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European Journal of Neurology 2012, 19 (Suppl.

1), 90457

Poster Session 1, Sunday 9 September

Ageing and dementia 1


P1002
P1001 Added diagnostic value of 11C-PiB-PET in
Surrogate decision and advance memory clinic patients with uncertain
directives authorization to diagnosis
institutionalization and spontaneous K.S. Frederiksen1, S.G. Hasselbalch1,2, A.-M. Hejl1,
refusal of cardio-pulmonary resuscitation I. Law3, G. Waldemar1
Memory Disorders Research Group, Dept. of Neurology,
1
in dementia: a Brazilian perspective Neurobiology Research Unit, 3Nuclear Medicine and PET,
2
Y. Correa-Neto1,2, G. Rego2, R. Nunes2 Copenhagen University Hospital, Rigshospitalet,
1Neurology Unit, Federal University of Santa Catarina,
Copenhagen, Denmark
Florianopolis, Brazil, 2Bioethics, University of Porto,
Introduction: PIB-PET imaging measures beta-amyloid,
Portugal
and may be an important diagnostic tool. We examined the
Introduction: Institutionalization and cardio-pulmonary added value of PiB-PET in patients from a memory clinic
resuscitation (CPR) authorization are substantial end of life with uncertain clinical diagnosis with regards to diagnostic
decisions in demented patients with geographical variation. classification and confidence in diagnosis.
Objectives: To assess family caregivers and non-caregivers Methods: Patients who underwent a PiB-PET study as part
attitudes concerning institutionalization and cardio- of their diagnostic work-up were eligible for inclusion. The
pulmonary resuscitation authorization to patients and to standard diagnostic evaluation included physical and
them if hypothetically later demented. neurological examination, cognitive and functional
Patients and methods: Family caregivers of 40 consecutive assessment and a cranial CT or MRI. Additionally, most
patients with Alzheimers disease assisted at an outpatient patients had an 18F-FDG-PET and cerebrospinal fluid
private practice clinic in Florianpolis, Brazil, and 40 sampling. Based on anonymized case reports, three
subjects without demented family members (non-caregivers) experienced clinicians reached a consensus diagnosis and
were enrolled. After written informed consent, subjects rated confidence in diagnosis before and after disclosure of
received a questionnaire consisting of authorizations PiB-PET ratings.
requests to institutionalization and CPR of demented Results: 57 patients (Gender, f/m: 27/30; Age: mean 65.7
patients (surrogate decision) and to themselves (advanced years (range 44.2-82.6); MMSE: mean 24.4 (range 13-29))
directive) if they hypothetically became demented later in were included. 27 had a positive PiB scan. 16 patients were
life. initially given a clinical AD diagnosis (87.5% PiB positive).
Results: The lower institutionalization authorization rate 13 patients (23%) were diagnostically reclassified after
was observed on family caregivers early dementia stages PiB-PET results were disclosed and clinicians overall
surrogate decisions (3%) and the greater on same group late confidence in their diagnosis increased in 28 cases (49%)
stages advance directives (87.9%). Institutionalization and remained unchanged in 27 cases (47%). Moreover,
authorization was overall greater on late versus early stages there was an increase in the number of patients rated as
and on advanced directives versus surrogate decisions. No clear AD (0% to 31.6%) and clear non-AD (12.3% to
differences were observed between family caregivers and 45.6%).
non-caregivers. The lower spontaneous CPR refusal rate Conclusion: Amyloid imaging may have a significant
was observed on family caregivers early dementia stages impact on the diagnostic classification of patients with
surrogate decisions (3%) and the greater on same group late uncertain diagnosis in a memory clinic as indicated by the
stages advance directives (42.4%). Spontaneous CPR number of reclassifications. Increased confidence in
refusal rate was overall greater on late versus early stages, diagnosis may lead to more aggressive treatment.
on advanced directives versus surrogate decisions, and on
family caregivers versus non-caregivers (this only on early
stage dementia).
Conclusions: Dementia progression improves
institutionalization authorization and CPR refusal among
Brazilians. Deciding for others decrease institutionalization
authorizations and CPR refusals. Daily interaction with
demented improves CPR refusal rates.

90 2012 EFNS
Posters, Sunday 9 September 91

P1003 P1004
Cell therapy with NGF in patients with Subjective cognitive impairment: who are
Alzheimers disease: changes in CSF these individuals? A review of patients
cholinergic markers receiving a diagnosis of subjective
A. Karami1, H. Eyjlfsdttir1,2, S. Vijayaraghavan1, cognitive complaints in ordinary clinical
A. Kadir1, B. Linderoth3,4, G. Lind3,4, N. Andreasen1,2, practice
A. Wall5, . Seiger1,6, P. Almqvist3,4, L. Wahlberg1,7, S. Garcia-Ptacek1,2, L. Cavallin3,4, I. Kreholt5,
A. Nordberg1,2, T. Darreh-Shori1, M. Eriksdotter1,2 M. Kramberger1,6, B. Winblad1,7, V. Jelic1,7,
1Dept Neurobiology, Caring Sciences and Society,
M. Eriksdotter1,7
Karolinska Institutet, 2Dept Geriatric Medicine, 3Dept 1Department of Neurobiology, Care Sciences and Society,
Clinical Neuroscience, Karolinska Institutet, 4Dept Karolinska Institutet, Stockholm, Sweden, 2Department of
Neurosurgery, Karolinska University Hospital, Stockholm,
5Dept of Radiology, Oncology and Radiation Sciences,
Neurology, Hospital Clnico San Carlos, Madrid, Spain,
3Department of Clinical Science, Intervention and
Uppsala, 6Stockholms Sjukhem, Stockholm, Sweden, 7NsGene Technology, Karolinska Institutet, 4Department of Radiology,
A/S, Ballerup, Denmark Karolinska University Hospital-Karolinska Institutet,
Background: Nerve growth factor (NGF) improves Stockholm University, 5Aging Research Center, Karolinska
cholinergic functions, important for cognition. Recently we Institutet and Stockholm University, Stockholm, Sweden,
have demonstrated that the acetylcholine synthesizing 6Department of Neurology, University Medical Centre

enzyme, choline acetyltransferase (ChAT) is present in Ljubljana, Slovenia, 7Department of Geriatric Medicine,
human cerebrospinal fluid (CSF). Karolinska University Hospital-Karolinska Institutet,
Objectives: To investigate whether NGF treatment affects Stockholm University, Stockholm, Sweden
the levels of cholinergic markers, ChAT and Background: Subjective cognitive impairment (SCI),
acetylcholinesterase (AChE) in CSF of patients with defined as cognitive complaints with normal cognitive
Alzheimers disease (AD). testing, is common in clinical practice. Longitudinal studies
Patients and methods: Samples from 6 AD patients were demonstrate increased risk of decline. We analyze this
included. The patients had received treatment with groups characteristics at the time of diagnosis.
encapsulated NGF releasing cells, implanted bilaterally in Patients and methods: SCIs diagnosed at Karolinska
the lateral basal forebrain in all 6 patients, and also in the Memory Clinic (2007-2009). Review of epidemiological,
medial basal forebrain in 3 of the patients. The levels of clinical and paraclinical data, including CSF analysis and
ChAT and AChE in CSF were determined by colorimetric temporal atrophy staging by a blinded rater. SCI, MCI and
assays prior to and after 3 and 12 months of NGF treatment. AD group means were compared with logistic regression.
Results: CSF AChE activity was increased by 18% and by Results: 453 SCI patients were identified. SCIs were
37% after 3 and 12 months of NGF treatment. Patients with significantly younger (57.7 years) than the MCI (64.2 years)
double implants had a 31-61% increase, while patients with and AD (70.1 years) groups. 64.2% were women. SCIs had
single implants had a 5-13% non-significant increase. more years of education and were more likely to have family
Patients with no significant cognitive decline over 12 history of dementia than AD or MCI patients. SCIs scored
months (responders) showed a 25% significant increase in higher on MMSE (28.4), RAVLT delayed retention and Rey
CSF ChAT activity. The changes in CSF ChAT activity visual memory than the other groups. 5.7% of SCI were
showed high correlations with cognition, as assessed by homozygote for ApoE4 allele, lower than AD (28.4%).
MMSE (r=0.79, p<0.014) and ADAS-Cog (r=-0.82, Blinded medial temporal lobe score for SCI was 1.0, lower
p<0.009), as well as with nicotine-binding (r=0.941, than MCI or AD. SCIs had higher beta-amyloid (903.7),
p<0.0003) and glucose metabolism (r=0.81, p<0.010), lower total Tau (245.1), lower p-tau (49.5) and were less
assessed by positron emission tomography. likely to have confluent white matter lesions, hypertension,
Conclusion: NGF cell therapy showed increases in the heart disease or treatment with acetyl-salicylic acid. Cornell
cholinergic markers in CSF, suggestive of a positive score of depression was higher in SCI than AD.
treatment outcome, but these findings warrant further Conclusions: SCI is a distinct group. SCIs are more likely
investigation in a larger patient sample. to be younger, have family history of dementia, and less
likely to be homozygote ApoE4 carriers and have a
cardiovascular risk profile than others. Follow-up is
essential to investigate long-term outcome of their cognitive
function.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


92 Posters, Sunday 9 September

P1005 P1006
Genetic risk score predicting accelerated Effect of Ginkgo biloba on cognitive
progression from mild cognitive function of elderly adults over a 20-year
impairment to Alzheimers disease period
E. Rodrguez-Rodrguez1,2,3, P. Sanchez-Juan1,2,3, H. Amieva, C. Meillon, J.F. Dartigues
J.L. Vazquez-Higuera1,2,3, I. Mateo1,2,3, J. Infante1,2,3, INSERM 897 Epidemiology and Biostatistics, University of
D. Alcolea4,5, S. Cervantes6,7, J. Clarimn4,5, Bordeaux, France
P. Martinez-Lage8, A. Lle4,5, P. Pastor6,7, Introduction: The study assesses the effect of Ginkgo
O. Combarros1,2,3 biloba extract (EGb761) on cognitive function of elderly
1Neurology, Hospital Universitario Marqus de Valdecilla, adults over a 20-year period.
2CIBERNED, 3IFIMAV, Santander, 4Neurology, Hospital Sant Methods: The data are gathered from the prospective
Pau, 5CIBERNED, Barcelona, 6Neurology, Clnica community-based cohort study Paquid. Within the study
Universitaria de Navarra, 7CIBERNED, Pamplona, sample (n=3612), three groups were compared: 589 subjects
8Neurology, Fundacin CITA-Alzheimer, San Sebastian,
reporting use of EGb761 at one of the ten assessment
Spain visits, 149 subjects reporting use of another nootropic drug
Objective: The genetic factors that influence progression of (piracetam) at one of the assessment visits and 2874 subjects
mild cognitive impairment (MCI) to AD remain largely were not reporting use of either EGb761 or piracetam.
unknown. We assessed whether a genetic risk score (GRS), Decline on the MMSE, IST (verbal fluency) and BVRT
based on 8 SNPs previously associated with AD risk in (visual memory) over the 20-year follow-up was analysed
GWA studies, is associated with either risk of conversion or with a mixed linear effects model accounting for numerous
with rapid progression from MCI to AD. confounding variables.
Methods: Among 288 subjects with MCI, follow-up (mean Results: A significant difference in the MMSE score
26.3 months) identified 118 MCI-converters to AD and 170 decline over the 20-year follow-up was observed in the
MCI-non-converters. We genotyped ABCA7rs3764650, EGb761 and piracetam treatment groups compared to the
BIN1rs744373, CD2APrs9296559, CLUrs1113600, neither treatment group in an opposite direction: EGb761
CR1rs1408077, MS4A4Ers670139, MS4A6Ars610932, group declined less rapidly than the neither treatment
and PICALMrs3851179. We calculated for each subject a group (clinically relevant difference of 5 points over the 20
cumulative GRS, defined as the number of AD-associated years), whereas piracetam group declined more. Regarding
risk alleles from our list of 8 SNPs (0-16), with each allele the IST and the BVRT, no difference was observed between
unit multiplied by the OR for that allele as described in the EGb761 group and the neither treatment group,
AlzGene database. Estimates of association between GRS whereas the piracetam group declined more. When
divided into tertiles and rapidity of conversion from MCI to comparing the EGb761 and piracetam groups directly, a
AD were calculated with logistic and Cox regression significantly different decline was observed for the 3 tests.
models. Conclusion: MMSE decline in a non-demented elderly
Results: GRS was not associated with risk of conversion to population is lower in subjects who reported using EGb761
AD. MCI-converters harbouring 6 or more risk alleles than in those who did not. This effect may be a specific
progressed 2-fold more rapidly to AD when compared with medication effect of EGb761, since it is not observed for
those with less than 6 risk alleles. In fact, both MCI- another nootropic medication.
converters in the second GRS tertile (mean GRS=6.9;
HazardRatio=1.89,95% CI=1.01-3.56,p=0.047) and in the
third GRS tertile (mean GRS=9.9; HR=2.06,95% CI=1.07-
3.98, p=0.031) showed a faster progression when compared
with those in the first tertile (mean GRS=4.6).
Conclusion: The combination of 8 validated non-APOE
risk SNPs for AD aggregated into a GRS predicted
accelerated progression to AD. The GRS calculation is a
first step towards development of prediction models that
incorporate aggregate genetic factors.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 93

P1007 P1008
Diffusion tensor changes according to Altered caudate morphology in
age-at-onset and apolipoprotein E leukoaraiosis is associated with motor
genotype in Alzheimers disease dysfunction in a subset of LADIS
M.-J. Kim1, S.W. Seo2, S.T. Kim3, J.-M. Lee4, D.L. Na2 (leukoaraiosis and disability in the elderly
1Department of Neurology, Seoul National University
study)
Hospital Healthcare System Gangnam Center, 2Department
M.D. Macfarlane1, M. Walterfang2, J.C.L. Looi1,3,
of Neurology, Samsung Medical Center, Sungkyunkwan
G. Spulber3,4, D. Velakoulis2, L.-O. Wahlund3,
University School of Medicine, 3Department of Radiology,
Samsung Medical Center, Sungkyunkwan University School
LADIS Study Group
1Academic Unit of Psychological Medicine, Australian
of Medicine, 4Department of Biomedical Engineering,
National University Medical School, Canberra, ACT,
Hanyang University, Seoul, Republic of Korea 2Melbourne Neuropsychiatry Centre, Melbourne University,
Introduction: Age-at-onset is one of the most important Melbourne, VIC, Australia, 3Department of Neurobiology,
factors that affect clinical course of Alzheimers disease Caring Sciences and Society, Division of Clinical Geriatrics,
(AD), and apolipoprotein E (apoE) genotype may also play Karolinska Institute, Stockholm, Sweden, 4Institute of
an important role in determining the clinical phenotype. We Clinical Medicine, Unit of Neurology, University of Eastern
aimed to investigate the change of white matter integrity Finland, Kuopio, Finland
according to age-at-onset and apoE genotype in AD using Background: Leukoaraiosis is a common finding on MRI
diffusion tensor imaging (DTI). brain scans of the elderly and is associated with cognition,
Methods: DTI was obtained in 72 patients with AD and 66 falls and disability. Frontostriatal neural circuits are
subjects with normal cognition (NC), and apoE genotype hypothesised to be involved, with specific areas of striatal
was available in 48 patients with AD. Multiple regression structures linked to specific cognitive and motor circuits.
analysis was conducted including the interaction of age-at- Objectives: To determine whether morphology of
onset (or current age in the NC group) and group difference neostriatal structures, as components of frontostriatal
as one of the independent variables, and fractional circuits, are correlated with severity of leukoaraiosis and
anisotropy (FA) and mean diffusivity (MD) values as the motor dysfunction.
dependent variables in all subjects. In order to assess the Methods: A representative subset of LADIS (n=66) was
effect of apoE, 48 AD patients whose apoE genotypes were analysed. Shapes of caudate nuclei and putamina were
available were divided into two groups according to e4 determined through standardised manual tracing methods
allele positivity, and each group was analyzed with the NC on MRI brain scans. We assessed relationships between
group using a similar method as above. leukoaraiosis and striatal volume differences, as well as
Results: Without consideration of apoE genotype, younger motor function, measured using the Short Physical
age-at-onset was associated with lower FA and higher MD Performance Battery (SPPB). Analysis was controlled for
in the cingulum and fronto-temporo-parietal association age and intracranial volume (ICV). Shape analysis was
fibres. When the data were analyzed individually in e4 performed using the UNC Spherical Harmonic (SPHARM)
carriers and non-carriers, the carriers showed correlation tools, correcting for age and ICV, for correlations of striatal
between younger age-at-onset and both of the cingulum and shape with SPPB.
association fibres while the non-carriers showed correlation Findings: There was no association between severity of
with only association fibres. leukoaraiosis (Fazekas score) and volumes of striatal
Conclusion: Our results suggest that characteristic structures via ANOVA. On linear regression, smaller
topographical distribution of pathological changes in volumes of the left caudate were associated with lower
patients with AD is determined by not only age-at-onset but SPPB (Beta 0.442, p=0.005). There was no association
also by apoE genotype. between SPPB scores and putaminal volumes. Inward
deformation of left caudate shape was found, in regions
corresponding to the inputs of the dorsolateral prefrontal,
premotor and motor cortex, correlating with SPPB
(p=0.0017).
Conclusions: Deficits in motor functioning seen in patients
with leukoaraiosis are associated with altered morphology
of the left caudate, a potential substrate of frontostriatal
circuit dysfunction and clinical biomarker.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


94 Posters, Sunday 9 September

P1009 P1010
Dementia drug treatment in clinical Exploration of a composite nano-medical
practice: data on 7570 patients from device for the delivery of neuroprotectant-
SveDem, the Swedish Dementia Registry nanocarriers into targeted neuronal cells
D. Religa1,2, K. Johnell3, M. Eriksdotter1 of Alzheimers disease
1NVS, Karolinska Institutet, Stockholm, Sweden, M.S. Mufamadi1, Y.E. Choonara1, L.C. duToit1,
2Mossakowski Medical Research Centre, Polish Academy of G. Modi2, D. Naidoo3, P. Kumar1, V.M.K. Ndesendo4,
Science, Warszawa, Poland, 3Aging Research Center, L. Meyer5, S.E. Iyuke6, V. Pillay1
Karolinska Institutet and Stockholm University, Stockholm, 1Department of Pharmacy and Pharmacology, 2Department
Sweden of Neurology, 3Department of Neurosurgery, University of
Background: In Alzheimers disease (AD), cholinesterase the Witwatersrand, Johannesburg, South Africa, 4School of
inhibitors and NMDA receptor antagonists, such as Pharmacy and Pharmaceutical Sciences, St. Johns
memantine, are used. Psychotropics are also used for University of Tanzania, Dodoma, Tanzania, 5Central Animal
treatment of neuropsychiatric symptoms. The aim was to Services, 6School of Chemical and Metallurgical
study whether there are differences between dementia Engineering, University of the Witwatersrand, Johannesburg,
disorders in the use of anti-dementia drugs and South Africa
antipsychotics (neuroleptics) in a large population of Purpose: The purpose of this study was to explore an
dementia patients. implantable composite nano-medical device that is capable
Methods: Information about dementia disorders was of prolonged delivery of the neuroprotectant-nanocarrier
obtained from the national Swedish Dementia Registry into targeted neuronal cells of Alzheimers disease (AD).
(SveDem) (n=7,570). Multivariate logistic regression Method: The device was fabricated by embedding
analysis was performed to investigate the association galantamine-loaded/flourescein-isothiocyanate (FITC)-
between dementia disorders and the use of anti-dementia labelled functionalized nanoliposomes [f(x)NLPs]
drugs and antipsychotics, after adjustment for age, sex, [phosphocholine(DSPC), cholesterol, ethanolamine (DSPE-
residential setting, living alone, MMSE score and number mPEG2000)] with synthetic peptide [KVLFLS, KVLFLT
of other drugs (used as a proxy for overall co-morbidity). and KVLFLM] within a neuro-compliant scaffold
Results: More than 80% of the AD and 86% of dementia (glutaraldehyde crosslinked chitosan, Eudragit-RSPO, and
with Lewy bodies (LBD) patients used anti-dementia drugs. polyvinylalcohol or polyethyleneoxide) followed by freeze-
Women were more likely than men to be treated with drying. Drug release from drug-loaded f(x)NLPs was
cholinesterase inhibitors. A higher MMSE score was performed using an orbital shaker-bath (50days) and at
positively associated with use of cholinesterase inhibitors, predetermined time intervals samples were drawn and
but negatively associated with NMDA receptor antagonists analyzed via UV (max=288nm). PC12-cells were seeded
and antipsychotics. The use of antipsychotics in all dementia on the device, ensued by air-drying/freeze-drying. Cells on
patients was 6%, but in LBD, the use of antipsychotics was the device were examined under SEM and stereomicroscopy.
significantly higher (16%), with an adjusted odds ratio of LDH release and antipoliferation were assayed using the
4.2 compared to AD patients. CytoTox-96 Non-Radioactive and CytoTox-Glu
Conclusions: The use of anti-dementia drugs in AD was in Cytotoxicity assays, respectively, thereafter Lactate
agreement with Swedish guidelines. The use of Dehydrogenase (LDH)/dead cells was quantified employing
antipsychotics in patients with LBD was high. LBD patients plate reader. At pre-determined time intervals cell uptake
suffer from psychotic symptoms early in the disease, but the was analyzed for 30 days employing plate reader and
high use of antipsychotics is worrying, taking into CLSM.
consideration side-effects of antipsychotics. Results: In vitro study demonstrated that prolonged release
of f(x)NLPs (60-100%) from the device over a period of 50
days. SEM and stereomicroscope results demonstrated that
the device was suitable for neuronal cell attachment and
proliferation. Ex vivo results exhibit less than 25% LDH
release which validate that the designed device did not
significantly induce cell injury. CSLM and plate-reader
studies revealed that f(x)NLPs facilitated the drug uptake
into PC12-cells via LDL receptor family.
Conclusions: The results revealed that the designed device
had superior cytocompatibility and was suitable to fulfil
prolonged delivery of neuroprotectants-nanocarrier into
targeted neuronal cells of AD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 95

P1011 P1012
A multicentre randomized clinical trial of Olfaction in frontotemporal lobar
physical exercise in Alzheimers disease degeneration
(AD): rationale and design of the ADEX H. Magerova1, M. Vyhnalek1,2, J. Laczo1,2, R. Andel3,
study A. Kadlecova1,2, M. Bojar1, J. Hort1,2
1Neurology, Charles University, 2nd Faculty of Medicine and
S.G. Hasselbalch1, K. Hoffmann1, K.S. Frederiksen1,
Motol Hospital, Prague, 2International Clinical Research
N.A. Sobol2, N. Beyer2, A. Vogel1, A.H. Simonsen1,
Center, St. Annes University Hospital Brno, Czech Republic,
G. Waldemar1 3University of South Florida, School of Aging Studies,
1Memory Disorders Research Group, The Neuroscience
Tampa, FL, USA
Centre, Rigshospitalet, 2Institute of Sports Medicine,
Bispebjerg University Hospital, Copenhagen, Denmark Introduction: Olfactory impairment is well documented in
Alzheimers disease or Parkinsons disease. On the other
Background: Physical exercise has the potential to improve
hand, only limited data are available in frontotemporal lobar
cognition, psychological symptoms, physical performance
degeneration (FTLD), however the distribution of brain
and quality of life, but evidence is scarce. Previous trials
pathology in some FTLD subtypes suggest, that olfaction
have been short, often underpowered and involving home
might be impaired even in these disorders
based light exercise programs. Most have included nursing
Methods: Following FTLD subgroups were included in the
homes residents with severe undefined dementia. The aim
study: FTLD-behaviour variant (FTD-bv, n=7), semantic
of the ADEX study is to establish whether exercise can
dementia (SD, n=4) and progressive supranuclear palsy
improve symptoms and quality of life in patients with mild
(PSP, n=8). Smell identification was assessed using a test
to moderate AD.
developed at our memory clinic - the Motol Hospital Smell
Methods: The study is a multicentre, single-blind,
Test (MHST). Results were compared with the control
randomized clinical trial. We plan to recruit 192 patients
group (n=15). In our previous study, the MHST and UPSIT
aged 55-85 years with mild to moderate AD. The participants
results correlated with each other (r=0.68, p<0.001).
will be randomly allocated into two groups: An intervention
Results: The subjects were similar in age (0.889) and
group attending 16 weeks of continuously supervised
gender (0.357), but they differed in Mini-Mental State
moderate aerobic exercise 1 hour three times a week and a
Examination (MMSE) score (p=0.002). The one-way
control group only receiving usual care. Primary outcome
analysis of covariance adjusted for MMSE score showed
measure is change in cognitive performance (Symbol Digit
impaired smell identification in the FTLD-bv group
Modalities Test) and change in quality of life (Euro-Qol-
(p<0.009) and intact in SD (p<0.405) and PSP (p<0.105)
5D-5L). Secondary outcome measures include changes in
groups.
other cognitive functions, neuropsychiatric symptoms, and
Conclusion: We have confirmed the hypothesis that smell
ADL. Blood sampling will be performed in all subjects to
identification is impaired even in specific FTLD subtypes
examine effects on biomarkers. A subgroup of the patients
reflecting the localization of neurodegenerative process.
will undergo MRI, PiB-PET and CSF studies to investigate
The atrophy in FTLD-bv involves the orbitofrontal cortex
structural changes and -amyloid accumulation.
and basis of frontal lobe that represent a part of olfactory
Discussion: This is the first controlled large study to
pathway. In SD and PSP the neurodegeneration takes place
investigate the effects of continuously supervised moderate
out of structures important for olfactory processing.
aerobic exercise. Recruitment was started in January 2012
and results will be available in late 2013. Complex
interventions of this kind present unique challenges to study
design, but may provide evidence for non-pharmacological
treatments in AD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


96 Posters, Sunday 9 September

P1013 P1014
Localisation of attentional function in Pharmacological manipulation of
Lewy body dementia cholinergic pathways affects the duration
X. Kobeleva1,2, M. Firbank2, J.-P. Taylor2 of silent period after cortical magnetic
RWTH Aachen University Hospital, Aachen, Germany,
1
stimulation in Alzheimers disease
Institute for Ageing and Health, Newcastle General
2
C. Balla, J.L. Pepin, A. MaertensdeNoordhout
Hospital, Newcastle Upon Tyne, UK
CHR Citadelle, Lige, Belgium
Introduction: A core symptom of Lewy Body Dementia
When transcranial magnetic stimulation is applied during a
(LBD) is fluctuations in cognition and attention. However
sustained isometric contraction, the electromyographic
the aetiology of this symptom is poorly understood but may
activity ceases temporarily. This interval is called silent
relate to fronto-parietal network dysfunction. To address
period. It can be divided in two parts (SP1 and SP2) under
this issue we investigated the performance and brain
control of cortical and spinal mechanisms. As the literature
activation in LBD patients applying a modified ANT
showed changes in cortical excitability in AD, we
(attention network task) using an event-related functional
investigated if these changes are reflected in silent period
magnetic resonance (fMRI) paradigm.
and if they are under the control of cholinergic mechanisms.
Methods: 14 LBD patients and 14 age-matched healthy
The duration of silent period was studied in 11 de novo AD
controls (HC) underwent an assessment of global cognitive
patients before and after two months of treatment with
function before performing the ANT in the scanner.
10mg donepezil. As control group, we used 11 age-matched
Reaction times (RT) and error rates were compared between
normal subjects. In the control group, the mean duration of
groups. Imaging data were submitted to a GLM analysis
SP1 (SD) was 77.23ms (27.5ms). In the AD group before
using SPM (Statistical Parametric Mapping) to detect
treatment it was 103.05ms (48.7ms). In the AD group after
within and between group effects.
treatment, the mean duration of SP1 was 72.19ms
Results: The LBD group was significantly slower in RT
(28.5ms). The difference is significant between control
performance (p<0.001) and had higher error rates (p=0.02).
and AD before treatment. It is also significant between AD
However, SPM(t) analysis revealed comparable fronto-
before and AD after treatment. There is no significant
parietal network activations to the executive component of
difference between control group and AD after treatment.
the ANT between controls and LBD patients even in
For SP2, no difference was found between AD and controls
uncorrected analyses (p<0.001) and this observation held
and between AD before and after treatment.
true when co-varying for RT. In contrast using a region of
In conclusion, silent period in its first component increases
interest analysis there was an over-activation of the anterior
significantly in AD patients. Donepezil reduces the duration
cingulate and infraparietal lobule with a delay in the
of silent period (SP1 component). Functional mechanisms
hemodynamic response.
modulate probably the motor excitability in AD, at a spinal
Discussion: These findings suggest a spatially intact
or a cortical level, possibly through descending cholinergic
network with no evidence of topologically specific deficits
pathways.
in LBD. The data rather point towards poor efficiency in
neural networks associated with executive dysfunction and
increased cortical activation, which might be interpreted as
an ineffective compensation mechanism. Comparison to
other disease groups (Alzheimers) is warranted.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 97

P1015 P1016
Hippocampal GABAergic interneurons are Encapsulated cell biodelivery of nerve
highly vulnerable in Alzheimers disease growth factor in Alzheimers disease
E. Sanchez-Mejias1, V. Navarro2, R. Sanchez-Varo1, M. Eriksdotter1,2, G. Lind3,4, H. Eyjolfsdottir1,2,
L. Trujillo-Estrada1, M. Aneiros1, S. Jimenez2, B. Linderoth3,4, P. Almqvist3,4, . Seiger1, L. Wahlberg1,5
V. De Castro1, M.L. Vizuete2, J.C. Davila1, J. Vitorica2, 1Dept Neurobiology, Caring Sciences and Society,

A. Gutierrez1 Karolinska Institute, 2Dept Geriatric Medicine, Karolinska


1 Cell Biology, University of Malaga/CIBERNED, Mlaga, University Hospital, 3Dept Clinical Neuroscience, Karolinska
2 University of Seville/CIBERNED, Seville, Spain Institute, 4Dept Neurosurgery, Karolinska University
Introduction: Alzheimers disease (AD) is characterized by Hospital, Stockholm, Sweden, 5NsGene A/S, Ballerup,
typical neuropathological changes including extracellular Denmark
amyloid-beta (Abeta) deposition and intracellular Background: Degeneration of cholinergic neurons in the
neurofibrillary tangles. According to the amyloid cascade basal forebrain correlates with cognitive decline in
hypothesis, accumulation and deposition of Abeta in the Alzheimers disease (AD). These neurons depend on nerve
brain are the major events in the pathology of AD. Specific growth factor (NGF) for survival. Local delivery of NGF
neuronal networks are preferentially affected by Abeta has emerged as a potential AD therapy due to its regenerative
pathology during disease progression. Among these the effects on the forebrain cholinergic neurons. We have
distinct subpopulations of hippocampal inhibitory developed a cell therapy technique where encapsulated cells
GABAergic interneurons have been shown to be highly release NGF to the basal forebrain with the aim to halt the
vulnerable. In this sense, we have previously reported a degeneration of cholinergic neurons in AD.
substantial loss of somatostatin (SOM) containing Methods: Patients with AD have been implanted with
interneurons in the hippocampus of APP-based transgenic encapsulated NGF producing cells into the basal forebrain
mouse models, with a linear correlation with the Abeta bilaterally using stereotactic neurosurgery. The catheter-like
content and glial activation. device consists of an NGF producing, genetically engineered
Methods: We have analyzed the expression of SOM, human cell line encapsulated behind a semipermeable fibre
neuropeptide Y (NPY), parvalbumin (PV) and membrane allowing efflux of NGF. The device can easily be
cholecystokinin (CCK) as markers of the major inhibitory removed. Patients are monitored regarding safety,
populations in human hippocampus. Furthermore, we also tolerability, cognitive and biological parameters.
quantified the hippocampal area occupied by extracellular Results: 10 patients have hitherto successfully received
Abeta immunoreactivity (plaque load) and the levels of bilateral implants. The procedure in the first 6 patients was
different Abeta forms. For this purpose, RT-PCR, Western safe, well tolerated and positive effects on cognition and
blots and immunostaining were performed in non-demented nicotine binding were found in 2 patients. However, since
controls and clinically diagnosed mild (Braak II) to severe the encapsulated cells, which were successfully removed
(Braak V-VI) AD cases. after 12 months, showed low NGF release, effort to improve
Results: Results showed a significant declined expression the release has been made. Recently, 4 additional AD
of SOM, NPY and, also PV, in severely affected AD patients patients were implanted with encapsulated cells that release
compared to the mild and control groups. 5-10 times more NGF/day.
Conclusion: SOM neuropeptide regulates the metabolism Conclusions: The first steps towards a possible therapy for
of Abeta through modulating proteolytic degradation AD based on local delivery of NGF to the cholinergic
catalyzed by neprilysin. Thus, the high cellular vulnerability neurons in the basal forebrain have been taken. The initial
of these GABAergic interneurons may have substantial results of this novel cell therapy approach will be discussed.
functional repercussions on Abeta accumulation during
disease progression as well as on hippocampal inhibitory
networks and memory processes.
FIS-PS09/00099(AG),FIS-PS09/000151(JV),CTS-
4795(JV).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


98 Posters, Sunday 9 September

P1017 P1018
The determination of aggregated tau in Plasma amyloid-beta and serum amyloid-
solution and its potential as a tool for the beta auto-antibody levels in patients with
diagnosis of tauopathies Alzheimers disease
S.T. ODowd1,2, P. Johansson3,4, M.T. Ardah5, L. Zhou1, L.W. Chu2, J.S.C. Kwan1, K.S.L. Lam1,
K.A. Roberts2, G. Cummins2, O. ElAgnaf5, O.Y. Cheng1, K.H. Chan3
J. Svensson4,6, H. Zetterberg7, T. Lynch1,2, D.M. Walsh1,8 1University of Hong Kong, 2Medicine, University of Hong
1Laboratory for Neurodegenerative Research, Conway Kong, 3Medicine, Research Center of Heart, Brain, Hormone
Institute of Biomolecular and Biomedical Research, and Healthy Aging, University of Hong Kong, Hong Kong
University College, 2Dublin Neurological Institute at the S.A.R.
Mater Misericordiae University Hospital, Dublin, Ireland, Introduction: Amyloid beta (A), especially A oligomers,
3Department of Neuropsychiatry, Skaraborg Hospital,
is important in Alzheimers disease (AD) pathogenesis.
Falkoping, 4Dept. of Endocrinology, The Sahlgrenska Aim: Study plasma A40, A42, A oligomers, serum A
Academy at University of Gothenburg, Sweden, 5Dept. of monomer and oligomers antibodies levels in AD patients,
Biochemistry, Faculty of Medicine and Health Sciences, and their correlation with cognitive functions.
United Arab Emirates University, Al Ain, United Arab Patients and methods: 44 AD patients and 22 non-
Emirates, 6Dept. of Endocrinology, Skaraborg Hospital, demented controls were studied. Cognitive functions were
Skovde, 7Dept. of Psychiatry and Neurochemistry, Institute of
assessed by Chinese version of mini-mental state
Neuroscience and Physiology, The Sahlgrenska Academy at
examination (MMSE), Abbreviated Metal Test (AMT),
University of Gothenburg, Sweden, 8Harvard Medical School
Alzheimers Disease Assessment Scale Cognitive Subscale
Center for Neurologic Diseases, Brigham and Womens
(ADAS-cog). A and A antibodies levels were measured
Hospital, Boston, MA, USA
by ELISA.
Introduction: Burgeoning evidence suggests soluble tau Results: Median plasma A40 and A42 levels were similar
assemblies may mediate neurotoxicity, but no specific assay between AD and controls. In women with AD, plasma A42
exists for their detection. The Innogenetics ELISA is the level was positively correlated with cognitive functions.
leading commercial assay in analysis of cerebrospinal fluid Plasma A oligomers level was higher in AD than in
(CSF) tau, but its ability to detect different aggregation controls (642.54ng/ml [range 103.33-2676.93] versus
states is undetermined. 444.18ng/ml [range 150.19-1311.18], p=0.047), and
Methods: Recombinant isoforms of human tau, and a negatively correlated with cognition. Serum A monomer
monoclonal antibody capable of their detection, were antibodies level was similar between AD and controls, but
expressed, purified and characterised, and tau aggregated in in AD patients, serum A monomer antibodies level was
vitro. A novel ELISA was devised and optimised for negatively correlated with cognition. Serum A oligomers
detection of aggregated tau. The ability of the Innogenetics Ab level was higher in AD than in controls (42.81ng/ml
platform to detect different aggregation states of versus 24.15ng/ml, p=0.014) without significant correlation
recombinant tau was interrogated and total-tau and phospho- with cognition.
tau dosage in CSF from 53 subjects (neurodegenerative Conclusion: Plasma A oligomers and serum A oligomers
disorders and controls) determined. antibodies levels facilitate AD diagnosis. Plasma A42 and
Results: Aggregated recombinant tau was detected A oligomers levels may reflect AD severity.
sensitively and specifically by the novel ELISA. The
Innogenetics assay reliably detected aggregated and
monomeric tau and evinced good recovery of both species
when spiked into human CSF. Total-tau levels were
significantly higher in AD than in controls (p<0.001) or in
patients with other neurodegenerative diseases (p<0.01).
Elevated phospho-tau levels similarly discriminated AD
from controls and other neurodegenerative diseases
(p<0.01).
Discussion: This is the first report of the capability of the
Innogenetics assay to detect different aggregation forms of
CSF tau. We also report total-tau and phospho-tau levels in
a unique cohort of patients with neurodegenerative diseases,
highlighting the variation in tau levels between groups,
despite comparable extents of neurodegeneration. This
suggests that tau dosage in CSF reflects more than merely
burden of neuronal death and suggests mechanistic
differences underlying the tauopathies.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 99

P1019 P1020
Nutritional intervention with Fortasyn Psychometric properties of DQoL in
Connect: beneficial effects in patients with Alzheimers disease and
experimental models of Alzheimers carers
pathology and functional decline M. Gomez-Gallego1, J. Gomez-Garcia2
1Catholic University of Murcia, 2University of Murcia,
N. vanWijk1, M.C. deWilde1, A.A.M. Kuipers1,
M. Balvers1, J. Sijben1, P.J. Kamphuis1,2, H. Koivisto3,4, Murcia, Spain
H. Tanila3,4, D. Jansen5,6, V. Zerbi5,6, A.J. Kiliaan5,6, Introduction: Quality of life is an important outcome
L.M. Broersen1 measure in the assessment of the interventions with
1Nutricia Advanced Medical Nutrition, Danone Research - Alzheimers disease (AD) patients. DQoL is a self-rated
Centre for Specialised Nutrition, Wageningen, 2Utrecht scale that covers the domains of sense of aesthetics, feelings
Institute for Pharmaceutical Sciences (UIPS), Utrecht of belonging, negative effect, positive affect and self-
University, Utrecht, The Netherlands, 3A. I. Virtanen esteem. The aim of this study is to determine the
Institute, University of Eastern Finland, 4Neurology, Kuopio psychometric properties of the Spanish version of DQoL.
University Hospital, Kuopio, Finland, 5Anatomy, 6Cognitive Methods: A sample of 102 AD patients and their 102 carers
Neuroscience, Donders Centre for Neuroscience, RUNMC, were administered DQoL. Internal consistency of both the
Nijmegen, The Netherlands whole scale and the subscales was assessed using alpha
Introduction: The neurotoxic cascade of Abeta initiates at coefficient. Agreement was assessed using the concordance
neuronal membranes and soluble oligomers are thought to correlation coefficient (CCC). Convergent and divergent
disturb membrane properties by binding to membrane validity were assessed by testing the relation between DQoL
components. As such, the precise composition of neuronal scores and both demographic factors and the scores of
membranes influences membrane disrupting properties of Minimental State Examination, Neuropsychiatric Inventory,
Abeta. Moreover, membrane composition directly Geriatric Depression Scale (GDS), Barthel Index, and Zarit
influences APP-processing and the generation of Abeta. The Burden Inventory. Factor analysis was used to test construct
hypothesis that nutritional interventions that positively validity.
affect membrane formation and composition will reduce Results: The internal consistency of DQoL was excellent
Abeta production, toxicity and pathology was tested in a for both patients and carers ratings. However, this was not
series of experiments using rodent models of AD. the case for some subscales (alpha< 0.7). The patient-carer
Methods: Fortasyn Connect (FC) is a nutritional agreement was good (CCC=0.877; accuracy=0.981;
composition containing precursors and cofactors for precision=0.893). Patients and carers DQoL ratings
neuronal membrane synthesis, viz. DHA, EPA, UMP, correlated significantly with GDS scores but were not
choline, folate, vit.B12, vit.B6, phospholipids, vit.C, vit.E, associated with the other clinical and demographic
and selenium. We assessed the effects of dietary enrichment measures. The factorial analyses showed that all 29 items
with FC in the Abeta infusion model, and the APP/PS1 loaded on component 1.
mouse model. Conclusions: Our results indicate that DQoL functions as a
Results: In the Abeta infusion model, dietary FC protected scale with good internal consistency and inter-rater
cholinergic neurons from Abeta-induced toxicity as reliability. The existence of subscales is not clear. DQoL
evidenced by preserved immunoreactivity for the does not relate to other constructs but depression, so it could
membrane-bound enzymes ChAT and VAChT. FC also really measure perceived psychological well-being.
prevented the Abeta-induced reduction in exploratory
activity. In young APP/PS1 mice, FC decreased brain Abeta
levels, amyloid plaque burden in the hippocampus, and
neurodegeneration in the neocortex. In aged APP/PS1mice,
FC normalized hippocampal levels of the neuronal marker
N-acetylaspartate. Additionally, FC alleviated poor spatial
learning in aged mice.
Conclusion: FC intervention positively influenced the
outcome of membrane-bound processes in different rodent
models of AD. These effects on AD-like pathology and
behaviour in AD models warrant further evaluation in AD
patients. Fortasyn is a trademark of N.V. Nutricia.
Funded by EU FP7-Food project LipiDiDiet
(Grant_211696).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


100 Posters, Sunday 9 September

P1021 P1022
Borrelia Burgdorferi: risk factor in Cognitive-enhancing activity of thymol
Alzheimers disease and carvacrol in two rat models of
R.P.W. Marquard1, A. Kurz2 dementia
1Psychiatry and Neurology, Klinikum Taufkirchen,
N. Majlessi, Z. Azizi, S. Ebrahimi, E. Saadatfar
Taufkirchen, 2Psychiatrie, TU Mnchen, Germany Department of Physiology and Pharmacology, Pasteur
Introduction: Inflammatory factors are presumably Institute of Iran, Tehran, Iran
involved in Alzheimers disease (AD). We examined if AD Introduction: Alzheimers disease (AD) is known to be the
is related with non-symptomatic borreliosis. most common cause of dementia in elderly patients. The
Method: 100 patients (mean age: 72.9 years) met NINCDS- limitations of current AD therapeutics have prompted
ADRDA diagnostic criteria for probable AD after a investigation into innovative therapeutics over the last two
thorough diagnostic evaluation which comprised a decades. This study evaluated the efficacy of thymol and
neuropsychological exam, blood tests and CT or MRI brain carvacrol against cognitive deficits induced by amyloid
imaging. The age and gender matched control group (72.8 (A) or scopolamine.
years) scored 28 or more points on the Mini Mental State Methods: Rats received bilateral intrahippocampal
and had no family history of dementia. All patients and injections of A (25-35) or intraperitoneal injections of
controls were Caucasian. Lyme ELISA and Western blot scopolamine, and the effect of different doses of thymol, or
test were used to examine serum samples of patients and carvacrol (0.5, 1, or 2mg/kg) on cognitive function was
controls and cerebrospinal fluid of patients with positive determined. Animals were subjected to 5 days of training in
blood test. the Morris water maze: 4 days with an invisible platform to
Results: 28 (28%) of the AD patients but only 8 (8%) of the test spatial learning and the 5th day with a visible platform
controls had Lyme Borreliosis antibodies (IgG) in the serum to test motivation and sensorimotor coordination. The acute
sample, indicating that individuals with Borrelia IgG toxicities of thymol and carvacrol were also studied.
antibodies have an increased risk suffering Alzheimers Results: The results showed increases in escape latency and
disease by 3.5. None of them had intrathecal antibody decreases in target quadrant entries in A or scopolamine-
production as examined in the CSF. treated groups. These impairments were reversed by pre-
Discussion: We found a significantly higher rate of Borrelia training administration of either thymol or carvacrol. The
IgG antibodies in individuals with Alzheimers disease. As calculated LD50s of thymol (565.7mg/kg) and carvacrol
several authors found similarities between the surface of (471.2mg/kg) were found to be much higher than their
Borrelia and neuronal tissue, cross-reactivity of B. therapeutic doses (thymol 0.5 mg/kg, carvacrol 1mg/kg).
burgdorferi antibodies with neuronal tissue or the triggering Conclusion: These findings provide preliminary positive
of a non-specific inflammatory response are possible. evidence for the effectiveness and safety of thymol and
Further research should include investigation of the carvacrol in alleviating cognitive impairments caused by
following questions: increased A levels or cholinergic hypofunction.
1. Is the frequency of Alzheimers disease in individuals Anticholinesterase, antioxidant, and anti-inflammatory
with history of non-symptomatic borrelia IgG antibodies activities may be the mechanisms contributing towards their
increased? beneficial effects in these models.
2. Is there any effect of early antibiotic treatment of Lyme
disease?

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 101

P1023 P1024
A preliminary study of the association Systemic lipid peroxidation in mild
between Cystatin C gene polymorphism cognitive impairment (MCI) and
and late-onset Alzheimers disease in a Alzheimers disease (AD)
sample from Iran S. Leoncini1, C. DeFelice2, C. Signorini1, A. Pecorelli1,
Z. Azizi1, M. Noroozian2, Z. Kaini-Moghaddam3, T. Durand3, J.-M. Galano3, C. Oger3, V. Bultel-Ponc3,
E. Saadatfar1, N. Majlessi1 A. Guy3, S. Boschi4, L. Ciccoli1, J. Hayek5
1Department of Physiology and Pharmacology, Pasteur 1Department of Pathophysiology, Experimental Medicine &

Institute of Iran, 2Tehran University of Medical Sciences, Public Health, University of Siena, 2Neonatal Intensive Care
3Department of Biotechnology, Pasteur Institute of Iran, Unit, University Hospital Azienda Ospedaliera Universitaria
Tehran, Iran Senese (AOUS), Siena, Italy, 3Institut des Biomolcules Max
Mousseron (IBMM) - UMR 5247 CNRS - UM I - UM II,
Introduction: Alzheimers disease (AD) is the most
Montpellier, France, 4Internal Medicine Unit, 5Child
common age-associated neurodegenerative disease caused
Neuropsychiatry Unit, University Hospital, AOUS, Siena,
by complicated interactions between genetic and
Italy
environmental factors. Structural genomic studies
demonstrated that more than 200 genes might be involved Introduction: Oxidative stress (OS) has been involved in
in AD pathogenesis regulating dysfunctional genetic the pathogenesis of Alzheimers disease (AD) and mild
networks leading to premature neuronal death. Most genes cognitive impairment (MCI). However, it is unclear whether
conferring susceptibility to AD are related to A deposition, OS is a primary or a secondary contributor to the MCI/AD
oxidative stress and inflammatory response. Recent studies phenotype, and whether OS markers in peripheral samples
have shown that the endogenous protein cystatin C binds represent reliable indicators for the presence of
soluble A and inhibits A oligomerization and neurodegenerative disease.
amyloidogenesis, protecting the brain against amyloid- Methods: The study included patients with AD (n=3) and
induced toxicity. Moreover, a decreased cystatin C secretion MCI (n=6). Healthy control subjects (n=8) comparable for
is associated with a polymorphism found in the cystatin C age were also included. Informed consent and Institutional
gene (CST3). Molecular epidemiological studies have Review Board were obtained. Plasma was used for
presented contradictory results concerning a potential role determination of free F2-Isoprostanes (F2-IsoPs),
of CST3 G73A polymorphism in AD. F2-dihomo-isoprostanes (F2-Dihomo-IsoPs),
Methods: In this study, to define a possible association of F4-neuroprostanes (F4-NeuroPs) and non-protein-bound
the G73A polymorphism in CST3 gene with late-onset AD iron (p-NPBI) and erythrocytes were used for determination
(LOAD) in an Iranian population, we conducted a case- of intra-erythrocyte NPBI (IE-NPBI). F2-IsoPs,
control study including a clinically well-defined group of F2-Dihomo-IsoPs, and F4-NeuroPs were measured by gas
61 LOAD patients and 63 age-matched controls. G73A chromatography/negative ion chemical ionization tandem
polymorphism was determined by polymerase chain mass spectrometry method (GC/NICI-MS/MS), while
reaction-restriction fragment length polymorphisms (PCR- IE-NPBI and p-NPBI were determined by HPLC.
PFLP) assay. Results: Levels of IE-NPBI [median (inter-quartile range)]
Results: Chi-square analysis showed a significantly in MCI [1.14 (0.45-1.92) nmol/ml erythrocyte susp.] and
increased number of individuals with the G/A genotype in AD [1.3 (0.92-2.35) nmol/ml] were significantly higher as
AD patients compared with controls (p<0.05). compared to controls [0.67 (0.55-0.75) ] (p=0.031), as well
Conclusions: The results obtained from our study as those of p-NPBI [MCI: 1(0.3-1.07)nmol/ml; AD:
demonstrate an association between G73A polymorphism 0.63(0.52-0.75)nmol/ml; controls: 0.25(0.1-0.4)nmol/ml]
and LOAD in an Iranian sample. However, as the level of (p=0.004). Plasma F2-IsoPs were significantly higher in
significance is low, the G/A genotype seems to have only MCI [53.4(44.4-162.8)pg/ml] and AD [85(54-96)pg/ml]
minor effects in the development of AD or probably interact compared to controls [15.35 (13.4-21.8)] (p=0.032). The
with other risk factors. combined group of MCI and AD patients showed
significantly higher levels of plasma F2-Dihomo-IsoPs than
controls [MCI+AD: 7.5(0-25.2)pg/ml; controls: 0.85(0.5-
0.96)]. No significant differences were observed for
F4-NeuroPs (p=0.257).
Conclusions: Systemic lipid peroxidation is detectable in
peripheral blood in MCI and AD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


102 Posters, Sunday 9 September

P1025 P1026
Physicomechanical transitions of an Development of novel lysosomal
implantable nano-enabled biorobotic modulators for treating protein
intracranial device for Alzheimers disease accumulation diseases
management for validation of in vivo M.L. Wisniewski1, D.J. Hoover2, J. Hwang1,
behaviour in a simulated brain K. Viswanathan2, D. Butler3, D. Wright2,4, B.A. Bahr1,2
1William C. Friday Laboratory, University of North Carolina
environment
Pembroke, NC, 2Synaptic Dynamics Inc., Farmington, 3Dept.
M.S. Mufamadi1, Y.E. Choonara1, L.C. duToit1, of Pharmaceutical Sciences, 4Medicinal Chemistry,
G. Modi2, D. Naidoo3, P. Kumar1, V.M.K. Ndesendo4, University of Connecticut, Storrs, CT, USA
L. Meyer5, S.E. Iyuke6, V. Pillay1
Lysosomes are the primary site for removal of old and
1Department of Pharmacy and Pharmacology, 2Department
of Neurology, 3Department of Neurosurgery, University of
misfolded proteins and to maintain cellular homeostasis,
the Witwatersrand, Johannesburg, South Africa, 4School of and positive lysosomal modulation has been shown to
Pharmacy and Pharmaceutical Sciences, St. Johns enhance protein clearance to protect against protein
University of Tanzania, Dodoma, Tanzania, 5Central Animal accumulation pathology. Small-molecule lysosomal
Services, 6School of Chemical and Metallurgical modulators, for instance Z-Phe-Ala-diazomethylketone
Engineering, University of the Witwatersrand, Johannesburg, (PADK), at appropriate concentration elicit marked
South Africa up-regulation of cathepsins and other lysosomal enzymes
without any indications of synaptic compromise,
Purpose: The purpose of this study was to evaluate the
behavioural abnormalities, or major organ malfunctions.
physicomechanical transitions of an implantable nano-
Recent work designed and synthesized compounds with the
enabled Biorobotic Intracranial Device (BICD) within
objective to improve potency, selectivity, and metabolic
simulated brain environment.
stability. We utilized the PADK structure as a departure
Methods: The BICD was fabricated by embedding
point to develop a non-peptidyl lead series. Initial structures
galantamine-loaded functionalized nanoliposomes [f(x)
(SD1001, SD1002, and SD1006) demonstrated that we
NLPs] [Phosphocholine (DSPC), cholesterol, ethanolamine
could remove the internal amide linkage, eliminate the
(DSPE-mPEG2000) with synthetic peptides] within a
highly reactive diazoketone moiety, and develop highly
neuro-compliant scaffold [glutaraldehyde crosslinked
flexible, enantioselective routes for first-in-class cathepsin
chitosan, eudragit RSPO, and polyvinyl alcohol or
modulators. The resultant lead series maintained or
polyethyleneoxide] followed by freeze-drying. Rheological,
improved the modulatory profile of PADK, providing
textual and SEM studies were employed to characterize the
protection in the hippocampal slice model of protein
BICD behaviour in vitro. Hydration and degradation studies
accumulation that exhibits experimentally-induced
were conducted within artificial cerebrospinal fluid (aFCS).
phosphorylated tau deposits, ubiquitinated inclusions, and
Images were acquired using a bench-top MRI over a period
synaptic compromise. Results from animal studies include
of 50 days. Labelled-f(x) NLPs distribution within the
selective lysosomal modulation, improved synaptic integrity
BICD was examined by micro-ultrasound imaging (Vevo
and cognition, without any indications of obvious adverse
2100) and CLSM.
effects amongst major organ tissue sections. The novel
Results: SEM images validated the porous surface
compounds will be used to further design efficacious
morphology of the scaffold, with pores that are relatively
modulators for protective agents that
uniform in size and shape. Rheological and textural
i) enhance cathepsin trafficking and protein clearance,
parameters demonstrated that the crosslinked scaffold was
ii) promote synapse function, and
more firm and resilient when compared with the non-
iii) slow the progression of proteinopathies.
crosslinked scaffold. MRI images showed complete scaffold
hydrated within 30min after submergence within aFCS,
however the hydrated scaffold exhibited swelling and slight
erosion. CLSM micrographs confirmed labelled f(x)NLPs
encapsulation and distribution within the BICD.
Fluorescence patterns postulate intact vesicles after BICD
lyophilization. Ultrasound images confirmed that more
even f(x)NLPs distribution was achieved in the slightly less
viscous rather than highly viscous BICD.
Conclusions: The results validate that evaluation of BICD
physicomechanical transitions is a useful tool for further
investigation of the optimized formulation designed for
prolonged delivery of the neuroprotectant-nanocarrier into
targeted neuronal cells of Alzheimers disease.

2012 EFNS European Journal of Neurology 19 (Suppl. X), 66343


Posters, Sunday 9 September 103

P1027 P1028
Myeloid microvesicles as potential Different cerebrospinal fluid biomarker
biomarkers of disease progression in patterns in dementia patients with
dementia patients cerebrovascular disease and Alzheimers
D. DallaLibera1, F. Agosta2, A. Bergami3, G. Magnani3, disease
V. Martinelli4, G. Comi5, C. Verderio6, M. Filippi4, M. Bjerke1, M. Jonsson1, H. Zetterberg1, K. Blennow1,
R. Furlan7 R. Schmidt2, A. Wallin1
1Neurology, San Raffaele Scientific Institute, 2Neurology, San 1Dep of Psychiatry & Neurochemistry, Inst of Neuroscience
Raffaele Scientific Institute Neuroimaging Research Unit, & Physiology, The Sahlgrenska Academy, University of
3San Raffaele Scientific Institute, 4San Raffaele Scientific
Gothenburg, Sweden, 2Department of Neurology, Division of
Institute Neuroimaging Research Unit, 5Neurology, San Special Neurology, University Clinic of Neurology, Medical
Raffaele Scientific Institute, INSPE, 61CNR Institute of University of Graz, Austria
Neuroscience and Department of Medical Pharmacology,
Introduction: Alzheimers disease (AD) and vascular
Universit di Milano, 7San Raffaele Scientific Institute,
dementia (VaD) are intertwined by mixed dementia (MD)
INSPE, Milano, Italy
harbouring varying degrees of AD pathology in combination
Introduction: Myeloid microvesicles (MMVs) have been with cerebrovascular disease (CVD) with white matter
indicated as important mediators of intercellular lesions (WML). The diseases may be hard to clinically
communication and are emerging as new biomarkers of separate due to the overlapping pathologies. A cerebrospinal
tissue damage. MMVs, shed from reactive microglia, store fluid (CSF) biomarker pattern representative of WML
and release the IL1beta, an inflammatory cytokine increased would aid in the clinical evaluation process and provide
in AD induced by amyloid deposition. Aim of the study was information about disease specific mechanisms.
to evaluate whether MMVs reflecting microglia activation Methods: Study one included 30 controls, 30 AD patients
state- may contribute to early dementia diagnosis and and 26 patients with subcortical WML (9 VaD and 17 MD).
predict disease rate progression. Study two included 46 normal or only slightly cognitively
Methods: We recruited 169 patients from the Neurological impaired individuals of whom all had subcortical WML
Department of San Raffaele Hospital affected by mild assessed by magnetic resonance imaging. Assessment of
cognitive impairment (MCI), probable Alzheimers disease white matter hyperintensity (WMH) volume, atrophy and
(AD) according to Dubois criteria, Frontotemporal diffusion weighted imaging (DWI) of normal appearing and
Dementia (FTD) and other dementia. Myeloid MVs were WMH matter was performed. Proteins measured in both
detected by flow cytometry in the cerebrospinal fluid of studies were total tau (T-tau), hyperphosphorylated tau 181
dementia patients and healthy controls (HC) and were (P-tau181), amyloid b 1-42 (A1-42), neurofilament light
correlated with clinical features, CSF data (Tau, bAmyloid) (NF-L), myelin basic protein (MBP), matrix
and neuroimaging in a longitudinal study of two-year metalloproteinases (MMPs), and tissue inhibitors of
follow-up. metalloproteinases (TIMPs).
Results: AD patients showed a higher amount of MMVs Results: The biomarkers MBP, TIMP-1, NF-L, and MMP-9
than MCI and HC. An increase in MMVs was found in MCI were altered in patients with WML (VaD and MD) and
patients early converting to AD. MMVs correlate with contributed to the separation between CVD and AD
p-tau/bamiloid and with hippocampal atrophy in AD, but (sensitivity 97% and specificity 81% (AUC=0.93)) in
not in MCI. Correlation with other neuroradiological combination with T-tau, P-tau181 and A1-42. MBP, TIMP-
measures (DTI,fMRI) is ongoing. No correlation with 1 and NF-L were related to WMH volume, the latter two to
clinical data (MMSE, CDR, age, sex) was evident. WMH DWI and ventricular atrophy and MMP-9 to
Conclusions: MMVs reflect the course of neuro progression of WMH.
degeneration, thus they may represent a suitable biomarker Conclusion: The finding of different neurochemical
in monitoring disease progression and response to treatment. fingerprints of CVD with WML and pure AD speaks in
favour of different disease mechanisms of the disorders.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


104 Posters, Sunday 9 September

P1029 P1031
Correlation exploration between executive Quantitative EEG in Alzheimers disease
function impairment and white matter before and after short and long-term
structure in patients with subcortical treatment
ischemic vascular disease A. Medvedeva, T. Vosnesenskaya
Y. Dong, P. Lv, L. Li, J. Wang Neurological Department, First Moscow Medical University,
Department of Neurology, Hebei General Hospital, Moscow, Russia
Shijiazhuang, China Introduction: Alzheimers disease (AD) is involving
Objective: To investigate the correlation between executive cerebral neuronal networks facilitating higher cognitive
function and cerebral white matter structure change by MRI functions.
and diffusion tensor imaging (DTI) in subcortical ischemic Objective: AD is characterized by changes in spectral
cerebrovascular disease (SIVD). power and EEG-coherence, which become stronger with the
Methods: 24 SIVD patients and 14 normal control subjects progression of dementia. The aim was to evaluate spectral
(NC) were evaluated with Stroop colour words test (CWT) power and EEG-coherence in AD group and healthy elderly
and trail making test (TMT) to assess executive function. controls, to find their reactivity in functional tasks before
They were divided into vascular dementia (VaD, n=12) and and after 3 months and 2.7 years of treatment with
vascular cognitive impairment no dementia (VCIND, n=12) galantamin, memantin and their combination, to determine
group. Age-related white matter change rating scale was the correlations between EEG changes, cognitive and
used to measure the WML. Fractional anisotropy (FA) and neuropsychiatric impairments before and after the treatment.
mean diffusivity (MD) of normal- appearing white matter Materials and methods: 22 patients without treatment, 23
(NAWM) were determined in the splenium of corpus patients on long-term treatment with mild and moderate
callosum (SCC), frontal (FL), parietal (PL), temporal (TL) AD, 25 age-matched controls were examined using EEG-
and occipital lobes (OL) by DTI. Correlation analysis was recordings (spectral power and coherence), neuro
used to investigate the relationship between executive psychological investigations and neuropsychiatric inventory
function and the imaging parameters. (NPI).
Results: Results: High level of apathy, depression, emotional
(1) Compared with NC group, the scores of VaD decreased liability was investigated in both AD groups. We have found
in each test and the scores of VCIND decreased in TMT significant increase of slow-wave activity in frontal, central
(p<0.05). and temporal regions, decreased alpha rhythm in the same
(2) The scores of WML were (12.823.57) and (10.502.95) regions in AD group vs. controls (p<0.05). Coherence in
in VaD and VCIND group (p<0.05). MD values of SCC same regions was lower in AD patients vs. controls (p<0.05).
increased in two groups (p<0.05). Decreased FA values of After short and long-term treatment there was decrease of
NAWM on FL, right TL, right OL and PL were demonstrated slow wave activity and increase of alpha band and coherence
in VaD, as well as of left FL and PL in VCIND patients. in the same regions (p<0.05).The combination of galantamin
They were significant compared to NC group (p<0.05) and memantin was more effective than monotherapy
(3) The parameters related with executive function in SIVD (p<0.05).
patients were FA values of NAWM in left FL, right PL, TL Conclusion: EEG changes, cognitive and neuropsychiatric
and MD values of SCC. impairments have positive dynamic on short and long-term
Conclusions: The executive function impairment in SIVD treatment without significant difference. The study revealed
patients could be associated with the degree of WML. significant correlations between increased slow-wave
Microstructural white matter impairment showed by DTI activity, decreased EEG-coherence, low levels of cognitive
might be used for evaluating executive function decline. tests and high level of apathy, depression, emotional
liability.

P1030
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 105

P1032 P1033
Caregiver preference for rivastigmine Memory performance is related to
patch in the OPtimising Transdermal functional connectivity alterations in
exelon In Mild-to-moderate Alzheimers Alzheimers disease
disease (OPTIMA) Study A. Fukuda, F. Pereira, T. Lopes, E. da Silva, A.C. Coan,
R. Blesa1, P. Martnez-Lage2, A.U. Monsch3, P. Downs4, B. Campos, B. Damasceno, F. Cendes, M.L.F. Balthazar
C. Strohmaier5 UNICAMP, Campinas, Brazil
1Hospital Sta Creu i Sant Pau, Barcelona, 2Department of Introduction: New methods in functional magnetic
Neurology, Center for Research and Advance Therapies, resonance imaging, especially during resting state, may help
Fundacin CITA-Alzheimer, San Sebastian, Spain, to clarify the functional organization of cognitive networks.
3Department of Acute Geriatrics, University Hospital Basel,
While their alterations have been reported in AD, especially
Switzerland, 4Novartis Pharmaceuticals Corporation, East in the Default Mode Network (DMN), their relationship
Hanover, NJ, USA, 5Novartis Pharma AG, Basel, Switzerland with cognitive symptoms remains unclear. In this study, we
Introduction: Caregivers of patients with Alzheimers aimed to verify the relation between DMN connectivity and
disease (AD) are often responsible for the administration memory performance in AD patients.
and management of medications. During the OPTIMA Methods: We studied 20 mild to moderate AD patients.
Study, caregivers took part in a questionnaire to evaluate High-resolution Magnetic Resonance Imaging (MRI) was
perceived treatment compliance, satisfaction and preference. performed using a 3.0T scanner (Philips - Achieva). All
Methods: Patients meeting pre-specified decline criteria patients underwent a 10 minutes task-free functional MRI
during a 24-48-week initial open-label (IOL) phase with (fMRI). We used FSLs Melodic Independent Component
9.5mg/24 h rivastigmine patch, entered a 48-week, Analysis to study fMRI images in order to select each
randomised, double-blind phase (9.5 versus 13.3mg/24h patients DMN. They also underwent a cognitive evaluation,
patch). Caregivers of patients taking oral AD medication including the Rey Auditory Verbal Learning Test (RAVLT).
prior to the study completed the Caregiver Medication We correlated RAVLTs scores (encoding, delayed recall
Questionnaire at baseline and week 24 of the IOL phase. and recognition) with individual maps of DMN connectivity,
The questionnaire focused on medication administration, considering age, education and dementia severity. We also
perceived compliance and satisfaction, and at week 24 only, performed VBM analysis to obtain grey matter maps to
treatment preference (prior oral medication or patch). correct these data for atrophy.
Results: Of 1584 participating AD caregivers, 57.8% were Results: We found significant areas of correlation between
females [mean (SD) age; 64.0 (13.7) years]. Most caregivers DMN functional connectivity and RAVLT encoding and
were a spouse (59.5%) or child (29.7%) of the patient and delayed recall with left precuneus (MNI coordinates: -13,
were living with them (74.7%). Of 882 caregivers of patients -67, 34). We did not find any areas of correlation with
on prior AD treatment, 642 (72.8%; 95% CI 69.7, 75.7) recognition, after correction for atrophy.
stated a preference for rivastigmine patch. Ease-of-use Conclusion: We report the association of changes in DMN
(27.9%) and administration (10.9%) were rated as the top connectivity (atrophy corrected) with episodic amnesia in
two most important reasons. From baseline to week 24, the AD, especially in left precuneus. These findings confirm
number of caregivers reporting that they were never the importance of neurofunctional networks alterations in
concerned that the patient missed taking medication the origin of AD symptoms.
(compliance) increased from 55.1% to 69.5% and mean
(SD) treatment satisfaction increased from 6.3 (2.7) to 7.3
(2.6) out of 10.
Conclusion: The majority (>70%) of caregivers of AD
patients preferred rivastigmine patch to oral medication,
and reported improved compliance and satisfaction
compared with previous oral medications.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


106 Posters, Sunday 9 September

P1034 P1035
Comparing language deficits in mild -Aminobutyric acid type A (GABAA)
cognitive impairment and mild Alzheimers receptor activation modulates tau
disease phosphorylation
E. Tsantali1,2, D. Economidis1, S. Rigopoulou2 N.-P. Nyknen1, K. Kysenius1, P. Sakha1, P. Tammela2,
1Medical School, B Internal Medicine, Geriatric Unit, H. Huttunen1
Aristotle University of Thessaloniki, Hippocration Hospital, 1Neuroscience Center, 2Center for Drug Research, Faculty of
2Neurologic Clinic, Dep. Panagia, Agios Pavlos Hospital,
Pharmacy, University of Helsinki, Finland
Thessaloniki, Greece Introduction: Abnormal phosphorylation and aggregation
Introduction: Alzheimers disease (AD) patients except of of the microtubule-associated protein Tau are hallmarks of
memory loss exhibit naming impairments from mild to various neurodegenerative diseases, such as Alzheimers
moderate stages of the disease, though Mild Cognitive disease (AD). Tau phosphorylation regulates its functions
Impairment (MCI) individuals tend to exhibit greater and is increased in AD. Molecular mechanisms that regulate
impairments in category fluency than on naming tasks. Tau phosphorylation are complex and currently incompletely
Aims: The aims of this study were to: understood. Peptidyl-prolyl cis-trans-isomerase 1 (Pin1) is
1) investigate and compare language abilities of non- a critical regulator of Tau dephosphorylation at several
demented elderly (ND), MCIs (amnestic type) and demented disease-associated proline-directed phosphorylation sites.
participants (AD), Reduced Pin1 activity has been associated with Tau
2) study the diagnostic value of language deficits according hyperphosphorylation in vitro and in vivo.
to the cognitive state of the participants. Methods: We have developed a novel live-cell reporter
Methods: 125 participants, 41 ND, 31 MCI and 53 mild AD system based on protein-fragment complementation assay
patients matched on age and education were recruited (PCA), using split humanized Gaussia princeps luciferase
randomly and administered clinical, laboratory, (hGLuc), to study dynamic changes in Tau phosphorylation
neuropsychological and neuroimaging assessment. status. In this assay, fusion proteins of Tau and Pin1 carrying
Language abilities were assessed by expert complementary fragments of the hGLuc protein serve as a
neuropsychologists using the Greek edition of the Boston sensor of altered protein-protein interaction between Tau
Diagnostic Aphasia Examination (BDAE). and Pin1.
Results: Our results indicate that verbal fluency, auditory, Results: We identified several structurally distinct GABAA
reading comprehension and narrative ability are the main receptor modulators as novel regulators of Tau
language abilities to be affected in mild AD, but they are phosphorylation in a chemical library screen. GABAA
vulnerable as well as in MCI. Six useful language subtests receptor activation promoted specific phosphorylation of
of the BDAE discriminated ADs from the rest 2 groups Tau at the AT8 epitope (Ser-199/Ser-202/ Thr-205) in
92.5% of the time, NDs 86.8% and MCI 67.9% of the time cultures of mature cortical neurons. Increased Tau
in order to save time and to be accurate in clinical practice. phosphorylation by GABAA receptor activity was
Conclusion: Language impairments are affected MCIs as associated with reduced Tau binding to protein phosphatase
well as AD patients but in a slighter degree, but not elderly 2A and was dependent on Cdk5 but not GSK3 kinase
without dementia. Language tests cannot distinguish MCI activity.
from non-demented elderly and mild AD patients safely. Conclusions:
(1) GABAA receptor activity is associated with regulation
of Tau phosphorylation.
(2) hGLuc-based PCA is an efficient novel method for
studying Tau interactome dynamics and for high-throughput
screening of modulators of Tau function in live cells.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 107

P1036 P1037
PCSK9 regulates neuronal apoptosis by Early onset Alzheimers disease
adjusting ApoER2 levels and signalling N. Behrem1, A. Altinkaya2, N. Karagoz Sakall1,
K. Kysenius1,
P. Muggalla1, K. Mtlik2, U. Arume2, P.N. Sutlas1, D. Kirbas1, B. Topcular2,3
H. Huttunen1
1Department of Neurology, Bakirky Teaching and Research
1Neuroscience Center, University of Helsinki, 2Insititute of Hospital for Mental Health and Neurological Disorders,
2Department of Neurology, 3Center for Research in Emotion
Biotechnology, University of Helsinki, Finland
and Cognition, Istanbul Bilim University, Istanbul, Turkey
Introduction: Proprotein convertase subtilisin/kexin type 9
(PCSK9) binds to low-density lipid (LDL) receptor family Introduction: Early-onset dementia is defined as dementia
members LDLR, VLDLR and ApoER2 promoting their before 65 years of age. The prevalence of dementia <64
degradation in intracellular acidic compartments. LDLR years of age is approximately 54-260/100,000. Studies on
controls the systemic levels of circulating LDL, whereas early onset Alzheimers dementia (AD) show different
ApoER2 and VLDLR primarily mediate Reelin signalling clinical and sociodemographic features compared to late
in the brain, critical for the development and plasticity of onset AD. We aimed to study the clinical and
the central nervous system. Expression level of PCSK9 is sociodemographic features of early onset AD patients in our
highest in the cerebellum during perinatal development, but department.
is also increased in the adult brain after ischemia. The Material and method: We included consecutive early onset
function of PCSK9 in apoptotic context is currently poorly AD patients admitted to our Behavioural Neurology and
understood. Dementia outpatient clinic between September 2011 and
Aim: To investigate the role of PCSK9 in neuronal January 2012 in the study.
apoptosis. Results: There were 48 early onset AD patients. Female/
Methods: Potassium deprivation of cerebellar granule male ratio was 0.92. Mean age at symptom onset was 53.32
neurons (CGN) was used as an apoptosis model and cell (36-64) years, mean age at onset was 56.82 (41-66) years.
death assessed by activation of caspase-3 and c-Jun and Mean education duration was 4.96 years. Mean follow-up
nuclear morphology. PCSK9, VLDLR and ApoER2 were time was 2.84 years. Mean MMSE and CDR scores at first
targeted by lentiviral RNAi to investigate their role in CGN visit were 13.34 and 1.66 respectively. The first symptom
apoptosis. was memory impairment in 79.16% (n=38) of patients
Results: PCSK9 RNAi reduced the activation of c-Jun and followed by executive dysfunction (n=6), language
caspase-3 by 44% and 59%, respectively, at 6h post- impairment (n=2) and visuospatial dysfunction (n=2). The
deprivation and the resulting cell death by 41%. Protection female/male ratio is lower than in the late onset AD cohort
was observed up to 24 hours post-deprivation. RNAi of in our department. The duration of education and CDR at
ApoER2 but not of VLDLR reversed the protection elicited first visit were similar to late onset AD patients. The
by PCSK9 RNAi. Pharmacological studies suggested that frequency of cognitive domains other than memory as initial
PCSK9 regulates neuronal apoptosis independently of presentation was higher in early-onset AD patients. The rate
NMDA receptor function but in concert with ERK and JNK of decline in MMSE and CDR was slower in early onset AD
signalling pathways. PCSK9 RNAi also protected CGN patients.
from staurosporine-induced apoptosis and dorsal root Conclusion: The frequency of early onset AD is increasing.
ganglion neurons from NGF-deprivation. Studies show that early and late onset AD have different
Conclusions: Our results indicate that PCSK9 potentiates characteristics in terms of clinical features.
neuronal apoptosis via downregulation of ApoER2 levels
and related anti-apoptotic signalling pathways.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


108 Posters, Sunday 9 September

P1038 P1039
Influence of age in CSF biomarkers of Cognitive impairment in general paresis:
Alzheimers disease from control subjects AD-like pattern?
J.A. Monge-Argils1, C. Muoz-Ruiz2, J. Snchez-Pay3, A. Altinkaya1, M. Ozerden2, N. Behrem2, M. Colak2,
J. Montoya-Gutirrez1, C. Leiva-Santana1 N. Karagoz Sakall2, P.N. Sutlas2, D. Kirbas2,
1Service of Neurology, 2Inmunology Laboratory, 3Preventive
B. Topcular1,3
Medicine, General Hospital of Alicante, Alicante, Spain 1Department of Neurology, Istanbul Bilim University,
Introduction: Cerebrospinal fluid (CSF) biomarkers of
2Department of Neurology, Bakirky Teaching and Research
Alzheimers disease (AD) are currently being assessed in Hospital for Mental Health and Neurological Disorders,
3Center for Research in Emotion and Cognition, Istanbul
patients with cognitive impairment. In a few studies, it has
been demonstrated the influence of age in the CSF Bilim University, Istanbul, Turkey
concentration of these biomarkers. Our objective was to Objective: Neurosyphilis is a treatable cause of dementia.
confirm this influence in our cohort of control subjects. Recent studies report an Alzheimers disease (AD)-like
Material: We included 55 CSF samples from control pattern in general paresis patients. We retrospectively
subjects, without cognitive impairment and MMSE over 27, investigated the clinical, cognitive and radiological features
with ages between 50 and 87. This population were patients of patients with general paresis of the insane (GPI) in
who underwent spinal anaesthesia for orthopaedic or comparison to early onset AD patients.
urological non-malignant conditions. Using xMAP Methods: GPI and early onset AD patients followed at the
technology and INNO-BIA Alzbio-3 reagents from Dementia and Behavioural Neurology outpatient clinic were
Innogenetics, we quantified CSF A1-42, T-tau and retrospectively compared in terms of clinical,
P-tau181p proteins. We analysed the results with the neuropsychological and radiological features.
Spearman p correlation. Informed consent was obtained Results: There were 16 patients with GPI and 44 patients
before the procedure. with early onset AD. The mean age of onset and mean
Results: Our results showed a moderate correlation between follow-up duration for GPI patients were 48 years and 2.12
age and T-tau (r=0.5) or P-tau181p (r=0.4). Nevertheless, no years, respectively. The mean age of onset and mean follow-
correlation was found with A1-42 protein CSF up duration for early onset AD patients were 56.82 years
concentrations. These results are very similar to those and 2.84 years, respectively. All GPI patients were male
published in the last year. whereas female/male ratio was 0.92 in early onset AD
Conclusion: In our experience, as others published recently, group. GPI showed a similar neuropsychological pattern to
age has a moderate influence on T-tau and P-tau181p CSF early onset AD patients. GPI and early onset AD patients
protein levels, but it is hardly detectable in A1-42 protein had similar performances in memory, language and
CSF concentrations. executive function tests. Attention was more severely
impaired in GPI patients whereas, visuospatial functions
were more severely impaired in AD patients. GPI patients
had more psychiatric symptoms compared to AD patients.
Both patient groups had medial temporal atrophy which was
more severe in the AD group.
Conclusion: Out results support previous studies that
demonstrate GPI that patients show AD-like pattern in
terms of clinical feature but also show that GPI and AD
might show similar radiological features, too.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 109

P1040 P1041
Paraoxonase 1 (PON1) and anti-oxidised Topographical disorientation (TD) in
LDL antibodies in a group of elderly ageing: preliminary results in healthy
subjects in Poland subjects and MCI patients
M. Bednarska-Makaruk1, H. Wehr1, M. Rodo1, A. Suardi1, M.L. Rusconi1, L. Rozzini2, M. Zanetti2,
W. Szirkowiec1, D. Ryglewicz2 M.R. Tamborrino3, L. Carelli1
1Department of Genetics, 2First Department of Neurology, 1Human Sciences, University of Bergamo, 2Neurological

Institute of Psychiatry and Neurology, Warsaw, Poland Clinic, University of Brescia, 3Santa Chiara Institute, Lecce,
Introduction: Paraoxonase 1 (PON1) is an enzyme Italy
associated with high density lipoproteins (HDL). PON1 has Introduction: Some evidences have suggested TD as a
antioxidant properties and its very important role consists possible indicator of Mild Cognitive Impairment (MCI)
of preventing low density lipoproteins (LDL) from oxidative conversion into Alzheimers disease (Hort et al., 2007;
modification. Antibodies against oxidised LDL (anti- Cushman et al., 2008). The purpose of our study was to
oxLDL) were considered to be a good index of LDL validate a new ecological instrument for clinical practice in
oxidation. The aim of the study was to investigate PON1 order to evaluate TD in elderly healthy subjects and MCI
and anti-oxLDL in a Polish population of elderly individuals. patients.
Methods: The investigated group consisted of 3154 Materials: We built an ideal city, consisting of 14 Legos
individuals (1570 men and 1584 women) mean age pieces, 12 road signs, 1 toy car and 1 lay-figure, all
76.310.0 years. The subjects were participants of removable and supported on a thick sheet (180x80 cm)
multicenter PolSenior project and were recruited randomly where 18 roads and 4 squares are printed.
from the Polish population. The cognitive status of the Methods: We enrolled 12 right-handed healthy participants
participants was estimated using MMSE. PON1 activity [10 females (F), 2 males (M); mean age =70.67, SD=4.6;
was determined in serum basing on the method of Kitchen mean schooling=9.75,SD=5.3] and 12 right-handed MCI
[1973] using phenyloacetate as substrate. Anti-oxLDL patients (10 F, 2 M; mean age=72.25, SD=3.9; mean
antibodies level was determined in serum by ELISA schooling =7.75, SD=4). All participants were submitted to
method. a neuropsychological battery and to an experimental test
Results: Significant negative correlation of PON1 activity which included: route learning forward and backward test;
with age and anti-ox LDL and positive correlation with Free recall landmark; Photographs recognition and location
HDL-C and MMSE were stated. Correlation of anti-oxLDL on the map; Map drawing; Recall and replace landmark on
with age was significantly positive but correlation the map; Replace landmark on the city; Routes description.
coefficient was very low. PON1 activity and anti-oxLDL Results: MCI patients showed difficulties in episodic
level were significantly lower in males as compared to memory, spatial planning and selective attention tasks. They
females. Mean PON1 activity was also decreased in 360 performed significantly worse in time learning forward and
subjects with MMSE<70% as compared to a group of 2667 backward test, map drawing and routes description; a trend
persons with MMSE70%. was observed in free recall landmark and photographs
Conclusions: PON1 activity was diminishing with location on the map.
advancing age which could contribute to cognitive Discussion: The interesting differences observed between
impairment development in the elderly. Implemented under subjects and patients allow us to consider the new instrument
publicly-funded project no. PBZ-MEIN-9/2/2006, Ministry as a promising ecological test to study the various
of Science and Higher Education. components involved in TD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


110 Posters, Sunday 9 September

P1042 P1043
Monitoring brain changes in mild Nerve growth factor treatment in
cognitive impairment and a biomarker for Alzheimers disease: EEG changes and
structural changes and correlations with relation to FDG-PET
serial PET-FDG scans, PET-PIB scans, and H. Eyjolfsdottir1,2, M. Eriksdotter2,3, P. Almqvist4,5,
MMSE scores O. Almqvist2,3, A. Kadir3, A. Karami3, G. Lind4,5,
W.R. Riddle1, S. Shokouhi2, S.C. DonLevy3 B. Linderoth4,5, L. Wahlberg6, . Seiger2,3,7, V. Jelic2,3
1Dept Neurobiology, Caring Sciences and Society,
Radiology and Radiological Sciences, Vanderbilt University,
1

Nashville, 2Radiology and Radiological Sciences, Vanderbilt Karolinska Institute, 2Dept Geriatrics, Karolinska University
University, Nasnville, 3Pediatric Nurse Practitioner, Hospital, 3Dept Neurobiology, Care Sciences and Society,
4Dept Clinical Neuroscience, Karolinska Institute, 5Dept
Nashville, TN, USA
Neurosurgery, Karolinska University Hospital, Stockholm,
Introduction: The objective of this study was to show how Sweden, 6NS Gene A/S, Ballerup, Denmark, 7Stockholms
a biomarker for structural changes generated with Sjukhem, Stockholm, Sweden
deformation-based morphometry can be used to monitor the
Background: Nerve growth factor (NGF) is important for
progression of Alzheimers disease and to correlate these
the growth and survival of central cholinergic neurons, a
changes with serial PET F-18 labelled fluorodeoxyglucose
signalling system impaired in Alzheimers disease (AD). We
(FDG) uptake scans and C-11 labelled Pittsburgh Imaging
treated 6 AD-patients with NGF by encapsulated NGF-
Compound B (PIB) retention scans.
releasing cells implanted into the forebrain. This report
Methods: 20 subjects with mild cognitive impairment and
focuses on the effects on EEG and the relation to glucose
20 normal subjects were downloaded from the Alzheimers
metabolism on PET (FDG-PET).
Disease Neuroimaging Initiative database. For each subject,
Objective: To investigate if NGF treatment improves EEG
serial Mini-Mental Status Exam (MMSE) scores, FDG
parameters and if EEG improvement correlates with
uptake volumes, PIB retention volumes, and T1-weighted
increased glucose metabolism on FDG-PET.
MR volumes were obtained. A biomarker for structural
Methods: 6 AD-patients (4 women and 2 men, mean age 62
changes was generated from the MR volumes using
years, mean MMSE at baseline 22) received treatment with
deformation-based morphometry. Binary masks for ten
encapsulated NGF-releasing cells implanted into the basal
brain regions were created for each MR image volume.
forebrain stereotactically. They were evaluated clinically,
After registering the FDG and PIB scans to the MR scans,
with quantitative EEG (qEEG) and PET at baseline, 3 and
the correlation coefficient between each subjects initial
12 months. Peak frequency (PF) (4-20 Hz) and spatial
normalized FDG vector and subsequent normalized FDG
distribution of alpha generators was analyzed from qEEG.
vector was calculated and displayed as a function of the
Cerebral metabolic ratio of glucose (CMRGlu) was
time
calculated from FDG-PET data.
Results: The normal subjects preserved their structural
Results: 3 patients had PF within normal range (8.5Hz) at
biomarkers, FDG correlation vectors, and cognitive scores.
baseline and showed significant increases in PF in the beta
In mild cognitive impaired subjects, the FDG correlation
frequency range during treatment. They had a higher MMSE
resembled the MMSE scores and the biomarker for
score at baseline, positive z-scores in Block Design, positive
structural changes identified the location of brain changes.
ADAS-cog changes and increased cortical nicotine binding
Conclusions: Our multi-modality approach may provide
at 12 months. There was a significant reduction in frontal
useful information for early detection and understanding the
shift of alpha generators during treatment which correlated
pathogenesis of Alzheimers disease.
with increased CMRGlu.
Conclusions: A more normalized EEG pattern with an
increased PF in the beta range and a reduced frontal shift of
alpha generators was found in a subset of patients suggestive
of a treatment effect with NGF.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 111

P1044 P1045
The rate of conversion to dementia in Enzyme antioxidant defences are
mild cognitive impairment (MCI) patients diminished in early stages of Alzheimers
L. Prodan1, A. Draghici2, C. RomanFilip1 disease
1Neurology, University Lucian Blaga Sibiu, Clinical Hospital
M.J. Ramirez-Exposito1, M.C. Puertas1, M.P. Cobo2,
County, 2Universiy Lucian Blaga Sibiu, Romania M.P. Carrera1, M.D. Mayas1, J.M. Martinez-Martos1
Aim: The aim of our study was to investigate the risk of 1Health Sciences, University of Jaen, 2Service of Neurology,

conversion to dementia for different MCI subtypes and University Hospital of Jaen, Spain
identify the clinical risk factors of conversion. Alzheimers disease (ATD) is the most common
Methods: We recruited 365 patients, >65 years, who met neurodegenerative disorder worldwide. It is characterized
the criteria for MCI and evaluated their global cognitive neuropathologically by the formation of senile plaques and
performances at baseline and annual for the next three neurofibrillary tangles, and clinically by the progressive
years. The diagnostic of dementia was established using deterioration of memory and other cognitive functions.
DSM IV TR criteria. Senile plaques are primarily composed of a 40-42 amino
Results: There were 365 cases of incident MCI, 199 patients acid peptide denoted as -amyloid (A). The accumulation
(54%) of amnestic MCI and 1666 patients (46%) of non- and deposition of A in selective brain regions is a major
amnestic MCI. The rate of conversion in the first year was cause of neurotoxicity and is assumed to be a culprit for
10.4%, in the second year 17.8% and in the third year inducing the pathologic process of ATD. Importantly, A is
23.3%. The mean rate of conversion during the follow-up a major source of reactive oxygen species (ROS) and other
period was 18.9% for Alzheimers disease (AD), 11.8% for toxic radicals. In our study, we measured the plasma levels
vascular dementia (VD) and 13.2% for Alzheimers disease of the enzyme defence system glutathione peroxidase
with cerebrovascular disease (AD with CVD). The rate of (GPx), catalase (CAT) and superoxide dismutase (SOD) in
conversion for specific MCI subtype was 17.1%/year for patients diagnosed of ATD and their age-matched controls.
patients with amnestic MCI and 11.5%/year for non- We found decreased levels of catalase and GPx in ATD
amnestic MCI. During the follow-up period 69 patients patients when compared with control groups, although
converted to AD, 71% were patients with amnestic MCI; 43 changes in catalase activity were found in men but not in
patients converted to VD; 46.5% were patients with women. On the contrary, we did not find changes in SOD
amnestic MCI; and 48 patients converted to AD with CVD; activity, although Western blotting analysis showed
68.7% were patients with amnestic MCI. The mean age for increased protein levels of SOD-1 in both men and women
converters was 72.8 year and for non-converters 74.2 year. with ATD. Our results suggest that there is a defect in the
The AD converters have high serum cholesterol levels, high enzyme antioxidant defence system that is incapable of
density lipoprotein, and the debut of hypertension before 65 responding to increased free radical production, which may
year. lead to oxidative damage and the development of the
Conclusion: The combination of amnestic MCI subtype pathological alterations that characterize the
with the clinical risk factors increased the risk of conversion neurodegenerative disorder of patients with ATD.
to dementia. Furthermore, this defect could be detected early during the
development of the disease. Supported by Junta de
Andaluca, Spain.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


112 Posters, Sunday 9 September

P1046 P1047
Baseline characteristics of subjects with Agitation - relation to clinical and
prodromal Alzheimers disease: the dementia biomarkers in cerebrospinal
LipiDiDiet study fluid
Y. Freund-Levi1, P.J. Visser2,3, M. Kivipelto4, V. Bloniecki1, Y. Freund-Levi2, D. Aarsland1,
P.J.G.H. Kamphuis5, R.L. Wieggers5, T. Hartmann6,7, K. Blennow3
H. Soininen8 1 Karolinska Institute, 2NVS, Karolinska Institute,
1Department of NVS, Section of Clinical Geriatrics, 3 Sahlgrenska Universitetssjukhuset, Stockholm, Sweden
Karolinska Institutet, Karolinska University Hospital, Introduction: Dementia leads to deterioration of patients
Huddinge, Sweden, 2Department of Psychiatry and cognitive abilities and neuropsychiatric symptoms in
Neuropsychology, Alzheimer Center Limburg, University of dementia (NPSD). NPSD contribute to the disease burden
Maastricht, 3Department of Neurology, Alzheimer Center, of patients and care-givers, increase early hospitalization
VU University Medical Center, Amsterdam, The Netherlands, and constitute approximately 30% of dementia associated
4Aging Research Center, Karolinska Institutet and Stockholm
treatment costs. The objective of this study is to examine the
Gerontology Research Center, Stockholm, Sweden, 5Nutricia correlation between agitation and cerebrospinal fluid (CSF)
Advanced Medical Nutrition, Danone Research - Centre for
biomarkers (tau, phosphorylated tau and A1-42), clinical
Specialised Nutrition, Wageningen, The Netherlands,
6Deutsches Institut fr DemenzPrvention (DIDP),
and gender aspects of agitation.
Methods and material: 100 patients 45 years (mean
Neurodegeneration and Neurobiology, 7Experimental
78.77.5), 67% female, BMI (mean 24.34.3) and MMSE
Neurology, Homburg, Germany, 8Department of Neurology,
University of Eastern Finland and Kuopio University
(mean 20.04.6) fulfilled diagnostic criteria of dementia
Hospital, Kuopio, Finland according to DSM-IV. All patients underwent a physical
exam including lumbar puncture for analysis of biomarkers
Introduction: Souvenaid, containing the specific nutrient and assessment of the patients agitation level using the
combination Fortasyn Connect1, is designed to support Cohen Mansfield Agitation Inventory (CMAI).
synapse formation and function in patients with Alzheimers Results: No statistically significant correlation was found
disease (AD). Two earlier randomised controlled clinical between agitation and all CSF-biomarkers. Men had
trials have shown that Souvenaid improves memory statistically significant higher scores on the CMAI- scale as
performance in drug-nave mild AD patients (MMSE 20-26; compared to women (F=4.0, p=0.05). Men with Alzheimers
Scheltens, 2010) (MMSE 20; Scheltens, 2011), indicating dementia have a significant negative correlation between
that Souvenaid may have a promising effect in the early BMI and agitation relating to non-aggressive physical
phases of AD. The LipiDiDiet study2 was designed to behaviour (r=-0.7, p=0.006).
investigate the effects of Souvenaid on cognition in the Conclusions: The absence of correlation between agitation
prodromal phase of AD. and biomarkers in CSF could suggest that neurofibrillary
Methods: The LipiDiDiet study is a 24-month, randomised, tangles and amyloid plaques are not involved in the cerebral
controlled, double-blind parallel-group study investigating pathology causing NPSD. High levels of agitation in males
the effects of Souvenaid in 300 prodromal AD patients with low BMI raise the interesting question whether
(criteria Dubois, 2007). Primary outcome measure is agitation amongst males with low BMI can be treated by
cognitive functioning as assessed by a modified version of increasing the daily calorie intake.
the Neuropsychological Test Battery (Harrison, 2007).
Secondary outcome measures include progression to AD,
cognitive performance (MMSE, ADAS-cog), functional
abilities (ADCS-ADL), depression (MADRS), MRI atrophy
rate, dementia biomarkers, safety, tolerance and nutritional
parameters.
Results: The LipiDiDiet study started in 2009. Enrolment
is expected to be completed in 2012 and the first results of
the intervention are expected to be available in 2014.
Conclusion: Baseline data from the total group of
prodromal AD patients will be presented.
1Souvenaid is a registered trademark of N.V. Nutricia.

Fortasyn is a trademark of N.V. Nutricia.


2Funded by the EU FP7 project LipiDiDiet, Grant

Agreement N211696.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 113

P1048 P1050
Short-term memory binding in mild Clinical significance of serum
cognitive impairment: a preclinical marker inflammatory markers in patients with
of Alzheimer's disease? subcortical ischemic vascular disease
E. Cerea, S. Caratozzolo, M. Riva, B. Vicini Chilovi, P. Lv, Y. Dong, L. Li
L. Rozzini, A. Padovani Department of Neurology, Hebei General Hospital,
Institute of Neurology, University of Brescia, Italy Shijiazhuang, China
Introduction: It is increasingly important to identify Objectives: To evaluate the levels of soluble CD40 ligand
neuropsychological measures easy to perform at low costs (sCD40L), interleukin-6 (IL-6) in the serum of patients with
that can help the clinician to predict subsequent cognitive SIVD and the correlation among the serum levels, cognition
decline. The aim of the study is to examine two and white matter lesions (WML).
neuropsychological tests, recently proposed in literature, the Methods: 39 SIVD patients and 15 normal elderly controls
Short Term Memory Binding Test (Parra and Della Sala, (NC) were evaluated with MMSE and MoCA to assess the
2009) and five words of Dubois (2002), and comparing cognitive function. The patients were divided into vascular
them with neuropsychological tests regularly used during dementia (VaD, n=19) and vascular cognitive impairment
routine neuropsychological practice. no dementia (VCIND, n=20) group. Age-related white
Methods: 36 patients with a diagnosis of Mild Cognitive matter change rating scale was used to qualitative measure
Impairment (MCI) and 35 healthy controls were assessed the WML. The serum levels of sCD40L and IL-6 were
with a neuropsychological battery (including tests of detected by enzyme linked immunosorbent assay (ELISA).
memory, attention, visuospatial, executive functions and The relationship was described between these inflammatory
phonemics and semantic fluency), the Dubois test of five factors and WML, cognitive impairment in patients with
words and a visual short-term memory task estimating the SIVD.
recognition of shape, colours and shape-colour binding Results:
(STMB). (1) A statistically increase of sCD40L serum level of SIVD
Results: Patients with MCI performed significantly worse patients versus healthy controls was detected (F=9.019
than healthy controls on most parts of neuropsychological p=0.000).
assessment (MMSE-TMT B-Reward Rey list), on the five (2) Compared with controls, patients with SIVD had
words of Dubois test and on the STMB. The STMB test elevated IL-6 serum levels (F=10.605 p=0.000).
correlated with episodic memory and executive functions (3)There was no statistically difference in the VaD and
tests whereas the test of five words of Dubois correlates VCIND patients (p>0.05).
only with memory tests. (4) There was no statistically correlation between the serum
Conclusions: The test of the five words of Dubois and levels of sCD40L, IL-6 and MMSE, MoCA, WML scores.
STMB test are, by their high sensitivity and specificity, tests (5) Slight positive correlation between sCD40L and IL-6
indicated in the diagnostic process of patients with memory was observed (r=0.318, p=0.023).
disorder. The deficit in memory domain and executive Conclusions:
function has been claimed to be predictive of conversion to (1) Increased serum levels of sCD40L and IL-6 in patients
Alzheimers disease in patients with MCI; thus, it is possible with SIVD might indicate that inflammatory processes may
to assume that the STMB test might be a good predictor of be activated in SIVD.
conversion to AD. (2) Enhanced serum levels of sCD40L, IL-6 could not imply
the association with the severity of cognitive impairment
P1049 and WML in SIVD patients.
(3) sCD40L and IL-6 may be promoting the pathological
Abstract cancelled changes of SIVD in common.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


114 Posters, Sunday 9 September

P1051 P1052
Saturated very long chain fatty acid- Acceptability, safety and feasibility of
induced neuronal dysfunctions playing aerobic exercise in patients with mild to
essential roles in Alzheimers disease: moderate Alzheimers disease: a pilot
impairment of mitochondrial activity, study
induction of oxidative stress and K.S. Frederiksen1, N. Sobol2,3, N. Beyer2,3, K. Madsen4,
cytoskeleton disorganization I. Law5, E. Garde6, S.G. Hasselbalch1,4, G. Waldemar1
1Memory Disorders Research Group, Dept. of Neurology,
A. Zarrouk1,2, T. Nury1, A. Vejux1, G. Lizard1
1Laboratoire Biochimie du Peroxysome, Inflammation et Copenhagen University Hospital, Rigshospitalet, 2Institute of
Sports Medicine, Bispebjerg Hospital, Denmark,
Mtabolisme Lipidique, Universit de Bourgogne, Dijon, 3Musculoskeletal Rehabilitation Research Unit, Copenhagen,
France, 2Laboratoire de Biochimie, Facult de Mdecine de
Bispebjerg Hospital, 4Neurobiology Research Unit, 5Nuclear
Monastir, Tunisia
Medicine and PET, Copenhagen University Hospital,
In Alzheimers disease (AD), some lipid alterations point Rigshospitalet, 6Danish Research Center for Magnetic
towards a peroxisomal dysfunction. A cortical accumulation Resonance, Copenhagen University Hospital, Hvidovre,
of saturated very long chain fatty acids (VLCFA: C22:0, Copenhagen, Denmark
C24:0 and C26:0), substrates for peroxisomal -oxidation,
Introduction: Aerobic exercise may modulate
has been found in AD patients. Human neuronal cells
neuropathology and symptoms of Alzheimers disease
(SK-NB-E) were treated with VLCFAs (5-20M; 48h). The
(AD). We assessed the feasibility of conducting a study of
impact of VLCFAs on mitochondria was evaluated by the
moderate intensity aerobic exercise in patients with mild to
MTT test, flow cytometric analysis (DiOC6(3), MitoSOX),
moderate AD, which included assessment of beta-amyloid
fluorescence microscopy (MitoTracker Red staining),
with PiB-PET.
transmission electron microscopy. Oxidative stress was
Methods: Home-dwelling patients with mild to moderate
estimated by flow cytometric analyses (DHE, DHR123, and
AD were recruited. An aerobic exercise program supervised
DAF), fluorescence microscopy (monochlorobimane), and
by a physiotherapist was administrated 3 times a week for 1
gas chromatography coupled with mass spectrometry.
hour over 14 weeks. Feasibility was assessed on attendance
Cytoskeleton organization (actin, tubulin and neurofilament
and retention rates, safety and a semi-structured interview.
network) was evaluated by flow cytometry and/or
Patients underwent PiB-PET pre- and post-intervention, for
fluorescence microscopy with Rhodamine-phalloidin and
quantification of beta-amyloid. Assessment of cognitive
specific antibodies. Mainly with VLCFAs used at 10 and
function (MMSE, Symbol Digit Modalities Test (SDMT)),
20M, some mitochondrial changes were observed: reduced
Quality of Life (QoL-AD patient- and proxy-rated),
succinate dehydrogenase activity, loss of m, increased of
Activities of Daily Living (ADCS-ADL) and depressive
the mitochondrial mass, identification of elongated
symptoms (Geriatric Depression Scale-15) were also
mitochondria, and overproduction of mitochondrial
carried out.
superoxide anions. An overproduction of superoxide anions
Results: Results are reported as meanSD. 8 patients were
and H2O2 was also revealed, no or slight overproduction of
included in the study. Attendance (90%) and retention
NO was found, and a decrease of GSH associated with an
(87.5%) was high. No serious adverse effects were observed.
increased lipid peroxidation was detected. A more or less
Patients had fewer depressive symptoms after the
pronounced disorganization of the cytosqueleton was
intervention (mean change on GDS: -1, (1); p=0.04).
observed with VLCFAs at 10 and 20M. VLCFAs are able
Proxy-rated QoL (-3.4 (1.7); p=0.002) decreased whereas
to induce neuronal damages (mitochondrial dysfunctions,
patient-rated QoL did not (0.4 (2.9); p=0.7). SDMT score
induction of oxidative stress and disorganization of the
(-4.4 (4.1); p=0.03)) decreased indicating poorer mental
cytoskeleton) which can contribute to the development of
speed and processing. PiB retention increased in all patients
AD. Thus, increased VLCFA levels in cortical lesions of AD
(pre-test: 2.60 (0.3); post-test: 2.71 (0.31); p=0.005).
patients might contribute to the development of this disease.
Conclusion: It is feasible and safe to carry out a study of
moderate intensity aerobic exercise in patients with AD. Our
uncontrolled study was not designed to examine the
effectiveness of aerobic exercise, which must be evaluated
in a large-scale randomized controlled trial.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 115

P1053 P1054
Caregiver burden and consumption of Human amniotic fluid stem cells over-
social services among patients with early expressing choline acetyltransferase gene
onset Alzheimers disease compared to restore cognitive function of kainic acid-
late onset Alzheimers disease induced Alzheimers disease model rats
H. Grnning1, S. Kristiansen2, D. Dyre2, A. Rahmani1, J. Kyung1, D. Kim1, D. Park1, Y.-H. Yang1, E.-K. Choi1,
J. Gyllenborg1, P. Hgh1 H.J. Lee2, S.U. Kim3, Y.-B. Kim1
1Neurology, 2Geriatrics, Roskilde University Hospital, 1College of Veterinary Medicine, Chungbuk National

Roskilde, Denmark University, Cheongju, 2Medical Research Institute, Chung-


Introduction: Alzheimers disease (AD) is the most Ang University Medical School, Seoul, Republic of Korea,
3Division of Neurology, University of British Columbia
common dementia disorder. For patients with early onset
AD (onset before 65 years of age, EOAD) the impact of the Hospital, Vancouver, BC, Canada
disease appears to be more devastating than in late onset AD We recently established an immortalized amniotic fluid
(onset after 65 years of age, LOAD). The purpose of the stem cell line (AF2) over-expressing human choline
study was to analyse caregiver-burden and the consumption acetyltransferase (ChAT) gene and transplanted these AF2.
of social services in a consecutive group of patients with ChAT cells to kainic acid (KA)-induced Alzheimers disease
EOAD compared to a matching LOAD-group. (AD) model rats. Intrahippocampal injection of KA (0.4g/
Materials and methods: Case-control study with 42 rat) to hippocampal CA3 region caused severe neuronal
patients, 21 EOAD (mean 58.5 years) and 21 matched loss, resulting in profound memory deficit. Intra cere
LOAD patients (mean 78.2 years). Patients were matched broventricularly transplanted AF2.ChAT cells (1x 106 cells/
according to disease severity at the time of diagnosis. rat) were found to express ChAT as early as at week 2, in
Caregivers in both groups went through an interview using parallel with the recovery of acetylcholine (ACh) levels in
the Neuro Psychiatric Inventory (NPI), Activities of Daily cerebrospinal fluid (CSF). AF2.ChAT treatment fully
Living (ADCS-ADL) and Resource Utilization in Dementia restored the impaired learning and memory function of AD
(RUD). The quantitative results were statistically compared. animals 4 weeks post-transplantation, in which AF.ChAT
Results: The EOAD-group had a significantly higher ADL- cells were superior to their parental AF cells. The cells
function compared to the LOAD-group. There was a trend migrated to the KA-induced injury site (CA3) and
towards caregivers in the LOAD group spending more time differentiated into neurons, but not into astrocytes. The
helping the patients, and they needed more social services present study demonstrates that human amniotic fluid cells
than the EOAD-group. NPI-scores were not significantly expressing ChAT have lesion-tropic property and improve
different in regards to neuropsychiatric symptoms, but had cognitive function of AD model rats with hippocampal
a tendency to a higher caregiver-burden in the EOAD- injury by increasing ACh level.
group.
Conclusion: The higher caregiver-burden in patients with
EOAD despite a better ADL-function compared to LOAD-
patients indicates that the existing psychosocial services are
insufficient to caregivers in EOAD. Suggestible efforts are
improved information during and after diagnostic
evaluation, an extended follow-up in the clinic, strengthening
of cooperation with the primary health care sector and
psychosocial intervention.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


116 Posters, Sunday 9 September

P1055 P1056
Dorsal hippocampus serves as site of Comparison of statistical methods for
action for angiotensin IV in cognitive handling incomplete data in the
enhancement evaluation of clinical efficacy of
L.A. Sandusky, E.C. McNay memantine in the treatment of
Behavioral Neuroscience, State University of New York, Alzheimers disease
Albany, NY, USA
O.M. Lemming1, S. Bineau2, C. Marre2, F. Marteau2
Introduction: Angiotensin IV (Ang IV) is an endogenous 1International Clinical Research, H. Lundbeck A/S,

pentapeptide inhibitor of the insulin-regulated Copenhagen, Denmark, 2Global Outcomes Research


aminopeptidase (IRAP), and is part of the Renin- Division, Lundbeck SAS, Issy-Les-Moulineaux, France
Angiotensin System (RAS) that resides within the brain. In Introduction: Alzheimers disease (AD) is a
adipocytes, IRAP redistributes with the glucose transporter neurodegenerative disease resulting in irremediable
4 (GLUT4) to the plasma membrane in response to insulin. deterioration in patient health over time. In the context of
In vivo Ang IVs mechanism and specific site of action randomized clinical trials on efficacy and safety of
remains unknown despite having been implicated in antidementia drugs, the progressive nature of AD leads to
numerous studies investigating its memory enhancing some methodological issues when handling missing data
effects. related to patient drop-outs.
Methods: Male Sprague Dawley rats were given a single In the absence of a gold standard method for managing
microinjection cannula in the left dorsal hippocampus. missing data, this study will compare the consistency of
Administration of aECF, 20pM Ang IV, 40pM Ang IV, or results from analysis on observed cases (OC) to the use of
80pM Ang IV was administered 10 min prior to spatial the last observation carried forward (LOCF) method for
working memory testing in the spontaneous alternation imputing missing data, as well as to the Mixed-Effect Model
task. 24 hours later the animals were re-tested in a novel Repeated Measures (MMRM) modelling approach.
spontaneous alternation maze to test for residual effects of Methods: Three approaches have been compared on the set
administration. of pooled data from three pivotal 6-month, double-blind,
Results: Analysis of group means revealed a significant randomised, placebo-controlled trials of memantine (full
improvement in spatial working memory when administered analysis set N=480 on placebo, N=488 on memantine) in
40pm Ang IV compared to aECF alone (p = 0.04) on Day 1. the treatment of moderately severe to severe AD (MMSE
No group differences were detected on Day 2, demonstrating 5-14).
the effects of Ang IV as acute but transient. The data on the main clinical dimensions, i.e. cognition,
Conclusion: Previous research exploring the effects of Ang global status, activities of daily living and behaviour,
IV on cognition has administered the drug via measured through standard scales, have been pooled and
intracerebroventricular injection. Our findings demonstrate analysed using OC, LOCF and MMRM approaches.
the dorsal hippocampus as a site of action for cognitive Results: The efficacy of memantine compared to placebo
enhancement by Ang IV and support the notion that this was demonstrated for all clinical outcomes. These results
drug might be utilized in populations where cognition is were comparable and consistent between the 3 statistical
compromised, such as ageing and dementia. Future research approaches.
will explore additional brain regions to extend current Conclusion: The consistency of the results supports the
findings. reliability of the three statistical methods (LOCF, OC and
MMRM) and reinforces the clinical evidence of memantine
efficacy as compared to placebo in the treatment of
moderately severe to severe AD patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 117

P1057 P1058
Comparison of two analytical platforms Age features of cognitive impairment in
for CSF biomarkers of Alzheimers the early rehabilitation period of ischemic
disease stroke
J.A. Monge-Argils1, J. Snchez-Pay2, C. Muoz-Ruiz3, E. Morozova, L. Pustokhanova
M. Blanco-Cant1, C. Leiva-Santana1 Department of Neurology, Academy of Medicine, Perm,
1Service of Neurology, 2Preventive Medicine, 3Inmunology Russia
Laboratory, General Hospital of Alicante, Spain Background: One of the major risk factors for cognitive
Introduction: Cerebrospinal fluid (CSF) biomarkers of impairment in patients after ischemic stroke is elderly age.
Alzheimers disease (AD) are currently being assessed with Methods: 156 patients (aged 38-84, 97 males and 59
two different assays. Our objective was to study if there is a females) after the first hemispheric ischemic stroke were
correlation between values obtained by both techniques, to examined. Clinical and neuropsychological investigation
compare their validity and search for a conversion factor was carried out within 1 and 3 months after the stroke with
between values obtained for every protein. the use of NIHSS, Barthel index (BI), MMSE, FAB,
Material: We compared the performances of the two most Semantic Verbal Fluency test (SVF), CES-D. The control
commonly used platforms, INNOTEST, an enzyme-linked group consisted of 53 people without stroke.
immunosorbent assay, and INNO-BIA AlzBio-3 for Results: The degree of neurological deficit did not depend
measurement of CSF A1-42, total tau (T-tau) and on age. MMSE decline was found in 100 patients, FAB in
phosphorylated tau 181 (p-tau181p) proteins, in 30 AD 126 patients at the first visit. Data differences in patients
patients and 28 control subjects. The relations between the and control group were confirmed in MMSE (26.223.22
variables of both techniques were evaluated using the and 27.791.55, p=0.0009), FAB (12.653.29 and
Spearman p correlation coefficient (=0.05). ROC and 15.672.25, p=0.0000), SVF (15.786.15 and 20.196.35,
AUC analyses were calculated for the variables of both p=0.0001). Significant differences between the average
techniques. CES-D in patients group and controls were not detected. At
Results: The two assay platforms yielded different absolute the first visit age was associated with the tests: MMSE
values for the various analytes, always higher in INNOTEST. (R=-0.21, p=0.0084), FAB (R=-0.25, p=0.0016), SVF
We found some correction factor between values: 2.1 to 3 (R=-0.28, p=0.0006), CES-D (R=0.20, p=0.0143), BI
fold for A1-42; 4,1 to 4.6 fold for T-tau and 1.4 to 1.6 fold (R=-0.21, p=0.0082). Reduction of cognitive impairment
for p-tau181p. In addition, those values were highly was established at the second visit: S (28.022.23,
correlated (A1-42: r=0.70, p<0.01; T-tau: r=0.90, p<0.01; p=0.0000), FAB (15.592.19, p=0.0000), SVF (18.026.35,
p-tau181p: r=0.85, p<0.01) and the AUC for the variables p=0.0084) increased. The average CES-D was lower than at
showed very similar values. the first visit (11.178.41 and 8.478.01, p=0.0122).
Conclusion: The results obtained with INNOTEST and Correlations between age and FAB (R=-0.24, p=0.0154),
INNO-BIA were highly correlated and its validity very SVF (R=-0.25, p=0.0185), CES-D (R=0.24, p=0.0163)
similar. Differences in absolute values point to the need for preserved.
a clear description of the technique used. However, we Conclusions: Cognitive impairment in post-stroke patients
found some conversion factor between values of every are mainly associated with frontal dysfunction. The patients
protein that may be useful for transformation between both age has a negative effect on the cognitive status, degree of
techniques. depression and social activity.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


118 Posters, Sunday 9 September

P1059 P1060
Patient dependence and clinical measures Relationship between healthcare, social
of cognitive impairment, functional care costs, dependence on others as
disability and behaviour in Alzheimers illness progresses in Alzheimers disease
disease: results from dependence in AD in (AD): results from dependence in AD in
England (DADE) study England (DADE) study
R.W. Jones1, L. Lacey2, M. Knapp3, R. Romeo4, A. Sato3, M. Knapp1, R. Romeo2, A. Sato1, R.W. Jones3,
T. Niecko5, R. Trigg6, DADE Investigator Group L.A. Lacey4, DADE Investigator Group
1Research Institute for the Care of Older People, Bath, UK, 1London School of Economics, 2Kings College London,
2Janssen Alzheimer Immunotherapy, Dublin, Ireland, London, 3RICE, Bath, UK, 4Janssen Alzheimer
3London School of Economics, 4Kings College London, Immunotherapy, Dublin, Ireland
London, UK, 5Niecko Health Economics, Naples, FL, USA, Introduction: The DADE sought to examine how
6Nottingham Trent University, Nottingham, UK
healthcare and social care costs in England change with
Introduction: The DADE study determined the relationship increasing dependence on others.
between dependence and clinical measures of cognition, Methods: 249 patients with possible/probable AD
function and behaviour in a UK population of patients with according to NINCDS/ADRDA criteria participated in a
Alzheimers disease. multi-centre, cross-sectional, observational study. Patient
Methods: A multi-centre, cross-sectional, observational dependence (Dependence scale, DS), healthcare and social
study was conducted. 249 patients with possible/probable resource utilization over the past 3 months (Client Service
AD according to NINCDS/ADRDA criteria, MMSE (3-26) Receipt Inventory (CSRI) were assessed. The costs of
participated. services used by patients were derived by combining
Patient cognition (Mini- Mental State Examination, medication, health and social care resource utilization data
MMSE), function, (Disability Assessment for Dementia, with estimated 2009/2010 unit costs. Costs were classified
DAD), behaviour (Neuropsychiatric inventory, NPI), global into 2 categories: direct medical costs (DMC; i.e.
disability (Clinical Dementia Rating Scale- Sum of Boxes, medication, primary care contacts, hospital outpatient visits,
CDR-SOB) and dependence (Dependence scale, DS) were inpatient admissions, A&E attendance) and direct non-
assessed. Higher scores indicate lower severity for MMSE medical costs (DNMC; i.e. accommodation, social care
and DAD, and greater severity for DS, CDR SOB and NPI. services, aids and adaptation). Relationships were examined
Associations were determined using Ordinary least squares using ordinary least squares regression.
regression (OLS). Results: The average time since AD diagnosis was
Results: The mean (SD) age of patients was 79 (8.5) years. approximately 3 years with 14% living in the nursing home
Average time since AD diagnosis was approximately 3 years or residential care. Bivariate analysis suggested a statistically
and 86% of patients lived in the community and 14% in a significant association (p<0.01) between DS and total DMC
nursing home or residential care home. The mean (SD) plus DNMC: a one point rise in DS is expected to increase
scores for DS, MMSE, DAD, CDR SOB and NPI were 8 the total DMC plus DNMC by 760 over 3 months. The
(3), 14.6 (6.8), 44.8 (27.7) 10.7 (4.94) and 21.9 (19.14), effect is largely driven by increases in DNMC. Multivariate
respectively. analyses suggests a positive association (p<0.01, R-squared:
OLS analyses support statistically significant (p<0.01) 0.29-0.48) between DS and total DMC and DNMC with a
associations between DS and the other clinical severity one point rise in DS associated with 8-21% increase in
measures. A one point increase in MMSE and DAD was costs.
associated with DS point decreases of 0.292 (R-squared: Conclusions: Patient dependence on others, as measured by
0.44) and 0.089 point (R-squared=0.68). A one point the DS, provides information for explaining variations in
increase in NPI and CDR-SOB was associated with DS cost of care, in particular direct non medical care costs.
increases of 0.046 (R-squared: 0.09) and 0.469 (R squared:
0.59).
Conclusions: Patient dependence, as assessed by the DS,
responds to changes in functional disability, cognitive
impairment and less significantly to changes in behavioural
problems.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 119

P1061 P1062
Hypothyroidism and cognition Pro-apoptotic function of Pin1-mediated
W.-D. Mller1, G. Brandt1, S. Sprenger1, P. Kropp2 Notch activation
1HG Creutzfeldt Institute, Kiel, 2University Medicine, S.-H. Baik, D.-G. Jo
Institute of Medical Psychology and Medical Sociology, School of Pharmacy, Sungkyunkwan University, Suwon,
Rostock, Germany Republic of Korea
Introduction: In our memory consultation a lot of
comorbidities have been observed in patients with cognitive
impairment. Besides cardiological disorders, angiopathies, P1063
and diabetes mellitus patients with thyroid dysfunction were Calsenilin contributes to neuronal cell
observed who also suffered from cognitive problems. In a death in ischemic stroke through Notch
recent study, Resta et al. (2012) point out, that in the elderly,
subclinical hypothyroidism is associated with cognitive
signalling
impairment, but its impact on specific aspects of cognition S.-H. Baik, J.-S. Park, D.-G. Jo
(long-term memory and selective attention) is less evident. School of Pharmacy, Sungkyunkwan University, Suwon,
Aim of our study is to quantify the occurrence of Republic of Korea
hypothyreodism in patients with cognitive disorders.
Methods: 750 patients were collected who consecutively
visited our memory consultation. Median age was 79 years
P1064
(female: 52%). For quantifying cognitive dysfunction all Oxidative lipid modification of -secretase
patients were tested by Mini-Mental-State-Examination in Alzheimers disease
(MMSE, Folstein, 1990) and were graded into mild, median, S.-H. Baik, A.-R. Gwon, J.-S. Park, D.-G. Jo
or severe affected. School of Pharmacy, Sungkyunkwan University, Suwon,
Results: Within the last five years 32 Patients (4,3%) Republic of Korea
suffered from hypothyroidism (70% mild). No differences
were seen in frequency of female or male patients.
Hypothyroidism was correlated with mild cognitive P1065
impairment in MMSE. After euthyroid substitution Striatal morphology as a biomarker in
cognition was improved.
Discussion: Especially in elderly patients hypothyroidism neurodegenerative disease: morphometric
may be correlated with cognitive impairment. In these methods for detecting and monitoring
patients cognitive functions can often be improved after disease impacting on corticostriatal
euthyroid substitution. Despite of the small frequency of circuits
occurrence hypothyroidism should be excluded especially
J.C.L. Looi1, M. Walterfang2
in patients with mild cognitive impairment. 1Academic Unit of Psychological Medicine, Australian
Reference: Resta F, Triggiani V, Barile G, et al. Subclinical National University Medical School, Canberra, ACT,
Hypothyroidism and Cognitive Dysfunction in the Elderly. 2Melbourne Neuropsychiatry Centre, Melbourne University,
Endocr Metab Immune Disord Drug Targets. 2012 Mar 5. Melbourne, VIC, Australia
[Epub ahead of print].

P1066
The effect of Thai traditional music on
cognitive function, psychological health
and quality of sleep among older Thai
individuals with dementia
P. Sithinamsuwan1, S. Saengwanitch1,
A. Pinidbunjerdkool1, M. Mungthin2
Department of Medicine, Phramongkutklao Hospital,
1

Department of Parasitology, Phramongkutklao College of


2

Medicine, Bangkok, Thailand

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


120 Posters, Sunday 9 September

P1067 P1071
An MEG study on the effects of medical Effectiveness of galantamine in patients
food on brain networks in patients with with mild to moderate dementia:
Alzheimers disease 12 months outcome data
E.C. van Straaten1, H. de Waal1, M.M. Lansbergen2, U. Richarz1, M. Djelani2, K. Rettig3, B. Schauble2,
P. Scheltens1, R.L. Wieggers2, P.J. Kamphuis2, C.J. Stam1 M. Gaudig4, E. Jedenius5
1Alzheimer Centre, VU University Medical Centre, 1Medical Affairs, Janssen Cilag, Baar, Switzerland, 2Medical

Department of Neurology, Amsterdam, 2Nutricia Advanced Affairs, Janssen Cilag, Neuss, 3Biostatistics, GEM,
Medical Nutrition, Danone Research - Centre for Specialised Meerbusch, 4Health Economics, Janssen Cilag, Neuss,
Nutrition, Wageningen, The Netherlands Germany, 5Janssen Cilag Sweden, Gteborg, Sweden

P1068 P1072
Human neural stem cells over-expressing Tooth loss and cognitive decline
choline acetyltransferase gene improve S.-H. Suk1, Y.-J. Kim2, I.-S. Koh3, Y.-K. Minn4,
cognitive and physical activities of ageing J.-H. Lee5, S.-H. Hwang6, J.-H. Park7
1Department of Neurology, Wonkwang University Sanbon
mice
Medical Center and Ansan Municipal Geriatric Hospital,
D. Kim1, J. Kyung1, D. Park1, Y.-H. Yang1, E.-K. Choi1, Gunpo-si, 2Neurology, Sungae Hospital, 3Neurology,
H.J. Lee2, S.U. Kim3, Y.-B. Kim1 National Medical Center, 4Neurology, Hallym University
1College of Veterinary Medicine, Chungbuk National
Hangang Sacred Hospital, Seoul, 5Neurology, National
University, Cheongju, 2Medical Research Institute, Chung- Health Insurance Coorperation Ilsan Hospital, Koyang,
Ang University Medical School, Seoul, Republic of Korea, 6Neurology, Hallym University Gangnam Sacred Heart
3Division of Neurology, University of British Columbia
Hospital, 7Neurology, Inje University Sangae Paik Hospital,
Hospital, Vancouver, BC, Canada Seoul, Republic of Korea

P1069 P1073
Dementia and thrombolysis: a systematic
Supporting synapse formation and
review
function in Alzheimers disease:
K. Murao, D. Leys, R. Bordet, F. Pasquier, C. Cordonnier
Departments of Neurology and Pharmacology, University
mechanism of action of the specific
Lille North of France (EA 1046), CHU Lille, France nutrient combination Fortasyn Connect
J.W. Sijben1, P.J. Kamphuis1,2, M. deWilde1,
R.J. Hageman1, L.M. Broersen1, M. Groenendijk1
P1070 1Nutricia Advanced Medical Nutrition, Danone Research -
Disease progression in patients with mild- Centre for Specialised Nutrition, Wageningen, 2Utrecht
to-moderate Alzheimers disease treated Institute for Pharmaceutical Sciences (UIPS), Utrecht
University, Utrecht, The Netherlands
with galantamine: results of a 2-year open
label study
U. Richarz1, M. Djelani2, M. Gaudig3, K. Rettig4,
P1074
B. Schauble2, E. Jedenius5 The awareness of everyday memory
1Medical Affairs, Janssen Cilag, Baar, Switzerland, 2Medical deficits in patients with Parkinsons
Affairs, 3Health Economics, Janssen Cilag, Neuss,
4Biostatistics, GEM, Meerbusch, Germany, 5Janssen Cilag
disease
Sweden, Gteborg, Sweden I. Tamura1,2, S. Hamada2, S. Homma2, H. Soma2,
A. Takei2, K. Hamada2, F. Moriwaka2, K. Tashiro2
1Communication Disorders, Health Sciences University of

Hokkaido, 2Neurology, Hokuyukai Neurological Hospital,


Sapporo, Japan

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 121

P1075 P1079
Neuroprotective effect of edaravone Hypertension: a risk factor for Alzheimers
against hydrogen peroxide induced disease?
neuronal cell death M. BenDjebara1,2, I. Belhouane1,2, I. Kacem1,2,
J. Liu1, Z. Zhao1, S. Huang2, S. Xiao1, B. Zhang1 I. BenArbia1,2, I. Rebai1, Y. Hizem1,2,
1Dept. of Neurology, Sun Yat-sen Memorial Hospital, A. Gargouri-Berrechid1,2, R. Gouider1,2
Guangzhou, 2Dept. of Neurology, Hainan Provincial Peoples 1Department of Neurology, Razi Hospital, La Manouba,
Hospital, Haikou, China 2Research Unit of Clinical Neurophysiology and
Electrodiagnosis 03/UR/08-09, Tunis, Tunisia

P1076
Hippocampal presynaptic alterations are
P1080
associated to amyloid plaques in young Genetic genomics analysis of Ptgs2 gene
PS1/APP Alzheimer mice in BXD recombinant inbred mouse
R. Sanchez-Varo1, E. Sanchez-Mejias1,
hippocampus
L. Trujillo-Estrada1, V. De Castro1, S. Jimenez2, Y. Chen, Y. Zhang, Y. Li, X. Wang, L. Lu
A.J. Jimenez1, J.M. Garcia-Verdugo3, M. Vizuete2, Nantong University, Nantong, China
J.C. Davila1, J. Vitorica2, A. Gutierrez1
1Cell Biology, Faculty of Sciences, University of Malaga/ P1081
CIBERNED, Mlaga, 2Biochemistry and Molecular Biology,
Faculty of Pharmacy, University of Seville/CIBERNED, Biochemical alterations of tau protein as
Seville, 3University of Valencia/CIBERNED, Valencia, Spain a healing physiological compensatory
mechanism of cytoskeleton integrity and
P1077 brain neuroplasticity in Alzheimers
disease and other types of
Temporal pattern of neuritic plaques
neurodegeneration
deposition in an app751sl/ps1m146l
A. Koudinov1,2, N. Koudinova1,2, T. Berezov1,2
Alzheimer transgenic model 1Biochemistry, TT Berezov Laboratory, Russian People
L. Trujillo-Estrada1, R. Sanchez-Varo1, Friendship University, 2Orechovich Institute of Biomedical
E. Sanchez-Mejias1, D. Baglietto-Vargas1, Chemistry, Russian Academy of Medical Sciences, Moscow,
I. Moreno-Gonzalez1, S. Jimenez2, M.L. Vizuete2, Russia
J.C. Davila1, J. Vitorica2, A. Gutierrez1
Cell Biology, University of Malaga/CIBERNED, Mlaga,
1

Biochemistry and Molecular Biology, University of Seville/


2 P1082
CIBERNED, Sevilla, Spain Role of the cell adhesion molecule F3/
contactin in hippocampal synaptic
P1078 plasticity and memory in old mice
Clinical characteristics of Alzheimers D. Puzzo1, L. Privitera1, D. Furnari1, A. Bizzoca2,
disease at the initial visit at the Belgrade G. Gennarini2, A. Palmeri1
1Bio-Medical Sciences - Section of Physiology, University of
Memory Centre: a Serbian report Catania, 2Department of Basic Medical Sciences, University
T. Stojkovic, I. Despotovic, G. MandicStojmenovic, of Bari, Italy
E. Stefanova, V.S. Kostic
Memory Centre, Neurology Clinic Clinical Center of Serbia,
Belgrade, Serbia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


122 Posters, Sunday 9 September

P1083 P1087
Vessel wall changes on ultrasound in Estradiol is neuroprotective against the
renal patients with intellectual decline neurotoxic effects of -amyloid (25-35)
T.J. Tegos1, G. Dimas2, C. Pitsalidis1, A. Valavanis1, in ageing female rat brains
A. Chatziapostolou1, A. Papadimitriou1, P. Kumar, R.K. Kale, N.Z. Baquer
I. Chrysogonidis3, D. Grekas2, A. Orologas1 School of Life Sciences, Jawaharlal Nehru University, New
1A Department of Neurology, AHEPA Hospital, 2First Delhi, India
Medical Propaedeutic Department, AHEPA Hospital,
3Radiology Department, AHEPA Hospital, Aristotelian

University of Thessaloniki, Greece P1088


How do patients get their information?
P1084 W.-D. Mller1, G. Brandt1, S. Sprenger1, P. Kropp2
1HG Creutzfeld Institute, Kiel, 2University Medicine, Institute
Neuroprotective effects of of Medical Psychology and Medical Sociology, Rostock,
metallothionein-I against amyloid toxicity: Germany
glial connections
J.-H. Kim1, Y.-P. Nam1, S.-M. Jeon1, J. Park1, H.-S. Han1, P1089
K. Suk1,2
1Kyungpook National University School of Medicine, 2Brain To determine the level of antioxidant
Science & Engineering Institute, Daegu, Republic of Korea markers in guinea pigs exposed to
huperzine A
P1085 L. Drtinova, M. Pohanka, V. Sepsova
The validity of the MoCA-K in non- Faculty of Health Sciences, University of Defense, Hradec
Krlov, Czech Republic
amnestic MCI
J.-Y. Ahn1, J.-W. Cho2, J.-M. Chung3, H-T. Kim4
1Neurology, Seoul Medical Center, 2Neurology,
Sungkyunkwan University School of Medicine, 3Neurology,
Seoul Paik Hospital, Inje University College of Medicine,
4Hanyang University Hospital, Seoul, Republic of Korea

P1086
Neuroprotective and anti-ageing roles of
dehydroepiandrosterone in aging male rat
brains
P. Kumar, R.K. Kale, N.Z. Baquer
School of Life Sciences, Jawaharlal Nehru University, New
Delhi, India

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 123

Autonomic nervous system disorders P1091


Abdominal compression compensates for
P1090 compromised sympathetic superior
Electrogastrography in pure autonomic mesenteric artery innervation and
failure restores blood pressure in hypotensive
D.A. Low1,2, E. Vichayanrat1,2, W. Seligman1,3, familial dysautonomia patients
Y. Yamanaka4, M. Asahina4, C.J. Mathias1,2 E.C. Ehmann1, J. Koehn1, S. Moeller1, R. Baltadzhieva2,
1Medicine, Imperial College London, 2Autonomic Unit, F.B. Axelrod3, M.J. Hilz1,2
University College London Hospital, London, 3Department Department of Neurology, University of Erlangen-
1
of Physiology, Anatomy and Genetics, University of Oxford, Nuremberg, Erlangen, Germany, 2Departments of Neurology,
Oxford, UK, 4Department of Neurology, Chiba University, Medicine, Psychiatry, 3Dysautonomia Center, New York
Chiba, Japan University, New York, NY, USA
Gastric motility is controlled, in part, by the autonomic Background: Familial Dysautonomia (FD) patients have
nervous system. Gastrointestinal symptoms can occur in severe orthostatic hypotension (OH) that recovers faster
Pure Autonomic Failure (PAF), which is characterized by upon supine-repositioning with than without abdominal-
postganglionic sympathetic dysfunction. No studies have compression. Influences of abdominal-compression on
investigated gastric motility in PAF patients however. splanchnic perfusion are unknown but may contribute to
Aim: To evaluate gastric motility by recording gastric blood pressure recovery.
myoelectrical activity using cutaneous electrogastrography Therefore we assessed superior mesenteric artery (SMA)
(EGG) before and after standard liquid meal ingestion in blood-flow changes during orthostasis, supine-repositioning
PAF. without and with abdominal-compression.
Methods: 7 PAF patients (669 yr) and 5 healthy individuals Methods: In 9 FD-patients (17.83.9 years) and 13 controls
(665 yr) ingested a 300ml standard liquid meal (20g (18.85 years) we monitored mean blood pressure (BP) and
glucose, 60g Complan and milk) whilst supine. Gastric heart rate (HR). Using real-time Doppler ultrasound, we
myoelectrical activity was measured using four-channel assessed cross-sectional SMA areas (SMA-area), time-
EGG. Running spectral analysis was performed using Fast average velocities (SMA-TAV), SMA blood-flow (SMA-
Fourier Transformation. Dominant frequency (DF) was BF), SMA-vascular resistance (SMA-VR=BP/SMA-BF),
defined as the frequency at which the overall power during supine-rest, standing, supine-repositioning, another
spectrum showed peak power between 2.0-4.0 cycles per supine-rest, second standing, and supine-repositioning with
minute (cpm). The instability coefficient of dominant abdominal-compression by leg elevation and flexion
frequency (ICDF) was calculated as the ratio of the standard towards the thorax. Differences between groups and
deviation and mean of the peak frequencies. positions were assessed by ANOVA and post-hoc testing
Results: There was no difference between groups in (significance: p<0.05).
baseline pre-prandial DF (2.80.1 vs. 3.00.3 cpm) and the Results: FD-patients had pronounced OH without HR
minimum and maximum post-prandial DF (both P>0.05). changes during positional changes. Controls had stable BPs
There was a borderline interaction effect of time and group and significant HR increase upon standing and HR decrease
for ICDF (P=0.086). ICDF was higher in PAF at baseline upon supine-repositioning. Upon standing, SMA-area,
(10.27.9 vs. 2.30.4%) and remained unchanged early SMA-TAV, SMA-BF decreased in both groups; yet, SMA-
(0-15 min, 12.55.9%) and late (30-45 min, 9.49.5%) area decreased more in controls than in patients. SMA-VR
post-prandial. In contrast, ICDF increased in controls early increased significantly in controls only. Upon repositioning,
post-prandial (11.03.9%) and returned to baseline during SMA-area, SMA-TAV, SMA-BF re-increased significantly
late post-prandial (1.90.6%). in both groups. SMA-VR re-increased in controls and
Conclusion: Irregular gastric myoelectrical activity in PAF remained unchanged in patients. With abdominal-
patients, as suggested by high ICDF and lack of post- compression, both groups had similar SMA-TAVs and
prandial ICDF changes, suggest gastric dysrhythmia in PAF. smaller SMA-areas; but only patients had higher SMA-VRs
and lower SMA-BFs than without compression.
Conclusion: Upon standing, FD-patients cannot adequately
increase SMA-VR with subsequent SMA-constriction.
Abdominal-compression compensates def icient
sympathetic splanchnic vasoconstriction and lowers SMA-
BF in patients. The reduced blood-flow towards the
splanchnic bed contributes to accelerated BP-recovery.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


124 Posters, Sunday 9 September

P1092 P1093
Direct evidence of sympathetic nervous Tako-Tsubo cardiomyopathy: direct
system hyperactivity in patients with evidences of sympathetic nervous system
vasospastic angina hyperactivity
F. Despas, J. Van Rothem, N. Boudou, A. Vaccaro, F. Despas1, A. Vaccaro2, M. Lebrin2, C. Delmas2,
M. Lebrin, D. Carrie, M. Galinier, M. Elbaz, A. Pathak, M. Galinier2, A. Pathak2, J.-M. Senard2
J.-M. Senard 1Clinical Pharmacology, 2Toulouse University Hospital,

Toulouse University Hospital, Toulouse, France Toulouse, France


The pathogenesis of vasospastic angina remains Tako-Tsubo Cardiomyopathy (TTC) is an acute reversible
incompletely elucidated. Among multiple mechanisms, condition that involves left ventricular apical ballooning
vagal withdrawal can act as a trigger for spontaneous and mimics acute myocardial infarction with no detectable
coronary spasm, changes in sympathetic activity have also coronary arterial disease. TTC typically affects aged post-
been suggested. To date no direct assessment of sympathetic menopausal women and is usually triggered by emotional or
nerve activity (MSNA) has been performed. physical stress. The exact pathophysiology remains
We evaluated haemodynamic parameters and MSNA in 31 unknown but data suggest a link between sympathetic
patients: 15 having definite vasospastic angina confirmed hyperactivity and TTC. Up to now, no direct evidence of
by ergonovine provocation test during angiography and 16 sympathetic hyperactivity has been established. The aim of
matched patients (age, gender, body mass index, risk our study was to determine by microneurography if patients
factors) with negative response to provocation test. with TTC present an increase of muscle sympathetic nerve
Parameters were collected during baseline and during a activity (MSNA) in comparison to matched heart failure
mental stress known to further increase MSNA. controls.
During baseline period, blood pressure and heart rate did We enrolled 13 TTC patients (80.12.1 years, all female,
not significantly differ between groups. Baseline MSNA Body Mass Index: 23.20.8kg/m, Left Ventricular Ejection
was significantly increased in patients with vasospam Fraction: 402%) and 13 control patients matched for age,
compared to non-vasospasm patients (56.81.3 vs. 50.31.8 sex, BMI, LVEF, renal function and haemoglobinemia.
burst/min; p<0.001). During mental stress period, spasm Within 36 hours after admission, all patients underwent a
patients presented a significant higher mean blood pressure microneurography and an arterial baroreflex gain
and MSNA in comparison to non-spasm patients (66.11.8 assessment (slope of the relationship between MSNA and
vs. 54.31.8 burts/min; p<0.001). For spasm patients group, diastolic blood pressure). There is no difference between
mental stress test produced a significant increase of blood groups on haemodynamics parameters (BP and HR) and
pressure, heart rate, oxygen saturation and MSNA (56.81.3 oxygen saturation. TTC patients presented a significant
vs. 66.11.8 bursts/min, p<0.001). For non-spasm patients increase of sympathetic activity (66.32.7 vs. 55.62.6
group, mental stress test produced a significant increase of bursts/min; p=0.0089). Arterial baroreflex gain is
heart rate, oxygen saturation and MSNA (50.31.8vs significantly decreased compared to control patients
54.31.8 bursts/min, p<0.05), but no significant (1.20.3 vs. 2.50.4 % MSNA/mmHg; p=0.005).
modification on blood pressure parameters. This study showed for the first time with a direct technique,
Our results show a direct evidence of increased sympathetic that TTC patients present a sympathetic hyperactivity. The
activity in patients with vasospastic angina, during mental increase of sympathetic nerve activity is associated to a
stress. This propensity to further increase MSNA during decrease of arterial baroreflex gain. During the acute phase,
stress may play a key role in the pathogenesis of coronary the benefit of adrenergic antagonist has to be evaluated.
spasm.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 125

P1094 P1095
Prolonged survival in multiple system Quantitative cardiovascular autonomic
atrophy (MSA) function in patients with primary chronic
P. Guaraldi1, F. Mastrolilli2, A. Cecere1, autonomic failure
G. Calandra-Buonaura1, L. Sambati1, G. Barletta1, E. Vichayanrat1,2, D. Low1,2, K.B. Nilsen3,4, V. Iodice1,2,
F. Provini1, F. Vernieri2, P. Cortelli1 C.J. Mathias1,2
1Dipartimento di Scienze Neurologiche, IRCSS Istituto delle 1Autonomic & Neurovascular Medicine Unit, Imperial
Scienze Neurologiche di Bologna, Alma Mater Studiorum - College London at St. Marys Hospital, 2Autonomic Unit,
Universit di Bologna, Bologna, 2Department of Neurology, National Hospital for Neurology and Neurosurgery, London,
Campus Bio-medico University, Rome, Italy UK, 3Department of Neuroscience, Norwegian University of
Introduction: MSA is a sporadic neurodegenerative Science and Technology, Trondheim, 4Department of
disorder characterized by dysautonomia associated with Neurology, Section for Clinical Neurophysiology, Oslo
parkinsonian (MSA-P) or cerebellar (MSA-C) features with University Hospital, Oslo, Norway
a mean survival of 7-9 years. Few reports described patients It can sometimes be difficult to differentiate the diagnosis
surviving more than 15 years (1). We retrospectively between Parkinsons disease with autonomic failure
reviewed the medical records of 108 MSA patients, assessed (PD+AF), Pure Autonomic Failure (PAF) and Multiple
in our Clinic between 1991 and 2012 in search of patients System Atrophy (MSA), owing to overlapping autonomic
with a disease history of >15 years. features, such as orthostatic hypotension (OH).
Methods: We identified 6 patients (5 MSA-P, 1 MSA-C, 4 Aim: We investigated the usefulness of a variety of
males). Demographic, clinical and polysomnographic data quantitative cardiovascular autonomic measures in
were analyzed. Up-to date follow-up information was distinguishing these patients with primary chronic AF.
obtained by telephonic interviews. Methods: Patients underwent cardiovascular autonomic
Results: MSA-P presented at 507 years (range 41-58 function tests that included head-up-tilting, Valsalva
years) with a rigid-bradikinetic syndrome in 3 patients and manoeuvre (VM) and deep breathing (HRDB). Blood
genito-urinary dysfunction in 2. MSA-C developed at 49 pressure (BP) and heart rate (HR) were continuously
years with cerebellar dysfunctions. Nocturnal stridor was recorded and analyzed off-line. The adrenergic component
present in 2 patients (1 MSA-C) that underwent of baroreflex sensitivity (BRS-a), blood pressure recovery
tracheostomy. All patients were bedridden at their final time during VM (PRT) and Valsalva ratio (VR) were derived
assessment (145 years after onset, range 8-20). One patient from the VM. All measures were compared between patients
with MSA-P and stridor is still alive; mean disease duration and normal controls.
was 203 years (range, 16-23 years) in the whole population Results: Supine systolic BP (SBP) was not different
and 214 years (range, 18-23 years) in the subgroup with between groups. OH was present in patients but not in
stridor. Causes of death were respiratory arrest (n=2) or controls (p<0.05). Absolute SBP was significantly lower
unknown (n=3). and the change in SBP was significantly greater during
Conclusions: Few previous studies reported MSA patients tilting in PAF compared to MSA (p<0.05). Responses in
with a survival >15 years, but with poor clinical description. PD+AF were intermediate. BRS-a and VR were significantly
5.6% of our MSA had a disease history of >15 years. lower in all patient groups compared to controls (p<0.05)
Gathering clinical information regarding MSA patients with but there was no difference between patient groups (p>0.05).
prolonged survival may help to identify useful prognostic MSA patients had a significantly prolonged PRT compared
and protective factors. to PD+AF (p=0.035) and HRDB was significantly lower in
1. Kim et al. (2011) Survival of Korean patients with PAF compared to MSA (p=0.04).
multiple system atrophy. Movement disorders 26(5):909- Conclusion: PRT may be useful for differentiating MSA
912 from PD+AF and HRDB for PAF from MSA. These
findings suggest that sympathetic function is more affected
in MSA compared to PD+AF while parasympathetic
dysfunction is more prominent in PAF than in MSA.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


126 Posters, Sunday 9 September

P1096 P1097
Immunohistochemical analysis reveals Diagnostic significance of urethral
sympathetic column involvement in the sphincter electromyography in patients
SOD1 (G93A) transgenic mouse model of with multiple system atrophy
amyotrophic lateral sclerosis X. Qi1, L. Li2, W. Yao2, F. Qiu2
B. Kandinov1, A.D. Korczyn2, V.E. Drory2, D. Offen3,
1 Neurology, 2Navy General Hospital, Beijing, China
N.C. Grigoriadis4, O. Olga Touloumi4 Objective: To evaluate the significance of urethral sphincter
1Pharmacology and Physiology, Tel-Aviv University, 2Tel- electromyography (US-EMG) for the diagnosis of multiple
Aviv Sourasky Medical Center, 3Tel-Aviv University, Tel Aviv, system atrophy (MSA).
Israel, 4Aristotle University of Thessaloniki, Thessaloniki, Methods: 15 patients who were diagnosed as MSA and 17
Greece non-MSA patients were examined as controls were enrolled
Amyotrophic lateral sclerosis (ALS) is a progressive and in the study and US-EMG was performed in both groups.
fatal disorder caused by degeneration of the motor neurons Spontaneous activities during relaxation, parameters of
in the cerebral cortex, brain stem and spinal cord. Several MUPs mean duration and amplitude, percentage of
clinical reports indicated dysfunction of sympathetic and polyphasicity, satellite potential, recruited pattern and
parasympathetic innervation in ALS patients. In addition, amplitude during strong contraction were recorded and
we have recently reported elevated heart rate and blood analyzed.
pressure in transgenic (TG) mice carrying the SOD1 mutant Results: US-EMG changes of various abnormalities were
form of the human SOD1 transgene (SOD1G93A) even found in 13 cases of the MSA group (86.7%). There was
prior to the appearance of motor symptoms. In order to significant difference of electromyographic findings
further elucidate the underlying mechanisms of autonomic between the MSA group and the control group including the
impairment in ALS we performed an immunohistochemical mean duration and amplitude of motor unit action potentials
study of the superior cervical ganglia (SCG) and adrenal (MUAPs) as well as recruited pattern and amplitude during
glands (AG) in TG and wild-type (WT) mice at the age of heavy contraction.
75-80 days. SCG and AG sections were stained with primary Conclusions: US-EMG has certain values for the diagnosis
antibodies against tyrosine hydroxylase (TH) and choline of MSA. It could be used as a routine electrophysiological
acetyltransferase (ChAT). TH expression in AG revealed a method for those suspected of MSA and could be a surrogate
24% decrease in the SOD1G93A group compared to WT of EAS-EMG.
(p<0.0001). ChAT expression in the SCG was reduced by
28% in the SOD1G93A group compared to WT (p<0.0001).
No significant differences were found for TH in SCG or for
ChAT in AG. Since ChAT is predominantly located at pre-
ganglionic nerve terminals which innervate the AG, our
results point to a pre-ganglionic sympathetic denervation as
a potential contributor to the abnormal sympathetic
regulation in ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 127

P1098 P1099
Diagnostic significance of urethral Sleep motor activity in parkinsonism at
sphincter electromyography and external disease onset: a possible biomarker
anal sphincter electromyography in useful for differential diagnosis
patients with multiple system atrophy M. Alessandria, G. Calandra-Buonaura, A. Cecere,
F. Qiu1, X. Qi1, L. Li2, J. Liu2 L. Sambati, R. Terlizzi, P. Guaraldi, F. Provini, P. Cortelli
1 Neurology, 2Navy General Hospital, Beijing, China IRCCS Istituto delle Scienze Neurologiche, University of
Bologna, Bologna, Italy
Objective: To assess the significance of urethral sphincter
electromyography (US-EMG) and external anal sphincter Introduction: Differential diagnosis of parkinsonism at
electromyography (EAS-EMG) for the diagnosis of multiple disease onset may be difficult. Aim of this study is to
system atrophy (MSA). describe videopolysomnographic (VPSG) motor findings in
Methods: US-EMGs and EAS-EMGs were performed in patients with recent onset parkinsonism.
15 patients diagnosed as MSA. Duration, motor unit action Methods: We studied 22 consecutive patients with
potentials (MUAPs) amplitude, and polyphasicity, as well as parkinsonian features and disease duration up to 3 years part
recruited pattern and amplitude during heavy contraction of the motor and non-motor prospective study on
were recorded and analyzed. parkinsonism at onset (BO-ProPark). Patients were
Results: Among 15 patients who were diagnosed as MSA, diagnosed following standard diagnostic criteria as
neural injuries were shown by both US-EMG and EAS- Parkinsons disease (PD, 12 patients), PD plus (PD with
EMG in 13 cases. There was significant difference of cognitive impairment or dysautonomia, 4 patients) and as
electromyographic findings between US-EMG group and parkinsonian syndromes (PS, 6 patients). All patients
EAS-EMG group (P=0.033 and 0.011, for comparison of underwent a full night VPSG, scored by a neurologist
means of MUAPs amplitude and medians of polyphasicity, blinded to the diagnosis.
respectively). Results: All patients showed reduced sleep efficiency with
Conclusions: Although US-EMG may be difficult to normal sleep macrostructure. In 3/6 PS patients apnoea/
perform, US-EMG may have the same specificity as EAS- hypopnoea index was >5. A sustained muscle EMG activity
EMG for the diagnosis of MSA, especially for the diagnosis or an excessive phasic muscle activity during REM sleep
of MSA patients with only urination disorders due to were shown in 14 patients (4/12 PD; 4/4 PD plus; 6/6 PS).
involvement of Onufs nucleus. RBD episodes were recorded in 5 patients (3/4 PD plus; 2/6
PS); 6 patients showed excessive fragmentary myoclonus
(1/12 PD; 1/4 PD plus; 4/6 PS); PD plus and PS patients
showed whole body jerks during sleep; in 14 patients the
PLMS index was >15 (7/12 PD; 2/4 PD plus; 5/6 PS).
Conclusion: Our preliminary data suggest that an impaired
sleep motor control is more frequent at disease onset in
patients with PS and PD plus compared to PD patients.
More data are needed to establish if these features may have
a diagnostic value in the differential diagnosis of
parkinsonism.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


128 Posters, Sunday 9 September

P1100 P1101
Quantitative electromyography of the Is cognitive impairment associated with
external anal sphincter in Huntingtons orthostatic hypotension in parkinsonism
disease patients at onset?
S. Podnar1, M. Kolenc2, J. Kobal1 L. Sambati, G. Calandra-Buonaura, F. Oppi, R. Poda,
University Medical Centre Ljubljana, Ljubljana, 2Novo
1
M. Stanzani Maserati, P. Guaraldi, R. Terlizzi,
Mesto General Hospital, Novo Mesto, Slovenia M. Alessandria, L. Solieri, R. Gallassi, P. Cortelli
Aims: In several extrapyramidal diseases Onufs nucleus IRCCS Istituto delle Scienze Neurologiche, University of
degeneration was also demonstrated by histology and Bologna, Italy
electromyography (EMG). Although Huntingtons disease Background: Parkinsonism at onset can be associated with
(HD) patients also often report bladder, bowel and sexual cognitive impairment and autonomic dysfunctions.
dysfunction Onuf s nucleus has not been systematically Objective: To describe the cognitive features of patients
studied in this population. This was the aim of the present with parkinsonism at onset and to investigate their eventual
study. association with orthostatic hypotension (OH).
Methods: From our registrar of patients with genetically Method: We consecutively selected patients with
confirmed HD all patients willing and capable to participate parkinsonism and disease duration up to three years to take
in the study using several standard questionnaires were part in the Bo-ProPark Study (Bologna-motor and non
recruited. Those reporting bladder and/or bowel dysfunction motor Prospective study on Parkinsonism at onset). Each
were also asked to undergo quantitative anal sphincter patient underwent, at baseline and after sixteen months,
EMG. neuropsychological assessment (Brief Mental Deterioration
Results: Of 72 patients (25 men) with genetically con Battery, Stroop Test, Semantic Fluency Task, Beck
firmed HD, 33 reported bladder and/or bowel dysfunction. Depression Inventory) and tilt table test (continuous
Anal sphincter EMG was performed in 14 of them (8 men), monitoring of arterial blood pressure and heart rate) to
33 to 67 years of age (meanSD, 4810 years). Their detect the presence of OH. The clinical diagnosis was made
disease durations were 3 to 15 years (85 years), and the at the second evaluation, according to international
number of huntingtin CAG repeats 40 to 52 (464). In all diagnostic criteria.
but 3 patients decreased tonic activity and/or decreased Results: We recruited 27 patients (21 Parkinsons disease
voluntary activation were found in the anal sphincter. (PD); 2 Progressive Supranuclear Palsy (PSP); 1 Multiple
However, in no patient quantitative EMG abnormalities System Atrophy (MSA); 3 Parkinsonian Syndromes not
were found. otherwise specified (PS)). 13 of 27 patients showed
Conclusions: Our study did not demonstrate EMG signs of neuropsychological impairment (NI). In these patients we
the Onuf s nucleus degeneration in HD patients. Decreased observed 3 different patterns of NI: executive functions
anal sphincter tonic activity and voluntary activation, as impairment (8 patients: 1MSA, 1PS, 6 PD); executive
well as bladder and bowel dysfunction could probably be function, verbal memory and fluency impairment (4
explained by basal ganglia or central autonomic structures patients: 1 PSP, 3 PD); dementia, (1 PSP patient). These
degeneration. patterns were not associated with the clinical diagnosis.
2 of 27 patients (1 MSA and 1 PD) fulfilled the criteria for
OH. These patients were impaired in executive functions.
The other patients with NI did not suffer from OH.
Conclusions: In parkinsonism at onset, we observed three
different patterns of NI which were neither associated with
the clinical diagnosis nor with OH.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 129

P1102 P1103
Heart rate variability in chronic vs. Association of EEG scalp potentials with
frequent migraine patients autonomic innervations of the heart
O. Grosu1, S. Odobescu1, V. Lacusta2, L. Rotaru1, A. Subhani, L. Xia, A.S. Malik
D. Concescu1, I. Moldovanu1,2 Department of Electrical and Electronics Engineering,
Neurology, Institute of Neurology and Neurosurgery, 2State
1 Universiti Teknologi Petronas, Tronoh, Malaysia
Medical and Pharmaceutical University, Chisinau, Moldova Background: Cerebral activation and autonomic nervous
Introduction: Heart rate variability (HRV) is a marker of system have significance in studies such as mental stress.
autonomic activity and reduced HRV correlates with sudden This study aims to analyse variations in electroencephalogram
death. Data on HRV in migraine are inconclusive and (EEG) scalp potentials that may influence autonomic
restricted to episodic type, with reports of increased HRV innervations during video games.
especially in the young. The aim of the study was to evaluate Methods: 12 healthy participants (2 females) were recruited
autonomic cardiac regulation in chronic migraine (CM) in this study. EEG and electrocardiogram (ECG) signals
compared with frequent migraine (FM) patients by mean of were measured during game playing and rest condition.
HRV. Sympathetic (high heart rate (HR)) and parasympathetic
Methods: The study included 109 CM and 61 FM patients (lower normalized high frequency (HFnu)) innervations
corresponding to IHS criteria. In all subjects, a 5-min from ECG were evaluated from heart rate variability (HRV).
electrocardiographic recording during paced breathing was Meanwhile ratio of theta at Fz to alpha at Pz power (Fz()/
performed, and HRV was analyzed in time and frequency Pz()) was calculated from the EEG scalp potentials to
domain according to international guidelines. SDNN measure workload.
represents the standard deviation of the NN (R-R) interval Results: Results show a significant surge in both Fz()/
and reflects the total power of HRV. HRV is reduced when Pz() and HR (p<0.001, paired t-test) and a drop in HFnu
SDNN <100ms. power (p<0.05, paired t-test) as evident in table 1 during
Results: CM presented a reduced SDNN value vs. FM games (0.364, 0.311, 0.361) as compared to the rest
group (56.584.95 vs. 99.0416.17, p<0.05) which reflect condition (0.419).
a reduced HRV. LF (low frequency) was increased
Condition Rest Game level1 Game level 2 Game level3
(53.842.32 vs. 45.193.04, p<0.05) and HF (high
Averagestd 0.4190.18 0.3640.13 0.3110.12 0.3610.17
frequency) reduced (46.162.32 vs. 54.813.04, p<0.05) in
CM. LF/HF ratio representing an index of sympatho - vagal [Table 1. HFnu during rest and games]
balance was higher in patients with CM vs. FM (1.730.23
vs. 1.050.18, p<0.05). Conclusion: It is concluded that enhanced cerebral
Conclusion: Decreased HF suggested a reduced activation (high Fz()/Pz() ratio) is accompanied with
parasympathetic influence, increased LF/HF ratio reflecting increment in sympathetic innervations and decrement in
a sympathetic over activity in the cardio-vascular system in parasympathetic innervations. Moreover, the results indicate
CM patients. The reduced HRV in CM found in our study that correlation of cerebral activation and autonomic
shows a strong relationship between autonomic cardiac innervations can be used to measure mental stress since they
function and central and peripheral sensitization (as a can provide an assessment of workload.
mechanism of migraine chronification) and may provide
some links to ischemic stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


130 Posters, Sunday 9 September

P1104 P1105
The immediate effect of paced breathing 24-hour ambulatory blood pressure
with biofeedback and active cycling on profiles in diabetic autonomic neuropathy,
heart rate variability in patients in the amyloid neuropathy and Holmes-Adie
sub-acute post-stroke phase syndrome
P. Ginsburg1, G. Bartur2, S. Peleg2, M. Katz-Leurer1 E.M. Hagen1,2,3, D.A. Low2,3, E. Vichayanrat2,3,
1 Tel Aviv University, 2Reuth Medical Center, Tel Aviv, Israel V. Iodice2,3, A.P. Owens2, V. Ponnusamy2, M. Peche2,
Introduction: Autonomic instability is a common K. Bleasdale-Barr3, L. Mason3, C.J. Mathias2,3
1Department of Clinical Medicine, University of Bergen,
phenomenon following stroke with signs and symptoms of
hyper stimulation of the sympathetic nervous system. There Bergen, Norway, 2Autonomic & Neurovascular Medicine
are at least two known non pharmacologic methods which Unit, Imperial College Faculty of Medicine, 3Autonomic
can alter autonomic balance; aerobic exercise and heart rate Unit, UCL Institute of Neurology, National Hospital for
variability (HRV) biofeedback, both showed success in the Neurology and Neurosurgery, London, UK
rehabilitation program among cardiac or diabetic patients Objective: The aim of this study was to investigate 24-hour
with autonomic impairment. ambulatory blood pressure monitoring (AMBP) results in
The aim of the present study was to assess the feasibility patients with insulin-dependent diabetic autonomic
and the possible impact of slow abdominal breathing with neuropathy, Amyloid neuropathy and Holmes-Adie
biofeedback and aerobic exercise stimuli on autonomic Syndrome.
regulation, by assessing HRV, among patients at the sub- Design: Data from 24-hour AMBP and activity diaries of 13
acute phase post stroke. diabetic autonomic neuropathy, 15 Amyloid neuropathy and
Methods: 15 patients up to one month post stroke underwent 12 Holmes-Adie Syndrome patients were analysed.
measurements of HRV under three conditions while sitting Diagnoses were verified by medical records.
down: Results: Mean age was 44.316.1 for diabetic autonomic
(a) at rest with self-select breathing frequency, neuropathy, 47.811.8 for Amyloid neuropathy and
(b) performing paced breathing with biofeedback and 56.87.7 years for Holmes-Adie Syndrome. Male
(c) cycling while sitting. percentages were 38.5, 73.3 and 33.3, respectively.
Results: Patients were by mean 10 days post event with Orthostatic hypotension was detected on tilt-table testing in
Barthel mean score of 75/100. Patients could reduce their 46.0, 26.7 and 8.3% of the patient groups, respectively.
breathing rate during paced breathing by mean of 6 breaths Mean daytime vs. night-time systolic/diastolic BP (SBP/
per minute (from 15 to 9) and could tolerate for 9 (3) DBP) was 130/81 vs. 122/72mmHg for diabetic autonomic
minutes in constant cycling. Performing paced breathing neuropathy, 123/81 vs. 113/73mmHg for Amyloid
with biofeedback showed a significant increase in HRV neuropathy and 127/81 vs. 118/72mmHg for Holmes-Adie
time domain parameters as compared to rest, for example; Syndrome. There was an impaired nocturnal systolic blood
RMSSD at rest 314.7ms, during paced breathing pressure profile in 61.5% diabetic autonomic neuropathy,
41.64.5ms p<0.05. Patients exhibit significant decreased 60.0% Amyloid neuropathy and 58.3% Holmes-Adie
HRV parameters during cycling, as compared to rest values Syndrome patients. When assessing the AMBP and diary
(RMSSD during cycling 21.82.5ms, p<0.05). activities, there was a significant SBP and DBP decrease in
Conclusions: Patients post stroke may alter autonomic diabetic autonomic neuropathy patients and a significant
balance by performing paced breathing and cycling exercise. SBP decrease in Amyloid neuropathy and Holmes-Adie
patients, when sitting and/or standing compared to lying (all
p<0.05). There were significant increases in heart rate when
sitting or standing versus lying in all three groups (all
p<0.01).
Conclusion: These findings indicate that 24-hour AMBP
can reveal impaired 24-hour BP profiles and autonomic
dysfunction during postural challenges in insulin-dependent
diabetic autonomic neuropathy, Amyloid neuropathy and
Holmes-Adie Syndrome patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 131

P1106 P1107
Postural tachycardia syndrome (PoTS) Parasympathetic dysfunction in ALS
and 24-hour ambulatory blood pressure patients
and heart rate monitoring H. Lahrmann1, E. Toeglhofer2, W. Struhal3
E.M. Hagen1,2,3, D.A.
Low2,3, E.Vichayanrat2,3,
1Private Practice, 2Department of Biomedical Engineering,
V. Iodice2,3, A.P. Owens3, K. Bleasdale-Barr2, L. Mason2, Technical University, Vienna, 3Autonomic Laboratory and
I. Skeavington2, C.J. Mathias2,3 Department of Neurology, General Hospital, Linz, Austria
1Department of Clinical Medicine, University of Bergen, Background: Amyotrophic lateral sclerosis (ALS) is a
Norway, 2Autonomic Unit, UCL Institute of Neurology, neurodegenerative disease with progressive loss of
National Hospital for Neurology and Neurosurgery, motoneurones. Recent studies have shown involvement of
3Autonomic & Neurovascular Medicine Unit, Imperial
non-motor structures within CNS, e.g. frontotemporal
College Faculty of Medicine, London, UK dementia, autonomic dysfunction. The correlation of
Objective: Postural tachycardia syndrome (PoTS) is respiratory, bulbar and parasympathetic dysfunction has not
characterized by orthostatic tachycardia (30 beats/min), in been clarified yet.
the absence of orthostatic hypotension, with orthostatic Aim of the study: We investigated the prevalence of
intolerance. We analyzed data from 24-hour ambulatory parasympathetic dysfunction in ALS patients compared to a
blood pressure monitoring (AMBP) and heart rate (HR) in control group and its association with bulbar symptoms and
patients with PoTS. respiratory failure.
Study design: Data from 24-hour AMBP, HR and diary Methods: 34 ALS patients were included and divided into
activities of 15 women with PoTS were analysed. The diary four subgroups according to their bulbar status and their
comprises a schedule of autonomically focused activities lung function. The control group consisted of 11 age
design to provoke changes in BP and HR. All patients matched healthy persons. All study participants underwent
underwent a 60 head-up tilt prior to the AMBP as part of the following procedures: history taking, neurological
their autonomic cardiovascular investigations. examination, lung function test, blood gas analysis,
Results: Mean age was 35.47.8 years. All patients met the autonomic testing (Ewing battery).
criteria for PoTS on head-up tilt. Results: A significantly higher prevalence of
Mean (SD) daytime vs. night-time systolic/diastolic BP parasympathetic dysfunction was observed in ALS patients
(SBP/DBP) was 118(12)/77(11) vs. 100(10)/59(10) compared to the control group. Bulbar involvement did not
mmHg (p<0.001). Mean (SD) daytime vs. night-time heart correlate with the measured autonomic parameters. Patients
rate (HR) was 94(21) vs. 74(14) beats/min (p<0.001). A with respiratory failure showed significantly higher resting
normal nocturnal systolic blood pressure fall was evident in heart rates (657 vs. 8527 bpm, p<0.018) and a significant
13/15 patients. Combining AMBP with diary activities reduction of heart rate variability (e.g. coefficient of
showing significant increases in SBP, DBP and HR during variation 4.72.1 in control group and 1.91.0 in respiratory
sitting and/or standing vs. lying (p<0.001). 13 patients met failure group, p<0.034).
the criteria for PoTS during these manoeuvres. There was Discussion: The results of this study indicate a significantly
no evidence for postprandial tachycardia or hypotension. higher rate of parasympathetic dysfunction in ALS patients.
SBP and HR were significantly higher during post- vs. pre- Dysautonomia correlates more with respiratory failure than
exercise standing (p<0.001 and p=0.001, respectively). with the patients bulbar status. The autonomic dysfunction
Conclusion: Except for the abnormal responses to standing, in ALS seems to be related to the progression of the disease,
these findings indicate normal 24-hour BP and HR profiles reflected by the degree of respiratory impairment.
in most PoTS patients. The use of 24-hour AMBP combined
with diary recording can be a supportive diagnostic tool
when evaluating patients with PoTS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


132 Posters, Sunday 9 September

P1108 P1109
Postural tachycardia syndrome, joint Analysis of heart rate variability to assess
hypermobility syndrome and Chiari type I the background lead exposure in children
malformation: clinical description and during orthostatic test and exercises
autonomic evaluation S. Tymchenko, E. Evstafyeva
V. Iodice1, D. Low1, R. Grahame2, C. Mathias1 Physiology Department, Crimea State Medical University
1Imperial College London, 2University College Hospital, named after S. I. Georgievsky, Simferopol, Ukraine
London, UK Heart rate variability (HRV) method is already an established
Postural Tachycardia Syndrome (PoTS) is characterized by tool in early detection of disorders of the autonomic cardiac
an increase in heart rate of 30 beats/min on head-up tilt/ regulatory system. The present study was designed to
standing without orthostatic hypotension associated with investigate whether the background lead exposure could
orthostatic intolerance (OI). PoTS can be associated with modify HRV during orthostatic test and exercises. Time
Joint Hypermobility Syndrome (JHS), a heritable disorder (SDNN, RMSSD, pNN50) and frequency (TP, VLF, LF, HF,
of connective tissue. The Chiari type I malformation (CM1) LF/HF, LFn and HFn) domain indexes of RR interval
is characterized by herniation of cerebellar tonsils 3-5 mm variability were assessed in 30 healthy 10-11 year old
below the plane of the foramen magnum and can present children who completed orthostatic test and exercise (step-
with a variety of clinical symptoms, frequently including test).
occipital headaches, other rare presentations are OI and A normal individual reaction was observed with a
syncope. An association between PoTS, CM1 and EDS III characteristic HRV altered in a predictable manner in
has not been previously described. response to orthostatic test and exercise (comprising a
Objective: To evaluate the clinical features and significantly reduced RRNN, pNN50, HFn and elevated
cardiovascular autonomic function in patients with OI and LFn, LF/HF). While SDNN was significantly higher during
CM1. exercises, that can indicate changes in the state of the
Methods: We described 5 females with OI and CM1. sympathovagal balance.
Symptoms were recorded and patients underwent autonomic During orthostatic test and exercises significantly higher
function and MRI brain assessments. HRV (increased RMSSD, pNN50, HF and HFn and reduced
Results: Mean age was 24.28.2 years. The most frequent LF/HF, LFn) was associated with elevated lead levels
presenting symptoms were: OI (palpitations, fatigue and (0.31<rs<0.48; 0.01<p<0.05) measured in hair by X-ray
syncope, 60%) and headache (40%). Additional symptoms spectrophotometry. No correlation was present between
were: bladder (60%) and gastrointestinal dysfunction HRV parameters recorded at rest and lead concentration.
(60%). Autonomic function tests showed PoTS features in These data indicate that effects of background lead exposure
all patients. All patients met the Brighton criteria for JHS on HRV were found during orthostatic test and exercise and
and MRI brain scans confirmed CM1. 4 patients underwent mainly for the spectral components of HRV. Results suggest
surgical decompression with the following clinical that background lead exposure may increase vagal tone,
outcomes: temporary improvement of headache (1 patient), resulting in elevated HRV.
improvement of PoTS (1 patient) and unchanged symptoms
(2 patients).
Conclusion: PoTS could be the presenting feature in
patients with CM1, the symptoms of which only partially
resolve after surgical intervention. Postural tachycardia
warrants investigation of CM1 as part of the cluster of
PoTS/JHS patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 133

P1110 P1111
Effects of martial arts on heart rate Reproducibility of sympathetic skin
variability and hostility response: within-subject consistency of
S. Tymchenko, K. Zavgorodnyaya wave-form and size across different
Physiology Department, Crimea State Medical University stimulation modalities
named after S.I. Georgievsky, Simferopol, Ukraine
M. Toyokura
Hostility has been associated with an increased risk for Rehabilitation Medicine, Tokai University Oiso Hospital,
cardiovascular disease. This study aims to investigate Oiso, Japan
whether this relationship could be explained by impaired Objective: The aim of the present study was to investigate
cardiac autonomic control assessed by heart rate variability the specific, intra-subject consistency of the waveform type
(HRV). Measures of HRV were obtained in 25 martial arts and size of sympathetic skin response (SSR) evoked by
(aikido) participants and 28 age matched (17-25 years) different modalities of stimulation. The results were
healthy non-athlete control subjects from both time- and discussed based on the neurophysiological mechanism of
frequency analysis of 5 minutes recordings of HRV. Time generating SSR.
domain indexes included mean NN (RRNN), SDNN, Methods: SSRs were obtained from 38 normal subjects
RMSSD and pNN50. Total-, low- (LF) and high (HF) using three different modalities of stimulation: auditory
frequency spectral power were calculated, as well as their (a-SSR), electrical (e-SSR), and magnetic (m-SSR). Four
ratio (LF/HF). Results suggest that resting heart rate was stimuli of each modality were applied. The wave-forms
lower in Aikido athletes. Compared with control subjects were classified into two types, P (positive component larger
athletes had significantly increased SDNN, RMSSD, than the negative component) and N (vice versa) (Toyokura,
pNN50 (p<0.05), showing evidence of increased vagal 1998).
activity. Hostility scores (Buss-Durkee Hostility Inventory) Results: P-type wave-form was less frequent in the a-SSR
showed significant differences between the groups than the e- and m-SSRs. The maximum amplitude of a-SSR
(p<0.001) with higher scores in non-athletes. Aikido was significantly smaller than those of e- and m-SSRs. The
athletes have significantly higher values in the BDHI size of e- and m-SSRs did not significantly differ. The
assault subscale, while controls got significantly higher incidences of the respective waveforms and maximum
values in verbal aggression (p<0.01). Correlation analysis amplitude values showed intra-subject correlation among
revealed that in Aikido athletes verbal aggression had a the SSRs evoked by different types of stimulation.
significant negative correlation with SDNN, RMSSD, HF Conclusions: The SSR waveform and size seemed to be
and positive association with LF/HF (0.44<rs<0.50; rather consistent in individuals even if the stimulation
p<0.05). These findings suggest that HRV and hostility are modality was changed. The results suggested that
affected by exercise, especially martial arts (aikido), which endogenous factors in an individual related to the
can have beneficial effects on the cardiovascular risk development of SSRs, e.g., the individuals emotional state,
profile. susceptibility to the surprise effect, and anatomical
characteristics of sweat glands, were important determinants
of the SSR wave-forms and maximum amplitudes.
Difference in these factors between individuals can explain
a large inter-subject variation of the SSR parameters.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


134 Posters, Sunday 9 September

P1112 P1113
Syncope due to concomitant cardiac Autonomic nervous system reactivity in
disease and neurogenic orthostatic normotensive subjects with a family
hypotension: a case report history of hypertension during Valsalva
C. Di Stefano, V. Milazzo, G. Bruno, S. Maule, F. Veglio manoeuvre
Autonomic Unit and Hypertension Unit, Department of K. Cankar, Z. Melik, M. Strucl
Medicine and Experimental Oncology, University of Turin, Institute of Physiology, Medical Faculty, University of
San Giovanni Battista Hospital, Turin, Italy Ljubljana, Slovenia
Syncope may be classified into several forms, characterized Introduction: This study was designed to address
by a different prognosis. alterations in autonomic nervous system activity in
We report a case of a 59-year-old man, referring to our Unit normotensive subjects with a family history of hypertension.
in 2011. He complained of orthostatic syncope and We compared the autonomic nervous system activity in 11
intolerance since 2004, when he was diagnosed with normotensives with a family history of hypertension (age
coronary artery disease, treated with PTCA. In 2008 pauses 23.30.4) and 14 normotensives with no family history of
>3 were found during Holter ECG. After implantation of hypertension (age 22.90.3).
biventricular PM, symptoms were only reduced. He Methods: In all of the participants their cardiovascular
underwent ABPM (113/68 mmHg day-time, 129/78 mmHg parameters, including impedance cardiography, were
night-time; high BP variability; orthostatic symptoms measured at rest. In addition, the Valsalva manoeuvre was
related to hypotension), tilt test (stopped after 15 because performed and Valsalva Index was obtained. On the basis of
of fainting and hypotension), and echocardiography (left the arterial blood pressure change, provoked by the Valsalva
ventricular hypertrophy and a preserved systolic function). manoeuvre, the latency of baroreflex response was
During our evaluation, he also reported anhydrosis and determined.
erectile dysfunction; we found orthostatic hypotension Results: Normotensive subjects with a family history of
(clinostatism: 117/75 mmHg; orthostatism: 90/60 mmHg hypertension, compared to the control group, showed
after 1; 79/57 mmHg after 3). significantly higher heart rate (75.03.4 vs. 62.41.8 beats/
Ewings battery of autonomic tests could not be performed min), cardiac output (7.60.4 vs. 6.70.3L/min), left
because of PM; hand grip test was pathological; basal ventricular weight index (4.60.3 vs. 3.90.1) and shorter
plasma adrenaline (30 pg/ml) and noradrenaline (20 pg/ml) ejection time (298.94.9ms vs. 316.84.5ms) (t-test,
were low and not responsive to orthostatism; cardiac SPECT p<0.05). In addition, the normotensives with a family
scan with 123I-MIBG showed a cardiac sympathetic history of hypertension have decreased latency of the
denervation. Neurological evaluation was normal. baroreflex response (7.00.5s) compared to the control
Secondary forms of autonomic neuropathy were excluded. group (10.50.9s) (p<0.001).
Diagnosis of probable pure autonomic failure was made and Conclusions: Our results indicate that even normotensives
therapy with fludrocortisone and dihydroergotamine was with a family history of hypertension exhibit changes of
started; orthostatic symptoms greatly improved. At 12 some cardiovascular parameters at early age. The changes
months of follow-up, the patient remains asymptomatic. in Valsalva manoeuvre response also show alteration of the
Diagnosis and management of syncope may be complicated autonomic nervous system reactivity.
by concomitant clinical conditions leading to syncope. In
our case, a potentially harmful arrhythmic cause was treated
and subsequent follow-up allowed the identification of an
autonomic-related non-cardiogenic cause of syncope.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 135

P1114 P1115
Juvenile migraine and the role of the A case of severe orthostatic hypotension
autonomic system: a preliminary clinical and sensory ataxia as presenting
study symptoms of Sjgrens syndrome
G. Giordano1, C. Spitaleri2, F. Brighina3, I. Camarda2, M. Kirbi, D. Georgiev, R. Berlot, B. Megli,
F. Consolo2, M. DAmelio3, L. Paziente2, V. Raieli2, N. Zupani Krinar
G. Santangelo2, E. Vanadia2, F. Vanadia2 Department of Neurology, University Medical Centre
1U.O. Di NPI Ospedale di Cristina, ARNAS Civico, 2Child Ljubljana, Slovenia
Neuropsychiatry Unit - Di Gristina Hospital - ARNAS Introduction: We present a case of a 61-year-old patient
CIVICO, 3Neurological Department, University of Palermo, with subacute autonomic and sensory polyneuropathy
Italy associated with Sjgrens syndrome.
Background and aims: Cranial Autonomic Symptoms Methods: The patient presented with transitory itching in
(CAS) are frequently reported in adult migraineurs, but the the chest and progressive symptoms of genitourinary and
prevalence of CAS in children affected by primary later cardiovascular autonomic neuropathy, followed by
headaches is unknown. In addition, recent studies suggest a predominantly large fibre sensory neuropathy for all
role of the autonomic nervous system in paediatric migraine modalities. Four months after the first symptoms, he was
by the involvement of trigemino autonomic reflex. bedridden due to severe ataxia and orthostatic hypotension.
Therefore, the aim of this study was to evaluate the He also developed severe urinary retention. Repeated
prevalence of CAS during cephalalgic attacks in a juvenile autonomic cardiovascular function tests confirmed
population with primary headaches and to study the parasympathetic and sympathetic failure; nerve conduction
correlation between CAS and the main symptoms of studies revealed severe, predominantly sensory axonal
migraine. polyneuropathy. Rheumatologic tests were negative. CSF
Methods: A total of 193 children suffering from headache protein levels were mildly elevated, with normal cell counts;
(M 86 F107, aged 4-17 years) were consecutively enrolled tests for paraproteinemia, amyloidosis, infectious and
in an 18-months period. A short questionnaire investigating paraneoplastic causes were negative. Imaging studies of
the presence of CAS was administered to all children. The spine and head were unremarkable. Empirical treatment
following CAS were included in our study: conjunctival with intravenous immunoglobulins (IVIG) showed poor and
injection, tearing, palpebral oedema, nasal congestion, short-lasting effects.
rhinorrhoea, red ear, facial flushing, miosis, ptosis, forehead Six months after presentation, he reported dry eyes and
or facial sweating. mouth. Schirmers test was positive. Rheumatologic
Results: 168 children (86%) were affected by migraine, the screening now showed positive anti-SSB(Ro)/SSB(La)
remaining 25 (14%) by other primary headaches. CAS were antibodies. Sedimentation rate and plasma cryoglobulins
present in 99 with migraine (59%) and only in 6 with others were elevated. Salivary gland biopsy was consistent with
type of headache (24%). Two or more CAS were found in Sjgrens syndrome. Infusions of corticosteroids led to a
(75%) children suffering from migraine during their attacks. remarkable improvement: autonomic and sensory symptoms
The most common signs were flushing, red ear and reduced, ataxia and orthostatic hypotension almost
conjunctival injection. CAS were more significantly disappeared. Corticosteroids were successfully tapered
frequent in migraineurs (p<0.5) . down to a low maintenance dose without symptom
Conclusion: These preliminary findings indicate that CAS deterioration.
are rather common in the course of paediatric migraine Conclusion: Severe autonomic dysfunction as presented
attacks. Besides, these results support the role of the here is rare in Sjgrens syndrome. Disabling orthostatic
trigemino-autonomic reflex in the pathophysiology of hypotension and ataxia were the first symptoms in the
migraine. presented case, followed only later by sicca symptoms and
serologic abnormalities. All symptoms were successfully
treated with corticosteroids with remarkable improvement.
Treatment with IVIG was not successful.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


136 Posters, Sunday 9 September

P1116 P1117
Autonomic disorders in patients with Chronic intestinal pseudo-obstruction
ischemic stroke with anti-Hu antibody without neoplasia
I. Darii1, I. Moldovanu1, M. Sangheli2, S. Plesca1 I. Moreira1, R. Pimentel2, E. Santos1, M. Salgado2
1Institute of Neurology and Neurosurgery, Institute of
2 1Neurology Department, 2Gastroenterology Department,
Neurology and Neurosurgery, State Medical and Hospital Santo Antnio, Centro Hospitalar do Porto,
Pharmaceutical University, Chisinau, Moldova Portugal
Background: Stroke is the most common life-threatening Introduction: Chronic intestinal pseudo-obstruction
neurologic disease and the leading cause of serious long- (CIPO) is characterized by mechanical obstruction of the
term disability. Autonomic disorders are common in the small/large bowel, without an anatomical lesion. Anti-Hu
acute post-stroke phase, but little is known about the long syndrome is one classic, but rare, cause of CIPO, occurring
-term effects in the recovery period. in approximately 6.5% of the cases. These antibodies are
Objective: To determine the type of autonomic function usually associated with neoplasms, particularly small cell
impairment during stroke recovery and their influence on lung cancer.
stroke rehabilitation. Case report: A 40-year-old woman, with history of one
Methods: The study involved 32 patients (mean age 643.2 spontaneous abortion and Raynaud phenomenon, 29 yo and
years) with ischemic stroke and 32 healthy controls; no other diseases/complaints, developed marked
prospective evaluation of 12 cases in 1 month and 20 after constipation, with multiple dejections of liquid stools, crises
6 months post hemispheric stroke. The patients underwent of abdominal distension and vomiting. Extensive gastro-
neurological examinations, and answered an originally intestinal work-up was compatible with CIPO. When 36 yo
designed questionnaire assessing the autonomic on set of dysphagia for solids, achalasia was diagnosed, and
interoceptive profile. improved after oesophagic dilatation. When 38 yo worsened
Results: The patients who had an ischemic stroke, besides requiring multiple hospitalizations by intestinal occlusive
main symptoms, presented different autonomic dysfunctions periods, without response to domperidone, erythromycin or
at 1 month versus 6 months. The most common clinical laxatives. Had sharp weight loss needing to escalate to
problems included abnormalities in heart rate and blood cisapride, artificial enteral nutrition and then total parenteral
pressure regulation (58.1% vs. 66.8%), reflecting nutrition. Gastrointestinal manometry was compatible with
cardiovascular autonomic dysfunction and asymmetric intestinal neuropathy. Neurological examination, nerve
sweating with cold hemiplegic limbs (61.3% vs. 77.6%), conduction and QST were normal. Rectal biopsy was
reflecting changes in the sudomotor and vasomotor negative for amyloid. Anti-Hu antibodies were positive.
regulatory systems. Stroke-induced gastrointestinal, sexual Wide-ranging investigation showed no evidence of
and urinary disorders are not uncommon. Most of the malignancy. Steroids and azathioprine were attempted, but
patients presented vegetative dysfunctions, the most severe not maintained because of vomiting. Now shes under total
ones being observed 6 months after stroke. parenteral nutrition and gastrostomy is planned.
Conclusion: Although autonomic disturbances often are Comments: This is a case of autonomic neuropathy
most pronounced immediately after the stroke, they are not involving myenteric plexus with anti-Hu antibodies with
limited to the acute phase only, but are long-term sequelae, eleven years of follow-up without evidence of malignancy.
being the cause of serious long-term disability. Impaired We hypothesized that it is more likely an auto-immune
autonomic function may increase the risk of all-cause disease than paraneoplastic or an immune tumour regression
mortality and cardiovascular mortality in older stroke mediated by anti-Hu antibodies.
survivors.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 137

P1118 P1119
A preliminary study on blood pressure Investigating the feasibility of using
variability assessed from left and right objective motion data to assist the
arms in the elderly diagnosis and management of
M. Hsu1, J. Lin1, Y. Chang2, Y. Chou3 cardiovascular autonomic dysfunction
1Department of Physical Therapy, Koahsiung Medical
R.M. Kwasnicki, D.A. Low, C. Wong, D. Jarchi, B. Lo,
University, Kaohsiung, 2Department of Physical Therapy, C.J. Mathias, A. Darzi, G.Z. Yang
Chang Gung University, Taoyuan, 3Department and Imperial College London, UK
Graduate Institute of Neurology, Koahsiung Medical
University, Koahsiung City, Taiwan R.O.C. Introduction: Cardiovascular autonomic dysfunction (AD)
manifests itself through various symptoms such as light-
Introduction: Autonomic dysfunction is associated with headedness, palpitations and syncope. Stimuli for such
higher risks of cardiovascular disease. Autonomic function events include physical activity, stress, and posture changes.
can be assessed non-invasively through analyzing blood Although the relationship between physical activity and
pressure variability. There are differences in blood pressure symptoms is important, activity data is currently limited to
between the two arms, which may possibly influence subjective, and potentially inaccurate, patient diaries.
autonomic function assessment. Therefore, the purpose of Our aim was to assess the feasibility of using a lightweight
this study was to compare the autonomic function assessed motion sensor (e-AR lite, ICL) to collect an objective record
between the two arms. of motion for analysis alongside clinical cardiovascular
Methods: 6 healthy old people were recruited from a autonomic function parameters.
community recreation center. The ECG (Biopac MP100 Methods: We recruited a patient with suspected
A/D converter and Biopac ECG module) and blood pressure cardiovascular AD, who wore an e-AR sensor throughout
(CNAP Monitor 500, CNSystems Medizintechnik AG) laboratory cardiovascular autonomic function testing and
were monitored continuously under three conditions: 24-hour ambulatory monitoring. Tri-axial accelerometer
supine, sitting, and a mental test. In each condition, the data from the e-AR sensor was analysed alongside blood
blood pressure for both arms and the ECG were recorded, pressure/heart rate data and the patients activity diary.
each arm for five minutes. The order of arm tested was Results: Body orientation during head-up tilting was visible
randomized. Blood pressure power spectrum was analyzed from the e-AR sensor data. Cervical head movements were
afterwards. Two-way repeated ANOVA was used to analyze also clearly recorded and were related to a fall in blood
between arm differences under three conditions for low pressure and heart rate with associated pre-syncopal
frequency (0.04-0.15, LF), high frequency (0.15-0.40, HF), symptoms. Although no unusual cardiovascular events
and LF/HF ratio. occurred during 24-hour ambulatory monitoring, we were
Results: Significant interactions were found between arm able to accurately quantify both the duration and nature of
and condition for LF and HF, while there was no interactions activities recorded in the patients diary using the e-AR
for the LF/HF ratio. LF and HF from the right arm were sensor, with corresponding physiological changes.
significantly higher than from the left arm in sitting Conclusion: We have demonstrated the feasibility of using
condition (p<0.0005). There are no significant differences a lightweight motion sensor to relate the symptoms and
between arms for the LF/HF ratio under any condition. objective data of cardiovascular AD to an objective account
Conclusions: Differences in blood pressure variability of patient activity and posture. The true value of objective
between arms may exist to some extent. Further studies with motion capture in assisting with diagnosis and management
a larger sample size are needed to provide insights into of AD must now be investigated in small study cohorts.
methodological considerations for autonomic function
assessment.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


138 Posters, Sunday 9 September

P1120 P1121
The effect of levetiracetam therapy on the RR interval variation and the sympathetic
autonomous nervous system in epilepsy skin response in the assessment of
patients autonomic function in diabetic neuropathy
K. Ekmeki, Y. Altun, Y. Tumay, Y. Ozkul H. Ulvi, U. Avar, R. Demir, L. zel, G. zdemir,
Neurology, Harran University, Sanliurfa, Turkey A. Ertekin, R. Aygul
Aim: It was aimed to research the effects of levetiracetam Department of Neurology and Family Medicine, Faculty of
on some autonomic functions by comparing autonomous Medicine, Atatrk University, Erzurum, Turkey
nervous system tests in epilepsy patients using levetiracetam The aim of our study was to evaluate possible autonomic
monotherapy with the tests of healthy volunteers who do not nervous system (ANS) dysfunction in patients with diabetic
use drugs. neuropathy (DN), and its correlation with abnormalities of
Method: 41 patients diagnosed with partial epilepsy using sensorimotor nerve conduction study and clinical autonomic
levetiracetam were included in this study. The control group symptoms. We studied 44 patients with DN; the mean age
was selected from 35 healthy volunteers who do not have was 55.5612.92 years (range 30 yrs to 70 yrs; 24 female
epilepsy. RR interval variation (RRIV), valsalva, and tilt and 20 male) and 35 age-matched healthy subjects (control);
tests were applied to patient and control groups in order to mean age was 34.1912.74 years (range 24 yrs to 48 yrs; 20
assess the autonomous nerve system functions. female and 15 male). Mean RR interval and SSR in patients
Findings: No statistically significant differences were with DN was significantly more abnormal than that of the
found in the results of RRIV, valsalva, and tilt tests in control subjects (p<0.05). No correlation was observed
patients in comparison with the control group (p>0.05). No between patient age, sex, duration of DN history,
statistical significances were observed when the results of abnormalities of sensorimotor nerve conduction and
upright position and the postural blood pressure changes abnormalities in RR interval, SSR. These data indicate that
were compared with the control group (p>0.05). patients with diabetic neuropathy show abnormalities of
Result: Our findings had shown that using levetiracetam ANS function. We conclude that SSR and RR interval
therapy had no effect on the responses of heart rate and variation, both of which can easily be performed in the
blood pressure in epilepsy patients. electromyography laboratory, are helpful in combination in
the assessment of autonomic function in diabetic neuropathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 139

P1122 P1123
Dispersion of ventricular repolarisation as Autonomic dysfunction: causes and
diagnostic tool for the assessment of presentations. A retrospective study in a
autonomic function in diabetic neuropathy tertiary hospital
H. Ulvi, U. Avar, Z. Avar, Z. Cansever, R. Demir, J.J.Y. Ong, K.W.P. Ng, Y. Gao, E.P. Wilder-Smith,
L. zel, R. Aygul Y.C. Chan
Departments of Neurology and Family Medicine and Neurology, National University Health System, Singapore,
Medical Education, Faculty of Medicine, Atatrk University, Singapore
Erzurum, Turkey Introduction: Due to increasing availability, tests for
Diabetes mellitus has been shown to affect almost all autonomic function are increasingly utilized to detect
systems of the human body and abnormalities in functions autonomic dysfunction. We did a retrospective study to
of autonomic nerves innervating various parts have been determine the causes and presentations of patients with
observed in several studies. Dispersion of repolarisation has autonomic dysfunction tested in our tertiary hospital.
been a proposed measure of repolarisation heterogeneity, Methods: This was a retrospective descriptive study of
and increased dispersion has been found to be associated patients with autonomic dysfunction tested in our
with an increased incidence of malignant ventricular neurodiagnostic laboratory between January 2009 and
arrhythmias. However, in literature, the assessment of the December 2011. Depending on the indication, patients
parameters of ventricular dispersion and their correlation underwent one or more of the following studies: tilt-table
with abnormalities of sensorimotor nerve conduction study test, heart rate variability (HRV) during deep breathing,
in diabetes mellitus patients have not been reported. Valsalva manoeuvre (VM) or sympathetic skin response
We studied 44 patients with diabetic neuropathy (DN) and (SSR) test.
41 age-matched healthy subjects. In all persons 12 leads Results: A total of 219 patients (72 females, 147 males,
ECG were recorded at a speed of 50mm/s and somatic nerve mean age 58.217.9) were tested. 40.2% (n=88) of patients
conduction studies were carried out. Ventricular had abnormal findings in one or more test categories. The
repolarisation parameters (QT, QTc, JT, and JTc) and most common presentations were: orthostatic hypotension
ventricular repolarisation dispersion (d) parameters (QT-d, (n=21), syncope (n=19), giddiness (n=26), pre-syncope
QTc-d, JT-d, JTc-d) were detected. QT and JT intervals were (n=2), parkinsonism (n=11) and others (n=6). Of those who
measured from the beginning and from the end of the QRS had abnormal findings, 36.4% (n=32) had abnormal tilt-
complex, respectively, to the end of T-wave to baseline while table results. Despite normal tilt-table findings, 56 patients
excluding the U-wave. Using Bazett formula, these intervals still had evidence of autonomic dysfunction based on our
were corrected for heart rate. Repolarisation dispersion remaining testing battery (31 abnormal SSR, 26 abnormal
parameters were calculated the difference between minimal HRV, 6 abnormal VM). The most common cause of
and maximal values of QT, JT, QTc, and JTc among 12 autonomic dysfunction was diabetes mellitus. 6.8% (n=6)
leads. Mean QT-d, QTc-d, JTd, JTc-d in diabetes mellitus had deceased.
patients was significantly longer than that of the normal Conclusion: Diabetes mellitus was the most common cause
subjects (p<0.05). No correlation was observed between of autonomic failure presenting at our neurodiagnostic
abnormalities of sensorimotor nerve conduction and laboratory. Orthostatic hypotension by lying and standing
ventricular repolarisation dispersion parameters. We blood pressure measurement was present in only 9.6%
conclude that ventricular repolarisation dispersion (n=21) and cannot be relied on alone to detect autonomic
parameters evaluation is a helpful and cheap method in the dysfunction.
assessment of autonomic function in diabetic neuropathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


140 Posters, Sunday 9 September

P1124 P1125
Epidemiologic characteristics of patients Correlations between parameters of
with the first episode of syncope sympathetic skin response tests and
diagnosed by head-up tilt test with parameters from the 24-hour ECG Holter
transcranial Doppler monitoring in patients after troponin-
J.J. Lee1, S.S. Jang2 positive acute coronary syndrome
1Neurology, Daegu Fatima Hospital, Daegu, 2Neurology, M. Fila1, U. Cielik-Guerra2, M. Kamiski3,
Pohang Sunlin Hospital, Pohang, Republic of Korea J. Chapiski3, A. Bogucki4, J. Kasprzak2, M. Kurpesa2
Introduction: Syncope is common in the general population 1Department of Neurology, 3rd City Hospital, 2Cardiology,
and many patients visited the hospital due to a single first Bieganski Hospital, Medical University of Lodz,
3Department of Microelectronics and Computer Science,
episode. Most epidemiological studies have shown the
bimodal distribution of syncope incidence over a broad age Technical University of Lodz, 4Departement of
range. We evaluated epidemiologic aspects of patients with Extrapyramidal Disorders, Medical University of Lodz,
the first episode of syncope from patients visit to hospital Poland
due to syncope with first or multiple episodes. Purpose: To compare the results and determine correlations
Methods: We have performed head-up tilt test with between parameters obtained in a basic neurophysiological
transcranial Doppler in all patients who visited the hospital test of SNS - Sympathetic Skin Response Test (SSRT) and
due to syncope between January 2009 and December 2011. parameters derived from 24-hour Holter ECG analysis.
We evaluated retrospectively epidemiologic aspects of Methods: The study group consisted of 52 patients aged
patients with the first syncope from all patients. 59.569.11 yrs (41 male) after troponin-positive acute
Results: The study population included 270 patients of coronary syndrome (1-6 months before they were included).
whom 105 (38.9%) in the first episode and 165 (61.1%) in In all of the pts direct recording of SSR potentials on both
multiple episodes. 105 patients of the first episode: 47 hands and feet was performed. In standard, latency (L) and
(44.8%) men and 58 (55.2%) women, average age 46.2 amplitude (A) of P0 potential were evaluated. In all pts ECG
years (range 12-88 years), male dominant in the 2nd and 7th Holter monitoring was performed using DMS monitors and
decade. 165 patients of multiple episode, 74 (44.8%) men analysis software CardioScan 12. Heart rate variability
and 91 (55.2%) women, average age 44.2 years (range (HRV) was analyzed with time and frequency domain
11-91years), male dominant in 2nd- 4th and 8th decade. The methods to calculate: the standard deviation of all normal
positive response rate of head-up tilt test are 79 (75.2%) in RR intervals (SDNN), the mean of all the 5-minute standard
the first episode group and 122 (73.9%) in multiple episodes deviations of RR (SDNN index), the standard deviation of
group with no meaningful difference in both groups. The all the 5-minute RR interval means (SADNN index), the
same finding was made in sex ratio, average age and root-mean-square successive difference (rMSSD), the
positive response rate of head-up tilt test. percentage of differences between successive RR intervals
Conclusion: Many patients visited the hospital due to over 24 hours that are greater than 50 ms (pNN50) and total
syncope with a single first episode. The same epidemiologic power spectrum (TPS), very low frequency (VLF), low
finding was made in sex ratio, average age at onset time frequency (LF), high frequency (HF) of spectrum.
with same positive response rate of head-up tilt test in both Premature ventricular contractions were observed and
groups. Therefore we can perform a study for evaluating in analyzed for turbulence onset (To) and slope (Ts). We
syncope even for the single first episode. performed also deceleration capacity (DC) analyses using
non-commercial software.
Results: Correlations have been observed between
potentials latency (L) of SSR on hand and parameters
derived from 24-hour Holter ECG analysis, but there were
not any significant correlations between these Holter
parameters and characteristics of SSR potentials recorded
from lower limbs.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 141

P1126 P1127
Sympathetic over-reactivity as underlying Complex regional pain syndrome type I
cause of recurrent syncope and following cervical medullary lesion: a case
accommodation reflex loss: a report of report
two cases L. Isidoro, F. Matias, C. Macrio
M.A. Sierra-Beltrn1, C.M. Hernndez-Crdenas2, Neurology, Coimbra University Hospital, Coimbra, Portugal
F.A. Villegas Lpez3 Introduction: Complex regional pain syndrome (CRPS)
1CIDyT, Mdica Sur, 2Critical Care Medicine, INCMNSZ, describes a variety of painful conditions following injury
3CGCA, Mdica Sur, Mexico City, Mexico
that appear regionally with a distal predominance of
Introduction: To present two clinical cases of patients abnormal findings, exceeding in magnitude and duration
suffering both of recurrent syndromes, fixed pupils and loss the expected clinical course of the inciting event. Peripheral
of the accommodation reflex. In both cases the underlying but also central mechanisms are involved.
cause was sympathetic over-reactivity. The first case was a Case report: Female, 56-year-old, admitted in the
23-years-old woman who has suffered for the last 13 years emergency department with a 4-months progressive history
from recurrent syncopes (in 4 occasions it evolved into a of right upper extremity (RUE) pain, oedema, impaired
convulsive syncope) evoked after postural changes and movement, and cold skin. She denied recent trauma, surgery
visual acuity defects. The second case was a 36-years-old or infection and reported mild relief with analgesics.
man who has suffered, for the last five years of recurrent Cervical MRI performed at the beginning of the symptoms
syncopes and progressive visual acuity defects. showed C4-C5 and D1 lesions, hyperintense at T2 and
Methods: Routine clinical and specialized rheumatological contrast enhancement. Brain image showed unspecific
imaging and neurophysiological tests were requested. white matter lesions.
Results: No alterations were found on the Complete Blood Examination: RUE hyperalgesia, which was swollen,
tests. Rheumatological test proved that there were not glossy and cold. Abnormal hair and nail growth. On EMG
antibodies of this sort. Images Scans discarded structural there was no sign of peripheral nerve lesion. Bone
central nervous system abnormalities. The Autonomic scintigraphy: unilateral hyperperfusion on right joints. Plain
Function test showed, in both cases, sympathetic over- radiographs: severe osteoporosis of the right hand. RUE
reactivity. echography revealed only mild degenerative abnormalities.
Conclusions: Sympathetic overreactivity has been observed Investigation of the cervical lesion (including lumbar
in upper spinal cord injuries, subarachnoid haemorrhage, puncture) was negative for an infectious or autoimmune
neuroleptic malignant syndrome, Guillain Barr Syndrome, etiology and on the control MRI there was minor extension
poliomyelitis and as toxin mediated diseases (botulism and of the cervical lesion and with no longer contrast
diphtheria). It is a rare circumstance that it happened in a enhancement.
clinical set of no other proven etiology. The analgesic treatment was optimized with analgesics,
pregabalin and antidepressives. A 3-day corticotherapy
pulse was also done and the patient started with calcitonin,
calcium and vitamin D. After pain relief, physical therapy
was started with good response.
Conclusion: We report a case of a patient who presented
with a CRPS I following a cervical inflammatory lesion.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


142 Posters, Sunday 9 September

P1128 P1129
Arnold-Chiari malformation presenting Changes in cardiovascular autonomic
with recurrent syncope attacks function and peripheral nervous system in
B. Gke, A.K. Erdemolu type 2 diabetes mellitus
Neurology, Kirikkale University School of Medicine, R.K. Goit1, R. Khadka2, S.K. Sharma2, N. Limbu2,
Kirikkale, Turkey B.H. Paudel2
The Chiari type I malformation (CM1) is characterized by Physiology, Nepalgunj Medical College, Banke, 2B P
1

herniation of cerebellar tonsils to at least 3-5 mm below the Koirala Institute of Health Sciences, Dharan, Nepal
plane of foramen magnum and can present with a wide
variety of symptoms like occipital headache, vertigo
secondary to bulbar or medullary distress. Rarely syncopal P1130
episodes have also been described. Sudomotor failure with
We report a 39-year-old male patient suffering from hypogonadotrophic hypogonadism
occipital headaches and recurrent syncopes for two years.
presenting as heat stroke
The patient had a normal neurological examination;
electroencephalogram (EEG), routine blood tests, thyroid H.J. Im1, J. Oh2, D.W. Kim2
function tests and vitamin B12 levels were normal. His Neurology, 2Konkuk University Hospital, Seoul, Republic of
1

cardiological examination also showed no abnormalities. Korea


His brain magnetic resonance imaging (MRI) showed a
Chiari type I malformation with tonsillar herniation of 9mm
below the plane of foramen magnum.
P1131
Rarely, syncopal episodes have also been described and Abstract cancelled
attributed to either compression of the midbrain ascending
reticular system or vascular compromise (vertebrobasilar
artery compression, hypotension). Chiari malformation
must be thought of in the differential diagnosis in patients
suffering from recurrent syncope attacks.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 143

Cerebrovascular diseases 1 P1133


High blood pressure, physical and
P1132 cognitive disability and risk of stroke in
Point-of-care laboratory halves door-to- the oldest old: the Leiden 85-plus study
therapy-decision time in acute stroke B. Sabayan1, P. van Vliet2, J. Gussekloo3,
S. Walter1, P. Kostopoulos1, A. Haass1, Y. Liu1, A.J.M. de Craen4, R.G.J. Westendorp4
P. Papanagiotou2, C. Roth2, I. Grunwald3, K. Fassbender1 1Gerontology and Geriatrics, Radiology, 2Neurology, 3Public
1Neurology, Diagnostic and Interventional Neuroradiology,
2 Health and Primary Care, 4Gerontology and Geriatrics,
University Hospital of the Saarland, Homburg, Germany, Leiden University Medical Center, Leiden, The Netherlands
3Acute Vascular Imaging Centre, John Radcliffe Hospital,
Introduction: Outcomes of studies on the association
Oxford, UK between high blood pressure and risk of stroke in very old
Time until beginning of a causal therapy is critical for age have shown conflicting results. In this study we
favourable outcome of acute stroke. In ischemic stroke examined whether levels of physical and cognitive disability
treatment, application of rt-PA thrombolysis requires moderate the association between high blood pressure and
information about coagulation, blood count and liver risk of stroke in the oldest old.
laboratory parameters of the patient in order to reduce Methods: Study participants included 571 subjects aged 85
bleeding complications. Currently, stroke laboratory years from the Leiden 85-plus Study. Disability in activities
examinations are usually performed in the centralized of daily living (ADL) and Mini Mental State Examination
hospital laboratory, which can be time consuming. (MMSE) were assessed at age 85 years and participants
Therefore, planned thrombolysis is often given before all were followed for stroke events over a period of five years.
results are available as off-label use. In this study, we Associations between various measures of blood pressure
examined the feasibility of gaining valuable time by and risk of stroke were estimated by Cox regression
transferring the complete stroke laboratory workup required analysis.
by stroke guidelines to a point-of-care laboratory system, Results: Higher pulse pressure at age 85 years was
that is, placed at a stroke treatment room contiguous to the associated with lower risk of stroke (HR: 0.81 95% CI,
computed tomography, where the patients are admitted and 0.68-0.96). In the stratified analysis, subjects with low
where they obtain neurological, laboratory, and imaging disability showed an increased risk of stroke for higher
examinations and treatment by the same dedicated team. blood pressure albeit not statistically significant. Conversely,
Our results showed that reconfiguration of the entire stroke higher blood pressure measures were associated with lower
laboratory analysis to a point-of-care system were feasible risk of stroke in subjects with high physical and cognitive
for 200 consecutively admitted patients. This strategy disability (all P<0.05). The associations between various
reduced the door-to therapy-decision times from 8426 to measures of blood pressure and risk of stroke were
4024 min (p<0.001). Results of most laboratory tests significantly different in subjects with low and high physical
(except activated partial thromboplastin time and and cognitive disability (all P for interaction <0.05)
international normalized ratio) revealed close agreement Conclusions: In very old subjects with high physical and
with results from a standard centralized hospital laboratory. cognitive disability, high blood pressure is associated with
These findings may offer a new solution for the integration lower risk of stroke. High blood pressure, in the presence of
of laboratory workup into routine hyperacute stroke advanced vascular damage, may preserve cerebral perfusion
management. and lower risk of low-flow infarcts.

P1134
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


144 Posters, Sunday 9 September

P1135 P1136
Asymptomatic Moya-moya Registry Early clinical implications of
(AMORE) study 2012-2019 - a nation-wide, microalbuminuria in patients with acute
multicenter prospective survey on ischemic stroke
prognosis of asymptomatic Moya-moya B.-H. Cho, J.-T. Kim, H.-J. Jung, K.-H. Choi, T.-S. Nam,
disease in Japan S.-M. Choi, S.-H. Lee, M.-S. Park, B.-C. Kim,
S. Kuroda, AMORE Study Group M.-K. Kim
Neurosurgery, Hokkaido University Hospital, Sapporo, Department of Neurology, Chonnam National University
Japan Hospital, Gwangju, Republic of Korea
Objective: The clinical features, prognosis, and treatment Background: Microalbuminuria is considered to be
strategy of asymptomatic Moya-moya disease are still independently associated with an increased risk and
unclear. Therefore, we have designed the Asymptomatic mortality of stroke. However, it has not been studied
Moya-moya Registry (AMORE) Study in Japan. The whether microalbuminuria could be associated with the
objectives of this nation-wide, multi-center prospective early clinical outcomes of acute ischemic stroke. Therefore,
study are to clarify long-term prognosis of asymptomatic we sought to investigate the association between
patients with Moya-moya disease and to determine the risk microalbuminuria and early clinical characteristics
factors to cause ischemic and hemorrhagic stroke in them. including outcomes at discharge in acute ischemic stroke.
Study design: AMORE Study is conducted in Japan, using Methods: We studied 361 patients with acute ischemic
a multicenter prospective observational design. The stroke within 72 hours of symptom onset. Early clinical
AMORE Study Group is composed of 16 Japanese outcomes were assessed by early neurological deterioration
neurosurgery/neurology centres. In this study, all (END) and modified Rankin Scale (mRS) at discharge. In
consecutive patients diagnosed as asymptomatic Moya- addition, early radiological outcomes were assessed by
moya disease between January 2012 and December 2014 hemorrhagic transformation (HT) and lesion changes on
are registered. Inclusion criteria is as follows: Thus, all follow-up diffusion-weighted imaging (FU DWI). We
patients must categorized UACR as 30 mg albumin/g creatinine (normal)
(a) be between 20 and 70 years at initial diagnosis; and 30-300 (microalbuminuria).
(b) fulfil the guidelines for the diagnosis criteria Moya- Results: 139 of 361 patients had microalbuminuria. In 41
moya disease; of 361 patients occurred END. In multivariate analysis,
(c) experience no ischemic or hemorrhagic cerebrovascular urine albumin-to-creatinine ratio (UACR) was independently
events since they were born; and associated with END (OR, 1.008; 95% CI, 1.004-1.013;
(d) be independent in daily life. p<0.001). In addition, microalbuminuria was independently
Their clinical and radiological data are registered. associated with HT and lesion changes on FU DWI by
Subsequently, they are followed up at outpatient clinic for multivariate analysis.
five years after enrolment. MR imaging and angiography Conclusion: In the early phase of ischemic stroke, patients
are repeated every year during follow-up period. The with microalbuminuria were related with worse clinical
following items constitute the primary endpoint: TIA with characteristics (including END, mRS>2 at discharge, HT,
cerebral infarct, ischemic stroke or hemorrhagic stroke. The and lesions changes on FU DWI) than those without.
following items constitute the secondary endpoint: TIA,
radiological occurrence of silent cerebral infarction, silent
intracranial bleeding, or silent disease progression or death.
Conclusion: We emphasize the importance to determine the
prognosis of asymptomatic Moya-moya disease, and also
present the precise protocol of AMORE Study.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 145

P1137 P1138
Knowledge of risk factors and stroke Ischemic stroke and asymptomatic
symptoms among non-stroke patients carotid stenosis are strongly associated
M. Wiszniewska1, M. Guszkiewicz2, A. Kobayashi2, with metabolic syndrome
A. Czonkowska2,3 M. Mijajlovic1, A. Jotic2, N. Lalic2,
1Neurological Department, Specialist Hospital, Pila, N. Covickovic-Sternic3
22nd Department of Neurology, Institute of Psychiatry and 1Department for Cerebrovascular Disorders, Neurology
Neurology, 3Department of Experimental and Clinical Clinic, 2Institute of Endocrinology, 3Neurology Clinic,
Pharmacology, Medical University, Warszawa, Poland Clinical Center of Serbia, Belgrade, Serbia
Background and purpose: This study aimed to evaluate Background and aims: Insulin resistance (IR) plays a
knowledge of stroke warning signs, risk factors, treatment crucial role in the pathogenesis of atherosclerosis, but their
and prevention among patients not suffering from stroke. role in ischemic stroke (IST) has not been elucidated. The
Methods: Patients admitted to one of five neurology study was aimed to analyze IR and plasma insulin (PI)
departments in Poland for diseases other than stroke were levels, dyslipidemia pattern, obesity and plasminogen
asked to answer a questionnaire regarding awareness of activator inhibitor-1 in 100 patients with atherothrombotic
cerebrovascular risk factors (CVRF) and stroke. The study IST (group A), 100 patients with asymptomatic carotid
was performed between November 1 and December 31 stenosis (ACAS) 50% (group B), 100 patients with lacunar
2008. stroke (Group C) and 65 healthy controls (group D). Patients
Results: 481 patients were included in the study (59.7% with diabetes mellitus and ischemic heart disease were
women). Proper definition of stroke and TIA were given by excluded.
90.3% and 60.5% of respondents respectively. Hypertension Methods: IR was determined by Homeostasis Assessment
was reported as a risk factor by 91.1% of participants. Model, PI levels by Radioimmunoassay. Total-, LDL- and
Approximately 70% knew that hypercholesterolemia and HDL-cholesterol and triglyceride levels were measured in
smoking are risk factors of stroke, but only one-third all groups. Obesity was determined by waist circumference
identified diabetes mellitus as a risk factor. Cardiac and hypercoagulable state by plasminogen activator
arrhythmia was recognized as a risk factor by 8.4% . 25% inhibitor (PAI-1) levels.
of the participants did not know any symptom of stroke. Results: IR was significantly higher in group A compared
Identification of stroke signs was worse in participants from to group B, C and D (4.820.27 vs. 3.690.22, p<0.05;
rural areas. 10% identified disturbances of consciousness, 4.820.27 vs. 2.710.21, p<0.01, 4.820.27 vs. 1.500.19,
numbness, and dizziness as stroke symptoms. p<0.01). PI levels were significantly higher in group A in
Conclusions: The knowledge of fundamental risk factors comparison to group B, C and D (19.001.2 vs. 15.950.88,
was sufficiently good, but recognition of cardiac arrhythmia p<0.05; 19.001.2 vs. 11.120.19, p<0.01, 19.001.2 vs.
and diabetes mellitus was unsatisfactory. The knowledge of 7.121.08). Different patterns of dyslipidemia were
stroke symptoms was unsatisfactory particularly in rural observed in ACAS and IST. PAI-1 levels, and waist
areas. Additional education programs are still necessary. circumference were significantly higher in group A, B, and
C in comparison to controls (p<0.01, respectively).
Conclusion: Our results indicate that all subtypes of
ischemic stroke as well as ACAS are strongly associated
with IR and increased PI and PAI-1 levels. Specific patterns
of dyslipidemia between ACAS and IST were observed.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


146 Posters, Sunday 9 September

P1139 P1140
Therapeutic drug monitoring of Less complications in shunt surgery:
clopidogrel in neurointervention: a single gravitational valves are proven to be
centre experience effective in the therapy of the idiophatic
J.J. McCabe, E. Kim, H. Villegas, W. McAuliffe normal pressure hydrocephalus
Neurological Intervention & Imaging Service of Western (SVASONA)
Australia (NIISWA), Sir Charles Gairdener Hospital, Perth,
J. Lemcke1, U. Meier1, C. Mller2, M. Fritsch2,
WA, Australia
U. Kehler3, N. Langer3, M. Kiefer4, R. Eymann4,
Introduction: Clopidogrel resistance is an increasingly M.U. Schuhmann5, A. Speil5, F. Weber6, V. Remenez6,
concerning phenomenon in the neurointervention setting, V. Rohde7, H.-C. Ludwig7, D. Stengel2,8
with an estimated incidence of 4-44%. This high incidence 1Neurosurgery, Unfallkrankenhaus Berlin, 2Ernst-Moritz-
is of clinical relevance as it is associated with increased Arndt-University, Greifswald, 3Asklepios Hospital Altona,
rates of thromboembolic events following carotid artery or Hamburg, 4Saarland Medical University, Homburg / Saar,
cerebral aneurysm stenting. Routine therapeutic drug 5Eberhard-Karls-University, Tbingen, 6Hospital Cologne-

monitoring to identify clopidogrel resistance has been Merheim, Cologne, 7Georg-August-University of Gttingen,
suggested. However there is a dearth of evidence as to its 8Center for Clinical Research, Unfallkrankenhaus Berlin,

usefulness in guiding therapy in practice. Germany


Aims: To evaluate the impact of therapeutic drug monitoring Background: Among dementia diseases idiopathic normal
of clopidogrel on patient management. pressure hydrocephalus is the only entity susceptible to a
Methods: Point-of-care testing to identify clopidogrel neurosurgical therapy. The risk of overdrainage
resistance using the VerifyNow assay was carried out on all complications had a major influence on the indication for
treated patients undergoing neurointerventional procedures surgery in the past. The aim of the SVASONA study is to
in a tertiary referral centre during the twelve month period analyse in a randomised multi-center study whether the risk
from 01/12/2010 to 01/12/2011. Clopidogrel resistance was of overdrainage complications can be eliminated by the use
defined as platelet inhibition <20% with a Platelet of gravitational valves.
Reactivity Unit of >240. A retrospective review of the case Methods: Patients diagnosed for iNPH by clinical
notes of all patients was carried out. Patient demographics, examination, radiological assessment and cerebrospinal
co-morbidities, medications and any alteration to treatment fluid (CSF) infusion test, CSF tap test and or continuous
plan following the point-of-care test were recorded. ICP measurement were recruited in seven centres and
Results: A total of 65 patients were included in the study: randomly assigned to either receive a ventriculoperitoneal
31 male and 34 female with a mean age of 57. The incidence (vp) shunt with a programmable valve without a gravitational
of Clopidogrel resistance was 20% (n=14). unit or a programmable valve with a gravitational unit. The
Long-term treatment was altered in 89% of these cases in patients were followed-up 3, 6 and 12 months after surgery.
light of platelet inhibition testing. Concomitant treatment The primary endpoint was the incidence of overdrainage
with Atorvastatin (Risk ratio 5.3, p=0.01) and age >65 (Risk complications after 6 months.
Ratio 2.8, p=0.05) was associated with resistance. Results: 150 patients were included. The study was stopped
Conclusion: A high incidence of Clopidogrel resistance due to a massive advantage for the patient group with
was observed in our study. Routine testing is recommended gravitational units.
to identify non-responders and alter management Six months after surgery in the intention-to-treat population
accordingly. the patients group treated with programmable valves
without a gravitational unit showed overdrainage
complications in 32.9%, and the patient group treated with
programmable valves with gravitational units showed
overdrainage complications in 2.7% (p<0.001).
Discussion: Significantly more patients had overdrainage
complications in the treatment group without gravitational
units whilst the outcome was equal in both groups. Thus, the
use of gravitational units should evolve as a new standard of
care for iNPH.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 147

P1141 P1142
Inhibiting TLR4 signal protects against The VISTA collaboration: exploratory
neuronal death in transient focal ischemia analysis of trial data to inform RCT design
Y. Suzuki1, T. Murase2, J. Hamanaka1, K. Hattori2, M. Ali1,2, M. Brady2, H.-C. Diener3, D. Hanley4,
K. Tsuruma1, M. Shimazawa1, H. Nagasawa2, H. Hara1 N. Wahlgren5, S. Warach6, K.R. Lees1, On behalf of the
1Molecular Pharmacology, Department of Biofunctional
VISTA Steering Committees
Evaluation, 2Medicinal and Pharmaceutical Chemistry, 1University of Glasgow, 2Glasgow Caledonian University,
Department of Organic Chemistry, Gifu Pharmaceutical Glasgow, UK, 3University Duisburg-Essen, Essen, Germany,
University, Gifu, Japan 4The John Hopkins Medical Institutions, Baltimore, MD,

Introduction: Toll-like receptors (TLRs) are signalling USA, 5Karolinska Institute, Stockholm, Sweden, 6UT
receptors in the innate immune system. Recently, we have Southwestern Medical Center, Austin, TX, USA
reported that TLR4, but not TLR3 or TLR9, contributes to Introduction: Practical and financial barriers hinder the
ischemic brain injury induced by middle cerebral artery pace of stroke research. Data from completed RCTs often
occlusion (MCAO) in mice (Hamanaka et al., Neuroscience, reside in industry or academic archives, however exploratory
171, 258-67). In the present study, we investigated whether analyses of these valuable data can generate hypotheses,
treatment with resatorvid, Ethyl (6R)-6-[N-(2-chloro-4- describe natural history patterns and pilot trial design
fluorophenyl)sulfamoyl]cyclohex-1-ene-1-carboxylate, a elements to inform future RCTs. We established the Virtual
TLR4 signal inhibitor, exerts neuroprotective effects against International Stroke Trials Archive (VISTA), a collaborative
the ischemic injury. stroke trials resource.
Methods: Mice were subjected 2h MCAO followed by 22h Methods: VISTA collates data in 6 specialist archives
reperfusion. Resatorvid was intracerebroventriculary including acute stroke, rehabilitation, secondary prevention,
injected immediately after reperfusion. Infarct volume and intracerebral haemorrhage, imaging, and observational
neurological score were evaluated at 24h after MCAO. To studies. Each section is governed by a Steering Committee
estimate the mechanism of neuroprotection by resatorvid, comprising contributing trialists. Access to VISTA has been
Western blotting and immunostaining were performed. granted to external investigators for novel analyses.
Results: Resatorvid at 0.01g significantly reduced infarct Results: To date VISTA contains data on 49,134 patients
volume and improved neurological score compared with across 6 archives and has facilitated 40 peer-reviewed
vehicle treatment. The levels of p38 phosphorylation, research publications and 42 conference presentations. Data
nuclear factor-kappa B (NF-B) activation, matrix from VISTA have been used to inform the design of the
metalloproteinase 9 (MMP9) and NADPH oxidase 4 SAINT acute stroke trials, investigate thrombolysis in the
(NOX4) expressions were significantly suppressed in elderly, post-stroke atrial fibrillation monitoring, prediction
the resatorvid-treated group. Furthermore, resatorvid of outcomes after ischaemic stroke, inclusion and attrition
significantly reduced DNA oxidation, tyrosine nitration, of patients in RCTs, and validation of stroke outcome
and neuronal apoptosis. measures.
Conclusion: These data suggest that inhibiting the TLR4 Conclusion: VISTA extends the value of clinical research
signalling after ischemia shows the neuroprotective effects at low cost, without threatening commercial or intellectual
against cerebral ischemia and reperfusion injury in mice. property interests and independently delivers valuable
Furthermore, resatorvid may be useful for a therapeutic research output on a wide range of stroke topics. VISTA is
agent for stroke. not a meta-analysis dataset; rather it is used to investigate
epidemiology, stroke outcomes and pilot trial design
elements. VISTA provides young researchers with access to
specialised committees for collaboration. We encourage the
contribution of further trials to the resource and collaboration
to develop new projects.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


148 Posters, Sunday 9 September

P1143 P1144
A meta-analysis on the efficacy and Is CHA2DS2-VASc the best score in
safety of cilostazol against aspirin predicting thrombo-embolic risk in atrial
P.A.D. Canto, M.C. San Jose fibrillation?
Philippine General Hospital, Metro Manila, Philippines N. Diaconu1, A. Grosu1, C. Gratii1, G. Pavlic2, V. Racila1
1Institute of Cardiology, 2Institute of Neurology and
Introduction: Stroke is a leading cause of adult disability
and death worldwide. A key aim to management of patients Neurosurgery, Chisinau, Moldova
with ischemic stroke is to reduce the risk of recurrent stroke Introduction: Atrial fibrillation (AF) is a risk factor for
and serious fatal and non-fatal vascular events. Aspirin ischemic stroke (IS) and thrombo-embolism. The stroke in
remains the cornerstone antithrombotic therapy, and has AF is associated with severe disability and high mortality,
been demonstrated to have a proportional reduction in its prevention being an important factor in management of
vascular events: 19% with 500-1500mg daily, 26% with AF patients.
160-325mg daily, and 32% with 75-150mg daily. Aspirin Objective: To determine the influence of atrial fibrillation
was associated with a 22% increase in odds of symptomatic on stroke development and evolution and to compare the
intracranial haemorrhage and 69% increase in major stroke predictive value of several schemas published in the
extracranial haemorrhage. In a study of 1095 patients with last years.
recent ischemic stroke on Cilostazol, the annual relative risk Methods: Retrospective study of all ischemic stroke
of serious vascular events was reduced by 41.7%. patients admitted during one year in a municipal hospital.
Objectives: Primary objective was to determine the efficacy Results: The study included 735 patients of whom 519 had
and safety of Cilostazol versus Aspirin in the secondary primary stroke (70.6%). AF was determined in 206 cases
prevention of stroke through meta-analysis of randomized, (28.4%), these patients being older (70.10.65 vs. 64.30.46
controlled studies. A secondary objective was to determine years; p<0.001), majority females: 57.8% (119/206) with
if Cilostazol, compared with Aspirin, decreased the more vascular risk factors. AF patients have had more
occurrence of ischemic stroke, hemorrhagic stroke and rate severe signs of cerebral lesion on admission (79% vs. 37%,
of intracranial haemorrhage. p<0.01), also having a higher hospital mortality rate (30.6%
Methodology: CASISP and CSPS2 were included in the vs. 13.2, p<0.001). The scores of thromboembolic risk
study. Meta-analysis was done using RevMan. estimation are presented in the table below.
Results and analysis: Rates of overall stroke occurrence,
hemorrhagic stroke occurrence, overall hemorrhagic events Scores of thromoembolic risk estimation
and intracranial hemorrhage were lower among those who Low Low risk Medium Medium High risk
took Cilostazol. Although ischemic stroke occurrence was risk with without risk with risk without High risk without

not statistically significant, there were less ischemic stroke previous previous previous previous with previous previous

events among patients on Cilostazol compared to Aspirin. Scores stroke stroke stroke stroke stroke stroke p
Framingham 15/8.6 % 10.9% 54/30.9% 33.6% 106/60.6% 55.6% <0.01
Conclusion: There is an advantage in using Cilostazol for
CHADS2 10/4.85 % 6.8% 45/21.8% 30.6% 151/73.3% 62.6% <0.01
secondary stroke prevention due to a better risk reduction in CHA2DS2-
stroke occurrence and safety compared to Aspirin. VASc 0 0 6/2.9% 4.1% 200/97.1% 95.9% <0.01

Conclusions: Patients with AF without previous thrombo-


embolic events have a high risk of stroke with more severe
evolution and consequences. CHA2DS2-VASc is the most
sensible score in determining thrombo-embolic risk in
patients with non-valvular AF.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 149

P1145 P1146
Safety and efficacy of sonothrombolysis Are we getting better in prescribing
using bilateral TCD monitoring by vitamin K antagonists for atrial fibrillation
diagnostic 2 MHz probes: a pilot study before and after stroke?
R. Herzig1, P. Bardo1,2, M. Kuliha1,3, P. Kaovsk1, M. Karlinski1, J.P. Bembenek1, A. Kobayashi1,2,
D. koloudk1,3 A. Baranowska1, A. Czlonkowska1,3
1Comprehensive Stroke Center, Department of Neurology, 12ndDepartment of Neurology, 2Interventional Stroke
Faculty of Medicine and Dentistry, Palack University and Treatment Centre, Institute of Psychiatry and Neurology,
University Hospital, Olomouc, 2Department of Neurology, 3Department of Experimental and Clinical Pharmacology,

Hospital Tinec-Sosna, Trinec, 3Comprehensive Stroke Medical University of Warsaw, Poland


Center, Department of Neurology, University Hospital and Introduction: Effective anticoagulation with vitamin K
Ostrava University, Ostrava, Czech Republic antagonists (VKA) is the treatment of choice for stroke
Introduction: Sonothrombolysis is a new therapeutic prevention in the majority of patients with atrial fibrillation
method used in patients with acute ischemic stroke (AIS). (AF). Considering growing vascular awareness, their
Various frequencies and intensities of used ultrasound are everyday use should be increasing.
being tested. The aim was to assess the safety and efficacy Aim: Our aim was to evaluate changes in the use of VKA
of sonothrombolysis using two diagnostic probes and before stroke onset in patients with pre-existing AF over the
bilateral monitoring in patients with acute occlusion of the past 15 years.
middle cerebral artery (MCA). Methods: We analyzed consecutive acute stroke patients
Methods: 12 consecutive AIS patients (7 males; age 47-78, admitted to our center between June 2006 and January
average 64.19.4 years) with acute MCA occlusion and 2011. Data were prospectively collected using a detailed
contraindication to thrombolysis were included in the study. stroke registry. We distinguished between three periods:
60-minute bilateral Doppler monitoring of the area of 1995-1999, 2000-2004 and 2005-2011.
occlusion was performed in all patients (Group 1). The Results: In the analyzed periods pre-stroke history of AF
control group consisted of 37 AIS patients (20 males; age was reported with similar frequency (years 1995-1999:
32-78, average 62.212.1 years) treated with standard 139/626, 22.2%; years 2000-2004: 346/1501, 23.0%; years
onothrombolysis and selected from the Thrombotripsy 2005-2011: 442/1872, 23.6%). It was present on their
Study database (Group 2). The differences in the number of routine ECG after admission in 22.4%, 20.7% and 20.1%,
recanalized arteries after a 60-minute treatment, number of respectively. We observed a significant (p<0.001)
independent patients (defined as a modified Rankin scale improvement in the use of VKA before stroke in patients
value of 0-2) after 90 days, number of symptomatic with pre-existing AF (8.0%, 14.3%, 27.9%), and fluctuating
intracerebral haemorrhages (SICH) were statistically prescription rate of VKA upon discharge (34.8%, 24.7%,
evaluated. 49.0%) in those patients. The use of subtherapeutic doses of
Results: Complete recanalization was detected in 4 (30.0%) VKA was much higher than the doses exceeding therapeutic
of Group 1 and 12 (32.4%) of Group 2 patients. 7 (58.3%) window (64.7% vs. 5.9%, 72.6% vs. 5.5%, 77.4% vs.
of Group 1 and 22 (59.5%) of Group 2 patients were 10.3%). During the hospital stay AF was diagnosed in
independent after 90 days. SICH was detected in none of additional 3.8%, 3.4%, and 6.9% of patients.
Group 1 and in 1 (2.7%) of Group 2 patients (p>0.05 in all Conclusion: The number of stroke patients with AF
cases). receiving VKA before stroke onset is systematically
Conclusion: According to the presented results, growing. However, the high ratio of patients receiving
sonothrombolysis using two probes and bilateral monitoring suboptimal doses points at constantly low guideline
is safe, but not more effective than standard sonothrombolysis adherence among physicians and patient non-compliance.
in AIS patients with MCA occlusion. Acknowledgement:
Supported by IGAMHCR grants NT/11046-6/2010,
NT/11386-5/2010, NT/13498-4/2012.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


150 Posters, Sunday 9 September

P1147 P1148
Predictive value of routine C-reactive Predictors of early seizures in acute
protein in ischaemic stroke patients stroke
treated with rtPA I. Volonghi1, A. Pezzini1, E. del Zotto1, A. Giossi1,
J.P. Bembenek1, M. Karlinski1, K. Grabska1, P. Costa1, L. Poli1, M. Gamba2, L. Antonini2, M. Magoni2,
A. Baranowska1, A. Kobayashi1, A. Czlonkowska1,2 A. Padovani1
1 nd
2 Department of Neurology, Institute of Psychiatry and 1Universit degli Studi di Brescia, 2Spedali Civili, Brescia,
Neurology, 2Department of Clinical and Experimental Italy
Pharmacology, Medical University of Warsaw, Poland Aims: To assess incidence and predictors of early seizures
Introduction: Blood biomarkers may be important (ES) in a prospective cohort of patients with acute first-ever
predictors of outcome after ischaemic stroke. However, stroke.
their clinical usefulness is not fully established yet, Methods: Patients consecutively recruited in the setting of
especially in a particular group of patients treated with the Brescia Stroke Registry were entered into the analysis.
intravenous rtPA. The aim of our study was to investigate ES (7 days) were recorded and correlated to clinical and
the predictive value of serum C-reactive protein (CRP) in demographic data, disease characteristics, and risk factors,
ischaemic stroke patients undergoing thrombolytic as well as to medical complications occurring within 7 days
treatment, adjusting for a history of infection recently of symptoms onset.
before stroke. Results: 516 patients [314 men; mean age 66.414.7; 436
Methods: Consecutive patients receiving intravenous rtPA (84.5%) cerebral infarct (IS), 80 (15.6%) intracerebral
in our stroke center (October 2003 - February 2010) were haemorrhage (ICH)] were enrolled. ES were observed in 20
included. Data were prospectively collected using a detailed patients (3.9%), 12 with IS (2.7%), 8 with ICH (10.0%). As
questionnaire including information about pre-existing expected, mean NIHSS at admission was higher in patients
conditions, laboratory findings and medications. Routine with ES than in those without (13.26 vs. 8.45), while
serum CRP was measured within 24 hours from admission. haemorrhagic lesion (RR, 1.56, 95%CIs 1.07-2.28),
Results: Serum CRP was measured in 197/232 patients particularly with lobar location (RR, 11.7, 95%CIs 4.63-
treated with intravenous rtPA. In 20/232 were reported signs 29.30), cortical involvement (RR, 1.42, 95%CIs 1.00-2.08),
of infection within 7 days before stroke onset. Patients with and haemorrhagic transformation of the infarct (RR, 1.51,
elevated CRP (>5 ng/ml) had more pre-existing 95%CIs 1.01-2.38) were independent predictors of ES, as
comorbidities, more severe strokes but a similar pre-stroke opposed to the neutral effect of vascular risk factors. The
disability, when compared to those with normal levels. In a occurrence of medical complications turned out to increase
multivariate analysis neither elevated CRP nor recent the risk of ES in the whole group (RR, 1.16; 95%CIs 1.07-
infection were associated with 3-month mortality. However, 1.26) as well as in that of patients with IS (RR, 1.17;
elevated CRP was independently associated with increased 95%CIs, 1.06-1.28), but not in that of patients with ICH
risk for death or dependency (OR 2.09, 95%CI: 1.03-4.26). (RR, 1.16; 95%CIs, 0.94-1.42).
Patients with not elevated CRP demonstrated high Conclusion: The occurrence of medical complications in
probability of not having sICH according to SITS (100%, the acute phase of stroke should be considered a further
95%CI:97-100%) and ECASS (97%, 95%CI:92-99%) predictor of ES in patients with brain infarction, but not in
definitions. those with ICH.
Conclusions: Elevated CRP may be an additional predictor
of unfavourable long-term outcome in thrombolysed
patients that could also facilitate bedside decision making
in cases with borderline eligibility for thrombolysis.
However, this should be verified in further studies.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 151

P1149 P1150
Effect of pre-treatment with statins on Factors affecting blood glucose and
ischemic stroke severity: do the doses blood pressure level in hyperacute
matter? ischemic stroke. Is mismatch a
P. Martinez-Sanchez, B. Fuentes, G. Ruiz-Ares, contributing factor?
E. Correas-Callero, M. Martinez-Martinez, S. Gur-Ozmen1, B. Aksay-Koyuncu1, S. Tavukcu-Ozkan2,
L. Cullar-Gamboa, E. Dez-Tejedor R. Tolun1, Y. Krespi2
Neurology, La Paz University Hospital. IdiPAZ Health 1Neurology, Istanbul Science University, 2Stroke Unit, Sisli
Research Institute, Madrid, Spain Florence Nightingale Hospital, Istanbul, Turkey
Objective: To examine the effect of pre-treatment with Background: Hyperglycaemia is detected in 30-50% and
statins, at high (80mg/d) and non-high (<80mg/d) doses, on high blood pressure (BP) is detected in 75% of acute
ischemic stroke (IS) severity in clinical practice. ischemic stroke (AIS) patients admitted to the emergency
Methods: Prospective study of IS admissions to our Stroke room (ER); most of them do not have history of diabetes
Unit during a 3-year period (2008-2010). Basal data, mellitus (DM) or hypertension (HT). Patients with
previous treatments (including statins), stroke severity salvageable brain tissue at admission can have higher
(NIHSS), stroke subtype, in-hospital complications, length glycaemic and BP levels and potentially be harmed by, or
of stay and functional status at discharge (mRS) were benefit the most from insulin and anti-hypertensive therapy.
collected. Mild stroke severity was defined as NIHSS 5 on Methods: The relationship between DWI/PWI mismatch,
admission. Good outcome was defined as mRS 2 at admission blood glucose (ABGL) and blood pressure level
discharge. Multivariable regression models and matched (ABPL) in patients admitted in the first 12 hours of AIS
propensity score analyses were used to quantify the onset is examined. Data of patients registered to Florence
association of statins pre-treatment, at high and non-high Nightingale AS database between 2006 and 2009 were used.
doses, with a mild stroke severity. Correlation between mismatch and ABGL or ABPL was
Results: Of 969 IS patients, 23% were using non-high analyzed with multivariate logistic regression analysis.
doses and 4.1% high doses of statins before the stroke. The Results: The sample consisted of 212 patients. The adjusted
frequency of mild stroke on admission was higher in the odds of mismatch in predicting high ABPL was 2.297
statin groups (57.9% for non-statins, 63.2% for non-high (p=0.064, 95%Cl 0.953-5.536) in the whole group, and it
doses of statins and 77.5% for high doses of statins, was 3.801 in the stress hyperglycaemia group (n: 67, %35)
p=0.026). After multivariable adjustment, pre-treatment (p=0.033, 95%Cl 1.110-13.015). Lower level of
with statins was associated with higher odds of mild stroke consciousness, anti-DM usage, Hba1c level and
severity and this effect was greater at high doses (OR=1.637, cardioembolic etiology were the other independent
95% CI 1.156-2.319 for the non-high doses and OR=3.297, predictors of high ABGL. Mismatch was not associated
95% CI 1.480-7.345, for the high doses of statins). with ABPL. Female gender (p=0.048) and total anterior
Conclusion: Pre-treatment with statins is associated with circulation syndrome (p=0.008) were found to be associated
lower stroke severity and this effect could be greater at with higher ABPL.
higher doses. Conclusion: Hyperacute ischemic stroke patients with
mismatch which is considered as potentially harbouring
salvageable penumbral brain tissue were more likely to
present with hyperglycaemia. On the other hand, a similar
relationship with mismatch and ABPL could not be
demonstrated.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


152 Posters, Sunday 9 September

P1151 P1152
The role of endothelial dysfunction in Functional outcome of acute ischemic
stroke stroke patients: association with
A. Inzhutova, O. Lopatina, E. Mervaala, A. Salmina concentrations of thyroid hormones and
Institute of Biomedicine, Helsinki University, Helsinki, C-reactive protein
Finland
H. Kazlauskas1, A. Bunevicius2,3, N. Raskauskiene2,
We investigated the role of endothelial dysfunction in A. Anskoliene1, R. Radziuviene2, V. Janusonis1,
development and progression of stroke compared to R. Bunevicius2
progressive angina or arterial hypertension. Known, 1Klaipeda University Hospital, Klaipeda, 2Institute of
endothelial cells are multifunctional organ with endocrine Psychophysiology and Rehabilitation, Lithuanian University
properties. Severe endothelial dysfunction in stroke leads to of Health Sciences, Palanga, Lithuania, 3Department of
disability of patients. Understanding of molecular Psychiatry, University of North Carolina at Chapel Hill,
mechanisms of endothelial dysfunction and detection of USA, Chapel Hill, NC, USA
biomarkers of endothelial disorders severity is the first step Objective: To evaluate the association of concentrations of
to successful prevention and treatment of stroke. In our thyroid axis hormones and C-reactive protein (CRP) with
study endothelial dysfunction (measured by ultrasound functional outcome of acute ischemic stroke patients.
examination of brachial artery diameter) was more Methods: 88 hospitalized patients (57 men and 31 women;
pronounced in patients with stroke (60 women, 50 men), mean age 7211 years) within 48 hours from the onset of
than in progressive angina (50 men, 40 women). It was ischemic stroke were included to this study. All patients
associated with the increase of intracellular calcium and were evaluated for severity of stroke using the Scandinavian
reactive oxygen species levels, endothelial apoptosis and the Stroke Scale (SSS) and Glasgow Coma Scale (GCS), and
rise of circulating endothelial progenitor cells number in the for serum concentrations of free tri-iodothyronine (T3), free
blood. Endothelial derived microparticles play an important thyroxin, and thyroid stimulating hormone and C-reactive
role in cell-cell communication and the development of protein (CRP). Functional outcome at discharge was
endothelial dysfunction. We discovered that specific antigen evaluated using the modified Rankin scale (mRS).
composition of circulating membrane microparticles Results: In univariate regression analyses worse functional
depends on cardiovascular pathology. Moreover, membrane outcome at discharge was associated with lower free T3
microparticles contain signal proteins such as MAPK and concentrations (=-0.230, p=0.04), with higher CRP
e-NOS and they are good biomarkers of endothelial concentrations (=0.486, p<0.001), with female gender
dysfunction severity. Stabilization of endothelial cell (=0.281; p=0.01), with advanced age (=0.386; p=0.012),
membrane by cytoprotectors had a beneficial effect on with lower SSS score (=-0.689, p<0.001), with lower GCS
endothelial function and recovery in patients after stroke. In score (=-0.290, p=0.01) and with more disability prior to
opposite, without targeted treatment of endothelial stroke (=0.398, p<0.001). In multivariate analyses, after
dysfunction mortality was 10% (p<0.01) and disability was adjustment for significant predictors in univariate analyses,
40% (p<0.001) in patients with stroke. In conclusion we higher CRP concentrations (=0.213, p=0.02), higher SSS
designed a method of endothelial dysfunction severity score (=-0.644, p<0.001) and more disability prior to
diagnosis by detecting novel biomarkers in the blood and an stroke (=0.251, p=0.003) were independently associated
algorithm for treatment of patients with stroke by the with worse results and together explained 57% (p<0.001) of
correction of endothelial function. variance of mRS score.
Conclusions: Worse functional outcome of acute ischemic
stroke patients at discharge is associated with lower free T3
concentrations and with higher CRP concentrations on
admission. After adjustment for clinical, demographic and
neuroendocrine factors, higher CRP concentrations are
independently associated with worse functional outcome.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 153

P1153 P1154
Effect of weekend or night compared with Endovascular coil embolisation for
daytime admission on outcome in acute wide-necked unruptured intracranial
ischemic stroke patients treated with aneurysms with enterprise stent:
endovascular therapy safety & efficacy
N. Nakai1, T. Kojima2, K. Shintai2, M. Hattori1, D. Itou1, S.-K. Hwang1, O.-K. Kwon2
R. Nishi1, S. Yamada1, K. Kawabata1, S. Yokoi1, Department of Neurosurgery, Ewha Womans University,
1

N. Mitsuma1, K. Yasui1, Y. Hasegawa1 College of Medicine, Mokdong Hospi, Seoul, 2Seoul National
1Neurology, 2Neurosurgery, Nagoya Daini Redcross Hospital, University Bundang Hospital, Seongnam, Republic of Korea
Nagoya, Japan Background: Stent assisted coil embolisation for wide-
Background and Purpose: A stroke weekend effect has necked unruptured intracranial aneurysms (UIA) is a
been demonstrated in many countries, but there is no report widely-accepted treatment modality. But, its technical
for effect of weekend admission in acute ischemic stroke results and clinical outcome are not well known. The aim of
(AIS) patients treated with endovascular therapy. We studied this study was to evaluate the safety and efficacy based on
AIS patients treated with endovascular therapy for weekend result of consecutive patients in a single center.
effect. Methods: This study included 116 patients (29 male
Methods: We report 38 AIS patients treated with patients, 87 female patients, mean age; 55.4 years) with 121
endovascular therapy between 2005 and 2011: 11 daytime UIA treated in one hospital by means of Enterprise stent
on working days (office hours) admissions and 27 weekend assisted coil embolisation between November 2008 and
or night time (non-office hours) admissions. December 2010. The clinical and radiological (magnetic
We compared the following data between office hours resonance angiography) results were evaluated.
admission group (OH) and non-office hours admission Results: Successful embolisation without complications
group (NOH); pre-treatment NIHSS, stroke onset to was achieved in 94%. 6 patients had procedure related to
admission time, admission to MRI time, admission to thromboembolic events. This resulted in neurologic
puncture time, recanalisation rate, and modified Rankin symptoms in 4 cases, and transient angiographically visible
scale (mRS) at 3 months. thromboembolism without symptoms in 2 cases. Aneurysm
Results: Between OH and NOH, there was no difference in occlusion was complete in 30.5%, small neck remnant in
pre-treatment NIHSS (24 vs. 19), and admission to MRI 49.5%, and residual contrast filling in 19.8%. Magnetic
time (34 minutes vs. 29 minutes). But there was statistical resonance angiography follow-up was performed at least 6
difference in stroke onset to admission time (77 minutes vs. months after coiling in all patients. The mean follow-up
56 minutes, p=0.034), admission to puncture time (105 duration was 13.4 months (range 6-34 months). 8 patients
minutes vs. 136 minutes, p=0.015) and recanalization rate (6.6%) demonstrated any visible recanalization. 1 patient
(90.9% vs. 33.3%, p=0.001). The rate of good outcome (0.8%) with major recanalization was successfully recoiled
(mRS 0-2) at 3 months in OH was similar to that of NOH and the others with minor recanalization were conservatively
(27.2 % vs. 25.9%). Most of our cases were internal carotid followed.
artery or proximal middle cerebral artery occlusion, as a Conclusion: Enterprise stent assisted coil embolisation for
result higher recanalization rate in OH has no influence on UIA appears to be effective and safe. Future studies
good outcome. including controlled prospective trials and careful follow-
Conclusions: There was no weekend effect in AIS patients ups are required to assess indications and efficacy in the
treated with endovascular therapy. long term.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


154 Posters, Sunday 9 September

P1155 P1157
Outcome analysis of the randomized Acute basilar artery occlusion: safety and
multi-center study on idiopathic normal efficacy of different treatment strategies
pressure hydrocephalus (SVASONA) and predictors of good outcome
J. Lemcke1, U. Meier2, C. Mller3, M. Fritsch3, T. Dork1, R. Herzig1, D. ak1, M. Krl1, T. Veverka1,
U. Kehler4, N. Langer4, M. Kiefer5, R. Eymann5, A. Brtkov1, P. Hlutk1, D. koloudk1, M. Heman2,
M.U. Schuhmann6, A. Speil6, F. Weber7, V. Remenez7, M. Roubec3, M. Kuliha3, V. Prochzka4, M. Kocher2,
V. Rohde8, H.-C. Ludwig8, D. Stengel9 P. Kaovsky1
1Department of Neurosurgery, 2Unfallkrankenhaus Berlin, 1Neurology, Comprehensive Stroke Center, Faculty of
Berlin, 3Ernst-Moritz-Arndt-University, Greifswald, Medicine and Dentistry, 2Radiology, Comprehensive Stroke
4Asklepios Hospital Altona, Hamburg, 5Saarland Medical Center, Faculty of Medicine and Dentistry, Palacky
University, Homburg, 6Eberhard-Karls-University, Tbingen, University and University Hospital, Olomouc, 3Neurology,
7Hospital Cologne-Merheim, Cologne, 8Georg-August- 4Radiology, Comprehensive Stroke Center, Ostrava

University of Gttingen, 9Center for Clinical Research, University and University Hospital, Ostrava, Czech Republic
Unfallkrankenhaus Berlin and Ernst-Moritz-Arndt-University Introduction: Acute ischemic stroke (AIS) caused by
of Greifswald, Berlin, Germany basilar artery occlusion (BAO) is often associated with a
Background: The aim of the prospective randomized severe and persistent neurological deficit and a high
SVASONA study was to prove, that an excellent clinical mortality rate. Various treatment concepts are being used.
outcome and a low rate of overdrainage complications can The aim was 1) to assess the association between outcome
be achieved with gravitational valves. and treatment type and 2) to identify predictors of good
Methods: In seven centres patients were enrolled in the outcome.
SVASONA study after passing clinical examination, Methods: The set consisted of 50 consecutive AIS patients
radiological assessment and CSF infusion test, CSF tap with radiologically confirmed BAO (37 males; mean age
test and or continuous ICP measurement. Patients were 64.712.4 years). Stroke severity at time of treatment was
randomly assigned in treatment groups with or without a assessed as severe (coma, locked-in state, tetraplegia) or
gravitational unit. The primary endpoint was the incidence mild to moderate. 30-day outcome was assessed using a
of overdrainage complications. Secondary endpoint was the modified Rankin scale (mRS) with good outcome defined
clinical outcome after 6 and 12 months. as score 0-3. The following treatments were used:
Results: 150 patients were included in the study. Due to a antithrombotics (AT); intravenous thrombolysis (IVT); IVT
significantly lower risk of overdrainage complications in the with subsequent intra-arterial therapy (IVT+IAT); intra-
treatment group with gravitational units, the study was arterial therapy alone (IAT). Other observed factors were
stopped according to the protocol after the planned interim age, mRS score before stroke onset, time to treatment,
analysis. recanalization rate.
69% of the patients in the treatment group without a Results: AT was used in 8, IVT in 12, IVT+IAT in 13, IAT
gravitational valve and 86% of the patients in the group in 17 patients. Good outcome was found in 0 AT,2 (16.7%)
with gravitational valves showed an excellent, improved or IVT,7 (53.8%) IVT+IAT, 3 (17.6%) IAT patients (p>0.05).
fair outcome according to the Black grading scale after 6 The following statistically significant differences were
months. After 12 months 75% respectively 86% were found between patients with good vs. poor outcome: mean
registered. The difference was not significant. age 54.2 vs. 68.0 years (p=0.0004); recanalization rate 91.7
Discussion: Whereas a significant difference between the vs. 47.4% (p=0.008); presence of severe neurological deficit
complication rates of the two treatment groups was evident, 25.0 vs. 65.8% (p=0.02). Logistic regression analysis
there is only a tendency in the difference between the identified age (OR=0.875; p=0.004), presence of severe
outcomes measured by specific iNPH scores. These findings neurological deficit (OR=0.079; p=0.023), time to
underline that gravitational valves prevent the risk of treatment (OR=0.602; p=0.035) as significant independent
overdrainage but do not suppress the favourable outcomes predictors of good outcome.
achieved with LPVs. Conclusion: In the presented study, an apparent (although
statistically insignificant) trend for better outcome was
P1156 observed in the IVT+IAT group. Age, severe neurological
deficit, time to treatment were identified as significant
Abstract cancelled independent predictors of good outcome. Supported by
grants NT/11386-5/2010,NT/11046-6/2010, NT/13498-
4/2012 (IGAMHCR),CZ.1.05/2.1.00/01.0030.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 155

P1158 P1159
Factors associated with platelet inhibition Prognostic significance of hemodynamic
in patients undergoing neurointerventional and hemostatic factors in the course of
procedures different manifestations of cerebral small
J. Savic, S. Vucetic, N. Radojkovic-Gligic, M. Savic vessel disease - study rationale and
Special Hospital for Cerebrovascular Diseases St. Sava, protocol
Belgrade, Serbia
J. Staszewski1, R. Piusiska-Macoch1, E. Skrobowska2,
Introduction: Significance of antiplatelet resistance is B. Brodacki1, A. Stepien1
not well defined among patients with cerebrovascular 1Clinic of Neurology, 2Department of Radiology, Military
diseases. We tried to determine factors associated with Institute of Medicine, Warszawa, Poland
antiplatelet resistance in a cohort of patients undergoing Cerebral small vessel disease (CSVD) is one of the most
neurointerventional procedures. important and common vascular diseases of the brain. It is
Material and method: In this retrospective study, we an important cause of ischemic and hemorrhagic strokes,
analyzed data of 65 consecutive patients in a one year period dementia and vascular parkinsonism. Although main
that underwent neurointerventional procedure and clinical and radiological signs of CSVD are well established
previously received antiplatelet therapy. We used impedance its prognostic factors and course have not been well studied
aggregometry method to determine the level of platelet so far.
inhibition. The aim of the study is to evaluate hemodynamic and
Results: Indications for neurointerventional procedures hemostatic factors and to assess clinical and radiological
were: carotid stenosis (56.4%), vertebral stenosis (48.4%), course of different manifestations of CSVD in long-term
and symptomatic intracranial aneurysm (24.2%). Among 56 observation.
patients receiving aspirin in a median dose 700mg per week, Method: A prospective, observational, with 24-month
10.7% were resistant. Among 24 patients receiving follow-up study in 150 CSVD patients (50 with lacunar
clopidogrel in a median dose 525mg per week, 21 of them stroke, 50 with vascular dementia, 25 with vascular
were on a dual antiplatelet therapy, and 60.4% of all were parkinsonism, 25 with deep intracerebral hemorrhage) and
resistant to clopidogrel. We found no differences between 50 healthy subjects from control group has been planned.
antiplatelet-responders and non-responders regarding The clinical and radiological course will be evaluated basing
gender, age, hypertension and concomitant statin therapy. on detailed neurological, neuropsychological and MRI
There were significantly less aspirin-responders among examinations. Hemodynamic (cerebral vasoreactivity, 24h
patients with diabetes and hyperlipidaemia. Stenoses of blood pressure control, MRI PWI/DWI) and hemostatic
neck blood vessels were more frequent in aspirin-non- (markers of endothelial and platelet dysfunction, brachial
responders. artery flow-mediated dilatation test) factors will be
Conclusions: Our results show that clopidogrel resistance determined.
is very common in patients undergoing neurointerventional Discussion: The study has the potential to further unravel
procedures, and aspirin resistance is common among these the predictors and course of different manifestations of
patients with diabetes and hyperlipidaemia. It emphasizes a CSVD. The scheduled trial will better define the prognosis
need for individualized approach for antiplatelet therapy in and prognostic factors of various clinical manifestations of
this group of patients. CSVD. These data may be helpful in considering the
development of preventive and therapeutic strategies in
CSVD especially in the context of individualization of the
treatment.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


156 Posters, Sunday 9 September

P1160 P1161
Utility of 64-section multi-detected The usefulness of time intensity curve
cardiac CT in detection of thrombus and generated from MRI/PWI to predict
circulation in left atrium appendage: neurological deterioration after onset in
comparison with trans-oesophageal acute ischemic stroke patients
echocardiography M. Nakazaki, T. Mori, H. Tajiri, T. Iwata, Y. Miyazaki,
F. Konoeda1,T. Dembo2, S. Yamada1, Y. Itoh1, K. Mizogami
M. Jinzaki3, N. Suzuki1 Department of Stroke Treatment, Shonan Kamakura General
1Neurology, School of Medicine, Keio University, 2Neurology, Hospital, Stroke Center, Kamakura, Japan
School of Medicine, Kyorin University, 3Radiology, School of Purpose: To investigate whether or not MRI/time intensity
Medicine, Keio University, Tokyo, Japan curve (TIC) can easily predict the neurological deterioration
Introduction: Trans-oesophageal echocardiography (TEE) of acute ischemic stroke patients.
has been utilized as the assessment for the embolic source Methods: Included for retrospective analysis were acute
in cardiogenic cerebral infarction with atrial fibrillation. stroke patients
However there is the problem that TEE imposes a heavy 1) who were admitted from 2008 to 2011 within 48hours
burden on the patient within the early phase of cardiogenic from onset,
stroke. Hence we assessed 64-section multi-detected cardiac 2) with mild neurologic symptoms of NIHSS10 without
CT in place of TEE in patients with cardiogenic stroke. disturbance of consciousness,
Methods: We investigated 22 patients with stroke (17 men 3) whose emergency MRI demonstrated an ischemic lesion
and 5 women; mean age 69.4 years) who had undergone in the MCA territory,
both CT and TEE within the previous 1-month period. We 4) who were treated without reperfusion therapy, and
decided the CT and TEE grades as below and compared 5) who had severe stenosis (70% or more) or occlusion of
each grade; CT the ICA or MCA in the affected side by DSA.
grade 1: no defect, We evaluated PWI findings by time-intensity-curve (TIC)
grade 2: no left atrium appendage thrombus and both defect grade, which were classified into four grades according to
during early phase and clear during late phase, the time to peak and the reduction value of the peak signal.
grade 3: defect (+), We investigated patients baseline features, TIC grades,
TEE grade 1: no thrombus, NIHSS on admission (adm_NIHSS), NIHSS on the 7th day
grade 2: we can detect spontaneous echocardiographic (7d_NIHSS) and difference between 7d_NIHSS and adm_
contrast (SEC) without thrombi, NIHSS (7d-adm_NIHSS). We defined neurological
grade 3: thrombi (+). deterioration as 7d-adm_NIHSS of more than 0.
Result: 14 of 22 (63.6%) were good match with the findings Results: We analyzed 71patients, median. 2 patients had
between the CT and TEE. Furthermore, SEC of TEE was grade 1 of TIC, 11 had grade 2, 38 had grade 3, and 20 had
well accorded with the feature of CT grade 2 (Sensitivity= grade 4. In 11 patients, 7d-adm_NIHSS was more than 0.
72.2%, Specificity=75%, Positive Predictive Value= Among the 11 patients, 9 patients (82%) had grade 1-2 of
92.8%). TIC at admission. Logistic regression analysis also
Conclusion: We have proved a significant correlation demonstrated grade 1-2 of TIC that was an independent
between enhancement patterns of multi-detected CT and significant predictor of neurological deterioration (OR,62.5;
TEE findings (p=0.036). 64-section contrast enhanced 95%CI,10.0-333, p<0.001).
multi-detected CT of the left atrium is a useful test of the Conclusion: In acute ischemic stroke patients with severe
method for evaluating thrombus and flow stasis in left stenosis or occlusion of the IC or MCA, MRI/TIC was
atrium appendage. useful in predicting neurological deterioration after
admission.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 157

P1162 Documented close relationships between ANS dysfunction


(HRV/BPV/BRS) iCI onset, and its contribution to
Heart and brain. Their mutual prognosisLP-PLA2. The largest study regarding LP-PLA2.
relationships. How can we explain this Significant increase in AH, CAD, iCI groups comparing to
connection and why is it important? controls. close direct correlation with changes in carotid
arteries. New era in understanding inflammatory
D. Bartko1, P. Blazicek2, Z. Gombosova1, L. Bubelkova3,
mechanisms of atherosclerosis. Important implication for
I. Combor1, L. Danihel3, J. Fabcin4, F. Rusnak5,
clinical practice.
A. Dukat6, J. Midriak7, Z. Prieselova7, J. Kubala8,
Conclusions: AF, ectopic abnormalities, HF, HRV, BP
E. Kurca9, V. Nosal9, I. Fillip1
variability, BRS and PL-PLA2 are major predictors of onset
1Institute of Medical Sciences, Neurosciences, Military
and outcome of stroke. All are associated with increasing
Health, Central Military University Hospital, Ruzomberok,
2Divison of Clinical Biochemistry, Alpha Medical Company, age, BMI, diabetes, dyslipidemia. These findings are
Bratislava, 3Dept. of Radiology, 4MRI Center, 5Dept. of
associated with stiff blood vessels and impaired baro-
Vascular Surgery, Central Military University Hospital, receptor function. Implications of these findings for
Ruzomberok, 62nd Dept. of Internal Medicine, Comenius guidelines on prevention and practice have yet to be
University, University Hospital, Bratislava, 7Department of established. Supported by EUgrants No2622022009 &
Neurology, 8Institute of Clinical Biochemistry, Immunology 26220220153
and Allergology, Central Military University Hospital,
Ruzomberok, 9Deparment of Neurology, Comenius
University, University Hospital, Martin, Slovak Republic
Introduction: Rhythmic abnormalities, HFs, ANS
dysfunction can contribute to decreasing cardiac output and
to onset of iCI. The not are always sufficiently estimated
and implicated in practice.
Aim: To analyse an impact of AF, rhythm disturbances,
heart failures (CAD, IHD, CVD), impaired ANS functions
on iCI onset/prognosis.
Material and methods: 3064 pts with iCI, confirmed by
CT/MRI, divided in 5groups:
1. AF (n=450),
2. ectopic abnormalities (n=320),
3. CAD, IHD, CVD (n=660),
4. HRV (n=160), BPV (n=212) BRS (n=590),
5. Heart-CBF (n=192), LP-PLA 2 (n=480).
Results:
AF: Risk of iCI is 6-fold higher when AF is present.
Implantable devices discovered association with subclinical
AF and significantly increase the ascertainment of AF.
Age: Risk increases with age. 50-59yrs: 6.7%, 80-89yrs:
36.2%.
Outcome: AF is independent RF for recurrent iCI (46.5%).
Mortality: 28-day mortality: 19.1% vs. 12.0% in non-AF
group, adjusted mortality risk: 20% higher, 3-month
mortality: 32.8% vs. 19.9%.
Neurological deficit: NIHSS is significantly more severe
compared to non-AFpts.
Lesion size: iCIs significantly larger, more disabling.Silent
infarction found in 87% pts..
ECG abnormalities found in 68.1% vs. 21.3% (Holter: conv.
ECG). In improved pts: 43.0% vs. 18%. In deteriorated pts:
68.0% vs. 33.0%.
Heart and Brain: isolated arrhythmia: CBF-8.0%,
AF-12.0%, A/V tachyarrhythmia 25.0%.
CI prognosis: Five ECG abnormalities created (peaked
P-wave, T-wave, longer QT arrhythmia, ST-changes).
Combination of two/three abnormalities are associated with
higher incidence, death, complications. CAD, IHD, CVD
(HF). Close correlation between HFs with or without
congestion and NIHSS was found (n=660) HRV/BPV/BRS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


158 Posters, Sunday 9 September

P1163 P1164
Ankle brachial index is a strong predictor Intra-hospital delays in stroke patients
of stroke events in the general population treated with rt-PA: impact of pre-
J. Gronewold1, M. Bauer2, N. Lehmann3, K. Krger4, admission notification to the emergency
S. Moebus3, K.-H. Jckel3, R. Erbel2, D. Hermann1, medical system and to the neurologists
Heinz Nixdorf Recall Study Investigative Group B. Casolla1,2, M. Bodenant1, M. Girot3, C. Cordonnier1,
1Vascular Neurology and Dementia, 2Cardiology, University
J.-P. Pruvo4, E. Wiel3, D. Leys1, P. Goldstein3
Hospital Essen, 3Medical Informatics, Biometry and 1Department of Neurology, Univ Lille Nord de France, CHU
Epidemiology, University of Duisburg-Essen, 4Angiology, Lille, EA 1046, Lille, France, 2Dep. of Neurosciences,
Helios Klinikum Krefeld, Germany Mental Health and Sensory Organs (NESMOS), SantAndrea
Introduction: Predictors of cerebrovascular events gain Hospital, University Sapienza, Rome, Italy, 3Department of
importance in secondary stroke prevention. Herein, we Emergency Medicine and SAMU 59, 4Department of
investigate the value of the ankle brachial index (ABI), a Neuroradiology, Univ Lille Nord de France, CHU Lille, EA
non-invasive marker of atherosclerosis, as stroke predictor 1046, Lille, France
in addition to established risk factors that are part of the Introduction: Pre-hospital notification enhances
Framingham Risk Score (FRS). thrombolysis rate and improves intra-hospital delays, but
Methods: 4299 subjects from the population-based Heinz- the impact of notification to the neurologist by the
Nixdorf-Recall Study (45-74 years; 47.3% men) without emergency medical system (EMS) call centre remains
previous stroke, coronary heart disease and myocardial unknown.
infarction, which received detailed baseline assessments, Our aim was to compare pre- and in-hospital delays in
were followed up for ischemic and hemorrhagic stroke stroke patients treated by intravenous (i.v.) rt-PA with and
events over 7.91.7 years. Cox regressions were used to without pre-hospital notification.
evaluate FRS variables [sex, age, smoking, diabetes, LDL, Methods: We compared baseline characteristics and
HDL, systolic blood pressure] and ABI as stroke predictors. in-hospital delays in stroke patients treated by rt-PA with a
Results: A total of 69 strokes (61 ischemic, 8 hemorrhagic) high-level notification (call to EMS and discussion EMS-
occurred during the observation period. Patients suffering neurologist), a low-level (call to EMS without discussion
stroke had significantly lower ABI values at baseline (1.02 EMS-neurologist) and no pre-hospital notification.
vs. 1.13, p<0.001). In multivariable regressions, ABI was a Results: Of 302 consecutive patients (165 women, 54.6%;
strong independent stroke predictor (hazard ratio=0.76 per median age 74 years, interquartile range [IQR] 59-83),
0.1, confidence interval=0.67-0.87; p<0.001) in addition to patients with high-level, low-level and no notification
age (1.49 per 5 years, 1.23-1.79; p<0.001) and systolic differed for the severity at admission (median national
blood pressure (1.34 per 10mmHg, 1.22-1.48; p=0.001). institutes of health stroke scale respectively of 12, IQR
ABI similarly predicted strokes in men and women and in 7-17; 9, IQR 6-15, and 8, IQR 6-14, p=0.029). Patients with
subjects above and below 65 years. In subjects belonging to high-level notification had shorter (i) admission-to-
the highest FRS (>13%) and lowest ABI (1.08) tertile completion of imaging times (27 min, IQR 14-35) than
stroke risk was particularly elevated. In the latter, low ABI patients with low-level notification (35 min, IQR 17-54) or
values significantly increased stroke risk (p for trend=0.009). no notification (36 min, IQR 30-58) (p<0.01); (ii) door-to-
Conclusion: ABI is a strong independent stroke predictor needle times (49 min, IQR 39-62 vs. 57 min, IQR 39-81 vs.
in addition to age and blood pressure, particularly in 63 min, IQR 51-97; p = 0.003); and (iii) onset-to-needle
subjects with vascular risk profile, where ABI identifies times (140 min, IQR 110-175 vs. 155 min, IQR 106-230 vs.
subjects at high stroke risk. 182 min, IQR 131-234; p < 0.001). They did not differ for
onset-to-admission time and imaging-to-needle time.
Conclusion: Pre-hospital notification by the EMS reduces
intra-hospital delays in patients eligible for rt-PA, but the
benefit is higher in case of discussion between the EMS and
the neurologist before admission.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 159

P1165 P1166
The immune response impairment was Intracerebral haemorrhage (ICH) patients
more pronounced in patients who as organ donors
developed severe ischemic stroke T. Sairanen1, A. Koivisto2, A. Koivusalo3, H. Isoniemi3,
P.A. Chemrukov1, N. Goncharova2, M. Potapnev2, T. Tatlisumak1
1Department of Neurology, Helsinki University Central
U. Shabalina1, I. Petrovich1, E. Oganova3, E. Sidorovich3
1Emergency Hospital, The Belarus Research Center of
2 Hospital, 2Medical Faculty, University of Helsinki,
3Department of Surgery, Division of Transplantation and
Transfusiology and Medical Biotechnologies, 3The Belarus
Research Center of Neurology and Neurosurgery, Minsk, Liver Surgery, Helsinki University Central Hospital,
Belarus Helsinki, Finland
Introduction: The association of inflammatory and Introduction: Intracerebral haemorrhage is the most
infectious processes with ischemic stroke has been devastating type of stroke. ICH is among the most common
identified by so far. The aim of our study was to define the reasons for brain death and thus carries the potential for
role of immune response impairment in patients who organ donation. We identified potential organ donors among
develop severe ischemic stroke. ICH patients.
Methods: In 89 patients with ischemic stroke the relative Methods: A retrospective chart review of consecutive ICH
content of T- and B-lymphocytes among peripheral blood patients treated at the Helsinki University Central Hospital
mononuclear cells were analyzed using the monoclonal (HUCH), 2005 to 2010 (n= 1 013). To assess the number of
antibodies CD3/CD4/CD8, CD4/CD154 and CD19/CD40 potential organ donors we identified patients who died
(Beckman Coulter) by flow cytometry. The serum levels of within 14 days, were 75 years of age, had no cancer or
separate inflammation markers were also determined. All other medical conditions that would be a contraindication to
patients were divided into four groups, i.e. the patients with organ donation, nor did they experience severe infection
mild (n=29), moderate (n= 24), severe (n=24) and extremely during hospitalization. The number of actual donations was
grave stroke (n=12). Healthy patients comprised the controls also assessed.
(n=13). Results: The number of deaths was 254/1013 (25%). Of
Results: Decrease in CD3+ (p=0.00007), these deceased 149 were 75 years (59%). There were 14
CD3+CD4+(p=0.02), CD3+CD8+ cells (p=0.02) was found with cancer (3 prostate, 2 breast, 2 lung, and one of bone,
in four groups of patients with stroke as compared to the liver, maxillary, lymphoma, sigmoid, melanoma, and
controls. The contents of CD3+ (54.010.4% vs. 63.68.7%, bladder). Of the remaining 135 9 had a prior medical
=0.028), CD3+CD4+ (30.210.4% vs. 39.65.7%, condition (3 rheumatoid arthritis, 2 type II diabetes with
=0.01), CD4+CD154- (21.25.9% vs. 32.75.3%, liver disease, and 1 microscopic polyangiitis, drug abuse,
=0.02), CD40+CD19- cells (51.20.03% vs. 64.44.4%, Eisenmenger syndrome, and carrier of ESBL). For 1 patient
=0.01) were lower, while those of CD19+ (13.66.5% vs. organ donation was denied by the bereaved. Among the
9.43.5%, =0.02), CD4+CD8+ cells (3.61.8% vs. remaining 126 patients 8 became donors. Of the remaining
1.81.3%, =0.02) were higher in patients with extremely 118 patients 24 had pneumonia and/or sepsis, and thus
grave stroke as compared to patients with mild stroke. The finally 94 of 254 (37%) patients who deceased were
reduction in T-helper cells correlated with the rise in hsCRP identified as missed potential donors. The conversion rate
(R=-0.34, p=0.01), while the increase in CD4+CD8+ and (actual donors divided by potential donors) was 8.5%.
CD3-CD8+ correlated with the severity of ischemic brain Conclusions: A considerable number of potential organ
lesions (R=0.049 , p=0.02). donors exists among deceased ICH patients.
Conclusions: Our findings suggest that stroke-induced
immune response impairment should be considered in
developing treatment strategy, especially in cases of
extremely grave stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


160 Posters, Sunday 9 September

P1167 P1168
Successful use of intravenous Endoscopic removal of intraventricular
recombinant tissue plasminogen (rtPA) in haemorrhage combined with post-
a pregnant woman with cardio-embolic operative intraventricular thrombolysis
stroke: a case report J. Kuzibayev
B. Ratajczak, S.B. Sando, A.H. Aamodt Neurosurgery, Republican Research Center of Emergency
Department of Neurology, St. Olavs Hospital, Trondheim, Medicine, Tashkent, Uzbekistan
Norway The treatment of acute intraventricular haemorrhage is still
Introduction: Pregnancy-related ischaemic stroke is a debatable. Positioning of an external ventricular drainage is
challenging medical problem. Historically, the use of rtPA a common procedure although with the high risk of
thrombolysis in pregnancy and early post-partum period has infection. Lately few authors have described the endoscopic
been regarded relatively contraindicated. The major evacuation of intraventricular clots as a treatment option for
concerns are the risk of bleeding and complications in both such cases. Intraventricular thrombolysis has been described
mother and child. The safety of thrombolysis in acute stroke with rewarding results since more than a decade. We used
in pregnancy is not clarified, as these patients are regularly endoscopic removal of intraventricular haemorrhage
excluded from trials. Case reports that show a beneficial combined with postoperative intraventricular thrombolysis
effect of thrombolysis in acute ischaemic stroke in in 24 patients with intraventricular haemorrhage. A rigid
pregnancy are scarce. endoscope (Aesculap) was inserted in the frontal horn
Results: The patient was a 33-year-old woman who through, after prolonged irrigation, partially organized
developed acute stroke symptoms, with right-sided haemorrhagic clots were detected and removed with the aid
hemiparesis and expressive aphasia in the 7th gestational of the suction tip. At the end of the procedure an external
week. Acute MRI of the brain showed restricted diffusion in ventricular drainage was positioned. A post-operative CT
three different areas in the left hemisphere. An intra-venous scan was obtained in all patients to assess the organized
rtPA treatment was administered according to a standard clots and post-operative intraventricular thrombolysis was
protocol. Her neurological symptoms improved rapidly, no performed to evacuate the clots. In no patient we observed
complications developed. The diagnostic work-up did not complication related to the procedure. The results of
reveal any carotid atherosclerosis, coagulopathy or treatment were compared with the group of patients (21)
immunological disease. Echocardiography showed PFO treated with single external ventricular drainage. All patients
with right-left shunt. She was discharged with prophylactic in the main group survived: good recovery was in 12
therapeutic enoxaparin treatment, and had no further patients, moderate disability in 7, severe disability - in 5. In
embolic events. At 38 weeks gestation, she delivered a the group treated with single external ventricular drainage
healthy boy. 7 patients died, good recovery was in 3, moderate disability
Conclusion: As of now, about 200 patients are reported to 3, severe disability in 8. Our experience suggests that the
have been treated with fibrinolytics in pregnancy for various endoscopic removal of intraventricular haemorrhage
medical conditions (thrombosed prosthetic valves, combined with post-operative intraventricular thrombolysis
myocardial infarction, pulmonary embolism and ischaemic of intraventricular clots is a useful treatment option for
stroke). Complication rates of thrombolytic therapy in patients with intraventricular haemorrhage.
pregnant patients are not higher than in the large randomized
trials. Careful consideration of individual risk and benefits
must be made before using thrombolysis in pregnancy. An
international registry of patients may be useful.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 161

P1169 P1170
The effect of a public information media Effect of weekday compared to weekend/
campaign on acute stroke presentation holidays admission on stroke outcome in
and management in an Irish hospital a comprehensive stroke unit
U. Moffatt1, N.A. Faheem2, D. Kelly3, P. Hickey4 J.C. Portilla1, R.M. Romero1, F. Lopez1, A. Falcon1,
1Medical 7, Sligo General Hospital, Sligo, 2Medical, M. Calle1, A. Serrano1, G. Gamez-Leyva1, M. Gomez1,
University College Hospital Galway, 3West of Ireland GP P.E. Jimenez1, M.D.M. Caballero1, J.M. Ramirez2,
Training Scheme, UCHG, Galway, 4Medicine, Sligo General I. Casado1
Hospital, Sligo, Ireland 1Hospital San Pedro de Alcntara, Caceres, 2Hospital
Introduction: Stroke is the third most common cause of Infanta Cristina, Badajoz, Spain
death and the most common cause of acquired major Background: Several studies have reported disparities in
physical disability in Ireland. In people with sudden onset treatment and clinical outcome between stroke patients
of neurological symptoms a validated tool such as FAST admitted on weekend compared to a weekday because of
(Face Arm Speech Time) should be used outside the hospital staffing level is reduced in those days. However, there is
to screen for the diagnosis of stroke or TIA. evidence that protocolized care in a Comprehensive Stroke
Objectives: To determine whether the FAST Campaign Center should overcome this effect. Our objective is to
increased public awareness of stroke and thus presentation compare the outcome of patients admitted for stroke in a
patterns of stroke/stroke-like symptoms to hospital, reduced Comprehensive Stroke Unit (CSU) according to the period
the time from onset of symptoms to presentation to hospital, of admission.
whether patients had CT brain within 3 hours, with view to Methods: Retrospective study. We included consecutive
thrombolysis if applicable. patients with acute stroke admitted in our CSU for 36
Methods: Retrospective audit undertaken on all patients months. Established protocols were applied. Two groups
admitted to Sligo General Hospital with stroke/TIA, six were categorized: A (weekday) and B (weekend/holidays).
weeks before and after, the official launch of the FAST We compared thrombolytic use, complications and outcome
campaign in Ireland, 6th May 2010 (i.e. between 25th March between two groups with adjustment for stroke type,
and 17th June). A list of audit candidates was compiled by severity and other demographic factors.
the Stroke Clinical Nurse Specialist using the Stroke Results: We included 622 patients (A:311, B:311). There
Register. Data was collected between October 2010 and were no significant differences in demographics, type or
January 2011 using a pro-forma. Data was analysed by the severity of stroke (NIHSS A: 6.9 and B: 7.1). Thrombolysis
Clinical Audit Support Team. was performed in A 8.7 % and B 7.7% (p0.05). Favourable
Results: Reduced time from onset of symptoms to hospital outcome (Rankin 2) were achieved in A: 59.5% and B:
presentation. Faster access to CT brain. Increased activation 58.8 % (p0.05). No differences in mortality were observed
of Stroke Pathway. Increased number of patients received (A 7.7% y B: 8%. p0.05).
thrombolysis. Increased numbers received anti-platelet Conclusions: At our CSU protocolized and continous care
within 48 hours. to the patients by neurologists and specialized nursing
Conclusion: Information media has a big influence on Irish overcome the effect that the admission period may have on
society and should be used as much as possible. 50% thrombolytic treatment and stroke outcome.
increase in suspected stroke hospital presentation within 3
hours. 50% increase in number thrombolysed.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


162 Posters, Sunday 9 September

P1171 P1172
Predictive value of acute measurement of HAT score predicts hemorrhagic
multiple plasma neuronal and astroglial transformation and functional outcome in
markers on neuronal damage following acute ischemic stroke patients treated
ischemic stroke with intravenous alteplase
S.-H. Oh1, S.-Y. Park1, J. Kim1, Y.-K. Kim2, D.-A. Shin3, D.-I. Chang, S.H. Heo
J. Song4, O.-J. Kim1 Department of Neurology, Kyung Hee University, College of
1Department of Neurology, CHA University, 2Institute for Medicine, Seoul, Republic of Korea
Clinical Research, CHA Bundang Medical Center, Background: Intravenous thrombolysis with alteplase is
3Department of Neurosurgery, CHA University, Seongnam,
the most effective therapy for acute ischemic stroke, but
4CHA Stem Cell Institute, CHA University, Seoul, Republic
hemorrhagic transformation (HT) is a potentially dangerous
of Korea complication of thrombolysis. However, there was lack of
Background and aims: Prediction of extensive neuronal research to find predictors of HT after thrombolysis in
damage in the acute phase of ischemic stroke is important Korean stroke patients.
for prevention of secondary neuronal injury. The objective Methods: From 2003 to 2009, patients with acute ischemic
of the present study is to investigate the predictive value of stroke who received intravenous alteplase were prospectively
plasma neuronal and astroglial protein levels on extent of enrolled into Kyung Hee University Hospital stroke registry.
neuronal damage in ischemic stroke. CT or MRI scan with gradient echo sequences was
Methods: We measured plasma concentrations of neuronal performed within 12-36 hours after IV thrombolysis.
(neuron specific enolase (NSE), neuroglobin (Ngb) and Hemorrhage after thrombolysis (HAT) score of each patient
visinin-like protein 1 (VSNL-1)) and astroglial (glial (range; 0- minimum risk, to 5- maximum risk) was
fibrillary acidic protein (GFAP) and S100B) markers in 175 collected. We evaluated the predictability of HAT score for
patients with acute ischemic stroke (6-24 hours after HT and symptomatic intracranial haemorrhage (sICH)
symptom onset). Total 109 non-stroke individuals were using c statistics.
included as control group. The extents of neuronal injury Results: Among 151 consecutive patients, 35 patients had
were assessed by measurement of infarct volume (5 to 7 HT on follow-up brain images. In multivariate logistic
days after symptom onset). regression analysis, atrial fibrillation (OR=2.709, 95%
Results: Among 5 neuronal and astroglial markers, plasma CI=1.118-6.567) and low density on CT scan (OR=3.419,
S100B, GFAP, and NSE levels were significantly elevated 95%CI=1.281-9.121) increased the risk of HT after IV
in the stroke group compared to the control group. In thrombolysis. Patients with sICH and unfavourable (mRS
Pearson correlation analysis, plasma S100B (r=0.72), GFAP 2-6) outcome at 3 months had higher HAT scores.
(r=0.68) and NSE (r=0.41) levels were significantly Conclusions: HAT score can be used for predicting the risk
correlated with infarct volume. In a multiple regression of sICH in intravenous thrombolysis. In addition, it can
model of biochemical markers (S100B, GFAP, NSE) after predict long-term clinical outcomes.
adjustment of confounding factors (age, sex, systolic BP,
fasting glucose, NIHSS at admission, C-reactive protein
(CRP), white blood cell count), NIHSS at admission
(=0.41, p<0.01), plasma GFAP (=0.31, t=5.7, p<0.01),
S100B (=0.29, t=4.9, p<0.01), CRP (=0.11, t=2.6,
p=0.01) were independent predictors for infarct volume.
Conclusions: Acute measurement of plasma S100B and
GFAP levels in addition to initial clinical severity score
provides valuable information for prediction of extensive
neuronal damage following ischemic stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 163

P1173 P1174
Intravenous rt-PA therapy for very old Complex interrelations between
stroke patients inflammation and vascular risk factors
C. Atsumi, H. Akiyama, H. Mizukami, K. Shimomura, determine cognitive impairment in acute
F. Maki, T. Yanagisawa, Y. Hasegawa ischemic stroke
Department of Neurology, St. Marianna University School of M.P. Danovska1, B.B. Stamenov1, M.L. Alexandrova2
Medicine, Kawasaki, Japan 1Neurology, 2Physics & Biophysics, Medical University
Introduction: In Japan, demographic changes will result in Pleven, Pleven, Bulgaria
a rapid increase of patients aged 90 years, but little is Introduction: Cognitive impairment following acute
known about outcomes in these patients after intravenous ischemic stroke is common, yet its determinants are not
recombinant tissue plasminogen activator (rt-PA) for acute fully understood
ischemic stroke. We aimed to assess safety and functional Objective: This study aims to identify significant
outcome in very old stroke patients treated with rt-PA. determinants, associated with increased risk of cognitive
Methods: We analyzed retrospectively collected data of 30 impairment in the acute phase of ischemic stroke.
consecutive acute stroke patients aged 80 years among 101 Patients and methods: The study was carried out on 47
all stroke patients treated with rt-PA in our hospital from patients (26 males and 21 females), median age 63 years,
October 2005 to September 2011. Their presenting with no baseline dementia. All the patients were assessed
characteristics, favourable outcome (modified Rankin scale with NIHSS, MMSE and mRS. Routine biochemical
0-2), mortality at discharge were compared between age 80 analyses and CT scan were performed within the first 24
to 89 (octogenarians) and 90 to 99 (nonagenarians). hours of stroke onset. Serum concentrations of hs-CRP
Results: 23 octogenarians (mean age; 83.73.0 years) and were measured on admission.
7 nonagenarians (mean age; 910.8 years) were eligible for Results: Our results showed that 57% of the patients had
our study. Gender and onset-to-needle time were similar cognitive impairment at discharge. We also established that
(60.7% women and 13441 minutes in octogenarians, and age (p=0.000), education (p=0.001), baseline systolic blood
57.1% women and 13925 minutes in nonagenarians). pressure (p=0.015), hyperglycaemia (p=0.021) and serum
Cardioembolic stroke was the most frequent stroke subtype hs-CRP level (p=0.050) on admission were significant
in both groups (19 of 23 octogenarians (82.6%) and 7 of 7 determinants of early cognitive deterioration. Of all the
nonagenarians (100%). Median NIHSS scores at admission variables, serum hs-CRP level (OR 1.12 (1.00-1.25),
and at discharge were 17 and 5 in octogenarians and 14 and p=0.049), gender (OR 5.97 (1.05-34.00), p=0.044) and age
10.5 in nonagenarians. Nonagenarians less often had a (OR 1.30 (1.09-1.55), p=0.004) were identified as
favourable outcome (14.2% vs. 30.4%, respectively) and independent predictors of post-stroke cognitive impairment.
had a higher incidence of mortality (14.2% vs. 4.3%, After baseline systolic blood pressure was entered in the
respectively) than did octogenarians. predictive model, gender lost its statistical significance of
Conclusions: Our study suggests less favourable outcomes independent predictor, which could be explained with
in nonagenarians as compared with octogenarians after gender-related variations of arterial hypertension.
rt-PA. Conclusion: Measuring serum hs-CRP level along with
some clinical and neuroradiological predictors is a rational
approach in the complex assessment of acute ischemic
stroke in view of treatment strategies and prevention of
cognitive deterioration.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


164 Posters, Sunday 9 September

P1175 P1176
Aspects of venous thrombo-embolism Stroke epidemiology in Shiraz, Southern
prophylaxis in patients with acute stroke Iran: a hospital-based single-center
I. Macavei1, I. Huza2, A. Macavei3 retrospective longitudinal study
1Neurology, University of Medicine and Pharmacy, Tirgu
A. Safari1,2, R. Safari1, A. BorhaniHaghighi1,
Mures, 2Neurology, Emergency Clinical County Hospital M. Sharifian1
Mures, 3General Medicine, Clinical County Hospital Mures, 1Shiraz University of Medical Sciences, 2Shiraz University,
Trgu-Mures, Romania Shiraz, Iran
Introduction: Stroke is the leading neurological disorder Background: Published data about stroke epidemiology in
which causes prolonged motor impairment, associated with Southern Iran are scarce. We tried to find the demography,
an increased risk of venous thrombo-embolism. socioeconomy and mortality of patients with stroke in a
Objectives: To assess the incidence of deep vein referral center in south of Iran.
thrombosiss (DVT) of lower limbs, appreciate the use of Methods: This was a hospital-based single-center
thromboprophylaxis in patients admitted to hospital with retrospective longitudinal study performed in Namazi
stroke with severe motor deficits and bedridden. Hospital, Shiraz, Southern Iran. Patients with the
Patients and methods: The study included 1101 patients, International Classification of Diseases, 9th edition-Clinical
out of which 975 (81.11%) had an ischemic stroke, 132 Modification (ICD-9-CM) codes of 160-169 were extracted
(10.98%) had a hemorrhagic stroke and 86 (6.90%) had a from hospital. database. Age, sex, inhabitation, coverage by
history of ischemic stroke associated with hemorrhagic charity insurance indicating for low socioeconomic status,
stroke and severe motor deficits. In order to diagnose DVT, length of hospitalization, discharge destination were
peripheral venous vascular Doppler was performed. Stroke investigated.
severity at admission was assessed using NIHSS. Work Results: 16,351 patients, 8759 (53.6%) males and 7592
methodology consisted of retrospective, partially (46.4%) females, were recruited. Mean age of the patients
prospective, clinical study. Sources of information were 63.4 (95% CI 63.1 to 63.6) years. 2326 (14%) patients were
clinical observation sheets, with gathering data about younger than 45 years was (young adult stroke). 428(0.026
thromboprophylactic treatment, type of therapy, side effects, %) of patients were in the paediatric age group (equal or
duration of bedridden period. less than 18 years old. 2935 (19.95%) of the patients
Results: Out of the followed patients, 16 had signs of DVT inhabited rural areas and 13,416 (80.05%) were from urban
on Doppler examination, but only 6 had clinical symptoms. areas. 875 (5.35%) patients were from very low
According to NIHSS, 2.90% had severe neurological socioeconomic status covered by charity insurance. Mean
impairment (>25 points), 33.60% had moderate impairment hospital stay was 6.3 (95% CI 6.2 to 6.4) days. Stroke
(5-14 points) and 63.48% had mild impairment (<5 points). hospital mortality rate was 20.5%, significantly higher in
277 patients (25.19%) received low molecular weight females, paediatric age group, patients from low
heparin, while in 63 patients (5.72%) mechanical methods socioeconomic status. (all P values <0.001). Mortality rate
were used. Hemorrhagic side effects were encountered in of the stroke patients was significantly increased from 2001
small percentage, not exceeding the numbers from the till 2009. (P value <0.001).
reference literature. Thromboprophylactic treatment was Conclusions: Stroke is a major health issue in Iran with
received within 24 hours from admission in 90.5% of the higher mortality and larger proportion of young adults in
patients. comparison to Western countries.
Conclusions: Thromboprophylaxis in patients with stroke
is underused or improperly used. Risk is perceived as being
smaller than in reality, because half of patients with DVT
are asymptomatic.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 165

P1177 P1178
Evaluation of the lesions in the left A case of only peripheral type facial palsy
thalamus in patients with subcortical in ponto-medullary junction area
vascular cognitive impairment using 1H infarction
magnetic resonance spectroscopy and J.H. Han, D.E. Kim
diffusion tensor imaging Department of Neurology, Seoul Veterans Hospital, Seoul,
Republic of Korea
P. Lv, Y. Dong, L. Li
Department of Neurology, Hebei General Hospital, Introduction: Unilateral facial palsy of the peripheral type
Shijiazhuang, China without other neurologic deficits is mostly diagnosed as
Objective: To investigate the lesions of the left thalamus Bells palsy. We present an unusual case of ipsilateral
and the correlation with cognition in patients with SIVD by peripheral facial palsy in dorsolateral ponto-medullary
using 1H magnetic resonance spectroscopy (1H-MRS) and infarction without other neurologic deficits.
diffusion tensor imaging (DTI). Case report: A 69-year-old woman was admitted with
Methods: 14 patients with VaD, 14 patients with VCIND, sudden left facial weakness. She had no other neurologic
and 12 normal controls (NC) were recruited. The quantitative deficit, except for left peripheral type facial palsy. She had
analysis of N-acetylaspartate (NAA), myoinositol (mI), a presumptive diagnosis of Bells palsy. Nerve conduction
Choline (Cho) and Creatine (Cr) resonance signals in region study showed normal terminal latency and compound
of interests (ROI) located in the left thalamus were muscle action potential (CMAP) amplitude in right and left
measured. Fractional anisotropy (FA) and mean diffusivity facial nerves. Blink reflex test showed normal R1 responses
(MD) in the same region were measured by DTI. The on both sides. But, ipsilateral R2 and contralateral R2
relationship was described among the MRS, DTI ratios and responses were not obtained by the stimulation on the
cognitive impairment reflected in MMSE and trail making affected side. These findings were indicative of left facial
test (TMT). neuropathy, due to left medullary lesion. Diffusion weighted
Results: (DWI) brain MRI demonstrated high signal lesion in left
(1)The NAA/Cr ratio showed a gradual decrease in the left dorsolateral ponto-medullary junction. Apparent diffusion
thalamus of SIVD patients compared with controls coefficient (ADC) brain MRI showed low signal lesion in
(F=3.656, p=0.038). No significant difference was found the same area.
between VaD and VCIND patients. Conclusion: Occasionally, peripheral type facial palsy was
(2)Compared with controls, patients with VaD and VCIND found in pontine infarction. But, isolated peripheral facial
demonstrated increased MD value in the left thalamus palsy without other neurologic defects is a rare condition in
(F=3.882, p=0.030). No significant difference between VaD brainstem infarction. Generally, if a patient presents with
and VCIND patients was observed (p>0.05). peripheral type facial palsy without other neurologic
(3)No correlation was found between MRS value and DTI abnormalities, brainstem lesions were not considered. This
parameters in the ROI (p>0.05). (4)A significant positive case shows us that ponto-medullary infarction presents only
correlation was observed between TMTb time and Cho/Cr peripheral type facial palsy without other neurologic
(r=0.520, p=0.001) and also MD value in the left thalamus abnormalities. So, if a patient admits with peripheral type
(r=0.305, p=0.044). facial palsy, we must make up electrophysiological study
Conclusions: The axonal loss or dysfunction and diffusion and brain image.
abnormalities in the thalamus could be found in early SIVD.
The Cho/Cr value in the left thalamus and MD values in
bilateral thalamus were correlated with executive function.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


166 Posters, Sunday 9 September

P1179 P1180
Improving factors for patients with left Real time measurement of extracellular
unilateral spatial neglect glutamate release in a global ischemic rat
M. Yamada1, Y. Nakamura1, M. Kawaguchi1, Y. Satoh1, model treated with nimodipine
A. Satoh2, M. Tsujihata2 H.-K. Park1,2, G.-J. Lee1,2, S.-W. Kang1,2, S. Choi1,2,
Rehabilitation, 2Neurology, Nagasaki Kita Hospital,
1
S.-K. Choi3
Nagasaki, Japan 1Kyung Hee University, College of Medicine, 2Healthcare

Objectives: The aim of this study was to determine the Industry Research Institute, 3Kyung Hee University Hospital,
factors to ameliorate USN. Seoul, Republic of Korea
Patients and methods: 51 patients (32 male and 19 female, Background and aims: It is generally thought that cerebral
ages 40-85, average educational level 11.3 years, all right- ischemia is associated with extracellular concentrations of
handed) with a right hemisphere stroke who were admitted the excitatory amino acids. It was known that nimodipine
to the hospital for rehabilitation were screened for USN. All combined with L-type voltage-gated calcium channels
patients were evaluated at 1 month and 3 months using The prevented calcium influx inside the cell. In order to define
Behavioural Inattention Test (BIT). The cut-off value of 131 the effect of nimodipine on glutamate release, we monitored
was used to determine whether USN present or not. The extracellular glutamate release in real-time in a global
patients who showed a BIT score over 131 at 3 months were ischemia rat model with eleven vessel occlusion.
classified as the improved group (17 patients) and those Methods: Changes in cerebral cortical blood flow (CBF)
with less than 131 were the unimproved group (34 patients). were monitored by laser-Doppler flowmetry with cortical
In total 17 items were studied to determine the factors glutamate levels by amperometric biosensor. A ten minute
associated with the improvement of USN; ages at onset, 11VO cerebral ischemia was initiated by pulling the snares
educational level, MMSE, KOHS block design test, Simple on the common carotid arteries. Nimodipine was permeated
test for evaluating hand function (STEF), Trail making test from the burr hole beside of CBF probe during the beginning
(TMT-partA), Functional Independence Measure (FIM) of the ischemic period. We performed Nissl staining for a
(total score, mobility ADL and cognition), BIT (total score, histological assessment of neuronal cell damage.
and its subitems; line, letter and star cancellation, line Results: During the ischemic period, the mean maximum
bisection, and figure and shape copying). change in glutamate concentration was 133.222.57M in
Results: The average scores of 12 items except for age, the ischemia group and 75.424.22M (p<0.001) in the
education level, MMSE, TMT part 2, and FIM cognition at group treated with nimodipine. The total amount of
1 month were significantly higher in the USN improved glutamate released was significantly different (p<0.001)
group than those in the unimproved group. between groups during the ischemic period. The %cell
Conclusions: The present study indicated that high scores viability in hippocampus was 47.505.64 (p<0.005) in the
on the Bit behavioural test, shape copying, and STEF ischemia group, compared with the sham group. But, the
evaluated within 1 month after the onset of stroke serve to %cell viability in the nimodipine treatment group was
predict a good prognosis of USN. 95.466.60 in the hippocampus (p<0.005).
Conclusion: We suggest that nimodipine treatment is
responsible for the protection of neuronal cell death through
the suppression of extracellular glutamate release in the
11-VO global ischemic model of rats.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 167

P1181 P1182
Prevalence of combinatorial genetic Successful intra-arterial thrombolysis in a
polymorphisms of CYP2C9 and VKORC1 patient with subarachnoid haemorrhage
in acute stroke patients: implications for developed after bridging intravenous and
warfarin management intra-arterial thrombolysis
S. upe1, Z. Poljakovi2, N. Boina3, V. Matijevi2, J.-H. Kwon1, W.-J. Kim1, M.-S. Kim2, S.-H. Lee2
A. Bazina2, A. Miima2 Neurology, 2Ulsan University Hospital, Ulsan, Republic of
1
1Department of Neurology, University Hospital Center Korea
Zagreb, 2Department of Neurology, 3Department of Subarachnoid haemorrhage (SAH) is a contraindication of
Pharmacology, University of Zagreb, School of Medicine, intravenous thrombolysis (IVT) with rt-PA. Reocclusion
Zagreb, Croatia after IVT is a well-known complication and intra-arterial
Background: Patients with cardioembolic stroke or stroke thrombolysis (IAT) is a relatively common procedure to
due to extracranial artery dissection or cerebral sinus open the re-occluded vessels. We report a patient who had
thrombosis require urgent anticoagulant therapy initiation. recurrent acute ischemic stroke which was successfully
Among those patients, risk of embolic stroke recurrence is recanalized with IAT in spite of localized SAH developed
5-7% within the first week with an increased risk of after IVT and IAT for acute ischemic stroke.
bleeding into the infarct zone. Many reports confirmed that A 60-year-old woman with atrial fibrillation developed left
CYP2C9 and VKORC1 genetic polymorphisms have strong hemiparesis and gaze preference to the right side suggesting
influence on interindividual warfarin sensitivity and right middle cerebral artery (MCA) territory infarction.
significantly alter the effective warfarin dose. In acute Brain CT at 20 minutes after symptom onset showed no
stroke patients it is important to select initial doses based on haemorrhage and IV rt-PA was administered. MR
individual genotype in order to achieve earlier angiography performed at 1 after IVT showed M1 occlusion
anticoagulation and to reduce the risk of developing of the right MCA. The right MCA was recanalized after IAT
warfarin side effects in early initiation of therapy, with urokinase and tirofiban. Her focal neurologic deficits
particularly among carriers of multiple allelic variants. were fully recovered despite localized SAH in the left
Methods: We determined the frequency of alleles of parietal lobe. We treated her with anticoagulation for
CYP2C9(*2*3) and VKORC1(-1173C>T) genes among prevention of recurrent ischemic stroke in spite of SAH.
106 patients hospitalized for acute stroke with indications However, she re-developed a right MCA territorial infarct
for anticoagulant therapy. Among them were 36 previously again at 3 days after successful thrombolysis. MR
anticoagulated patients. The impact of CYP2C9(*2*3), angiography at that time revealed re-occlusion of the right
VKORC1(-1173C>T) genetic polymorphisms on warfarin MCA. IAT with penumbra and urokinase was re-tried and
dosage was gauged using a published algorithm. We the right MCA was completely recanalized. She recovered
analyzed which alleles of CYP2C9(*2,*3) and VKORC1- to 2 of modified Rankin Score at 3 months and her SAH
1173C>T contribute to achieve earlier anticoagulant effect, was not aggravated.
stable maintenance dose, which allelic variants spent the In conclusion, localized SAH could occur after IVT. IAT is
longest time in therapeutic INR range and which contribute a considerable treatment option when a recurrent acute
to undercoagualtion. ischemic stroke developed in a patient with SAH after IVT
Results: Target INR was firstly achieved among the patients and IAT.
with polymorphism for both defective alleles (VKORC1TT,
CYP2C9*3/*3). Application of pharmacogenetic algorithm
enabled the correct assessment of dose in 81.5% patients
who required a higher or lower warfarin dose. The most
common genotype among previously under-anticoagulated
patients who developed stroke with a share of 34% was a
wild CYP2/C9 wt/wt genotype.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


168 Posters, Sunday 9 September

P1183 P1184
Ischemic stroke as a late complication of The territorial division and clinical
brain radiotherapy during childhood: a characteristics of anterior circulation
case report cerebral infarction
C. Parmentier, Y. Vandermeeren, P. Laloux, E. Mormont I. Stakena1, I. Zimaisa1, G. Enina1, I. Kikule1,
Neurology, CHU Mont-Godinne, Yvoir, Belgium A. Platkajis2
1Department of Neurology and Neurosurgery, 2Department of
Background: Neurologists should be aware that survivors
of childhood brain cancer have an increased risk of stroke. Radiology, Riga Eastern University Hospital Gailezers,
Stroke can occur as a delayed complication of brain Riga, Latvia
radiotherapy. We report a case that highlights the increased Introduction: In the classification of anterior circulation
risk of long-term cerebrovascular complications in such cerebral infarctions (ACCI) into subtypes there is not
patients. sufficiently considered the involved artery, the territorial
Case-report: A 26-year-old patient presented with acute area of blood supply and the size of infarction.
sensory loss involving the right face, arm and leg and Patients and methods: 424 patients were examined with
clumsiness of the right arm. His previous medical history ACCI who were treated at the Stroke Unit. All cases CI was
was remarkable for smoking, alcohol abuse and a cerebellar the first presentation and lacunar CI were not included. The
pilocytic astrocytoma discovered at the age of four, treated atherotrombotic CI were detected in 68.9%, but
by surgery followed by radiotherapy and chemotherapy. cardioembolic CI - in 31.1% of patients.
Neurological examination revealed right hemihypesthesia, Results: The vast majority of the patients were admitted
right hemiataxia, right Babinski sign and a weakness of the with MCA CI (98.1%) and only 1.2% had ACA and 0.7%
right arm (NIHSS=7). Head computed tomography revealed AChA infarcts. According to the damaged brain area MCA
calcifications of the left cerebellum, left temporal lobe and and ACA CI were divided into territorial areas of
of the basal ganglia. A brain MRI showed left thalamic and leptomeningeal (lm) and perforal (perf) arteries. The
parahippocampal hyperintense lesions in FLAIR and DWI analysis of the territorial area was only performed for the
with a decrease of the apparent diffusion coefficient (ADC), MCA blood supply area. Most often MCA infarction was at
consistent with an acute ischemic stroke in the territory of territory of lm arteries (73.6%) and of perf arteries only in
the left cerebral posterior artery. Magnetic resonance 26.4%. Of all ACCI partial large infarction of the MCA lm
angiography confirmed an occlusion of the left P2 segment. area was found significantly more often (44.1%) (p<0.005).
Carotid and vertebral duplex-ultrasound and cardiac workup The incidence of other subtypes of MCA infarction (total
were normal. Blood tests only showed a moderate MCA, partial small MCA lm, large MCA perf and small
hyperlipemia. Thrombophilia screening was negative. MCA perf) were similar and <15%. The clinical
Alpha-galactosidase activity was normal. characteristics of different MCA infarction subtypes differ
Discussion: This case illustrates a late manifestation of mainly according to the size of CI and territorial blood
radiation-induced complication, namely intracranial steno- supply area.
occlusive disease and mineralizing microangiopathy. In Conclusion: Elaborated supplement of ACCI classification
brain cancer survivors, the stroke workup should be based on the involved arteries, territorial blood supply area
extensive and take into account unusual aetiologies such as and the characteristics of infarction size is suitable for use
radiation-induced intracranial steno-occlusive disease. in neurovascular clinical practice.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 169

P1185 P1186
Predilection for antihypertensive therapy Stroke in atrial fibrillation can it be
in patients from the stroke-risk group prevented?
E. Vereshchagina, E. Isakova, A. Kotov V. Turcanu1, A. Grosu2, N. Diaconu2, C. Gratii2,
Neurology, M.F.Vladimirsky Moscow Regional Clinical and G. Pavlic3
Research Institute, Moscow, Russia Clinical Military Hospital, 2Institute of Cardiology,
1

Introduction: Arterial hypertension (H) is a most Institute of Neurology and Neurosurgery, Chisinau,
3

investigated risk factor (RF) of cerebrovascular diseases Moldova


available for correction, but efficiency of AH therapy is low. Introduction: Despite the known increased stroke risk
Materials and methods: A total of 197 AH patients were associated with atrial fibrillation (AF) and the benefit of
asked to fill in questionnaires in order to study their medical oral anticoagulation (OAC) in high-risk patients, still ~20%
literacy and predilection to antihypertensive therapy (AHT). of all ischaemic strokes are atrial fibrillation related.
Results: The patients were asked what index of arterial Aims: We aimed to evaluate the frequency of inappropriate
blood pressure (ABP) they considered normal: 12.7% - anticoagulation in all patients admitted with ischemic stroke
BP>140/80mmHg; 32.9% - 140/90-70; 23.4% - <140/90, and AF and to calculate the theoretical number of
but >120/80; 25.4% - 120/80; and 5.6% patients could not preventable strokes in case of proper guideline adherence
determine their normal ABP values. Regularly 58.4% and also to assess secondary stroke prevention at discharge.
patients underwent AHT, not regularly - 37.1%; never - Methods: In this cross-sectional study, all patients with
4.7%. The causes of irregular or refuse of AHT; 58.5% ischemic stroke admitted to our hospital during January
BP was not always elevated being normal sometimes 2004 and December 2004 in which the diagnosis AF was
without preparations; 34.1% drugs were harmful for their either known or established during hospital stay were
stomach, liver and kidneys, etc., 25.6% did not feel their identified.
elevated ABP; 17.1% forgot to take preparations; 17.1% Results: We have included 206 patients with AF and
were afraid to get accustomed to the drugs; 1.2% ischemic stroke. We have calculated the number of
complained of the unpleasant taste of the tablets; 12.2% preventable strokes in case AF patients would have received
complained of the drug side effects; 12.2% considered adequate antithrombotic treatment on admission. On
the drug cost too high; 15.9% refused to take so many admission, in 59% of the OAC eligible known AF patients
tablets; and 4.9% considered AHT ineffective. the drug was withheld. Improved antithrombotic guideline
Conclusion: The results obtained are indicative of the low adherence potentially would have prevented 54 out of the
predilection to AHT, which is due to a low medical literacy 206 (26%) ischemic strokes. At discharge at least 48% of
of the population. It should be noted that just the personal the patients were still insufficiently protected against
attitude to the drugs (and not their high cost) was a main recurrent stroke.
cause of patients refusal of AHT. Conclusion: Many known AF patients admitted with
ischemic stroke lack adequate antithrombotic treatment on
admission. Antithrombotic guideline adherence in these
patients could prevent a substantial number of strokes, but
secondary stroke prevention at discharge is also suboptimal.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


170 Posters, Sunday 9 September

P1187 P1188
Mechanical thrombectomy with Solitaire Clinical and ultrasonographic
AB device in basilar artery occlusions characteristics in anterior ischemic optic
E.J. PalacioPortilla1, A. GonzlezSurez1, neuropathies (AION)
J.M. Navasa Melado2, I. GonzlezAramburu1, D.C. Jianu1, S.N. Jianu2, L. Petrica3, D.F. Muresanu4,
O. AchaSalazar3, F.J. NovoRobledo3, S.M. Deme5, M. Petrica1
M.A. RevillaGarca1, M. RebolloAlvarez-Amandi1, 1Neurology, Victor Babes University of Medicine and

A. GonzlezMandly2 Pharmacy, County Emergency Hospital, 2Ophthalmology,


Neurology, 2Radiology, 3UARH, Hospital Universitario
1
Military Emergency Hospital, 3Internal Medicine-
Marqus de Valdecilla, Santander, Spain Nephrology, Victor Babes University of Medicine and
Introduction: Acute ischemic stroke (AIS) due to basilar Pharmacy, County Emergency Hospital, Timisoara,
4Neurology, University of Medicine and Pharmacy, Cluj-
artery occlusion (BA) has a poor natural history. Early
recanalization of occluded brain arteries in AIS is associated Napoca, 5Neurology, West University, Arad, Romania
with better clinical outcome. Our aim is to describe four Introduction: AION represent a segmental infarction of the
patients with AIS due to BA occlusion who had been treated optic nerve head supplied by the posterior ciliary arteries
with mechanical thrombectomy with Solitaire AB stent. (PCA).
Methods: Description of four clinical cases of acute BA Purpose: To describe characteristic clinical features and
occlusion treated with mechanical thrombectomy with ultrasound findings which help to differentiate newly
Solitaire AB stent in our hospital. diagnosed arteritic AION (A-AION) from non-arteritic
Results: Four patients (three females and one male, age AION (NA-AION).
range 52-81 years) with acute BA occlusion received Patients and methods: In this prospective study, 37
treatment with mechanical thrombectomy. Intravenous consecutive patients with clinical suspicion of unilateral
thrombolysis was previously administered in two cases. AION were examined following a complex protocol
Thrombectomy revascularization, defined as a final grade including Color Doppler imaging-CDI of orbital vessels
of 2b or 3 according to the thrombolysis in cerebral (Logic 500, GE with 9MHz linear probe).
infarction (TICI) score, was successful in all cases. At 3 Results: The final diagnoses were A-AION due to GCA in
months, one patient had died and the other three were 5 patients, all of them with biopsy-confirmed disease, and
functionally independent (modified Rankin scale 0-2). 32 patients with NA-AION. A combination of a history of
Conclusion: Thrombectomy with Solitaire AB stent amaurosis fugax before an abrupt, painless, and severe loss
achieved immediate recanalization with good clinical of vision of the involved eye, with a diffuse pale optic disc
outcome in most cases. Endovascular therapy with this oedema was extremely suggestive of A-AION. However,
technique may be considered as an efficient tool for the none of these symptoms was ever found in NA-AION. The
treatment of AIS due to BA occlusion. CDI of the orbital vessels in A-AION revealed low blood
velocities, especially end-diastolic velocities, and high RI in
all retrobulbar vessels, in both orbits (with severe diminished
blood flow velocities in the PCA, especially on the affected
side). In nonarteritic AION blood velocities and RI in PCA
were relatively preserved.
Conclusions: CDI of retrobulbar vessels data supported the
evidence of involvement of the entire trunk of the PCA in
the A-AION. In contrast, in the NA-AION, the impaired
flow to the optic nerve head was distal to the PCA
themselves, possibly at the level of the paraoptic branches
(1/3 of the flow of the PCA).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 171

P1189 P1190
Interhemispheric differences of cerebral Clinical features of the perimesencephalic
perfusion and brain DC potentials non-aneurysmal subarachnoid
influence verbal fluency in patients with haemorrhage: a report of 35 cases
vascular encephalopathy L. Yin
V. Fokin, N. Ponomareva, M. Krotenkova, R. Konovalov, Department of Neurology, 2nd Hospital of Dalian Medical
M. Tanashian, O. Lagoda University, Dalian, China
Research Center of Neurology RAMS, Moscow, Russia Objective: To analyse clinical manifestations and imaging
Aim: In healthy subjects left frontal and temporal cortex features of perimesencephalic non-aneurysmal subarachnoid
plays a main role in verbal fluency performance, however in haemorrhage (PNSAH).
patients with chronic cerebrovascular disease there is not Materials and methods: Clinical data of 35 cases with
much information about the brain structures participating in PNSAH were collected in this study. All cases were admitted
this cognitive process. This study was aimed at determining to the Department of Neurology, the Second Affiliated
brain structures influencing verbal fluency in patients with Hospital of Dalian Medical University, from January 2001
vascular encephalopathy (VE). to June 2010. The clinical manifestations, imaging features
Methods: DC potentials and cerebral perfusion were and prognosis, were retrospectively investigated and
investigated in 40 patients with VE. DC potentials were concluded.
registered from head using 12 Ag/AgCl electrodes placed Results:
according to 10-20 system with reference on right wrist. DC 1. Clinical symptoms are mild in these cases with Hunt-
potentials reflect intensity of cerebral energetic metabolism Hess grade I-II, with no consciousness disturbance, focal
(Fokin, Ponomareva, 2003). Standard characteristics of neurological deficits and hydrocephalus, with few (8.6%)
computed tomography perfusion in the brain were measured cerebral vasospasms which recovered quickly by
before and after word fluency test (WFT). symptomatic treatment.
Results: Characteristics correlating with word production 2. CT showed that the blood mainly distributed in
in WFT was minimized in multiple regression model surrounding mesencephalon cisterna and anterior pontine
(R=0.96; F=34.5; p<0.00001). According to this model left cisterna. The blood spread into the cerebral longitudinal
frontal, right parietal DC potentials as well as fissure and fissure of Sylvius in few cases (5.71%, 2/35).
interhemispheric differences between CBF in right and left Most CT images were classified into Fisher Grade 1~2, 2
frontal cortex, CBV in right and left thalamus, difference of cases were Grade 3, there was no Grade 4 case. All cases
DC potentials between right and left parietal cortex had a negative DSA examination performed within one
influenced the WFT performance. week after onset, and repeated DSA showed no abnormality
Conclusion: Interhemispheric differences of brain after 3 months of onset.
perfusion and energetic metabolism correlating with verbal 3. Average hospitalization duration was 16 days. After a
fluency appear to reflect the differences in activation of mean 14 months follow-up, there was no re-bleeding and
right and left brain structures involved in WFT performance permanent nerve dysfunction.
in patients with VE. Conclusions: There is no positive finding by DSA
examination in PNSAH. The clinical symptoms are mild.
All patients recovered fast with few complications and good
prognosis. There was no obvious effect for the future quality
of life.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


172 Posters, Sunday 9 September

P1191 P1192
Analysis of risk factors associated with Thrombolytic treatment of acute ischemic
bleeding of AVM patients treated with stroke. How to harmonize logistics in the
gamma knife radiosurgery (GKRS) healthcare system to meet the high-level
J.H. Yun, D.H. Kwon, B.D. Kwun professional requirements of the
Neurological Surgery, Asan Medical Center, Ulsan thrombolytic treatment protocol?
University College of Medicine, Seoul, Republic of Korea
A. Folyovich, K.A. Bres-Molnr, V. Varga, E. Horvth,
Objectives: The two purposes of our study are comparison K. Vadasdi, N. Kaszs
of annual bleeding rate before and after GKRS and analysis Neurology and Stroke Center, Szent Jnos Hospital,
of risk factors associated with bleeding before and after Budapest, Hungary
GKRS. Introduction: The efficacy of thrombolytic treatment (TT)
Materials and methods: This retrospective analysis was for acute ischemic stroke has been established. The number
performed for AVM patients treated with GKRS in single of patients eligible for thrombolysis remains low, due to the
center. Between 2001 to 2010, a total of 433 patients were strict treatment protocol. This number could be increased by
selected in this study and all charts were reviewed. We highly organized logistics of the ambulance service and
analyzed annual bleeding rate and bleeding risk factor by hospitals. Almost all medical specialties should be available
multivariate Cox proportional hazards model analysis. 24 hours a day. We investigated the timely distribution of
Results: The 299 cases of initial bleeding presentation TTs during regular working hours (between 8-16 hours) and
before GKRS represented 69% (299 of 433). Among 35 on-call duty hours in our department, providing 24-hour
bleeding AVM patients after GKRS, 15 patients showed comprehensive care for patients with stroke and acute
initial non-bleeding presentation and the remaining 20 were neurological conditions.
initial bleeding ones. In analysis on factors affecting with Patients and methods: Time and date of admissions of
bleeding before GKRS, highly significant associations were 6076 patients treated in our Stroke Center between 2009
found with single feeder, deep draining vein and and 2011 were analyzed, as well as distribution between
periventricle location. Also we could demonstrate that AVM regular working hours and on-call duty hours.
patients who had a single feeder or total obliteration were at Results: Percentage of acute admissions was 86.12%.
low risk of bleeding after GKRS (62%, 56%), but one with 51.15% of total patients and 66.66% of patients with TT
nidal aneurysm was high risk of bleeding after GKRS as who were admitted during on-call duty hours. On weekends,
(4 times). percentage of admissions was 20.19%, and that of patients
Conclusion: Annual bleeding rate of AVM was decreased with TT was 17.64%. Thrombolysis was most often
after GKRS (from 5.9% to 3.0%). AVM patients who had a performed on Thursday and Friday, and between 12 and 13
single feeder or total obliteration would have a low bleeding p.m. On weekends, the peak time for TT was between 17
risk after GKRS and on the other hand, ones with nidal and 18h. 23.53% of TTs were performed between 10 p.m.
aneurysm would have a higher bleeding risk than ones and 6 a.m.
without. Meticulous clinical and radiological follow-up Conclusions: The rate of admissions for acute stroke was
study is mandatory for detection of post-radiosurgery higher during on-call duty hours, adding to the already high
bleeding in AVM patients with these risk factors. workload during on-call duty. This type of high-level care,
requiring a tight interdisciplinary cooperation, can only be
safely provided in an institute, where a high-level standby
or on-call duty is available in all specialties.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 173

P1193 P1194
Differences in changes of Stupefaction phenomena of peripheral
interhemispheric EEG coherence in neurons in stroke patients
patients after carotid ischemic stroke and O. Schiopu1, M. Gavriliuc1, A. Grumeza1, A. Bodiu2
transient ischemic attack 1Neurology, 2Neurosurgery, Institute of Neurology and

Neurosurgery, Chisinau, Moldova


A. Solkin, M. Bialiauski, U. Kuzniatsou, K. Bialiauskaya,
K. Pashkova, R. Santhalingam, C.S. Ilapperuma Background and aims: Preconditioning of cerebral tissue
Neurology and Neurosurgery, Vitebsk State Medical is a known phenomenon. Our work group suggests a new
University, Vitebsk, Belarus term - stupefaction of neurons that means capacity to
Aim: To determine the dynamics of changes of recovery of neurologic functions after delayed in time
interhemispheric EEG coherence in patients after carotid reperfusion. The aim of study derived from observation of
ischemic stroke (IS) and transient ischemic attack (TIA). several post-stroke patients in which carotid endarterectomy
Patients and methods: 28 patients (18 patients with TIA was performed and remarkable clinical improvement was
and 10 patients with IS in the region supplied by the left noticed reduction of neurological deficits.
carotid artery) aged 46-68 were examined by coherence Patients and methods: The study comprised 32 post-stroke
analysis of the EEG on the 2nd-14th day after TIA and on patients operated for carotid artery stenosis greater than
the 60th-180th day after IS. The control group included 24 70% by carotid endarterectomy. The period between stroke
age-matched practically healthy volunteers. onset and surgery varied from 6 weeks to 1 year. Control
Results: A decrease of EEG coherence predominantly in group, 21 patients, included the same population - post-
the delta, theta and alpha frequency bands between different stroke patients with symptomatic carotid artery stenosis
cortical areas of hemispheres (frontal, central, parietal, who refused surgery. Electrophysiological exam comprising
temporal and occipital) was revealed on the 2nd-4th day neurography and needle EMG was performed in operated
after carotid TIA. Positive changes of EEG coherence in the patients by the following schedule: before surgery, 10-14
majority of frequency bands were revealed on the 10th-14th days after and 30 days after. Control group was examined
day after TIA. An opposite trend of changes of EEG twice: 14 days after stroke onset and 3-4 months later.
coherence (increase) was observed at the same time between Results: In all patients first exam of the paralyzed side
some cortical areas in the beta-2 band. A relatively persistent showed: decreased M-responses compared to the healthy
decrease of EEG coherence in all frequency bands with a side, spontaneous activity, and unstable motor units
focus on alpha, beta-1 and beta-2 bands was revealed depending on duration from stroke onset. After surgery first
between different cortical areas of hemispheres in patients exam showed increased motor responses, bigger amount of
after carotid IS during the observation period. motor units, richer interference pattern. The evolution of
Conclusion: The revealed changes of interhemispheric changes in peripheral nervous system was totally different
EEG coherence give evidence of the reversible and in the operated and the control group.
functional damage of central nervous system in patients Conclusions: Peripheral neurons in stroke patients after
after TIA and structural and relatively persistent CNS long-term carotid stenosis preserve their function in
damage in patients after ischemic stroke. hibernating state-stupefaction. Reperfusion, in our cases
carotid endarterectomy, leads to rapid improvement of
motor functions, based on clinical and electrophysiological
examinations, especially compared to control group.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


174 Posters, Sunday 9 September

P1195 P1196
Transplantation of autologous bone Stroke subtypes and risk factors
marrow stromal cells (BMSC) for ischemic associated with vascular dementia
stroke: strategy and tactics for clinical B. Ince, G. Benbir
application Neurology, Istanbul University Cerrahpasa Medical Faculty,
Istanbul, Turkey
S. Kuroda, K. Houkin
Neurosurgery, Hokkaido University Hospital, Sapporo, Vascular contributions to cognitive impairment and
Japan dementia are very important. As we know more about the
Objective: There is increasing evidence that the transplanted risk factors of vascular dementia (VaD), the prophylactic
bone marrow stromal cells (BMSC) significantly promote managements would better help us in controlling the
functional recovery after central nervous system damage in morbidity of this disease. We investigated the patients
the animal models of various kinds of CNS disorders, followed in stroke outpatient clinic between 1996 and 2011.
including cerebral infarct. However, there are several The diagnosis of VaD was made on the basis of DSM-IV
shortages of information when considering clinical criteria and mini mental test. A total of 2798 patients were
application of BMSC transplantation for patients with included in the study with a mean age of 61.613.4 years;
neurological disorders. Here, we discuss what we should and 53.9% of them were males. Of these, 90.5% were of
clarify to establish cell transplantation therapy in clinical ischemic origin and 7.1% were intracerebral haemorrhages.
situation and describe our recent works for this purpose. Among patients with ischemic stroke, the pathogenesis was
Methods and results: The BMSC have multiple abilities to atherothrombotic in 15.8%, cardioembolic in 20.1%, small
differentiate into the neural cells and to promote neuronal vessel disease in 18.2%, other causes in 5.1%, and unknown
survival and axon elongation, contributing to rebuild the in 31.6%. A total of 127 patients (4.5%) had dementia. VaD
neural circuits in the injured CNS. Using optical imaging was present in 2.2% of patients with atherothrombotic
and MRI techniques, the transplanted BMSC can non- stroke, 4.4% of patients with cardioembolic stroke, 11.8%
invasively be tracked in the living animals for at least 8 of patients with small vessel disease, and 3.5% of patients
weeks after transplantation. Clinical MR apparatus can with intracerebral haemorrhage. The frequency of VaD is
visualize the tagged BMSC in the brain. 18F-FDG PET is significantly higher in small vessel disease (p<0.001).
quite valuable to monitor the recovery of brain metabolism Hypertension was more frequent in patients with VaD
after transplantation. The BMSC can be expanded using the (81.9% vs. 69.6%, p=0.001), and the use of more than one
animal protein-free culture medium within a clinically antihypertensive drug was more common in patients with
relevant period. G-CSF is useful to enhance their small vessel disease and without VaD (p=0.008), suggesting
proliferation when the BMSC are obtained from the aged a better control for hypertension. As the studies investigated
patients. There is optimal dose and timing of BMSC the characteristics of VaD increased, we could better identify
transplantation to yield significant therapeutic benefits. the risk factors to reduce the burden of dementia, the
Conclusion: It is anurgent issue to develop a clinical incidence of which seems to increase as life expectancy
imaging technique to track the transplanted cells in the CNS advanced.
and evaluate the therapeutic significance of BMSC
transplantation to establish it as a definite therapeutic
strategy in clinical situation in very near future.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 175

P1197 P1198
Basilar artery thrombosis in an 11-year- Changes in plasma matrix
old boy treated with intravenous t-PA and metalloproteinase-9 levels in patients with
endovascular mechanical thrombectomy acute ischemic stroke
D. Kondziella1, J. Fink2, L. Sonneborg3, L.L. Larsen4, I.M. Cojocaru1, M. Cojocaru2, V. Sapira3, G. Socoliuc3,
A.P. Born5, M. Holtmannsptter6 C. Hertea3
1Department of Neurology, Copenhagen University Hospital, 1Neurology, C Davila Univ of Med and Pharm, Colentina

Rigshospitalet, Copenhagen, 2Department of Radiology, Clinical Hospital, 2Physiology, Titu Maiorescu Univ,
3Department of Paediatrics, 4Department of Neurology, Faculty of Medicine, 3Neurology, Colentina Clinical
Roskilde Hospital, Roskilde, 5Department of Paediatrics, Hospital, Bucharest, Romania
6Department of Neuroradiology, Copenhagen University
Background: Matrix metalloproteinase (MMP) have been
Hospital, Rigshospitalet, Copenhagen, Denmark thought to be involved in stroke pathogenesis. MMP-9
Basilar artery occlusion (BAO) in children is rare. BAO is contributes to tissue destruction.
associated with a high mortality and morbidity if Objective: Our aim was to analyze the MMP-9 levels in
recanalization of the basilar artery is not achieved before blood within 24 hours of acute ischemic stroke onset to
extensive brainstem damage has occurred. An 11-year-old observe the role of MMP-9 in the pathogenesis of
boy presented with a clinical and radiological top-of-the- atherothrombotic stroke.
basilar syndrome. Intravenous tissue plasminogen activator Material and methods: In this study we investigated
(t-PA) was administered and the patient immediately prospectively MMP-9 levels in serum from 106 patients (42
referred to the regional stroke center. Mechanical men and 64 women, mean age 71.526.32 years) with acute
thrombectomy using a Solitaire stent resulted in clot ischemic stroke in the middle cerebral artery area in the first
removal and recanalization of the basilar artery 4 hours after 24 hours from the onset (mean duration 7.84.5 hours) as
stroke onset. The patient made a full clinical recovery. To compared to 112 controls (48 men and 64 women, mean age
our knowledge this is the first report on BAO in a child 70.366.8 years). Exclusion criteria for patients and
treated with bridging therapy, the combination of controls were: atrial fibrillation, manifest ischemic
intravenous thrombolysis and endovascular thrombectomy. cardiopathy, infections, neoplasias, surgical interventions,
vasculitides, concurrent major renal or hepatic disorders,
major trauma in previous months. Serum samples were
collected under sterile conditions and stored in aliquots at
-700C until assay. Serum MMP-9 levels were determined by
enzyme-linked immunosorbent assay (ELISA) in blood
samples obtained on admission. Statistical analysis was
performed by Mann-Whitney and Log-Likeliwood Ratio
tests. All values reported are expressed as mean (x)SD.
Results: Mean serum MMP-9 concentrations were higher
in the group with ischemic stroke 17232.4ng/mL, range
139.6-204.4ng/mL vs. controls 579.6ng/mL, range 47.4-
66.6ng/mL (95% CI, 3.17 to 14.18; p<0.014).
Conclusion: MMP-9 activity is associated with early acute
ischemic stroke. The high levels of MMP-9 in acute
ischemic stroke document the involvement of this enzyme
in the regulation of inflammation in stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


176 Posters, Sunday 9 September

P1199 P1200
Off-label thrombolysis in a case of Role of the phosphatidylinositol 3-kinase
ischemic hemiballism: a case report and extracellular signal-regulated kinase
A. Correnti1,2, E. Puca3, A. Pieroni3, C. Colosimo3, pathways in the neuroprotective effects of
D. Toni3 cilnidipine on primary cultured cortical
1 Neurologia e Psichiatria, 2UTN-Policlinico Umberto I, neurons against hypoxia
3 Sapienza University of Rome, Italy
H.-J. Yu1, S.-H. Koh2, K.-Y. Lee2, S. Kim3, H.-H. Park2,
Introduction: Hemiballism is a rare hyperkinet movement Y. Lee2, J. Kim2
disorder. A lesion of the contralateral subthalamic nucleus, Neurology, Bundang Jesaeng Hospital, Seongnam,
1
due to a vascular etiology, is involved in more than half of Hanyang University College of Medicine, 3Department of
2
the cases. We report a case of hemiballism with an Neuroscience, KAEL-Gemvax Co., Ltd, Seoul, Republic of
extraluysian lesion who was treated off-label with Korea
intravenous alteplase.
The phosphatidylinositol 3-kinase (PI3K) and extracellular
Case report: A 74-year-old man, with hypertension and
signal-related kinase (ERK) pathways are crucial for
dyslipidemia, reported a left motor disorder on his arrival at
neuronal survival. Cilnidipine, a calcium channel blocker,
the Emergency Room. He had left-sided hemiballism
has been reported to have neuroprotective effects. We
involving the face and both limbs and a cerebral CT-scan
investigated whether cilnidipine could protect neurons from
showed only chronic signs of vascular leucoencephalopathy.
hypoxia and explored the role of the PI3K and ERK
Despite having a normal NIHSS, in accordance to the
pathways in the neuroprotection of cilnidipine. The viability
clinical signs and after exclusion of other possible causes
of primary cultured cortical neurons injured by hypoxia,
related to the development of this hemiballism (e.g. marked
measured by trypan blue staining and LDH assay, was
hypoglycaemia), he was thrombolysed with alteplase iv at
dramatically restored with the treatment of cilnidipine.
4 hours from stroke onset. After this treatment, choreiform
TUNEL and DAPI staining showed that cilnidipine
movements were less pronounced. The following day a
significantly reduced apoptotic cell death induced by
brain MRI showed an ischemic lesion of the right capsula
hypoxia. Free radical stress induced by hypoxia was
extrema and insular cortex and the integrity of both
markedly decreased by the treatment with cilnidipine.
subthalamic nuclei. Another smaller lesion was found in the
Survival signalling proteins associated with the PI3K and
right temporal-parietal cortex. The patient recovered
ERK pathways were significantly increased and death
completely in three days without receiving any further
signalling ones were markedly decreased in the primary
symptomatic therapy. Conclusion: Hemiballism has been
cultured cortical neurons simultaneously treated with
classically characterized as pathognomonic of a lesion in
cilnidipine and hypoxia when compared with the neurons
the contralateral subthalamic nucleus. However, several
treated with only hypoxia. These neuroprotective effects of
cases due to a lenticular or caudate lesion or even cortical,
cilnidipine were blocked by the treatment with a PI3K
as in the present case, have been described. Prognosis is
inhibitor, LY294002, or an ERK inhibitor, FR180204. These
benign in most cases, but it seems logical to treat these cases
results show that cilnidipine protects primary cultured
with thrombolysis when they are seen within an appropriate
cortical neurons from hypoxia by reducing free radical
time frame in the ER.
stress and death-related signalling proteins and by enhancing
survival-related proteins associated with the PI3K and ERK
pathways and that activation of those pathways plays
important roles in the neuroprotective effects of cilnidipine
against hypoxia. These findings suggest that cilnidipine
with neuroprotective effects against hypoxia through
various mechanisms as well as blood pressure-lowering
effect might help preventing ischemic stroke and reduce
neuronal injury caused by ischemic stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 177

P1201 P1202
Coenzyme Q10 protects neural stem cells Inflammatory process evaluation by
against hypoxia/reoxygenation by serum Hs CRP and sVCAM-1 in patients
enhancing survival signals with carotid stenosis
K.-Y. Lee1, S.-H. Koh1, H.-J. Yu2, J. Park3, Y. Lee1, C. Revnic1, C. Ginghina2, I.A. Popescu3, C. Popa3,
H.-T. Kim1, S. Kim1, J. Kim1 F. Revnic4
1Hanyang University College of Medicine, Seoul, 1UMF Carol Davila Bucharest, Faculty of General Medicine,
2Neurology, Bundang Jesaeng Hospital, Seongnam, 2Cardiology, C.C. Iliescu Cardiovascular Disease Institute,
3Neurology, Inje University Paik Hospital, Pusan, Republic 3Neurology, Vlad Voiculescu Neurology Institute,

of Korea 4Gerontology & Geriatrics, Ana Aslan National Institute of

Recanalization and secondary prevention are the main Gerontology and Geriatrics, Bucharest, Romania
therapeutic strategies for acute ischemic stroke. Objectives: Inflammation is a part of the process of
Neuroprotective therapies have also been investigated atherosclerosis; evaluation of serum Hs-CRP together with
despite unsuccessful clinical results. Coenzyme Q10 sVCAM-1 seems to be an important predictor for stroke and
(CoQ10), which is an essential cofactor for electron cardiovascular events in patients with atherosclerosis. The
transport in mitochondria, is known to have an antioxidant levels of circulating inflammatory markers may express the
effect. We investigated the protective effects of CoQ10 extent of the inflammatory response, their participation in
against hypoxia/reoxygenation in neural stem cells (NSCs). plaque progression and rupture needs to be evaluated.
We measured cell viability and levels of intracellular Materials and methods: The study evaluated 86 patients
signalling proteins after treatment with several (62 men) (40 controls without carotid atherosclerosis, 22
concentrations of CoQ10 under hypoxia-reperfusion. patients with asymptomatic carotid stenosis and 24 patients
CoQ10 protected NSCs against hypoxia-reperfusion in a with symptomatic carotid stenosis) mean age 61.35.8
concentration-dependent manner by reducing growth years; range 42 to 86 years. Serum Hs-CRP and sVCAM-1
inhibition and inhibiting free radical formation. It increased levels were determined in all patients using a Dade Behring
the expression of a number of survival-related proteins such hsCRP assay and Quantikine Human sVCAM-1
as phosphorylated Akt (pAkt), phosphorylated glycogen Immunoassay by ELISA method.
synthase kinase 3- (pGSK3-), and B-cell lymphoma-2 Results: The mean values of Hs-CRP and sVCAM-1 in
(Bcl-2) in NSCs injured by hypoxia-reperfusion and reduced control patients were 1.38mg/ml0.63 and 636ng/dl
the expression of death-related proteins such as cleaved 0.11(p=0.025); 1.780.84mg/ml and 672ng/dl0.14 in
caspase-3. We conclude that CoQ10 has effects against patients with asymptomatic carotid stenosis (p=0.014);
hypoxia-reperfusion induced damage to NSCs by enhancing 1.820.92mg/ml and 693ng/dl 0.46 in patients with
survival signals and decreasing death signals. symptomatic carotid stenosis (p=0.001). There is an
association between the higher levels of hsCRP and
sVCAM-1 with low echogenicity of carotid plaques,
suggesting a link between inflammation and potential risk
of plaque rupture.
Conclusions: The results of our study showed that highest
levels of hs-CRP and sVCAM-1 were associated with
morphological and clinical progression of the carotid
atherosclerotic disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


178 Posters, Sunday 9 September

P1203 P1204
Intravenous thrombolysis in stroke Intra-cerebral haemorrhage in patients
patients with atrial fibrillation warfarinized for non-valvular atrial
V. Padjen1, M. Jarakovic2, P. Stanarcevic1, fibrillation
M. StefanovicBudimkic1, D. Jovanovic1, O. Savic1, M.K. Fong, Y.P. Chu, B. Sheng, K.K. Lau
I. Berisavac1, M. Ercegovac1, L. BeslacBumbasirevic1 Princess Margaret Hospital, Kowloon, Hong Kong S.A.R.
1Emergency Neurology Department, Neurology Clinic
Aim: To study the characteristics of intra-cerebral
Clinical Center of Serbia, School of Medicine, University of
haemorrhage (ICH) in warfarinized non-valvular atrial
Belgrade, 2Cardiology, Institute of Cardiovasular Diseases
fibrillation (wNVAF) patients.
of Vojvodina, Sremska Kamenica, Serbia
Methods: Retrospective review on wNVAF patients who
Background: The purpose of our study was to compare the had developed ICH and were treated at Princess Margaret
severity and the outcome of acute ischemic stroke among Hospital, Hong Kong from 2006-2011.
patients with atrial fibrillation (AF) who received Results: 87 warfarinized patients developed ICH during the
intravenous thrombolysis (IVT) compared to those who did study period, accounted for 5.34% of all ICH. Among them,
not receive this therapy. 37 (24 male and 13 female) were wNVAF patients. Their
Methods: We performed a retrospective analysis of data mean age was 74.8. The baseline risk for thromboembolism
prospectively collected of stroke patients with AF that were and bleeding, as represented by their median CHA2DS2-
treated in the Stroke unit of the Emergency neurology VASc and HAS-BLED score, were 4 and 2, respectively. All
department. Neurological deficit was evaluated using the patients had moderate to high bleeding risk, the lowest
NIHSS on admission while the 90-day clinical outcome was HAS-BLED score was 1. 15 patients (40.5%) had both
assessed using the modified Rankin scale (mRS). scores in the high risk category (CHA2DS2-VASc2 &
Results: There were 91 stroke patients who had AF, 47 HAS-BLED3). The volume of ICH in these 37 patients
(51.6%) were men IVT was given to 22 patients (24.2%). was 19.223.0cm3. Their initial INR was 2.50.7 (range
Comparing the two groups there was no significant 1.1-5.0), which was corrected to 1.60.5 (range 1.1-3.1) at
difference between the baseline NIHSS which was 12hrs. There was no correlation between ICH volume and
12.9+2.86 in AF patients who received IVT,and 16.2+3.11 INR, and a majority (72.9%) developed ICH at INR 2 to 3.
in AF patients who did not receive IVT (p=0.053). Patients 3 patients required neurosurgical intervention. The mortality
with AF and IVT were more likely to have excellent was 48.6%, which was not statistically different from the
functional outcome (mRS 0-1) (63.6% vs. 21.7%; OR 0.16 overall mortality of ICH. The ICH score showed good
[95% CI 0.06-0.45]; p=0.000), and favourable functional correlation with mortality (c=0.75, p=0.001). All patients
outcome (mRS 0-2) (68.2% vs. 28.9%; OR 0.19 [95% CI with ICH score 3 died. 8 patients had good neurological
0.07-0.54]; p=0.001) than patients with AF who did not recovery at 6 months with modified Rankin scale score 3.
receive IVT. Also, patients with AF and IVT were less likely Conclusion: Most of our ICH-wNVAF patients had
to have a fatal outcome as outcome at month 3 (13.6% in appropriate risk/benefit balance. The clinical course of ICH
patients with AF and IVT vs. 49.3% in patients with AF was similar to other ICH patients, and INR did not correlate
without IVT; OR 6.15 [95% CI 1.67-22.7]; p=0.005). with ICH volume or clinical outcome.
Conclusion: Our study has shown that stroke patients with
AF who received IVT had significantly better outcome than
stroke patients with AF who did not receive this therapy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 179

P1205 P1207
25-Hydroxyvitamin D associated with Post-partum stroke: a multifactorial
acute ischemic stroke and subtypes: complication of pregnancy
a case control study M.G. Kortushvili, I.V. Verulashvili
J.R. Chaudhuri1, V.S. Bandaru1, R. Ramapriya1, Tbilisi State Medical University, Tbilisi, Georgia
A. Anamika2, B. Balaraju1 Objectives: The aim of this study was to analyse the
1Neurology, 2Biochemistry, Yashoda Hospital, Hyderabad, influence of pregnancy on the risk of post-partum stroke
India and possibilities of prevention recurrent cerebral events in
Background: Vitamin D deficiency is common in all age future.
groups and may contribute to cardiovascular diseases. Methods: Issued from a multicenter (3 centres) prospective
Recent reports have incriminated low 25-hydroxyvitmain D study, 21 women (mean age 26.9 years) with post-partum
levels causing ischemic stroke. stroke (PS), were screened from over 1200 patients of acute
Aim: To investigate association of 25-Hydroxyvitamin D in cerebral stroke between January 2005 and September 2009.
acute ischemic stroke. PS was diagnosed clinically and by CT/MRI/angiography.
Methods: We recruited 85 consecutive stroke patients and The selected patients were followed up for 2 years:
85 age and sex matched control subjects attending the information on death, neurological status, recurrent cerebral
department of neurology for non-vascular diseases at events were obtained from direct observation, written
Yashoda hospital Hyderabad South India, from October questionnaire or by telephone interviews (every 6 months).
2011 to February 2012. All patients under went evalution Results: Causes of post-partum stroke included cerebral
for stroke subtypes and risk factors. We measured infarction (7 arterial, 8 venous) and 6 intracerebral
25-Hydroxyvitamin D by Chemiluminiscen test, serum haemorrhage with the poorest outcome (1 maternal death
calcium, phosphorus and alkaline phosphatase for case and and 3 residual neurologic deficits). The median time at
control subjects. onset of stroke was 6 days post- partum (range, 2-25 days).
Results: Out of 85 patients there were 63 men (74.1%), During a mean follow-up of 2 years, 2 arterial recurrent
mean age 60.4 range 26-89 years. 25-Hydroxyvitamin D ischemic strokes occurred (both within the second year and
deficiency was observed in 44 (51.7%) of stroke patients outside pregnancy); there were no cases of recurrent
and 30 (35.2%) in control subjects (p=0.01). Our data cerebral venous thrombosis.
showed hypertension in 48 (56.4%) with stroke patients and Conclusions: Post-partum stroke is a multifactorial and
28 (32.9%) in controls (p=0.003), diabetics in 32 (37.6%) non-preventable complication of pregnancy. The post-
in stroke patients and 18 (21.7%) in controls (p=0.02). partum period, not the pregnancy itself, is associated with
25-Hydroxyvitamin D deficiency was found 100% (4/4) in an increased risk of recurrent stroke. A previous cerebral
extracranial large artery atherosclerosis, 61.9% (13/21) in stroke is not a contraindication to a subsequent pregnancy.
intracranial large artery atherosclerosis, 50% (6/12) in Secondary prevention after PS is based on similar principles,
cardioembolic stroke, 52.9% (9/17) in small artery diseases, as in general population: an optimal management of
27.2% (3/11) in stroke of other determined etiology and vascular risk factors and appropriate antithrombotic therapy
45% (9/20) in stroke of un-determined etiology. After (at least during the first year after post-partum stroke).
adjustment, multiple logistic regression analysis showed
hypertension (Odds ratio: 3.1 95%CI 1.6-6.2), low
25-Hydroxyvitamin D (Odds ratio:3.0 95%CI 1.5-5.8) and
diabetics (Odds ratio:2.6 95%CI 2.1-4.8)
Conclusions: Low levels of 25 Hydroxyvitamin D is
significantly associated with acute ischemic stroke and it
may be an independent risk predictor for stroke.

P1206
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


180 Posters, Sunday 9 September

P1208 P1209
Intravenous thrombolytic treatment in Foetal middle cerebral and arteries of
stroke patients with recent transient umbilical blood flow velocity in normal
ischemic attack: a Serbian experience and gestational diabetic pregnancies
with thrombolysis in ischemic stroke - S.-M. Fereshtehnejad1, M. ShabaniZanjani2,
SETIS register R. Nasirzadeh2, S.-A.P. Alemzadeh3, S. Askari3
1Neurobiology, Care Sciences & Society (NVS), Karolinska
M.S. Budimkic1, D.R. Jovanovic2,
Institute, Stockholm, Sweden, 2Tehran University of Medical
L. Beslac-Bumbasirevic2, V. Padjen3, P. Stanarcevic3,
Sciences, 3Student Scientific Research Committee, Tehran
SETIS group
1Department of Emergency Neurology, Neurology Clinic,
University of Medical Sciences, Tehran, Iran
Clinical Center of Serbia, Faculty of Medicine, University of Background: Gestational diabetes mellitus (GDM) is one
Belgrade, 2Department of Emergency Neurology, Faculty of of the most common complications in pregnancies.
Medicine, University of Belgrade, 3Department of Evaluating other conditions including intra uterine growth
Emergency Neurology, Neurology Clinic, Clinical Center of restriction and pre-eclampsia, some studies have shown
Serbia, Belgrade, Serbia significant changes in blood flow velocity of foetal middle
Introduction: Patients with prior transient ischemic attack cerebral artery (MCA). Our study is one of the first which
(TIA) may have diffusion-weighted lesions on MRI imaging has aimed to assess the effects of GDM on Doppler
despite normal CT brain findings. This raises the concern of parameters of the foetal MCA and umbilical artery (UI) and
higher risk for post-thrombolytic intracerebral haemorrhage to compare with normal pregnancies.
in stroke patients with recent TIA. The aim of this study was Methods: This cross-sectional study was performed in 66
to evaluate efficacy and safety of intravenous thrombolysis pregnant women, including 33 women with GDM and the
(IVT) in stroke patients with recent TIA. others without it, in Akbar-Abadi University Hospital in
Methods: We analysed 651 stroke patients treated with IVT, Tehran, Iran during 2010-2011. Peak systolic and diastolic
from the SETIS register. Data were collected about recent velocities, pulsatility index (PI), resistance index (RI) and
ipsilateral TIA, clinical characteristics of patients, systolic/diastolic ratio (S/D) were recorded in UA as well as
occurrence of symptomatic ICH (defined with SITS MOST both right and left foetal MCAs for every recruited pregnant
criteria) and 3 month outcome (using mRS). women by means of Doppler ultrasonography.
Results: From analysed stroke patients 34 of them (5.2%) Results: The mean gestational age at the time of examination
had one or more previous TIA episodes within a month was 34.45 (SD=2.62) weeks in GDM-positive group.
prior to the current stroke. Most of the patients had TIA Although all of the measured Doppler parameters had
within the first week before stroke (67%) and a majority of higher values in GDM-positives, but the differences were
those had TIA on the day of the current stroke. There was not significant between the two groups of study; except for
no sex and age difference among TIA and non-TIA group the left foetal MCA-PI which was significantly higher in
of patients (male 67.6% vs. 64.1%; mean age 57.31.6 vs. GDM associated group [2.07 (SD=0.07) vs. 1.85 (SD=0.74),
59.60.5). Patients with prior TIA presented with less severe P=0.03].
stroke (mean NIHSS score 9.77.4 vs. 13.45.7), had more Conclusion: Our results show that gestational diabetes
frequent hyperlipidemia (84.4% vs. 55.4%, p=0.002) and could also correlate with the increased PI of the foetal
occurrence of lacunar stroke (26.4% vs. 11.6%, p=0.01). MCA. This result could be due to the brain-sparing
Occurrence of sICH was equally present in both phenomenon; and as it was demonstrated that
groups-2.9% (OR 0.99, CI 0.13-7.67). The good recovery (3 hyperglycaemia affects the diabetic adult vessels, it might
month mRS 0-2) gained 76.6% of patients in TIA group and also involve foetal vessels in GDM associated pregnancies.
64.6% of patients in non-TIA group (OR 1.7, CI 0.75-4.26).
Conclusion: Thrombolytic treatment in stroke patients with
recent TIA is safe.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 181

P1210 P1211
The benefit of early Holter-ECG Evaluation of total serum MMP-9 and
monitoring for the detection of their inhibitors TIMP-1 as a markers of
paroxysmal atrial fibrillation in patients carotid plaque instability in elderly
with acute ischemic stroke: a pilot study patients with carotid stenosis
M. Kral1, D. Sanak1, M. Hutyra2, T. Veverka1, C.R.S. Revnic1, G.I. Prada2, C. Pena3, F. Revnic3,3,
A. Bartkova1, R. Herzig1, D. Skoloudik1, T. Dornak1, B. Paltineanu4
M. Taborsky2, P. Kanovsky1 1Cardiology, UMF Carol Davila Bucharest/Faculty of

Comprehensive Stroke Center, Department of Neurology,


1 General Medicine, 2Geriatrics, UMF Carol Davila,
3Biology of Aging, 4NIGG Ana Aslan, Bucharest, Romania
Department of Internal Medicine I - Cardiology, Faculty of
2

Medicine and Dentistry, Palack University and University Introduction: Matrix metalloproteinases (MMPs) and their
Hospital, Olomouc, Czech Republic inhibitors (TIMPs) regulate extracellular matrix (ECM)
Background: Atrial fibrillation (AF) is the most common remodelling, a key feature of atherosclerosis, and their
cause of cardioemboligenic ischemic stroke (IS). Patients circulating concentrations can be assayed..
with cardioemboligenic IS and AF often present with more Aim: To relate circulating biomarkers of extracellular
severe neurological deficit associated with the occlusion of matrix (ECM) turnover to site-specific measures of carotid
the large cerebral artery, worse clinical outcome and higher artery atherosclerosis on Echo Doppler ultrasound.
mortality. Especially the paroxysmal form of AF is very Material and methods: 110 patients admitted to the
risky, because it is usually not being registered during a Cerebrovascular Clinic (85 males) aged:44-89 years,
routine electrocardiogram (ECG) examination. The aim was divided into three groups (44 patients with ischaemic stroke
to assess the benefit of early 24-hour Holter-ECG related to carotid stenosis, 36 patients with asymptomatic
monitoring in patients with ischemic stroke and a negative carotid stenosis and 30 controls) were included in the study.
history of AF. All patients underwent carotid Echo-Doppler examination
Methods: In the prospective study, the set consisted of 114 and serum levels of total MMP-9 and TIMP-1 were
consecutive IS patients (57 males, average age 75.49.8 evaluated using Quantikine Human MMP-9 (total) and
years) with a negative history of AF and without the TIMP-1 ELISA R&D Systems Kits with a ChemWell 2019,
presence of AF on the admission ECG exam. A standard Awarness,Inc. USA Autoanalyser.
24-hour Holter-ECG was performed in all patients. Results: Serum MMP-9 and TIMP-1 levels were associated
Results: 33 (28.9%) patients had concurrent ischemic heart with risk of all-cause mortality (Cox proportional hazard
disease, 14 (12.3%) had a positive history of myocardial ratio [HR] per standard deviation 1.10, 95% confidence
infarction, and 25 (22%) a history of previous IS. Holter- interval [CI] 1.03-1.18; and 1.11, 1.02-1.18; respectively).
ECG was performed on average 4.12.5 days after the IS TIMP-1 levels were mainly related to risks of cardiovascular
onset. Newly detected AF was found in 10 (8.8%) patients mortality and stroke (HR per standard deviation 1.20, 85%
(paroxysmal form of AF in 90.0% out of these patients). No CI 1.09-1.35; and 1.16, Carotid Echo-Doppler study showed
acute ischemic changes were recorded on admission ECG that in the stroke group most of plaques found were
in all patients. echolucent with irregular surface compared with the
Conclusion: Holter-ECG monitoring is a non-invasive and asymptomatic group where echogenic plaques with smooth
inexpensive method used for the early detection of AF, surface are found frequently (p=0.04).
particularly of its paroxysmal form. The risk of recurrent Conclusions: In this community-based cohort of elderly
stroke can be significantly reduced in IS patients with men, serum MMP-9 and TIMP-1 levels were related to
diagnosed AF using the anticoagulant therapy. mortality risk. An altered extracellular matrix metabolism
Acknowledgement: Supported by the IGA MH CR grant may be involved in several detrimental pathways, and
number NT/11046-6/2010. circulating MMP-9 or TIMP-1 levels may be relevant
markers thereof.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


182 Posters, Sunday 9 September

P1212 P1213
Post-stroke depression: predictive factors Correlation between white matter lesions
at six months follow-up and neurological complication after
J.M. Ramrez-Moreno1, M.J. Gmez-Baquero1, cardiac surgery
F. LpezEspuela2, J.C. PortillaCuenca2, A. OlleroOrtiz1, B. Zllami1, E. Basha2, B. Cekrezi3, J. Kruja4
T. GavilnIglesias2, J.D. PedreraZamorano3, 1Department of Neurology, American Hospital, 2Department
P.E. JimenezCaballero2, I. Casado-Naranjo2 of Neurology, Central Military Hospital of Tirana,
1Neurology, Stroke Unit, Hospital Universitario Infanta 3Department of Radiology, American Hospital, 4Department

Cristina, Badajoz, 2Neurology, Stroke Unit, Hospital San of Neurology, UHC Mother Theresa, Tirana, Albania
Pedro de Alcntara, 3Nursing, Universidad de Extremadura, Background and purpose: With the advances of surgical
Cceres, Spain procedure, cardiac surgery can now be performed on older
Introduction: Post-stroke depression affects approximately patients with multiple chronic diseases. The white matter
one third of ischemic stroke survivors. In validation studies, lesions are hyperintensities that are located in the deep
the Hamilton Rating Scale for Depression (HRSD) proved white matter, and are often seen with small vessel disease.
to be an acceptable screening instrument. To identify We looked for the correlation between the pre-surgery white
variables that could predict depression after stroke using matter lesion (MRI- T2, Flair) and the adverse neurological
this score. outcome following the surgery.
Methods: A total of 200 consecutive patients with acute Method and evaluation: We have seen thirty (n 30) patients
stroke, admitted to the Stroke Unit, were included in the undergoing cardiac surgery (mainly valve repair and
study. After six months follow-up, 173 survivors [71 CABG- coronary artery bypass graft surgery). Preoperative
(35.5%) females and 129 (64.5%) males; average age (SD): and intraoperative variables include patient age,
71.16 (12.3) years] were evaluated, with the HRSD. comorbidities are evaluated. They are also evaluated with
Univariate and multivariate logistic regression analyses neurological and cognitive examination (MMSE). Also
were conducted to determine predictive factors. Age, head MRI is performed before the surgery and after surgery
gender, socioeconomic class, education, residence, social only in patients with new neurological deficits.
support, Charlson index, previous depression, type and side Results: 12 (40%) of them were presented with multiple
of stroke, National Institutes of Health Stroke Scale score periventricular, subcortical white matter lesions, 18 (60%)
(NIHSS) at admission, Hemiparesis, Barthel index (BI) and without cerebral lesions. After the surgery 2 patients 6.6%
modified-Rankin scale (mRS) at discharge, and length of with prior white matter lesion had a stroke; 6 of them 20%
stay were evaluated and included in the models. had prolonged encephalopathy (3-5 day coma after surgery).
Results: When a cut-off point of 13 in the HDRS was used 1 ischemic stroke (3.3%) happened in the group of patients
as the criterion, depression was rated in 42% of patients. without white matter lesions patients (1-2 weeks after the
Univariate analysis showed that post-stroke depression was surgery).
significantly associated with: previous depression, type and Conclusion: White matter hyper intensities are more
side of stroke, stroke severity assessed by the NIHSs, common and extensive in patients with cardiovascular risk
hemiparesis, BI and mRS at discharge, and length of stay. A factors and symptomatic cerebrovascular disease. White
multivariate regression model demonstrated that the most matter lesions can be predictive for neurological
significant factors associated with post-stroke depression complication after cardiac surgery. The small number of our
were previous depression, side of stroke, mRS at discharge, cases does not allow us to have a statistically significant
and length of stay. conclusion.
Conclusions: Post-stroke depression measured by the
HRSD is highly prevalent in the follow-up, and is associated
with previous depression, length of stay and with the degree
of disability at discharge.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 183

P1214 P1215
Evaluation of ESSEN and SPI-II scale for Contribution of antiplatelet resistance to
short-term prognosis of minor stroke and vascular pathology in patients undergoing
TIA neuroendovascular procedures
J. Liu, J. Liu S. Vucetic1, N. Gligic2, J. Savic3
The 3rd Hospital of Hebei Medical University, Shijiazhuang, Special Hospital for Cerebrovascular Diseases St. Sava,
1

China Neurology, 3Special Hospital for Treatment of


2

Background and purpose: The evaluation of stroke risk Cerebrovascular Diseases Sveti Sava, Belgrade, Serbia
may improve the outcome of stroke, especially for minor Aims: Antiplatelet resistance is not sufficiently investigated
stroke. We investigated the validity of ESSEN and SPI-II among patients undergoing neuroendovascular procedures.
scale for predicting the short-term risk of minor stroke the Its contribution to vascular pathology is also not well
clinic. understood. The purpose of this study was to determine
Methods: 167 patients with minor stroke or transient contribution of antiplatelet resistance to different
ischemic attack (TIA) were enrolled in this study. The cerebrovascular pathoanatomical features.
recurrent ischemic events were recorded within 90 days Material and method: In this retrospective study, we
after onset and the curve of accumulative survival rate was analyzed data of 65 consecutive patients who underwent
obtained to observe the recurrent risk. The prognostic value neuroendovascular procedure in a one year period and
of ESSEN and SPI-II scale for recurrent risk was evaluated previously received antiplatelet therapy. We used impedance
by calculating the area under receiver operating aggregometry method to determine level of platelet
characteristic curve respectively. inhibition.
Results: The total recurrent rate of ischemic events within Results: Indications for neurointerventional procedures
90 days was 12.57% while the recurrent rate was 7.78% in were carotid stenosis and vertebrobasilar stenosis and
the first week after onset. Steep change of the cumulative symptomatic intracranial aneurysm. Among 56 patients
survival rate was observed in the acute phase of minor receiving aspirin at a median dose 700mg per week, 10.7%
stroke. The area under receiver operating characteristic were resistant. Among 24 patients receiving clopidogrel in
curve of ESSEN scale was 0.677 (95%CI: 0.557~0.797) a median dose 525mg per week, 21 of them were on a dual
compared to 0.553 (95%CI: 0.413~0.694) of SPI-II scale. antiplatelet therapy, and 60.4% of all were resistant to
Conclusions: As screening tool, both ESSEN and SPI-II clopidogrel. We found carotid and vertebrobasilar stenosis
scale could evaluate the short-term risk for recurrent events and aneurysms to be significantly more often in aspirin non-
of minor stroke and TIA. The ESSEN scale was more responders than in responders. However, clopidogrel
suitable for predicting the short-term risk at the clinic. response affected neither of these vascular features.
Conclusion: These results show contribution of aspirin
response to pathomorphology of cerebral vessels. This
emphasizes a need for detailed assessment of underlying
mechanisms of vascular pathology in this group of patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


184 Posters, Sunday 9 September

P1216 P1217
Validation of transcranial duplex Frequency of post-stroke shoulder pain
sonography as screening test for the D. Dobi1,2, M. Kapisyzi2, S. Grabova2, L. Stefanidhi2,
detection of right-to-left shunt F. Myslimi2, S. Koci2, E. Ranxha2, J. Kruja2
1Dept. of Neurology, UHC, 2UHC Mother Theresa, Tirana,
P. Martinez-Sanchez, E. Correas-Callero, M. Lara-Lara,
Albania
G. Ruiz-Ares, B. Fuentes, M. Martinez-Martinez,
L. Cullar-Gamboa, E. Dez-Tejedor Background and purpose: Shoulder pain is known to
Neurology, La Paz University Hospital. IdiPAZ Health retard rehabilitation after stroke. Its causes and prognosis
Research Institute, Madrid, Spain are uncertain. This study describes the frequency of post-
Objective: To evaluate the diagnostic accuracy of contrast- stroke shoulder pain prospectively, in an unselected stroke
enhanced transcranial colour-coded sonography (c-TCCS) population in the first 3 months after stroke.
against contrast-enhanced transcranial Doppler monitoring Methods: 92 patients entered the study. 88 of them, mean
(c-TCD) for the detection of right-to-left shunt (RLS). age 70.62.4 years , were examined at 2 weeks, 4, and 3
Methods: Prospective study of ischemic stroke patients months after stroke. The data about shoulder pain, Barthel
studied at our neurosonology laboratory for the presence of index, anxiety and depression score were recorded. Full
RLS (March 2011-January 2012). All patients underwent neurological examination was undertaken, using the
c-TCD by contrast injection (9cc of saline solution + 1cc of contralateral side as a control. Pain outcome and stroke
air), both at rest and after Valsalva manoeuvre (VM). After outcome was recorded at subsequent visits. Non-steroid
15 minutes of rest, patients underwent a c-TCCS by a anti-inflammatory drugs and physical treatment is used in
neurosonographer blinded to the results of the c-TCD, who all patients, in order to treat the pain.
used the same methodology as in c-TCD. The extent of CDI Results: 35 (40%) patients developed shoulder pain on the
was measured according to international criteria: absent (no same side of their stroke. There was a strong association
HITS), small (<10 HITS), medium (>10 HITS shower between pain and abnormal shoulder joint examination, ipsi
pattern) and large (>10 HITS, curtain pattern). lateral sensory abnormalities and arm weakness. Shoulder
Results: 50 patients were enrolled, 23 men. Mean age: 43 y. pain had resolved or improved at 3 months in 70 (80%) of
At rest, c-TCD showed 7 RLS and 6 were detected by the patients with non-steroid anti-inflammatory drugs and
c-TCCS. After VM, c-TCD showed 23 RLS and 21 were physiotherapy.
detected by c-TCCS. After VM c-TCCS showed, compared Conclusions: Shoulder pain after stroke occurred in 40% of
with c-TCD: sensitivity 91.3%, specificity 92.6%, positive 88 patients surviving, consenting to participate. 80% of
predictive value (PPV) 91.3%, negative predictive value patients made a good recovery with pharmacological and
(NPV) 92.3% and accuracy 92%. Taking into account only physical treatment. Patients with sensory and/or motor
medium-large RLS, c-TCCS showed, compared to c-TCD: deficits represent at risk sub-groups. Non significant
sensitivity 100%, specificity 100%, PPV 100%, NPV 100% correlation was observed among male and female patients
and accuracy 100%. The concordance with echocardiography in Barthel Index.
was similar in c-TCD and c-TCCS.
Conclusion: The accuracy of c-TCCS as screening test for
the detection of RLS, compared with c-TCD, is high and is
greater in case of medium or large RLS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 185

P1218 P1219
Intravascular perfusion of carbon black Effect of anodal versus cathodal
ink allows reliable visualization of cerebral transcranial direct current stimulation on
vessels in mice stroke recovery: a pilot randomized
M.R. Hasan, J. Herz, D.M. Hermann, T.R. Doeppner controlled trial
Department of Neurology, University of Duisburg-Essen E. Khedr1, O. Shawky1, A. Tohamy1, E. Darwish1,
Medical School, Essen, Germany D. El Hamady2
Introduction: Study of the rodent cerebrovascular anatomy 1Assiut University Hospital, 2Physical Medicine and
provides significant insight in the experimental stroke Rehabilitation, Assiut University Hospital, Assiut, Egypt
research. For several years, intravascular perfusion of Purpose: To compare the long-term effect of anodal vs.
colored latex has been considered as a standard tool to cathodal transcranial direct current stimulation (tDCS) on
visualize cerebral vessels. However, this technique has motor recovery of subacute stroke.
certain technical limitations, which compromise its Methods: 36 patients with subacute ischemic stroke
reproducibility. Here, we described a simple method to participated in the study. The patients were randomly
visualize the cerebral vessels in mice in a reproducible assigned to one of three groups; the anodal, cathodal and
manner. sham groups. They received l tDCS at an intensity of 2mA
Methods: Diluted/undiluted latex mixed with carbon black for 25 minutes daily for 6 consecutive days. Patients were
ink was injected through the left cardiac ventricle. assessed with NIHSS, Barthel index (BI) and Medical
Alternatively, a mixture of two commercially available Research Council (MRC) muscle strength scale at baseline,
carbon black inks, i.e. CB1 and CB2 at a 1:9 ratio was after end of sessions, and then 1, 2 and 3 months later.
injected. The minimal diameter of the stained vessels was Cortical excitability was measured at baseline and after the
quantified in each method in cryosections of brains. Finally, 6th sessions.
CB1+CB2 staining was done in combination with Results: By the end of the follow-up, all groups had
triphenyltetrazolium chloride (TTC) staining in animals improved on all scales with P-values ranging from 0.01 to
subjected to transient focal cerebral ischemia. 0.0001. Improvement was equal in the anodal and cathodal
Results: Perfusion with CB1+CB2 yielded adequate filling groups with no significant differences between them in any
of the cerebral vessels with high contrast visualization. This of the rating scales. When these treatment groups were
technique was applied to identify the anastomotic points combined and compared with Sham, significant time X
between cerebral vascular territories of mice with different group interaction terms were seen for BI (p=0.002) and
genetic backgrounds and strains. Furthermore, perfusion NIHSS (p=0.046). Interactions were also seen in MRC
with combined carbon black inks allowed visualization of scores for all muscles. There was increased cortical
significantly smaller vessel diameters at a higher vessel excitability of the unaffected hemisphere after the end of the
density in comparison to perfusion with diluted/undiluted last session in all groups with the changes being more
latex. We finally gave evidence that this method for cerebral significant in the real than sham groups.
vessel staining could be combined with TTC staining - a Conclusion: Combined rehabilitation training with anodal
widely used tool to observe and analyze infarct volumes in or tDCS may improve mobility in the upper and lower
rodents. extremities in subacute ischemic stroke.
Conclusion: Thus, perfusion with combined carbon black
inks offers a simple, cost-effective and reproducible
technique in order to visualize cerebral vasculature in mice.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


186 Posters, Sunday 9 September

P1220 P1221
Predicting survival in haemorrhagic New score ABN for TIA: prediction of
stroke: the prognostic value of routine short-and long-term risk of stroke
markers O.Y. Fartushna
A.M.H. Young1, C.K. Weerasuriya1, A.J. Procter1, Bogomolets National Medical University, Kiev, Ukraine
S. Singh1, A. Rinnert1, N. Wang1, U. Rai2 Transient ischemic attacks (TIA) are strong predictors of
1Cambridge Medical School, University of Cambridge, subsequent stroke, disability and death. Thats why,
Cambridge, 2Department of Stroke Medicine, Queen improved methods to predict risk of stroke after TIA are
Elizabeth Hospital, Kings Lynn, UK very important.
Introduction: Serum markers of inflammation are Aim was to create a neurological prognostic score for stroke
becoming recognised as prognostic factors in ischaemic risk after TIA using the clinical and neuroimaging criteria
stroke. However, this relationship in haemorrhagic stroke is and methodology of mathematical evaluation of the
less well defined. In this study, we examined admission probability of a set of independent events, and combinatorics.
markers of inflammation and applied a logistic regression Methods: The clinical, Doppler ultrasound, MRI,
model to predict outcome in haemorrhagic stroke. transthoracic echocardiography diagnosing has been carried
Methods: A retrospective study was performed between out in 178 patients with TIA. Cases were reviewed by two
September 2009 and 2011 at a general admission stroke unit neurologists to establish the correlation with the diagnosis.
in the UK. Over this time, approximately 1400 patients were Results: During the 2 years observation period stroke
admitted with acute stroke; of these, 114 cases were developed in 34 (41.5%) TIA patients. To determine the
haemorrhagic in nature. Venous blood samples were stroke risk after TIA we have created a new score ABN
obtained on admission. Pre- and post-morbid (i.e. at (Age, Blood pressure, Neuroimaging), which is an improved
discharge or death) modified Rankin scores were also version of the group of ABCD scales to predict short- and
recorded. Patients were controlled for age, sex, smoking long-term risk of stroke in patients with TIA. The ABN
status, hypertension status and co-morbidities using the score is calculated by summing up points for 3 independent
Charles co-morbidity index (CCI). Logistic regression factors associated and optimized to predict the risk of stroke
models were generated using SPSS. within 2, 7, 30, 90 days after a TIA, but also to predict
Results: Of the cohort examined 58 were male and 55 stroke risk within 1 and 2 years with a sufficiently high
female. The median [quartiles] glucose values of patients diagnostic accuracy, p<0.05. Total scores ranged from
differed significantly (p=0.009) between those who survived 0 (lowest risk) to 7 (highest risk).
(n=59) at 6.5mmol/L [5.8, 8.1] compared to those who did Conclusions: Comparative analysis of scales ABCD2 and
not (n=34) at 8.9mmol/L [6.1, 10.1]. Leukocytes ABN in the first 90 days after TIA showed a higher
demonstrated a similar trend with a median of 9.32x109/L sensitivity and specificity of the ABN (Se=0.83; Sp=0.69)
[8.0,12.0] in the survival group compared to 13.4x109/L scale compared with the scale ABCD2(Se=0.77; Sp=0.58)
[9.7, 17.4] those deceased (p=0.001). Using these data, our in 90 days and in the 2 years after TIA (ABN: Se=0.85,
logistic regression model predicted survival correctly on Sp=0.75; ABCD2: Se=0.79, Sp=0.62).
83.9% of occasions. Separating groups by sex improved this
to 96% in females. P1222
Conclusions: These results suggest that a logistic regression
model using plasma glucose, leukocytes, clinical outcomes Abstract cancelled
and sex might be a useful tool for prognosis in haemorrhagic
stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 187

P1223 P1224
A case of Susacs syndrome The influence of carotid endarterectomy
T. Kasikci, S. Bek, E. Tokgoz, S. Demirkaya, Z. Odabasi on cognitive and affective functions in
Gulhane Medical Faculty, Ankara, Turkey patients with atherosclerosis of carotid
Background: Susacs syndrome is a rare angiopathy with arteries
the triad of encephalopathy, retinal artery occlusion and N.N. Spirin1, N.N. Malyshev2, I.V. Malysheva2,
acute hearing loss. It causes retinal, cochlear and cerebral O.A. Fadeeva1
infarcts usually in young females. 1 Neurology, Yaroslavl State Medical Academy, Yaroslavl,
Case: A 33-year-old male patient after headache for one 2 Neurology, Regional Hospital #1, Vologda, Russia
week described attention deficiency, dysmnesia and acute
right hearing loss. In neurological examination his Aim: To investigate the dynamics of neuropsychological
cooperation and orientation were inadequte. He was not status in patients after carotid endarterectomy (CEA).
able to hear with his right ear. His gait was ataxic, deep Methods: 52 patients were examined, 39 (75%) male and
tendon reflexes were increased. Right Babinski sign was 13 (25%) female, mean age 627.5 (M). In anamnesis 21
positive. His electroencephalography showed generalised (40.4%) patients had TIA, 14 (26.9%) had stroke, 17
delta activity. In brain magnetic resonance imagings T2 and (32.7%) did not have TIA or stroke. For the assessment Mini
FLAIR hyperintense, T1 iso/hypointense lesions especially Mental State Examination (MMSE), Hospital Anxiety and
in corpus callosum were detected. Right sensorineural Depression Scale (HADS), the test for remembering of 10
hearing loss was confirmed by audiogram. Cerebrospinal words and Shultz tables were used.
fluid analysis was normal except mild protein increase. Results: The second assessment was performed 12.50.4
Recanalized vascular occlusions were shown in peripheral months after CEA. The positive dynamics of cognitive
retina by fundus fluorescein angiography (FFA). As a result status was stated based on MMSE (from 28.52.0 to
of laboratory findings the patient was diagnosed as Susacs 29.20.9, =0.037) and the test for remembering of 10
syndrome and treatment was initiated with acetylsalicylic words (for immediate reproduction from 5.80.6 to 6.20.6
acid, metylprednisolon and intravenous immunoglobulin for (=0.003), for postponed reproduction from 4.31.5 to
five days and continued with oral corticosteroid. Within the 5.11.4, =0.007). Also the concentration of attention
following days encephalopathy had recovered but no improved based on Shultz tables (time for the first table
improvement was provided in hearing loss. from 58.82 to 562 sec (=0.015), for the last table from
Conclusion: Despite the clinical and radiological similarity 862 to 77.62 sec (p=0.04)). Based on HADS assessment
with multiple sclerosis Susacs syndrome is an uncommon were decreased the level of anxiety from 9.31.8 to 8.51.5
microangiopathy resulting involvement of vessels. (=0.014) and of depression from 7.12.1 to 6.381.41
Etiopathogenesis of the syndrome could not be clearly (=0.031)).
defined but occlusions caused by the immune response Conclusion: Revascularization of carotid artery positively
against precapillary endothelium may be responsible for the influences cognitive and affective functions in patients with
pathogenesis. atherosclerosis of carotid arteries.

P1225
Abstract Cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


188 Posters, Sunday 9 September

P1226 P1232
Haemorrheology of clinically silent Mortality in stroke patients
multifocal vascular cerebral lesions I.V. Pershyna
P. Kowal1, I. Siemieniak2, A. Marcinkowska-Gapinska3 Kharkiv Medical Academy of Postgraduate Education,
1Medical University, Strus Hospital, Rheological
2 3 Kharkiv, Ukraine
Laboratory, Poznan, Poland
P1233
P1227 Development and validation of the Asian
Intravenous and intra-arterial stroke disability scale (ASDS)
thrombolysis for acute ischemic stroke: K. Ghandehari1, K. Ghandehari2, G. Saffarian-Toosi3,
the first experience in Cyprus S. Masoudinezhad3, S. Yazdani3, A. Nooraddin3,
B. Kaymakamzade1, G. Akansoy1, S. Akpinar2, A. Eker3, S. Ebrahimzadeh3, F. Ahmadi3, F. Abrishamchi4
Neurology, 2Neuroscience Research Center, 3Mashhad
1
I. Ipekdal3, L. Erguven1, S. VudalDogruyol2,
University of Medical Sciences, Mashhad, 4Isfahan
M. Ozmenoglu3, T. Akalin1
1Neurology, 2Radiology, Dr. Burhan Nalbantoglu State University of Medical Sciences, Isfahan, Iran
Hospital, 3Neurology, Near East University, Faculty of
Medicine, Nicosia, Cyprus P1234
The rate of disability after cerebrovascular
P1228 disease
Opalski syndrome developing after R. Aliyev
radiotherapy: a case report Neurology Department, Azerbaijan State Advanced Training
Institute for Doctors named after A.Aliyev, Baku, Azerbaijan
S.G. zmen, B. Kocer, . Kenan, H.Z. Batur
Department of Neurology, Gazi University School of
Medicine, Ankara, Turkey P1235
Is malnutrition a risk factor of stroke?
P1229 M. Habib, A.N. Rizvi, M.S.I. Bhuiyan, M. Bhuiyan,
Three-year consequences of ischemic A. Haque
stroke in patients with arterial Neurology, Bangabandhu Sheikh Mujib Medical University,
Dhaka, Bangladesh
hypertension and C-reactive protein
content
O. Markulan1, T. Cherenko2 P1236
1 National Medical Academy of Post-graduate Education, Influence of Cerebrolysin on white blood
2 National Medical University, Kiev, Ukraine cell and red blood cell aggregation,
deformability and morpho-densitometry
P1230 parameters of erythrocytes in patients
Cerebral amyloid angiopathy presenting with cerebrovascular disease
as recurrent intracerebral haemorrhage A.V. Anisimova, K.V. Anisimov, T.I. Kolesnikova
Neurology, Russian State Medical University, Moscow,
Z. Ozozen Ayas, D. Kotan, G. Unal, Y. Guzey Aras
Sakarya University Department of Neurology, Sakarya, Russia
Turkey
P1237
P1231 Exertional periodic breathing augments
Mortality in patients with asymptomatic anaemia-related cerebral hemodynamic
carotid atherosclerosis dysfunction in patients with heart failure
I. Kolos, O. Syvakova, S. Boytsov, AMTEC Study Group J.-S. Wang
Research Center for Preventive Medicine, Moscow, Russia Chang Gung University, Tao-Yuan, Taiwan R.O.C.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 189

P1238 P1243
The difference in the biochemical indices Cerebral blood flow and head-up tilt in
of blood plasma in patients with the earlier period of acute stroke
symptomatic and asymptomatic course of L.N. Anisimova1, I.A. Voznyuk1, D.V. Polyakov2,
different degree atherosclerosis of the A.V. Polyakova3
internal carotid arteries
1Medical Military Academy, 2I. I. Mecnikov North West State
University, 3I. P. Pavlov State Medical University, St.
M.L. Pospelova1, O.D. Barnaulov2 Petersburg, Russia
1Neurology, I. P. Pavlov State Medical University,
2Pharmacology, The Bektereva Human Braun Institute, RAS,
Saint Petersburg, Russia P1244
Comparison of clinical-neuroimaging
P1239 diagnoses and cognitive profile diagnoses
Diagnostic value of Transcranial Doppler of mild cognitive impairment subtypes:
and computer tomographic angiography preliminary experience in a VAS-COG
in acute basilar artery ischemia clinic
N.G. Dadamyants1,2, A.I. Ikramov2 R. Valenti, E. Salvadori, P. Anna, F. Pescini, L. Ciolli,
Radiology, Republic Research Center of Emergency
1 S. Nannucci, D. Inzitari, L. Pantoni
Medicine, Khorezm, 2Tashkent Institute of Postgraduate Department of Neurological and Psychiatric Sciences,
Medical Education, Tashkent, Uzbekistan University of Florence, Italy

P1240 P1245
Territorial division and clinical Cerebellar haemorrhage - clinical course
characteristics of posterior circulation and prognosis
cerebral infarcts (PCCI) A.P. Bezmarevic
I. Stakena1, G. Enina1, I. Kikule1, A. Platkajis2 Special Hospital for Cerebrovascular Diseases St. Sava,
1Department of Neurology and Neurosurgery, 2Department of Belgrade, Serbia
Radiology, Riga Eastern University Hospital Gailezers,
Riga, Latvia P1246
Cardiovascular risk factors control in
P1241 patients after ischemic stroke in Western
Three-year catamnesis of survivability and Ukraine
functional outcomes in stroke patients N. Sydor1, O. Shulga1, A. Yagensky1, L. Shulga2
I. Pershyna 1 Volyn Region Hospital, 2Lutsk City Hospital, Lutsk, Ukraine
Neurology and Neurosurgery, Kharkiv Medical Academy of
Postgraduate Education, Kharkiv, Ukraine
P1247
Characteristics of stroke mimics in
P1242 intravenous thrombolysis
The implication of immediate neurological D. Leupold, M. Galovic, G. Kgi, B. Weder
improvement in acute ischemic stroke Department of Neurology, Cantonal Hospital St. Gallen,
D. Kim St. Gallen, Switzerland
Neurology, Songdohyoja Geriatric Hospital, Incheon,
Republic of Korea

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


190 Posters, Sunday 9 September

P1248 P1254
Characteristics of transient ischemic Melatonin prevents the cerebral ischemic
attacks preceding ischemic stroke brain injury-induced decrease in
O. Stojiljkovic, M. Zarkov, M. Jerkovic parvalbumin expression
Department of Neurology, Clinical Center of Vojvodina, Novi P.-O. Koh1, J.-H. Sung2, F.-A. Shah2, E.-H. Cho1,
Sad, Serbia M.-O. Kim2
1Department of Anatomy, College of Veterinary Medicine,

P1249 Institute of Agriculture and Life Science, 2Division of Life


Science and Applied Life Science, Gyeongsang National
Echocardiogram in young ischaemic University, Jinju, Republic of Korea
stroke patients: TTE versus TOE
A. Murphy1,2, J. Kinsella2, C. McGuigan2 P1255
School of Medicine and Medical Science, University
1

College Dublin, 2Department of Neurology, St Vincents Cognitive decline in patients with stroke
University Hospital, Dublin, Ireland A.K. Thacker1, N. Chaturvedi2, A.M. Verma2, M. Mishra2
1Neurology, 2Medicine, BRD Medical College, Gorakhpur,

India
P1250
Cerebral vein thrombosis and fluid
P1256
deprivation of the post-partum
N. Slimani1, R. Ghazali2, I. Eltibani2, M. Izem3,
Thrombolysis during menstruation
A. Ait-Slimane4, D. Hakem1, A. Berrah1 A. Iljicsov1, C. Ertsey1, I. Sipos1, E. Cspny1, M. Sim1,
Internal Medicine, Dr Mohammad-Lamine Debaghine,
1 S. Ilniczky1, G. Vrallyay2, I. Szl3, D. Bereczki1
University Hospital, Algiers, 2EPH Djelfa, Djelfa, 3Internal 1Neurology Department, 2Szentgothai MRI Research Center,
Medicine Emergency, 4Emergency, Dr Mohammad-Lamine Semmelweis University, 3National Institute of Rehabilitation,
Debaghine, Universitarys Hospital, Algiers, Algeria Budapest, Hungary

P1251 P1257
Depression after stroke Outcome of acute basilar artery
A. Jaoua1, S. Benamor2, S. Benammou2 occlusions followed at the intensive care
1 Neurology, 2CHU Sahloul, Sousse, Tunisia unit
N. Oztekin1, M.F. Oztekin2
P1252
1Neurology, MOH Ankara Numune Education and Research

Hospital, 2Neurology, MOH Ankara Yildirim Beyazit


CNS vasculitis associated with HCV Education and Research Hospital, Ankara, Turkey
infection
A. Castro Caldas, L. Neto, P. Canho, T. Pinho e Melo, P1258
R. Geraldes
Hospital Santa Maria, Lisbon, Portugal A retrospective analysis of cerebral vein
and dural sinus thrombosis (CVT) cases
hospitalized at our neurology service over
P1253
a two-year period
Abstract cancelled M.I. Gutirrez-Cid, B. Venegas-Prez, G. Torres-Gaona,
C. Feliz-Feliz, M.A. Aranda-Calleja, S. Bellido-Cullar
Neurology, Fundacin Jimnez Daz, Madrid, Spain

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 191

P1259 P1264
Post-stroke dementia syndrome Date of birth and gender as risk factors
D. Nao1, J. Kruja,2, S. Gjei,2, D. Dobi2 for ischemic stroke of middle cerebral
1Specialistic Polyclinic 2, 2Neurology, UHC Mother Theresa, artery vasculature
Tirana, Albania
A. Kutera, E. Szczepocka, K. Jastrzebski
Medical University of Lodz, Lodz, Poland
P1260
Mortality and morbidity rate in P1265
hemorrhagic stroke patients with different Management of acute ischaemic stroke in
risk factors tertiary care hospital in UAE: an audit
V. Davoudi, K. Keyhanian, M. Saadatnia, F. Haghdoost report
Neurology Department, Isfahan University of Medical
M.C. Thakre1, J.S. Inshasi2,3
Sciences, Isfahan, Iran 1Neurology, Canadian Specialist Hospital, 2Neurology,

Rashid Hospital, Dubai Health Authority, 3Medicine, Dubai


P1261 Womens Medical College, Dubai, United Arab Emirates

The assessment of carotid wall thickness


as a predictive value of risk of stroke in P1266
hypertensive diabetes and non-diabetes Verification of oversight in the
patients cancellation tests and the subscale of the
C.R.S. Revnic1, B. Paltineanu2, G.I. Prada3, F.G. Revnic4 behavioural inattention test (BIT) in
1Cardiology, UMF Carol Davila, 2Geriatrics, NIGG Ana
patients with right hemisphere stroke and
Aslan, 3Geriatrics, UMF Carol Davila Bucharest/Faculty of normal subjects
General Medicine, 4Cell and Molecular Biology of Aging,
NIGG Ana Aslan, Bucharest, Romania M. Kawaguchi1, M. Yamada1, Y. Nakamura1, K. Kawase1,
Y. Satoh1, A. Satoh2, M. Tsujihata2
Rehabilitation, 2Neurology, Nagasaki Kita Hospital,
1

P1262 Nagasaki, Japan


Cerebral venous thrombosis presenting
with subarachnoid haemorrhage: shady of P1267
3 cases study Dynamics of the quality of life in the
L. Raji, R. Ibn Moufti, H. Elotmani, M.A. Rafai, post-stroke patients restoration period
B. Elmoutawakil, I. Slassi
M. Romanova
CHU IBN Rochd, Casablanca, Morocco
Department of Neurology, M.F.Vladimirsky Moscow
Regional Clinical and Research Institute, Moscow, Russia
P1263
Correlation between high sensitive P1268
C-reactive protein (hsCRP) and infarction Predictors of stroke recurrence or cardiac
volume and brain CT-scan findings in death in non-rheumatic atrial fibrillation
patients with ischemic stroke C. Gratii1, A. Grosu1, N. Diaconu1, G. Pavlic2
P. Petramfar1, A. Shariat2, S. Mosallaei2, 1Institute of Cardiology, 2Institute of Neurology and

M. SharifianDorche2 Neurosurgery, Chisinau, Moldova


1Shiraz University, 2Shiraz University of Medical Sciences,

Shiraz, Iran

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


192 Posters, Sunday 9 September

P1269 P1275
The impact of pulmonary infections on Long-term methylphenidate treatment in
the course and outcome of stroke a young woman with ADHD and agenesia
S.T. Ristic1, D.T. Ristic2, I. Marinkovic3 of left carotid and vertebral artery without
Department of Neurology, University Clinical Centre Nis,
1
cerebrovascular complication
Institute of Pulmonary Diseases, 3Medical Faculty of Nis,
2
A. Lavillaureix1, F. Pico2
Nis, Serbia 1Neurology Department, Versailles Hospital, Versailles
Saint-Quentin-en-Yvelines University, Paris Descartes
P1270 University, Paris, 2Neurology Department, Versailles
Hospital, Versailles Saint-Quentin-en-Yvelines University,
Stroke outcomes depending on the terms Versailles, France
of hospitalization and the severity of
patientts condition P1276
V. Yavorskaya1, I. Pershyna1, A. Grebenyuk1, O. Bondar1,
A. Scoryj2, S. Kryvchun2 Abstract cancelled
1Neurology and Neurosurgery, Kharkiv Medical Academy of
Postgraduate Education, 2Kharkiv City Clinical Hospital P1277
No7, Kharkiv, Ukraine
Neurophysiological evaluation of cortical
inhibitory activity in acute stroke:
P1271 correlation with long-term functional
Sneddons syndrome observed in internal recovery
medicine practice F. Ranieri1, M. Dileone1, P. Profice1, F. Pilato1,
D. Hakem, A. Berrah F. Capone1, L. Florio1, E. Pravat2, F. Iodice1,
Internal Medicine, Bab-El-Oued Hospitaal Universitary C. Colosimo2, V. Di Lazzaro1
1Institute of Neurology, 2Institute of Radiology, Universit
Centre, Algiers, Algeria
Cattolica del Sacro Cuore, Roma, Italy

P1272
Abstract cancelled
P1278
A comprehensive functional assessment
P1273 of cerebral blood flow in ischemic brain
L. Bokeria1, I. Aslanidi1, T. Serguladze1, N. Darvish1,
Intra-arterial thrombolytic therapy with
L. Pyshkina2, M. Bekuzarova2, A. Kabanov2
streptokinase. Experience of application 1Cardiovascular Surgery, Bakoulev Scientific Center for

in Uzbekistan Cardiovascular Surgery of the Russian Academy of Medical


S. Mubarakov1, A. Zakhidov1, N. Dadamyants2 Sciences, 2Department of Neurology and Neurosurgery,
1Neurology Department, Republican Research Center of Russian State Medical University, Moscow, Russia
Emergency Medicine, 2Functional Diagnosis Department,
Republican Research Center of Emergency Medicine,
Khorezm, Tashkent, Uzbekistan
P1279
DSA analysis and etiological exploring of
P1274 stroke in 86 young adults
L. Yin
Functional outcome and quality of life in Department of Neurology, 2nd Affiliated Hospital of Dalian
patients with aneurismal subarachnoid Medical University, Dalian, China
haemorrhage
I. Gabrielyan, H. Manvelyan
Department of Neurology, Yerevan State Medical University,
Yerevan, Armenia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 193

P1280 P1285
Macaroni sign, Takayasu arteritis and A novel hypothalamic peptide proline-rich
stroke peptide-1 as an agent for stroke
S. Calado1,2, G. Matias1, C. Reizinho3, C. Jordo4, treatment
M. Viana-Baptista1,2 E. Yeritsyan1, A. Galoyan2, M. Balasanyan3
1Neurology Department, Centro Hospitalar Lisboa Ocidental 1Pharmacy, Yerevan State Medical University after Mkhitar
(CHLO) - Egas Moniz Hospital, 2CEDOC, Medical Sciences Heratsi, 2H. Buniatian Institute of Biochemistry of the
Faculty, New University of Lisbon, 3Neurosurgery National Academy of Sciences, 3Pharmacology, Yerevan
Department, 4Neuroradiology Department, Centro State Medical University after Mkhitar Heratsi, Yerevan,
Hospitalar Lisboa Ocidental (CHLO) - Egas Moniz Armenia
Hospital, Lisbon, Portugal

P1286
P1281
Clinical analysis of 1003 cases of
Biomarker-based test for acute stroke transient ischemic attack
diagnosis and stroke types identification W. Ju1, Y. Yang2, J. Wu2
G. Mehtiyeva, V. Yagubov, R. Shiraliyeva 1 Neurology, 2Jilin University, Changchun, China
Neurology Department, Azerbaijan State Institute of
Advanced Training for Doctors, Baku, Azerbaijan

P1287
P1282
Cerebral venous sinus thrombosis:
Tentorial dural arteriovenous fistula association with primary varicella zoster
presenting as recurrent attacks of infection in the absence of Purpura
periodic paralysis fulminans
D. Singh, A. Garg, A. Bansal, G. Goel, A. Gupta
S.A. Siddiqi1, S. Nishat2, D. Kanwar3, F. Ali3,
Neurosciences, Medanta The Medicity, Gurgaon, India
M. Azeemuddin4, M. Wasay3
1Neurology, 2Aga Khan University Hospital, 3Medicine,
P1283 4Radiology, Aga Khan University Hospital, Karachi,
Pakistan
Treatment of cerebrovascular disorders
by new hypothalamic proline-rich
PRPTIDE-1
E. Yeritsyan1, A. Galoyan2, M. Balasanyan3
1Pharmacy, Yerevan State Medical University after Mkhitar
Heratsi, 2Buniatian Institute of Biochemistry, The National
Academy of Sciences, 3Pharmacology, Yerevan State Medical
University after Mkhitar Heratsi, Yerevan, Armenia

P1284
Association of the
methylenetetrahydrofolate reductase
gene polymorphism with carotid
distensibility and intima-media thickness
in patients with cerebral infarctions
N.D. Dimitrov1, A. Savov2, E.V. Todorova-Dimitrova3,
I. Velcheva4
1Department of Neurology, Second City Hospital Sofia,
2National Genetic Laboratory, Medical University Sofia,
3Clinic of Neurology, Military Medical Academy Sofia,
4Department of Neurology, Medical University Sofia,

Bulgaria

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


194 Posters, Sunday 9 September

Child and developmental neurology P1289


Oxygen toxicity is reduced by
P1288 acetylcholinesterase inhibition in the
Primary cilium, NPC1 and Patched1 developing rat brain
proteins in Niemann-Pick type C disease M. Sifringer1, C. von Haefen1, S. Endesfelder2, A. Kalb1,
C. Battisti1, M. De Santi2, P. Formichi1, B. Rossi1, I. Bendix3, U. Felderhoff-Mueser3, C. Bhrer2,
E. Radi1, S. Tripodi2, E. Tarquini2, A. Federico1 C.D. Spies1
1 University of Siena, 2AOU Senese, Siena, Italy 1Anaesthesiology and Intensive Care Medicine,
2Neonatology, Charit - Universittsmedizin Berlin,
We assessed the presence of NPC1 and Patched1 proteins 3Paediatrics I - Neonatology, University Hospital Essen,
and evaluated the relative distribution and morphology of
Germany
primary cilium in fibroblasts from Niemann-Pick type C
(NPC) patients and controls. NPC1 disease is a neurovisceral Objectives: The cholinergic anti-inflammatory pathway is
storage disorder, characterized by an abnormal intracellular a neural mechanism that suppresses the innate inflammatory
accumulation of unesterified cholesterol and glycolipids. response and controls inflammation by acetylcholine as the
Two distinct disease-causing genes have been isolated, key endogenous mediator. In this study, we investigated the
NPC1 and NPC2, involved in the processing and utilization effects of the cholinergic agonists, physostigmine and
of endocytosed cholesterol. The NPC1 protein functions in donepezil, on neurodegeneration, oxidative stress and
the sorting and recycling of cholesterol and inflammation during oxygen toxicity in the developing rat
glycosphingolipids in the late endosomal/lysosomal system. brain. The aim of this study was to investigate the level of
This protein exhibits extensive homology with Ptc1 cell death, glutathione, lipid peroxidation and the expression
receptor, a transmembrane protein localized in the primary of cytokines after hyperoxia and treatment with the
cilium and involved in the hedgehog signalling pathway. We acetylcholinesterase (AChE) inhibitors, physostigmine and
evaluated morphological aspects, relative distribution and donepezil in the brain of neonatal rats.
length of primary cilia in fibroblasts from five patients Methods: Six-day-old Wistar rats were exposed to 80%
affected by NPC1 disease, using anti-acetylated--tubulin oxygen in the presence or absence of physostigmine or
and anti--tubulin antibodies. Moreover we evaluated the donepezil and were sacrificed at defined time points (6, 12,
cytoplasmic expression of the NPC1 protein and Ptc1 24 h) following treatment. Sex-matched littermates were
receptor by fluorescence microscopy in fibroblasts from the kept in room air, and hyperoxia-exposed pups injected with
same patients. Immunofluorescence analysis showed a normal saline serving as controls. Brains were examined
significantly decreased immunostain for both NPC1 and histologically by Fluoro-Jade B staining or were processed
Ptc1 in NPC1 patients. Moreover, the NPC1 patient for molecular studies (real-time PCR, Western blotting,
fibroblasts showed a lower percentage of primary cilia spectrophotometric analysis, HPLC) to measure IL-1 and
distribution and a significant reduction in median cilia TNF-, malondialdehyde and glutathione.
length compared to controls. Our results confirm the Results and conclusions: Treatment with physostigmine
hypothesis that Ptc1 and NPC1 have a similar aminoacidic and donepezil significantly reduced hyperoxia-induced
sequence, and that they might also present a functional neurodegeneration and upregulation of the pro-
convergence. Moreover, we have, for the first time, inflammatory cytokines IL-1 and TNF- in the brains of
demonstrated altered ciliogenesis in fibroblasts from NPC1 infant rats. In parallel, both AChE inhibitors reduced
patients, suggesting an important role for the primary cilium hyperoxia-induced oxidized glutathione and
in the pathogenesis of the disease. malondialdehyde levels, whereas reduced glutathione was
upregulated through physostigmine and donepezil treatment
in the developing rat brain.
These findings are highly relevant from a clinical aspect
because oxygen administration to neonates is often
inevitable, and AChE inhibitors may serve as an adjunctive
neuroprotective therapy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 195

P1290 P1291
The distribution and changes in immune Brachial plexus ultrasound in obstetric
inhibitory molecule CD200 and its brachial plexopathy
receptor in the developing C57BL/6 mice S. Pillen1, A. Verrips1, J. Meulstee1, B. Semmekrot1,
brain following hypoxia/ischemia N. van Alfen2
Canisius Wilhelmina Ziekenhuis, 2RU Nijmegen Medical
1
K. Shrivastava, M. Chertoff, G. Llovera, M. Recasens,
Centre, Nijmegen, The Netherlands
L. Acarin
Dept of Cell Biol. Physiol. & Immunology, Inst. of Background: Obstetric brachial plexus injury (OBPI) is the
Neuroscience, Universitat Autonoma Barcelona, Bellaterra, most common peripheral nerve injury in children. In case of
Spain severe nerve damage surgical intervention is indicated.
Introduction: CD200-CD200R system has been widely However, it is difficult to decide which child needs surgery
shown to be involved in inducing immune tolerance and in because both reversible and irreversible nerve damage (i.e.
contributing to immune privileged status of the CNS. neurotmesis and/or root avulsion) are initially clinically
However, the developing brain exhibits distinct identical. Ultrasound can visualize peripheral nerves with
morphological as well as physiological characteristics excellent resolution. As ultrasound is a painless bedside
determining a peculiar response to injury showing an technique, it can be used in even very young children.
aggravated susceptibility to excitotoxicity and pro- Ultrasound of traumatic brachial plexus injury in adults
inflammatory cytokines, along with an exacerbated showed that root avulsion could be predicted correctly in
inflammatory response. Hence, the aim of this study was to 75%. Our study aimed to 1) assess if the neonatal brachial
characterize the expression pattern of CD200-CD200R in plexus can be reliably visualized with ultrasound, 2)
the developing mice brain following Hypoxic/Ischemic establish reference values and 3) use them to predict root
(H/I) injury by immunofluorescence. lesions in OBPI patients. Here we present the results of a
Methods: Wild-type C57/BL6 mice post-natal day 7 were pilot measurement and the first results in healthy neonates.
used for H/I injury using Vannucci model modified for Methods: 20 healthy neonates with a gestational age of at
neonatal mice (8% O2, 55 min) and samples were collected least 32 weeks and a negative family history for neuropathy
3h, 12h, 24h, 48h, 72h & 7 days after hypoxia. were included during the first week of life. Ultrasound
Results: In control neonatal brains, CD200 was expressed images were made using a Philips IU22 ultrasound device
on neurons, blood vessels and some astrocytes, while with a linear 7-15MHz hockey stick probe. They included
CD200R was expressed on pial and perivascular the C4 to T1 nerve roots and interscalene and supraclavicular
macrophages. After H/I, CD200 immunolabelling was brachial plexus. Aspects of the nerve and surrounding
increased in the hippocampal fissure until 7 days post- tissue, continuity and cross-sectional area were evaluated.
lesion. CD200R+ cells were a subpopulation of Iba1+ Results and conclusion: Our pilot studies showed that the
reactive microglia/macrophages, showing ameboid/ cervical roots and brachial plexus can be visualized in
pseudopodic morphologies, and located in the damaged neonates. Further results are presented at the meeting.
hippocampus and white matter tracts, persisting until 7 days
post-lesion. Characterization of CD200R+ cells by triple
immunofluorescence showed that most CD200R+ microglia
expressed the mannose receptor CD206, characteristic of
alternatively activated phenotype M2. In addition, some
CD200R+ microglial cells showed markers of antigen
presentation such as MHCII and CD86, and in specific
survival times also expression of scavenger receptor 1
(CD204).
Conclusion: CD200-CD200R molecules are involved in
microglial response following neonatal H/I injury.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


196 Posters, Sunday 9 September

P1292 P1294
Effects of maternal infection on anxiety Idiopathic optic neuritis revealing an
and depression-like behaviours of exceptional presentation of Parry
offspring Romberg syndrome in a Tunisian family
J. Solati, G.H. Moll, R.R. Dawirs, Y. Golub A. Riahi1,2, I. Bedoui1, I. Hmida1, H. Khaled1,
Dept. of Child and Adolescent Mental Health, University of M. Mansour1, J. Zaouali1, R. Mrissa1
Erlangen-Nrnberg, Erlangen, Germany 1 Department of Neurology, Military Hospital of Tunis,
Introduction: LPS and some cytokines can activate the
2 Facult de Mdecine de Tunis, Tunisia
hypothalamic-pituitary-adrenal (HPA) axis and affect brain Introduction: Parry-Romberg syndrome (PRS) or
development in pregnant mice. Maternal infection during progressive hemifacial atrophy is a rare neurocutaneous
pregnancy is a risk factor for several psychiatric illnesses syndrome of unknown etiology characterized by slowly
with neurodevelopmental origin. In this study we have progressive atrophy of a hemiface, spreading occasionally
evaluated the effects of exposure of pregnant mice to the to one side of the body. Extracutaneous manifestations such
bacterial lipopolysaccharide (LPS) on anxiety and as neurologic and ocular are common.
depression-related behaviour of male offspring. Case report: Patient A is a 19-year-old patient born to
Methods: Pregnant NMRI mice were treated with intra- consanguineous parents, presented with acute blurred
peritoneal administration of LPS (120, 240 and 480g/kg) vision. Examination showed left hemifacial atrophy with
at the 10th gestational day. Induction of the pro- limb length asymmetry. Visual acuity was impaired in both
inflammatory cytokines, TNF-, IL-1 and IL-6, was eyes. Cerebral MRI displayed hypotrophy with some non
measured in maternal serum 1.5h following maternal LPS unequivocal white matter lesions in the right cerebral
challenge. hemisphere. VEP indicated bilateral severe optic neuritis.
Results: Consequently we investigated the serum Further interrogation revealed similar cases in one maternal
concentration of corticosterone in both pregnant mice and uncle (Patient B, 32-year-old, hypotrophy of left side of the
their offspring at the age of 9 weeks. Anxiety-related body) and grandmother (Patient C, 69-year-old, severe right
behaviour of the male offspring (at postnatal day 70) was side hemiatrophy).
studied using elevated plus maze (EPM) test; and forced Discussion and conclusion: Usually, PRS appears to occur
swimming test was applied for evaluating the depression- randomly in sporadic cases and for unknown reasons.
like behaviour. Familiar cases of PRS are exceptional. All three affected
Conclusion: Our results demonstrate that LPS patients in our observation had extra facial extension of the
administration induces a significant increase in TNF-, atrophy, which is uncommon, too. Ocular manifestations
IL-1, IL-6 and corticosterone levels in maternal serum. associated with PRS are frequent, Patient A's optic neuritis
However, in offspring, prenatal LPS administration has no was partially responsive to high doses of corticoids.
significant effects on serum cytokines, corticosterone levels The particularities of this observation highlight the
and depression-like behaviour, while decreasing the anxiety heterogeneities of the PRS spectrum.
levels.

P1293
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 197

P1295 P1296
Biopsy-proven isolated neurosarcoid in a Clinical profile and follow-up of paediatric
7-year-old: presentation, management neurocysticercosis cases: a study from
and follow-up Eastern India
G. Anand, N. Wilkinson, M.A. McShane S. Bhattacharjee1,2, P. Biswas3, T. Mondal4, D.S.P. Saha5
Oxford University, Oxford, UK 1General Medicine, Peerless Hospital, 2Toxicology,

Isolated paediatric neurosarcoid is rare and there is little R.G. Kar Hospital, 3Senior Resident, Pediatrics,
information on its management and outcome. We report on R.G. Kar Hospital Kolkata, , Community Medicine, R.G. Kar
a 7-year-old with biopsy- proven neurosarcoidosis. Hospital, 5Consultant Neurologist,
Case history: A 7-year-old male presented with acute N.R. S. Hospital, Kolkata, India
weakness and continuous twitching of his right leg. Over Introduction: Neurocysticercosis is the most common
the next 48 hours he developed progressive weakness parasitic central nervous infection worldwide. Our present
affecting all 4 limbs. An EEG showed Right Epilepsia study attempts to evaluate the clinical profiles, treatment
Partialis Continua and T2-weighted brain MRI showed outcome and 2 years of mean follow-up results of 38
multifocal areas of high signal changes. CSF showed mild paediatric neurocysticercosis patients of an Eastern Indian
pleocytosis. Our preliminary diagnosis was Acute hospital.
Disseminating Encephalomyelitis and he was treated with Methods: Diagnosis was mainly based on clinical features,
steroids, immunoglobulin and anticonvulsants. Though he computed tomography (CT) / Magnetic resonance imaging
initially showed some improvement, he deteriorated with (MRI) scan and exclusion of other causes. Symptomatic,
further seizures that led to a brain biopsy. This showed active cyst patients were treated with albendazole for 28
multinucleate giant cells positive for CD68 KP1 and CD68 days, steroids and anticonvulsants.
PGMI but negative for factor 13a, S100 and CD1a. Results: Mean age of presentation was 8.473.19 yrs.
Immunostaining showed CD20 and CD79a positive small Patients presented with generalized seizures in 55.3%, focal
B- and CD3 positive small T-lymphocytes, none of which seizures, in 31.6%, headache vomiting in 63.2%, focal
expressed TDT and proliferation index was low. The neurodeficit in 10.5% and combination of symptoms in
features were in keeping with sarcoid. He was treated with 60.5% cases. Contrast CT brain showed solitary lesion in 27
intravenous cyclophosphamide followed by oral (71.1%) and multiple in the rest. Among the solitary lesions,
Azathioprine Serial brain scans showing consistent parietal lobe was involved in 74.1%, frontal lobe in 14.8%.
improvement and on 18 month follow-up, he could At presentation, lesions were transitional (ring enhancing)
independently walk and had made a good recovery apart in 58.2%, inactive in 20%, mixed in 14.6% and active in the
from some residual weakness of his right foot. His disease rest. After 2 years of mean follow-up, seizure persisted in 9,
stayed isolated to his brain. headache in 8 cases with permanent neurodeficit in none.
Conclusion: Neurosarcoid though rare does occur in 44.7% lesions calcified, 31.6% disappeared, 10.5%
children and it is important to get a tissue diagnosis as it can regressed and the rest persisted.
mimic neoplasms. Early intensive immunotherapy can be Conclusion: Solitary ring enhancing lesions (transitional
very beneficial and hence the urgency in diagnosis. stage) involving the parietal lobe was the commonest CT
picture at presentation. Generalized seizure was the
commonest presentation. Calcification of the lesion was the
commonest mode of healing followed by the disappearance
of the lesions. No particular risk factor was found to be
prognostically significant (p value>0.05).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


198 Posters, Sunday 9 September

P1297 P1298
Incoercible hiccups and vomiting as a Incidence of kidney stones related to
presentation of NMO in paediatric age topiramate treatment in paediatric
S. Machado1, J.P. Vieira2, C. Conceio3, R.L. Silva2, patients
P.R. Sousa2, M. Ramos4, G. Queiroz5, L. Ventura5, A.A.H. Mahmoud
E. Calado2 NNI, KFMC, Riyadh, Saudi Arabia
1Hospital Fernando Fonseca, Amadora, 2Pediatric
Aim: We ran this study to assess the incidence of
Neurology, 3Neurorradiology Department, Hospital
nephrolithiasis in a group of children on topiramate (TPM)
Peditrico Dona Estefania, CHLC, Lisboa, 4Pediatric
therapy for at least 1 year.
Rheumatology, Hospital Peditrico Dona Estefania, CHLC,
Method: In this retrospective observational surveillance
Lisbon, 5Pediatric Intensive Care Unit, Hospital Peditrico
Dona Estefania, CHLC, Lisboa, Portugal
study, we reviewed the medical charts of children on TPM
for at least one year seen at a paediatric neurology
Introduction: Neuromyelitis optica (NMO) affects department during the period from 2005 to 2010 at King
preferentially the optic nerve and spinal cord and its serum Fahad Medical City. Children with a normal baseline
marker, the antibody against AQP4, has completely ultrasound report were included. Follow-up ultrasound
revolutionized the diagnosis. AQP4 is heavily expressed in reports after at least one year were collected. However,
area postrema, which explains clinical presentations such as patients with any evidence of chronic illness or medications
incoercible vomiting. Nonetheless, it is also expressed in that may affect the kidney functions in addition to those that
other organs such as kidneys and stomach, and the reason are not compliant to the prescribed dose were excluded.
why they are spared is not understood. Family history of renal stones, symptoms suggestive of
Clinical Report: A previous healthy 14yo children urologic disorders, and co-morbidities were recorded.
presented to the A&E due to a subacute onset of vomiting Results: Medical charts of 96 children on TPM with mean
and hiccups. A couple of days later she experienced age of 6.9 (3.8) years were reviewed, 52 (54.2%) of the
weakness in the lower limbs with further progression to the children were males. The follow-up ultrasound showed that
upper limbs and urinary retention. On the examination, the 5 children (5.2%) had developed kidney stone. The
cranial nerves were spared (without RAPD), there was no occurrence of kidney stones was found in 4 females (80%)
Lhermitte sign, but a tetraparesis with a pyramidal pattern versus (20%), 1 male (p>0.05).
was found. Also, there was a sensory thoracic level, Discussion: Long-term use of TPM may result in incidence
hyperreflexia and bilateral Babinski sign. The aetiological increase of asymptomatic kidney stones in the paediatric
investigation disclosed: CSF with pleocytosis (PMN population. Hence, routine baseline and follow-up
predominance), extensive lesion from the postrema area ultrasound of the urinary system should be recommended
until the conus medullaris, with preferential involvement of during the use of TPM in children.
grey matter, a positive anti-AQPA4 ab and also both
hematuria and proteinuria. Methylprednisolone was
promptly started but due to clinical deterioration, with
necessity of invasive ventilation, cycles of cyclophosphamide
and rituximab were initiated. In spite of the aggressive
treatment strategy, she deteriorated with bilateral optic
neuritis, tetraplegia and finally fatal dysautonomia.
Conclusion: With this clinical report we intend to highlight
the potential pitfalls in the diagnosis of NMO, namely with
atypical presentations such as vomiting and renal
involvement which to our knowledge was not described
previously.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 199

P1299 P1300
Neuro-osteology - a new discipline in Early detection of minor
orthodontics neurodevelopmental dysfunction at the
I. Kjaer age of 6 months in prematurely born
Odontology, University of Copenhagen, Denmark neonates
Introduction: Neuro-osteology describes the inter- M.H. Tsai1, Y.-S. Huang2, S.M. Chu2, J.F. Hsu2
relationship between neural tissue and mineralized tissue Chang Gung Memorial Hospital, Yunlin, 2Chang Gung
1

(bone and teeth) under normal and pathological conditions Memorial Hospital, Taoyuan, Taiwan R.O.C.
(1). Special focus areas are pituitary gland/sella turcica (2), Introduction: Premature infants are known to have higher
CNS/neurocranium (3) and PNS/jaw and teeth (4). The risk of minor neurodevelopmental dysfunctions (MNDs)
prenatal interrelationship has been examined in human when 2 to 5 years old. We aimed to investigate the 6-month
foetuses in a long series of published studies now forming neurodevelopmental outcome of prematurely born neonates
the basis for analyzing malformations in postnatal jaw and find the determining neonatal factors of MNDs.
regions with different neural crest origins (5). Methods: 151 premature infants were assessed at 6 months
Methods: 17 children with Single Median Maxillary corrected age with the Bayley Scales of Infant Development-
Central Incisor (SMMCI) were examined by radiography 2nd Edition (BSID-II) and the Denver Developmental
and 2 of these by MR scan of the brain. Screening Test (DDST). Neonates with cerebral palsy or
Results: major neuromotor dysfunctions at discharge were excluded
Dentition: One single maxillary central incisor located in from analyses.
the midline. Results: Of 151 neonates born before 37 weeks, 20 (13.2%)
Maxilla and cranial base: No inter-incisal suture present. had MNDs at 6 months corrected age. These proportions
The jaw was retrognathic and the anterior cranial fossa was were 21.6%, 13.2%, and 8.2% for neonates born before 28
significantly shorter than normally. weeks, 29 weeks to 32 weeks, and 33 weeks to 36 weeks,
Brain: Midline structures of the brain, falx cerebri and the respectively. Half of neonates with MNDs have a birth body
pituitary gland were abnormal. The neuro-hypophysis was weight (BBW) of less than 1000g. BSID-II and DDST are
absent and interdigitized cortex was observed in the frontal highly correlated in assessing the MNDs of premature
lobes. Severe growth hormone deficiency was registered. neonates at 6 months corrected age. After multivariate
Conclusion: The study highlights the concurrence between logistic regression analysis adjusted to gestational age and
midline defects in dentition, jaw and frontal lobes and BBW, MND was independently associated with postnatal
pituitary gland. The condition may be part of the corticosteroid use (odds ratio [OR], 11.2; 95% confidence
Holoprosencephaly spectrum. The study provides guidelines interval [CI], 1.9-66.0, P= 0.008) and cholestasis (OR, 6.2;
for early diagnostics in the dentition with interrelation to 95% CI, 1.16-33.1, P=0.033).
CNS malformations. Conclusions: Premature neonates, even those born at 33 to
References: 36 weeks, are found to have MNDs as early as 6 months
1. Kjr I. Crit Rev Oral Biol Med 1998;9:224-44. corrected age by BSID-II and DDST, with risk increasing as
2. Kjr I et al. Eur J Pediatr Surg 1999;9:354-8. gestation decreases. Preterm infants at risk of MNDs justify
3. Kjr I et al. Neuropediatrics 2010;40:280-3. the requirement of intervention as early as possible.
4. Nielsen SH et al. Eur J Orthod 2006;28:529-34.
5. Kjr I. Eur J Orthod 2010;32:140-7.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


200 Posters, Sunday 9 September

P1301 P1302
West syndrome followed by juvenile Maternal alcohol drinking and
myoclonic epilepsy: a coincidental neurodevelopmental outcome in early
occurrence? infancy
G. Giordano, C. Spitaleri, A. Fontana, R. Nardello, G. Jeong1, J.Y. Han2,3, H.S. Kwak4
S. Mangano 1Pediatrics, 2Obstetrics and Gynecology, Cheil General
Dipartimento Materno Infantile di Urologia e Andrologia, Hospital and Women's Healthcare Center, Kwandong
Unit Operativa di Neuropsichiatria Infantile, Universit University, 3Division of Maternal Fetal Medicine, The
degli Studi di Palermo, Italy Korean Motherisk Program, 4Division of Endocrinology,
WS is an age-dependent epilepsy with onset peak in the first Department of Internal Medicine, Cheil General Hospital
year of life. According to the ILAE classification, the and Women's Healthcare Center, Kwandong University,
etiology of WS could be symptomatic or cryptogenic. An Seoul, Republic of Korea
idiopathic etiology was considered too. In literature, there Introduction: Fetal alcohol spectrum disorder (FASD) has
was never previously described a transition from WS to been identified as significant medical and public health
JME. problem. Many studies have reported morphologic
Methods: The proband, (male) was referred to our characteristics and long-term disabilities. However
Department at the age of 8 months because he showed neurodevelopment of early infancy has been rarely
clusters of symmetric spasms. Interictal EEG recording described.
displayed an hypsarrhythmic pattern. The clinical and EEG Methods: A prospective cohort study was conducted at
data suggested WS diagnosis. At 1 year of age increasing Cheil General Hospital Mother Risk Program. In total 236
long and thick hair in both elbow regions was observed. pregnant women participated in the research and filled out
This picture suggested an additional diagnosis of Hairy TWEAK questionnaire. After delivery and 12 months
Elbows Syndrome. During follow-up, the neurological postpartum, offspring5 were examined and mothers were
examination was normal and the EEGs showed age interviewed. Meconium was collected to measure fatty acid
appropriate background activity without abnormalities until ethyl ester (FAEE) in their offspring5. 106 babies being over
12 years of age, when he experienced some clusters of 10 months of age were performed the physically examined
bilateral, arrhythmic myoclonic jerks, synchronous with and Bayley scales of infant and Toddler Development III
generalized discharges of 4Hz spike-wave. This features (BSID-III) were performed. We compared the growth
suggested JME diagnosis. (height, weight, and head circumference), morphological
Results: We report a child with WS with onset at 8 months characteristics (palpebral fissure length and philtrum grade)
of age followed by JME at 12 years of age. This unusual and BSID-III results between maternal alcohol drinking
evolution, never reported previously, suggests that both group and non-drinking group.
seizure types may share some pathophysiological processes Results: The overall rate of alcohol drinking more than
genetically determined, which produce a susceptibility to once during pregnancy was 55% (66/120). In maternal
seizure. alcohol drinking group, TWEAK positive rate was 38%
Conclusion: This case documents a new transition type (25/66) and FAEE positive rate was 24% (8/33). One child
from WS to JME and improves the knowledge about the was diagnosed as foetal alcohol syndrome. For the growth,
spectrum of seizure susceptibility. These findings suggest children in the maternal alcohol drinking group showed less
that some genes other than those currently known and non- growth rate, shorter palpebral fissure length, higher
conventional genetic factors can play a role in seizure philtrum grade and lower BSID-III score than in the non-
predisposition. drinking group however statistical difference was not
significant.
Conclusion: Offspring of women drinking alcohol had
tendency of delayed neurodevelopment but that was not
prominent in early infancy. Development test alone could
not be a diagnostic tool for FASD without distinctive FAS
morphology.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 201

P1303 P1304
Paraneoplastic opsoclonus-myoclonus Audio-visual perception and related
syndrome in children memories among dyslexia disordered
N. Ben Achour, I. Ben Youssef-Turki, I. Kraoua, students in comparison with normal
H. Benrhouma, A. Rouissi, H. Touaiti, students
N. Gouider-Khouja A. Kazemi
Department of Child and Adolescent Neurology, National Psychology, Islamic Azad University Tehran Medical
Institute of Neurology, Tunis, Tunisia Branch, Tehran, Iran
Introduction: Opsoclonus-myoclonus syndrome (OMS), Introduction: Dyslexia is a disorder which causes some
called Kinsbourne syndrome is the most common problems in reading. Students with dyslexia suffer from
paraneoplastic neurological syndrome in children associated other improper functioning of the brain which is not
with neuroblastoma (NB) or more rarely with other tumours. necessarily because of physical injuries to it. The function
The objective is to study the epidemiological, clinical and of the brain in individuals with dyslexia differs from normal
therapeutic aspects of paraneoplastic OMS (POMS) people. The object of this study is to compare audio-visual
collected in our department. perception and related memories between a group of 2nd to
Methods: All children with POMS, followed up in our 4th grade elementary students with dyslexia disorder with a
department over 6 years (2005-2011), were reviewed. normal group.
Epidemiological data, clinical presentation and diagnostic Methods: The statistical sampling included 40 dyslexic
and therapeutic approach are discussed. students chosen according to available sampling method
Results: 4 children (3 boys and 1 girl) with POMS were and 40 normal students chosen according to simple random
collected. Mean age was 5.6 years. Acute ataxia was method. Both groups included boys and girls with equal
inaugural symptom. Sleep disturbances were found in 2 numbers from 2nd to 4th grades in elementary school in
patients. High rate of NSE was found in all patients. Tehran city. The participants were evaluated with Frostig
Onconeural antibodies were negative. Screening for a developmental visual perception test, Wepman's audio
tumour, including (CT scan, Whole-Body MRI and MIBG diagnosis scale, Andre-ray test and the sequence of audio
scintigraphy) allowed us to find the primary tumour, only in expressional memory scale. The results were analyzed using
2 patients (NB in the first case and rhabdod tumour of the T-Test method.
skin, in the second case). All patients received Results: Findings showed that there is a meaningful
immunomodulatory treatment (corticosteroids and difference between the groups in terms of visual -perception
intravenous immunoglobulin). Full recovery was obtained (t=9/39) and its five sub-scales including eye-motor
only in 1 patient who had etiological treatment. coordination(t=9/12), figure grand(t=9/03), form
Discussion and conclusion: POMS is quite a rare (4/1600 constancy(t=9/36), position in space(t=6/98) and spatial
children hospitalized in our department over 6 years) or relations(t=5/76); audio perception(t=9/74); visual
under-diagnosed entity. Patients may present with staggering memory(t=3/96) and audio memory(t=5/83) .
and falling, often leading to a misdiagnosis of acute Conclusion: The dyslexic disordered students compared to
cerebellar ataxia. Recognition of this disorder may lead to the normal students are less capable in audio visual
early detection and treatment of the paediatric neoplasms perception and related memories. There is also no difference
and may reduce the neurologic damage that is the major between boys and girls in both groups.
source of morbidity in children with successfully treated
tumours.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


202 Posters, Sunday 9 September

P1305 P1306
Role of glutathione redox status in the Diploid/tetraploid form of cutaneous
pathophysiology of autistic disorder mosaicism in a child with refractory
A. Corejova, A. Hrabovska, J. Kyselovic epilepsy and malformation of cortical
Department of Pharmacology and Toxicology, Faculty of development
Pharmacy, Comenius University, Bratislava, Slovak Republic
M.I. Sigatullina1, C. Fons2, F.V. Sanmarti3, M.I. Perez4
Introduction: Oxidative stress has been suggested to be 1Child Neurology Department, Tashkent Institute of
one of the key elements in the pathophysiology of autism. Postgraduate Medical Education, Tashkent, Uzbekistan,
Oxidative stress is produced by high levels of free radicals, 2Child Neurology Department, 3Epilepsy Unit, , 4Genetic

which, in physiological conditions, are under regulation by Department, Hospital San Joan de Deu, Barcelona, Spain
multiple antioxidative mechanisms. It has been proposed Objective: To analyze the concordance between the
that the loss of this regulation participates in the pathogenesis neurological symptoms and seizure semiology in a child
of autism. The aim of our project was to examine whether with a special form of cutaneous mosaicism.
potentiated antioxidative mechanisms would affect autistic Methods: The full spectrum of clinical, cytogenic,
disorder symptoms. continuous Video-EEG and MRI investigations were
Methods: Patients (n=43; 2-21 years old) with autistic performed.
disorder were enrolled. Exclusion criteria for subject Results: A 1-year-old girl was born from healthy non-
selection were as follows: Asperger syndrome, high- consanguineous parents. During the first 36 hours of life
functioning autism, epilepsy, selected pharmacotherapy she presented clonic seizures in the left hand and leg, with
affecting CNS. Methylcobalamine was given daily at a dose tonic head and eyes deviation to the right. During the first
of 500g. Venous blood was collected at d0, d90 and d180 year she developed refractory epilepsy with generalized
and redox status of glutathione as well as levels of tonic seizures. Family history was unremarkable for
homocystein and cobalamine were determined. The cutaneous and epilepsy disorders. Pregnancy and delivery
psychological profile was defined at d0, d90 and d180 by a were uneventful. The birth a lot of dismorphic features of
psychologist in combination with parent observations. the face have been noticed, heterocroma of iris, hypo- and
Results: Oral application of methylcobalamine (500g per hyper pigmented maculas at the skin of the right frontal area
day) influences glutathione redox status. Based on the and right leg, hypopigmented hair mixed with normal,
combination of parent and psychologist observations, which followed the Blaschko lines. Clinically the girl
language and social skills were improved in patients using presented severe mental retardation and diffuse muscle
methylcobalamine. Social interaction was increased, hypotonia. The EEG was characterized by focal slowing and
including social responsiveness and eye contact. epileptiform activity predominant in the right centro-
Conclusion: Oral application of methylcobalamine in parietal and centro-temporal areas. MRI of the brain
patients with autism seems to potentiate antioxidative revealed extensive right parieto-occipital lysencephaly. The
mechanisms and leads to changes in the psychological routine analysis of the caryotype in the peripheral blood was
profile of patients. normal: 46 XX. The cytogenic investigation of the
hypopigmented parts of the skin biopsy revealed the
Chromosomal Mosaicism 92, XXXX(10)/46,XX (20).
Conclusion: There is a possible close connection between
the cutaneous mosaicism and different types of
malformations, which show us the importance of caryotype
investigation in skin fibroblasts in patients with dysmorphic
features, pigmentation changes of the skin and brain
dysplasia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 203

P1307 P1308
Diagnostic brain imaging in congenital Leigh syndrome in 13 Tunisian patients
facial palsy E. Ellouz1, J. Abdelhedi2, I. Chamkha3, I. Ayadi2,
H. Uchikawa, K. Fujii, A. Takagi, Y. Kohno I. Hsairi2, F. Kamoun2, F. Fakhfakh4, C. Triki5
Pediatrics, Graduate School of Medicine, Chiba University, 1Pediatric Neurology, 2Hedi Chaker Hospital, 3Department
Chiba, Japan of Genetics, 4Genetics, Faculte de Medecine de Sfax,
5H. Buniatian Institute of Biochemistry, Sfax, Tunisia
Introduction: Congenital facial palsy is caused by various
disorders. But the pathogenesis of them often remains Introduction: Leigh syndrome is the most common clinical
unclear. We report three cases of congenital facial palsy phenotype of mitochondrial disorders in childhood. The
with identification of lesions by the radiological findings. diagnostic criteria include progressive neurological disease
Methods: We investigated the etiology of three Japanese with psychomotor delay, symptoms of brainstem or basal
patients with congenital facial palsy. ganglia disease, raised lactate levels and characteristic
Patient 1: The 11-month-old girl presented with left facial symmetric necrotic lesions in the basal ganglia or brainstem.
palsy noted at birth. During pregnancy her mother needed We try to determine clinical, radiological and genetic
insulin for diabetes mellitus. features of Leigh syndrome through the evaluation of 13
Patient 2: The 1-year-old girl was born as second of twins. Tunisian patients.
Right facial palsy was noticed after birth. Methods: We reviewed the observations of all patients with
Patient 3: The 6-year-old boy presented with bilateral facial a clinical presentation suggestive of Leigh syndrome; we
and abducens nerve palsy, and cleft of soft palate noted at determined the clinical and radiological features of these
birth. To investigate their etiology, we performed patients. Respiratory chain enzymes study was not
constructive interference in steady state (CISS) magnetic performed. The molecular study was performed in some
resonance imaging (MRI) and temporal bone computed patients.
tomography (CT). Results: We collected 13 patients belonging to 9 families.
Results: First CISS MRI were performed. In patient 1, the The mean age was 15 years; the mean age at onset was 2.1
left facial nerve was not observed. In patient 2, the bilateral years. Psychomotor delay was noted in 7 patients, gait
facial nerves were observed. In patient 3, the bilateral facial difficulties and movement disorders were noted in 9, spastic
nerves, abducens nerves and facial colliculuses were not paraplegia in 8, cerebellar ataxia and optic atrophy in 5,
observed. Next, temporal bone CT was performed in patient ophthalmoplegia in 4 patients. Brain MRI showed
2. The strictured right labyrinth portion of facial canal was involvement of putamen in 9 cases, caudate nucleus and
observed. Therefore, patient 1 and 3 were diagnosed with pallidi in 3 cases, brain stem in 5 cases. Molecular study
Mbius syndrome, patient 2 was diagnosed with congenital showed a novel missense mutation in the mitochondrial
unilateral facial palsy with strictured labyrinth portion. cytochrome C oxidase III gene in 2 patients, and a surf
Conclusion: We successfully identified the lesion causing mutation in 1 patient.
facial palsy using CISS MRI and temporal bone CT. Conclusion: Leigh syndrome has a clinical and genetic
Identification of the responsible lesion is important for heterogeneity. The diagnosis should be considered in
diagnosis and for considering the pathogenesis of congenital children with symptoms of brain stem or basal ganglia
facial palsy. disease. Radiological features with bilateral and symmetric
necrotic lesions of basal ganglia and brain stem are almost
constant.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


204 Posters, Sunday 9 September

P1309 P1310
Mucopolysaccharidosis VI in two siblings: Efficient generation of functional
an unusual clinical presentation dopaminergic neurons from hESCs
E. Ellouz1, E. Chtourou2, H. Ben Othmen2, I. Hsairi2, M. LI1, Y. Zou1, N. Tang2, G. Dawe2, I. Islam2, T. Cao2
F. Kammoun2, A. Miled3, C. Triki2 Oral Sciences, National University of Singapore, 2National
1
1Pediatric Neurology, 2EPS Hedi Chaker, Sfax, University Health System, Singapore, Singapore
3Hopital Farhat Hached Sousse, Sousse, Tunisia
Introduction: Years after the successful differentiation of
Introduction: Mucopolysaccharidosis VI or Maroteaux- neurons from human embryonic stem cells (hESCs) there
Lamy syndrome is a lysosomal storage disease characterized are still huge limitations of current protocols that are either
by systemic clinical manifestations. Diagnosis and difficult to replicate or unable to generate physiological
management are often challenging because of the functional cells. We have created a very simple and efficient
considerable variability in symptom presentation and rate of protocol in generating functional dopaminergic neurons
progression. from hESCs.
Methods: We report the observations of 2 siblings with Methods: hESCs colonies were first digested into a single
unusual clinical presentation of mucopolysaccharidosis VI. cell suspension and subsequently seeded onto poly-L-
Results: Our patients were 2 Lybian siblings (a boy and a ornithine coated plates with Stemgent and BMP inhibitor
girl) born from consanguineous parents, aged 20 and 4 LDN193189 over 10 days. The cells were subsequently
months respectively. The 2 patients presented with passaged on Matrigel coated plates for 7 days. Rosettes
psychomotor retardation. Neurological examination found were then selected through 1 hour treatment of rosette
a macrocephaly and a cerebellar ataxia in the boy. He did selection reagent followed by single cell dissociation with
not have any facial dismorphy or skeletal or joint accutase. Collected cells were then subjected to a stepwise
abnormalities. The girl has a macrocephaly, flaccid induced differentiation into dopaminergic neurons under
paraplegia, myelomeningocele and foot deformity. The neural basal media supplemented with N2, B27, shh, FGF-
cerebral MRI showed a T2 hyper intensity in the midbrain 8, GDNF, TGF-3, dc-AMP, laminin and fibronectin.
in the boy and hydrocephalus in the girl. The screening of Results: Differentiated cells gained neuron morphology
mucopolysaccharides in urine demonstrates an increase of with extending neurite network were observed within 10
dermatan sulphate at 22mg/l suggestive of days of induced terminal differentiation. Immunofluorescent
mucopolysaccharidosis VI. The enzymatic study was in staining showed positive expression of dopaminergic
progress. The boy died suddenly from dyspnoea. markers Tuj1, TH, DAT and pan Sodium channels.
Conclusions: Mucopolysaccharidosis VI or Maroteaux- Electrophysiological studies using Patch clamp confirmed
Lamy syndrome is a clinically heterogeneous condition. the neurons were physiological functional in firing action
Case studies reported in the literature have identified potential with clear evidence of threshold with 30pA current
subjects who presented with marked disease in the first year injection. Dopamine release was quantified by Dopamine
of life and others who presented with slowly advancing ELISA kit with sensitivity of 10-100ng/ml.
disease. Our patients belong to the first group. To our Conclusion: We successfully demonstrated the efficient
knowledge cerebellar ataxia, myelomeningocele and T2 generation of dopaminergic neurons from human embryonic
hyper intensity in the midbrain have not been reported stem cells and confirmed them as physiological functional
before. cells.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 205

P1311 P1312
Dysgraphia in children with asymmetric Chronic inflammatory demyelinating
bilingualism polyneuropathy in children
S.M. Golubovic, N. Novakovic, Z. Kasic A. Takagi1, K. Fujii1, H. Uchikawa1, S. Misawa2,
Faculty of Special Education and Rehabilitation, University Y. Kohno1
of Belgrade, Serbia Department of Pediatrics, 2Department of Neurology, Chiba
1

Objectives: The Vlachs of Serbia are an ethnic minority of University Graduate School of Medicine, Chiba, Japan
Serbia, culturally and linguistically related to Romanians. Background: Chronic inflammatory demyelinating
The Vlach languages, also called the Eastern Romance polyneuropathy (CIDP) is a slowly progressive disorder of
languages, have a common origin from the Proto-Romanian the peripheral nerves, causing demyelination and axonal
language. degeneration. In adults, the main treatments are intravenous
Methodology: The sample consisted of 22 children (from immune globulin (IVIg) and steroid therapy. However,
totally 113 tested) in early school age, from five rural treatment for paediatric CIDP still remains to be determined.
primary schools around Svilajnac and Zabar. 13 children We aimed to clarify the efficacy of IVIg and steroid
had dyslexic dysgraphia, and 9 had graphomotor dysgraphia. treatment in CIDP children.
The study was performed with the abbreviated version of Methods: We investigated the clinical course of 3 paediatric
Semantic test - S. Vladisavljevic, Three-dimensional test of CIDP patients. Mean age was 9.3 years (7-12), and their
reading (H. Sax) for assessment of written speech, Scale for gender was one male and two female. We performed
assessment of dysgraphia of writing (Ayiriagerra, Ozias), electrophysiological examinations to diagnose them as
and assessment of performance of graphomotor line (Lillian having CIDP by using the diagnostic criteria. We basically
Lirsa). treated these paediatric patients firstly with IVIg (400mg/
Results: Obtained results show that there is a statistically kg/day, 5 days), and secondary methylpredonisolone (30mg/
significant difference ( 0.02) on the semantic test in kg/day, 3days), following oral predonisolone (1-2mg/kg/
asymmetrically bilingual children regarding the type of day).
dysgraphia, which means that asymmetrically bilingual Results:
children with graphomotor dysgraphia showing better Patient 1: We treated a 7-year-old boy with IVIg, resulting
scores on the semantic test than asymmetrically bilingual in dramatical improvement. However, we failed to change
children with dyslexic dysgraphia. There was a statistically to steroid therapy, resulting in combination therapy.
significant difference ( 0.04) in the average speed of Patient 2: We treated a 9-year-old girl with IVIg and
reading between asymmetric bilingual children regarding methylpredonisolone with partial recovery. We finally could
the type of dysgraphia. There is also statistically significant finish the treatment.
difference ( 0.01) in the average number of errors regarding Patient 3: We treated a 12-year-old girl with IVIg, resulting
the type of dysgraphia. Asymmetric bilingual children with in partial improvement. After treatment with
graphomotor dysgraphia make less mistakes than the methylpredonisolone and oral predonisolone, she could
asymmetric bilingual children with dyslexic dysgraphia. walk again.
Conclusion: Asymmetric bilingual graphomotor dysgraphic Conclusion: All 3 patients showed improvement in motor
children on average remember more facts than asymmetric function by IVIg therapy. Subsequent steroid therapy is still
bilingual children with dyslexic dysgraphia. necessary for full recovery. IVIg is the most effective initial
treatment in patients with paediatric CIDP as well as adult
ones. Since IVIg has a potential risk of infection, the
transition from initial IVIg to oral predonisolone therapy
should be considered in children.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


206 Posters, Sunday 9 September

P1313 P1314
Partial agenesis of corpus callosum in Vestibular stimulation effect on quiet
Sanjad-Sakati syndrome (p-ACC): a case stance balance in 3- to 7-year-old children
report plus a review of articles) with cerebral palsy
N.S. Al Ghasab1, B. Janati2, A. Khan3 S.A. Hosseini1, H.A. Haghgoo1, B. Zeinal Zadeh1,
Qassim University, Hail, Saudi Arabia, 2Center for
1
E. Pishyareh1, S. Talebian2
Neurology, Virginia, VA, USA, 3ICU, King Khaled Hospital, 1Occupational Therapy, University of Social Welfare and

Hail, Saudi Arabia Rehabilitation Sciences, 2Tehran University of Medical


Introduction: Sanjad-Sakati syndrome (SSS) was first Sciences, Tehran, Iran
described in the Middle-East in children of consanguineous Background and aims: Children with cerebral palsy (CP)
parents. It is a rare autosomal recessive disorder known also show a variety of neurological deficiencies such as problem
as Middle-East syndrome or Richadson-Kirk syndrome or with processing tactile, somatosensory, proprioceptive,
hypoparathyroidism-retardation-dysmorphism (HRD) visual and vestibular information which leads to
syndrome. discoordination in bilateral movements, movement
Study objective: To describe a new finding in a rare genetic sequencing and planning and equilibrium disorders. The
disorder where literature is meagre. vestibular system plays an important role in the general
Methods: The clinical investigation was conducted at King development and balance which is distorted severely in CP
Khaled Hospital in Hail, Saudi Arabia in the year 2011. children. We aimed to investigate the effect of vestibular
Results: General physical examination showed the patient stimulation on quite stance balance in CP children.
to be short-statured with facial dysmorphic features Methods: Vestibular stimulation was performed twice
including a long narrow face, deep seated eyes, peaked weekly with a course of 12 sessions for 20 CP children.
nose, depressed nasal bridge, micrognathia, thin lips, low Using force plate, different parameters such as sideway
set and large floppy ears, small hands and small feet. The range, range fore-after, area and mean velocity were
patient had hypotonia, poor motor coordination, delayed measured. Data were analyzed statistically using U-Mann-
developmental milestones. Whitney and Wilcoxon tests.
The brain MRI in our patient showed a partial agenesis of Results: Mean velocity parameter as a postural control
the corpus callosum (p-ACC) characterized by absence of parameter showed significant changes but no significant
the splenium and rostrum, a finding which has not change was shown in stability postural parameters.
previously been reported in SSS. Conclusion: Application of vestibular stimulation for CP
Conclusion: Our patient showed partial agenesis of the children can lead to significant changes in postural control
corpus callosum which could explain the patient's motor, mean velocity. Accordingly, those children who have
behavioural and developmental abnormalities. The wide received vestibular stimulation in this project showed a new
variety of MRI presentations reported in the SSS literature balance strategy that would lead to an increase in exploratory
may represent different gene mutations. behaviour which would help them to maintain their balance.

[SSS Figure 1]

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 207

P1315 P1316
Effectiveness of topiramate and Neurophysiological perspectives of
levetiracetam in infantile spasms non- electroencephalography in children with
responsive to steroids attention deficit hyperactivity disorder
A.A.H. Mahmoud (ADHD)
NNI, KFMC, Riyadh, Saudi Arabia A. Dube1, K. Yadav1, A. Gupta2, Y. Yadav3
Aim: To compare effectiveness of two novel antiepileptic 1 Physiology, 2Pediatrics, S.M.S. Medical College,
drugs, topiramate and levetiracetam as a second line 3 Pediatrics, Sevayatan Hospital, Jaipur, India
treatment for infantile spasms when oral steroids fail. Objective: The present study was undertaken to digitally
Method: 40 infants within 2 years of life with clinically- evaluate the electroencephalographic signature in children
and EEG-proven infantile spasms that did not respond to with Attention Deficit Hyperactivity Disorder (ADHD).
prednisone (2mg/kg/day in two divided doses) were Method: 30 children in the age range of 3-11 years were
recruited and randomized into two groups. They were included in the present study and power spectral analysis of
randomly assigned to either topiramate (group 1; 1mg/kg/ electroencephalographic (EEG) output from the central
day for 3 days, then increased to 1mg/kg/day every third day EEG electrode pair location (preferably the Cz - Vertex pair)
up to 6mg/kg/day) or levetiracetam, (group 2; 10mg/kg/day was run to test the hypothesis that cortical slowing in the
for 5 days and then increased to 10mg/kg/day every 5 days prefrontal region can serve as a basis for differentiating
up to 60mg/kg/day). children with ADHD from healthy children.
Results: Of the 20 patients included in the final data Results: Quantitative electroencephalographic findings
analysis, 11 (55%) were administered topiramate and 9 indicated significant increased theta power and decreased
(45%) levetiracetam. 18 patients did not respond to the first delta power seen in patients with ADHD with lack of
drug and subsequently to the other drug when crossed-over, suppression of Mu-waves which suggested significant
if the first drug proved ineffective. 2 patients with infantile maturational dysfunction in cortical arousal in the prefrontal
spasms responded to either one drug respectively without cortex, cortical slowing and dysfunctional mirror neuron
crossover. Their EEGs improved with time. system in children with ADHD.
Discussion: The present study demonstrated the Conclusion: These findings constituted a positive initial
ineffectiveness of topiramate and levetiracetam suggesting test of a QEEG- based neurometric test for use in the
current treatment modalities are grossly inadequate assessment of ADHD and the significance of the mirror
underscoring the urgent need for more research efforts to neuron system in the disorders of the social mind.
overcome current deficiencies. 2 patients with cryptogenic
infantile spasms responded to treatment suggesting the
potential for treatment of such patients with these two
drugs, which merits further multicentre investigation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


208 Posters, Sunday 9 September

P1317 P1318
Treating children with epilepsy and Semantic-pragmatic abilities in children
comorbid attention-deficit/hyperactivity with graphomotor and dyslexic-dysphasic
disorder (ADHD) dysgraphia
I. Dumitru1, A. Salan2 S.M. Golubovic1, G.R. Colic2
Pediatric Neurology, 2Pediatric Psychiatry, Country
1 1Speech and Language Pathology/Logopedics, 2Faculty of
Hospital Dr. Fogolyan Kristof, Sfantu Gheorghe, Romania Special Education and Rehabilitation, University of
Introduction: 30-40% of children with epilepsy have Belgrade, Serbia
symptoms of ADHD. That causes problems at school and at Introduction: Since pragmatic deficits are often connected
home. Despite the evidence of impairment from ADHD, with language disorders, and specific learning disabilities,
clinicians have difficulty in deciding whether to treat the goal of this study was to investigate the semantic-
children for both epilepsy and ADHD. pragmatic abilities in children with graphomotor dysphasic-
Methods: We treated 30 children with epilepsy and ADHD dyslexic dysgraphia, in the age from 8 to 12 years of both
for 3 months with anticonvulsants and then added genders.
atomoxetine (1.2mg/kg/day), in the morning. We used Methodology: The sample consisted of 40 children of both
questionnaires for children, parents and teachers to evaluate genders, age from 8 to 12 years, from second to fifth grade
the school performance, behaviour at home and school, of elementary school. The sample was divided in two
relationships with friends and family every 3 months for a groups: the first group consisted of children with
year. We also performed EEG every month. graphomotor dysgraphia, and the second group consisted of
Results: 25 of these children had been seizure free before children with dysphasic-dyslexic dysgraphia, with IQ of 90
added atomoxetine; 3 were stable, 2 experienced a reduction and above 90. For examination of semantic-pragmatic
in seizures. In 28 children there was no effect on seizure abilities, we applied the Test of Pragmatic Language (TOPL)
frequency, or anticonvulsant drug levels the EEG findings and the Semantic test.
doesn't change after introducing atomoxetine. 2 of them Results: In the test for assessment of pragmatics, children
have an increased epileptic discharge. 24 children improved with graphomotor dysgraphia have achieved, in average, 36
compartmental and school performances. 1 no influence points (59.69%), while children with dysphasic-dyslexic
was seen. 3 of them had good scores at questionnaires dysgraphia achieved 29 points (19.8%). There is a
completed by teachers, but no improvement in family. statistically significant difference (p=0.000) in pragmatic
Conclusion: If there is ADHD that can be diagnosed abilities between children with graphomotor and dysphasic-
independently of epilepsy effects, then further improvement dyslexic dysgraphia. In the semantic test, children with
in inattention and hyperactive/impulsive symptoms can be graphomotor dysgraphia achieved on average 36 points. The
made by the addition of a specific ADHD treatment. best results were achieved in the category of word
Although there are no recommended guidelines, we have homonyms, and the worst in synonyms. Children with
the opinion that if inattention and hyperactivity symptoms dysphasic-dyslexic dysgraphia have on average achieved 29
persist for more than 3 months after the last epileptic points, best results were with the homonyms and were worst
seizure, one can go for specific treatment. with synonyms. The semantic ability assessed in children
with graphomotor and dysphasic-dyslexic dysgraphia was
statistically significant (p = 0.003).
Conclusion: Children with graphomotor dysgraphia differ
from children with dysphasic-dyslexic dysgraphia in all
analyzed variables, except for the subscales of synonyms
and antonyms.

P1319
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 209

P1320 P1321
Impact of non-pharmacological treatment A new case of Worster-Drought syndrome
on tics in children C.S. Spitaleri, F. Caramella, P. Glorioso, R. Nardello,
S. Bakhtadze, N. Geladze, N. Khachapuridze A. Fontana, S. Mangano
Child Neurology, Tbilisi State Medical University, Tbilisi, University of Palermo, Palermo, Italy
Georgia Introduction: Worster-Drought syndrome (WDS) consists
Object: Tics are involuntary, sudden, rapid, recurrent, of a congenital pseudobulbar palsy and is usually associated
stereotyped motor movements or phonic productions that with spastic tetraplegia, learning impairment, behavioural
involve discrete muscle groups. Pharmacological treatment problems, and epilepsy. Congenital bilateral perisylvian
was considered as the most effective approach for syndrome (CBPS) is characterized by bilateral perisylvian
management of tics for many years. In recent periods polymicrogyria on imaging. Clark et al, have previously
clinicians attempt to use behavioural methods for this proposed a WDS spectrum that includes CBPS, speculating
purpose. The aim of our study was to use non that it may be due to malformation of the perisylvian region
pharmacological treatment like EEG biofeedback-neuro- due to various perinatal or congenital causes, whether
feedback (NF) for the treatment of tics. demonstrable on imaging, or functional and not visible with
Methods: We have previously examined 15 non-treated current imaging techniques. Worster-Drought suggested
children (9 boys and 6 girls) with simple tics (average age that the syndrome is probably a developmental defect of the
10 years). All children with complex tics and with other motor tracts (corticobulbar) which run from the lower part
comorbidities were excluded from the study. Tics frequency of the motor (Rolandic) area of the cerebral cortex to the
and severity were assessed by Yale Global Tic Severity Scale 10th and 12th cranial nerve nuclei.
(YGTSS). Sensorimotor rhythm (SMR) training was used Methods: A 12-year-old boy presented with a long history
for NF therapy. 30 sessions of NF with duration of 30 of spastic tetraplegia, learning impairment, behavioural
minutes each was conducted in every patient. Data were problems, and epilepsy. The seizures were refractive to
analyzed by SPSS 10.0. ANOVA was used to determine the several antiepileptic drugs. He also had variable difficulty
effect of treatment on YGTSS parameters. in moving the tongue, soft palate and lips with weakness of
Results: The ANOVA showed a significant effect of the pharynx and laryngeal muscle.
treatment on YGTSS measures (F(1.37)=223.69, Results: Video-EEG monitoring captured paroxysms of PO
MSE=114.735, p<0.0001). These evidences suggest that NF in the center-front of both hemispheres, and that project
significantly improves the severity and frequency of tics. contralaterally. Neuroimaging studies do not revealed
Conclusions: Thus effectiveness of SMR training in abnormalities.
children with tics is important, since the drugs used for the Conclusion: Our patient presented the characteristic
treatment have severe side effects, compliance problems etc. symptoms of Worster-Drought syndrome (WDS) with mild
Cognitive behavioural therapy is effective not only for spastic tetraplegia, learning impairment, and epilepsy. MRI
reduction of tics but also for increasing self-esteem and did not show dysplastic cortex in the region of the sylvian
social competence as well. fissures, giving normal signal on T1- and T2-weighted
images. Although the white matter had a normal appearance,
an abnormality in the corticobulbar tracts cannot be
excluded.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


210 Posters, Sunday 9 September

P1322 P1323
Clinical characteristics of paroxysmal Clumsiness in children with ADHD
kinesigenic dyskinesia in paediatric compared with normal children based on
practice the Lincoln Ozeretski Scale
S. Kwon, J. Suh, H.-E. Seo, S.K. Hwang A. Kazemi1, F. Khaledi2, M. Kazemi2
Pediatrics, Kyungpook National University School of 1Psychology, Islamic Azad University Tehran Medical

Medicine, Daegu, Republic of Korea Branch, 2Islamic Azad University, Tehran, Iran
Purpose: Paroxysmal kinesigenic dyskinesia (PKD) is one Introduction: Clumsiness is a syndrome that has a
of the movement disorders in which dyskinesia occurs in a pathological meaning. It is mainly concerned with the motor
part of the body by a sudden movement under a certain activities of children. The children who are suffering from
circumstance. It is rare, but important because it mimics such a problem are not able to perform normal motor skills
epileptic seizures. This study was aimed to evaluate the even if they do not show any neurological symptoms in
clinical features of children and adolescents with PKD in laboratory tests. The object of the present study is the
Korea. evaluation of clumsiness in children with ADHD compared
Methods: A total of 7 children with PKD was involved in with normal children based on Lincoln Ozeretski Scale.
the study. The demographic and clinical characteristics were Methods: The statistical sample consisted of 30 boys with
retrospectively evaluated from their medical records. ADHD, who were selected according to an available
Results: The age of the subjects ranged from 10 to 21 years sampling method and 30 boys without ADHD, selected
(mean age: 15.75.7 years). Males were predominant (M:F; through random sampling, at the age of six. Measurement
6:1). They all presented with dystonias of abrupt onset with tools consisted of Motor Development Lincoln-Ozeretski
an average duration of 10.5 seconds (3.5-17.5 seconds), Scale. Data were analyzed using T-Test.
which were triggered by various sudden movements. They Results: The results showed that there is a significant
were multifocal, but mainly in limbs. No obvious difference between the kids motor skills and the dependent
comorbidities were found. 6 of the subjects, except for a variables (t=10.43).
non-compliant case, responded well to the medications and Conclusion: Children with ADHD compared to normal
remained symptom free. They were treated with either children are at a lower level of motor skills, and suffer from
oxcarbazepine (n=4, 14.95.8 mg/kg/day) or lamotrigine clumsiness. In other words, children suffering from this
(n=2, 1.50.9 mg/kg/day). There were no significant syndrome, show incompetent and abnormal motor-skills in
differences between the two groups in terms of effectiveness, learning and doing an activity expected from them.
adverse events, etc.
Conclusions: This study showed that clinical features of
Korean children with PKD are quite similar to those of
other Western countries. They responded well to the
medications. In addition, lamotrigine can be an alternative
treatment for PKD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 211

P1324 P1325
Some etiological factors of stroke in Neuron-specific enolase as marker of
children brain lesions in children with epilepsy
S.S. Shamansurov, S.H. Saidazizova, N.M. Tulyaganova C. Calcii, S. Hadjiu, I. Iliciuc
Tashkent Institute of Postgraduate Medical Education, Hospital of Mother and Child Health Care, Chisinau,
Tashkent, Uzbekistan Moldova
Introduction: Today, in adults much attention is paid to Aim: To reveal the correlation of serum NSE concentrations
question an acute vascular pathology, but not less interest is with the type of epilepsy, including seizure characteristics,
the problem of the sharp vascular accidents developing at frequency, age of onset, and presence of mental retardation.
childrens age. Methods: To achieve the proposed objectives, the study
Aim: To investigate etiology of stroke in children. included 108 children with age ranging from 2 weeks to 24
Methods: The analysis of clinical supervision of 27 months (mean age 9.21.2 months), 61(56,0%) boys and 47
children. All children were divided at the moment of acute (44.0%) girls. 43 patients (39.84.71%) manifested
stroke. Hemorrhagic Stroke in 18 patients, ischemic in 9 generalized seizures, 53 (49.14.81%) had partial seizures
patients. and 12 children (11.13.02%) had polymorph seizures.
Results: Infections were present in 11 patients (vasculitis), Results: Serum NSE concentrations were elevated in 35
DIC in 8, hemophilia in 1, AVM in 4, Mitochondrial disease (32.4%) children from the study group. Out of them 12
(syndrome MELAS) in 1, Neurophakomatosis in 2 patients. patients (22.65.74%) had focal epilepsy, 15 children
Distribution on age: 17 patients younger than till 40 days, (34.87.26%) manifested generalized type of seizures and
life (the picture of Hemorrhagic Stroke occurred 7 8 children (66.713.60%) were diagnosed with polymorph
newborns; Ischemic stroke in at 10); on average attacks seizures. Elevated sera NSE concentrations were identified
occured on days 34-38; in all cases Hemorrhagic Stroke was in following subgroups of patients: 15 out of 30 children
the DIC syndrome. The second group of children one year with several epileptic attacks per day (50%, rxy+0.43), 13
told included 5 patients: on a share of ischemic stroke - 2 out of 41 children with several epileptic attacks per week
patients (trauma); hemorrhagic stroke - 3 patients (infectious (32%, rxy+0.49), 4 out of 16 children with several epileptic
process). The third group included children up to 3 years old attacks per month (25%, rxy+0.29), and in 3 out of 21
(2 children): 1 child with a virus infection; 1 child with children with two unprovoked seizure attacks (15.0%,
MELAS syndrome. The fourth group included children rxy+0.31). A moderate correlation (rxy+0.44) was observed
from 4 till 13 years (3 patients) with traumatic damage (VB between the raise of the serum NSE concentration and onset
system). of epilepsy in the neonatal period. Strong correlation
Conclusions: The revealed reasons for the acute damage of (rxy+0.88) showed the development of mental retardation
the brain blood circulation at children, have revealed a and elevated serum NSE concentrations.
significant divergence in etiology and are also very often Conclusions: Increased concentrations of serum NSE were
occuring in children. revealed in children with epilepsy who complained daily or
weekly seizure attacks, who developed epilepsy in the first
month of life and who presented mental retardation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


212 Posters, Sunday 9 September

P1326 P1327
Neurophysiological perspectives of Neurotrophic factors and disorders of
electroencephalography in children with residual psychological development of the
attention deficit hyperactivity disorder child
(ADHD) A. Hadjiu1, S. Hadjiu2, E. Hadjiu3, C. Calcii2
A. Gupta
1Universit de Reims Champagne-Ardenne, Reims, France,
Paediatrics, SMS Medical College, Jaipur, India
2Neurology, 3State University of Medicine and Pharmacology
N. Testemitanu, Chisinau, Moldova
The present study was carried out in the Department of
Pediatrics & Dept. of Physiology in collaboration with the Objective: To highlight the relationship between mixed
Departments of Neurology, SMS Medical College, Jaipur. specific psycho-developmental disorders in children and
30 children in the age group of 3 to 11 years (median age BDNF levels.
range of 7-10 years) with an intelligence quotient (IQ) of Materials and methods: We assessed BDNF serum levels
more than 70, suffering from ADHD (male : female ratio via immunoenzymatic method in 48 children (study group)
being 3:1), diagnosed as per DSM IV criteria, were included presenting residual mixed specific psycho-developmental
in the study. An equal number of children matched for age disorders (aged between 2 and 5 years old) and 25 healthy
and sex acted as the control cohort. children (control group).
18 to 20 epochs were selected for Power Spectral Analysis, Results: Patients from the study group presented: language
each lasting 2-3 seconds, the time duration representing retardation, emotional, cognitive and behavioural disorders.
confocal and frequency matched neuronal pool based on the An overall analysis showed that BDNF serum levels were
theory of analysis of sharp changes or rapid transitional significantly lower in children from the study group in
processes (RTP) reflected in quasi-stationary segments of comparison to controls (p< 0.05). Significantly low levels
local EEGs Epochs with more than 100v on the electro- of serum BDNF were noted in children with severe cognitive
encephalogram representing artifacts were excluded from disorders frequently associated with language disorders.
the mean. However no significant difference could be seen Moreover, neuroimaging data revealed abnormalities of
in the absolute powers of beta and alpha waves. The nerve tissue maturation. There is statistical evidence that
increased theta/beta ratio in ADHD children and absence of psychological disorders are negatively correlated with
Mu-wave suppression [a characteristic feature of the mirror serum BDNF levels. Thus, BDNF represents an important
neurons operational architectonics (Oberman et al, 2005)] marker a of child's psychological development.
give an insight into the features of the disease process of Conclusions: Our data confirm the role of BDNF on the
ADHD, namely cerebral dysmaturation (Monstra et al, child's psychological development (it contributes to nerve
1996) and broken mirrors. In the present study no significant fibre maturation, language, behaviour and emotional
difference could be seen in the absolute powers of alpha and centres). We suggest that low levels of BDNF influence the
beta waves in ADHD children and the control group. synaptic plasticity and the interaction between neural and
glial cells, thereby playing a decisive role in the complex
child's behaviour including language acquisition, cognition,
affect etc. BDNF studies could have an important
implication in the comprehension of psycho-developmental
disorders, as well as in the treatment of neurodevelopmental
disorders.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 213

P1328 P1329
BDNF in children with motor disorders Rituximab in refractory seronegative
E. Hadjiu1, S. Hadjiu1, A. Hadjiu2, C. Calcii1 juvenile myasthenia gravis with crisis
1State University of Medicine and Pharmacology R.L. Koul, A.M. AlFutaisi, R.M. Abdwani
N. Testemitanu, Chisinau, Moldova, 2Universite de Reims Childhealth, Sultan Qaboos University Hospital, Muscat,
Champagne-Ardenne, Reims, France Oman
Objective: Assessment of serum BDNF levels in children
with motor disorders
Materials and methods: We assessed serum BDNF levels P1330
via immunoenzymatic method in the blood of 54 children, Giant axonal neuropathy - first case
aged between 1 and 5 years, presenting diverse motor report in Romania
disorders and who had CNS perinatal hypoxic-ischemic
antecedents, and 25 healthy controls. I. Dumitru1, A. Salan2, L. Delne3
1Pediatric Neurology, 2Pediatric Psychiatry, 3Radiology,
Results: The results suggest that there is a relationship
Country Hospital Dr. Fogolyan Kristof, Sfantu Gheorghe,
between serum BDNF levels and motor abnormalities in
Romania
children with cerebral maturation disturbances (data was
also confirmed by neuroimaging). Furthermore, we noted
that serum BDNF levels were significantly lower in children P1331
with severe motor disorders compared to controls, but this Neurologic problems in children with
tendency was not significant in those with soft motor
disorders. The results showed an increase of BDNF levels in
autism spectrum disorder and ADHD
the study group during their first years of life, though these H.J. Park1, J.Y. Sim2, W.-S. Kim3
levels were significantly lower in comparison to the control Eulji University Hospital, Daejeon, 2Ceongju Saint Mary
1

group. Hospital, Ceongju, 3Chungbuk National University Hospital,


Conclusions: Serum BDNF levels were significantly lower Cheongju, Republic of Korea
in children with motor disorders (aged 1-5 years) in
comparison to controls. This could suggest a delayed
increase of BDNF levels during those children's
P1332
development. Thus, low BDNF levels are negatively Types of cerebral palsy, developmental
correlated with the severity of motor disorders. This could delay and epilepsy in children after
indicate the role of BDNF in plasticity of motor neurons, neonatal seizures
developmental and maintenance processes of the brain.
C. Calcii, I. Iliciuc, S. Hadjiu, A. Antohi, M. Gotonoaga
Hospital of Mother and Child Health Care, Chisinau,
Moldova

P1333
Abstract cancelled

P1334
Effectiveness of iron therapy on breath
holding spells in children
E. Bidabadi1, M. Mashouf2
1Guilan University of Medical Sciences, 2Arya Hospital,
Rasht, Iran

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


214 Posters, Sunday 9 September

P1335 P1339
Apraxia of speech in children: attempt of Comparative study of febrile or afebrile
etiological diagnostics seizures associated with mild gastro-
M.Y. Bobylova1, K. Mukhin1, I. Kirova2, enteritis
M. Ryakhovskaya2 S. Chung, E.H. Lee
Svt. Luka's Institute of Child Neurology & Epilepsy, 2Child
1
College of Medicine, Kyung Hee University, Seoul, Republic
Diagnostic Center 'Razvitie Plus', Moscow, Russia of Korea

P1336 P1340
Idiopathic unilateral isolated hypoglossal Cognitive testing and video-EEG
nerve palsy: a case report monitoring in children with consequences
I. Dumitru1, S. Paripas2 of perinatal impairment of the central
1Pediatric Neurology, Country Hospital 'Dr. Fogolyan
nervous system and epilepsy
Kristof', Sfantu Gheorhhe, 2Neurosurgery, Emergency County
Hospital Brasov, Brasov, Romania S.Y. Lavrick, S.V. Domitrak, V.V. Shprakh, L.V. Kolesova
Department of Neurology, State Medical Academy of
Postgraduate Education, Irkutsk, Russia
P1337
Possible birth injuries of the newborns at P1341
Caesarean section Dysgraphia in gypsy children with
F.N. Samadov, S.S. Shamansurov, S.H. Saidazizova semilingualism
Tashkent Institute of Postgraduate Medical Education,
Tashkent, Uzbekistan S.M. Golubovic1, V. Nestorov2, Z.N. Kasic1
1Faculty of Special Education and Rehabilitation, University

of Belgrade, 2PI 'Savski Venac', Belgrade, Serbia


P1338
Seizures in neonates P1342
B. Medjo1, D. Nikolic2, M. Atanaskovic-Markovic3, Management of seizures in Bosnian
S. Rsovac1, J. Kalanj1, N. Vunjak1
1Neonatal Intensive Care Unit, 2Department of Neurology,
children
3Department of Pulmonology and Allergology, University A. Bajraktarevic1, S. Trninic1, M. Miokovic1,
Children's Hospital, School of Medicine, University of L. Kumasin1, I. Suljevic2, S. Uzicanin3, H. Niksic4
1Pediatrics Department, Public Health Institution of
Belgrade, Serbia
Sarajevo Canton, 2Department for Biochemical Diagnostics,
Clinical Medical Center Sarajevo, 3Neuropediatrics
Department, Pediatrics Clinic Sarajevo, 4Department for
Clinical Pharmacology, Pharmacy Faculty Sarajevo, Bosnia-
Herzegovina

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 215

Clinical Neurophysiology P1345


The way how various cognitive tasks
P1343 impair the activity of subthalamic nuclei
and upper limb tremor
Saccade-related beta-band
N. Kovacs1, I. Herndi1, G. Deli1, E. Bosnyk1, F. Nagy1,
desynchronization in the subthalamic J. Janszky1, I. Bals2
nucleus of Parkinsons disease patients 1Department of Neurology, 2Department of Neurosurgery,

during successful antisaccades and error University of Pecs, Hungary


trials Background: It is a clinically well-known phenomenon
A. Yugeta, W.D. Hutchison, R. Chen that different cognitive tasks (e.g. counting, thinking about
University of Toronto, Toronto Western Research Institute, the disease) may alter the tremor characteristics in idiopathic
Toronto, ON, Canada Parkinsons disease (PD).
The basal ganglia are involved in response selection. We Objectives: To analyze the changes in the firing pattern of
have shown that the saccade-related beta-band subthalamic nuclei (STN) and the concomitant tremor
desynchronization (beta-SRD) in the subthalamic nucleus parameters during different cognitive tasks.
(STN) may play a role in inhibition of unwanted movements Methods: 6 patients with PD undergoing bilateral STN
and in execution of movements. Antisaccades require an deep brain stimulator implantation were enrolled into our
inhibition of reflexive saccades towards the visual cue and study. During microelectrode recording, the preamplified
an initiation of voluntary saccades away from the visual cue. and unfiltered electrical activity of subthalamic nuclei was
If beta-SRDs are involved in voluntary saccades, we registered by Medtronic Leadpoint 5 system. The activity of
hypothesize that beta-SRDs would be deficient during STN and the bilateral limb tremor were simultaneously
antisaccade errors. To investigate this, we studied beta- captured by a CED Power 1401 AD converter during three
SRDs in the STN of Parkinsons disease (PD) patients different tasks: baseline resting condition, counting and
during successful antisaccades and error trials. 20 PD word-generation tasks. Raw recordings were filtered and
patients performed an antisaccade task 1 to 3 days after subsequently analyzed by Spike 2 software (CED Inc.,
deep brain stimulation surgery with local field potential version 7.08). We determined the frequency and discharge
recording from the STN via externalized leads. Electro- pattern of neuronal activity by spike sorting involving both
oculography was used to measure saccades. We analyzed template matching and principal component analysis
the wavelet power spectrum averaged on saccade onset. The techniques. Between the tremor and STN signals cross-
incidence of errors was 34.5%. Latencies of saccades were correlation and coherence analyses were also performed.
longer during successful antisaccades (426.977.4ms Results: In the baseline resting condition the intensity and
[meanSD]) than during errors (312.987.2ms; p<0.01). the frequency of tremor was significantly lower than that
Beta-SRDs were observed more often during antisaccades during counting (124 milli-g2 vs. 352 milli-g2 and 4.12Hz
(27 of 34 STN) than during errors (7 of 34 STN; p<0.01). vs. 4.32Hz, respectively). Meanwhile, we observed a
Onsets of beta-SRDs were not significantly earlier during tendency that the firing pattern of STN became more burst-
antisaccades (-180.5165.9ms) than during errors like (bursting narrow pos skew or burst-like bimodal)
(-76.4279.1ms; p=0.25). Beta-SRDs were sustained for during cognitive tasks.
longer during antisaccades (528.9205.7ms) than during Discussion: Cognitive tasks may influence the firing pattern
errors (374.5264.3ms; p=0.03). These results suggest that of STN and concomitant tremor. Further studies are required
beta-SRDs play a role in the suppression of unwanted to analyze the importance and possible clinical implications
saccades and in the successful execution of antisaccades. of this phenomenon.

P1344
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


216 Posters, Sunday 9 September

P1346 P1347
Cortical alternating generators of sleep Role of the cerebellum in the motor
spindles: electrical source imaging using control of Donders law during human
high-density EEG (256-channels) pointing tasks: an rTMS study with
recordings kinematic analysis of the wrist
A. Del Felice, C. Arcaro, S.F. Storti, P. Manganotti L. di Biase1, F. Giambattistelli1, F. Mastrolilli1,
Department of Neurological, Neuropsychological, F. Taffoni2, D. Formica2, P.M. Rossini3, F. Vernieri1,
Morphological and Motor Sciences, University of Verona, J.M. Melgari1
Italy 1Department of Neurology, 2Biomedical Robotics and

Introduction: Sleep spindles are the hallmark of N2 sleep, Biomicrosystems Lab, Universita Campus Biomedico di
with a frequency between 10-16Hz and a duration of 0.5- Roma, 3Universit Cattolica del Sacro Cuore, Roma, Italy
2sec, localizing over the fronto-centro-parietal cortical Introduction: Donders law well represents intrinsic
areas. Anterior spindles have a relative slower frequency strategies of motor control system during pointing tasks,
compared to posterior ones, although no consensus exists executed through eye, head or arm movements [1-2]. The
on the cortical localization of spindles generator. central nervous system (CNS) removes the degrees of
Methods: 136 spindles were obtained by visual inspection freedom that are not essential (solution of the redundancy
from a 256 channels EEG (Geodesic, Eugene, USA) sleep problem). Aim of the present study is to identify the CNS
recordings from 6 healthy volunteers. Offline analysis regions involved in the control of Donders law applied to
filtered the marked traces at 10-12Hz (slow spindles) and the motor control of human pointing tasks.
12-14Hz (fast spindles); traces of each category were Material and methods: 11 healthy subjects underwent a
segmented, baseline-corrected, bad channels rejected by session of inhibitory rTMS (1Hz) of right cerebellar
visual inspection and replaced, and averaged. Averaged hemisphere, while 13 control volunteers, underwent a sham
traces were used to reconstruct on the MNI (Montreal cerebellar rTMS session. Each subject executed a kinematic
Neurological Institute) magnetic resonance template via an analysis of the wrist during pointing tasks to evaluate
inverse solution method (LORETA algorithm) cortical Donders law [2] before and after the rTMS session. The
generators of slow and fast spindles. orientation matrix R of the wrist was measured by means of
Results: A total of 64 slow and 70 low spindles, temporally inertial magnetic unit.
alternating on the EEG, were analyzed. Visual rendering of Results: The MANOVA analysis shows significant
spatial distribution of spindles by EEG-signal projection on modifications in the curvature of Donders plans obtained
a cortical flat map confirmed the fronto-central distribution before and after rTMS cerebellar neuromodulation, in 78%
of slow spindles and the centro-parietal localization of fast of healthy subjects that underwent to real rTMS session,
ones. The source generator reconstruction localized the while no significative differences were found in sham
generator in the anterior prefrontal cortex (middle frontal group.
gyri, Brodmann area 10) for the slow spindles and in the Discussion: The present data suggest a cerebellar control of
parietal cortex (paracentral lobule, Brodmann 5; posterior Donders law applied to the redundancy problem solution in
parietal cortex, 7; supramarginal gyrus, 40) for the fast human pointing tasks. This method could have an
ones. application in clinical practice, particularly for functional
Conclusion: Sleep spindles show different, alternating evaluation. Since the curvature of Donders plan is like an
cortical generators according to their frequency and individual fingerprint, these findings could be useful in
topographical distribution on the scalp. This observation many neurodegenerative diseases, in which neuronal loss
suggests the existence of different cortical networks for occurs earlier than the onset of clinical symptoms and in
slow and fast spindles. individually-tailored and neurophysiology-based
rehabilitation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 217

P1348 P1349
Left prefrontal cortex influences motor Multivariate gait data analysis:
imagery comparison between older and younger
I.M. Tarkka1, D.S. Stokic2 healthy adults
Health Sciences, University of Jyvskyl, Finland,
1
C. Pradhan1, M. Whr1, K. Jahn2, R. Schniepp2
Mississippi Methodist Rehabilitation Center, Jackson, MS,
2 1Integrated Research and Treatment Center for Vertigo,
USA Oculomotor and Balance Disorders, IFB, LMU, University
Introduction: Our aim was to determine the role of the left of Munich, 2Integrated Research and Treatment Center for
dorsolateral prefrontal cortex (PFC) in facilitation in the Vertigo, Oculomotor and Balance Disorders, IFB, LMU, &
primary motor cortex (M1) of the muscle involved in motor Department of Neurology, University of Munich, Germany
imagery. We stimulated the left PFC during motor imagery Introduction: While gait analysis is a powerful non-
to induce a virtual lesion. invasive tool, its interpretation is often hindered by large
Methods: Transcranial magnetic stimulation to left M1 volumes of highly correlated data. We explore Principal
(round coil at Cz, 120% resting threshold, 8510% of Component Analysis (PCA) for dimension reduction and
maximal output) was used to elicit responses in the right linear discriminant analysis (LDA) to determine the relative
tibialis anterior (TA) muscle during relaxation and while power of extracted components in separating different
imagining right ankle dorsiflexion movements cued by a clinical groups.
visual display. A second stimulator (figure-of-eight coil at Methods: 224 discreet measurements using a GAITRite
F3, 7510%) was used to stimulate left PFC 300ms before sensor carpet were obtained from two groups of normal
M1 during relaxation and motor imagery. 5 healthy adults healthy adults (Group 1: 31-40 years (34.63.32), n=20;
(age 576 years, 4 males) were studied. Main outcome Group 2: 71-80 years (75.13.10), n=27). PCA was
measure was the amplitude of motor evoked potentials performed to determine significant components (PC) and
(MEPs) in the right TA during relaxation and motor imagery, loadings. Fishers LDA was used to determine the hierarchy
without and with left PFC stimulation (8-12 traces each). of discriminatory power among the components.
Results: Motor imagery alone significantly increased Results: 12 PCs explained 90% of the data variance with
MEPs in the right TA compared to relaxation (~400% on the 1st explaining 28.9%. Stepwise LDA indicated that 4
average). Stimulating the left PFC 300ms before the M1 PCs (PC3, PC4, PC6 and PC1 in order of discriminatory
significantly reduced the facilitatory effect of motor power) were optimal for the separation of the groups. Major
imagery by about one quarter (317%, p=0.0268). Paired left contributory measures to PC3 were Base of Support (BS)
PFC and M1 stimulation during relaxation, however, had no Left and Right (L&R) during self-selected, maximal-speed
appreciable effect on MEPs in TA (102%). and head-extended gait. In addition there were significant
Conclusion: The results indicate that left PFC is an integral contributions from BS (L&R), Stride Length (L&R) and
part of the network involved in motor imagery. Impaired Functional Ambulatory Profile (FAP) scores during dual-
output from the left PFC may hinder the potential to utilize task gait (verbal fluency and serial 7) as well as FAP scores
motor imagery for restoration of motor function after brain during eyes-closed gait.
injury. Conclusion: The most discriminatory gait features between
young and old healthy adults appear to be lateral
displacement variables. In addition dual-task and eyes-
closed gait appear to affect gait in elderly. PCA is useful for
gait data reduction and visualization and we intend to
explore similar methods in other clinical populations.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


218 Posters, Sunday 9 September

P1350 P1351
Quantitative EEG and cognitive Diagnosis of epilepsy by means of sleep-
impairment in Parkinsons disease deprived or melatonin-induced sleep EEG
L.C. Fonseca1, G.M. Tedrus1, P.N. Carvas2, G. Gustafsson, E. Svanborg
E.C. Machado3 Clinical Neurophysiology, Linkoping University Hospital,
1Neurology, 2Medicine - FAPIC/Reitoria, 3Medicine - Linoping, Sweden
FAPESP, Pontificia Universidade Catlica de Campinas, Recording EEG in children may be challenging, since
Campinas, Brazil reliable interpretation requires that the patient lies still.
Introduction: Cognitive impairment is frequent and Sleep during the recording is therefore desirable, and
sometimes precocious in Parkinsons disease (PD), but parents are often instructed to keep the patient awake for at
knowledge of its pathophysiology is still limited. least half the night before. However, sleep deprivation is not
Quantitative EEG has provided contributions to the theme easy to achieve in children. Therefore, melatonin is currently
and in the present study includes an approach to both used in a number of laboratories to induce sleep. This study
absolute power and coherence in different cognitive states was performed to determine 1) whether melatonin is equally
of PD. efficient in inducing sleep as sleep deprivation; and 2)
Methods: 46 patients with PD and 54 normal subjects were whether melatonin-induced sleep is as efficient in provoking
assessed using a neurological evaluation, CERAD epileptiform activity as sleep deprivation.
neuropsychological battery (consortium to establish a Method: 115 EEGs recorded between 2007 and 2008 after
registry for Alzheimers disease), Mini-mental Status sleep-deprivation and 130 EEGs recorded between 2010
Examination, executive functions examination and EEG. and 2011 after melatonin ingestion were studied. The
The mean global absolute powers at the various electrodes children in the two groups were matched for age (1-16 y.o)
for the delta, theta, alpha and beta bands were calculated, as and gender. Comparisons were made concerning how many
also the inter- and intra-hemispheric coherences. The theta/ children obtained sleep, acceptability of EEG recording and
delta global absolute ratio was also calculated. Four groups epileptiform activity.
were compared: PD with dementia (PD-D) - 12 patients; PD Results: 11 recordings in each group were not interpretable
with mild cognitive impairment (PD-MCI) - 12 patients; PD due to movement artifacts (n.s.). Sleep was obtained in 71%
with no cognitive disturbances (PD-NLCog) - 22, and the of recordings of children that got melatonin and in 60%
control group (p<0.05). sleep deprived children (n.s). Epileptiform activity was
Results: The global delta and theta absolute powers and the recorded in 34% of EEGs with melatonin-induced sleep and
beta frontal-occipital coherence were significantly greater in 39% of EEGs recorded after sleep deprivation (n.s.).
in the PD-D group than in the other groups, whereas the Conclusion: Melatonin had a small advantage over sleep-
theta/delta ratio was greater in the PD-D and PD-MCI deprivation in so far as sleep was obtained in a larger
groups than in the PD-NLCog and control groups (post-hoc number of subjects. It had no disadvantage concerning
analysis, ANOVA, p<0.001). ability to provoke epileptiform activity. Furthermore, the
Conclusions: Significant differences were identified procedure is much easier for the parents than to keep a
between the PD-D group and other groups and between the young child awake for half the night.
PD-MCI group and individuals with no cognitive
impairment using EEG absolute power and coherence. Our
findings suggest qEEG as a possible physiological tool in
the assessment of cognitive aspects in PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 219

P1352 P1353
Polysomnographic (PSG) recordings and Feasibility of a new multichannel surface
clinical evaluation in patients with electrode to detect neuropathy in diabetic
prolonged disorders of consciousness patients with normal results in
(DOCs) conventional nerve conduction studies
M.R. Valente, S. de Biase, F. Basaldella, M. Fuccaro, B.J. Kim1, K.Y. Jung1, Y.S. Koo1,2, J.H. Jang1, S.H. Lee3,
S. Lorenzut, G.L. Gigli K.W. Park1
Neurology Clinic, Sleep Disorder Center, University of 1Neurology, Korea University College of Medicine, Seoul,
Udine, Italy 2Jeju Medical Center of Jeju Special Self-Governing
Introduction: The aim of the study is to evaluate the Province, Jeju, 3Biomedical Engineering, Korea University
importance of sleep recordings in patients with prolonged College of Health Science, Seoul, Republic of Korea
DOCs looking at possible correlations between clinical Introduction: To increase sensitivity for detecting early
evaluation, sleep patterns and evoked potentials. At our neuropathy in patients with diabetes, we have recently
knowledge, studies evaluating these methods altogether in developed a multichannel surface electrode. This study was
patients with prolonged disorders of consciousness are performed to investigate reliability of the multichannel
lacking, at least with current scales. (8-electrodes) surface electrode in detecting neuropathy in
Materials and methods: 12 patients, 10 in vegetative state diabetic patients, but with normal results from the
(VS) and 2 in minimally conscious state (MCS) were conventional nerve conduction study (NCS).
evaluated from a clinical and neurophysiological point of Methods: Age and sex matched normal healthy subjects (24
view. Clinical evaluation included Coma Recovery Scale- men, 32 women; mean age, 58.71 yr) and diabetic patients
Revised (CRS-R), Disability Rating Scale (DRS) and with distal symmetric sensory symptoms (12 men, 12
Glasgow Coma Scale (GCS). Neurophysiological evaluation women; mean age, 59.92 yrs) who were normal at
included 24h PSG, SSEP, BAEP, VEP, P300 and MMN. conventional NCS were recruited. Eight compound muscle
Results: In 4 patients 24h PSG showed the presence of a action potentials (CMAPs) were recorded from the abductor
clear sleep-wake alternation, different stages of sleep, pollicis brevis using the electrode by stimulation of the
REM-NREM ciclicity, sleep spindles and K-complexes. median nerve at the wrist. Various latency and amplitude
These patients had a CRS-R score of 7.251.71 while 8 related variables including minimum and maximum
patients, lacking one or more of these sleep elements, had a latencies were obtained and analyzed to compare these
CRS-R score of 3.380.91 (p<0,05). Patients with each variables between the two groups using independent t-test.
single sleep element (such as REM sleep) had always a We also used multiple logistic-regression analysis to find
better clinical evaluation compared with those who had not. out which variables can discriminate control and diabetic
Patients with all sleep elements and preserved BAEPs had groups.
a better clinical evaluation, compared with patients lacking Results: All variables related to latencies in the diabetic
at least one of them. No correlation was found between patients group, especially standard deviation of CMAP
presence of SSEPs and better clinical evaluation. onset latencies in each subject (SDLat), showed significant
Conclusions: PSG recordings resulted as the best difference from control group (P<0.001). However, all
neurophysiological tool for assessment of patients with amplitude-related-variables did not show any differences.
prolonged DOCs and may be considered a non-expensive, Among all variables, minimum latency and the SDLat were
non-invasive and easy to perform examination able to give the most reliable variables to discriminate diabetic patients
a suitable support to clinical evaluation. from normal subjects.
Conclusions: The multichannel surface electrode is a
reliable tool to detect neuropathy in patients with diabetes
who did not have abnormality in conventional NCS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


220 Posters, Sunday 9 September

P1354 P1355
Vagus somatosensory evoked potentials Assessing the progress of mild cognitive
(VSEP) in subjects with multiple sclerosis impairment with the use of an ERP
T. Polak1, F. Metzger2, D. Zeller3, A.J. Fallgatter4 electrophysiological biomarker
1Dept. of Psychiatry, Psychosomatics and Psychotherapy,
V. Papaliagkas
University Clinic Wrzburg, 2Clinic of Psychiatry and 3rd Department of Neurology and Department of Physiology,
Psychotherapy, University Clinic Tbingen, 3Department of Aristotle University of Thessaloniki, Greece
Neurology, University Clinic Wrzburg, 4Department of
Aim: To study changes in auditory event related potentials
Psychiatry and Psychotherapy, University Clinic Tbingen,
Germany
(AERP) and their correlation with the memory status of
mild cognitive impairment (MCI) patients
Introduction: Vagus somatosensory evoked potentials Materials and methods: AERPs were recorded in 22 MCI
(VSEP) were proposed as an electrophysiological patients (meanSD age = 67.47.8, median (interquartile
contribution to the diagnosis of Alzheimers dementia (AD) range-IQR) MMSE score = 28 (27-29) in three consecutive
and Parkinsons disease (PD) where prolongation of exams, and in 30 age-matched controls at baseline. During
latencies as compared to healthy controls was explained by this time period, 3 patients progressed to Alzheimers
the disease specific degeneration of brainstem nuclei. We disease (AD). Latencies and amplitudes of the late waves
now searched for an independent confirmation of this (N2, P3 and Slow Wave) and N2-P3 peak-to-peak
hypothesis by examining VSEP in a disease where brainstem amplitudes and latencies were measured, as well as
affection may occur but which is different from the correlation coefficients (CC) between them and MMSE
neurodegenerative process in AD and PD. scores.
Method: We assessed VSEP in 15 subjects with multiple Results: Between exams, a significant increase in P3
sclerosis (37.515.1 years, 5 male; EDSS median=4,0 latency and a decrease in N2 amplitude were observed.
[range 0-7]) with brainstem affection in 8 cases and in 15 Only N2 latency correlated with baseline MMSE scores,
matched healthy controls. The auricular branch of the vagus whereas P3 and Slow Wave latencies correlated with age.
nerve was stimulated at the tragus applying electrical square Conclusions: A new index that incorporates changes in N2
impulses (0.1msec duration, interstimulus interval 2sec, and P3 latencies and amplitudes into a single
stimulus intensity 8mA). Evoked potentials were recorded electrophysiological marker is proposed in order to describe
bipolarly from the electrode positions C4-F4 and Fz-F4 with high accuracy the gradual progress of MCI and its AD
(band-pass 0.1Hz to 1kHz, analysis time 10msec, averaging conversion.
of 100 artefact-free epochs).
Results: ANOVAs for repeated measurements showed
significant main effects of the factor diagnosis only for
stimulation on the right side with significant longer
latencies in patients as compared to healthy controls for
latencies P1 (p0.005) and N1 (p0.026).
Conclusions: These results further support our view of
VSEP being far field potentials generated in brainstem
nuclei and thus point to VSEP as a possible tool for the
detection of brain stem pathology in neurodegenerative as
well as inflammatory diseases.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 221

P1356 P1357
Modulatory effects on human Acute motor axonal neuropathy:
sensorimotor cortex activation by whole reversible axonal conduction block or
hand vibration stimulation axonal degeneration?
A.B. Kunz1, M. Christova2, E. Gallasch2, M. Seidl1,3, Z. Arnyi1, A. brahm2
J. Bergmann4,5, Y. Krenn1, R. Nardone3,6, E. Trinka1,3,4, 1Dept. of Neurology, Semmelweis University, 2Dept. of

S.M. Golaszewski1,3,4 Neuroinfectology, St. Lszl and Istvn Hospital, Budapest,


1Department of Neurology, Paracelsus Medical University Hungary
Salzburg, 2Institute of Physiology, Medical University Graz, Objective: Acute motor axonal neuropathy (AMAN) is a
3Spinal Cord Injury and Tissue Regeneration Center,
variant of Guillain-Barr syndrome. AMAN apparently has
4Neuroscience Institute, Paracelsus Medical University
two distinct forms: In the Far-East, AMAN is a common
Salzburg, 5Institute of Psychology, Paris Lodron University, disease showing quick recovery, whereas in Europe and
Salzburg, Austria, 6Department of Neurology, F. Tappeiner North-America AMAN is rare and characterized by
Hospital, Merano, Italy complete denervation. We present 3 Hungarian patients, and
Objective: To investigate the effect of stimulation of the discuss the clinical and electrophysiological differences
nerve afferents of the hand on cortical activity elicited by between the two forms of AMAN.
whole-hand vibration (WHV) stimulation for sensation in Patients: Patient 1 and Patient 2 (32- and 61-year-old
healthy human subjects. males) acquired their disease in China and Thailand,
Methods: WHV stimulation was performed in 12 healthy respectively. Both patients developed generalized weakness,
subjects in separate sessions at different stimulation levels: with elicitable reflexes and no sensory symptoms.
sub-sensory at 50Hz, sensory at 50Hz and motor at 2Hz. To Electrophysiological assessment showed low amplitude
verify if stimulation at lower frequencies is less effective, an motor responses, with normal sensory potentials. Both
additional experiment at sensory level with 2Hz was patients improved within weeks. Follow-up examination of
performed. TMS was used to assess motor threshold (MT), Patient 1 showed complete restitution of motor responses
motor evoked potentials (MEP) recruitment curve (RC), after 2 months. Patient 3 is a 25-year-old male who
short latency intracortical inhibition (SICI) and intracortical developed tetraplegia, areflexia, and respiratory
facilitation (ICF) to paired-pulse TMS at baseline (T0), insufficiency within 2 days in Hungary. The first
immediately after (T1) and one hour (T2) after 30 minutes electrophysiological examination was performed before
of WHV stimulation. F-wave studies were performed to Wallerian degeneration had taken place, which showed
assess spinal motoneuron excitability. preserved motor responses with multiple amplitude
Results: WHV stimulation at sub-sensory/50Hz and reductions on intermediate nerve segments, and normal
sensory/2Hz level determines no significant cortical sensory responses. Follow-up examination showed complete
excitability changes; at sensory/50Hz level and at motor/2Hz denervation of all limb muscles.
level we found decreased MT, increased MEP RC as well as Conclusion: It is postulated that patients 1 and 2 with the
reduced SICI and increased ICF at T1 and T2. Far-Eastern form of AMAN had reversible axonal blocks,
Conclusions: WHV stimulation at sensory/50Hz and as opposed to the initial axonal blocks progressing to axonal
motor/2Hz level induces similar long-lasting modulatory destruction seen in the European form of AMAN in patient
effects on motor cortical excitability. Both, the strength of 3. In both forms, the immune response is elicited by
the corticospinal projections and the intracortical networks Campylobacter jejuni and directed against the axolemma,
are influenced to the same extend. The study provides but there appears to be a quantitative and/or qualitative
further evidence that stimulation intensity and frequency difference, perhaps related to geographical differences in
can independently modulate motor cortical plasticity. The Campylobacter jejuni.
selection of optimal stimulation parameters has potentially
important implications for the neurorehabilitation of
patients after brain damage (e.g. stroke, traumatic brain
injury) with hand motor deficits.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


222 Posters, Sunday 9 September

P1358 P1359
Ocular vestibular evoked myogenic Peripheral nerve function following
potentials in patients with multiple 24-week treatment with tanezumab,
sclerosis a nerve growth factor (NGF) inhibitor
E. Gozke, Z. Ayhan, N. Erdal, N. Gurbuzer, M. Kaydan M.T. Brown1, W.J. Litchy2, D.N. Herrmann3,
Department of Neurology, FSM Teaching and Research M. Goldstein4, A.M. Burr1, M.D. Smith1, C.R. West1,
Hospital, Istanbul, Turkey K.M. Verburg1, P.J. Dyck2
Introduction: Vestibular evoked myogenic potential 1Pfizer, Inc., Groton, CT, 2Mayo Clinic, Rochester, MN,
(VEMP) test is a technique based on activation of the
3University of Rochester, Rochester, NY, 4Medical Specialists
vestibular apparatus by acoustic stimulation. In this study of the Palm Beaches, Inc., Atlantis, FL, USA
we aimed to investigate the presence of vestibulopathy in Introduction: The reported cutaneous sensory symptoms
patients with relapsing remitting multiple sclerosis (RRMS) and novel analgesic mechanism of action of tanezumab, a
who have not vestibular complaints using ocular VEMP monoclonal antibody NGF-specific inhibitor, raises
(oVEMP) test. potential concerns regarding nerve function and safety.
Methods: 40 patients diagnosed as RRMS according to Mc Methods: This randomized, double-blind, placebo-
Donalds criteria and 30 healthy controls were enrolled in controlled study (NCT00863772) of neurologically normal
the study. oVEMP tests were performed through patients with knee or hip osteoarthritis investigated whether
contralateral inferior oblique muscles. Active electrode was tanezumab (3 administrations of 5 or 10mg IVq8w for 24
placed on the lower eyelid, and the reference electrode was weeks) caused clinically significant changes from baseline
attached 1cm below. The patients were requested to stare (cfBL) relative to placebo in a composite measure of
towards a superolateral direction so as to activate inferior peroneal, sural, tibial and autonomic nerve function
oblique muscle. Meanwhile 120dB click stimulation was (5NC+HRdb) or in intraepidermal nerve fibre density
delivered to the contralateral ear at 10/sec for 150 sec, and (IENFD), assessed by distal leg skin biopsy. Efficacy
1500 responses were averaged. The test was repeated twice (WOMAC pain) at week 16 was also assessed.
for each side and the latencies and amplitudes of the initial Results: Due to an FDA-mandated clinical hold, only 219
negative (N1) and positive (P1) waves were measured. of 369 planned patients were treated. Last observation
Availability of any response, their latencies and amplitudes carried forward least squares mean and standard error (LSM
were compared. (SE)) cfBL in 5NC+HRdb scores and IEFND to week 24,
Results: 9 cases of RRMS (22.5%) could not yield any and WOMAC Pain cfBL to week 16 are shown.
response in both sides, while in the control group no
bilateral response was obtained in 4 cases (13.3%). Also in Assessment Placebo Tanezumab 5 mg Tanezumab 10
the RRMS group no unilateral response was obtained in 5 (LSM (SE)) mg
(12.5%) cases. In the RRMS group mean latencies of N1
5NC+HRdb -0.11 0.17 (0.30) -0.06 (0.30)
and P1 were significantly longer, while N1-P1 amplitudes (0.29)
were found to be meaningfully lower.
IENFD -0.43 0.18 (0.57) -1.12 (0.57)
Conclusion: These findings demonstrate that by using (0.57)
oVEMP test subclinical vestibulopathy can be elicited in
RRMS patients without vestibular complaints. WOMAC Pain -1.31 -2.17 (0.28) -2.48 (0.28)
(0.28)

Conclusions: The magnitude of cfBL in 5NC+HRdb and


IENFD was small and unrelated to dose; treatment
differences versus placebo were not statistically significant.
Patients reported significant pain relief with tanezumab 5
and 10mg versus placebo treatment (=-0.86, p=0.024 and
=-1.16, p=0.002, respectively). Evidence linking
tanezumab to detrimental (neurotoxic) effects on the
peripheral or autonomic nervous system was not found.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 223

P1360 P1361
Long-term changes of visual evoked EEG in dementia with Lewy bodies: a
potentials in children with insulin- diagnostic tool?
dependent diabetes mellitus Y. Hizem1,2, M. Ben Djebara1,2, I. Abdelkefi1,2,
S.-S. Lee1, H.-S. Han2, H. Kim3, J.J. Lee4 I. Kacem1,2, A. Gargouri-Berrechid1,2, R. Gouider1,2
1Neurology, 2Pediatrics, 3Preventive Medicine, College of 1Department of Neurology, Razi Hospital, La Manouba,
Medicine, Chungbuk National University, Cheongju-si, 2Research Unit of Clinical Neurophysiology and
4Premedical Course, College of Natural Science, Seoul Electrodiagnosis 03/UR/08-09, Tunis, Tunisia
National University, Seoul, Republic of Korea Introduction: Frontal intermittent rhythmic delta activity
Objectives: To investigate the changes of central nerve (FIRDA) is an abnormal EEG pattern found in primary and
conduction in children with insulin-dependent diabetes secondary involvement of the brain in a wide variety of
mellitus (IDDM) prospectively using the visual evoked diseases. The occurrence of FIRDA in degenerative
potentials (VEP) and to know how those results were related dementia could be considered as an indicator supporting the
to clinical risk factors and parameters of peripheral nerve diagnosis of dementia with Lewy Bodies (DLB).
conduction studies (NCS). Objective: To investigate the correlation between FIRDA
Methods: A total of 76 patients (29 males and 47 females) and DLB in comparison with Alzheimers disease (AD).
aged 5-26 years (mean 14.44.8) with IDDM underwent Methods: 88 patients with dementia were ascertained: 44
visual evoked potentials and bilateral NCS of median, ulnar, DLB and 44 AD, age and sex matched. Diagnoses were
posterior tibial, peroneal, and sural nerves annually for 5 established according to international criteria. EEGs were
years. The control group was made up of 52 healthy performed on 47 patients as part of a routine dementia
children. assessment using standard techniques. Recordings were
Results: Out of 76 patients, 25 patients completed annual examined blind to diagnosis following these criteria:
studies for 5 years. Significant positive correlations were dominant frequency, presence of other frequencies,
found between the latency of P100 wave in the VEP and the asymmetry, mean amplitude and presence of focal
level of serum glycosylated haemoglobin. Latency and abnormalities including spikes, sharp waves, triphasic
amplitude values of P100 waves decreased progressively in waves or transient slow wave activity.
children with IDDM as the duration of the disease increased. Results: A total of 18 records from the DLB group and 29
The values of latency and amplitude were inversely related records from the AD group were examined. 8 of 18 patients
with the age of patients and duration of the disease. The (44.5%) with DLB showed a main pattern of FIRDA. In AD
values of latency of P100 were not related with parameters group, FIRDA occurred in 10 patients with severe dementia
of NCS. However, some of the parameters of NCS were (34.5%). The difference between the two groups was
weakly related with the amplitudes of p100. significant (p: 0.03).
Conclusions: Poor glycaemic control proved to be an Conclusion: We found a significant correlation between
important risk factor over 5 years as related to the FIRDA and DLB. Our results are in concordance with
development of subclinical central neural pathway recent findings in literature. This pattern when found in
abnormality. VEP could be considered as a valid non- early stages, could be helpful for diagnosis, however this
invasive tool for detecting an early diabetic central pattern could not be considered specific for DLB.
conduction abnormality such as retinopathy or optic
neuropathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


224 Posters, Sunday 9 September

P1362 P1363
Potential mechanisms underlying the HRAS-dependent perturbation of iTBS
neural effects of TDCS in post-stroke after-effects: a lesson from Costello
recovery disease
E.M. Khedr1, O.A. Shawky1,2, A. Tohamy1, J. Rothwell3 M. Dileone1,2, P. Profice1, F. Ranieri1, F. Pilato1,
1Assiut University Hospital, Assan University Hospital,
2
F. Capone1, L. Florio1, R. Di Iorio1, G. Musumeci1,
Assiut, Egypt, 3National Hospital for Neurology and C. Leoni1, M. Tartaglia3, G. Zampino1, V. Di Lazzaro1
Neurosurgery, London, UK 1Catholic University of Rome, 2San Bortolo Hospital,

The aim of this study was to investigate potential Vicenza, 3Istituto Superiore di Sanit, Roma, Italy
mechanisms underlying the neural effects of TDCS in post Introduction: Costello syndrome (CS) is a rare congenital
stroke recovery. disorder due to a G12S amino acid substitution in HRAS
Subjects and methods: 36 patients with subacute ischemic protoncogene and several lines of evidence suggest a key
stroke participated in the study. The patients were randomly role of HRAS in Long Term Potentiation (LTP). Paired
assigned to one of three groups; the anodal and cathodal Associative Stimulation (PAS) leads to an extremely
groups received real tDCS at an intensity of 2mA for 25 pronounced motor cortex excitability increase in CS
minutes daily for 6 consecutive days. The sham group patients. Intermittent Theta Burst Stimulation (iTBS) is
received sham stimulation for the same duration. Patients another facilitatory repetitive Transcranial Magnetic
were assessed with the Barthel index (BI) and the Medical Stimulation (rTMS) protocol that in normal subjects induces
Research Council (MRC) muscle strength scale at baseline, a similar increase in motor cortex excitability. For this
and after end of the sessions. Cortical excitability and reason, we evaluated the after-effects induced by iTBS in
transcallosal inhibition (TCI) were measured at baseline and CS and compared them with those induced by PAS.
after sessions for both hemispheres. Methods: In two different session we evaluated iTBS and
Results: In all groups, resting and active motor thresholds PAS after effects in 4 CS patients and a group of 17 age
decreased in the affected hemisphere but were unchanged in matched healthy controls. All CS patients underwent clinical
the unaffected hemisphere; the changes were more evaluation for dystonia.
significant after real tDCS than sham. The slope of the Results: PAS protocol led to a 250% increase of the FDI
input-output curve was steeper after real tDCS (anodal, MEP amplitude in CS patients: surprisingly iTBS induced
p=0.0001 and cathodal p=0.01) with no significant changes no change in MEP amplitude in CS patients whereas led to
in the sham group. There was a significant decrease in the an increase of about 50% in healthy subjects. Nevertheless
duration of TCI (pre-post tDCS) in the affected hemisphere all CS patients showed mild to moderate generalized
of all groups (p=0.0001); in the unaffected hemisphere the dystonia.
duration decreased only after cathodal stimulation (p=0.01). Conclusions: CS patients are characterized by dystonia
There was a significant correlation between improvement in and, moreover, by a concomitant enhanced PAS-induced
Hand grip or the BI and changes in cortical excitability. after-effect and an impairment of iTBS-related LTP-like
Conclusion: Both types of real tDCS increased cortical phenomena. The last finding seems to suggest that HRAS-
excitability of the affected hemisphere and these changes dependent signalling pathways could differently affect
correlated with the improvement in motor function. homotopic and heterotopic brain plasticity in humans.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 225

P1364 P1365
Motor cortex excitability in patients with Ocular vestibular evoked myogenic
vascular cognitive impairment-no potentials in evaluation of patients with
dementia: a follow-up study multiple sclerosis
M. Cantone1, G. Lanza1, M. Pennisi2, V. Puglisi1, T. Gabelic1, M. Krbot2, I. Adamec2, M. Habek2,3
L. Vinciguerra1, R. Ricceri1, G. Pennisi1, R. Bella1, Neurology, 2University Hospital Centre Zagreb, 3School of
1

M. Zappia1 Medicine, University of Zagreb, Croatia


1 Department GF Ingrassia, Section of Neurosciences, Objectives: We investigated ocular vestibular evoked
2 Department of Chemistry, University of Catania, Italy myogenic potentials (oVEMP) as supportive diagnostic toll
Introduction: Up to now only few studies have examined in assessing lesions of vestibulo-ocular pathways in patients
cortex excitability in patients with cognitive decline with relapsing remitting multiple sclerosis (RRMS).
following vascular brain damage. Recently, a transcranial Patients and methods: 60 patients treated at the Referral
magnetic stimulation (TMS) study showed an enhanced Centre for Demyelinating Diseases with established
motor cortex facilitation even though the absence of diagnosis of relapsing remitting multiple sclerosis were
changes in resting motor threshold (rMT), in patients with included in the study. The control group consisted of 16
vascular cognitive impairment-no dementia (VCI-ND). healthy subjects. oVEMP in response to acoustic clicks of
Aim: To evaluate neuropsychological and neuro 1ms duration at the intensity of 130dB SPL and the
physiological outcome in VCI-ND patients. stimulation frequency of 1Hz were studied. Signals were
Methods: 8 VCI-ND patients (mean age 72.46.4 years) divided in segments of 120ms duration (20ms before the
underwent to neuropsychological assessment and to single stimulus and 100ms after the stimulus) and averaged.
and paired-pulse TMS study at baseline and after two years. Latencies and amplitude of N10 and P13 waves of oVEMP
Resting motor threshold (rMT), cortical silent period, short were evaluated.
intracortical inhibition and intracortical facilitation were Results: Statistically significant prolongation of latencies
recorded bilaterally. was found in the group of RRMS patients (p<0.05) for N10
Results: After the follow-up period (22.13.5 months) response on the right and left side. There was also latency
patients exhibited a significant worsening of Stroop prolongation for P13 wave on right side. Statistical
T-scores (43.4 sec16.78 vs. 66.9 sec30.98; p<0.05) and a significance was not reached for left P13 latency. The
significant reduction in rMT over both hemispheres (left: difference in the amplitude between groups was not
47.3%9.88 vs. 40.9%10.67; right: 41.9%4.88 vs. observed. Conduction block was indentified in 18 patients
38.4%7.11; p<0.05). (30%).
Conclusion: After two years, VCI-ND patients showed an Conclusion: Ocular vestibular evoked myogenic potentials
overall trend towards a motor cortex hyperexcitability, are useful neurophysiological methods in evaluation of
although the mean rMT value was still within normal limits. brainstem lesions in patients with relapsing remitting
Previous studies reported that cortical excitability is stably multiple sclerosis.
affected in dementing process. In this study, patients with a
significant decrease of rMT exhibited a worse performance
in cognitive evaluation even without an overt dementia,
suggesting a correlation between neuropsychological and
neurophysiological data. TMS is valuable tool in the
monitoring and early detection of neurophysiological
changes in patients at risk for vascular dementia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


226 Posters, Sunday 9 September

P1366 P1367
Prognostic value of neuron-specific Acute hyperoxia and cerebrovascular
enolase level in patients with Parkinsons reactivity in diabetic patients with acute
disease stroke: a pilot study
Y. Madjidova, S. Fakhargalieva I. Hegedus1, I. Drenjancevic2, S. Butkovi-Soldo3,
Tashkent Medical Academy, Tashkent, Uzbekistan A. Cosic2
Aim: To study the role of neuron-specific enolase in the
1Neurology, University Hospital in Osijek, 2Physiology and
forecast of Parkinsons disease (PD). Immunology, 3Neurology, Faculty of Medicine University
Materials and methods: We examined 35 patients, 17 Josip Juraj Strossmayer Osijek, Croatia
(48.57%) women and 18 (51.42%) men, average age - Introduction: Impaired cerebral vascular reactivity has
(7310.3) with PD. Patients were divided into 3 subgroups: been described in hypertension and diabetes mellitus. Since
with trembling-rigidity, trembling, and akinetic-rigidity stroke is of vascular etiology, the aim of this study was to
form. NSE level was defined in the serum of patients with determine the effects of acute hyperoxia stimulation on
the method of immune-enzyme analysis which was made cerebrovascular reactivity in diabetic patients with acute
with specific test systems, on "LISA" analyzer according stroke.
to the instruction applied in a set. Methods: In this pilot study, 12 diabetic patients (DM
Results: In 24 (68.5%) from 35 patients, NSE level was group) (meanSD age 656 yrs) with acute ischemic stroke
elevated (>25ng/ml). Elevated NSE level was marked in (within 72 hours) and 12 healthy subjects (613 yrs) were
58.3% of men and 41.7% of women. From 3 subgroups only included. Blood flow velocity (BFV) of middle cerebral
in patients with the trembling form of PD fluctuation of artery (MCA), index of pulsality (IP) and index of resistance
NSE level was within the norm (19-24.73ng/ml). More (IR) were determined by transcranial Doppler before,
often elevated NSE level was defined in the early period of during 15 minutes, and after 15 minutes of acute hyperoxia
the disease. Depending on the rate of parkinsonism (inhalation of 100% O2 over facial mask). Tromboxane B2
progressing, it was revealed that more elevated NSE levels (TXB2) levels in blood of both groups were measured by
were marked in patients with fast rate of disease progressing, ELISA assay before and during acute hyperoxia.
than in patients with slow and moderate rate. Results: The results showed paradoxical increase in cerebral
Conclusions: Elevated NSE level was marked in 69% cases BFV in both brain hemispheres during hyperoxia in the DM
of patients with parkinsonism. Elevated NSE level met in group compared to controls, accompanied by decrease of
patients with the trembling-rigidity and akinetic-rigidity TXB2 during hyperoxia in the DM group and increase of
form of PD, and was more elevated in patients with fast rate TXB2 in controls during hyperoxia. Controls exhibit
of disease progressing, also at early stages of the disease. vasoconstriction in response to hyperoxia. PI and RI in the
NSE level can be used as an additional methods of DM group decreased during hyperoxia compared to their
diagnostics and the forecast of neurodestructive brain basal values and compared to controls (while PI and RI of
processes, including PD. controls increased compared to basal conditions).
Conclusion: This study demonstrated paradoxical MCA
vasodilation, accompanied by decrease in TXB2 levels in
DM patients with stroke compared to healthy controls,
suggesting the role of cyclooxygenase metabolites of
arachidonic acid mediating the change in cerebral BFV and
vasoreactivity in response to hyperoxia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 227

P1368 P1369
Effects of simultaneous bilateral tDCS of Endothelial dysfunction in two patients
the human motor cortex with posterior reversible encephalopathy
L. Mordillo Mateos1, L. Turpin Fenoll2, syndrome
J. Milln Pascual2, A. Oliviero1 M. Saadatnia, V. Davoudi, K. Keyhanian, S. Haghjoo
1FENNSI, Hospital Nacional de Parapljicos, Toledo, Javnmard, M. Mirmohammad Sadeghi, F. Haghdoost
2Servicio de Neurologia, Hospital La Mancha Centro,
Isfahan University of Medical Sciences, Isfahan, Iran
Alcazar de San Juan, Spain
Background: We report two patients, one with eclampsia
Introduction: tDCS is a non-invasive technique investigated and another one with Systemic Lupus Erythematosus
as a therapeutic tool for different neurologic disorders. presenting posterior reversible encephalopathy. We tried to
Neuronal excitability can be modied by application of DC find out whether endothelial cell dysfunction was present in
in a polarity-specic manner: anodal tDCS increases these two patients.
excitability, while cathodal tDCS decreases excitability. Case1: A 22-year-old woman who was pregnant and has
Objective: To determine whether simultaneous bilateral been diagnosed to have HELLP syndrome was admitted to
tDCS is able to increase cortical excitability in one Al-zzahra hospital. The day after caesarean section, she had
hemisphere whereas cortical excitability in the contralateral experienced two Generalized Tonic Colonic Seizure attacks
hemisphere decreases. Cortical excitability before and after with a consequent long time loss of consciousness. In brain
bilateral motor cortex tDCS was evaluated and compared Magnetic Resonance Imaging (MRI) scan, ischemic lesions
with unilateral motor cortex tDCS. were found in parietal lobes and basal ganglia. Serum level
Methods: Cortical excitability was evaluated before and of two adhesion molecules, ICAM-1 & VCAM-1 was
after unilateral or bilateral tDCS using transcranial magnetic obtained and they were 274.5 & 102.5, respectively.
stimulation. Case2: A 16-year-old girl with Systemic Lupus
Results: Simultaneous application of anodal tDCS over the Erythematosus was admitted to Al-zzhra Hospital while she
motor cortex and cathodal tDCS over the contralateral one was confused and had experienced five episodes of
induces an increase in cortical excitability on the anodal- Generalized Tonic Clonic Seizures. Cerebral vasospasm has
stimulated side and a decrease in the cathodal stimulated been recorded through Magnetic Resonance Angiography
side. The simultaneous bilateral tDCS induced similar (MRA). Serum level of ICAM-1 and VCAM-1 were found
effects to the unilateral montage on the cathode-stimulated to be 329.3 and 75.7, respectively.
side. On the anodal side, the simultaneous bilateral tDCS Conclusion: We found high amount of ICAM-1 and
seems to be a slightly less robust electrode arrangement VCAM-1 in serum of two patients with posterior reversible
compared with the unilateral montage. Intersubject encephalopathy syndrome. These adhesion molecules were
variability of the excitability changes induced by the anodal found under different circumstances within circulating
motor cortex tDCS using the bilateral montage was lower blood reflecting the endothelial damage.
than that with the unilateral montage.
Conclusions: This is the rst study in which cortical
excitability before and after bilateral motor cortex tDCS
was extensively evaluated, and the effects of bilateral tDCS
were compared with unilateral motor cortex tDCS.
Simultaneous bilateral tDCS seems to be a useful tool to
obtain increases in cortical excitability of one hemisphere
whereas causing decreases of cortical excitability in the
contralateral hemisphere (e.g., to treat stroke).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


228 Posters, Sunday 9 September

P1370 P1371
Relation between electrophysiological Small fibre conduction velocity (CV) by
findings and diffusion weighted magnetic contact heat evoked potential stimulation
resonance imaging in ulnar neuropathy in (CHEPS) is faster in peripheral than in
the elbow central spinothalamic pathways
Y. Altun1, S. Aygun2, M.U. Cevik3, A. Acar3, S. Varol3, B.E. Smith1, M.A. Ross1, B.P. Goodman1, E.P. Bosch1,
A. Arkanoglu3, H. Onder4, E. Uzar3 L.K. Jones Jr.2
Neurology, 2Radiology, Siirt State Hospital, Siirt,
1 1Neurology, Mayo Clinic, Scottsdale, AZ, 2Neurology, Mayo

Neurology, 4Radiology, Faculty of Medicine, Dicle


3 Clinic, Rochester, MN, USA
University, Diyarbakir, Turkey Introduction: Nerve conduction studies (NCS) assess only
Diffusion weighted imaging (DWI) provides information large diameter myelinated fibres. Data regarding small
about tissue integration by reflecting the random movement diameter fibre CV are sparse. CHEPS provides objective
of water molecules within the tissue. There is a limited measures of A delta fibre pathway CV from skin to cortex.
number of studies regarding DWI and conventional Methods: To assess CHEPS small (A delta) fibre CV in the
magnetic resonance imaging (MRI) in patients with ulnar periphery vs. in the CNS 13 neurologically normal controls
neuropathy in the elbow (UNE). In our study, we aimed to were recruited over 18-24 months with IRB approval. After
investigate the diagnostic value of non-invasive DWI consenting, all subjects underwent evaluations including
technique in patients with UNE. 26 elbows in 19 healthy CHEPS of limb and spine (L1 and C7) sites. Small fibre CV
controls (range 22-56 years) without any symptoms and 24 values were calculated between skin stimulation sites and
elbows in 21 patients (range 21-46 years) with cubital scalp recording electrodes.
tunnel syndrome underwent DWI. The electrophysiological Results: CV values from distal limb sites showed means of
and clinical criteria for the diagnosis of UNE were 3.7 m/s (foot) and 2.1 m/s (hand) while spine CV values had
performed. No signal from the ulnar nerve was detected in mean values of 1.5 m/s (L1) and 0.7 m/s (C7). CV figures
the healthy control group. We determined signal increase of for proximal limb sites (leg and forearm) were intermediate.
the ulnar nerve by DWI in all patients with UNE. T2 Conclusions: 1) Small (A delta) fibre CV values are higher
weighted (T2W) image showed increased signal intensity in from the distal limb than from corresponding spinal sites, 2)
20 elbows, while low signal intensity was observed in 4 since the vast majority of the conduction pathway between
elbows. A positive correlation was found between disease spine and cerebral cortex is central and the peripheral extent
duration and presence of hyperintensity (p=0.044, r=0.42) of this pathway is longer in peripheral than central segments
on T2W images. In addition, a positive correlation was also stimulating distal limb sites, it may be hypothesized that
found between electrophysiological severity scale score and peripheral small (A delta) fibre CV is greater than central
the largest nerve fibre diameter (p=0.017, r=0.48) and small (A delta) fibre CV, and 3) possible explanations may
cross-sectional area (p=0.017, r=0.48). In conclusion, DWI include: greater peripheral axon diameter, greater peripheral
might be used together with electrophysiological methods myelin thickness, and perhaps other differing physiologic
in diagnosis of UNE. Furthermore, DWI can be preferred in properties between Schwann cells and oligodendrocytes
some cases since it is a non-invasive method compared to such as sodium channel density or kinetics, PNS vs. CNS
electrophysiological methods in the diagnosis of UNE. axonal membrane properties, and central inhibitory
mechanisms.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 229

P1372 P1373
Can the Mismatch negativity (MMN) be Stimulus intensity and masseter volume
found in the nucleus subthalamicus? conduction on nerve conduction study in
E. Minks1, P. Jurak2,3, J. Halamek2,3, J. Chladek2, facial palsy
M. Bares1,4 S.J. Oh
1First Department of Neurology, St. Annes University Catholic University of Korea, Uijeongbu St. Marys Hospital,
Hospital and School of Medicine, Masaryk University, Uijeongbu, Republic of Korea
2Institute of Scientific Instruments of the ASCR, v.v.i., 3ICRC
Objectives: Electroneurography (ENoG) is used to
- International Clinical Research Center, St. Annes
determine the prognosis and the outcome in the acute phase
University Hospital, 4CEITEC - Central European Institute
of Bells palsy. However, we have experienced that inter-
of Technology, Brno, Czech Republic
rater and test-retest reproducibilities are relatively low. One
Introduction: In the protocol of mismatch negativity of the reasons is masseter volume conduction. This study is
(MMN) the frequent and target auditory stimuli are designed to obtain pure compound motor action potential
presented to the patient while unconcious. These evoked (CMAP) of the facial nerve without activating the masseter
potentials (EP) are considered to correlate with automatic muscle.
working memory and a primitive intelligence on an Methods: 13 patients with facial palsy were enrolled.
unconscious level. The aim of the study was to elucidate CMAPs were measured by using the 2 channel recording
whether nucleus subthalamicus (STN) is involved in the method, locating the surface electrode on nasalis muscle
processing of audio signals. and a concentric needle in the masseter muscle.
Methods: The EEG signal was recorded from STN during Supramaximal stimulations were done using stimulating
MMN protocol in 5 patients with Parkinsons disease with intensities of 20 to 60mA (sometimes over 60mA) at
9 established intracerebral electrodes. EP and event related intervals of 5 or 10mA. Stimulation intensities at which
time-frequency analysis was performed. Protocol masseter volume conduction arose were checked in every
parameters: 0.1-40Hz frequency band, target/frequent patient. The values of ENoG (%) with and without
stimulus 1kHz/2kHz, 100 ms, interstimulus interval 4s, 40 considering masseter volume conduction were assessed.
target stimuli. Results: Most of the masseter volume conductions arose
Results: When the intracerebral electrodes were connected between a stimulating intensity of 40 and 60mA except 4 of
with the reference electrode on the scalp - the N1 wave 26 masseter muscles, which are minimally 35mA in 1
(around 100ms) and MMN (peak around 200ms) dominated muscle, and 70mA in 2 muscles, and maximally 80mA in 1
at record. The most distinct N1 wave frequency was around muscle. When masseter volume conduction was neglected,
3Hz and this signal weakened while target motion was only 1 patient revealed over 90% axonal loss (ENoG< 10%).
presented. While intracerebral electrodes were connected in On the contrary, when masseter volume conduction was
the STN (near field potential) MMN-like potentials were taken into account, 3 patients revealed over 90% axonal loss
detected in 3 of 5 patients and in 4 of 9 intracerebral (ENoG< 10%), which means a poor outcome.
electrodes. These EP were in the same latency (19625ms) Conclusions: Masseter volume conductions arose in all
like the MMN from connection of intracerebral electrodes patients. Even though the ENoG is over 10%, one should
to the extracranial reference electrode. In 1 patient we consider masseter volume conduction all the time, especially
observed phase reversal of MMN-like potentials. when 80~90% axonal loss is represented.
Conclusion: The nucleus subthalamicus is involved in
processing of auditory signals on an unconscious level.
However, further verification will be needed. Supported
CZ.1.05/1.1.00/02.0068, CZ.1.05/1.1.00/02.0123 and
MUNI/A/0862/2011.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


230 Posters, Sunday 9 September

P1374 P1375
Chronic inflammatory sensory Acoustic shear wave signalling and
polyradiculopathy: a case report and calcium activation as the scientific basis
review of the literature of acupuncture physiology: TRP ion-
P. Zuberbuhler1, R. Reisin1, D. Miarro1, D. Binaghi2, channel experiments with fibroblast and
C. Marchesoni1, A. Pardal1 endothelial cells
1Neurology Department, Hospital Britnico Buenos Aires, J. Liang1, W.J. Xie2, X.Q. Yao3, P.Y.S. Cheung4,
2Radiology Department, Fundacin Favaloro, Buenos Aires,
S.K. Lam4, Q. Ma5, P.-W. Li6, X. Yang7, G. Li5,
Argentina
E.S. Yang4,6,8, P. Cheng9
Introduction: Chronic inflammatory sensory 1EEE, University of Hong Kong, Hong Kong, Hong Kong
polyradiculopathy (CISP) is a defined entity, frequently S.A.R., 2Health Science Center, Peking University, China,
underdiagnosed, and potentially treatable. It must be 3Chinese University of Hong Kong, Shatin, 4University of

suspected in patients with sensory ataxia, normal nerve Hong Kong, 5Time Medical Systems, Hong Kong, Hong
conduction studies (NCS), and MRI with thickened Kong S.A.R., 6Electrical Engineering, National Central
lumbosacral nerve roots and gadolinium enhancement. University, ChungLi, Taiwan R.O.C., 7Shenzhen Disease
Objective: Report of one case and review of the literature. Control Center, Shenzhen, China, 8Time Medical Systems,
Case report: A 57-year-old man with marked sensory Hing Kong, Hong Kong S.A.R., 9Peking University, China
ataxia on his left leg. Examination showed normal strength, A novel model of acupuncture physiology based on cellular
decreased knee and ankle jerks. Light touch and pinprick calcium activation by an acoustic shear wave (ASW)
sensations were reduced below the knees. Vibration and generated by the mechanical movement of the needle was
joint position sense were absent in the feet. Rhomberg was reported recently and its implication on integration of
positive. NCS were normal. Tibial sensory evoked potentials ancient Chinese medicine with modern science was
disclosed absent responses bilaterally. CSF was acellular discussed. Experimental evidences were observed using
with elevated protein. Lumbosacral MRI showed thickening latest instrumentation in MR Elastrography, confocal
of roots, with gadolinium enhancement. The patient was microscopy and two-photon fluorescent. To further
treated with IVIg 2g/kg for 5 days, with improvement of understand the biological nature of the ancient art, we have
symptoms. Currently, he receives IVIg every month, and developed an acoustic wave model that relates Ca2+
remains stable. activation to the total acoustic energy; not time and
Conclusions: The clinical course, elevated CSF protein, the amplitude alone. We have made use of siRNA in knockdown
evidence of root enhancement on the MRI, good response experiments in both fibroblast and endothelial cells and
to immunotherapy and the exclusion of other causes of found that acupuncture induced ASW activates Ca2+ influx
sensory ataxia, were compatible with the diagnosis of CISP. pathway of TRPC1, TRPV4 and TRPM7 channels in both
CISP is considered a restricted regional variant of chronic NIH 3T3 and H5V cells indicating the mechanosensitive
inflammatory demyelinating polyradiculopathy (CIDP), activities via the TRP channels. TRP channels, especially
clinically mimicking a purely sensory variant. There are TRPV4, have been implicated in the detection of acute
several reports of nerve root involvement in patients with nociceptive and neuropathic pains. In cooperation of
CIDP, but there are only a few of patients with sensory TRPC1, TRPV4 was found to mediate mechanical hyper-
ataxia, nerve root involvement, but normal nerve conduction algesia and primary afferent nociceptor sensitization. ASW
studies. CISP is probably an underdiagnosed but potentially activated Ca2+ influx pathway of TRPV4 might be the link
treatable disorder that requires the identification of isolated of the pain release observed in the large scale clinical trials
involvement of the sensory roots. of acupuncture performed mostly in Europe in the last 10
years.

P1376
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 231

P1377 P1378
Nucleotide interactions with K-ATP Cervical vestibular evoked myogenic
channels in hippocampal neurons potentials in cerebellar lesions
examined with open-cell patch-clamp E.S. Papathanasiou, S.S. Papacostas, M. Pantzaris
method Clinical Sciences, The Cyprus Institute of Neurology &
Genetics, Nicosia, Cyprus
R. Mollajew
Neuro and Sensory Physiology, Medical University of Introduction: Cervical vestibular evoked myogenic
Gttingen, Germany potentials (cVEMPs) represent a non-invasive method of
ATP-sensitive K+ (KATP) channels are widely expressed in evaluating the function of the vestibular nervous system
the brain and contribute in many physiological processes. using sound stimulation. Using air-conducted sound, the
Especially important is their protective role during hypoxia/ above is thought to occur as the saccule is located close to
ischemia and epilepsy. We examined hippocampal neurons the oval window. Our experience in patients with cerebellar
in primary culture using open-cell (OC) patch-clamp lesions is evolving, with only a few references published,
method. In the test experiments we monitored a loss of fura- and more illustrative cases are required. We describe two
2 or fluo-3 from preloaded neurons and estimated that cases with isolated cerebellar vascular lesions who had
equilibrium between cytoplasm and bath solution was cVEMPs and brainstem auditory evoked potentials
established within <1min after addition of -escin. In many (BAEPs).
neurons we observed generation of action potentials in the Methods: BAEPs were performed in the standard manner
cell-attached mode indicating normal viability. The cells using unilateral click stimulation at 70dB nHL with
were permeabilized in Ca2+- and Mg2+-free solutions by contralateral masking noise. cVEMPs were performed
brief exposure to 5-M -escin after which they are viable using unilateral 120dB peak SPL (pSPL) intensity 1kHz
for 5 to 60 min. The activity of KATP channels increased tone air-conducted sound stimulation with contralateral
within 2 min after permeabilization in ATP-free solutions. 90dB pSPL masking noise. Recording was performed from
Subsequent application of 1mM ATP inhibited KATP the tonically active sternocleidostoid muscle ipsilateral to
channels. 1mM ADP in ATP-free solution considerably the ear receiving tone stimuli, with the active recording
increased their activity. Most likely it happens due to electrode placed over the midpoint of the muscle, the
suppression ATP sensitivity of KATP by ADP. Combined reference electrode on the clavicle and the ground on the
application of 1mM AMP and 1mM ATP enhanced KATP forehead.
channel opening that might be due to disproportionate Results: The first case (28 years old) presented with a right
reaction AMP + ATP < -> 2 ADP. The effects were not middle cerebellar peduncle lesion, whereas the second case
observed when AMP was applied with non-hydrolyzable (65 years old) presented with a lesion in the right middle
ATP-PNP (1mM) and ADP-PNP (1mM). Further cerebellar peduncle and hemisphere. Both cases had normal
complexity produced intracellular Mg2+ whose complexes cVEMPs bilaterally. The first case revealed an abnormal
with ATP and ADP additionally increased channel openings. BAEP on the left and a disorganized BAEP on the right,
The data thus reveal the multifaceted role of adenine whereas the second case showed normal BAEPs bilaterally.
nucleotides in regulation of KATP channels that perhaps Conclusion: cVEMPs appear to be unaffected by middle
cannot be reduced to a simple rationale involving ATP/ADP cerebellar peduncle and cerebellar hemispheric lesions.
ratio.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


232 Posters, Sunday 9 September

P1379 P1380
Alpha coma as unusual EEG pattern of Uncommon neurophysiological patterns
sedation induced by Propofol in Lambert Eaton syndrome
M.P. Pirovano1,2, F. Sasanelli1 E. Agazzi
1Department of Neurology, Azienda Ospedaliera di Ospedali Riuniti di Bergamo, Italy
Melegnano, Vizzolo Predabissi, 2Neurology Residency
School, University of Milano-Bicocca, Monza, Italy
Introduction: Propofol, a short acting hypnotic drug, is
P1381
usually used in intensive care units in several clinical Median compound sensory nerve action
situations, including treatment of status epilepticus by potentials (CSNAPs) recorded
continuous infusion. In these cases the EEG pattern is antidromically from the finger using a
characterized by widespread delta activity, later joined by
burst-suppression.
distant reference electrode
Case report: A woman shows up at the emergency room A.S. Wee1,2, S.J. Wilson1
presenting abdominal pain, involuntary movements and
1Neurology, University of Mississippi Medical Center,
irreducible trismus; suspecting prolonged seizure, she
2Neurology, G.V. (Sonny) Montgomery VA Medical Center,
undergoes treatment with Propofol infusion. Brain and Jackson, MS, USA
abdominal CT scans and blood tests, including extensive
toxicology, were negative. After 36 hours of sedation with
Propofol, the EEG showed a persistent and widespread
P1382
activity at 13Hz, not reactive to stimuli. After 48 hours, Acute onset multifocal motor neuropathy
infusion of Propofol was discontinued and the patient woke with conduction block (MMNCB): a case
up without seizures. An EEG performed after 12 hours report of a benign variant
showed normal awake activity, characterized by alpha
D. Tsiptsios, D. Kiourtidis, T. Tsironis, M. Krommyda,
rhythm. Subsequent investigations led to the diagnosis of
E. Ameridis, P. Petrou, A. Mastrokosta, A. Tichalas,
urosepsis, secondary to acute pyelonephritis caused by
G. Xiromerisiou, E. Koutlas, X. Fitsioris, J. Rudolf,
Pseudomonas Aeruginosa, responsive to tetracycline
treatment. The antiepileptic treatment was not necessary. G. Deretzi, I. Tsiptsios
Neurology Department, Papageorgiou General Hospital,
Conclusion: Retrospective studies showed non-epileptic
Thessalonki, Greece
events classified as seizures in about 15% of patients. In this
case the misdiagnosis allowed describing an EEG pattern of
alpha coma during Propofol infusion. Studies in children P1383
suggest that propofol induces burst-suppression as typical
EEG pattern, but data in adults are poor. In the same way, Diagnostic sensitivity of cervical
there are few reports of alpha coma, usually described in vestibular evoked myogenic potentials in
brain stem lesions. It is therefore necessary to conduct acute vertigo distinction
further studies in adults to better stratify the possible EEG M. Jolic, M. Lazarevic, S. Jolic, V. Djuric, J. Stamenovic,
patterns about duration and doses of propofol infusion. S. Djuric
Neurophysiology Department, Clinical Center of Nis, Serbia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 233

P1384 P1389
Patients with central and peripheral Neurophysiological monitoring of the
neuropathic pain: evaluation of trans-procedure endovascular closure of
neuropathic pain with specific atrial septum defects (ASDs) in adults
neurophysiological reflex techniques and M.E. Habeych Sanmiguel1, W. Anderson2, J.R. Balzer1,
clinical approach D.J. Crammond1, P.D. Thirumala1
1Neurological Surgery, 2Invasive Cardiology, University of
N. Dogan1, D. Selcuki1,2
1Neurology, 2Algology-Neurophysiology, Celal Bayar Pittsburgh, Pittsburgh, PA, USA
University, Manisa, Turkey
P1390
P1385 Hepcidin mediates stress induced
Autoimmune encephalitis associated with depression
Graves disease: a case report A. Nahavandi1, F. Farajdokht2, M. Solaymani2
1Department of Physiology, Tehran University of Medical
M. Saadatnia, V. Davoudi, K. Keyhanian, M. Siavash,
Sciences/Physiology Research Centre/Cellular and
B. Ansari, F. Haghdoost
Molecular Research Centre, 2Tehran University of Medical
Isfahan University of Medical Sciences, Isfahan, Iran
Sciences, Tehran, Iran

P1386 P1391
Evaluation of cutaneous silent period Spastic cerebral palsy treated by anodal
parameters in patients with type-2 transcranial direct current stimulation:
diabetes mellitus: effects of pregabaline a pilot study
O. z1, H. Akgn1, M. Ycel1, A. Talpnar2,
B. Aree-Uea1, N. Auvichayapat2, T. Janyacharoen3,
. Erdoan3, .H. Ula1, Z. Odaba1
1Department of Neurology, 2Department of Endocrinology, S. Iamsaard4, J. Prasertnoo5, P. Auvichayapat6
1Physiology, Faculty of Medicine, Khon Kaen University,
Glhane Medical Academy, Ankara, 3Department of 2Department of Pediatrics, Khon Kaen University,
Neurology, Pamukkale University, Denizli, Turkey 3Department of Physical Therapy, Faculty of Associated

Medical Sciences, Khon Kaen University, 4Department of


P1387 Anatomy, Faculty of Medicine, Khon Kaen University,
5Physical Therapist of Srisangwal School, 6Department of
Congenital hypogenesis of bilateral Physiology, Faculty of Medicine, Khon Kaen University,
abductor pollicis muscle: report of two Khon Kaen, Thailand
cases
H. Yaar1, O. z2, M. Korkmaz2, H. Akgn2, H. Tekeli3 P1392
1Department of Neurology, Ankara Mevki Military Hospital,
2Department of Neurology, Glhane Medical Academy, How to predict axonal degeneration in
3Department of Neurology, Kasimpaa Military Hospital, carpal tunnel syndrome?
Ankara, Turkey M.-H. Chang
Neurology, Taichung Veterans General Hospital, Taichung,
P1388 Taiwan R.O.C.

Cervical vestibular evoked myogenic


potentials are efficacious in evaluation of P1393
vestibulospinal pathways in patients with Hereditary neuropathy with liability to
multiple sclerosis pressure palsy presenting with brachial
M. Krbot1, T. Gabeli1, I. Adamec1, M. Habek1,2 plexopathy
1University Hospital Centre Zagreb, 2University of Zagreb,
G. Genc1, S. Bek2, T. Kasikci2, B. Kurt2, Z. Odabasi2
School of Medicine, Zagreb, Croatia Maresal Cakmak Hospital, Erzurum, 2Gulhane Military
1

Medical Academy, Ankara, Turkey

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


234 Posters, Sunday 9 September

P1394 P1400
Prognostic value of quantative EEG prior Role of H-reflex latency in diagnosis of
to electroconvulsive therapy in psychotic diabetic neuropathy
disorders and major depression M. Budimirovi
E. Dal1, O. Kamisli1, M.E. Tagluk2, S. Kartalci3, Neurology, County Hospital Sremska Mitrovica, Sremska
Y. Kaplan1, C. Ozcan1 Mitrovica, Serbia
1 Neurology, 2Electric and Electronic Engineering,
Psychiatry, Inonu University, Malatya, Turkey
P1401
3

Movement disorder at pituitary


P1395 microadenoma
Treatment of spinocerebellar
M.G. Zhestikova1, V.A. Minenkov1, N.P. Shakhvoorost2,
degeneration with serotonin agonist E.I. Rybalko3, E.D. Vasenina4, S.D. Komissarova4,
A. Takei1, S. Hamada1, H. Soma1, S. Honma1, N.A. Korotkevich5, V.C. Van1, L.V. Van1
K. Hamada1, H. Moriwaka1, K. Tashiro1, H. Saaki2 1Neurology, State Budgetary Educational Institution of
1Hokuyukai Neurological Hospital, 2Hokkaido University Additional Education Novokuznetsk State Advanced
School of Medicine, Sapporo, Japan Medical Studies the Ministry of Health Care and Social
Development of the Russian Federation, 2Endokrinology,
Municipal Budgetary Medical Preventive Institution City
P1396 Clinical Hospital 1, 3Neurology, Municipal Budgetary
Abstract cancelled Medical Preventive Institution City Clinical Hospital 29,
4Neurology, Municipal Budgetary Medical Preventive

Institution City Clinical Hospital 2, Novokuznetsk,


P1397 5Neurology, Regional Budgetary Medical Preventive

Abstract cancelled Institution Regional Hospital, Kemerovo, Russia

P1398 P1402
Saccadic eye movements during aging in Abstract cancelled
healthy volunteers, patients with
Parkinsons disease and progressive P1403
supranuclear palsy Electromyography in obvious and
R. Bogdanov1, P. Ratmanova2, A. Litvinova2, subclinical hypothyroidism and its clinical
D. Napalkov2 correlation
1Department of Neurology, Moscow Regional Scientific
E. Oguz Akarsu1, H. Acar1, M.F. Genc1, S. Gunaydin1,
Research Clinical Institute n.a. M. F. Vladimirsky,
2Department of Higher Nervous Activity, M. V. Lomonosov S. Ozben2, F. Ozer1, M.B. Aktuglu3
1Neurology, Haseki Training and Research Hospital,
Moscow State University, Moscow, Russia
Istanbul, 2Neurology, Kafkas University, Kars, 3Internal
Medicine, Haseki Training and Research Hospital, Istanbul,
P1399 Turkey
Computerized posturographic analysis of
balance control in patients with P1404
Parkinsons disease and essential tremor Applying balance control systems in
R. Bogdanov1, D. Napalkov2, P. Ratmanova2 coordination disturbances diagnostics
1Department of Neurology, Moscow Regional Scientific N. Tarasevich, U. Lukashevich
Research Clinical Institute n.a. M.F. Vladimirsky, V&A Laboratory Limited Liability Company, Moscow,
2Department of Higher Nervous Activity, M.V. Lomonosov
Russia
Moscow State University, Moscow, Russia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 235

Epilepsy 1 P1406
Actions of sK-channel openers in a
P1405 pharmacoresistant seizure model in-vitro
M.L. Raza, I. Papageorgiou, U. Heinemann
Localizing epileptic networks in tuberous Institute of Neurophysiology, Charite Universittsmedizin,
sclerosis using arterial spin labelling MRI CVK, Berlin, Germany
perfusion sK-channels are reported to regulate neuronal
A. Azarion1, K.A. Davis2 hyperexcitability. Pharmacoresistance in epilepsy is one of
1Neurology, Hospital of the University of Pennsylvania, major problems; nearly 30% of patients are non-responsive
University of Pennsylvania School of Medicine, to antiepileptic drugs. To address this issue of
Philadelphia, 2Neurology, Crozer Chester Medical Center, pharmacoresistance and role of sK-channels whether
Chester, PA, USA effective or not, we tested sK-channel agonist using
Introduction: The goal of this study is to utilize the non- pharmacosensitive/ pharmacoresistant organotypic
invasive technique of arterial spin labelling (ASL) magnetic hippocampal slice cultures (OHSC) and human tissue from
resonance imaging (MRI) to identify epileptic networks in pharmacoresistant epileptic patients. Slice cultures were
patients with tuberous sclerosis complex (TSC), a subtype prepared from 7-8 day-old rat pups and field potential
of neocortical epilepsy. Given that patients with TSC often recordings were performed from 7-14 days after incubation.
have better seizure control after surgical resection of For induction of seizure-like events (SLEs) in slice cultures,
epileptic lesions as well as perilesional tissue, this cohort is we have used 4-AP (100M) & zero magnesium ACSF. In
ideal for testing the hypothesis that ASL can accurately human tissue protocol of high K+ (8 mM) + bicuculline
identify epileptic networks. Recent studies have shown that (50M) & 4-AP (100M) is followed to induce epileptiform
the quantitative ASL signal correlates with underlying activities. Results showed anticonvulsant actions of CyPPa,
pathological changes of tubers, as well as their epileptic interestingly 4-AP induced SLEs in both pharmacosensitive
potential. and pharmacoresistant OHSCs were blocked by CyPPa at
Methods: In this study we plan to analyze the perfusion 100M in 100% slices. Whereas, zero magnesium induced
patterns both within tubers and in the surrounding SLEs are blocked in 88% and 74% of pharmacosensitive
perilesional regions in two populations of TSC patients, and pharmacoresistant OHSCs respectively. Similarly,
those with epilepsy (n=6), and those with no history of CyPPa at same dose blocked SLE induced by 4-AP in all
seizures (n=6). Epileptic tubers will be cross-identified with human tissues. K+ + bicuculline induced SLEs are blocked
scalp EEG, and this data will be correlated with ASL in 3 out of 5 slices. CyPPa at 20M blocked SLEs in 100%
perfusion maps. pharmacosensitive slices whereas, same dose of CyPPa was
Results: We have collected and analyzed ASL and MRI ineffective against SLEs induced by 4-AP in
data on 4 TSC patients with epilepsy. Thus far, it appears pharmacoresistant OHSCs. In zero magnesium model,
that epileptic tubers and surrounding perilesional areas 20M dose failed to block SLEs in both pharmacosensitive
show hyperperfusion in comparison with average cerebral and pharmacoresistant OHSCs. Our findings suggests
blood flow. Analysis of the non-epileptic tubers reveals no CyPPa has potential to block both pharmacosensitive &
significant differences in perfusion compared to average pharmacoresistant type of seizures.
cerebral blood flow.
Conclusion: Our preliminary data show that ASL can
accurately identify epileptic networks in TSC. Using ASL
to identify epileptic networks can potentially be applied to
all patients with neocortical epilepsy, with the ultimate goal
of improving seizure freedom rates from resective surgery.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


236 Posters, Sunday 9 September

P1407 P1408
Risk factors for epileptic seizure of Founder c.1048-1049delGA mutation in
cavernous malformations in the central NHLRC1 gene in Laforas disease patients
nervous system: 52 cases from Serbia
C. Huang1, D. Zhou2 M. Kecmanovic1, N. Jovic2, M. Keckarevic-Markovic1,
1West China Clinical Medicine School of Sichuan University, D. Keckarevic1, G. Stevanovic2, S. Romac1
2Department of Neurology, West China Hospital of Sichuan
Faculty of Biology, University of Belgrade, 2Clinic of
1

University, Chengdu, China Neurology and Psychiatry for Children and Youth, School of
Purpose: To determine the risk factors for preoperative and Medicine, University of Belgrade, Belgrade, Serbia
postoperative epileptic seizure in patients with cavernous Objectives: Lafora's disease (LD) is an autosomal recessive,
malformations (CMs). progressive metabolic disorder characterized by intractable
Methods: We retrospectively studied 52 consecutive myoclonus and seizures, inexorable neurological
patients diagnosed with CMs who were surgically treated deterioration, cognitive decline, unfavourable clinical
and histopathologically enrolled in West China Hospital of course and poor prognosis. Despite the clinical homogeneity
Sichuan University from January 2009 to June 2010. there is genetic heterogeneity in LD. It is caused by
Clinical data, treatment procedure and follow-up mutations either in EPM2A or in NHLRC1. There is also at
information were collected. least one other gene that can be associated with LD. Here
Results: In the univariate analysis, factors associated with we report a haplotype study on 11 unrelated LD
preoperative epileptic seizure were low birth weight chromosomes from Serbia carrying c.1048-1049delGA
(p=0.017), temporal lobe involvement (p=0.003) and (p.E350fs) mutation in NHLRC1.
cortical lesion (p=0.025). In the multivariate analysis, the Methods: Using direct sequencing analysis, we identified 4
cortical lesion showed a high risk for preoperative epileptic different mutations in NHLRC1 gene in 11 patients from 7
seizure (OR=10.48; 95% CI 1.61-68.23). In the univariate Serbian families. As 11 unrelated LD chromosomes from 6
analysis, patients with temporal lobe CMs were found more patients carry c.1048-1049delGA mutation in NHLRC1, we
likely to be seizure free after surgery (p=0.016). The performed a haplotype analysis in order to estimate if this is
maximum diameter of CMs longer than 2.5cm (p=0.012) the founder mutation. 8 markers flanking NHLRC1:
and disease course longer than 1 year (p=0.050) predicates D6S289-D6S1605-D6S274-D6S1567-NHLRC1-D6S1688-
an unfavourable outcome. In the multivariate analysis, D6S1959-D6S1643-D6S1665 were amplified by PCR and
temporal lobe involvement still showed a favourable run on 4% polyacrilamide gel along with sequenced allelic
outcome (OR=0.038; 95% CI 0.002-0.833). Application of ladder.
ECoG did not make significant difference in seizure Results: We identified 8 different LD haplotypes carrying
outcome (p=0.430). p.E350fs mutation, but all analyzed chromosomes shared
Conclusion: Surgical treatment of patient with CMs is same alleles for markers D6S274(166)-D6S1567(108)-
satisfactory in most cases and temporal lobe involvement D6S1688(172). The size of common haplotype is correlated
usually predicts favourable postoperative seizure outcome to the number of generations from the ancestors on whose
whether under the monitoring of ECoG or not. Thus, chromosome mutation arose. So, considering those markers,
epileptic patients with CMs should be considered for we estimated how long ago this mutation arose on an
surgical treatment especially when cortical brain layer or ancestral chromosome. Shared haplotype covers 1.8cM
temporal lobe is involved. indicating that mutation arose 55 generations ago.
Conclusion: Mutations in NHLRC1 are the common cause
of LD in Serbia, what is mostly due to a founder effect. The
common ancestor carrier of p.E350fs mutation lived 55
generations ago.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 237

P1409 P1410
Age- and gender related risk of Partial epilepsy with auditory features:
developing unprovoked seizures after clinical and genetic features of 186
traumatic brain injury: a population-based sporadic cases
case-control study C. Stipa1, F. Bisulli1, F. Pittau1, L. Licchetta1, I. Naldi1,
B. Mahler1, C. Adelw1, T. Andersson2, A. Ahlbom2, L. Di Vito1, C. Leta1, B. Mostacci1, R. Michelucci2,
T. Tomson1 S. Striano3, P. Striano4, U. Aguglia5, E. Ferlazzo5,
1Department of Clinical Neurosciences, 2Institute of A.T. Giallonardo6, C. Nobile7, M. Seri8, T. Pippucci8,
Environmental Medicine, Karolinska Institute, Stockholm, P. Tinuper1
1IRCCS Institute of Neurological Sciences, University of
Sweden
Purpose: To study traumatic brain injury as risk factor for Bologna, 2Department of Neurological Science, Bellaria
unprovoked epileptic seizures in a population-based case- Hospital, Bologna, 3Department of Neurological Sciences,
Federico II University, Naples, 4Department of
control study with incident seizure cases.
Neurosciences, University of Genoa, Genoa, 5Institute of
Method: In this study, 1885 patients with new onset
Neurology, Magna Grcia University, Catanzaro,
unprovoked seizures were included in the Stockholm 6Department of Neurological Sciences, Sapienza University,
Incidence Registry of Epilepsy. 15080 controls matched for
Roma, 7Institute of Neurosciences, University of Padova,
gender, year of diagnosis, and catchment area were Padova, 8Medical Genetics Unit, University of Bologna,
randomly selected from the register of the Stockholm Bologna, Italy
County population. Exposure was defined as a hospital
discharge diagnosis of a traumatic head injury preceding the Introduction: In 2004 we described 53 cases of partial
date of seizure onset. Odds ratios (ORs) with 95% epilepsy with auditory features and good clinical outcome.
confidence interval (95%CI) were calculated using logistic The syndrome was named Idiopathic Partial Epilepsy with
regression to assess the risk of developing unprovoked Auditory Features (IPEAF). Since 2004 we observed 133
seizures after hospital admission for various categories of additional cases and the present study summarizes clinical
patients and type of head trauma defined by ICD codes from features of this group of 186 patients (85 females and 101
the Swedish Hospital Discharge Registry. males).
Results: OR for unprovoked epileptic seizures was 2.0 Methods: Patients were selected according to the following
(1.5-2.7) after cerebral concussion (66 cases); 2.5 (1.6-3.9) criteria: partial epilepsy with auditory symptoms, negative
for women and 1.8 (1.3-2.5) for men. For patients older than family history for epilepsy and absence of cerebral lesions.
65 years of age OR was 2.5 (1.5-4.3) and for younger All patients underwent full clinical, neuroradiological and
patients 1.8 (1.3-2.5). After brain contusion or traumatic neurophysiological examination. The majority of patients
intracranial haemorrhage (37 cases) OR was 8.9 (5.6-14.1). were screened for LGI1 mutations observed in more than
OR of developing unprovoked seizures was similar for half of familial cases with autosomal dominant partial
women 9.6 (4.3-21.5) and men 8.5 (4.8-15.1). For older epilepsy with auditory features (ADPEAF).
patients OR was 4.7 (2.3-9.6) and 10.9 (5.8-20.4) for Results: Age at onset ranged from 3 to 59 years (mean 22
younger patients. years). Secondarily generalized seizures were the most
Conclusion: We provide quantitative data on the risk of common type of seizures at onset (67%). Auditory auras
developing unprovoked seizures after various brain occurred either in isolation (38%) or associated with visual,
traumata. Our findings point towards a higher relative risk psychic or aphasic symptoms (62%). Low seizure frequency
for younger patients to develop epileptic seizures after at onset and good drug response were common, with 41%
severe traumatic brain injury. of patients seizure-free (minimum follow-up 6 months).
Seizures tended to recur after drug withdrawal. We observed
a de-novo mutation in LGI1 gene in only three patients of
the 118 tested.
Conclusions: Our data confirm the existence of a peculiar
form of non-lesional temporal lobe epilepsy, closely related
to ADPEAF, in which auditory aura could represent a
clinical marker of benign outcome in the majority of
patients. Although LGI1 mutations have been found in only
2.5%, another gene could be involved.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


238 Posters, Sunday 9 September

P1411 P1412
Power spectral frequency analysis of peri- Epileptic vertigo: overview of clinical
ictal suppression by spontaneous semiology
adenosine release in hippocampus in a M. Saad, S. Zakarya, M. Abu-Hegazy
kainic acid-induced limbic status Neurology, Mansoura University, Mansoura, Egypt
epilepticus rat model Introduction: Epileptic vertigo is a rare form of partial
Y.-I. Kim1, P.A. Kwak2, Y.-M. Shon3 seizures. It is often distinctive with sudden very brief
1Department of Neurology, St Paul's Hospital, The Catholic episodes followed by rapid recovery without sequelae.
University of Korea, 2Department of Neurology, Seoul St Objective of this study was to establish the contribution of
Mary's Hospital, The Catholic University of Korea, clinical semiology of epileptic vertigo in absence of
3Department of Neurology, Yeouido St Mary's Hospital, The convulsions and loss of consciousness.
Catholic University of Korea, Seoul, Republic of Korea Patients and methods: 30 epileptic vertigo patients (19
males and 11 females) were included. Their ages ranged
Background: We performed time-frequency analysis of
from 12 to 40 years and mean age at onset was 26.7 years.
ictal EEG during spontaneous adenosine (ADO) release,
All patients were subject to neurological examination,
accompanied by a synchronous neurotransmitter recording
interictal electroencephalogram (EEG), caloric otologic
by WINICS (wireless instantaneous neurotransmitter
test, audiogram, nystagmography, carotid Doppler; brain
concentration system) in a KA-induced acute hippocampal
stem evoked potential and brain MRI.
seizure model in rats.
Results: Family history of epilepsy was reported in 20%,
Method: Young adult male Sprague-Dawley rats (n=6)
no past history of febrile convulsion, tunnel voices aura was
were implanted with four cortical electrodes, bipolar
reported in 6.7%. Dizziness was report in 8 patients
hippocampal (HC) depth electrodes and a cannula for the
(26.7%), 14 patients with true vertigo (46.6%), nausea in 6
injection of KA (1mcg 4min) in the right hippocampus
(20%) and tinnitus in 2 patients (6.7%). EEG abnormality
(CA3) under urethane anaesthesia. WINCS recording
was documented in all cases: 15 (50%) and 6 (20%) patients
electrode was inserted in left hippocampus (mirror site of
illustrated focal left and right temporal epileptiform
R HC). EEG data were analyzed by fast Fourier
discharges consecutively. In addition, there were 2 (6.7%)
transformation to obtain the power spectral density (PSD)
bitemporal, 2 (6.7%) frontotemporal and 5 cases (16.8%)
for delta to gamma frequencies (range: 1.25-100Hz), as
temporal with secondary generalization discharges. MRI of
well as local field potential (LFP) taken by both HC with a
brain was normal in 20 (66.6%), 4 cases with temporal lobe
high-frequency band (100 - 500Hz, ripple and fast ripple).
tumours, cerebral infarction was in one and small cyst.
Results: During the spontaneous, intermittent and phasic
Frontal lobe lesions were detected in 4 patients. No mesial
release of adenosine, there was a sudden disappearance of
temporal sclerosis was detected.
polyspike or spike-wave discharges in its EEG and LFP the
Conclusion: Epilepsy is an important cause of transient
EEG. PSD showed an increased power in the theta and delta
dizziness and vertigo, but misdiagnosis due to absence of
frequency band, whereas higher frequency bands (beta and
convulsions and loss of consciousness is common.
gamma) showed a decrease. Furthermore, LFP from both
HC showed bursts of population spikes (BPS) immediately
before the phasic adenosine release. BPS showed increased
power in ripple or fast ripple frequency band (100-300Hz).
Conclusion: These data strongly support the hypothesis
that adenosine might play a key role as an endogenous anti-
seizure modulator.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 239

P1413 P1414
Ictal PET F18-FDG in refractory focal Acquired mirror writing in a patient with
epilepsy: report of 6 cases with surgically complex partial seizures after surgery for
removed cortical hypermetabolic lesions, left frontal meningioma
with histopathological correlation and D. Hodorog, D.I. Cuciureanu
follow-up Clinica I Neurologie, University of Medicine and Pharmacy
Gr.T. Popa, Iasi, Romania
D. Ladron de Guevara1, S. Marca2,
G. Kuester2,
L. Rios2,
F. Solari2, M. Galvez1, M. Campos2, F. Otaiza2 Mirror writing can be related to a variety of focal
1Radiology, 2Advanced Epilepsy Center, Clinica Las Condes, neurological disorders involving left parietal lobe, left basal
Santiago, Chile ganglia, and right supplementary motor area. It has also
Introduction: Unlike interictal PET (Positron Emission been associated with diffuse cerebral disorders. It is nearly
Tomography), ictal PET is not regularly used in focal always carried out with the left hand. Left-handers represent
refractory epilepsy study, and its usefulness in pre-surgical the majority of patients. Motor, spatial-orientation, visual
evaluation and prognosis value has not been established yet. word-form, involvement of thalamo-cortical circuitry
Objective: To present 6 cases of epilepsy patients whose hypotheses have been advanced to explain mirror writing.
F18-FDG PET showed hypermetabolic cortical lesion, and Mirror writing is rare and usually resolves over days to
to correlate with MRI, EEG, histopathology and clinical weeks. Acquired mirror writing accompanied by mirror
outcome. reading have been described in some patients with extensive
Methods: Histopathology and post-surgical outcome of 6 visuospatial deficits and left-right disorientation.
hypermetabolic F18-FDG PET of patients with focal We present the case of a right-handed women, with complex
epilepsy were reviewed. PET findings were correlated with partial seizures and acquired mirror writing after brain
Video-EEG, MRI, and intraoperative EcoG. surgery for a left frontal meningioma. Writing was tested for
Results: Ictal PET showed well-defined hypermetabolic regular and irregular words, whole sentences, numbers. She
lesions in all patients (3 temporal and 3 extratemporal wrote in mirror as fluidly as normal direction and preferred
lesions). There was coincidence between clinical, Video- mirror writing with either the right or left hand. Her
EEG, intraoperative EcoG and PET hypermetabolic foci in preference persisted for 4 years since her brain surgery. She
all cases. MRI showed correctly the seizure onset area in 4 was capable of mirror reading, which was preferred to
cases and was negative in 2 (female 38y hippocampal normal reading. Oral language skills were normal. Apraxia,
sclerosis/amygdala gliosis, and male 3y frontal type IA visual agnosia, neglect, left-right disorientation were absent.
FCD). Both PET and MRI allowed correct localization of It is unlikely that mirror writing of our patient is the
foci in 4 cases: male 16 m frontal type IIB FCD, male 3 m consequence of the release of mirror motor engrams. The
occipital type IIA FCD, male 7 m with temporal IIA FCD engrams within the right hemisphere are not associated with
and polymicrogyria, and female 40 y hippocampal type IIB mirror reading as in our case. We presume that mirror
FCD. Post-surgical mean follow-up was 14.8 month (range writing of this patient could be a consequence of mirror-
1-27 month), resulting all patients seizure free (Engel I). reversed visual-spatial representations.
Conclusion: Ictal F18-FDG PET presents a high
correlation with clinical and EEG findings, and allows a
precise localization of seizure onset area in FCD lesions.
Surgical resection of a PET hypermetabolic area predicts an
excellent post-surgical outcome.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


240 Posters, Sunday 9 September

P1415 P1416
Neuropsychological profile in childhood Influence of anti-epileptic monotherapy
benign epilepsy with centrotemporal on bone mineral density and bone
spikes (BECTS) metabolism biochemical markers in
P. Conde-Guzn1, M.T. Bartolom-Albistegui2, P. Quirs3 children and adolescents with epilepsy
1Psychology, Universidad de Len, 2Neurology, Hospital
M.D. Dimic1, N.A. Dimic2
Nuestra Seora de Regla, Leon, 3Psychology, UNED, 1Department of Paediatric Neurology, Clinical Center of Nis,
Madrid, Spain Nis, 2Clinic for Rheumatology, Institut for Therapy and
Objective: There is controversy regarding the Rehabilitation, Niska Banja, Serbia
neuropsychological profile of children with epilepsy. The Introduction: Enzyme-inducing (carbamazepine) or non-
aim of this study is to analyze the neuropsychological enzyme-inducing (valproate) antiepileptic drugs are
profile of children with benign epilepsy with centrotemporal associated with bone loss and mineral metabolism
spikes (BECTS). disturbances.
Methods: Sample: 49 children from 7 to 12 years (25 with Methods: We evaluated effects of the carbamazepine and
BECTS treated with carbamazepine and 24 controls). We valproate monotherapies on bone mineral density (BMD),
got the neuropsychological profile by applying the WISC-R calcium and alkaline phosphatase serum levels in 29
(Wechsler Intelligence Scale for Children-Revised) and ambulatory male epileptic patients aged 6-18 years, taking
Battery Luria-DNI (Manga and Ramos, 1991). therapy longer than 1 year and in age matched healthy male
Results: A) We have not found any differences in IQ control group (n=31). We measured BMD by Dual-energy
between children with BECTS and controls. B) The X-ray absorptiometry at lumbar spine (L1-L4).
neuropsychological profile Luria-DNI is significantly lower Results: The mean serum levels of carbamazepine and
in BECTS (p<0.05) than the control group in hand motor valproate were 7.651.46mg/ml and 78.6412.76mg/ml,
activity, verbal regulation, tact, spatial orientation, respectively, and the mean therapy duration for each drug
articulation, naming, phonemics analysis, reading, was 3.061.61years and 3.161.93years, respectively.
numerical structure and arithmetic. This is a severe Calcium intakes in diet were similar in both the control and
deterioration in verbal regulation, articulation, phonemics study groups. Bone mineral density values of both valproate
analysis and reading. C) Nevertheless, we have not found and carbamazepine groups were lower without statistically
deterioration in the memory and the attention. In children significant difference compared with control group
with crisis of absences we have found, in a previous study, (p>0.05). The serum calcium levels were significantly
a significant memory alteration (the short-term auditory and lower (p<0.01) in patients treated with carbamazepine.
visual memory and logical memory) and attentional Serum alkaline phosphatase levels were significantly higher
disorder. D) The time of oxcarbazepine treatment does not in boys treated with carbamazepine (p<0.02) and with
affect the neuropsychological profile, however, age at onset valproate (p<0.002), respectively. Therapy duration has
of the first crisis negatively affects the ability for naming showed no effect on bone mineral density and biochemical
and phonemics analysis. parameters.
Conclusions: In children, with this diagnosis and treatment, Conclusion: Carbamazepine and valproate caused lumbar
and neuropsychological profile described, we must consider bone mineral density decrease in boys with epilepsy.
strengthening the deficient neuropsychological and psycho- Hypocalcaemia was observed in patients treated with
educational areas. In addition, we must continue research in carbamazepine. Serum alkaline phosphatase level, as
future studies on the role of the antiepileptic drug in this marker of osteoblast activity, was significantly higher in
profile. both patient groups. Therefore, there are significant benefits
of regular screening and prophylactic supplementation with
vitamin-D and calcium in patients with epilepsy receiving
long-term antiepileptic therapy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 241

P1417 P1418
Defective inhibition in the visual system of Myoclonic Epilepsy and psychomotor
photosensitive patients with idiopathic delay associated with a novel SLC2A1
generalized epilepsy gene mutation
G. Strigaro, P. Prandi, L. Falletta, C. Varrasi, F. Monaco, L. Di Vito1, F. Bisulli1, D. Mei2, I. Naldi1, F. Pittau1,
R. Cantello L. Ferri1, C. Leta1, C. Stipa1, E. Pasini1, C. Marini2,
Department of Neurology, Amedeo Avogadro University, R. Guerrini2, P. Tinuper1
Novara, Italy 1IRCCS Istituto delle Scienze Neurologiche, University of

Introduction: To assess the visual system excitability of Bologna, Bologna, 2Pediatric Neurology Unit and
photosensitive patients with idiopathic generalized epilepsy Laboratories, A Meyer Children's Hospital, Florence, Italy
(IGE) with the paired-pulse flash-evoked potential (paired Introduction: GLUT1 deficiency syndrome is caused by
F-VEP) technique. mutations in SLC2A1 gene in most patients and results in
Methods: We studied 19 patients with IGE (16 women) impaired glucose transport into the brain. Its clinical
showing a photoparoxysmal electroencephalographic spectrum is heterogeneous.
response (PPR). 22 normal subjects of similar age and sex We describe a patient with a novel SLC2A1 gene mutation.
acted as controls (17 women). We recorded F-VEPs from Methods: This 14-years-old patient had a family history of
occipital and central electrodes. Stimuli were single flashes, febrile seizures and mental retardation in his mother. A mild
intermingled to flash pairs at the internal frequency of 3, 8, psychomotor delay was evident since childhood. At 8
16, 20, 30, and 60Hz. Recordings were done both with months: convulsive seizures in apyrexia. At 2 years he
closed and open eyes. The single F-VEP was split into a started an antiepileptic treatment. At 12 years he started
main complex and a late response, which were with diurnal episodes of myoclonic jerks at upper limbs and
measured separately. As to paired stimuli, the test F-VEP drop-head without consciousness impairment. At 13 years
emerged from electronic subtraction of the single F-VEP to presented almost continuous myoclonic face and upper
the paired F-VEP. Grouped data were analyzed by means of limbs jerks associated with an ideo-motor delay and
nonparametric analyses of variance (ANOVA). increase in epileptiform EEG abnormalities. His epilepsy
Results: In the eyes-closed state, the normal inhibition of was drug resistant.
the main complex of the test F-VEP was replaced by a Results: The neurological examination showed: facial
paradoxical facilitation, significant at the frequency of 16, dysmorphism, dysarthria, slow saccadic eye movements,
20 and 30Hz (p<0.05). This applied to the occipital as well hypotonus, dysdiachokinesia, upper limbs myoclonus,
as the central electrodes. The late response reacted in a weak deep tendon reflexes, mild ataxia, striae rubre.
similar way, with a significant (p<0.05) enhancement at 20 Psychometric evaluation confirmed moderate mental
and 30Hz (occipital electrodes) and at 20Hz (central retardation.
electrodes). In the eyes-open state no significant changes EEG disclosed frequent discharges of irregular spike/
in the main complex were detected. polyspike and waves diffuse over both hemispheres,
Conclusion: The paired F-VEP technique documents a increased during drowsiness, inconstantly associated with
defective inhibition in the visual system of photosensitive myoclonic jerks of the mouth and occasionally of neck
patients with IGE, whose features and timing likely underlie extensor. Brain CT and MRI were normal.
the PPR origin. He had altered palmitoyl sulfate and aldolase blood level.
Research for SCL2A1 mutation showed a heterozygous
nucleotidic deletion (c.130-135n of TACAAC) causing the
loss of 2 aminoacids of the protein (p.Tyr44_Asn45del).
Conclusion: We present a case of myoclonic epilepsy and
cognitive delay associated with a heterozygous nucleotidic
deletion of SLC2A1 gene, not previously described.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


242 Posters, Sunday 9 September

P1419 P1420
An epidemiological survey of epilepsy in Somatic co-morbidities in PWE and its
the Koung-khi department, western relationship with Quality of Life: the
Cameroon outlook in a developing economy
M.S.N. Dongmo1, C. Tume2, A. Tazoacha3 P.B. Adebayo1, S.A. Ogun2, A. Ogunniyi3
1Department of Biochemistry and Molecular Biology, 1Internal Medicine, Ladoke Akintola University, Ogbomoso,
University of Buea, 2Department of Biochemistry, University 2Internal Medicine, Olabisi Onabanjo University, Ago-
of Dschang, 3Department of Lofe Sciences, University of Iwoye/Sagamu, 3Internal Medicine, University College
Yaounde 1, Cameroon Hospital, Ibadan, Nigeria
Epilepsy is a chronic and invalidating neurological disease Introduction: Evaluating co-morbidity in people living
characterized by the repetition of seizures. The prevalence with epilepsy (PWE) is essential and its modification might
around the world is 50.000, this is generally higher in improve their Quality of Life. We aimed to determine the
developed than developing countries. In Africa, some profile of self reported somatic co-morbidities and its
epidemiological studies have been conducted in Mali, relationship with quality of life among PWE in Sagamu,
Uganda with different prevalence: 13.35 and 1.3% Nigeria.
respectively. In Cameroon a study conducted in the Mbam Methods: The Quality of Life in Epilepsy-31 item (version
Valley, revealed a prevalence of 49 one of the highest in 1.0) questionnaire was completed by 88 patients. The
the world. The koung-Khi division is one of the department presence and type of somatic co-morbidities was assessed
in Cameroon with a suspected prevalence of the disease, but using a performa check list. The Statistical Package for the
there is no data available. Hence the study conducted to Social Sciences (SPSS), version 16 was used for all
determine the prevalence of epilepsy and possible associated analyses.
causes. For the aim, a door to door survey was conducted in Results: 44 males and 44 females with mean ages (SD) of
the four villages of the department: Bandjoun, Bayangam, 31.82(13.58) and 31.68(10.98) years respectively were
Batoufam and Bandrefam The study revealed a prevalence evaluated. 66 (75%) of the PWE had no somatic co-morbid
which differs according to each locality with an overall rate condition while 18(20.5%) had only one co-morbid
of 7.1% in the department. Among the possible suspected to condition. Hypertension was the commonest co-morbidity
have a link with epilepsy were the type of food consumed, (8%). Acid peptic disease was found in 5(5.7%) patients.
genetic factors, itching. This study revealed the existence of Other co-morbid conditions included Asthma (1.1%),
the disease in the region, and the efforts made by epileptics pulmonary tuberculosis (1.1%) stroke (3.4%) and
and their families to ameliorate their wellbeing. Further osteoarthritis (1.1%). 4 (4.5%) had two concurrent
studies are needed for a better understanding of the co-morbid conditions comprising stroke and hypertension
management of epilepsy by populations, specially the use in three patients. There was no statistically significant
of medicinal plants for the cure of the disease. difference in the mean QoL scores between the groups with
or without concurrent co-morbid conditions.
Discussion: It appears that somatic co-morbidities have no
effect on the QoL of PWE in this study. A large comparative
study of the effect of psychological and somatic co-
morbidities on QoL of PWE in this population is desirable.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 243

P1421 P1422
Is there any difference between awake Risk of obesity during treatment of
EEG in benign rolandic epilepsy and epilepsy with valproate and possible role
continuous spike-and-waves during sleep of Pro12Ala PPAR2 polymorphism
syndrome? E.V. Krikova1, S.G. Burd2, A.S. Chukanova2,
K. El Alaoui Taoussi1, A. Satte2, J. Mounach2, H. Ouhabi2 M.A. Tushkanov3
1Clinical Genetics Laboratory, Mental Health Research
1Neurology B and Neurogenetics, Facult de Mdecine et de
Pharmacie de Rabat, 2Neurophysiology, Mohammed Vth Centre of Russian Academy of Medical Sciences,
2Department of Neurology and Neurosurgery, Russian State
Military Hospital of Rabat, Rabat, Morocco
Medical University, 3Molecular Genetics Diagnostic
Introduction: Benign rolandic epilepsy (BRE) and
Laboratory, A.N. Sysin State Research Institute of Human
continuous spike-and-waves during sleep syndrome
Ecology and Environmental Hygiene, Russian Academy of
(CSWS), are different entities that can have the same
Medical Sciences, Moscow, Russia
awakening electrical abnormalities. Diagnosis of both is
based on sleep EEG which needs long time recording and Background: Valproate (VPA), structurally analogue of
specialized structures. Our study aims to find a method to fatty acid, is one of the widely used antiepileptic drugs.
set apart the two syndromes on awake EEG. Based on the fact that several fatty acids have been
Methods: 15 awakening EEG records of 9 BRE and 6 identified as PPAR-2 ligands and common Pro12Ala
CSWS were compared. Electrical abnormalities were PPAR-2 polymorphism has been related to obesity, insulin
analyzed. The frequency and amplitude were calculated resistance, and risk of type 2 diabetes we hypothesized that
through a period of 20 minutes on awake EEG recorded at obesity, often observed during VPA treatment of epilepsy,
different day times. could be associated with the presence of polymorphic
Results: The 9 registered BRE includes 5 awake EEG variant of PPAR-2 gene and induced via insulin resistance
performed in morning and 4 in evening. For patients with mechanism.
CSWS, 4 awake EEGs were done in morning and 2 in Methods: The Pro12Ala polymorphism was genotyped in
evening. Biphasic abnormalities had the same appearance 69 subjects who fulfilled the diagnostic criteria of VPA-
in BRE and CSWS. Their average frequencies were treatment, taking the drug in a monotherapy mode for at
different between the two syndromes and during the day least 1 year. Insulin level and weight deviation were
with a statistically significant difference (p=0.02) between analyzed respectively.
the frequency of abnormalities recorded in the BRE in the Results: Mean insulin level in patients with wild Pro(C)
morning (9147 anomalies in 20 minutes) and that of allele without weight deviation was up to 2 times lower than
CSWS recorded the morning (19558 anomalies in 20 for Pro(C) group, exhibited weight gain (=0.00018). The
minutes) but there were no statistical difference between the statistical difference was also shown for Pro12 and Ala12
evening records. No statistical difference was found groups with obesity - mean insulin level was up to 2.4 times
between the amplitudes of the abnormalities in the various lower for Ala12 than for wild-type carriers (0.046), which
EEG. in fact may indicate the insulin-independent mechanism of
Conclusion: Our study suggests that diagnosis of BRE or obesity in the presence of Ala12 allele.
CSWS could be established on morning awake EEG by Conclusion: Positive association was shown for insulin
calculating the frequency of the biphasic spikes. However, level and weight gain in subjects with Pro12 allele, taking
the sample needs to be extended to confirm this hypothesis. VPA in a monotherapy mode. Clinically, these findings
could be the first prerequisite towards the rationales for the
value of combination of VPA treatment with omega-3 fatty
acids diet for patients with wild-type PPAR-2.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


244 Posters, Sunday 9 September

P1423 P1424
Can a nurse intervention promote the Clinical features and seizure semiology in
self-management of patients with children with malformations of cortical
established epilepsy who attend development
emergency departments? A nested M.I. Sigatullina, S.S. Shamansurov
qualitative study Child Neurology Department, Tashkent Institute of
Postgraduate Medical Education, Tashkent, Uzbekistan
A.J. Noble1, C. Virdi1, M. Morgan2, L. Ridsdale1
1Department of Clinical Neuroscience, Institute of Objective: To analyze the clinical features and seizure
Psychiatry, King's College London, 2Division of Health and semiology of Malformations of Cortical Development
Social Care Research, King's College London, London, UK (MCD) in children.
People with epilepsy (PWE) frequently attend emergency Material and methods: 45 children age 2 months till 14
departments (EDs). Attendance is associated with AED years with MCD were examined. Clinical, MRI, PET and
non-adherence, low knowledge of epilepsy and stigma. continuous video-EEG investigations have been performed.
Reducing unnecessary emergency hospitalisations could The types of MCD in patients: Focal Cortical Dysplasia
help patients and reduce costs. We are conducting a trial (FCD)-19, Hemimegalencephaly-7, Lysencephaly-7,
aimed to reduce epilepsy ED visits, by a self-management Polymicrogyria (PMG)-8, Shysencephalia-4.
intervention with an epilepsy nurse-specialist (ENS). We Results: Intractable Epilepsy was marked in patients with
here describe patients' perceptions of acceptability and FCD in 87% and hemimegalencephaly in 90%. In all
benefits. patients with Lysencephaly the muscular hypotonia and
Methods: N=85 adults with epilepsy were prospectively severe mental retardation were observed, in 2 patients-
recruited from 3 London EDs. The first 20 (age=41, SD=17; epileptic encephalopathy (West syndrome). In 6 children
10 males; median years since diagnosis=12) receiving the with precentral and perisylvian polymicrogyria motor
intervention were invited for semi-structured interview. deficiency in the form of a contralateral hemiparesis was
Transcripts were analysed thematically. observed. All patients with Hemimegalencephaly had
Results: Participants reported not having been confident in severe hemiparesis on the contralateral side, mental
managing their epilepsy, but that the intervention improved retardation and refractory epilepsy. All patients had the
this. It did this by meeting a need for epilepsy information, early debut of epileptic seizures. The spectrum of epileptic
with the ENS being available in a way GPs and consultants seizures was wide, from myoclonic (8), asymmetric tonic
are not: Responses included: It was the first time someone spasms (5), clonic (4) and complex partial seizures (10) to
had sat down and really told me a huge amount; she took generalized tonic-clonic (13) and polymorphic seizures as
the time to go through my needs, you felt you could ask Lennox-Gastaut (2) and West syndrome (in 3 patients). In
questions, not that wasting her time. Patients felt not so all patients with Hemimegalencephaly continuous slow
frightened of seizures. One said I don't feel as if I now activity in the involved hemisphere was registered of these
need to go to hospital every time. Learning about epilepsy and in 3 cases - burst-suppression pattern. In 5 patients
prevalence helped patients not feel so alone and less with severe progressive epileptic encephalopathy a
ashamed. They also felt AED adherence improved. functional hemispherectomy was performed with the result
Conclusions: This short intervention was acceptable to of 90% of seizure reduction in these patients.
PWE who attended ED. It satisfied a perceived need for Conclusion: In most cases a clear correlation was found
information and in turn increased confidence. We will between the type and location of the MCD and seizure
report whether the intervention leads to reduced ED use semiology which was also confirmed by the continuous
subsequently. Video-EEG register.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 245

P1425 P1426
Reversible homonymous hemianopia The syndrome gelastic seizures -
secondary to occipital lobe seizures hypothalamic hamartoma: description of
R. Sawaya, S. Daouk, W. Radwan, S. Hammoud a case study
Neurology, American University Medical Center, Beirut, M. De Vito, L. Santulli, M. Ianniciello, S. Striano
Lebanon University Federico II, Naples, Italy
Introduction: We report a case of acute homonymous Introduction: Hypothalamic hamartoma can be associated
hemianopia associated with simple partial seizures over the to a wide spectrum of epileptic conditions, from a mild form
contralateral parieto-occipital region. The hemianopsia with an urge to laugh and no cognitive impairment to a
subsided with control of the seizures. catastrophic encephalopathy with early onset gelastic
Material and method: A 53-year-old man, with controlled seizures, precocious puberty and mental retardation.
hypertension, presented with subacute visual hallucinations Surgical ablation and the use of other non-conventional
and distortion of visual perception associated with attacks techniques seem to be useful to revert epilepsy and
of gaze deviation and head rotation to the left. No loss of encephalopathy. The goal of the study is the observation and
consciousness. No focal motor symptoms. Neurological evaluation of the outcome of the patients followed at our
examination revealed only a left homonymous hemianopia centre.
which was confirmed by perimetry testing. Methods: We observed 10 patients (8 males and 2 female)
Results: MRI brain revealed diffuse cortical thickening and with hamartoma over about a 20-year period, very
high FLAIR signal intensity in the right parieto-temporo- heterogeneous for length of observation. 4 patient have a
occipital region with mild restriction in diffusion-weighted small hamartoma (< 1cm), 6 a large hamartoma (>1cm).
images without enhancement. No infarcts and no masses Among the patients with small hamartoma one has received
seen. MRA showed normal carotid and cerebral arteries. stereotactic implantation of radioactive I125 seeds and one
Visual perimetry revealed dense right homonymous was submitted to gamma-knife surgery. Among the patients
hemianopia. Blood studies were normal. CSF revealed with large hamartoma one was submitted to total resection,
elevated protein with normal IgG index, no cells and one to partial resection, 2 to gamma knife surgery.
negative bacteriology and virology results. EEG revealed Results: All patients developed drug-resistant focal or
near-status epileptic activity emanating from the right generalized epilepsy. Complete ablation of the hamartoma
parieto-occipital lobe. Upon treating the seizures with can stop and revert the epilepsy, the partial ablation is a
antiepileptic drugs the visual disturbances subsided and the temporary solution. Moreover long-term effect and efficacy
EEG normalized, but the hemianopia and MRI findings of gamma knife surgery and implantation of radioactive
persisted. The patient received 1 gram IV solumedrol which I125 need a better definition.
resolved the hemianopia and consequently the MRI Conclusions: Our study confirm the evolution of the
abnormality. syndrome towards a drug-resistant epilepsy. The surgical
Conclusion: We report that homonymous hemianopia can ablation of the amartoma can reverse epilepsy. The new
occur secondary to a seizure disorder without an underlying techniques request a long-term evaluation to establish the
infarct, tumour, or infection. It may be the result of focal real efficacy.
cortical oedema and reverses after the seizure is controlled
and oedema treated.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


246 Posters, Sunday 9 September

P1427 P1428
Predicting the epileptic seizure with beta Religious-spiritual coping in epileptic
rhythm of brain: a real time approach patients: correlation with clinical aspects
R.F. Ahmad1, A.S. Malik1, N. Kamel1, J.K.J. Tharakan2 G.M. Tedrus, L.C. Fonseca, G.C. Hoehr, F.D.P. Magri,
1Electrical & Electronics Engineering, Universiti Teknologi Neuropsicofisiologia em cognio e epilepsia
Petronas, Tronoh, 2Department of Neuroscience, School of Faculdade De Medicina, Pontificia Universidade Catlica
Medical Sciences, Universiti Sains Malaysia, Kota Bharu, de Campinas, Campinas, Brazil
Malaysia Introduction: How religiousness/ spirituality (R/S) acts on
Objective: Epilepsy is the brain disease that is second in the quality of life has been investigated, but there are few
number after stroke worldwide. Around 30% patients do not such studies in epileptic patients. The objective was to
respond to anti-epileptic drugs (AEDs). Therefore, seizure evaluate religious-spiritual coping and its relationship with
prediction can be helpful to improve the quality of life of clinical aspects in adult epileptics.
the patients having uncontrolled epilepsy. The study is Methods: 69 epileptic patients, (50.7% female), were
carried out in perspective to the real time monitoring and evaluated as follows: a clinical-neurological assessment and
prediction of the epileptic seizure. the Brazilian version of the RCOPE scale (CRE).
Method: Different methods are available to predict the Results: Mean total CRE:3.46, positive CRE:2.97, negative
seizure but still the results are not applicable for real-time CRE:2.40 and a negative/positive (N/P) ratio: 0.82 (0.16).
clinical application. The EEG is the major modality used for There was a significant negative correlation between
diagnosis of epilepsy. For real-time, the method scholastic level and the CRE scores (positive and negative
implemented is using the pre-processing, band pass filtering and the factors P1, P4, P6, P7, P8, N2), less years of formal
and observing its power spectrum. This method shows schooling corresponding to higher CRE values. The CRE
different brain rhythms during the pre-ictal and ictal period. scores were significantly greater in individuals who said
The SVM classifier is the best option for classifying the they only practiced one religion. There was negative
seizure occurrence or absence. correlation between the age of the first epileptic seizure
Results: The Physionet data set compiled by CHB and MIT (ES) and the factor N1 (Spermann Test: -0.242; p=0.047),
is used. The different rhythms of brain are observed for and between a greater ES frequency, a negative CRE
changes and synchronization. But the beta rhythm showed (-0.0292; p=0.034) and an N/P ratio of (-0.0292; p=0.021).
that during pre-ictal and ictal period, the beta power is The CRE (negative and factors) were significantly smaller
increased significantly than other frequency bands. in patients with generalized ES as compared to those with
Conclusion: The beta rhythm has shown significant focal ES. Other relationships were not significant.
changes during the pre-ictal period. Here, simple Conclusions: The results showed that the majority of
combinations of methods are used as compared to other patients showed high mean CRE scores (positive and
complex methods. The implementation requires less negative), which could portray aspects of the exercise of
computational power from hardware point of view. The spirituality. The R/S could be a link between health and
EEG signals for real-time can be acquired using wireless disease. Scholastic level correlated negatively with the R/S
EEG. This approach can be adapted towards real-time scale. There was a correlation between religious-spiritual
monitoring of epilepsy. coping and the frequency/ severity of the epilepsies.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 247

P1429 P1430
Correlation between religiousness/ Clinical semiology of psychogenic non-
spirituality and clinical aspects of epileptic seizures as a subset of post-
epilepsy: WHOQOL-SRBP traumatic stress related syndromes
G.M. Tedrus, L.C. Fonseca, G.C. Hoehr, F.D.P. Magri, L. Craciun1, G. Diaconu2,3, I. Mindruta4,5
Neuropsicofisiologia em cognio e epilepsia - FAPESP 1Neurology, Emergency Universitary Hospital Bucharest,
Faculdade De Medicina, Pontificia Universidade Catlica 2Psychiatry, Life Memorial Hospital, Bucharest, Romania,
3McGill Group For Suicide Studies, Douglas Hospital,
de Campinas, Campinas, Brazil
Introduction: Despite scientific evidence, questioning Montral, QC, Canada, 4Neurology, 5Epilepsy and Sleep
persists concerning the relationship between religiousness/ Monitoring Unit, Emergency Universitary Hospital,
spirituality (R/S) and epilepsy. The objective of this study Bucharest, Romania
was to correlate clinical aspects with R/S data in epileptic Introduction: Paroxysmal non-epileptic seizures (PNES)
patients. among patients referred to epilepsy centres are noticeably
Methods: 143 patients were evaluated as follows: a clinical- higher than in outpatient epilepsy population. The
neurological assessment, and WHOQOL-SRBP (World differential diagnosis between epileptic/non-epileptic
Health Organization Quality of life Spirituality, seizures can be difficult, and the mean latency between
Religiousness and Personal Beliefs) total and eight onset of PNES and adequate diagnosis is 7 years.
factors. Statistical tests were applied at p<0.05. Method: We retrospectively analysed 12 patients
Results: Mean total score WHOQOL-SRPB: 14.39 (2.25). considered to suffer from pharmacoresistant, difficult to
The scholastic level showed a significant negative classify epilepsy and finally diagnosed with PNES. The
correlation with WHOQOL-SRPB (factors 2; p=0.022 e patients underwent prolonged video-EEG monitoring in our
factors 6; p=0.02). Practicing religious individuals presented clinic and we recorded several seizure-like attacks. Patients
significantly higher WHOQOL-SRPB (p=0.47). The homemade videos displaying their habitual episodes were
individuals with more than one ES type and generalized ES also reviewed. The patients included in our study underwent
showed lower WHOQOL-SRPB values (p=0.02). psychiatric evaluation based on a standardized approach.
Significantly lower values were observed for WHOQOL- Results: We finally were able to define 4 clinical patterns:
SRPB in idiopathic generalized epilepsy (p=0.03). The hypermotor like, tonic/tonic-clonic like, absence-like,
occurrence of psychiatric co-morbidity was associated with atonic like with sudden fall. Particular attention was
lower WHOQOL-SRPB (factors 2 and 6) p=0.02. captured by a case displaying voluntary respiratory arrest
Conclusions: Scholastic level correlated negatively with during prolonged episodes, in a context of absence-like
the R/S scales. There was negative correlation between R/S status.
and the frequency/ severity of the epilepsies. High R/S Conclusion: The whole group showed signs of chronic
values can portray aspects of the practice of spirituality and depression and had a significant history of past traumatic
confirm studies that suggest that the R/S of epileptic patients events that were symbolically linked with the type of
differ in context and intensity. seizure-like activity, such as a history of repeated physical
and/ or sexual abuse, loss of a close member of the family
at an early age or exposure to family separation during the
attachment-forming period (0 - 8 yrs). We inferred that the
type of ictal activity may be operationalized as trauma
re-enactment process by which the patient re-lives the initial
event. Such cases may be re-defined as subsets of post-
traumatic stress-related syndromes.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


248 Posters, Sunday 9 September

P1431 P1432
Epileptic patients have an increased risk Does geographic region impact the
of carbamazepine toxicity during triple efficacy of retigabine/ezogabine in adults
therapy for Helicobacter pylori infection: with partial-onset seizures? Analysis of
a case presentation and review of the Central/Eastern Europe, Western Europe
literature and the rest of the world
S. Sabetay, L. Kaplan, B. Weller C. Tessier1, S. McDonald2, W.-J. Lee2, S. Derossett2
Neurology, Bnai Zion Medical Centre, Haifa, Israel 1GlaxoSmithKline, Middlesex, UK, 2GlaxoSmithKline,
Introduction: Over 50% of the world population harbours Research Triangle Park, NC, USA
H.pylori in their upper gastro-intestinal tract. H.pylori Introduction: Retigabine/ezogabine is effective and
testing is recommended especially in persons suspected generally well tolerated as adjunctive therapy in adults with
with peptic ulcer disease. Triple therapy administered in partial-onset seizures. The efficacy of retigabine/ezogabine
peptic ulcer disease patients with positive H.pylori tests by geographic region is reported.
includes proton pump inhibitors, the antibiotics Methods: A post-hoc analysis was conducted using data
clarithromycin and amoxicillin. When starting triple therapy from the double-blind, placebo-controlled trial, Study 302
in epileptic patients the drug-drug interactions between (NCT00235755), to evaluate responder rates (50%
clarithromycin and proton pump inhibitors on one hand and reduction in seizure frequency) during treatment
carbamazepine on the other hand are sometimes overlooked, maintenance and percentage change in 28-day total partial-
predisposing to carbamazepine toxicity. seizure frequency from baseline to the double-blind period.
Methods: Male, aged 58, with generalized tonic-clonic Geographic regions included Central/Eastern Europe
epilepsy treated for two decades with carbamazepine and (Hungary/Poland/Russia/Ukraine), Western Europe
phenytoin presented with acute ataxia and vertigo. No (Belgium/France/Germany/Spain/UK) and rest of the world
previous adverse effects related to his antiepileptic (ROW) (Australia/Israel/South Africa/USA).
medications were reported and drug blood levels were Results: Compared with placebo, retigabine/ezogabine 600
always in therapeutic range. A week prior triple therapy and 900mg/day increased responder rates in patients in
(omeprazole, amoxicillin, clarithromycin) for peptic ulcer Central/Eastern Europe (placebo [n=70], 19%; retigabine/
disease and positive H.pylori testing was started. Toxic ezogabine 600mg/day [n=73], 47%; and 900mg/day [n=64],
carbamazepine blood levels in ER were obtained and the 48%), Western Europe (placebo [n=45], 16%; retigabine/
drug was temporarily discontinued. Clarithromycin- ezogabine 600mg/day [n=50], 22%; and 900mg/day [n=40],
carbamazepine, omeprazole-carbamazepine drug 43%) and ROW (placebo [n=49], 22%; retigabine/
interactions were considered as causing carbamazepine ezogabine 600mg/day [n=42], 46%; and 900mg/day [n=49],
toxicity, therefore triple therapy was interrupted and, after 49%). Retigabine/ezogabine also reduced 28-day total
two days, the patient was asymptomatic and carbamazepine partial-seizure frequency compared with placebo in patients
levels returned to therapeutic range. in Central/Eastern Europe (median: placebo [n=74], 16.5%;
Results: Carbamazepine- clarithromycin and retigabine/ezogabine 600mg/day [n=79], 37.8%; and
carbamazepine-omeprazole interactions determined acute 900mg/day [n=75], 42.7%), Western Europe (median:
carbamazepine toxicity in our patient, symptoms placebo [n=47], 10.3%; retigabine/ezogabine 600mg/day
disappearing following interruption of the triple therapy. [n=58], 21.8%; and 900mg/day [n=51], 37.2%) and ROW
Conclusion: Carbamazepine toxicity represents a definite (median: placebo [n=55], 15.8%; retigabine/ezogabine
risk for patients started on triple therapy for peptic ulcer 600mg/day [n=42], 27.2%; and 900mg/day [n=49], 39.4%).
disease due to drug- drug interactions. This risk should be No significant difference on retigabine/ezogabine efficacy
known by neurologists and gastroenterologists, and dose- by the region was observed (p-value for interaction >0.3).
reduction of carbamazepine could be considered during Although some differences in baseline characteristics
triple therapy for H.pylori. among regions were observed, these differences did not
significantly affect retigabine/ezogabine efficacy.
Conclusion: Consistent with the overall results, retigabine/
ezogabine as adjunctive therapy demonstrated efficacy in
adults with partial-onset seizures in Central/Eastern Europe,
Western Europe and ROW.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 249

P1433 P1434
Stereotactic radiofrequency Therapeutic changes in patients on anti-
amygdalohippocampectomy for the epileptic polytherapy: looking for the
treatment of temporal lobe epilepsy: do "winner" combination
good neuropsychological and seizure V. Hernando-Requejo, N. Huertas, N. Juarez, A. Diez
outcomes correlate with hippocampal Neurology, University Hospital Severo Ochoa, Leganes,
volume reduction? Spain
H. Malikova1, L. Kramska2, R. Liscak3, Z. Vojtech2 Introduction: To analyze the clinical outcome of different
1Radiology, Neurology, Stereotactic and Radiation
2 3 antiepileptic drugs (AED) combinations in patients taking
Neurosurgery, Na Homolce Hospital, Prague, Czech at least three AED.
Republic Methods: Review of the neurological history of the last 668
patients attended in our Epilepsy Outpatient Clinic.
Introduction: Temporal lobe surgery bears the risk of a Results: From 42 patients on three or more AED, we
decline of neuropsychological functions. Stereotactic reviewed 151 AED combinations. Number of combined
radiofrequency amygdalohippocampectomy (SAHE) AED: 3.3 (2-6).
represents an alternative to mesial temporal lobe epilepsy Outcomes: Improvement (seizure frequency): 54
(MTLE) surgery. This study compared neuropsychological combinations (35.8%, the "good outcome" group, with
results with MRI volumetry of the residual hippocampus. follow up longer of 6 months in more than 90%), in 46
Materials and methods: We included 35 patients with (30,5%) seizure frequency didn't change, in 29 (19.2%)
drug-resistant MTLE treated by SAHE. MRI volumetry and increased the frequency (the "bad outcome" group), in 22
neuropsychological examinations were performed before (14.6%) the treatment failed due to adverse events.
and 1 year after SAHE. Each year after SAHE clinical Reduction of seizure frequency ("good outcome" group):
seizure outcome was assessed. 81.2% (reduction over 50% of seizure frequency: 81.8%),
Results: One year after SAHE 77% of patients were in 8 combinations (5.3%) seizure freedom was obtained. We
assessed as Engels Class I, 14% of patients were classified did not find statistical differences in the distribution of AED
as Engels II and in 9% of patients treatment failed. Two in both groups (good" and "bad" outcomes): p=0.269.
years after SAHE 76% of subjects were classified as Engels Lamotrigine was present in 10.29% of the "good outcome"
Class I, 15% of patients was assessed as Engels II and in and in 5.56% of "bad outcome" combinations (p=0.195),
9% of patients treatment failed. The hippocampal volume this difference is not related to the synergy of combination
reduction was 5817% on the left and 5427% on the right of lamotrigine and valproic acid (associated in 33% of
side. One year after SAHE the intelligence quotients of combinations in the "good outcome" and in 40% in the "bad
treated patients increased. Patients showed slightly outcome" group).
significant improvement in verbal memory (p=0.039) and Conclusion: Our data support the idea, less pessimistic than
semantic long-term memory subtest (LTM) (p=0.003). classically accepted, that active intervention in AED
Patients treated on the right side improved in verbal polytherapy can lead to good outcomes. Lamotrigine is
memory, delayed recall and LTM. No changes in memory slightly more present in combinations with good outcome,
were found in patients treated on the left side. There was a but in our series we find only a tendency.
trend between the larger extent of the hippocampus
reduction and the improvement in visual memory in speech-
side operated (p=0.057). No other relationships were
significant.
Conclusion: SAHE causes partial hippocampal destruction,
but seizure outcome is comparable with open surgery and
moreover SAHE does not cause neuropsychological
deficits.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


250 Posters, Sunday 9 September

P1435 P1436
Bone health in epilepsy: are we doing Epilepsy and Brugada syndrome
enough? A. Velazquez1, M.P. Gil Villar2, C. Garcia Arguedas1,
K. O'Connell, C. McGuigan C. Gonzalez Mingot2, R. Bascompte3,
Neurology Department, St Vincent's University Hospital, T. Corbaln Sevilla1
Dublin, Ireland 1Neurology, Lozano Blesa Hospital, Zaragoza, 2Neurology,
Arnau De Vilanova Hospital, Lerida, 3Cardiology, Lozano
Background: Recent publications support an association
Blesa Hospital, Zaragoza, Spain
between epilepsy, long-term anti-epileptic drug (AED) use
and osteoporosis. Poor bone health in people with epilepsy Introduction: Brugada syndrome is a cardiological
is multifactorial although there is evidence that AED channelopathy with some typical EKG signs and high risk
directly affect bone metabolism particularly enzyme of sudden death. It is put in relation with some epilepsy
inducing drugs and sodium valproate. This study was syndromes in the literature. We report two patients with
conducted to assess if high risk patients are being identified both pathologies.
and managed appropriately. Material and methods:
Methods: An audit of the medical records of consecutive First patient: 49-years-old male. Family history of sudden
patients attending an outpatient department with epilepsy death and personal history of febrile seizures in his
was performed. Patient demographics, AED history, fracture childhood. He was found unconscious with seizures while
history, previous DXA scanning and current bone protection he was sleeping. The urgent EKG showed Brugada signs
medication was recorded. (right precordial ST segment elevation). The EEG was also
Results: 79 patient records were reviewed. No fractures abnormal, showing right temporal spikes focus. The
were documented, 8 (10%) had been screened with a DXA flecainide test was positive, and all the other cardiological
scan and 12 (15%) were on bone protection medication. and neurological tests were unremarkable. Since then, he
Of the female patients 10 (27%) were over 50, 4 (40%) had started levetiracetam treatment and no more seizures have
a DXA scan and 5 (50%) were on bone protection. 7 were been reported.
on enzyme inducing drugs, 2 (29%) had a DXA and both Second patient: 37-years-old male, without family or
were on bone protection. personal history of seizures or heart disease. He had a first
39 (50%) patients were on either enzyme inducing AED or episode of tonic-clonic movements with post-ictal
sodium valproate for more than 5 years and only 8 (21%) confusion. All the neurological test were normal except the
had been screened. EEG, which showed generalized spikes and waves
Conclusion: There is no consensus with regard to screening discharges. The urgent EKG and the ajmaline test were
and treatment of AED induced osteoporosis. DXA scanning positive for Brugada syndrome patterns. He started
is the gold standard screening tool. Identifying at risk carbamazepine and an automatic implantable cardioverter
patients needs to encompass independent risk factors for defibrillator was placed.
osteoporosis including age and sex in combination with Conclusions: Many ion-channel pathologies have been
AED, duration of treatment and seizure control. The described, such as heart diseases (Brugada syndrome, long
relatively low screening rate across our patient group would QT syndrome) and neurological disorders (including many
suggest a need for improved awareness of this condition. epilepsy syndromes). In fact, there are some mutations in
the sodium channels like SCN5A and SCN1A-B that are
linked to Brugada syndrome and generalised epilepsy with
febrile seizures. Channelopathies can coexist in the same
patient and make the differential diagnosis and the treatment
more difficult.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 251

P1437 P1438
FET-PET visualizes increased cortical Glutamic acid decarboxylase auto-
neutral amino acid-uptake after epileptic antibodies in intractable temporal lobe
seizure epilepsy and juvenile myoclonic epilepsy
Y. Krenn1, S.M. Golaszewski1,2,3, A.B. Kunz1, S. Elibirlik, A. Dirican, S. Baybas, V. Sozmen,
M. McCoy4, B. Egger5, E. Trinka1,2,3, M. Hutterer1 M. Ozturk, N. Demir, E. Arslan, O. Soydemir,
1Department of Neurology, 2Spinal Cord Injury and Tissue
H. Kucukoglu
Regeneration Center, 3Neuroscience Institute, 4Division of Neurology, Bakrky Research and Training Hospital for
Neuroradiology, 5Department of Nuclear Medicine, Neurologic and Psychiatric Diseases, Istanbul, Turkey
Paracelsus Medical University Salzburg, Salzburg, Austria Purpose: High glutamic acid decarboxylase auto-antibodies
Objective: Molecular imaging with positron emission (anti-GAD) levels were reported in patients with intractable
tomography (PET) has become essential in the management epilepsy in some studies. In this study, we investigated the
of brain tumours. High amino acid uptake in biological role of anti-GAD antibodies in the etiopathogenesis of
active tumour tissue allows the detection of neoplastic intractable mesial temporal lobe epilepsy (MTLE) and
growth via radiolabeled amino acid tracers. One of the most compared these results with juvenile myoclonic epilepsy
important tracer of this group is [18F]-Fluoro-Ethyl- (JME) patients and healthy subjects.
Tyrosine (FET). However, little is known about the Method: The study group consisted of 26 patients with
specificity of FET-uptake. MTLE, 28 patients with JME and 26 healthy subjects as
Case report: We present two cases in which FET-PET control group. Anti-GAD levels were determined
shows significantly increased amino acid metabolism after quantitatively by Immunoradiodiagnostic Assay (IRMA).
epileptic events: A 44-year-old man with symptomatic Number Cruncher Statistically System (NCSS) 2007&PASS
epilepsy due to a left insular secondary malignant 2008 Statistical Software (Utah, USA) program was used
astrocytoma WHO grade II (in complete remission) entered for statistical analysis.
a prolonged postictal state after a complex focal seizure. Results: The serum anti-GAD levels were not different in
FET-PET revealed an extended and exclusively cortical MTLE, JME and the control group. Anti-GAD was positive
enhancement left frontal and left insular. The signal in 3 JME cases and in one control subject. Statistical
completely resolved with symptom remission after 2 analysis did not show any significant difference between
months. In a 58-year-old woman with an acute symptomatic anti-GAD levels and demographic data, medical history,
simple focal seizure due to a right-cerebral ischemic family history, disease duration, seizure types and frequency,
infarction, FET-uptake was enhanced in the right temporal antiepileptic drugs, history of status epilepticus and EEG
cortex next to the white matter infarction. findings.
Conclusion: It can be assumed that the discharge of Conclusion: Although we did not find any statistically
neurotransmitters during an epileptic event is responsible significant relationship of anti-GAD levels in our study
for an increased uptake of amino acids in the postictal groups, some studies reported that GAD autoimmunity may
phase. The latter can be visualized by FET-PET imaging. be associated with refractory localization-related epilepsies.
Studies with higher number of patients must be performed
to determine the prevalence and the role of anti-GAD
antibodies in the etiopathogenesis of intractable epilepsy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


252 Posters, Sunday 9 September

P1439 P1440
Intravenous lacosamide in status Resting motor threshold in idiopathic
epilepticus and cluster seizures generalized epilepsies: a systematic
Y. Handouk, S. Morresi, C. Bomprezzi, C. Minardi, review with meta-analysis
S. Malag, A.M. Mauro F. Brigo1,2, M. Storti3, F. Rossini1, R. Nardone2,4,
U.O. Neurologia Ospedale 'Bufalini', Cesena, Italy F. Tezzon2, A. Fiaschi1, L.G. Bongiovanni1,
Introduction: Some anti-epileptic drugs (AEDs) used in P. Manganotti1
status epilepticus (SE) are associated with potential 1Department of Neurological, Neuropsychological,

respiratory and cardiovascular complications. Lacosamide Morphological and Movement Sciences, University of
(LCM), a new AED, has an intravenous formulation with a Verona, Verona, 2Department of Neurology, Hospital Franz
potential role in the treatment of SE. Tappeiner, Merano, 3Department of Medicine, University of
Objective: To test the efficacy and safety of the intravenous Verona, Verona, Italy, 4Department of Neurology, Christian
drug formulation in SE and cluster seizures. Doppler Clinic, Paracelsus Medical University, Salzburg,
Patients and methods: 11 patients with symptomatic Austria
epilepsy (6 female and 5 male; mean age 66 (23-90); 8 with Introduction: Resting motor threshold (rMT) assessed by
SE and 3 with cluster seizures; were treated, after failure of means of Transcranial Magnetic Stimulation (TMS) is
other AEDs, with simultaneous administration of thought to reflect trans-synaptic excitability of cortico-
intravenous and oral LCM for two days (200mg intravenous spinal neurons. TMS studies reporting rMT in idiopathic
+ 50mg oral LCM twice daily), then switched to oral LCM generalized epilepsies (IGEs) yielded discrepant results, so
(400mg daily). that it is difficult to draw a definitive conclusion on cortico-
Results: EEG improvement occurred in the first 24 hours spinal excitability in IGEs by simple summation of previous
after the beginning of LCM therapy. All patients were results regarding this measure. Our purpose was to carry out
responsive with no serious adverse event, except two old a systematic review and a meta-analysis of studies
patients who died for severe comorbidities. evaluating rMT values obtained during single-pulse TMS in
Conclusions: LCM could be efficacious and safe in the patients with IGEs.
treatment of SE. Methods: Controlled studies measuring rMT by single-
pulse TMS in drug-naive patients older than 12 years
affected by IGEs were systematically searched and
reviewed. rMT values were assessed calculating mean
difference and odds ratio with 95% confidence intervals
(CI).
Results: 14 trials (255 epileptic patients and 424 controls)
were included. Patients with Juvenile Myoclonic Epilepsy
(JME) have a statistically significant lower rMT compared
with controls (mean difference: -6.78; 95% CI -10.55 to
-3.00); when considering all subtypes of IGEs, there was a
tendency toward reduced rMT values in IGEs, but the
difference did not reach statistical significance (mean
difference: -2.34; 95% CI -4.92 to -0.24).
Conclusion: Overall considered, the results are indicative
of a cortico-spinal hyper-excitability in JME, providing not
enough evidence for motor hyper-excitability when all
subtypes of IGE are considered as a whole. The considerable
variability across studies probably reflects the presence of
relevant clinical and methodological heterogeneity, and
higher temporal variability among rMT measurements over
time, related to unstable cortical excitability in these
patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 253

P1441 P1442
EEG photosensitivity phenomenon among Retrospective clinical and
children without epileptic seizure electrophysiological analysis of 33
I. Shchederkina surgically treated patients with
Neurology Clinic of Morozovsky Children Hospital, Moscow, pharmaco-resistant epilepsy
Russia
A.R. Beatriz
In some people, without epileptic seizure, generalized Clinical Neurophysiology, Hospital General Universitario
polyspike-wave activity under photo-stimulation Gregorio Maran, Ciudad Real, Spain
(electrophysiological photosensitivity) is registered in EEG. Objective: Analysis of clinical manifestations, ictal,
The frequency of such cases in population may reach 1.8%. interictal and intraoperative EEG monitoring in patients
Aim: To evaluate the frequency of photoparoxysmal with medical treatment refractory temporal epilepsy
response (PPR) in EEG for children with different successfully controlled surgically.
complaints. Methods: Retrospective analysis, 33 patients. Mean age
Methods: Clinical, EEG, video-EEG-monitoring. 3611.40; number of monthly seizures: 914.23 mean
Results: 44 children with PPR were examined with EEG: duration of disease: 22 years; number of anti-epileptic
22 boys and 22 girls. Mean age 7.5 years. Reasons for drugs: 30.93. Average monitoring duration on Intensive
seeing a neurologist were: tension headache - 31.8%, video-EEG Monitoring Unit: 6.423.61 days. Scalp EEG
vegetative dysfunction (syncope) - 27.2%, motor tics - was recorded with surface electrodes placed according to
22.7%, migraine - 20.4%, head trauma - 18.1%, other the 10-20 International System, plus additional 10-10
(attention deficient hyperactivity disorder, logoneurosis, System and sphenoidal electrodes to record medial temporal
retrocerebellar cyst, neuroinfection, learning disability) and temporobasal regions. In 12 patients, subdural
were represented with identical frequency of 4.5%. Girls electrodes were necessary.
were suffering under migraine twice as often, tics prevailed Results: 158 seizures were recorded, most of them focal
among boys. Syncopes were registered more often among complex. 82% of patients presented aura (36.1% gastric).
females. 77% of children had neurological Disturbances of consciousness appeared in almost 100%
microsymptomatics. Isolated PPR was registered for 42% (94.3%), being more precocious than the automatisms in the
of children, the rest showed a combination of this EEG majority of the sample. The most frequent and precocious
phenomenon with other types of epileptiform activity automatisms were oromandibular. A low percentage of
(sharp(spike)-slow wave): generalized in background contralateral dystonic position was observed, perhaps
record and at hyperventilation - 16%, with frontal explained by the detection criteria that were used to.
localization - 6%, with temporoparietal localization, with Intercritical: epileptiform activity was observed in 87.9% of
occipital localization - 2%. Patient surveillance period cases; sharp waves in 93.1%., increasing with sleep in 97%.
varied from 9 months to 4 years with occasional video- Polyspikes during REM sleep in 21.2%. Bilateral
EEG. During the surveillance period none of the patients epileptiform activity in 21.2%. Ictal activity: Changes on
had an epileptic seizure. EEG activity was observed previous to clinical manifestation
Conclusion: In order to rule out subclinical seizures, the in more than 69,1%. Type of ictal onset: flattening 46%,
carrying out of video-EEG monitoring with additional trials rhythmic slow activity 41.7%. Ictal onset was focal in
is necessary. Photoparoxysmal response at EEG is more 48.9% (posterior lateral temporal 16.5%, frontotemporal
often combined with generalized epileptiform activity on 15.1%).
background and in case of hyperventilation. Anticonvulsant
prescription is unreasonable unless epileptic seizures occur.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


254 Posters, Sunday 9 September

P1443 P1444
Epileptogenic potential of supratentorial Clinical features and follow-up of patients
brain tumours in paediatric patients with infantile spasms
V.S. Khalilov, A.A. Kholin M.J. Gonzalez1, F.X. Sanmart2, I. Alonso Colmenero3,
Department of Neurology, Neurosurgery and Medical O. Sans3, J. Campistol4
Genetic, Russian State Medical University, Moscow, Russia 1Child Neurology Department, 2Epilepsy Unit, Barcelona
Purpose: Symptomatic epilepsy is common in patients with University Hospital Sant Joan de Deu, 3Neurophysiology
supratentorial brain tumours. Epileptogenesis depends on Unit, 4Child Neurology, Barcelona University Hospital Sant
several factors including histology, location, changes in the Joan de Deu, Barcelona, Spain
peritumoral parenchyma and genetic predisposition. The Introduction: The frequency of infantile spasms (IS) is
aim of presented research was studying epileptogenic about 2% among the epilepsy in the children and about 25%
potential of brain tumours of different histology in children. occur in the first year of life. The incidence peak is between
Method: Among the paediatric patients investigated and 4 and 7 months of age.
treated in the Neurosurgical Department and Psycho- Objective: To describe the main clinical features, ictal and
Neurological Department 2 of Russian Children Clinical interictal video-EEG findings in 38 patients with infantile
Hospital at the period 2006-2011 60 children with spasms.
supratentorial brain tumours were revealed. Results: The mean age of beginning of the infantile spasms
Result: The group of patients (n=60) consists of 28 girls was 5.1 month. In 18 patients the infantile spasms had
and 23 boys (age range from 5 month till 17 years). 41 connection with concrete etiology and had relationship with
patients (68,3%) had symptomatic epilepsy. Among the severe degree of delayed psychomotor development. The 8
patients were revealed: fibrillary astrocytoma 8 cases (all 8 patients with infantile spasms which did not have another
patients had epilepsy), pilocystic astrocytoma 6 (3), type of seizures and Hypsarrhytmia pattern during video-
pleomorphic xanthoastrocytoma 5 (4), desmoplastic EEG evaluation had better response to Vigabatrin treatment.
infantile astrocytoma (DIA) 1 (0), protoplasmic astrocytoma In 79% the interictal EEG was abnormal and in 73% of
2 (1), ganglioastrocytoma 5 (5), mixed glioma 3 (1), mixed patients were observed abnormal sleep patterns. Only in 2
ganglioglioma 2 (1), anaplastic ganglioglioma 1 (0), patients during continuous video-EEG monitoring
desmoplastic ganglioglioma 1 (1), glioblastoma multiforme hypsarrhytmic activity was registered. The improvement of
2 (2), olygodendroglioma 1 (0), anaplastic ependymoma 6 the video-EEG findings were observed during 6 months
(3), choroid plexus papilloma 3 (0), atypical choroid plexus after first record in 18 patients. In 48.5% of patients the
papilloma 1 (1), choroid plexus carcinoma 1 (0), MRI was normal in the first examination. 27 patients were
dysembryoplastic neuroepithelial tumour (DNET) 5 (5), treated with corticosteroids with a good response in 60%.
primitive neuroepithelial tumour (PNET) 1 (1), atypical During the long follow-up 25 patients had delay of
meningioma 1 (1), thalamic hamartoma 3 (3), psychomotor development and in 22 patients other type of
meningioangiomatosis 2 (2). seizures appeared later.
Conclusion: Tumour types demonstrating obligate Conclusion: The most important factors of the good
epileptogenesis were: fibrillary astrocytoma, outcome in patients with infantile spasms are early diagnosis
ganglioastrocytoma, DNET, PNET, hamartoma, and effective treatment according to the etiology of the
meningioangiomatosis, desmoplastic ganglioglioma, spasms.
glioblastoma multiforme and atypical meningioma.
Pleomorphic xanthoastrocytoma was epileptogenic in 80%
of cases. Pilocystic and protoplasmic astrocytoma, mixed
ganglioglioma, anaplastic ependymoma had 50% risk of
epilepsy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 255

P1445 P1446
The selected NR1I2 polymorphism The peculiarities of post-stroke epileptic
rs1523130 T>C and rs2472677 C>T as a seizures related to post-stroke time
risk factor of drug resistant epilepsy T. Danilova, D. Khasanova, I. Kamalov
K. Jastrzebski, I. Klich, A. Klimek Neurology, Kazan State Medical University, ICDC -
Medical University of Lodz, Poland Interregional Clinical Diagnostic Center, Kazan, Russia
Introduction: The NR1I2 nuclear receptor subfamily 1, The aim of the study is to establish the clinical and
group I, member 2 (or PXR - Pregnane X Receptor) gene functional peculiarities of seizures-precursors (SP), early
product belongs to the nuclear receptor superfamily, (within the first 7 days) (ES) and late post-stroke seizures
members of which are transcription factors characterized by (LS). A total of 222 patients with ischemic stroke who
a ligand-binding domain and a DNA-binding domain. The experienced different types of epilepsy were under integral
PXR protein is a transcriptional regulator of the cytochrome medical supervision. The diagnostic complex comprised
P450 gene and ABC - transporters. PXR is expressed clinical analysis, MRI, EEG, extracranial and transcranial
mainly in the liver and intestine, where it plays important Doppler sonography. The simple partial seizures (45.3%,
roles in detoxification. p<0.01) developed more frequently in patients with ES, as
Methods: A total of 29 patients with drug resistant epilepsy compared to the ones with LS and SP. The secondary
and 185 healthy controls from central Poland were analyzed generalized seizures (35.7%, p<0.05) dominated in patients
for the two PXR polymorphisms. The diagnosis of drug with LS. SP (41.4%) and ES (49.5%) occurred more
resistant epilepsy was consistent with consensus Tasks frequently in patients with ischemic stroke in the left carotid
Force of the International League Against Epilepsy (ILAE). basin as compared to the right carotid one (34.5% and
The polymorphisms were detected by TaqMan RT-PCR 36.9%, p<0.05). LS developed in the left (43.6%) and right
assay. The results were tested by Chi-square and Fisher (47.5%) carotid basins with equal frequency. The SP often
tests. developed in patients with lacunar stroke (34.5%, p<0.01).
Results: The distribution of haplotypes between cases and The ES were more common in patients with cardioembolic
control was not important in difference and consistent with stroke (30.5%, p<0.05), the LS occurred equally in all types
Hardy-Weinberg equilibrium (cases: rs1523130T>C: CC - of stroke. More frequent occurrence of seizures in the
38%, CT - 41.3%, TT - 20.7% vs. control CC - 35.2%, CT dynamics in patients with SP (27.6%) and LS (24.5%) was
- 46.8%, TT - 16.2%, cases: rs2472677C>T: CC - 20%, CT observed more often. Patients with ES showed decrease in
- 45%, TT - 35% vs. control CC - 14.8%, CT - 48%, TT - frequency of seizures over time (32.6%, p<0.05).The focal
37.2%). The distribution of haplotypes CC/CC vs. others epileptiform and slow-wave activity were more typical in
haplotypes was almost statistical important - OR- 6.8519 patients with ES (60.2%) as compared to the late ones
(CI 95% 0.9262 to 50.6879). (53.6%) and SP (41.9%).
Conclusion: Our preliminary results indicate that genetic Thus, the revealed clinical manifestations of seizures
variability in PXR gene can play an important role in drug associated with different periods of stroke may indicate
resistant epilepsy but further studies are now required to their different pathogenesis.
define the significance of these associations.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


256 Posters, Sunday 9 September

P1447 P1448
Clinical and EEG findings in idiopathic Impact of regular counselling on epilepsy
generalized epilepsies: are there any clinic default: the Nigerian experience
distinctive features to distinguish E.O. Ezunu1, M. Owolabi2
syndromes? 1Medicine, Federal Medical Center, Asaba, 2Medicine,

University College Hospital, Ibadan, Nigeria


A. Asadipooya, M. Emami, A. Karimpourghanadi
Shiraz University of Medical Sciences, Shiraz, Iran Introduction: Epilepsy is one of the leading brain disorders
Background: Epidemiologic data concerning idiopathic in the developing countries. However, the impact of
generalized epilepsies (IGEs) are scarce or often unreliable. education on clinic attendance and drug compliance on
In the current study, we specifically analyzed the syndromes patients with epilepsy is unknown in our environment.
if IGE based on their demographic, clinical, and EEG Methodology: This is a prospective study of subjects with
findings. epilepsy seen at the outpatient neurology clinic, University
Materials and methods: In this retrospective study, all College Hospital, Ibadan Nigeria. A total of 184 (37 new
patients with a clinical diagnosis of IGE were recruited in and 147 old) epileptic patient were recruited consecutively.
the outpatient epilepsy clinic at Shiraz University of A standard questionnaire for obtaining information on their
Medical Sciences, from September 2008 through May demographic characteristics, monthly clinic attendance was
2011. Demographic variables and relevant clinical and EEG initially applied to each of the old patients without
variables were summarized descriptively. Statistical counselling. The extent of counsel they might have received
analyses were performed using Chi square and Fishers was evaluated. The new patients were separated and
Exact tests. counselled monthly on the need to attend clinic regularly.
Results: 2190 patients with epilepsy were registered at our The two groups were followed up monthly for six months,
epilepsy clinic and 442 patients (20.2%) were diagnosed as and then their clinic attendance was assessed. Informed
having IGE. Age of seizure onset was 127 years. Juvenile consent of the patients was obtained; ethical clearance from
myoclonic epilepsy was the most frequent syndrome, University of Ibadan/University College Hospital Ibadan
followed by epilepsy with generalized tonic-clonic seizures Ethical Committee was also approved.
only and juvenile absence epilepsy. Epilepsy risk factors, Results: 32 new cases (86.5%) attended the clinic as
physical examination, MRI and even EEG could not scheduled and were compared with 21 old cases (14.3%),
differentiate various syndromes. Chi2= 75.14, OR=38.40, p<0.0001, 95% CI=13.44-109.70.
Conclusion: The key element in making the correct Conclusions: Regular counselling has a positive impact on
diagnosis of an IGE syndrome is obtaining a detailed clinic default of epilepsy patients in our environment.
clinical history. However, sometimes these syndromes have Therefore, health care providers should provide regular
overlapping features. counsel to their patients in the clinic.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 257

P1449 P1450
Significance of metal content monitoring Can vitamin B12 and folate supplements
in children with epilepsy prevent the rise of serum homocysteine in
O. Guzeva epileptic patients receiving
Neurology Department, Saint-Petersburg Paediatric Medical carbamazepine?
Academy, Saint-Petersburg, Russia
M. Motamedi, S. Ahmadi Karvigh, A. Naser Moghadasi
Introduction: Metals play an important role in the Neurology, Tehran University of Medical Sciences, Tehran,
metabolism of the nervous system, including activation of Iran
enzyme reactions. Objectives: To assess the level of the serum homocysteine
Purpose: To study metal content in children with epilepsy in epileptic patients, who are under carbamazepine
in comparison with normal parameters. treatment and are also receiving Vitamin B12 and folate
Materials and methods: The content of 11 metals (Pb, Cu, supplements.
Mn, Ca, Mg, Li, Al, Fe, Zn, Cd, Hg) in hair of 100 children Methods: We studied 36 epileptic patients who were
(43 girls and 57 boys) and Se in 73 children with epilepsy receiving carbamazepine, folate (1mg/day) and Vitamin
(41 boys, 32 girls), age 1-14 years. 76% of children receive B12 (1mg/month) for one year. We measured the serum
valproic acid and 24% carbamazepin. levels of Homocysteine, carbamazepine, Folate and Vitamin
Results: There were no sex differences in metal content in B12.
hair, except for Cu level. The difference in level of Cu was Results: The meanSD of the carbamazepine therapeutic
6,3106,221ug/g (p=0.90). Contents of Zn, Cd, Mn and Hg dose was 536151.4mg/day. The folate levels were within
were below normal in all children, content of Cu - in 46%, normal limits in all of our patients. Nine patients had low
Se - at 34.25%, Mg - in 18%, Fe - 7%, Ca - only in 3% levels of serum vitamin B12 from whom, only one had
children. Content of Pb and Al was overweight, and of Cu, hyperhomocysteinemia. The meanSD of homocysteine
Ca, Mg, Li, Fe, Se below normal average. Significant levels was 13.38.1mol/l and 28% of our patients had
correlation (p0.95) between metal content was found in 29 homocysteine levels above normal limits. There was no
(43.94%) cases: contents of Pb, Cu, Ca, Al is in correlation significant association between the homocysteine and
with content of Cd - 6, Zn and Li - 5, Mg and Fe - 4, Hg - 3, carbamazepine, folate or vitamin B12 serum levels (pvalue=
Mn - 2, Se with Mn. 0.61, 0.17 and 0.55, respectively).
Conclusion: The study of metal concentrations in children Conclusion: This study suggests that the
with epilepsy may contribute to the discovery of new hyperhomocysteinemia that was detected in some of the
epileptogenic pathways and justification of therapy based epileptic patients with carbamazepine was independent of
on screening indicators of metals. the serum levels of folate and vitamin B12. Larger scale
controlled trials are needed to verify the possible role of
folate and Vitamin B12 or even vitamin B6 on controlling
the hyperhomocysteinemia following the treatment with
carbamazepine.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


258 Posters, Sunday 9 September

P1451 P1452
Recurrent symptomatic seizure in post- Blink reflex significance at estimation of
ischemic stroke prophylaxis patients epilepsy hypersynchronous activity
D.I. Cuciureanu1,2 V. Semashko, V. Kistsen, V. Evstigneev
1Neurology, University of Medicine and Pharmacy Belarusian Medical Academy of Postgraduate Education,
'Gr. T. Popa', 2Neurology, Emergency Hospital 'Prof. Dr. Minsk, Belarus
N. Oblu', Iasi, Romania Objective: The aim of our study is to estimate the self-
Background: To investigate the relationship between descriptiveness of Blink Reflex (BR) as a predictor of
recurrence rate of symptomatic seizures in post-ischemic epilepsy behaviour character (exacerbation or remission)
stroke patients receiving antiplatelet / anticoagulant therapy. and treatment efficacy.
Method: We reviewed the clinical findings and laboratory Methods: 37 patients (mean age 31.11.9 years) with
data of 212 patients with post-ischemic stroke symptomatic temporal epilepsy were studied. Latency, duration,
seizures, hospitalized in our neurological service for amplitude and configuration of R1, R2 components and R3
primary diagnoses between 2007 - 2010 and 1 year follow- presences were evaluated. 21 patients were treated AED
up in outpatient's service. All patient, aged 35 - 75, were with rTMS (1Hz, 20% of big circular coil MMI) during 10
investigated by clinical examination, brain computed consecutive days. Control group (n=16) took anticonvulsants
tomography, vascular related blood tests, EEG studies, only. We observed all patients every 4 weeks for 3 months
cerebrovascular ultrasound and transthoracic echography with seizure frequency registration and
searching possible new sources of emboli for every new electroencephalography (EEG) control.
seizure. All received first line antiepileptic drugs and 104 Results: Before anticonvulsive therapy correction in 28
patients received antiplatelet / anticoagulant treatment. (76%) patients presented hyperexcitable BR type. That BR
Results: We found 46 (22 %) patients with early-onset type is characterized by early R1 appearance, R1 and R2
seizures and 166 (78%) late-onset seizures patients. duration prolongation, R2 polyphasy and high amplitude,
Regarding the topography and types of infarcts 98 (47%) R3 presence. 9 patients (24%) had hypersynchronous BR
supratentorial infarcts, 28 (13%) watershed 48 (22%) with pattern with high non-polyphasic R2 amplitude. During
subcortical involvement, 24 (11%) lacunars strokes, 14 epilepsy exacerbation of R3 component was registered in
(7%) TIA. 38 (18%) patients with recurrent stroke. Seizure focus side at all cases. High seizure frequency correlated
type: 72 (34%) generalized tonic clonic, 62 (29 %) simple with early R1 appearance (r=0.57, p=0.042), prolongation
partial seizures, 78 (37%) partial seizures with secondary of R2 duration (r=0.55, p=0.049) and R3 presence (r=0.7,
generalization. Only 38 (18%) patients with recurrent p=0.006). During anticonvulsive therapy most R2 duration
stroke. 22 (3 vs. 8) patients with early-onset seizures, 82 (30 reduction was obtained in the subgroup of patients who took
vs. 52) late-onset seizures patients, 32 (10 vs. 21) simple AED with rTMS (r=0.74, p=0.003). BR type and its
partial seizures, 72 (22 vs. 40) patients with primary and parameter normalization correlated with seizure frequency
secondary generalized tonic clonic seizures received reduction (r=0.7, p=0.003) but had not correlations with
antiplatelet / anticoagulant treatment with lower rate of brain damage localization (neocortical dysplasia or mesial
seizure recurrence. temporal sclerosis) and epileptiform discharge occurrence
Conclusion: Rate of seizures recurrence was lower in in EEG (p<0.05).
groups with antiplatelet treatment, maybe due to lower rate Conclusions: Blink reflex pattern can be used as the
of recurrent small silent infarcts. criterion of estimating anticonvulsive therapy efficacy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 259

P1453 P1454
Non-invasive video-EEG monitoring in the Affective disorders and psychotic
diagnosis of focal epilepsy in children episodes in patients with chronic active
A. Bunduchi1, A. Bunduchi2, S. Hadjiu1 epilepsy
1Paediatrics, State Medical and Pharmaceutical University
L.M. Icevska
N. Testemitanu, 2Medical Centre "Excellence", Chisinau, JZU ZD Zeleznicar, Skopje, FYR Macedonia
Moldova
The aim of the study was to estimate frequency and types of
Introduction: FE (Focal Epilepsy) is met frequently in affective disorders and psychotic episodes in patients with
children, clinically expressed in polymorph seizures, often chronic active epilepsy. Evaluation of all patients with
with kinematic generalized characteristics. The onset of chronic epilepsy referred for hospitalization to department
seizures has a maximal incidence before the age of 3, with of epileptology for a period of one year, was done in this
a higher prevalence of tonic seizures. Method of choice in open, prospective, observational study. 34 patients, mean
assessing the diagnosis is Video-EEG monitoring that age 32.4 years, mean duration of epilepsy 19.1 years, were
identifies the type of seizure, the ictal and inter-ictal included. Comprehensive interview with patients, inquiry
epileptiform activity and establishes the clinical- with family and sometimes psychological testing ( MMPI,
electroencephalographic correlations. PIE) were used. 19 (56%) patients showed affective
Objective: To diagnose FE, determination of localization of symptoms. Two of them presented euphoric mood and four
the ictal and inter-ictal epileptiform activity. irritable-explosive affect. 13 experienced depressive mood
Patients and methods: Study included 89 patients, age 1 changes (ranging from major depressive episode with
month to 18 years with admission diagnosis of ,,Epilepsy''. suicide attempt in one, to low-grade disorder of dysthymia
The method was Video-EEG monitoring (1-12 hours), 21 and short-term course depressive and anxiety interictal
channels , 10-20 system. dysphoric episodes). 5 patients with depressive mood
Results: The diagnosis of epilepsy was confirmed in 76 changes required drug treatment. 69.2% of depressive
patients (85 %), while in 13 patients (15%) non-epileptic patients had complex partial seizures (CPS), which exceeds
paroxysms were diagnosed. Generalized epilepsy was the total number of patients with CPS in group (55,9%).
diagnoses in 11 patients (12%),FE in 65 patients (63%). Two-thirds of these patients had left-sided temporal foci in
According to localization, distribution was following: EEG. Only two of our patients have psychotic episodes, in
frontal - 30 patients (46,1%); temporal -22 (34%) ; occipital one of them treatment related (with Topiramate). Results
- 9 (13,9%) ; parietal - 1 (1%) ; multifocal - 3 (5%). 35% of showed high percentage of affective disturbances in our
patients with FE younger than 3 years had marked group, but it must be kept in mind that these patients were
generalized seizures known as infantile spasms, tonic. admitted to specialized clinic for severity or aggravation of
Conclusions: their illness. On the other hand, the importance of affective
1. Every sixth patient with primarily diagnosed epilepsy disorder recognition and treatment must be stressed, as they
suffered actually a non-epileptic paroxysm. are inherent, frequent manifestations of disordered cerebral
2. 73% of patients have FE, about half of them have frontal function in epileptic patients.
localization of the focus.
3. Presence of generalized types of seizures mimicking
generalized epilepsy is an important feature of FE in
patients younger than 3 years.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


260 Posters, Sunday 9 September

P1455 P1456
Seizure induction procedure during EEG Rubella encephalitis in children:
recording hot water epilepsy neurological and electro-encephalographic
B. Labovic1, R. Raicevic2, T. Lepic2, D. Veljancic2 features
1Clinic of Neurology, 2Military Medical Academy, Belgrade,
J. Abdelhedi1, I. Hsairi2, I. Kammoun3, I. Ayadi1,2,
Serbia E. Ellouz1,2, F. Kamoun1,2, N. Zouari3, C. Triki1,2
Introduction: Hot water epilepsy (HWE) represents a rare Department of Child Neurology, CHU Hedi Chaker, 2Search
1

clinical entity, classified in a group of reflex epilepsy in Unit Neuropdiatrie 01UR08_05, 3Department of
which seizures are precipitated by hot water during bathing. Neurophysiology, Sfax, Tunisia
Method: We report HWE in a 21-year-old male, with
symptoms on set appearing at the age seven years. After
initial provocative procedure when we applied a tea-pot P1457
filled with 38 C warm water and wrapped up with warm Temporal lobe epilepsy and stress
towels pressed against the back skin, with no effect; using N. Bukia1, M. Butskhrikidze2, L. Machavariani1,
the shower of wider surface of his shoulders and back with I. Bilanishvili1, N. Khizanishvili1, Z. Nanobashvili1
hot water of the same temperature 38 C which has been 1Neurophysiology, 2LEPL Center of Experimental
effective. Alongside with this, long term EEG monitoring Biomedicine, Tbilisi, Georgia
was proceeded.
Results: It is possible that stimulation of broad skin areas
involved hypersynchronisation of the parietal and P1458
frontotemporal regions that is critical to elicit seizure, not Neuron-specific enolase, a marker brain
the temperature alone.
As complex partial seizure was inducted, the EEG has inflammation, is increased in symptomatic
shown high voltage slow waves of 2.5 to 3.5/s over the left epilepsy
side, maximal over Fp1 and C3, followed by well developed N. Rashidova, K. Khalimova
spike and wave complexes over Fp1C3F3 and F7. The Tashkent Medical Academy, Tashkent, Uzbekistan
patient experienced a secondary generalized seizure in the
next 70 seconds, feeling sleepy, confused and recovered
gradually after cessation of the seizure over the following P1459
15 minutes. Status epilepticus and multiple sclerosis
Conclusion: We have special interest to present the case case overview
because of the novel method for seizure induction, which
we have applied. The importance of this observation is D. Sahovic, Z. Savic
related to the feasibility of seizure induction, in HWE cases, Neurology Clinic, Clinical Center University of Sarajevo,
under the conditions of EEG recordings. Sarajevo, Bosnia-Herzegovina

P1460
Review article: epilepsy in Ethiopia
D.K. Worku
Neurology, Addis Ababa University, Addis Ababa, Ethiopia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 261

P1461 P1465
Efficacy of anti-epileptic drugs (AED): Idiopathic generalized epilepsies focal
attempts of therapy study features
A. Kotov K.V. Voronkova1, A.A. Kholin2
Neurology, Moscow Regional Research and Clinical 1Neurological Department, 2Neurological Department,

Institute, Moscow, Russia Paediatric Faculty, Russian National Research Medical


University, Moscow, Russia

P1462
Vascular epilepsy P1466
A. Hancu1, C. Pascu2, C. Mocanu1, A. Cuciureanu2 Effectiveness of the first anti-epileptic
Ovidius University Constanta, 2Neurology, Ovidius
1 drug in elderly patients with epilepsy
University Constanta, Constanta, Romania S. Lukic, M. Spasic
Epilepsy Department, Clinical Center of Nis, Nis, Serbia
P1463
Executive dysfunction in one case of P1467
juvenile myoclonic epilepsy Endothelian dysfunction and oxidating
A. Revesz1,2, L. Visu-Petra3, L. Perju-Dumbrava2 stress in epileptogenesis
1Neurology, County Hospital Satu Mare, Satu Mare, M. Pityk1, D. Delva1, O. Pityk2
2University of Medicine and Pharmacy, 3Psychology, 1Department of Child Neurology, 2Department of Psychiatry,
Babes-Bolyai University, Cluj-Napoca, Romania Ivano-Frankivsk National Medical University, Ivano-
Frankivsk, Ukraine
P1464
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


262 Posters, Sunday 9 September

Motor neurone diseases P1469


Preliminary results of the French MSA-
P1468 fluoxetine study
UNC13A influences survival in an Italian O. Rascol, French MSA-Fluoxetine Study Group
population-based series Clinical Pharmacology, Toulouse University Hospital,
Toulouse, France
A. Calvo1, G. Restagno2, M. Brunetti2, I. Ossola2,
E. Majounie3, A.E. Renton4, A. Canosa1, U. Manera1, Introduction: Multiple system atrophy (MSA) is a sporadic
E. Bersano1, C. Moglia1, G. Mora5, B.J. Traynor4, neurodegenerative disorder characterized clinically by any
A. Chi1 combination of parkinsonian, autonomic, cerebellar or
1ALS Center, University of Torino, S. Giovanni Battista pyramidal symptoms. To date, no drug has proved sufficient
Hospital, 2Molecular Genetic Unit, A.S.O. O.I.R.M.-S. Anna, efficacy to halt the neurodegenerative process in MSA. The
Torino, Italy, 3Neuromuscular Diseases Research Unit, therapeutic strategy relies on the control of clinical
4Molecular Genetics Unit, Laboratory of Neurogenetics, symptoms of parkinsonism and autonomic dysfunction. Not
NIA, Bethesda, MD, USA, 5ALS Center, Salvatore Maugeri only a dopaminergic deficit, but also a serotoninergic one,
Foundation-IRCCS, Milano, Italy could be implicated in the physiopathology of MSA, as
Background: It has been recently shown that the common suggested by a recent study showing an improvement of
variant rs12608932 in gene UNC13A is associated with behavioural and neuropathological deficits with fluoxetine
amyotrophic lateral sclerosis (ALS) susceptibility and may in a model of MSA (Ubhi, et al. Exp Neurol 2012;234:405-
be an independent modifier of ALS survival in populations 16).
of North-European ancestry (Van Es, 2009; Diekstra, 2012). Objective: To test the clinical effect of fluoxetine 40mg/day
Aim: To evaluate the effect of UNC13A on survival in a on subjects with MSA, measured by change from baseline
population-based series of ALS cases of Italian ancestry. to month 3 in the total UMSARS (part I + part II) score.
Methods: 261 samples and 247 ethnically matched controls Methods: Multi-centred, randomized, double-blind,
were genotyped on Infinium HumanHap550 (Illumina), and placebo controlled study on 81 MSA subjects. Only baseline
236 on Infinium HumanHap610-Quad (Illumina); 535,468 data and preliminary result of main outcome are presented
SNPs were common across both platforms, including the here.
rs12608932 single nucleotide polymorphism (SNP). Results: The two groups were similar at baseline concerning
Survival was assessed with Kaplan-Meir curves and age (63.5 vs. 63.1), gender (men: 58.5% vs. 65%),
compared with the logrank test. UMSARS (I + II) (40.2 vs. 40.3), Beck Depression
Results: The study included 497 apparently sporadic ALS Inventory Scale (11.8 vs. 12.3), SCOPA-AUT score (20 vs.
patients (mean age 61.6 years [SD 11]) resident in Piemonte, 22.9), Epworth sleepiness scale (8.3 vs. 7.7), apathy, and
Italy. The minor allele frequency was similar in cases and orthostatic hypotension (40% vs. 60%). No significant
controls (33% vs. 32%, p=0.91). However, we found a difference according treatment group was found for change
significant association with survival for both additive and from baseline in the total UMSARS score after 3 months of
recessive genetic models. Patients carrying the CC (minor fluoxetine (3.1 vs. 1.4).
allele) genotype had a 12-months shorter survival than Conclusion: Further analyses with adjustment and imput
those carrying AA or AC genotypes (median survival time, for missing values are needed to confirm these preliminary
CC 2.5 years [95% CI, 1.5-3.4] vs. AA or AC 3.5 years [3.1- results.
3.8]; p=0.017). This effect was independent from patients'
age at onset, gender, site of symptom onset, phenotype and
use of NIV and PEG in a stepwise forward Cox multivariable
model (p=0.02; HR=1.42).
Comment: In the Italian population, UNC13A has a strong
independent modifier effect on ALS survival. This finding
has implications relevant to both, understanding ALS
pathogenesis and in defining therapeutic interventions.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 263

P1470 of the reviewed ALS-FRS and mean amplitude of motor


responses by phrenic nerve stimulation (PhrenAmpl). All
TDP-43 and FUS pathology in amyotrophic patients were on riluzole.
lateral sclerosis Results: Survival analysis was evaluated by Kaplan-Meier
Y. Fujita1, M. Kitani1, M. Takatama2, M. Ikeda1, log-rank and multivariate Cox proportional hazards.
K. Okamoto1 Independent factors negatively affecting survival were
Neurology, Gunma University Graduate School of Medicine,
1 bulbar-onset, elderly age, short diagnostic delay, FVC and
Internal Medicine, Geriatric Research Institute and Hospital,
2 small PhrenAmpl for the complete population. Small
Maebashi, Japan PhrenAmpl and short diagnostic delay were also independent
factors for G1 and G2, but age at onset and FVC were also
Introduction: TAR DNA-binding protein of 43-kDa (TDP- independent predictors for G2.
43) and fused in sarcoma (FUS), which are the key proteins Discussion: Prognostic factors in our population are in
of ALS, have structural and functional similarities. However, accordance with those described by others. PhrenAmpl
the relationship of these proteins have not been established. showed to be an independent prognostic factor. It is non-
Therefore, we examined the relationship of TDP-43 and FUS volitional and easily standardized and quickly performed.
proteins in ALS using immunohistochemical methods. FVC depends on patient co-operation and is not reliable in
Methods: We studied the spinal cord sections from 12 patients with facial weakness. Phrenic nerve stimulation
common sporadic ALS (SALS) patients and 4 ALS patients should be included in the routine assessment of ALS patients.
with FUS-positive cytoplasmic inclusions (including 3
familial cases and 2 patients with mutations in the FUS gene).
The sections were stained with haematoxylin and eosin (HE), P1472
and immunostained with anti-FUS, anti-phosphorylated- Rasch analysis of the Epworth Sleepiness
TDP-43 (pTDP-43), and anti-non-phosphorylated TDP-43 Scale in patients with post-polio syndrome
antibodies.
C.J. Gibbons1,2, I.M. Pomeroy1, A. Tennant3, C.A. Young1
Results: All pTDP-43-positive inclusions except one were 1Walton Centre Foundation NHS Trust, Liverpool,
negative for anti-FUS antibody in SALS. Although most of 2Collaboration for Leadership in Applied Health Research
the 42 neurons without abnormal pTDP-43 immunoreactivity and Care (GM CLAHRC), University of Manchester,
showed normal nuclear staining of FUS, 6 of 40 neurons with 3Academic Department of Rehabilitation Medicine, University
fine granular pTDP-43 immunoreactivity and 13 of 22 of Leeds, UK
neurons with skein-like or round inclusion did not show
nuclear staining of FUS. On the other hand, all basophilic Introduction: The Epworth Sleepiness Scale (ESS) has been
inclusions (BIs) and FUS-positive inclusions were not stained widely used to assess sleepiness in clinical trials for neurology
with anti-pTDP-43 and anti-TDP-43 antibodies. Furthermore, patients. The scale has not previously been validated for use
the nuclear staining of TDP-43 was well preserved in the all in patients with post-polio syndrome (PPS). Data collected in
neurons with BIs and FUS-positive inclusions. a PPS population were applied to the Rasch model to ensure
Conclusion: These results suggest that the pathogenesis of the scale was reliable, unidimensional and free from
FUS proteinopathy is independent from that of TDP-43 differential item functioning (DIF) or local dependency.
proteinopathy; however, abnormal TDP-43 may interact with Methods: A questionnaire pack containing the ESS was sent
the FUS protein in SALS pathogenesis. to 282 (88% response) consecutive patients with a confirmed
diagnosis of PPS.
Results: The ESS displayed reasonable fit to the Rasch
P1471 model [2(24) =37.80, p=0.04]. Differential item functioning
Survival in amyotrophic lateral sclerosis - was not present for either age or gender. Scale reliability was
prognostic factors acceptable (PSI=0.86). Mild local dependency (r=0.20) was
S. Pinto, A. Pinto, M. de Carvalho apparent between items 3 'Sitting inactive' and 6 'Sitting and
Institute of Molecular Medicine and Faculty of Medicine, talking'. Collapsing items 3 and 6 into a testlet improved fit
University of Lisbon, Portugal and revealed local dependency between items 1 'Sitting and
reading' and 2 'Watching TV'. Collapsing these items into a
Introduction: Amyotrophic lateral sclerosis (ALS) is a testlet led to a solution with excellent model fit [2(18)
severe neurodegenerative disease with short survival due to =22.55, p=0.21]. The final scale was unidimensional (0.71%
respiratory failure. We aimed to test the survival predictive T-tests significant) and free from DIF or local dependency.
value of the classical prognostic factors in addition to the Category thresholds were well ordered and item 'difficulties'
phrenic nerve motor responses in a large ALS population. were widely distributed (-3.2 to 3 logits).
Methods: We included 254 ALS patients followed in our Conclusion: The Epworth Sleepiness Scale demonstrates
tertiary center from 1997 and 2006 in whom phrenic nerve good psychometric and scaling properties in this population.
stimulation was performed and who fulfilled the inclusion The scale may be used with confidence in clinics and research
criteria. ALS was spinal-onset in 175 (group1- G1) and with PPS patients.
bulbar-onset in 79 (group2- G2). Features recorded at first
visit were: gender, age at symptom onset, onset region,
diagnostic delay, forced vital capacity (FVC), ALS functional
rating scale (ALS-FRS) including the respiratory sub-score

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


264 Posters, Sunday 9 September

P1473 these conditions first-hand. By creating and validating


longitudinal models of QoL, key intervention points can be
Work in progress: quality of life (QoL) and identified to enhance care delivery.
neurological disease - trajectories of
outcome in neurological conditions P1474
(TONiC): a study protocol Familial adult spinal muscular atrophy
C.J. Gibbons1,2, R. Cousins3, I.M. Pomeroy1, H. Ando1,3, associated with VAPB gene
A. Al-Chalabi4, J. Chataway5, C. Constaninescu6,
M.R.G. de Freitas, V. Kosac, O.J.M. Nascimento
J. Ealing7, C. Hawkins8, K. Morrison9, J. Palace10,
Neurology, Federal Fluminense University, Niteri, Brazil
N. Robertson11, D. Rog7, N. Scolding12, B. Sharrack13,
P. Shaw14, K. Talbot15, T. Williams16, A. Tennant17, Introduction: Familial spinal muscular atrophy (FSMA)
C.A. Young1, on behalf of the TONiC Group associated with VAPB gene is a rare autosomic dominant
1Walton Centre Foundation NHS Trust, Liverpool, disease of late onset and slow progression. Most cases were
2Collaboration for Leadership in Applied Health Research evaluated in Brazil and they were linked to the mutation
and Care (GM CLAHRC), University of Manchester, 3Health P56S. The phenotype may vary among families. The
Sciences, Liverpool Hope University, Liverpool, 4King's objective is to report four generations of four families in Rio
College Hospital, 5National Hospital for Neurology and de Janeiro.
Neurosurgery, London, 6University of Nottingham, 7Salford Methods: We studied 41 patients of 4 families with clinical
Royal Hospital, Manchester, 8University Hospital of North history, physical exam, electrophysiological study and
Staffordshire, Stoke-on-Trent, 9University Hospital genetic test.
Birmingham NHS Foundation Trust, Birmingham, Results: All patients presented a late onset and slow
10University of Oxford, Oxford, 11University of Cardiff, progressing disease with fasciculations, proximal weakness
12University of Bristol, 13Sheffield Teaching Hospitals, and amyotrophy, hypoactive deep tendon reflex, except one
14Sheffield Institute of Translational Neuroscience (SITRaN),
patient with brisk reflex. One patient showed tongue
University of Sheffield, 15John Radcliffe Hospital, Oxford, fasciculations and respiratory insufficiency. Electro
16The Newcastle Upon Tyne Hospitals NHS Foundation
physiological studies showed patterns of lower motor
Trust, Newcastle upon Tyne, 17Academic Department of neuron disease and genetic test showed P56S mutation of
Rehabilitation Medicine, University of Leeds, UK VAPB gene.
Background: Maintaining quality of life (QoL) in Conclusion: Although it is a rare motor neuron disease,
neurological illness is a key treatment priority for clinical FSMA with this mutation could be much more prevalent in
care teams. Understanding the complex variables and Brazil than expected. Moreover many cases may be
relationships that influence QoL and the manner these underdiagnosed. Genetic test should be performed whenever
relationships develop over time are important in improving it is suspected, mainly in our country.
care and designing better services.
Aims: We aim to examine the factors influential for QoL of
individuals with multiple sclerosis (MS), and motor neurone
disease (MND). This process includes longitudinal
assessment over four time points, development of new
disease-specific scales and validation of existing scales that
measure different factors related to QoL.
Methods: The study will be run in five phases across
fourteen hospital sites
Phase One: In depth interviews and focus groups conducted
with patients, to explore QoL issues and identify potential
items for disease-specific questionnaire scales.
Phase Two: Face to face interviews and focus groups
conducted with patients to identify gross questionnaire
problems. Professionals invited to participate in Delphi
discussion to discuss factors and model design to be tested.
Phase Three: Baseline questionnaire administration.
Phase Four: Test-retest assessment (four to six weeks).
Phase Five: 6-, 12- and 24-month questionnaire
administration.
Quantitative analyses will be conducted using Rasch
analysis and Structural Equation Modelling.
Discussion: The TONiC study is a large longitudinal study
designed to explore quality of life for people living with
neurological illness. It uses quantitative models based on
qualitative investigation with the patients that experience

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 265

P1475 P1476
Effects of hypertension in ALS. Results of Ataxin-1 and ataxin-2 intermediate-length
a population-based study PolyQ expansions in ALS patients
C. Moglia1, A. Calvo1, A. Ilardi1, A. Canosa1, U. Manera1, R. Spataro1, W. Sproviero2, R. Mazzei2, F. Cavalcanti2,
L. Mazzini2, G. Mora3, A. Chio'1, PARALS F. Condino2, T. Colletti1, V. La Bella1, F.L. Conforti2
1Neuroscience, University of Torino, Torino, 2Universit del 1Department of Experimental Biomedicine and Clinical

Piemonte Orientale, Novara, 3Fondazione Salvatore Neurosciences, University of Palermo, 2Institute of


Maugeri, Milano, Italy Neurological Sciences, National Research Council, Cosenza,
Background: Factors related to cardiovascular risk have Italy
been assessed in ALS with uneven findings. A recent paper Introduction: A number of studies have shown that
showed that a beneficial vascular risk profile is associated ataxin-2 (ATXN-2) intermediate poly-CAG expansions
with an increased risk of ALS (Sutjeda et al, 2011). with CAA interruptions are a risk factor for amyotrophic
Aim: To assess the effect of arterial hypertension in a lateral sclerosis (ALS). The present work was undertaken
population-based series of ALS patients. with the aim to investigate the frequency of Ataxin-1
Methods: The study population consisted of the 1260 ALS (ATXN-1) and ATXN-2 PolyQ expansions in a cohort of
cases incident in Piemonte and Valle d'Aosta in the period sporadic and familial ALS patients from South Italy.
1995-2004. Patients were affected by definite, probable or Methods: The PolyQ lengths of ATXN-1 and ATXN-2 in
probable laboratory-supported ALS. Arterial hypertension 405 sALS, 13 fALS and 296 unrelated controls without
was indicated as systolic pressure 160 and diastolic history of neurodegenerative disorders (NC) were assessed.
pressure 100. Genotyping of the ataxin-1 and ataxin-2 CAG repeat
Results: A total of 272 patients (21.6%) were affected by number was performed using fluorescent-labelled primer
hypertension at the time of onset of ALS. Patients with PCR with capillary electrophoresis on an ABI3130xl
hypertension had a significantly lower age at onset of ALS sequencer and analyzed with GeneMapper software version
than patients without hypertension in both genders (women, 4.0.
68.1 [SD 8.3] years vs. 64.2 [11.9] years, p=0.0001; men, Results: We found that 57 out of the 806 ATXN-1 alleles in
67.8 [8.9] years vs. 63.7 [11.5] years, p=0.0001). No the sALS cohort harboured a 32 PolyQ repeat length, as
relationship between hypertension and site of onset (bulbar compared to 13 out of the 544 NC alleles (p=0.0001, c2
vs. spinal) was found. Patients with hypertension were more test). For ATXN-2, a 28 PolyQ repeat length was found in
likely to have also diabetes mellitus (HR 2.9, 95% c.i. 1.8- 22 of the 808 sALS alleles and in only 3 of the 586 NC
4.6, p=0.0001) and frontemporal dementia (FTD) (HR, 1.8, alleles (p=0.0041, c2 test). Further, the analysis of ATXN-1
95% c.i. 1.1-2.9, p=0.01). Hypertension did not influence and ATXN-2 PolyQ repeat length expansions in fALS
ALS outcome. revealed that ATXN-1 might be a potential risk factor also
Conclusions: We have found that a preceding arterial in these patients. ATXN-1 CAT and ATXN-2 CAA
hypertension is associated to a delayed onset of ALS in interruptions were detected in ALS patients only. Age at
population-based series. This finding is in line with the onset, site of onset, and sex were not significantly related to
previous observation that a beneficial vascular risk profile the ATXN-1 and/or ATXN-2 PolyQ repeat length
is associated to an increased risk of ALS. expansions.
Conclusion: Both ATXN-1 and ATXN-2 PolyQ
intermediate expansions are independently associated to an
increased risk for ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


266 Posters, Sunday 9 September

P1477 P1478
High vascular risk profile is associated Inhibition of APP beta-cleavage site
with prolonged survival in ALS affects survival and motor functions of
M.A. Rubio Perez, J. Pascual Calvet, J. Jimnez Conde, ALS transgenic mice
A.J. Santiago Ois, E. Munteis Oliva, J. Roquer Gonzalez P. Rabinovich Toidman, M. Becker, B. Solomon
Neurology Department, Parc de Salut Mar, Barcelona, Spain Department of Molecular Microbiology and Biotechnology,
Introduction: Recent studies show that a beneficial George S. Wise Faculty of Life Sciences, Tel Aviv University,
vascular risk profile increases amyotrophic lateral sclerosis Tel Aviv, Israel
(ALS) susceptibility. Also, low levels of vascular endothelial Background: Amyotrophic lateral sclerosis (ALS) is a
growth factor (VEGF) represent a risk factor for ALS, and progressive neurodegenerative disease defined by motor
correlate with shorter survival. Chronic vascular hypoxic neuron loss. Recent studies have reported an increase in
situations as coronary, cerebral and peripheral arterial amyloid precursor protein (APP) levels and in its cleavage
diseases stimulate VEGF production. products in ALS patients indicating their possible
The aim of the study is to analyze the impact of the vascular involvement in this disease. APP is a transmembrane protein
risk profile in the survival of our population of ALS patients. processed either by -secretase or -secretase followed by
Methods: Retrospective analysis of our ALS registry of -secretase. The APP cleavage products mediate a reduction
patients diagnosed between 1991 and 2011. Demographic in synaptic transmission, synaptic loss, neurite retraction
and clinical data like age of onset and clinical form at onset and programmed cell death.
were collected. Objective: Elucidation of the role of APP cleavage products
Patients were classified in two groups according to their in pathology of ALS.
vascular risk profile. 'High Vascular Risk' profile was Methods: ALS mice models that express mutant superoxide
defined as having suffered a clinical event (stroke, coronary dismutase 1(SOD1) were treated intraventricularly with
or peripheral arterial disease) or the presence of at least 3 monoclonal antibody that blocks the -secretase cleavage
vascular risk factors. site on APP. Levels of APP cleavage product called soluble
Results: 161 patients were analyzed (49.7% men, 50.3% APP-, number of motor neurons, motor functions and
female), with median age of 64 years (IQR 55-73). 128 survival were assessed.
patients were dead (79.5%), with median survival time of Results: Inhibition of APP cleavage resulted in a decrease
32 months (IQR 20-53). Factors associated with survival in in the levels of soluble APP-, an increase in number of
univariate analysis were sex, age of onset, clinical form at motor neurons, delayed disease onset, improved motor
onset and high vascular risk profile. Multivariate analysis capacities of ALS affected mice and better survival.
showed an independent association with survival with the Conclusions: APP cleavage products might contribute to
following variables: age (HR 1.028;p<0.001), clinical form the degeneration in ALS, and early inhibition of APP
at onset (HR 1.705;p=0.009), and high vascular risk profile process may ameliorate disease progression.
(HR 0.592;p=0.014).
Conclusion: We found an association between a high
vascular risk profile and increased survival in our
population. Other prognostic markers were age of onset and
clinical form at onset.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 267

P1479 P1480
No heart involvement in SBMA patients Rigorous control of a supervised exercise
G. Querin1, P.Melacini1, L.Morandi2, L. Mazzini3, protocol improves function and survival in
V. Silani4, A. Gaiani1, C. D'Ascenzo1, E. Pegoraro1, amyotrophic lateral sclerosis patients
G. Sorar1 J.P. Lopes Almeida1, A. Cardoso Pinto1, M. de Carvalho2
1University of Padova, 2Istituto Neurologico 1Department of Physical Medicine and Rehabilitation,
'C.Besta', Milan, 3University of Novara, University of Lisbon Medical School, Santa Maria Hospital,
4University of Milan, Italy
2Neuromuscular Unit, Institute of Molecular Medicine,

Background: Spinal and bulbar muscular atrophy (SBMA) University of Lisbon Medical School, Lisbon, Portugal
is an adult-onset, X-linked, lower motor neuron disease, Introduction: The uncertainty about exercise in
characterized by slowly progressive muscle weakness and Amyotrophic Lateral Sclerosis (ALS) is mostly derived
atrophy. The disease is caused by an expansion of a CAG from paucity of randomized trials and lack of rigorous
repeat encoding a polyglutamine tract within the androgen control of exercise intensity. We hypothesized that
receptor (AR) gene. Nuclear accumulation of pathological exercising till fatigue (as evaluated by Borg scale and
AR, which is toxic to motor neurons, has been observed in cardiopulmonary exercise test, CPET), is suitable for
tissues other than the nervous system including the heart. ambulatory ALS patients.
Materials and methods: To test the hypothesis of the Methods: Prospective, quasi-randomized, single-blinded
presence of heart disease in SBMA we carried out a full controlled trial. 40 consecutive ALS patients, were assigned
cardiologic evaluation (12-lead ECG, Echocardiography to the control group (G1, n=20) and the intervention group
and 24-hour ECG Holter) in 26 genetically defined SBMA (G2, n=20). Patients in G1 followed standard care and
patients. exercised to fatigue with no supervision at home. G2
Results: Patients' age range was 32 - 75 yrs (mean age 54.4 underwent a supervised moderate progressively resisted
yrs). 10 patients had high blood pressure and were under exercise program 3 times/week (treadmill ramp protocol
antihypertensive medications. No patients displayed clinical levelled 20% lower than determined by CPET), while
signs of heart disorders at the cardiologic examination. The compensating lower limb weakness and respiratory failure
12-lead ECG findings were normal or consistent with left with body-weight-supporting-systems and non-invasive-
ventricle (LV) hypertrophy in the oldest patients suffering ventilation, correspondingly. All patients performed CPET
from high blood pressure. Similarly, echocardiography and respiratory function tests (RFT) at 3-month interval.
showed no abnormalities other than mild concentric LV Primary outcomes were ALSFRS-R scores and slopes and
hypertrophy in patients with hypertension. No patients RFT/CPET parameters. Secondary outcomes included
showed significant rhythm abnormalities at the 24-hour survival analysis.
ECG Holter. Results: No clinical or laboratorial differences were
Conclusions: Our findings do not support the hypothesis of observed between groups for any variable at admission. All
a primary heart involvement in SBMA. ALSFRS scores improved in G2 throughout follow-up
course (p<0.05); survival from the intervention was
statistically increased in G2 (p=0.021) and when adjusted to
exercise variables. A linear decline in respiratory function
was seen in both groups, but not significant. Peak oxygen
uptake, ventilation/minute and VO2/work rate at one-year
were significantly lower in G2 (p<0.01).
Conclusion: A well-controlled exercise protocol is feasible
and should be prescribed to ALS patients since it improves
survival and respiratory efficiency, with less deconditioning.
Determinants of exercise are significant predictors of
survival.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


268 Posters, Sunday 9 September

P1481 P1482
Reduced expression of BTBD10 in Novel candidate proteins for early
anterior horn cells in amyotrophic lateral diagnosis in animal models and patients
sclerosis of amyotrophic lateral sclerosis
N. Furuta1, Y. Fujita1, M. Takatama2, M. Matsuoka3, H. Hara, H. Tanaka, M. Takata, K. Tsuruma,
K. Okamoto1 M. Shimazawa
1Department of Neurology, Gunma University Graduate Molecular Pharmacology, Department of Biofunctional
School of Medicine, 2Geriatrics Research Institute and Evaluation, Gifu Pharmaceutical University, Gifu, Japan
Hospital, Maebashi, 3Department of Pharmacology, Tokyo Introduction: The diagnosis of amyotrophic lateral
Medical University, Shinjuku, Japan sclerosis (ALS) is difficult at an early stage due to lack of
Background: BTBD10 was shown to activate Akt by definitive biomarkers. Our aim was to identify characteristic
inhibiting protein phosphatase 2A (PP2A)-mediated serum protein patterns that could provide candidate
dephosphorylation. The overexpression of BTBD10 biomarkers for ALS.
suppressed motor neuron death. However, it remains Methods: We divided mutant superoxide dismutase 1
unclear whether expression of BTBD10 is related to motor (SOD1)H46R rats into 3 groups based on disease
neuron degeneration in human amyotrophic lateral sclerosis progression: pre-symptom (90 days), onset, and end-stage.
(ALS). After separation of serum proteins using two-dimensional
Materials and methods: We examined the spinal cords of electrophoresis, we selected clear protein spots and
22 patients with sporadic ALS and 5 control cases. Mirror identified 2 candidate proteins - inter-alpha-trypsin inhibitor
paraffin sections were prepared for immunohistochemistry, heavy chain H4 (ITIH4) and glutathione peroxidase 3
which was carried out using rabbit polyclonal anti-BTBD10 (GPX3).
antibody, anti-phosphorylation- dependent TDP-43 (pTDP- Results: The 120-kDa ITIH4 increased at the onset of the
43) antibody, and anti-TGN46 antibody, which recognizes disease and the cleaved 85-kDa ITIH4 at the end-stage in
an intrinsic membrane protein of trans-Golgi network the sera of the SOD1H46R rats. In clinical samples,
(TGN). We compared the neuronal immunoreactivity expression of the 85-kDa ITIH4 was substantial in the sera
between BTBD10 and TGN46 / pTDP-43. and in vascular endothelial cells in sporadic ALS patients,
Results: BTBD10 was expressed in neurons in the spinal and was not detected in controls or patients with Alzheimers
anterior horns and only minimally expressed in astrocytes. or Parkinson's disease. 100-kDa ITIH4 was specifically
In control cases, BTBD10-positve small granular increased in myelitis patients, but not in ALS. GPX3 protein
cytoplasmic immunostaining was observed diffusely in the levels in the sera of SOD1H46R rats were upregulated pre-
anterior horn cells. In ALS cases, BTBD10-positive neurons symptom and gradually decreased as the disease progressed.
were significantly decreased in the remaining neurons, and In clinical samples, GPX3 protein levels were lower in the
the reduction of BTBD10 immunoreactivity occurred more sera of the patients with ALS than in those of controls,
frequently in large neurons than in small neurons. Mirror patients with Alzheimers disease, Parkinson's disease, or
sections disclosed that neurons with reduction of BTBD10 myelitis.
immunoreactivity showed more frequently fragmentation of Conclusion: These results indicate that ITIH4 and GPX3
Golgi apparatus and pTDP-43 positive inclusions. Bunina are potential biomarkers for diagnosis of ALS and that
bodies and round inclusions showed no BTBD10 observing changes in these expression levels may be useful
immunostaining. in obtaining important clinical information on the
Conclusion: Reduction of BTBD10 immunoreactivity was progression of disease in ALS patients.
observed in the large anterior horn neurons, and the majority
of these neurons had fragmented Golgi apparatus and
pTDP-43 positive inclusions. These results suggest that
reduced BTBD10 expression may be related to the neuronal
degeneration in ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 269

P1483 P1484
Pattern of spread and survival in sporadic ALS incidence and prevalence in the U.S.:
ALS How does ALS progress? estimates from large administrative
F. Kimura1, C. Fujimura-Kiyono1, H. Tani2, S. Ishida1, databases
H. Nakajima1, T. Hirose1 D. Perlroth1,2, R. Conrad2,3, H. Cheung2,
1Division of Neurology, The First Department of Internal D. Lakdawalla2,3, L.A. White4
Medicine, Takatsuki-City, 2Osaka Medical College, Osaka, 1Center for Primary Care and Outcomes Research, Stanford
Japan University, Stanford, 2Precision Health Economics, 3Shaeffer
Objective: To define patterns of spread through the order of Center for Health Policy and Economics, University of
lower motor neuron involvement (first, second or third Southern California, Los Angeles, CA, 4Biogen Idec, Weston,
order), relationships between interval or sites of affected MA, USA
areas from onset to involvement of a second region, and Introduction: Most ALS epidemiologic estimates are based
prognosis, including 5-year survival, normal preservation of on samples of <1 million persons. We estimated U.S. ALS
motor function at onset of respiratory symptoms and incidence and prevalence using two large healthcare claims
cumulative occurrence of each region and direction of databases.
spread. Methods: We used 2005-2007 claims data from a Medicare
Method: 150 patients with sporadic amyotrophic lateral 20% sample for patients aged 65 years and over, and
sclerosis (ALS) underwent follow-up at 3 month intervals Ingenix Touchstone for patients aged 18 to 64 years. There
until the appearance of respiratory symptoms. Symptom were 18 million person-years of data in Medicare, and 7
appearances were determined using the revised version of million person-years in Ingenix. ALS cases were included
the ALS Functional Rating Scale. if they had 2 medical claims with ICD-9 diagnostic code
Result: Median survival with combined type onset (two 335.20 separated by at least 90 days. We extrapolated these
regions simultaneously) was shorter (18 months) than with findings to estimate the epidemiology of ALS in the overall
bulbar onset (26 months, p1/40.01). The interval from onset U.S. population.
to involvement of the second region correlated significantly Results: Annually on average, there were 477 (range 454 to
with survival, independent of particular combinations. 5 516) new ALS cases in Medicare sample and 42 (range 36
year survival rate was 21% for lower limb onset, 18% for to 53) in Ingenix. Mean prevalent ALS cases were 1,670 in
upper limb onset and 16% for bulbar onset. Early Medicare and 138 in Ingenix. This resulted in an estimated
manifestations of bulbar symptoms within 1 year were annual ALS incidence of 7.7 cases per 100,000 persons over
associated with worse survival (p<0.001) although no age 65, and 2.5 per 100,000 persons aged 18-64. The
significant difference in survival was seen between groups estimated annual ALS prevalence was 26.9 cases per
with and without bulbar symptoms (p1/40.51). In terms of 100,000 over age 65 and 8.2 per 100,000 aged 18-64. The
cumulative occurrence, symptoms spread longitudinally to overall U.S. adult population ALS incidence was 7,530 new
adjacent regions. Bulbar function remained preserved in cases annually, or 3.4 per 100,000 with an annual prevalence
27%, lower limb function in 10% and upper limb function of 25,400 cases, or 11.3 per 100,000.
in 2.7%. Conclusion: ALS incidence and prevalence estimates from
Conclusion: The interval between onset and involvement these data are slightly higher (7-10%) than previous
of the second region is an important predictor of survival. estimates based on smaller samples sizes. The number of
The data support the contiguous anatomical propagation of persons diagnosed and living with ALS may be larger than
lower motor neuron involvement in sporadic ALS. previously thought.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


270 Posters, Sunday 9 September

P1485 P1486
Potential biomarkers in the blood of ALS A functional variant in the PON1 gene is
patients: mitochondrial SOD and associated with shorter survival in
aconitase enzymatic activities patients with amyotrophic lateral
C. Gonzlez Mingot1, P. Iaerrea2, C. Iguez1, sclerosis
J.L. Capablo3, J. Costn4, F.J. Miana5, J. Garca5, N. Ticozzi1, F. Verde1, C. Morelli1, C. Tiloca1,
R. Osta6, P. Larrod1 D. Sangalli1, I. Fogh1,2, A. Ratti1,3, S. Messina1,
1Neurology Department, University Clinical Hospital of
V. Silani1,3
Zaragoza, 2Biochemical Department of Biology Faculty, 1Department of Neurology, IRCCS Istituto Auxologico
Zaragoza University, 3Neurology Service, Miguel Servet Italiano, Milano, Italy, 2MRC Centre for Neurodegeneration
Hospital of Zaragoza, 4Neumology Department, University Research, Institute of Psychiatry, King's College London,
Clinical Hospital of Zaragoza, 5Physiology Department of UK, 3Department of Neuroscience, University of Milan
Medicine Faculty, 6Genetics Department of Veterinary Medical School, Milano, Italy
Faculty, Zaragoza University, Zaragoza, Spain
Introduction: The paraoxonases (PON) are a family of
Background: Oxidative stress-mediated mitochondrial enzymes involved in preventing lipid membrane
degeneration plays a role in motor neuron death caused by peroxidation, and in detoxification of exogenous
mutant SOD1 effects in Amyotrophic Lateral Sclerosis compounds. Several studies have suggested an association
(ALS). It has not yet been established whether oxidative between single nucleotide polymorphisms (SNP) in the
stress is a cause or a consequence of this neurodegeneration. PON genes and increased risk of developing amyotrophic
There is altered antioxidant defence enzyme (ADE) activity lateral sclerosis (ALS). Our study aims to assess whether
in the peripheral tissues of familial and sporadic ALS the functional SNP rs661 in PON1, which alters the
patients. This study attempts to confirm the alteration in substrate specificity of the enzyme, has an effect on disease
mitochondrial ADE activity and to try to determine whether phenotype.
the altered enzyme activity in ALS is independent of a Methods: 341 Italian patients with sporadic ALS were
patients condition. genotyped using the Human 660W-Quad BeadChip
Methods: We measured mitochondrial SOD1, SOD2 and (Illumina), and rs661 genotypes obtained with the
aconitase enzymatic activities in 22 controls and 26 SALS GenomeStudio software. Phenotypic traits analyzed
patients at different stages of the disease. We correlated included: age at onset, site of onset, distribution and severity
mitochondrial antioxidant activity with clinical and of muscular involvement, global functional impairment,
prognostic variables. disease duration and the time from onset to the start of non-
Results: Mitochondrial antioxidant enzymatic activity was invasive ventilation (NIV).
significantly lower in the 26 ALS patients than in the 22 Results: In comparison with patients homozygous for the
controls (SOD 1 p<0.001, aconitase p<0.005). Cases with major allele (A) of rs661, individuals with at least one copy
lower SOD1 (p<0.05) and aconitase (p<0.005) activity of the G-allele presented more frequently with a bulbar
levels died before those with higher levels. Patients with (28.8% vs. 19.3%; p=0.01) or respiratory onset (10.7% vs.
higher levels of SOD1 (p<0.05) and aconitase (p<0.05) 1.6%; p<0.01). Also, GG patients often showed a
activity survived longer. Aconitase activity was also higher symmetrical (48.1% vs. 19.8%; p<0.01) and proximal
in patients with earlier onset ages (p<0.01) and in those with (50.0% vs. 27.6%; p=0.04) distribution of the motor deficit.
predominantly upper motor neuron (MN) signs. SOD2 Lastly, individuals homozygous for the G-allele had a
activity was significantly correlated with ALSFRS scores shorter time-to-NIV (14.3 vs. 31.2 months; p=0.01), and
and nutritional parameters. reduced survival (26.8 vs. 41.4 months; p<0.01).
Conclusions: Mitochondrial SOD1 and aconitase activities Conclusion: The G-allele of rs661 may increase the
are independent prognostic factors for ALS, whereas SOD2 susceptibility of motor neurons innervating bulbar and axial
activity is influenced by the functional and nutritional status muscles to neurodegeneration in ALS, ultimately resulting
of ALS patients. in a faster respiratory decline and shorter survival.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 271

P1487 P1488
The copy number of 5q13 locus genes Prevalence of medical comorbidity among
and the severity of spinal muscular ALS patients in a U.S. health insurance
atrophy in Russian patients claims database
V. Zabnenkova, E.L. Dadali, A.V. Polyakov J.R. Williams, D.A. Kerr, W. Farwell
Research Centre for Medical Genetics, Moscow, Russia Biogen Idec, Cambridge, MA, USA
Introduction: Proximal spinal muscular atrophy (SMA) is Introduction: Motor and respiratory decline in amyotrophic
an autosomal recessive disorder caused by the loss of lateral sclerosis (ALS) is well documented, but information
a-motor neurons in the spinal cord. With an incidence of 1 on medical comorbidities is limited. This study estimated
in 10,000 live births and a carrier frequency of 1 in 50, SMA the prevalence of comorbidities among ALS patients within
is the most frequent genetic cause of infantile death. SMA a large U.S. health insurance claims database.
patients are subdivided into types I-III according to age of Methods: Subjects (n=1845) with 2 ALS medical claims
onset and achieved motor abilities. These forms of proximal (ICD-9 code 335.20) between 2004 and 2011 were
SMA are caused by mutations in SMNt gene. About 95% of identified, then age- and gender-matched to controls without
SMA cases are caused by homozygous deletion of the a claim for any motor neuron diseases (n=3690).
SMNt gene or conversion events. The phenotype variability Comorbidity categories were defined using the Agency for
of the disease with such molecular homogeneity may be Healthcare Research and Quality's Clinical Classification
explained by presence of phenotype modifiers. System. Prevalent comorbidity was defined as 1 medical
Methods: It has been developed a quantitative assay based claim with an ICD-9 code within a particular category,
on Multiplex Ligation-dependent Probe Amplification unrelated to ALS sequelae. 12-month prevalence and odds
(MLPA) for this study. ratios (OR) were calculated.
Results: The SMNc and NAIP gene-copy number has been Results: The five most prevalent comorbidity categories
analyzed for establishing the phenotype-genotype were hypertension (41.4%, OR=1.4), disease of the
correlation in 200 SMA patients (SMAI n=77, SMAII n=58, heart (36.0%, OR=1.7), disorders of lipid metabolism
SMAIII n=65). 77.9% of SMAI patients showed two copies (28.2%, OR=0.8), diseases of arteries; arterioles; and
of SMNc, 60.4% of SMAII patients had three or four copies capillaries (19.0%, OR=2.1), and mood disorders
of SMNc and 54.1% of SMAIII had four to five copies. The (17.2%, OR=3.1). The five comorbidity categories with the
NAIP gene is deleted in 36.4%, 6.9% and 4.6% affected greatest increased odds relative to controls were immunity
individuals with SMA I, II and III types, respectively. disorders (3.9%, OR=16.4), systemic lupus erythematosus
Conclusions: The copy number of SMNc gene is a and connective tissue disorders (2.6%, OR=6.1), other
modifying factor of severity of SMA. The absence of the infection; including parasitic (5.1%, OR=5.8), delirium,
gene NAIP, indicating a considerable deletion in the locus dementia, amnestic, and other cognitive disorders (7.4%,
5q13, may be an additional criterion, modifying the severity OR=5.5), and epilepsy; convulsions (3.4%, OR=4.4). All
of the disease. ORs were statistically significant, p<0.05. Additional
comorbidity categories with the highest prevalence and
greatest increased odds will be presented.
Conclusions: Cardiac diseases were the most prevalent
comorbidities, while immune-related disorders, cognitive
impairment, and seizures were more increased compared to
controls. Understanding the full comorbidity profile in ALS
will aid in therapeutic decision making for patients with
ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


272 Posters, Sunday 9 September

P1489 P1490
Ocular motor apraxia: an uncommon early Over-representation of the L144F SOD1
sign in amyotrophic lateral sclerosis mutation in Serbian ALS patients due to
C. Morelli1, N. Ticozzi1, A. Doretti1,2, A. Lafronza1, founder effect
F. Verde1, D. Sangalli1, B. Poletti1, A. Ciammola1, D. Keckarevic1, Z. Stevic2, M. Keckarevic-Markovic1,
S. Messina1, V. Silani1,2 M. Kecmanovic1, S. Romac1
1Department of Neurology, IRCCS Istituto Auxologico 1Faculty of Biology, University of Belgrade, 2Clinic of
Italiano, 2Department of Neurosciences, University of Milan Neurology, School of Medicine, University of Belgrade,
Medical School, Milano, Italy Serbia
Introduction: Acquired ocular motor apraxia (AOMA) is Objectives: Approximately 5-10% of ALS cases are
characterized by loss of voluntary control of saccades and familial (FALS), and the remaining ones are sporadic or
smooth pursuit. To our knowledge, this is the first report of apparently sporadic (SALS). So far, more than 300 different
two patients with amyotrophic lateral sclerosis (ALS), also mutations have been identified in at least six major genes of
developing AOMA. which mutations in the gene encoding Cu/Zn superoxide
Methods: Patient 1 developed an atypical parkinsonism at dismutase (SOD1) are most numerous. Founder effect was
age 62, followed three years later by slowly progressive reported for A4V, G41S, I113T, and R115G SOD1
ALS and fronto-temporal dementia (FTD). Progressive mutations. Here we present results of the haplotype analysis
fixation of gaze, ocular and eyelid apraxia appeared at age in a group of Serbian ALS cases harbouring L144F mutation
67. Patient 2 started complaining of cramps and confirming the existence of a founder effect.
fasciculations in the lower limbs at age 77, and was Methods: Genomic DNA was isolated from blood samples
diagnosed with classic ALS. Six months later he developed taken from 191 unrelated ALS patients. Coding sequence of
ocular and eyelid apraxia, and, subsequently, FTD. The SOD1 gene was analyzed by direct sequencing, followed by
patient developed akinetic mutism and died nine months haplotype analysis of 8 surrounding STR loci.
after onset. In both cases, neuropsychological assessment Results: Sequencing analysis revealed presence of
suggested a frontal and parietal dysfunction. mutations in 37 ALS cases (26/37 FALS and 11/154 SALS).
Neuroradiological tests showed bilateral cortical atrophy of Mutation L144F was detected in 22 FALS and 4 SALS
the frontal and parietal lobes. Genetic screening was cases. Haplotype analysis showed that all L144F
negative. chromosomes could have common origin.
Results: The patients were diagnosed as having ALS-plus Conclusion: Results of the sequencing analysis confirmed
syndromes (corticobasal degeneration in case 1, and FTD in that SOD1 mutations are the most frequent cause in Serbian
case 2). Both patients showed a prominent involvement of ALS patients. Haplotype analysis suggests that over-
fronto-parietal areas, compatible with AOMA. representation of L144F mutation could be explained by
Conclusion: An unrecognized bilateral involvement of actual Balkanian origin of L144F mutation and founder
frontal and parietal lobes may underlie the impairment of effect.
ocular movements observed in some ALS patients. Early
detection of AOMA may predict an incoming dementia,
thus assuming a negative prognostic significance.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 273

P1491 P1492
VEGF evaluation in amyotrophic lateral Amyotrophic lateral sclerosis (ALS) in
sclerosis: interplay with respiratory Iran: focusing on functional disability and
function and exercise quality of life trends
S. Pinto1, R. Carrilho2, A. Pinto1, J. Costa2, S. Nafissi1, H. Shamshiri1, E. Mir2, B. Pourmirza2,
M. de Carvalho1 M. Etemadifar3, R. Abolfazli1, K. Basiri3, K. Gharagozli4,
1Institute of Molecular Medicine and Faculty of Medicine,
B. Zamani1, H. Ayromlou5, M.H. Harirchian1, on behalf
University of Lisbon, 2Instituto de Tecnologia Qumica e of Iran ALS Study Group
Biolgica, Lisbon, Portugal 1Tehran University of Medical Sciences, 2Sanofi-Medical
Objectives: The role of vascular endothelial growth factor Department, Tehran, 3Isfahan University of Medical
(VEGF) in the pathogenesis of ALS is a critical point. We Sciences, Isfahan, 4Shahid Beheshti University of Medical
have addressed this issue by evaluating VEGF expression Sciences, Tehran, 5Tabriz University of Medical Sciences,
in a large population of patients. Tabriz, Iran
Methods: We measured VEGF plasma level in 83 ALS Introduction: Many studies from different parts of the
patients, 20 controls and 10 patients with other world have reported disease course, disability and quality of
neuromuscular diseases matched for age. ALS patients were life of ALS patients. Very few studies on this devastating
divided into 4 groups: patients with severe respiratory disease have been conducted in the Middle East. The main
insufficiency undergoing non-invasive ventilation (NIV) goal of this study was to evaluate ALS progression,
(G1); patients chronically on NIV (G2); patients submitted disability and quality of life in a multicenter, prospective
to exercise (G3); and ALS patients with absent respiratory study in Iran.
impairment. Methods: 358 ALS cases were registered from 24 neurology
Results: 30 ALS patients were included in G1, 14 in G2, 12 centres all around the country. Demographic data, Manual
in G3 and 27 in G4. VEGF levels were similar in controls Muscle Test scoring (0-130), ALS Functional Rating Scale,
and in all ALS groups analysed. There was no correlation ALSAQ-40, ALS health scoring system, Rilutek
between VEGF level and gender, onset type, age, disease consumption, disease onset, presence of positive family
duration, ALS-FRS-R and respiratory function history and consanguinity were recorded. Follow-up visits
measurements, except for G2 in which VEGF was were performed after 6 and 12 months.
negatively correlated to forced vital capacity (p=0.025). Results: Patients were 17 - 89 years old (mean=54.96),
VEGF level increased significantly in G2 after NIV 62% were male. 22.9% of patients had bulbar onset
(p=0.01) and in G3 after exercise (p=0.02) but remained ALS(19.4% of males and 28.7% of females). 65.6% of
stable in the rest of patients. patients used Rilutek continuously. During 12 months
Discussion: VEGF is modulated in ALS by 2 relevant follow-up, 14.8% of patients died because of ALS
interventions, NIV in patients with severe respiratory complication (4.2% in mild, 8.6% in moderate, 23.2 in
impairment and following exercise. This observation severe and 41.7 in terminal stages). This rate was 14.1% in
suggests that both NIV and exercise can drive limb onset and 17.1% in bulbar onset groups. Death rate in
neuroprotective mechanisms in ALS. the Rilutek group was 13.6 vs. 17.1 in other patients.
Overall, for 0-6 and 6-12 months periods, mean score
decrements for MMT score was 13.94 and 10.93,
ALSFRS-R 4.77 and 3.66, ALSAQ score 13.85 and 14.61,
respectively. Also the scores were analyzed and compared
in all aforementioned groups and subgroups.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


274 Posters, Sunday 9 September

P1493 P1494
Novel crystalloid oligodendro-gliopathy in Methyl esterification of erythrocyte
complex hereditary spastic paraplegia membrane proteins in response to
A. Whrer1, L. Laszlo1, J. Finsterer2, C. Stllberger3, oxidative stress in amyotrophic lateral
J. Furtner1, W. Rinner1, K. Molnar1, H. Budka1, sclerosis: a case-control study
G. Kovacs4 L. Daniele
1Medical University of Vienna, 2Danube University Krems, Department of Neurological Sciences, Second University of
3KAR, 4Clinical Institute of Neurology, Vienna, Austria
Naples, Italy
Objectives: Hereditary spastic paraplegia (HSP), comprises Objectives: Amyotrophic lateral sclerosis is a degenerative
a group of so far 48 clinically and genetically heterogeneous disorder of upper and lower motor neurons. Aim of study
disorders associated with either isolated spastic paraparesis was to evaluate levels of methylation in erythrocyte
(pure HSP) or with additional neurological or non- membrane proteins of ALS patients as possible marker of
neurological manifestations (complicated HSP). oxidative stress
Methods and results: Here we present a case of an adult- Materials and methods: Blood samples of 17 ALS patients
onset, apparently autosomal dominant complicated form of (10 men, 7 women, 5723years) and 13 healthy age sex
HSP. Onset of clinical symptoms was at the age of 40 years matched controls were processed to assess methylation of
and characterised by slowly progressive corticospinal tract intact erythrocytes membrane proteins as index of abnormal
dysfunction, dysarthria, disorientation, extrapyramidal levels of L-isoaspartyl residues and intracellular
symptoms, and bilateral ptosis. Cranial MRI revealed concentrations of methyl donor S-adenosylmethionine and
hyperintensities on T2-weighted sequences mostly in the AdoMet demethylated product S-adenosylhomocysteine via
posterior limb of the internal capsule. The proband deceased high performance liquid chromatography
at the age of 64 years. As morphological substrate for the Results: Methyl accepting capability in erythrocyte
slowly progressive clinical symptoms, comprehensive membrane proteins of ALS patients was lower than in
neuropathological and ultrastructural evaluation revealed a controls and abnormal L-isoaspartyl residues were
novel oligodendrogliopathy with distinctive, partly significantly higher in ALS patients (p<0.05). AdoMet
ubiquitinated and p62 positive fibrillar inclusions evolving concentration was about 50% lower in ALS patients while
into crystalloid deposits, containing elements of the AdoHcy levels were equivalent measuring a lower AdoMet/
oligodendroglial cytoskeleton (a- and b-tubulin, TPPP/p25). AdoHcy ratio in ALS patients (p<0.05).
In the central nervous system accumulation of crystalloid Discussion and conclusions: Methyl transfer reactions
structures has been related to histiocytes but not to glial mediated by AdoMet are involved in oxidative damage
cells. repair. Increased formation of L-isoaspartyl residues is one
Conclusions: This study has implications for the of the major structural alterations occurring in erythrocyte
understanding on how the human central nervous system membrane proteins. Abnormal residues are converted into
reacts to protracted dysfunction and disruption of the normal L-aspartyl residues by methyltransferase (PIMT).
oligodendroglial cytoskeleton, including development of PIMT is ubiquitous and its repair function is crucial in
crystalloid structures, which have not yet been reported in erythrocytes. Our results suggest the presence of injured
neurodegenerative disease such as HSP. action of PIMT in ALS patients' erythrocytes with
accumulation of L-isoaspartyl residues responsible for
abnormal protein conformation. We hypothesize injured
mechanisms of DNA methylation dependent on AdoMet/
AdoHcy ratio with deregulated gene expression. DNA
hypomethylation damage epigenetic control of long-term
silencing genes expression with involvement in ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 275

P1495 P1496
Intravenous immunoglobulin for post- Increased neurotrophin-3 of the skin of
polio syndrome: a randomized, double- patients with amyotrophic lateral
blind, placebo-controlled study sclerosis
M. Turri1, L. Bertolasi1, E. Frasson2, M. Acler1, S. Ono, T. Yamano, T. Tsukie, H. Fukazawa,
M. Ferlisi1, F. Pimazzoni1, A. Gajofatto1, G. Didon2, K. Higashida, Y. Oketa, K. Yasui, H. Ishikawa,
M. Bordignon3, S. Vicentini1, E. Dall' Ora1, F. Brigo1, M. Nomura, H. Mikami, T. Watanabe, M. Suzuki
A. Fiaschi1, M. Martini4, B. Danzi4, S. Monaco1 Neurology, Teikyo University Chiba Medical Center,
1Clinical Neurology, University of Verona, 2Neurology, Ichihara, Japan
3Management Control, Cittadella Hospital, Padova,
Objectives: Recently, it was found that immunoreactivities
4Rehabilitation Medicine, Malcesine, Italy
of ciliary neurotrophic factor (CNTF), leukaemia inhibitory
Objective: Post-polio syndrome refers to an increase of factor (LIF) and insulin-like growth factor-I (IGF-I) were
disabilities experienced by many polio survivors decades markedly increased in the skin of patients with amyotrophic
after acute infection expressed by a clinical worsening or lateral sclerosis (ALS) compared with controls. These
appearance of new symptoms. Pro-inflammatory cytokines observations serve to emphasize the potential importance of
production within the CNS indicates an underlying neurotrophic factors in ALS. Neurotrophin-3 (NT-3) also
inflammatory process. The aim of this paper is to describe belongs to the neurotrophic factors and is known to promote
the effects of intravenous immunoglobulin in post-polio motoneuron survival. However, little is known concerning
syndrome. NT-3 of skin in ALS patients.
Design: Single-center randomized, double-blind, placebo- Methods: We examined NT-3 immunoreactivity of biopsy
controlled study of efficacy of intravenous immunoglobulin specimens of skin overlying the left biceps from 13 ALS
in post-polio syndrome. patients and 13 control subjects with other neurologic
Subjects : 50 patients were randomly assigned to receive disorders. A densitometric analysis was performed using an
infusion of either intravenous immunoglobulin or placebo. image analysis program.
Methods: Primary endpoint was to demonstrate the efficacy Results: The optical density for NT-3 immunoreactivity of
of immunoglobulin in improving quality of life measured the epidermis in ALS patients (meanSD, 1.070.38) was
with short-form-36 (SF-36) questionnaire increasing significantly higher (p<0.01) than in control subjects
physical component score (PCS). Secondary endpoints (0.520.22). The optical density of the reticular dermis in
were improvement of physical performance, pain, fatigue ALS patients (meanSD, 0.710.36) was also significantly
and muscle strength. Patients were tested before the first higher (p<0.001) than in controls (0.230.31). The above
infusion and two and four months thereafter. densities of NT-3 immunoreactivity in ALS patients showed
Results: SF-36 physical component score did not a progressive increase in relation to duration of illness. This
significantly improve in patients who received positive correlation was highly significant (r=0.89, p<0.001
immunoglobulin, but we obtained a statistically significant and r=0.73, p<0.001, respectively) in the epidermis and in
improvement in SF-36 mental component score (MCS) the reticular dermis.
(p=0.015) and the subscale scores for physical and Conclusion: These data suggest that NT-3 may have a
emotional roles (p=0.05, p=0.023) two months after trophic role in skin of ALS patients and may help to explain
treatment. No difference was found in physical performance, why decubitus formation is rare in ALS.
pain, fatigue and muscle strength between the two groups.
None of the outcome variables tested four months after
treatment differed significantly between the groups.
Conclusions: Single treatment of intravenous
immunoglobulin improves MCS and physical and emotional
role subscales, but does not improve SF-36 PCS. Further
studies are needed to identify responding subgroups and to
test the effects of immunoglobulin after repeated treatments.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


276 Posters, Sunday 9 September

P1497 P1498
Impaired cytoplasmic-nuclear transport Evaluation of autonomic dysfunction in
(intranuclear changes and nuclear patients with amyotrophic lateral
envelope alterations) occurs at the early sclerosis using the heart rate variability
pre-symptomatic stage of amyotrophic (HRV)
lateral sclerosis K.-W. Oh, S.-H. Lee, S.-I. Oh, S.H. Kim
Y. Nagara, T. Tateishi, S. Hayashi, R. Yamasaki, Neurology, Hanyang University, Seoul, Republic of Korea
M. Kawamura, H. Kikuchi, K.M. Iinuma, Y. Ohoyagi, Background and aims: Amyotrophic lateral sclerosis
J.-I. Kira (ALS) is a neurodegenerative disorder characterized by
Department of Neurology, Neurological Institute, Graduate progressive loss of motor neurons, however it is increasingly
School of Medical Sciences, Kyushu University, Fukuoka, recognized that non-motor manifestations may occur,
Japan including autonomic nervous system dysfunction.
The present study aimed to examine the temporal Therefore, our first objective in these studies was to evaluate
relationship of immunohistochemical changes in the autonomic dysfunction in patients with ALS. Second
anterior horn cells (AHCs) from mutant superoxide objective was to define associated clinical characteristics
dismutase 1 (mSOD1) transgenic mice related to impaired and heart rate variability (HRV)
cytoplasmicnuclear transport as a potential pathomechanism Methods: 86 patients with sporadic ALS were compared to
of amyotrophic lateral sclerosis (ALS) and to examine 213 healthy controls. HRV and clinical characteristics
whether these changes occur in animal and human (duration of the disease, site of onset, ALS functional rating
specimens. We performed immunohistochemical analyses scale-revised (ALSFRS-R) was collected. 40 of 86 patients
for six autopsied patients with ALS, 6 non-neurologic were examined twice within a month to evaluate reliability
disease controls, 18 mSOD1 transgenic mice, and 18 non- of HRV.
transgenic mice. Compared to non-transgenic mice aged 8 Results: A decrease in HRV was found in the ALS patients,
weeks, consequently, mSOD1 transgenic mice aged 12, 16, indicating dysfunction of autonomic cardiac control
and 18 weeks exhibited the following: 1) chronological (p<0.0001). ALSFRS-R was positively associated with
significant decreases in the immunostaining intensity of a HRV (p<0.05). There were no differences of HRV
nucleoporin Nup62 in the AHC nuclear envelope; 2) parameters between first and second exam of HRV.
chronological significant increases in morphological Conclusions: These results suggest that cardiac autonomic
irregularities of the AHC nuclear envelope immunostained dysfunction in patients with ALS has sympathetic-activated
with Nup62; and 3) chronological significant decreases in balanced with decreasing both sympathetic and
the immunostaining intensity of karyopherin b1 in the AHC parasympathetic components. HRV is related to disability
nucleus. mSOD1 transgenic mice showed significant of the disease.
decreases in the immunostaining intensity of vascular
endothelial growth factor (VEGF) and increases in hypoxia-
inducible factor 1a (HIF-a) in the AHC nucleus. Our study
indicated that impaired cytoplasmicnuclear transport
(intranuclear changes and nuclear envelope alterations)
occurred at the early presymptomatic stage (8 weeks after
birth) in the mSOD1 mice, with nuclear envelope alterations
(decreased immunostaining intensity and increased
morphological irregularities) presumably preceding
intracytoplasmic changes. These data indicate the
correlation of immunohistochemical changes in the AHCs
from ALS model animals and ALS patients. Further study
will be required to specify the cell compartment where the
changes occur first.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 277

P1499 P1500
Targeting GPNMB-mediated neuronal Review of the compliance with the Awaji-
protection as a novel therapeutic strategy Shima criteria for the diagnosis of MND in
for amyotrophic lateral sclerosis a clinical setting
H. Tanaka, M. Shimazawa, M. Kimura, M. Takata, R. Sans1, E. Villamil2
K. Tsuruma, H. Hara 1Clinical Neurophysiology, Betsi Cadwaladr University

Molecular Pharmacology, Department of Biofunctional Health Board, Rhyl, UK, 2Clinical Neurophysiology, Somnia,
Evaluation, Gifu Pharmaceutical University, Gifu, Japan Malaga, Spain
Introduction: Amyotrophic lateral sclerosis (ALS) is an Introduction: After the change in diagnostic criteria for
incurable and fatal neurodegenerative disease characterized MND there has been an enhanced role of EMG in the
by the loss of motor neurons. Despite substantial research, diagnosis of the condition.
the causes of ALS remain unclear. Understanding these Objective: This is an audit study to assess the compliance
causes may be critical for the development of effective with recommended guidelines in a General DGH.
therapies. The purpose of this study was to identify a novel Patients and methods: All patients referred to our
target factor involved in ALS. department to specifically exclude MND were included, in
Methods: Glycoprotein non-metastatic melanoma protein a period of 12 months (July 2010 to July 2011). The EMG
B (GPNMB) was identified as an ALS-related factor using studies were revised in order to determine the amount of
DNA microarray analysis with mutant superoxide dismutase muscle studies by EMG, its locations and the findings in
(SOD1G93A) mice. The crucial roles of GPNMB were each muscle. NCS were also reviewed to exclude other
demonstrated through in vitro and in vivo biochemical and causes of symptoms.
morphological methods, including clinical tissue sample Results: 37.5% of patients referred had definitive or
studies. probable MND according to Iwaji-Shima criteria; 37.5%
Results: GPNMB was greatly induced in the spinal cords had polyneuropathies and 18.75% had normal results. Other
of ALS patients and a mouse model as the disease findings were present in 6.25% of cases. Mean number of
progressed. It was especially expressed in motor neurons muscles sampled was 5, in 2 or 3 territories.
and astrocytes. In an NSC34 motor neuron cell line, Conclusions: Most patients had no need for extensive
glycosylation of GPNMB was inhibited by interaction with EMG sampling as the changes were diagnostic enough.
SOD1G93A, increasing motor neuron vulnerability, However, some patients might have benefited of more
whereas extracellular fragments of GPNMB secreted from extensive bulbar and thoracic regions EMG sampling.
activated astrocytes attenuated the neurotoxicity of
SOD1G93A in neural cells. Furthermore, GPNMB
expression was substantial in the sera of sporadic ALS
patients compared to that of other diseased patients.
Conclusion: This study suggests that GPNMB can be a
novel target for therapeutic intervention for suppressing
motor neuron degeneration in ALS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


278 Posters, Sunday 9 September

P1501 P1502
Perception of ALS patients, carers and The association between VEGF-2578C/A
doctors regarding clinical management polymorphism and amyotrophic lateral
C. Oreja-Guevara1, F.J. Rodriguez de Rivera1, sclerosis in a Russian population
J. Mascias2, J.L. Muoz-Blanco3, J. Esteban4, L. Galan5, E.V. Lysogorskaia, N.Y. Abramycheva, M.N. Zakharova,
J.L. Villanueva-Marcos6 S.N. Illarioshkin
1Neurology, University Hospital La Paz, Idipaz, 2Neurology, Research Center of Neurology Russia Academy of Medical
Hospital Carlos III, 3Neurology, Hospital Gregorio Science, Moscow, Russia
Maraon, 4Neurology, Hospital 12 de Octubre, 5Neurology,
Introduction: Amyotrophic lateral sclerosis is a devastating
University Hospital San Carlos, 6Advance Directives
neurodegenerative disorder with an important genetic
Register, Regional Health Service, Madrid, Spain
contribution. Studies of association of ALS with the
Non-invasive positive pressure ventilation (NIPPV) 2578C/A, 1154G/A and 634G/C polymorphisms in the
increases survival and improves patient's quality of life. vascular endothelial growth factor (VEGF) gene yielded
Invasive mechanical ventilation (IMV) can prolong survival contradictory results. We investigated the VEGF 2578/A
in ALS. Percutaneous endoscopic gastrostomy (PEG) polymorphism in a Russian cohort of patients with ALS.
improves nutrition but also no improvement in QOL is Methods: An ALS group comprised 192 patients (103/192
known. The use of these procedures has often been males and 89/192 females) aged from 20 to 83 years (52
controversial. 13.4); and a control group comprised 128 age- and sex-
Objective: To evaluate the point of view of ALS patients, matched persons. All studied individuals were Slavs.
carers and physicians about the use of NIPPV, IMV, PEG. TaqMan PCR was used for the VEGF 2578/A detection.
Methods: 30 ALS patients, 30 caregivers and 30 physicians Results: The significant difference of the allele distribution
from four different hospitals were examined with a cross- was observed between ALS cases and controls (2=11.1;
sectional survey. The survey consists of three groups of =0.004). The 2578A/A genotype increased the disease risk
questions related to accepting or refusing the procedures in to an adjusted odd of 1.66 (95% CI 1.03-2.29). Males
different levels. carrying 2578A/A had more increased risk of ALS
Results: Patients: mean age: 56.5; 76% males; mean (OR=2.18; 95% CI 1.90-2.47). We found significant
ALSFRS 31.6; duration of disease 568.6 days (sd 354.4); association of the 2578A/A genotype with earlier disease
FVC 72.6. 90% of ALS patients have a carer. All doctors onset (OR=1.3; 95% CI 1.21-1.40) and rapid progression
agree to use NIPPV in all patients and situations and 96% (OR=1.7; 95% CI 1.27-2.13).
of them to use the PEG for nutrition. With respect to NIPPV Conclusions: Our data show that VEGF 2578/A genotype
50% of patients agree with the opinion of physicians and can modulate the risk of ALS in a Russian population.
caregivers and 26.6% regarding PEG. IMV was the most
controversial procedure; only 20% of patients accepted
IMV, like the caregivers and doctors. Physicians showed
very different opinions: from acceptation to rejection of
IMV.
Conclusion: Our findings demonstrate that the perception
of the patients, caregivers and doctors in relation to PEG,
IMV and NIPPV is very different. This study identified
some issues to take into account in order to better meet their
needs. Decisions should be taken by all together.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 279

P1503 P1504
X-linked bulbar and spinal muscular IVIg treatment in post-polio patients;
atrophy; experiences from a single center quality of life before and after 6 months -
J.K. Kim1, D.H. Kim1, S.H. Choi2 results from an open clinical study
Dong-A University Hospital, 2Wallace Memorial Baptist
1
G. stlund, L. Broman, L. Werhagen, K. Borg
Hospital, Busan, Republic of Korea Division of Rehabilitation Medicine Department of Clinical
Background and objectives: Bulbar and spinal muscular Sciences Karolinska Institutet Danderyd Hospital,
atrophy (BSMA, Kennedy disease) is an X-linked Stockholm, Sweden
degenerative disease of the motor neuron caused by an Introduction: Vitality (Gonzalez et al 2006) and pain
expansion of CAG repeat within the androgen receptor (Farbu et al 2007) were significantly improved in post-polio
gene. We tried to characterize the presenting manifestation (PPS) patients after IvIg treatment. Characteristics of
for early and easy clinical diagnosis of BSMA. responders and non-responders have not been pin pointed.
Methods: 9 clinically compatible BSMA patients were Aims: To evaluate quality of life after IvIg treatment in PPS
included. All patients were from a single center during the patients before and after 6 months and to identify parameters
period 2010 to 2011. Initial clinical manifestations, influencing the outcome.
neurological examinations and laboratory findings including Methods: Open trial, prospective follow-up study. 113 PPS
genetic study were investigated. patients from a Swedish PPS outpatient-clinic who had
Results: Mean age was 47.9 years and mean duration from received one IvIg treatment were included. Quality of life,
symptom onset to diagnosis was 45.9 months. From the pain and physical activity were measured by Short form 36
neurological manifestations, the most frequent and (SF-36), Visual Analogue Scale (VAS) and Physical Activity
consistent finding was perioral tremor (100%) typically Scale for the Elderly (PASE) and were answered before
provoked by movements such as talking and postural hands treatment and after 6-month. Clinical examination of
tremor (100%). Gynecomastia was observed in 5 patients medical records was performed before IvIg treatment.
(62.5%) and only half of cases had bulbar symptom and Descriptive statistics were done for all variables before
sign. Fasciculations and hypo/a-reflexia were found only in treatment. To compare SF-36, VAS-pain and PASE before
4 patients (50%). All patients showed the pattern of chronic treatment and at 6-month follow-up, Wilcoxon non-
motor dominant polyneuropathy with axonal involvement parametric tests were performed.
in NCSs and needle electromyography. Decremental Results: SF-36: Bodily pain (BP) (p=0.002), and Vitality
responses in Jolly test were found in all cases from 6 (VT) (p=0.008) were significantly increased after 6 month.
patients studied. This means abnormal neuromuscular Increased BP and VT scores were seen in those under 65
transmission is not a rare manifestation of BSMA during a years of age and in those with paresis only in lower
certain stage of this disease. extremities. Increasing BP, was seen in those who were
Conclusion and discussion: Perioral tremor is the most working, increasing VT was seen in patients with pain.
frequent and consistent finding in BSMA and it showed a Conclusions: SF-36: Bodily pain and vitality were
typical action induced pattern. This supports us making improved 6 months after IVIG treatment. Age below 65
clinical diagnosis of BSMA easier. The pattern and years and pain before treatment may be indicators for future
distribution of motor weakness in tongue and limb muscle identification of responders.
are variable.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


280 Posters, Sunday 9 September

P1505 P1506
The involvement of GPNMB in neuronal Compound heterozygosity with two
cell death by endoplasmic reticulum mutations in the HEXB gene produces
stress adult Sandhoff disease presenting as a
M. Takata, H. Tanaka, K. Tsuruma, M. Shimazawa, motor neuron disease
H. Hara S.-Y. Kang
Molecular Pharmacology, Department of Biofunctional Neurology, School of Medicine, Jeju National University,
Evaluation, Gifu Pharmaceutical University, Gifu, Japan Jeju, Republic of Korea
Introduction: Glycoprotein transmembrane non-metastatic Introduction: Sandhoff disease is a rare autosomal
melanoma B (GPNMB) is a single transmembrane protein. recessive metabolic disorder of GM2 gangliosides. It is
GPNMB is involved in the pathogenesis of several cancers caused by a lack of functional N-acetyl--D-glucosminidase
and glaucoma. However, the involvement of GPNMB in the A and B due to mutations in the HEXB gene. Little
central nervous system is unclear. Recently, it has been information is available on molecular defects involved in
reported that GPNMB is localized in the rough endoplasmic adult Sandhoff disease presenting as motor neuron disease
reticulum (ER), suggesting that GPNMB has several roles phenotype.
in ER. The purpose of this study was to investigate the Case report: We describe a 55-year-old woman with adult
effects of GPNMB against ER stress-induced neuronal cell Sandhoff disease presenting as motor neuron disease. The
death. assay of total hexosaminidase involving A and B showed
Methods: We used mouse motor neuron (NSC34) cells and decreased level of these activities. Analysis of HEXB gene
evaluated the roles of GPNMB against ER stress by demonstrated two point mutations. The two mutations were
GPNMB knockdown using siRNA and treating with located at the exon 5 (c.619A>G) and exon 11 (c.1250C>T).
recombinant extracellular fragments of GPNMB. Conclusion: Compound heterozygosity of these two
Furthermore, we investigated the protein and mRNA mutations may trigger the development of adult Sandhoff
expressions of glucose-regulated protein 78 (GRP78/BiP) disease with a motor neuron disease phenotype. In patients
and C/EBP homologous protein (CHOP), ER stress-related with motor neuron disease in the setting of a possibly
factors. recessive disorder, Sandhoff disease should be suspected,
Results: The protein expression of GPNMB was increased even when the onset age is over 50 years.
by thapsigargin-induced ER stress as well as the protein and
mRNA expressions of BiP and CHOP. GPNMB knockdown
significantly increased the ER stress-induced neuronal cell
death, and suppressed the induction of BiP protein and
mRNA, but not CHOP. Moreover, the recombinant GPNMB
had protective effects against ER stress-induced neuronal
cell death with no effects on BiP expression.
Conclusion: These results suggest that intracellular
GPNMB may regulate the transcription of BiP and have
protective effects against thapsigargin-induced ER stress.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 281

P1507 P1508
Functional gait and pulmonary function in A case report of a patient with ALS and
patients with amyotrophic lateral an obsessive-compulsive disorder
sclerosis: a direct relationship carrying expansion of C9ORF72
B. Heredia Camacho1, M. Castillo1, A. Hochsprung1, C. Moglia, A. Calvo, A. Canosa, E. Bersano, A. Ilardi,
G. Izquierdo Ayuso2 G. Restagno, A. Chio'
Biofunctional Neuropyisiotherapy, 2Neurology, University
1 Neuroscience, University of Torino, Italy
Hospital Virgen Macarena, Sevilla, Spain It has been recently reported that a large proportion of
Introduction: Amyotrophic lateral sclerosis (ALS) is a patients with familial ALS and frontotemporal dementia are
neurodegenerative disease marked by progressive loss of associated with a hexanucleotide repeat expansion in the
motor neurons, muscle wasting and respiratory dysfunction. first intron of c9orf72. We describe a patient with a diagnosis
Hypothesis: one of the factors influencing the progressive of ALS-FTD with psychiatric onset. At the age of 50 hour
loss of functional gait is respiratory dysfunction. patient developed a depressive disorder. Some months later,
Methods: Participants: 11 patients with suspected or muscle weakness at the right hand occurred, followed by
definitive diagnosis of ALS, with a functional gait, included worsening of the mood disorder. The collection of familial
in a Physical Therapy programme of once-a-week history revealed that the patient's father died after a 5-year
assistance. Informed consent was obtained from each history of ALS. Neither cognitive nor behavioural
subject. Five consecutive measures of VC and FEV1 were impairment were reported. Genetic analysis revealed a
obtained from spirometry. Walking was documented using hexanucleotide expansion in the first intron of the c9orf72
an 8-meters GaitRite electronic walkway, and a Functional gene. MRI documented a marked hyperintensity along the
Ambulance Profile (FAP) was obtained. Each measure was corticospinal tract; MRI fibre-tracking revealed bilateral
observed and a graphic was made in order to compare each reduction of fractional anisotropy along the corticospinal
parameter (FEV1, VC and FAP). A 2-way ANOVA and a tract. Brain PET-CT presented reduced uptake of the
Bonferroni post-test were made. radioactive tracer in the motor cortex bilaterally, in the
Results: 6 patients obtained a negative progression in FAP. fronto-mesial cortex bilaterally, between the anterior and
Of them, 5 registered a lower FEV1 and 3 subjects, a lower the middle cingulate gyrus and in the postero-lateral
VC. 2 of them, obtained the same measure in VC. At the occipital cortex bilaterally. The clinical and
same time, from 3 patients with a better FAP score, 2 neuropsychological assessment was consistent with a
obtained a better VC and also a higher FEV1. Both measures diagnosis of FTD, associated to OCD, hallucinations and
have a significant interaction (p<0.0001). depressive mood disorder. Later the patient developed
Conclusions: FAP score is positively correlated with CV dysarthria, dysphagia, lower limbs weakness and
and FEV1 measures. Furthermore, these functional hypotrophy and worsening of spasticity at upper and lower
respiratory measures demonstrate a good correlation with limbs. 14 months after the onset of the motor neuron
the functional evolution in patients with ALS. In addition, a disease, he is still alive, wheelchair-bound.
specific respiratory program must be included in a The association of ALS, FTD, depression, psychotic
Biofunctional Neurophysiotherapy program. manifestations and OCD could set up a distinctive
Because of the small number of patients included, more phenotype related to c9orf72 gene expansion. Nevertheless,
studies are necessaries. this hypothesis needs to be confirmed by further
observations.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


282 Posters, Sunday 9 September

P1509 P1515
Effects of neurotrophic factors on Metabolomics as a tool for studying
oxidative and nitrosative stress in neurodegenerative diseases
amyotrophic lateral sclerosis G93A A. Wuolikainen1,2, the Erling-Persson Metabolomics
murine model Project at Ume University
1Department of Chemistry, 2Computational Life Science
I. Kochergin1,2, L. Brylev1,2, M. Onufriev2, I. Barskov1,
Cluster (CLiC), Ume University, Ume, Sweden
M. Zakharova1, I. Zavalishin1, N. Gulyaeva2
1Research Center of Neurology RAMS, 2Institute of Higher

Nervous Activity and Neurophysiology, RAS, Moscow, Russia P1516


Neurolathyrism in Ethiopia
P1510 H.D. Belay, H. Demissie
Amyotrophic lateral sclerosis as a Department of Neurology, Addis Ababa University, Addis
paraneoplastic syndrome Ababa, Ethiopia
A. Riahi, M. Messelmani, H. Khaled, M. Mansour,
J. Zaouali, R. Mrissa P1517
Neurology, Military Hospital, Tunis, Tunisia
Cervical flexion myelopathy caused by a
spinal intradural cyst
P1511 D. Kondziella1, L.R. Damhave1, A. Wagner2, K. Hansen1
Neurodegenerative overlap syndrome: 1Department of Neurology, 2Department of Neuroradiology,

Copenhagen University Hospital, Rigshospitalet,


a case report
Copenhagen, Denmark
M. Minar, P. Valkovic
Second Department of Neurology, Comenius University,
Bratislava, Slovak Republic P1518
The therapeutic efficacy of herbal
P1512 medicine for olivopontocerebellar atrophy
Vestibular evoked myogenic potentials in versus genetic spinocerebellar ataxia 6
amyotrophic lateral sclerosis T. Okabe
Department of Integrated Traditional Medicine, University
N. Tarasevich, S. Likhachev, U. Lukashevich,
of Tokyo, Japan
Y. Rushkevich
Republican Research and Practical Centre of Neurology and
Neurosurgery, Minsk, Belarus P1519
The use of sulbactam treatment in ALS
P1513 patients
Hirayama disease: a report of two cases N. Polat1, H.A. Idrisoglu2, A. Sazci2
1Merd Company, 2Department of Neurology, Medical
A. Jaoua, S. Benamor, S. Benammou
CHU Sahloul, Sousse, Tunisia Faculty of Istanbul, Turkey

P1514 P1520
Cramp-fasciculation syndrome in the The controversy behind Mills syndrome
differential diagnosis of amyotrophic E. Tufanoiu, A.M. Corfu
Neurology, Fundeni Clinical Institute, Bucharest, Romania
lateral sclerosis
J. Domnguez Brtalo1, A. Hernndez Gonzlez1,
B. Miguel Martin1, C. Valencia Guadalajara1,
A. Lopez Garca1, G. Martn Palomeque2, A. Castro
Ortiz2
1Neurology, 2Neurophysiology, Hospital General Ciudad

Real, Spain

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 283

Movement disorders 1 P1522


A panel of five CSF biomarkers can be
P1521 used with high accuracy in the differential
Interim results from an international, diagnosis of patients with dementia
open-label study of levodopa-carbidopa and/or parkinsonism
intestinal gel in patients with advanced S. Hall1,2, A. hrfelt3, R. Constantinescu4,
Parkinsons disease: efficacy results by U. Andreasson3, Y. Surova1,2, F. Bostrm1,5, C. Nilsson6,
country H. Widner1,2, H. Decraemer7, K. Ngga1,5, L. Minthon1,5,
E. Londos1,5, E. Vanmechelen7, B. Holmberg4,
P. Odin1,2, A.J. Espay3, A.D. Vanagunas4, R.A. Hauser5,
H. Zetterberg3, K. Blennow3, O. Hansson1,2,5
H.H. Fernandez6, D.G. Standaert7, W.Z. Robieson8, 1Department of Clinical Sciences, Lund University,
Y. Pritchett8, K.L. Widnell8, K. Chatamra8, J. Benesh8, 2Department of Neurology, Skne University Hospital, Lund,
R.A. Lenz8 3Department of Psychiatry and Neurochemistry, Institute of
1Klinikum-Bremerhaven, Bremerhaven, Germany, 2Skane Neuroscience and Physiology, The Sahlgrenska Academy at
University Hospital, Lund, Sweden, 3University of Cincinnati University of Gothenburg, 4Department of Neurology,
Academic Health Center, Cincinnati, OH, 4Northwestern Sahlgrenska University Hospital, Gothenburg,
University, Chicago, IL, 5University of South Florida, 5Neuropsychiatric Clinic, Skne University Hospital, Malm,
Tampa, FL, 6Cleveland Clinic, Cleveland, OH, 7University of 6Department of Cognitive Medicine, Skne University
Alabama at Birmingham, Birmingham, AL, 8Abbott, Abbott Hospital, Lund, Sweden, 7ADx NeuroSciences, Gent, Belgium
Park, IL, USA
Introduction: Due to overlapping symptomatology, it can
Introduction: Fluctuating blood levels of levodopa are be difficult to clinically distinguish Parkinsons disease
associated with motor complications in Parkinsons disease (PD) from atypical parkinsonism, i.e. multiple system
(PD). Levodopa-carbidopa intestinal gel (LCIG) is delivered atrophy (MSA), progressive supranuclear palsy (PSP) and
continuously via a percutaneous endoscopic corticobasal degeneration (CBD). There is also an overlap
gastrojejunostomy (PEG-J) tube. in clinical symptoms and brain pathologies between
Methods: An international, 54-week, open-label study of dementia disorders, including Alzheimers disease (AD),
LCIG in patients with advanced PD experiencing motor dementia with Lewy bodies (DLB) and PD with dementia
fluctuations despite optimized standard therapy is ongoing (PDD). There is thus a need for biomarkers to improve the
(NCT00335153). Individualized LCIG dosing was diagnosis of these disorders.
instructed to be 16 hr/day. Efficacy outcomes included Method: CSF samples (n=453) were obtained from
Off time and On time with and without troublesome controls, subjects with PD, PDD, DLB, AD, PSP, MSA and
dyskinesia (TD). Adverse events (AEs) were monitored. CBD. A newly developed Luminex multiplex assay was
This interim analysis included assessments performed on or used for simultaneous quantification of -synuclein, A42,
prior to the data cut-off date of Nov. 25, 2011. Efficacy and T-Tau and P-tau. ELISA was used to analyze NF-L.
safety endpoints were tabulated across 16 enrolling Results: CSF -synuclein was decreased in patients with
countries. PD, PDD, DLB and MSA, but increased in AD. CSF
Results: At data cut-off, 354 subjects had nasojejunal tube -amyloid1-42 was decreased in DLB, but even further
insertion and 323 had received subsequent PEG-J tube. decreased in AD. CSF T-Tau and P-Tau were increased in
Across countries, mean [SD] baseline values ranged from: AD. Multivariate analysis revealed that these four
Age, 53.9[6.3]-70.1[6.9] yrs; PD duration, 9.2[3.9]- biomarkers could differentiate AD from DLB and PDD with
16.0[6.4] yrs; Off time, 5.7[1.9]-8.4[3.3] hr/day; On an AUC of 0.90, where -synuclein and T-Tau contributed
time without TD, 6.7[2.9]-8.5[2.7] hr/day; On time with most to the model. NF-L was substantially increased in
TD, 0.2[0.6]-2.3[2.2] hr/day. In each country, the change atypical parkinsonian disorders (PSP, MSA, and CBD).
from baseline in Off time and On time without TD was Multivariate analysis revealed that NF-L alone could
significantly improved (p<0.05) as early as week 4, and differentiate PD from atypical parkinsonian disorders with
sustained through week 54. At week 54, mean [SD] Off an AUC of 0.93.
time improvements ranged from -3.4[3.9] to -5.8[2.7]hr/ Conclusions: CSF -synuclein improves the differential
day, and improvements in On time without TD ranged diagnosis of AD vs. DLB and PDD, and when combined
from 4.1[3.8]-6.3[3.5]hr/day. Additional analyses of with established AD-biomarkers the diagnostic accuracy
baseline characteristics among countries will be included in reaches 90%. Neurofilament light chain alone can
the presentation. AE profiles were similar across countries. differentiate PD from atypical parkinsonian disorders with
Conclusions: LCIG consistently improved motor symptoms an accuracy of 93%.
and resulted in similar AE profiles across countries.
Support: Abbott

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


284 Posters, Sunday 9 September

P1523 P1524
Assessing postural stability in early Dystonia in corticobasal degeneration:
Parkinsons disease - how valid are a review of the literature on 404
commonly used clinical assessment pathologically proven cases
tests? M. Stamelou, A. Alonso-Canovas, K. Bhatia
I.M. Claesson1,2, A. Sthle1 UCL Institute of Neurology, London, UK
Institution of Neurobiology, Care Sciences and Society,
1
Background: Dystonia is considered one of the classical
Karolinska Institutet, 2Division of Physiotherapy, Karolinska features of corticobasal degeneration and is reported in up
Universitetssjukhuset, Stockholm, Sweden to 83% in clinical series.
Background: Postural instability is an important symptom Methods: We reviewed the literature on 404 pathologically
of Parkinsons disease (PD) that may cause problems in proven cases and noted the presence, age at onset,
daily life. It is, therefore, important to screen for this distribution, evolution and treatment of dystonia, associated
problem to identify those at risk for falling and to reduce features and final clinical diagnosis.
hazardous falls. The primary aim of this study was to Results: Dystonia was present in only 37.5% of the 296
examine the suitability of different clinical assessment tests cases, with adequate information. The majority of them
for the screening of postural stability in early PD. presented with a corticobasal syndrome and dystonia
Methods: 28 people with idiopathic PD were assessed with occurred in the first two years from disease onset, affecting
commonly used clinical assessment tests, i.e. the Berg the upper limb. In phenotypes where dementia was the
Balance Scale (BBS), Timed Up and Go (TuG), Timed Up cardinal feature, dystonia tended to appear later in the
and Go-cognition (TuG-cog), the Modified Figure of Eight disease course and to affect more cervical region and face.
(MFE), walking 30 meters at self-paced and maximum 54% of 374 cases, where the phenotype was available,
speeds, together with disease severity rating, self-reported presented as corticobasal syndrome, 15% as frontotemporal
fall history, the Falls Efficacy Scale-International (FES-I) dementia and 10.7% as progressive supranuclear palsy.
and the Freezing of Gait Questionnaire (FOG-Q). The 48.8% of corticobasal syndrome cases presented with
Bckstrand, Dahlberg, and Liljens Balance Scale (BDL), dystonia, 51.5% with myoclonus, 86.3% with apraxia and
with more complex balance demands, was also validated. 100% with akinetic-rigid syndrome.
Correlations between the different assessment tests, Conclusion: Despite dystonia being an inclusion criterion
questionnaires, ratings of disease severity, and fall history in all sets of clinical criteria for corticobasal degeneration,
were calculated. this was present in only one third of the pathologically
Results: Tests that included walking with a turning task proven cases presented here. Dystonia and myoclonus were
were more predictive of risk of fall and more highly present in about half of all cases with corticobasal syndrome,
correlated to the disease severity rating scales than were the implying that these features may not be that frequent in
other tests. The questionnaires FES-I and FOG-Q did not corticobasal syndrome as are akinetic-rigid syndrome and
correspond to actual falls or to disease severity. apraxia. Dystonia and myoclonus almost co-occurred in our
Conclusions: To evaluate postural stability in early PD, we analysis, suggesting a possible association. More accurate
suggest a battery of tests that includes the TuG, the TuG- characterization of dystonia in corticobasal degeneration
cog, and the MFE. For evaluating exercise intervention in would be of importance for clinical diagnosis and
early PD the BDL is a better test than the BBS. development of treatment strategies.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 285

P1525 P1526
Clinical features of corticobasal syndrome Characterization of gait disturbance in
with midbrain atrophy patients with normal pressure
R. Hayashi, T. Oeda, A. Umemura, M. Kousaka, hydrocephalus
S. Tomita, H. Nakano, H. Sawada R. Schniepp1, M. Wuehr2, C. Pradhan2, K. Jahn1
Clinical Research Center and Neurology, National Hospital 1Neurology, University of Munich, 2Integrated Research and
of Utano, Kyoto, Japan Treatment Center for Vertigo, Oculomotor and Balance
Introduction: Mesencephalic tegmentum atrophy can be a Disorders, University Munich, Germany
maker for progressive supranuclear palsy (PSP). However, In normal pressure hydrocephalus (NPH) a disturbance of
patients with corticobasal syndrome (CBS) often share gait is typically the first symptom to appear. The most
common pathological and clinical features with PSP, and common test for the outcome of a shunt operation is a CSF
some cases show midbrain atrophy (MA). The purpose of tap test. In this study we investigated which gait parameters
this study was to clarify clinical features in CBS with MA positively respond to CSF tapping and at which time period
comparing that without MA. after tapping this response is most pronounced. The study
Methods: 34 patients with CBS (male 32%, age 72.77.6), cohort comprised 41 patients (72.210.0 years, 12 females).
46 patients with PSP (male 67%, age 73.17.3), and 31 Gait analysis was performed on a pressure-sensitive gait
healthy controls (male 45%, age 66.712.1) were carpet (GaitRITE). Patients were tested under two different
investigated. Midbrain area was measured on planimetry speed-, two cognitive dual-task- and one motor dual-task-
using mid-sagittal MRI. The cut-off point of midbrain area condition. A Principal Component Analysis was performed
discriminating between PSP and control was 112mm2 in order to identify relevant gait parameters. Extracted
(sensitivity 91.3%, specificity 90.3%). We classified CBS parameters were then analyzed at different time points and
patients into two groups, CBS with MA (112mm2) and under different gait analyses. Positive effects of CSF
without MA (>112mm2), and compared clinical features tapping were most pronounced during walking with
such as age, sex, FAB, MMSE, postural instability with fall preferred walking speed. Patients did also show
2 years (PIF) and vertical gaze palsy (VGP) between the improvements while walking with maximal speed and for
groups. The clinical factors which are independently the motor dual-task but not for the two cognitive dual task
associated with MA were identified by a general linear conditions. Velocity, stride length, stride time and double
model. support time were the parameters that responded most
Results: Comparing the clinical factors in the two groups, positively (p<0.01). In nearly all cases improvements were
patients with MA were older and more often showed PIF detectable not till 12h or even 48h after the CSF tapping.
and VGF than patients without MA. The general linear These results indicate that in a multi-parametric analysis of
model analysis revealed that MA was independently the preferred walking pattern covers a positive CSF tap test
associated with PIF ( 21.3, p=0.004), VGP ( 13.8, best. In addition, gait examinations immediately after the
p=0.043) and FAB score ( 3.4, p=0.007). CSF tap test will overlook most gait improvements and
Conclusion: MA in patients with CBS was associated with therefore gait assessments at later time points are necessary
PSP-like features. to detect all positive effects on the walking performance.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


286 Posters, Sunday 9 September

P1527 P1528
Prepulse inhibition in idiopathic REM Hip fractures in people with idiopathic
sleep behaviour disorder, Parkinsons Parkinsons disease: incidence and
disease and multiple system atrophy outcomes
M. Zoetmulder1,2,3, H.B. Biernat1, M. Nikolic4, R. Rutherford1, R. Hancock1, A. Chaplin2, W. Gray3,
L. Korbo1, P.J. Jennum2,3 R. Walker3
1Neurological Department, Bispebjerg Hospital, 1Medical School, Newcastle University, Newcastle Upon

Copenhagen, 2Danish Center for Sleep Medicine, Tyne, 2Medicine, Northumbria Healthcare NHS Foundation
Department of Clinical Neurophysiology, Glostrup Hospital, Trust, Ashington, 3Medicine, Northumbria Healthcare NHS
Glostrup, 3Center for Healthy Aging, University of Foundation Trust, North Shields, UK
Copenhagen, 4Department of Clinical Neurophysiology, Introduction: People with Parkinsons disease (PD) are
Glostrup Hospital, Glostrup, Denmark thought to be at increased risk of hip fracture compared to
Introduction: Prepulse inhibition (PPI) of the acoustic the general population, with poorer outcomes post-surgery.
startle response is a measure of sensorimotor gating, in The aim of this audit was to establish the incidence of, and
which a weaker prestimulus (prepulse) inhibits the reaction outcomes from, hip fracture in people with and without PD
of an organism to a subsequent strong startling stimulus living in north-east England.
(pulse). The objective of this study was to determine Methods: Data from two previous prevalence studies in the
whether patients with idiopathic REM sleep behaviour same geographical area was used to estimate the number of
disorder (iRBD), Parkinsons disease (PD) and multiple people with PD living in the study area. Using data collected
system atrophy (MSA) have altered PPI. prospectively for the UK national hip fracture database, the
Methods: 21 PD patients with RBD, 20 PD patients without annual incidence of hip fracture in people with and without
RBD, 12 with iRBD, 10 with MSA and 20 healthy and PD was calculated. Type of fracture, time to surgery, time to
gender-matched controls entered and completed the study. discharge and 30-day outcomes from surgery were
A passive acoustic PPI paradigm was applied with prepulses compared for PD and non-PD patients.
5dB and 15dB above background noise at 30-, 60-, 120- and Results: The annual incidence of hip fracture was
300-ms intervals. significantly higher in people with PD across all age bands.
Results: Startle-response characteristics (latency and In those aged 60 years and over it was 2032 (95% CI 1945
habituation) did not differ between patients and controls to 2121) per 100.000 in people with PD and 552 (95% CI
(p0.05). Non-parametric analyses showed that MSA 507 to 599) per 100.000 in people without PD. The
patients had a significantly lower PPI than the other groups experience of PD and non-PD patients within hospital was
for the 60ms - 85dB and 120ms - 85dB prepulses. No remarkably similar. However, PD patients had poorer
differences in PPI were found between the other groups. mobility prior to hip fracture, took longer to be discharged
Conclusion: The present study suggests that prepulse to the community, and were less mobile post-surgery.
inhibition is markedly altered in MSA. Since striatal Conclusions: People with PD have a significantly higher
dopaminergic function and the pedunculopontine nucleus incidence of hip fracture than people without PD and post-
(PPN) play a major role in prepulse inhibition, these results surgical outcomes are poorer. Specific guidelines for
are in line with the functional involvement of the striatum managing people with PD who sustain a hip fracture may
and PPN in the pathogenesis of multiple system atrophy. improve outcomes.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 287

P1529 P1530
Cost effectiveness of Apomorphine The influence of striatal dopamine on the
infusion in the treatment of advanced modulation of upper limb locomotor
Parkinsons disease in the UK synergies
E. Walter, D. Mercsanits I.U. Isaias1,2,3, A. Cattaneo1, J. Volkmann3, A. Marzegan1,
Institute for Pharmaeconomic Research, Vienna, Austria G. Marotta4, P. Cavallari1, G. Pezzoli2
1Dipartimento di Fisiologia Umana, L.A.M.B. P.&L.
Objectives: Parkinsons disease (PD) is the second
commonest neurological disability and affects 100-180 Mariani, Universit degli Studi di Milano, 2Centro per la
people per 100.000 of the population and increases with Malattia di Parkinson e i Disturbi del Movimento, Istituti
age. Continuous subcutaneous apomorphine represents an Clinici di Perfezionamento, Milan, Italy, 3Neurologische
alternative treatment option of advanced PD with motor- Klinik und Poliklinik, Universittsklinik Wrzburg, Germany,
4Dipartimento di Medicina Nucleare, Fondazione IRCCS Ca
fluctuation. The purpose of this analysis was to estimate the
cost-effectiveness of Apomorphine infusion (APO) Granda Ospedale Maggiore Policlinico, Milan, Italy
compared with Levodopa/carbidopa intestinal gel (LCIG), Introduction: Reduced arm swing while walking is a
Deep-Brain-Stimulation (DBS) and Standard-of-care clinical hallmark of Parkinsons disease (PD) suggesting a
(SOC). direct involvement of the basal ganglia.
Methods: We developed a Markov-Model to simulate the Aim: We investigated the role of striatal dopamine on the
long-term consequences, disease progression (Hoehn&Yahr- modulation of upper limb synergies during walking at
stages 3-5, percentage of waking-time in the OFF-state), different velocities.
complications and adverse-events. Complications are Method: Patients were recruited according to several
different for the alternatives (e.g. pump problems in case of inclusion criteria, including normal spatio-temporal
LCIG, temporary/permanent complications in case of parameters of the stride at preferred gait speed. After 3-day
DBS). We include moderate and severe adverse-events and drugs-off state, 13 patients and 10 healthy controls (HC)
death. Monte-Carlo-simulation accounted for uncertainty. were asked to walk at preferred, slow and fast speed (12
The model includes 25 health-states. Probabilities were trials). Kinematics were measured using an optoelectronic
derived from RCT and open-label studies; direct costs system. Striatal dopaminergic innervation was measured by
(2012 ) from published sources from the payers FP-CIT and SPECT.
perspective. QALYs, life-years and costs were projected Result: At preferred gait speed, all patients showed reduced
over a life-time horizon and discounted at 3.5%. range of motion (ROM) at one arm and four bilaterally. In
Results: Over a life-time horizon, costs associated with PD, ROM of the most affected arm (median: 7, range:
apomorphine amounts to 46,053.42 and 2.05 QALYs (4.4 3.9-16.3) was significantly reduced compared to HC
LYs). Costs associated with LCIG are 92,753.42 and 2.40 (median: 25.27, range: 14.6-29.1; p<0.01). The range of
QALYs (5.3 LYs). The cost-saving amounts to 46,700 per gait velocities (Km/h) was similar between patients (range:
patient and the incremental-cost per QALY gained (ICER) 1.93-7.81, median: 3.49) and HC (range: 1.24-8.15, median:
was 129,903. Costs associated with DBS are 61,893.08 3.76). While walking at different velocities, patients
and 2.16 QALYs (4.98 LYs). Apomorphine dominates DBS. modulated arm swing similarly to HC (RSquare average,
ICER amounts to 143,215.73 favourable for apomorphine. PD: 0.51; HC: 0.71; p<0.001 both), although to a lesser
SOC associated total-costs are 60,312.19 and 1.75 QALYs extent (no statistical significance). Such a modulation,
(4.04 LYs). Apomorphine dominates SOC. measured as linear correlation slope, as well as arm ROM
Conclusion: Apomorphine is a cost-effective alternative, reduction at preferred gait speed, was not predicted by
reducing OFF-time and improving quality-of-life, and is contralateral striatal dopaminergic loss and clinically tested
associated with a cost-advantage. rigidity or bradykinesia.
Conclusion: Rather than striatal dopaminergic tone per se,
mesencephalic centres (i.e. PPN and reticular system) might
be involved in the modulation of upper limb locomotor
synergies.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


288 Posters, Sunday 9 September

P1531 P1532
Association between irritable bowel Longstanding idiopathic dystonia
syndrome and restless legs syndrome: preceding Parkinsons disease
a comparative study with control group R. Modreanu, Y. Compta, M.J. Marti
R. Borji1, S.-M. Fereshtehnejad2, S. Taba Taba Vakili3, Parkinsons Disease & Movement Disorders Unit, Hospital
N. Ebrahimi Daryani1, H. Ajdarkosh1 Clnic, Barcelona, Spain
1Tehran University of Medical Sciences, Tehran, Iran, Introduction: There are few descriptions of idiopathic
2Neurobiology, Care Sciences & Society (NVS), Karolinska dystonia preceding in years or even decades the onset of
Institutet, Stockholm, Sweden, 3Shaheed Beheshti University idiopathic Parkinsons disease (PD). These reports pointed
of Medical Sciences, Tehran, Iran to a possible common etiologic mechanism.
Background: As a common gastrointestinal (GI) disorder, Patients and methods: We reviewed the medical records of
irritable bowel syndrome (IBS) has been reported to be patients with initial focal dystonia who had been followed
associated with some psychological and neurological in our clinic and further developed PD. Data about
factors. This study aimed to evaluate the prevalence rate of demographic, clinical and genetic features, and response to
restless legs syndrome (RLS) in a sample of IBS patients treatment of dystonia and parkinsonism were collected
and to compare this prevalence with that of matched healthy retrospectively.
controls. Results: There were 7 patients (5 men and 2 women), with
Methods: This prospective comparative study was a mean age of dystonia onset of 47 (35 to 52) years and a
conducted in Tehran, Iran during 2010 and 2011. Based on mean age of PD onset of 60 (55 to 63) years. The time
the Rome III criteria, a total number of 225 definite IBS between dystonia and parkinsonism ranged from 3 to 28
patients and 262 age- and sex-matched healthy controls years (median 15.3). 2 patients presented with writers
were recruited in the final assessment to compare the cramp, 3 with blepharospasm, 1 with cervical and 1 with
prevalence rate of RLS between the two groups. segmental (cranio-cervical) dystonia. 5 patients had a mixed
Results: RLS was significantly more frequent in IBS group parkinsonism (with resting tremor) and 2 a rigid-akinetic
(25.3% vs. 6.5%, p<0.001) which leads to an odds ratio type. Genetic tests for LRKK2 and parkin mutations were
(OR) of 4.89 (95%CI: 2.75-8.70). IBS patients with performed in 5 patients and were negative. 5 patients
comorbid RLS suffered significantly more from stomach received anticholinergics for dystonia without improvement.
pain [96.5% vs. 86.3%, OR=4.36 (95%CI: 1-19.12)], All received levodopa and 5 of them also dopaminergic
nausea [40.4% vs. 21.4%, OR=2.48 (95%CI: 1.3-4.73)] and agonists with good response of parkinsonism, but no
vomiting [10.5% vs. 2.4%, OR=4.82 (95%CI: 1.31-17.76)]. changes in dystonia.
Conclusion: Having enrolled a considerable number of IBS Conclusions: The co-existence of dystonia with
patients and healthy controls, our study showed a parkinsonism suggests a possible common patho-
significantly higher prevalence of RLS in IBS patients. physiological mechanism. The previous descriptions of
Surprisingly, a higher prevalence rate of RLS was also dystonia preceding parkinsonism in PD antedated the
accompanied by a more severe discomfort and stomach discovery of monogenetic causes of parkinsonism which
pain in IBS patients. It seems that screening patients with can in turn present with dystonia, but in our short series
IBS for RLS may lead to greater identification of RLS and such mutations do not appear to underlie this uncommon
improved treatment of both conditions. association.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 289

P1533 P1534
Unmyelinated axons are more vulnerable Eye movement abnormalities in essential
to degeneration than myelinated axons of tremor and other neurodegenerative
the cardiac nerve in Parkinsons disease diseases (Parkinsons disease, spino-
S. Orimo1, T. Uchihara2, Y. Itoh3, A. Kakita4, cerebellar ataxia, Huntingtons disease)
K. Wakabayashi5, H. Takahashi4 M. Wojcik-Pedziwiatr1, M. Rudzinska1, K. Zajdel2,
1Kanto Central Hospital, 2Tokyo Metropolitan Institute of T. Tomaszewski1, A. Szczudlik1
Medical Science, 3Yokufukai Geriatric Hospital, Tokyo, Departament of Neurology, 2Departament of
1
4University of Niigata, Niigata, 5Hirosaki University
Otolaryngology, Jagiellonian University Medical College,
Graduate School of Medicine, Hirosaki, Japan Krakow, Poland
Aims: The aim of this study is to determine whether there Introduction: Eye movement abnormalities are a common
is a difference in the degenerative process between sign of many neurodegenerative diseases like Parkinsons
unmyelinated and myelinated axons of the cardiac nerve in disease (PD), spino-cerebellar ataxia (SCA) or Huntingtons
Parkinsons disease (PD). disease (HD). Even though essential tremor (ET) is the most
Background: We recently reported cardiac sympathetic prevalent movement disorder, the spectrum of eye
denervation in PD and dementia with Lewy bodies, which movement abnormalities for this disease has not been
accounts for the reduced cardiac meta-iodobenzylguanidine entirely explored yet.
(MIBG) uptake on MIBG myocardial scintigraphy in these Aim: To evaluate eye movement abnormalities in ET
disorders. Furthermore, we demonstrated accumulation of patients in comparison with controls matched according to
[alpha]-synuclein aggregates of the cardiac sympathetic age and gender and patients with other neurodegenerative
nerve in PD and a possible relationship between diseases like PD, SCA and HD and to assess the relations
degeneration of the cardiac sympathetic nerve and [alpha]- between eye movement abnormalities and other signs as
synuclein aggregates. well as course of ET.
Methods: We immunohistochemically examined cardiac Material and methods: 50 ET and 50 PD matched
tissues from 4 pathologically verified PD patients, 9 patients according to age and gender, 42 SCA, 50 HD patients as
with incidental Lewy body disease (ILBD) and 5 control well as 42 healthy controls were included in the study.
subjects, using antibodies against neurofilament, myelin Saccades were recorded using portable Saccadometer from
basic protein (MBP) and -synuclein. First, we counted the Ober Consulting company. EOG (Hortman) was used for
number of neurofilament-immunoreactive axons not recording of smooth pursuit, OKN and fixation.
surrounded by MBP (unmyelinated axons) and those Results: The study showed that eye movement abnormalities
surrounded by MBP (myelinated axons). Next, we counted are considerably more common in ET patients than in
the number of unmyelinated and myelinated axons with healthy subjects and their frequency in ET is similar to other
-synuclein aggregates. neurodegenerative diseases like PD, SCA and HD. The
Results: (i) The percentage of unmyelinated axons in PD most typical eye movement disturbances in ET were:
(77.59.14%) was significantly lower compared to that in reflexive saccades dysmetria, smooth pursuit and OKN
control subjects (92.22.40%). (ii) The ratio of unmyelinated impairment. In ET patients with concomitant cerebellar
axons with -synuclein aggregates to total axons with signs volitional saccade latency prolongation was
-synuclein aggregates in ILBD ranged from 94.4 to 100 additionally detected. The frequency of reflexive saccades
(98.2 2.18%). Among axons with -synuclein aggregates, dysmetria in ET increases with disease progression.
unmyelinated axons were the overwhelming majority, Conclusion: Eye movement abnormalities are a common
comprising 98.2%. sign of ET like other neurodegenerative diseases and their
Conclusion: These findings suggest that in PD unmyelinated frequency increases with disease progression.
axons are more vulnerable to degeneration than myelinated
axons of the cardiac nerve, because -synuclein aggregates
accumulate much more abundantly in unmyelinated axons.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


290 Posters, Sunday 9 September

P1535 P1536
White matter damage in Parkinsons Clinicopathological correlation of
disease patients with glucocerebrosidase progressive supranuclear palsy
gene mutations: a study using diffusion presenting with corticobasal syndrome
tensor imaging H. Ling1, R. de Silva1, L. Massey1, N. Bajaj2, J. Lowe2,
F. Agosta1, K. Davidovic1, L. Sarro2, N. Kresojevi3, J. Holton3, A. Lees1, T. Revesz3
1Reta Lila Institute of Neurological Studies, Institute of
M. Svetel3, I. Stankovi3, G. Comi4, V.S. Kostic5,
M. Filippi2 Neurology, University College London, 2University of
1Neuroimaging Research Unit, 2Neuroimaging Research Unit Nottingham, 3Queen Square Brain Bank for Neurological
and Department of Neurology, Vita-Salute San Raffaele Studies, Institute of Neurology, University College London,
University and San Raffaele Scientific Institute, Milan, Italy, UK
3Clinic of Neurology, Faculty of Medicine, University of Aim: We aimed to characterise the clinical and pathological
Belgrade, Serbia, 4Department of Neurology, Vita-Salute San features of 10 progressive supranuclear palsy (PSP) cases
Raffaele University and San Raffaele Scientific Institute, with a clinical presentation of corticobasal syndrome (PSP-
Milan, Italy, 5Clinic of Neurology, Faculty of Medicine, CBS) and to compare these with 10 PSP cases with a
University of Belgrade, Serbia classical presentation of Richardsons syndrome (PSP-RS).
Objective: To investigate brain white matter (WM) damage Background: The clinical presentation of CBS has been
in patients with Parkinsons disease (PD) carrying associated with PSP pathology and is clinically characterized
glucocerebrosidase (GBA) gene mutations. by asymmetric limb dystonia, rigidity, apraxia, the alien
Methods: Among 360 PD patients screened for mutations limb phenomenon and cortical sensory loss.
of the GBA gene, 19 (5.3%) heterozygous mutation carriers Methods: Morphometry with a stereological approach was
were identified. 15 (mean age 64 years, mean age at onset used for tau load quantitation.
57 years, median Hoehn and Yahr [HY] stage score 3.0), Results: Only 3.9% of PSP cases in the Queen Square Brain
were found to be heterozygous for N370S (6 cases), D409H Bank presented with a CBS. All PSP-CBS cases had marked
(6 cases), D380V (1 case), E388K (1 case) and L444P asymmetrical features with a clumsy useless limb, in
(1 case) GBA mutations, were enrolled in this study. 20 PD association with apraxia, hand dystonia, myoclonus and the
patients without GBA mutations (mean age 63 years, mean alien limb phenomenon, cortical sensory loss and delayed
age at onset 56 years, median HY stage score 3.0) and 16 initiation of horizontal saccades. Neuropathologically, both
healthy controls (mean age 64 years) were also studied. PSP groups had similar total tau load (p=0.18). However,
Diffusion tensor imaging (DTI) scans were obtained from analysis of the regional tau load revealed a shift of tau
all subjects. Tract-based spatial statistics were used to pathology from the basal ganglia (p=0.003) towards the
perform a brain voxel-wise analysis of mean diffusivity cortical regions (p<0.001) in PSP-CBS when compared to
(MD) and fractional anisotropy (FA). PSP-RS.
Results: Compared to controls, GBA mutation PD carriers In PSP-CBS, the presence of delayed initiation of horizontal
showed an increased MD of the genu of the corpus callosum saccades was associated with a greater parietal tau load
and a decreased FA of the corpus callosum, cingulum, (r=0.6; p<0.001).
external capsule, anterior thalamic radiations, bilaterally, Conclusions: We conclude that PSP-CBS is a cortical
and right superior longitudinal fasciculus (p<0.05, family- predominant subtype of PSP characterized by a shift of tau
wise error corrected). PD patients without GBA mutations burden from the basal ganglia towards the cortices. Early
did not show significant DT MRI abnormalities when falls and vertical supranuclear gaze palsy, when present,
compared with healthy controls. might be helpful in predicting PSP pathology in patients
Conclusions: PD patients carrying GBA mutations show presenting with CBS.
WM abnormalities involving the interhemispheric, limbic
and associations tracts. Future research will clarify whether
WM damage in these patients may have an impact on the
clinical phenotype, in particular on the development of
cognitive impairment.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 291

P1537 P1538
Randomized, double-blind, double- Recognition of adult-onset dystonia over
dummy study of levodopa-carbidopa time (1970 to 2007): data from a
intestinal gel in patients with advanced multicenter Italian series
Parkinsons disease: efficacy analyses by A. Macerollo1, G. Abbruzzese2, A.R. Bentivoglio3,
subgroups R. Liguori4, L. Santoro5, A. Berardelli6, G. Defazio1
1Dept. of Neurosciences and Sense Organs, University of
A. Antonini1,2, K. Chatamra3, W.Z. Robieson3,
Bari, 2Department of Neuroscience, University of Genoa,
Y. Pritchett3, K.L. Widnell3, J. Benesh3, R.A. Lenz3 3Department of Neurosciences, Catholic University of Rome,
1 IRCCS San Camillo, Venice, 2University of Padua, Italy, 4Department of Neurological Sciences, University of
3 Abbott, Abbott Park, IL, USA
Bologna, 5Department of Neurological Sciences, University
Introduction: Fluctuating blood levels of levodopa are Federico II of Naples, 6Department of Neurology and
associated with motor complications in Parkinsons disease Psichiatry, Sapienza University of Rome, Italy
(PD). Levodopa-carbidopa intestinal gel (LCIG) is delivered
Introduction: A dataset of 470 Italian patients, recruited
continuously via an intrajejunal percutaneous gastrostomy
between 2007 and 2008 at 7 Italian movement disorder
tube.
centres, was checked in order to analyse the time elapsing
Methods: A double-blind, double-dummy trial evaluated
between dystonia onset and diagnosis taking into account
LCIG compared with oral levodopa-carbidopa immediate-
sex, education, place of birth and residence, year of birth,
release (IR) therapy in patients with advanced PD and
and type of dystonia.
motor fluctuations. Levodopa-responsive patients received
Methods: The dataset included 318 women and 155 men
LCIG infusion+placebo capsules or encapsulated levodopa-
with adult-onset dystonia aged 6214 years. Mean age at
carbidopa IR tablets+placebo gel infusion for 12 weeks.
dystonia onset was 5114 years. Dystonia at presentation
The primary endpoint was the change from baseline to week
was blepharospasm (BSP, n=235), cervical dystonia (CD,
12 in Off time normalized to 16 waking hours. On time
n=185) and hand dystonia (FHD, n=50) .
without troublesome dyskinesia was a key secondary
Results: The time elapsing from dystonia onset to diagnosis
outcome. Efficacy was analyzed by subgroups including
was 6 years on average. The diagnostic delay was less
gender, age (<65 or 65 years), disease duration (<10 or
marked in the patients presenting with blepharospasm than
10 years), and number of PD medications at baseline (2
in the other groups (57 vs. 88 years, p<0.05). The time
or 3).
elapsing from dystonia onset to diagnosis significantly
Results: 71 patients were randomized (n=37 LCIG; n=34
decreased by increasing the year of diagnosis, but the
IR), and 66 (93%) completed the trial. LCIG significantly
percentage of patients who had <1 year elapsing between
improved Off time (LS mean difference = -1.91hr;
dystonia onset and diagnosis did not exceed 50% in the
p=0.0015) and On time without troublesome dyskinesia
most recent time bin (> year 2000) (all patients, 47%;
(LS mean difference =1.86hr, p=0.0059) compared with IR.
cranial dystonia, 50%; extra-cranial dystonia, 43%). It was
Subgroup analyses of the change from baseline in Off
chosen one year because this is the average diagnostic
time revealed no statistically significant interaction of
delay for Parkinsons disease in western countries.
treatment group by gender (p=0.589), disease duration
Confirmation that the year of diagnosis is a significant
(p=0.135), age (p=0.935), or number of PD medications at
predictor of the percentage of patients who had <1 year
baseline (p=0.237).
elapsing between dystonia onset and diagnosis was obtained
Conclusions: These results demonstrate the robust and
by binomial regression models.
consistent efficacy of LCIG, compared with optimized oral
Conclusions: Outside movement disorder centres the
levodopa-carbidopa therapy, irrespective of differences in
recognition of dystonia in Italy remains poor even in the
gender, age, PD duration, or number of PD medications at
years 2000. We suspect that a similar situation may be
baseline.
present in other western countries.
Support: Abbott

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


292 Posters, Sunday 9 September

P1539 P1540
Nutritional status as a predictor of the Maintenance of constant steady state
mortality in patients with Parkinsons therapeutic plasma concentrations of
disease levodopa following its continuous
R. Motoyama1,2, T. Oeda1,2, Y. Hari3, R. Hayashi1,2, subcutaneous administration with
A. Umemura1,2, S. Tomita1,2, M. Kohsaka1,2, carbidopa
K. Yamamoto1,2, T. Konishi2, H. Sawada1,2 O. Yacoby-Zeevi1, S. Oren1, P.A. LeWitt2
1Clinical Research Center, 2Department of Neurology, 1Neuroderm, Ltd., Ness-Ziona, Israel, 2Neurology, Wayne
3Department of Nutritional Management, Utano National
State University School of Medicine and Henry Ford
Hospital, Kyoto, Japan Hospital, West Bloomfield, MI, USA
Purpose: It has been reported that hypoalbuminemia Objective: To determine the pharmacokinetic (PK) profile
increases the mortality rates in cancer or surgical operations. of levodopa (LD) following continuous subcutaneous (SC)
To evaluate the association between the nutritional status administration of carbidopa (CD) / LD.
and mortality in patients with Parkinsons disease (PD). Background: In PD, wearing-off between doses and
Methods: The cohort of 501 consecutive PD patients dyskinesias are often related to variable circulating
enrolled from July 2004 to March 2010 was followed until concentration of LD occurring with oral administration.
June 15th 2010. The concentrations of serum albumin, urea Unfortunately, with available oral LD preparations, most
nitrogen, and creatinine, the lymphocyte counts and the patients with treatment fluctuations do not experience much
body mass index (BMI) were regarded as nutritional status, improvement and the option of administering LD
and in addition, age, sex, disease duration, Hoehn-Yahr intravenously or intraduodenally (Duodopa) is costly, and
stages (HY), and dementia (yes/no according to DSM-IV) ) invasive. Neuroderm proposes a practical alternative for
were obtained at the study entry. The association between maintaining more constant plasma concentrations of LD.
the nutrition status and mortality was analyzed using Cox Methods: A proprietary LD/CD solution (designated
proportional hazard models and the relative risk of death ND0612), was continuously administered SC in pigs with
was estimated as hazard ratios (HR). PK measurements of LD and CD.
Results: In the study period 22 patients died. Serum Results: Constant, dose-dependent steady-state plasma
albumin concentration was negatively associated with the concentrations of LD were achieved during continuous SC
mortality in the Cox model adjusted for age, sex, disease administration of ND0612 for both LD and CD. By using
duration, HY and dementia (yes/no) (p=0.014). The HR was only 2-4ml/day of SC ND0612, constant LD plasma
0.38 (per 0.5g/dl, 95% CI 0.18-0.82). Patients with albumin concentrations could be maintained at values that are
<3.8g/dl had a shorter lifetime than those with albumin generally effective at maintaining anti-Parkinsonian control
3.8g/dl (Log rank test p=0.010). in humans. No safety or tolerability issues were detected.
Conclusion: Serum albumin concentrations were a Conclusions: The limitation of oral LD products is the
significant prognostic factor for the mortality in patients marked variability in plasma concentrations of the drug.
with PD. Using SC delivery of an LD/CD solution, we demonstrated
a promising option for achieving consistency in plasma LD
concentrations. A clinical application of this approach
should achieve improved control of motor fluctuations in
PD. Furthermore, frequent daily oral dosing with CD/LD
can be avoided. This approach is currently under
investigation in a randomized clinical trial (double-blind) of
ND0612, evaluating the plasma LD PK profile as well as
safety and tolerability.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 293

P1541 P1542
Rapid eye movement (REM) sleep Motor learning in primary cervical
behaviour disorder (RBD): a risk factor for dystonia
cognitive impairment in Parkinsons P. Katschnig1,2, P. Schwingenschuh1,2, M. Davare1,
disease (PD)? A. Sadnicka1, R. Schmidt2, J.C. Rothwell1, K.P. Bhatia1,
R. Di Giacopo, D. Quaranta, C. Piccininni, C. Marra, M.J. Edwards1
1Sobell Department of Motor Neuroscience and Movement
A.R. Bentivoglio
Neuroscience, Catholic University of Rome, Italy Disorders, UCL Institute of Neurology, London, UK,
2Department of Neurology, Medical University of Graz,
Background: RBD is strongly associated with Austria
synucleinopathies, often heralding and later accompanying
Introduction: Motor sequence learning and motor
motor symptoms of PD and Lewy Body Dementia (DLB).
adaptation rely on overlapping circuits predominantly
The neuropsychological assessment in 24 idiopathic RBD
involving basal ganglia and cerebellum. Given the
patients revealed a cognitive impairment similar to those
importance of these neuro-anatomical structures to the
observed in patients with PD and DLB; it suggests a
pathophysiology of primary dystonia, and the previous
common pathophysiological mechanism underlying these
finding of abnormal motor sequence learning in DYT1 gene
conditions, according to Braaks hypothesis. However, this
carriers, we explored motor sequence learning and motor
theory in not confirmed in all PD cases. Our objective is to
adaptation in patients with primary cervical dystonia.
assess the relationship between the presence of RBD and
Methods: 12 patients with cervical dystonia and 11 healthy
the cognitive profile of non-demented patients with PD.
age-matched controls were included. Subjects used a
Methods: 47 patients with PD without dementia were
joystick to move a cursor from a central starting point to
evaluated by a standard neuropsychological battery
radial targets as fast and accurately as possible. Using this
assessing intelligence, episodic verbal and spatial memory,
device, we recorded baseline motor performance, motor
recall capacity, fluency, executive and visuospatial abilities.
sequence learning and a visuomotor adaptation task.
25 patients fulfilled polysomnographic criteria for RBD.
Results: Baseline motor performance with random target
Bilateral t-tests were performed to compare differences
presentation was normal except for higher peak velocity in
between RBD-PD group and non-RBD-PD group.
patients with cervical dystonia. Patients and controls had
Results: No significant differences between groups were
similar levels of motor sequence learning and motor
found regarding age, schooling, depression assessment,
adaptation.
Hoehn and Year stage (I-II), UPDRS III score, disease
Conclusion: Our patients had significantly higher peak
duration, levodopa equivalent daily doses (LEDD). Groups
velocity compared to controls, with similar movement
differed significantly for execution time in interference
times, implying a different performance strategy. The
condition of the Stroop colour word test (p=0.002).
preservation of motor sequence learning in cervical dystonia
Conclusions: Our data confirm a previous study in that
patients contrasts with the previously observed deficit seen
RBD-PD patients showed a lower cognitive performance
in patients with DYT1 gene mutations, supporting the
compared to non-RBD-PD. The cognitive involvement in
hypothesis of differing pathophysiology in different forms
executive functions is very early, because our patients
of primary dystonia. Normal motor adaptation may signify
presented a mild motor phenotype (H&Y:1,87) compared to
that previously documented cerebellar abnormalities in
literature. Thus RBD may indicate a more pervasive
cervical dystonia might simply reflect unimportant
neurodegenerative process in PD and can increase the risk
contamination of a structure directly connected to the
of cognitive impairment in patients.
basal ganglia, or might represent a compensatory response
to the primary pathophysiology within the basal ganglia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


294 Posters, Sunday 9 September

P1543 P1544
PRRT2 mutations are a major cause of Peripheral selective denervation for
paroxysmal kinesigenic dyskinesia in the torticollis - long-term follow-up
European population T. Bergenheim1, M. Hariz2, R. Libelius3, E. Nordh3,
A. Meneret, D. Grabli, C. Depienne, C. Gaudebout, E. Larsson1
F. Picard, A. Drr, I. Lagroua, D. Bouteiller, C. Mignot,
1Neurosurgery, Inst of Clinical Neuroscience, Ume, Sweden,
D. Doummar, M. Anheim, C. Tranchant, P. Burbaud,
2Neurosurgery, UCL Institute of Neurology, National
C.P. Jedynak, D. Gras, D. Steschenko, D. Devos, T. Hospital for Neurology and Neurosurgery, London, UK,
3Neurophysiology, Inst of Clinical Neuroscience, Ume,
Billette de Villemeur, M. Vidailhet, A. Brice, E. Roze
INSERM, UMRS 975 and CNRS 7225 - CRICM, Paris, Sweden
France Introduction: Spasmodic torticollis is a focal cervical
Introduction: Paroxysmal kinesigenic dyskinesia (PKD) is dystonia that most often responds to botulinum toxin.
a rare disorder characterized by recurrent attacks of However, not all patients respond to this treatment and
hyperkinetic movements. Mutations in the PRRT2 gene some develop secondary resistance. In those cases, there are
were recently identified in PKD patients. We studied the two surgical options, selective peripheral denervation
prevalence of PRRT2 mutations and characteristics of the (Bertrand procedure) and pallidal Deep Brain Stimulation
patients in a European PKD population. (DBS). We report here on the Ume experience of selective
Methods: 34 consecutive PKD index cases (20 sporadic denervation.
cases and 14 cases with family history) were recruited Methods: 63 patients were operated. 4 patients were later
through the 1996-2011 database of our DNA bank. Each re-operated due to recurrence or change in the pattern of the
patient had a standardized neurological assessment. dystonia. The patients were assessed with the Tsui torticollis
Molecular analysis of the PRRT2 coding sequence was scale, VAS for pain, and the Fugl-Meyer Life Satisfaction
performed in all index cases and their family members scale before surgery, at 6 months, and long-term follow-up
when available. In addition, 3 microsatellite markers were (mean 42 months).
genotyped in the patients and their relatives. Results: 6 months follow-up was available for 57 patients
Results: Mutations introducing premature termination and long-term for 35. Mean score of Tsui scale was
codons were identified in 22/34 patients (65%) including preoperatively 10.2, early post-op 4.1 (p<0.001), 6 months
13/14 families and 9/20 sporadic cases. The previously post-op 4.8 (p<0.001), and at long-term follow-up 5.3
described c.649dupC/pArg217ProfsX8 and c.629dupC/ (p<0.001). Mean VAS for pain was pre-operatively 6.2, at 6
pAla211SerfsX14 were present in 19 and 1 cases months 4.3 (p<0.001), and at long-term follow-up 4.1
respectively; we also report 2 novel mutations, each in one (p<0.01). Life satisfaction total score improved moderately
case: c.562C>T/pGln188X and c.649C>T/pArg217X. from a mean of 43.3 to 46.6 at 6 months (p<0.05), and to
Segregation analysis of the mutations in the parents of 6 51.4 at long-term follow-up (p<0.05). No severe or long-
sporadic cases revealed 3 de novo mutations and incomplete lasting side effects were noted.
penetrance in a transmitting parent in the 3 other cases. No Conclusions: From our experience it can be concluded that
founder effect was found for the c.649dupC mutation. The selective peripheral denervation is a satisfactory
mutated group was characterized by a younger age of onset neurosurgical option for treatment of otherwise refractory
(9 years) compared to the negative patients (15 years; spasmodic torticollis when the muscles involved are
p<0.01). surgically accessible for denervation. In patients with a
Conclusion: Mutations in PRRT2 are a major cause of PKD widespread muscular involvement, pallidal DBS may be
in familial and sporadic cases in the European population. preferable.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 295

P1545 P1546
Familial congenital mirror movements: Copper metabolism and diagnostic
phenotype and neurophysiological problems in clinically asymptomatic
findings in a new family with incomplete Wilsons disease patients
penetrance K. Dzieyc1, A. Czonkowska1,2
12nd Department of Neurology, Institute of Psychiatry and
M. Bologna1, A. Fasano1, E. Iezzi1, F. Di Biasio1,
L. Rocchi2, M. Srour3, A. Levert3, G. Rouleau3, Neurology, 2Department of Clinical and Experimental
A. Berardelli1,2 Pharmacology, Medical University of Warsaw, Poland
1Neuromed Institute, Pozzilli, 2Sapienza University of Rome, Background: Wilsons disease (WD) is a rare autosomal
Italy, 3Hpital Notre Dame, Montral, QC, Canada recessive inherited disorder of copper metabolism.
Introduction: Mirror movements (MMs) are involuntary Diagnosis of WD is based on combination of clinical,
movements of one side of the body that accompany biochemical, genetic testing and may be problematic. The
intentional movements on the opposite side. Few families aim of the study was the evaluation of initial copper
with members presenting MMs without other neurological metabolism parameters in asymptomatic cases diagnosed
disorders have been reported and mutations in DCC gene upon family history of WD.
were found to underline this condition. In the present study Methods and results: We have assessed 85 WD patients
we aimed to report the clinical and neurophysiological diagnosed by family screening in our center between 1964
features of a novel family with congenital MMs. and 2010. All of them had no history of hepatic or
Methods: 13 members of a 4-generation family with neurological symptoms and were without any symptoms at
individuals affected with congenital MMs were clinically the time of diagnosis. WD diagnosis was based on copper
evaluated using the Woods & Teuber scale. Subjects were metabolism, DNA analysis and in uncertain cases using
blood sampled for the screening of the DCC gene mutations. radiocopper study. The initial copper metabolism test results
5 subjects underwent neurophysiological evaluation of asymptomatic patients were compared to their
including recordings of voluntary and involuntary EMG symptomatic siblings (n=60). Serum ceruloplasmin levels
activity, motor evoked potentials (MEPs) and were decreased in asymptomatic patients but were
interhemispheric inhibition (IHI) studied with transcranial significantly higher than in symptomatic siblings
magnetic stimulation. The neurophysiological parameters (14.37.7mg/dl vs. 10.26.2mg/dl, respectively, p=0.001),
were recorded before and after delivering continuous theta in 11 (13%) cases were even in normal range. Urinary
burst stimulation (cTBS) on the dominant primary motor copper excretion was significantly lower in asymptomatic
cortex. patients (168181.5ug/24h vs. 388.2678.6ug/24h,
Results: Transmission of MMs was consistent with an p=0.003), and was normal in 11 (15%) patients. Total
autosomal dominant fashion with incomplete penetrance copper serum concentration levels were similar in both
and variable degree of expression. The screening of the groups.
DCC gene mutations was negative. Among the subjects Conclusion: Copper metabolism test results in clinically
who underwent neurophysiological evaluation, ipsilateral asymptomatic patients may cause diagnostic difficulties.
MEPs were detectable in the two most severe cases. IHI did Ceruloplasmin levels and urinary copper excretion may be
not correlate with MMs severity; cTBS tended to reduce the less significant for diagnosis comparing with symptomatic
EMG mirror activity and this was clinically relevant in one patients. In case of lack of genetic confirmation, further
of the two most affected case. diagnostics such as the radiocopper study may be necessary,
Conclusions: Neurophysiological features suggest that because missed diagnosis and delay of therapy may lead to
MMs in this newly reported family are mainly mediated development of symptoms.
through uncrossed cortico-spinal tracts and that cTBS might
be a potential therapeutical tool in congenital MMs.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


296 Posters, Sunday 9 September

P1547 P1548
The impact of micro-electrode recording Epidemiological (cross-sectional) study to
on selecting the most optimal trajectories evaluate and describe fatigue in patients
for electrode implantation with Parkinsons disease in Italy - The
N. Kovacs, E. Gasparics, E. Bosnyk, F. Nagy, J. Janszky, FORTE Study
I. Bals, G. Deli F. Stocchi1, G. Abbruzzese2, R. Ceravolo3, P. Cortelli4,
Department of Neurology, University of Pecs, Hungary M.F. De Pandis5, G. Fabbrini6, W. Liboni7, C. Pacchetti8,
Background: Micro-electrode recording (MER) is an G. Pezzoli9, M. Canesi9, C. Iannacone10, M. Zappia11,
electrophysiological tool for finding the most optimal FORTE Study Investigators
trajectory and level during implantation of chronic 1Institute of Neurology, IRCCS San Raffaele Pisana, Rome,
stimulation electrode. Although it might be associated with 2Department of Neurosciences, Ophthalmology,
a higher risk for intra-operative intracranial bleedings, most Rehabilitation and Genetics, University of Genoa,
DBS center routinely apply it. 3Department of Neuroscience, University of Pisa,
4Department of Neurological Sciences, Alma Mater
Objectives: To analyze the number of DBS surgeries in a
large prospective cohort where the planned trajectory was Studiorum, IRCCS, Institute of Neurological Sciences,
modified by the results of MER signals. University of Bologna, 5Parkinson Operative Unit, San
Methods: 184 DBS electrode implantations were evaluated. Raffaele Institute, Cassino, 6Department of Neurology and
We applied Leadpoint 5 system (Medtronic Inc, MN) to Psychiatry and IRCSS Neuromed, La Sapienza University
MER. Simultaneously 5 micro-electrodes were inserted of Rome, 7Complex Structure of Neurology, Gradenigo
(center, anterior, posterior, medial and lateral) 2mm and the Hospital, Turin, 8Parkinson Operative Unit, IRCCS
electrophysiological activity was recorded in the level of National Neurological Institute C. Mondino Foundation,
-10 and +5mm from the planned target level. The signals Pavia, 9Parkinson Institute, I.C.P., 10SPARC Consulting,
were evaluated offline and macro-electrode stimulation was Milan, 11Department G. F. Ingrassia, Neurosciences Area,
performed subsequently. University of Catania, Italy
Results: The central electrode at the level of the planned Introduction: Fatigue is a common non-motor symptom in
target demonstrated pathological activity during 134 patients with Parkinsons disease (PD) and one of the most
electrode placements (72.8%); however, the central disabling symptoms of this disease, as reported by patients,
trajectory was used for chronic stimulation only in 102 with serious impact on patients quality of life. It is generally
(55.4%) of instances. Anterior trajectory was chosen in 53 accepted that about 50% of PD patients will complain of
cases (28.8%). Chronic electrodes were placed in the lateral, fatigue and clinical observation revealed that its severity
medial and posterior positions less frequently (12, 15 and 6 remained mostly unchanged during progression of the
instances, respectively). disease. This study aims to assess, in a non-interventional
Conclusion: Although we reached the planned target in the epidemiological setting, the incidence and severity of
vast majority of cases, we selected the central trajectory for fatigue in PD patients and its correlation with PD severity.
electrode implantation only in nearly half of the cases based Methods: This is an epidemiological, cross-sectional,
on signal quality and intra-operative stimulation. multicentre study, in patients diagnosed with PD according
to the UKPDS brain bank diagnostic criteria. The primary
objectives included the evaluation of the incidence and
severity of fatigue in PD patients measured by Parkinson
Fatigue Scale (PFS-16) and assessment of its predisposing
factors (age, gender, marital status, disease duration,
presence of depression, presence of sleep disorders, PD
severity, UPDRS total score).
Results: 402 patients (mean age: 66.9 years; mean disease
duration: 7.5 years) were enrolled in 27 PD centres in Italy.
33.8% of them showed fatigue (PFS-16 score 3.3 points).
Logistic regression analysis showed that UPDRS total
score, female gender, presence of depression and presence
of sleep disorders significantly increase the odds of
occurrence of fatigue in patients with PD.
Conclusion: FORTE is the largest study ever conducted in
Italy to assess fatigue in PD patients. The large amount of
data collected will allow us to investigate in depth the
fatigue in these patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 297

P1549 P1550
Usefulness of brain perfusion SPECT for Early-onset dystonia with brain
differentiation between idiopathic manganese accumulation due to
Parkinsons disease and multiple system SLC30A10 mutations: a new treatable
atrophy disorder
K.-S. Lee1, I.-U. Song1, J.-W. Park1, Y.-A. Chung2 M. Stamelou1, K. Tuschl2, P. Mills2, K. Chong3,
1Department of Neurology, 2Department of Nuclear A. Burroughs4, P. Clayton2, K. Bhatia1
Medicine, The Catholic University of Korea, Seoul, Republic 1UCL Institute of Neurology, 2UCL Institute of Child Health,
of Korea 3Department of Radiology, 4The Wellington Hospital,
Objectives: Statistical parametric mapping was performed London, UK
to investigate differences in regional cerebral blood flow Background: Recently, the first gene causing early-onset
(rCBF) between patients with idiopathic Parkinsons disease generalised dystonia with brain manganese accumulation
(IPD), patients with multiple system atrophy (MSA), and has been identified. Mutations in the SLC30A10 gene,
healthy volunteers. encoding a manganese transporter, cause a syndrome of
Methods: Tc-99m HMPAO SPECT was performed on 23 hepatic cirrhosis, dystonia, polycythemia and hyper
IPD patients (10 men, 13 women; aged 57-80 y), 9 MSA manganesemia.
patients (4 men, 5 women; aged 60-83 y), and 12 age- Methods: We present ten-year longitudinal clinical features,
matched healthy volunteers (5 men, 7 women; aged 56-81y). magnetic resonance imaging data, and treatment response
Results: Significant hypoperfusion was observed in IPD to chelation therapy of the originally described patient with
compared with healthy subjects in a symmetric subcortical- proven SLC30A10 mutation.
cortical network including the basal ganglia, thalami, Results: The patient presented with early-onset generalized
prefrontal and lateral frontal cortex, and parietal cortex dystonia and jaundice accompanied by elevated whole
(voxel size: 50, corrected p<0.001). For MSA, only blood manganese levels. T1 sequences in magnetic
symmetric hypoperfusion was seen in both prefrontal resonance imaging showed manganese deposition in the
cortices with respect to healthy subjects and to IPD (voxel basal ganglia and cerebellum. Treatment with intravenous
size: 50, corrected p<0.001). disodium calcium edetate, led to a slow but continuous
Conclusions: Tc-99m HMPAO perfusion SPECT shows clinical improvement and reduction of the manganese
detailed differences between IPD and MSA, which may be deposition in brain imaging.
of use in the differentiation of both disease entities. Conclusions: We wish to highlight this rare disorder which,
together with Wilsons disease, is the only potentially
treatable inherited metal storage disorder to date, that
otherwise can be fatal due to complications of cirrhosis.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


298 Posters, Sunday 9 September

P1551 P1552
Digitalised spirography and clinical Movement disorders in Ethiopia
examination based decision support D.K. Worku
system for differentiating between Addis Ababa University, Addis Ababa, Ethiopia
tremors Introduction: In a country of 80-million plus people, there
D. Georgiev1, V. Groznik2, A. Sadikov2, M. Moina2, is a shortage of physicians - only 15 neurologists serve the
M. Guid2, V. Kragelj3, I. Bratko2, S. Ribari3, Z. Pirtoek1 entire country and most are concentrated in the capital city
1Laboratory for Movement and Gait Disorders, Department - making it difficult for all who need care to see a
of Neurology, University Medical Centre Ljubljana, neurologist.
2Artifitial Intelligence Laboratory, Faculty of Computer and Methods: A review of journals was done on papers:
Information Science, University of Ljubljana, 3Institute of movement disorders in Ethiopia available until the end of
Pathophysiology, Faculty of Medicine, University of June, 2011 at the main Library of Addis Ababa University,
Ljubljana, Slovenia Department of Neurology.
Results: In one retrospective study done over a 1-year
Objective: To analyse the usefulness of computer decision
period a total of 15.1% of the neurological patients were
support system (DSS) that combines digitalised spirography
seen for movement disorders. Of these, most were
and clinical data for differentiating between essential tremor
parkinsonism (47.7%), followed by ataxia (16.5%), dystonia
(ET) and Parkinsons disease (PD), including the mixed
(8.3%), essential tremor (8.3%), chorea (7.3%), and
type tremor (MT) (comorbidity).
miscellaneous (11.9%). Data on Parkinsons disease in sub-
Introduction: ET and PD are very often misdiagnosed
Saharan Africa is limited but it appears that the age-adjusted
when standard clinical examination is applied (37-50% and
prevalence may be a little lower than in other parts of the
24%, respectively, Quinn et al., 2011). DaTSCAN can be
world. The likely explanation for this is a lack of diagnosis,
used to more precisely differentiate between ET and PD, but
and therefore treatment, leading to early fatality. Different
it is costly and not widely available. In a previous work
studies indicated that movement disorders is one of the
(sample size 69 patients), we have estimated the accuracy
neurologic disorders associated with malnutrition which is
of the argument-based machine learning (AMBL) DSS for
rampant in the country. HIV/AIDS adds more to this
tremor differentiation as 81%.
problem and makes the diagnosis and the management even
Methods: 122 (median age 69 years) patients with ET, PD
more challenging. Diagnostic evaluations are limited, but
or MT were included in the study. DSS was built using
treatment is available, although expensive. In spite of the
ABML, which combines machine learning (ML) with
limitations, patients with movement disorders require and
expert knowledge based on the clinical as well as
seek care in Ethiopia in proportions comparable to
quantitative data from digitalised spirography.
developed nations.
Results: The estimated classification accuracy of the model
Conclusion: These findings underline the need for adequate
was 91%, which shows an improvement of 10% compared
training in movement disorders for physicians and
to the previously reported model. Measured specificities of
neurologists, and community education in Ethiopia.
the model were 0.97 for ET, 0.89 for PD, and 0.98 for MT;
measured sensitivities were 0.93 for ET, 0.97 for PD, and
0.70 for MT.
Conclusions: Digitalised spirography has a potential to be
used as a stand-alone screening method for tremor analysis.
We plan to further evaluate and validate the system by
enrolling a larger number of patients and compare it to
DaTSCAN, in order to improve the diagnostic precision of
the system.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 299

P1553 P1554
Obliquus capitis inferior muscle plays an Dopamine therapy in primary pain
important role in dystonic torticaput syndromes in patients with Parkinsons
A. Stenner, G. Reichel disease
Movement Disorders Department, Paracelsus Clinic, A. Alekseev, E. Podchufarova, M. Nodel
Zwickau, Germany First Moscow Medical University, Moscow, Russia
Introduction: Idiopathic cervical dystonia (CD) is the most Introduction: Many researchers discussed the role of
prevalent defined dystonia in adults. The response rate to dopamine in the functional activity of the anti-nociceptive
botulinum toxin in open and double-blind studies is usually system (ANS). Parkinsons disease (PD) is an example of
about 60-70%. Nevertheless, there are cases of primary common dopaminergic failure, accompanied by widespread
treatment failure or unsatisfactory treatment outcomes. The pain syndromes.
main reasons for this are most probably the selection of Aim: To investigate the relationship of primary pain (pain
muscles to be injected. In addition, in such cases the without peripheral sources) in patients with PD and activity
problem is probably not only that not all affected muscles of ANS using nociceptive-flexion reflex (NFR).
are treated, but that healthy muscles are also affected by the Patients and methods: 12 patients with PD and primary
application of botulinum toxin. pain (PD+PP), mean age 63.359.04, mean duration of PD
Methods: The results of the examinations of 94 patients 5.680.48, 15 patients with PD and low back pain
with primary CD treated in our clinic, were analysed. The (PD+LBP), mean age: 63.2310.11, mean duration of PD
cervical spine and the soft parts of the neck, including deep 5.424.8, and 20 patients with low back pain without PD
neck muscles, were examined by CT in 5mm slices. For (LBP), mean age: 62.099.27, were enrolled in the study.
comparison, the CT-images of 50 patients who did not Pain intensity by visual analogue scale (VAS), NFR
suffer from CD were analyzed. thresholds (NFRTh) were determined. Patients underwent
Results: Measurement of the maximum muscle diameters clinical examination before and after treatment of motor
by CT revealed one distinctive feature: in 73% of the sign by dopaminergic drugs. Patients had no significant
patients the only small muscle that was asymmetrical, in differences in the therapy received, the stage of PD or motor
addition to the large neck muscles, was the obliquus capitis symptoms PD.
inferior muscle. Dystonia of this muscle induced rotation to Results: Patients PD+PP had significantly (p<0.05) higher
the same side in the lower head joints. pain intensity (7.251.76 VAS) than PD+LBP (6.731.39
Conclusions: Obviously, the obliquus capitis inferior VAS), not significantly different from LBP (7.381.50
muscle plays an important role in the dystonic rotation of VAS). Patients PD+PP had significantly (p<0.05) more
the head. If treatment of the large neck muscles with Btx types and localizations of pain (2.751.48), than PD+LBP
does not show satisfactory results, this small muscle should (1.630.67) and LBP (1,120.38). NFRTh was significantly
be included in the therapy plan. (p<0.05) higher in PD+PP (19.256.21), and PD+LBP
(18.314.76), than in LBP (18.34.76). After dopaminergic
therapy all PD patients showed decreased intensity of pain.
Decreasing was more significant (p<0.05) in PD+PP
(5.11.72 VAS). NFRTh significantly (p<0.05) decreased
(14.374.78). Any correlations between pain reduction or
NFRTh increase and improvement of motor signs were not
noted.
Conclusion: Primary pain in PD may be an example of
dopaminergic-associated pain due to dopaminergic
dysfunction and incompetence of ANS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


300 Posters, Sunday 9 September

P1555 P1556
Surface electromyogram of neck extensor Xenologous transplantation of human
and flexor muscles in Parkinsons disease adipose derived mesenchymal stem cells
patients with antecollis and retrocollis in an animal model of Huntingtons
K. Kashihara, T. Imamura disease: behavioural and micro-
Neurology, Okayama Kyokuto Hospital, Okayama, Japan anatomical outcomes
Objective: Patients with Parkinsons disease (PD) may M.A. Edalatmanesh1, Z. Alizadeh2, F. Vafaee-Bagheri3,
present with abnormal neck posture, including antecollis E. Abbasi2, M. Hosseini3, H. Nikfarjam4
and retrocollis. In order to study the mechanism of such 1Department of Physiology, Science & Research Branch,
postural disorders of the neck, we evaluated the electrical Islamic Azad University, Shiraz, 2School of Medicine, Islamic
activity of the neck extensor and flexor muscles in PD Azad University, Mashhad Branch, 3Neuroscience Research
patients presenting with postural disorders. Center and Department of Physiology, School of Medicine,
Methods: We examined 15 PD patients without postural Mashhad University of Medical Sciences, 4Department of
disorder of the neck, 14 with antecollis, and 6 with Biology, Faculty of Science, Ferdowsi University of
retrocollis. Surface electromyogram (EMG) was recorded Mashhad, Iran
from the splenius capitis, trapezius, sternocleidomastoideus, Introduction: Human adipose derived mesenchymal stem
and deltoid muscles on both sides of the spine while the cells (hMSCs) have been proposed as alternative sources of
subjects were at rest in the sitting position and while they cells for transplantation into the brain in neurodegenerative
tried to lift their head in the same position. disorders. We tested the efficacy of hMSCs transplants to
Results: The EMG amplitude of the sternocleidomastoideus reduce behavioural and micro-anatomical deficits in a
muscles at each position was the highest. The amplitude at Quinolinic acid (QA) rat model of Huntingtons disease.
the spine position was at maximum in patients with Methods: After unilateral lesion in striatum was caused by
retrocollis, followed by those with antecollis, and then by QA, hMSCs, which were isolated and purified from
those without neck postural disorder. In PD patients with liposuction of healthy male donors, were transplanted into
antecollis, the amplitude increased in the sitting position the damaged striatum of rats. Animals were tested by motor
and showed a greater increase when patients tried to lift function tests at different times after cell transplantation.
their head. In patients with retrocollis, changes in position The volume of striatum, lateral ventricles and atrophy
from supine to sitting hardly produced increased muscle percentage of striatum and volume extension of lateral
discharges. ventricles were measured in all treated rats.
Conclusion: Antecollis in PD patients may be associated Results: MSCs survived 42 days without inducing a strong
with disproportionately increased muscle tonus of both inflammatory response from the striatum. Behavioural
flexor and extensor muscles, though antecollis of the amelioration was observed on tests of apomorphine induced
sternocleidomastoideus is the most prominent. In case of rotation, cylinder, beam balance and rotarod tasks for
patients with retrocollis, muscle tonus increased to the MSCs-treated rats. Relative to QA controls, the MSCs
maximum level. treated group was protected from QA-induced enlargement
of the lateral ventricles. Histological results showed
significant difference in amount of striatum atrophy
between QA controls and MSCs-treated rats.
Conclusions: These results confirm the potential of hMSCs
in treatment of behavioural and micro-anatomical defects in
Huntingtons disease. Taken together, these data demonstrate
that xenologous transplantation of hMSCs could be
considered as a good candidate for treatment of
neurodegenerative diseases, especially Huntingtons
disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 301

P1557 P1558
Neuromelanin activates dendritic cells: Brain MRI apparent diffusion co-efficient
implications for Parkinsons disease (ADC) in Parkinsons disease with
U. Oberlnder1, E. Gschmack1, K. Pletinckx1, A. Dhler1, dementia
N. Mller1, M.B. Lutz1, T. Arzberger2, P. Riederer3, A. Umemura, T. Oeda, M. Kohsaka, S. Tomita,
M. Gerlach4, E. Koutsilieri1, C. Scheller1 R. Hayashi, K. Yamamoto, H. Sawada
1Institute of Virology and Immunobiology, University of Clinical Research Center and Neurology, Utano National
Wrzburg, 2Institute of Neuropathology, University of Hospital, Kyoto, Japan
Munich, 3Clinical Neurochemistry (National Parkinson
Purpose: ADC reflects pathological changes in the brain.
Foundation Center of Excellence Research Laboratory),
To evaluate ADC in the brain regions and explore which
Clinic and Policlinic of Psychiatry, Psychosomatics and
brain regions are associated with dementia in Parkinsons
Psychotherapy, 4Laboratory of Clinical Neurobiology,
Department of Child and Adolescent Psychiatry, disease.
Psychosomatics and Psychotherapy, University of Wrzburg, Methods: In 249 patients with Parkinsons disease (157
Germany without dementia and 92 with dementia), ADC was obtained
in the hippocampus, amygdala, putamen, thalamus, caudate,
Introduction: Neuromelanin (NM) is a complex polymer the superior frontal, middle frontal, superior temporal, and
pigment that is present in the dopaminergic neurons of the inferior temporal gyri, the anterior cingulate, the posterior
substantia nigra (SN). It is these neurons that degenerate in cingulate, the primary visual area and the substantia nigra.
PD. In the past decade evidence for an autoimmune By a logistic regression model using a likelihood test it was
mechanism in PD pathogenesis has accumulated. NM determined, which brain regions were associated with
represents an attractive potential target for an autoimmune dementia.
pathomechanism, as the distribution of NM in the brain Results: The ADC in the amygdala was significantly
correlates with the pattern of neurodegeneration in PD. increased in demented patients compared to the non-
Here we investigated whether NM triggers maturation of demented after adjustment for age, sex and Hoehn-Yahr
dendritic cells (DCs), the major antigen-presenting cell type (p=0.005). The odds ratio was 2.1 (>0.1 x 10-3 mm2/s, 95%
involved in adaptive (auto-) immune response. Moreover, CI 1.25-3.56).
we studied whether NM-mediated DC-activation is Conclusion: It was supposed that dementia was related to
functional in order to trigger a T-cell response. degeneration in the amygdala in Parkinsons disease.
Methods: Murine DCs were treated for 48h with NM from
human SN and synthetic dopamine melanin (DAM) in a
concentration of 50g/ml. Uptake of NM was studied by
confocal microscopy. Characterization of activation/
maturation of DCs was performed by flow cytometry and
ELISA. T-cell activation was assessed with mixed
lymphocyte reaction (MLR).
Results: DCs phagocytosed NM, leading to DC maturation
(CD86high, MHC-IIhigh), activation (release of
proinflammatory cytokines TNF- and IL-6) and T-cell
proliferation in a mixed lymphocyte reaction. DAM was
also phagocytosed, but did not cause significant DC
activation.
Conclusions: The maturation and activation of DCs
following contact with NM represents the initial step in
triggering an adaptive immune response. If operative in
vivo, this could lead to an autoimmune response directed at
NM and subsequent degradation of dopaminergic neurons
in PD. (The data has been published in BMC Neurosci.,
doi:10.1186/1471-2202-12-116.)

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


302 Posters, Sunday 9 September

P1559 P1560
Prognostic value of nocturnal stridor in Pharmacokinetics, pharmacodynamics
multiple system atrophy and tolerability of opicapone, a novel
F. Mastrolilli1, P. Guaraldi2, G. Calandra-Buonaura2, COMT inhibitor, during the first
G. Barletta2, A. Cecere2, L. Sambati2, F. Provini2, administration to healthy male subjects
F. Vernieri1, P. Cortelli2 T. Nunes1, J.-F. Rocha1, R. Pinto1, R. Machado1,
1Policlinico Universitario Campus Bio-Medico, Roma,
2IRCCS, Istituto delle Scienze Neurologiche, University of
L. Wright1, A. Falco2, L. Almeida3, P. Soares-da-Silva1,4
1Research & Development, BIAL - Portela & C S.A.,
Bologna, Italy S. Mamede Coronado, 2Faculty of Pharmacy, University of
Introduction: Multiple system atrophy (MSA) is a Coimbra, 3Health Science Section, University of Aveiro,
neurodegenerative disease characterized by parkinsonian, 4Dept. Pharmacology & Therapeutics, Faculty of Medicine,

cerebellar and autonomic features. Nocturnal stridor is University of Porto, Portugal


frequent associating with poor prognosis. We conducted a Introduction: Opicapone was developed to fulfil the need
retrospective study to determine the survival of 111 patients for more potent, safer and longer acting catechol-O-
and examine the incidence and prognosis of stridor in methyltransferase (COMT) inhibitors (1). This study was
relation to standard therapy. aimed to assess the tolerability, pharmacodynamics, and
Methods: 111 patients were analyzed (68 MSA-P, 43 MSA- pharmacokinetics of single oral doses of opicapone in
C; mean age at motor onset was 589 years). 86 of 111 healthy male subjects.
patients died; mean disease duration was 84 years. 80 of Methods: Single rising oral doses of 10mg to 1,200mg of
111 patients underwent polysomnography. opicapone were studied under a double-blind, randomised,
Results: Stridor was diagnosed by polysomnography in 36 placebo-controlled design. 8 sequential groups of 8 subjects
patients. Mean disease duration was 94 years in patients were enrolled. Within each group, 6 subjects were
with stridor; 84 years in patients without stridor. Disease randomised to receive opicapone and 2 subjects to receive
duration after stridor onset was 74 years. 28 of 36 patients placebo. Opicapone/placebo was administered after a 10h
with stridor died: 14 underwent tracheostomy (62 years overnight fasting.
after disease onset), 5 were treated with CPAP (41 years Results: The extent and rate of systemic exposure (AUC
after disease onset), 9 patients refused any treatments. and Cmax) to opicapone increased in an approximate dose-
Statistical analyses showed a mean survival from disease proportional manner. Despite the relatively short half-life of
onset of 10 years for tracheotomised patients and 8 years for opicapone (1 to 4h), inhibition of soluble COMT (S-COMT)
CPAP treated patients; a mean survival after stridor therapy activity in erythrocytes was long-lasting, ranging from 6%
of 43 years for tracheotomised patients and 22 years for (10mg) to 55% (1,200mg) at 72h post-dose. Maximum
CPAP treated ones. S-COMT inhibition (Emax) occurred between 1 and 6 h
Conclusions: In our series stridor does not appear to be a post-dose (tEmax), and was 34.5%, 71.7%, 93.8%, 96.3%,
marker for reduced life expectancy in MSA. Despite the 100%, 100%, 100% and 100% with the doses of 10, 25, 50,
treatment, the mean survival time is not significantly 100, 200, 400, 800 and 1,200mg, respectively. Urine levels
different. A prospective study is needed to shed light on the of opicapone and its metabolites usually remained below
prognostic effect of the nocturnal stridor and its treatment the limit of quantification, showing that the kidney is not
that is one of the most reliable clinical hallmarks of MSA. the primary route of excretion. Opicapone was well
tolerated at all doses tested.
Conclusion: Opicapone was well tolerated at oral single
doses of 10 to 1,200mg, presented a dose-proportional
kinetics and effectively inhibited erythrocyte soluble
COMT activity.
[1] J Med Chem 53, 3396-3411, 2010.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 303

P1561 P1562
Effect of verbal auditory cues on cortical Genetic features and dopaminergic
motor excitability in Parkinsons disease. imaging in benign hereditary chorea
Evidence from motor evoked potentials T. Konishi1, S. Kono1, M. Fujimoto2, T. Terada1,3,
E. Shahine1, N.A. Elsawy1, G.A. Achmawi2 Y. Ouchi3, H. Miyajima1
1First Department of Medicine, Hamamatsu University
1Physical Medicine and Rehabilitation, 2Neurology
Department, Faculty of Medicine, Alexandria University, School of Medicine, Hamamatsu, 2Department of Neurology,
Alexandria, Egypt Iwata City Hospital, Iwata, 3Laboratory of Human Brain
Imaging Research, Molecular Imaging Frontier Research
Introduction: Parkinsons disease (PD) patients rely on
Center, University School of Medicine, Hamamatsu, Japan
external sensory inputs to guide movements.
Aim: To study the effect of verbal auditory cues on cortical Introduction: Benign hereditary chorea (BHC) is a rare
motor excitability of PD patients. autosomal dominant movement disorder caused by a
Participants and methods: The study included 17 PD mutation in the TITF-1 gene, which is essential for the
patients and 15 healthy controls. Motor evoked potential development, differentiation and organization of the basal
(MEP) was recorded from abductor pollicis brevis muscle ganglia. Previously reported TITF-1 mutations were located
at baseline, following repetitive rhythmic thumb abduction- in the exons or splice sites adjacent to the exons. Some
adduction at preferred speed and with verbal cues. Number patients showed a response to levodopa therapy, suggesting
of repetitive movement cycles (RMC), resting motor a functional impairment in the dopamine neuronal pathway
threshold (RMT), central motor conduction time (CMCT), of the basal ganglia.
MEP amplitude ratio and cortical silent period (CSP) mean Methods: We investigated the clinical features in Japanese
duration were measured. BHC family members by genetic analyses of the TITF-1
Results: At baseline, PD patients had significantly higher gene and positron emission tomography (PET) imaging to
MEP amplitude ratio and shorter CSP mean duration than assess the integrity of the striatal dopaminergic system
controls (p=0.9, 0.01, respectively). At their preferred using [11C]-CFT for the presynaptic dopamine transporter
speed, PD patients had significantly lower RMC compared function and [11C]-raclopride for the postsynaptic D2
to controls (p=0.005); and compared to baseline, they had receptor function.
significantly lower RMT, prolonged CMCT and increased Results: The BHC patients presented non-progressive
CSP mean duration (p=0.04, 0.05 and 0.01, respectively). generalized choreic movements initiated in childhood and
With verbal cues, both PD patients and controls could no response to levodopa treatment. The genomic DNA
increase significantly RMC (0.000, 0.028 respectively) but analyses showed a novel heterozygous c.464-9C>A
still lower in patients (p=0.002). Following verbal cues, mutation in intron 2. A sequencing analysis of the lung
none of the MEP parameters had changed significantly TITF-1 mRNA by a reverse transcription-PCR assay
among patients compared to controls and compared to revealed an abnormal transcript with a 7bp insertion,
performance without cues. Controls had significant identical to sequence in intron 2, at the boundary of exons 2
shortening in CMCT and prolongation of CSP mean and 3. Dopaminergic PET studies revealed decreased
duration (p=0.046, 0.001 respectively). raclopride binding in the striatum compared to age-adjusted
Conclusion: Parkinsons disease patients have significant control values, while the CFT binding was not altered.
cortical hyperexcitability compared to healthy subjects. Conclusions: The intronic mutation created a new acceptor
Performing a repetitive motor task with or without verbal splice site leading to the production of an aberrant transcript
auditory cues may normalize cortical excitability level in affecting the expression of TITF-1 gene. Dopaminergic
PD patients. PET imaging revealed striatal D2 receptor impairment
which may be attributed to a lack of response to levodopa.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


304 Posters, Sunday 9 September

P1563 P1564
Ocular application of oxybuprocaine Resting state functional connectivity in
hydrochloride against apraxia of lid early Parkinsons disease
opening: a randomized controlled trial K. Szewczyk-Krolikowski1,2, R. Menke3,4, M. Hu1,2,
M. Kohsaka, T. Oeda, H. Nakano, A. Umemura, K. Talbot1,2, C. Mackay1,2,3
1Nuffield Department of Clinical Neurosciences, 2Oxford
S. Tomita, R. Hayashi, K. Yamamoto, H. Sawada
Clinical Research Center and Neurology, Utano National Parkinson Disease Centre (OPDC), 3FMRIB Centre,
4Department of Psychiatry, University of Oxford, UK
Hospital, Kyoto, Japan
Introduction: Apraxia of lid opening (ALO) is often seen Background: RS-fMRI is a promising new method for
in patients with Parkinsons disease. ALO is usually developing an imaging biomarker in PD. It investigates
aggravated by bright lights or irritants to the cornia such as functional connectivity between different brain regions at
cold wind, suggesting that it could be caused by abnormal rest and requires minimal cooperation from the patient.
reaction to trigeminal sensory input from the cornea. The Previous resting state studies in PD used Region of Interest
purpose of the study is to examine an effect of corneal (ROI) analyses which are very investigator-dependent. In
aesthetic against ALO. our study we employed a data driven approach of
Methods: A randomized placebo-controlled cross-over trial Independent Component Analysis to look at changes in
of ocular application of oxybuprocain hydrochloride, a Resting State Networks (RSN).
short acting corneal anaesthetic, was conducted in 8 Methods: 19 early stage idiopathic PD patients and 19
Parkinsons disease patients. Time to complete opening normal controls (NC) were selected from the Oxford
after 10 minutes eye closure (complete opening time Parkinson Disease Centre cohort. Patients were scanned on
(COT)) was measured 14 times (before and every minute two sessions: OFF and ON medication. Scans were
from 1 to 13 minutes after eye drops of oxybuprocain performed under resting conditions with eyes open in a 3T
hydrochloride or saline as placebo). Primary outcome MRI scanner. Data was analysed with Independent
measure was a difference of % change of COT (%COT) Component Analysis implemented in MELODIC, FSL.
from baseline between the two arms. The data were Results: We isolated 25 components. Comparing OFF state
analyzed statistically by a two-way repeated-measure PD patients to NC, the Basal Ganglia/Thalamic network
ANOVA. showed reduced connectivity in the putamen bilaterally.
Results: In the oxybuprocaine hydrochloride arm, the mean The left putamen showed increased connectivity with the
COT was reduced from baseline COT at every point. In Default Mode network. Dopaminergic medication
contrast, every mean COT was increased in the saline arm. normalised all the differences. We found significant
Comparing %COTs between the two arms, eye-drop of correlations with UPDRS part III subscores in the Sensori-
oxybuprocaine hydrochloride improved COT significantly Motor and Cerebellar networks.
compared to saline eye-drop (p<0.05). In 5 patients (63%), Conclusions: Independent Component Analysis of
the effect of oxybuprocain hydrochloride appeared within 1 RS-fMRI is a sensitive method for investigation of
minute after the eye drop. functional connectivity in PD. Identified connectivity
Conclusion: Oxybuprocain hydrochloride is useful for differences correspond very well to results of task-related
ALO in Parkinsons disease patients as a rescue drug. fMRI studies. In particular, reduced activation in the basal
ganglia has been one of the most commonly reported
findings. Our study shows additionally that this phenomenon
may be related to a stronger correlation of the putaminal
activity with the Default Mode Network.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 305

P1565 P1566
Differences in non-motor symptoms in Botulinum toxin therapy of cervical
patients with young-onset and late-onset dystonia (CD): duration of therapeutic
Parkinsons disease effects
I. Stankovic1, V. Markovic1, M. Svetel1, T. Pekmezovic2, D. Dressler, P. Tacik, F. Adib Saberi
V. Kostic1 Movement Disorders Section, Department of Neurology,
1Movement Disorders Department, Neurology Clinic, Hannover Medical School, Hannover, Germany
Clinical Center of Serbia, 2Neuroepidemiology Unit, Institute Introduction: We sought to explore the duration of
of Epidemiology, School of Medicine, University of therapeutic effect of botulinum toxin (BT).
Belgrade, Serbia Methods: Patients with CD, receiving Xeomin, Botox or
Objective: To assess prevalence of different non-motor both treatments in sequence, entered a non-interventional
symptoms (NMS) in young-onset Parkinsons disease study. Outcome measures included treatment duration (TD;
patients (YOPD) in comparison to their older counterparts time between application of BT and patient-reported waning
(LOPD). of therapeutic effect), inter-injection interval (II) and excess
Introduction: In some studies it was suggested that YOPD time (ET; ET=II-TD).
patients have an increased risk of non-motor symptoms. Results: 59 patients (male, n=22; mean age, 52.6 years;
Methods: This cross-sectional study comprised 101 mean Tsui score, 9.0) and 1289 treatment cycles (mean
consecutive YOPD and 107 LOPD patients. All PD patients number per patient, 21.8; standard deviation [SD], 14.0;
were clinically evaluated and completed the revised NMS range, 4-66) were evaluated. Mean (SD) TD per patient
Quest. The two separate backward stepwise linear ranged from 7.8 (1.4) to 21.0 (3.9) weeks (overall mean,
regression analyses were used to examine how the various 11.8 [2.7]). Mean TD was 12 weeks in 83.1% of patients
demographic and clinical characteristics contribute to the and 10 weeks in 35.6%. Mean (SD) II per patient ranged
total number of NMS. from 11.3 (1.3) to 27.8 (11.6) weeks (overall mean, 15.4
Results: The mean total NMS (NMSQ-T) was 11.96.0 in [3.4]). Overall mean (SD) ET was 3.5 (2.4) weeks (22.7%
LOPD and 7.75.8 in YOPD (p<0.05). Dribbling of saliva, of overall mean II). TD and II were generally stable,
loss of taste/smell, nocturia, forgetfulness, loss of interest, although 16.9% of patients experienced treatment delays,
hallucinations, lack of concentration, anxiety, change in most frequently due to appointment difficulties. Prolonged
libido and difficulty in sexual activities were more prevalent treatment effects, probably due to CD fluctuations, occurred
in LOPD. The only NMS more prevalent in YOPD were in 18.6% of patients. Singular unexplained therapy failure
restless legs and sweating. Significant positive correlations (SUTF) occurred following 0.4% of injection series. No
were registered between the NMSQ-T and actual age antibody-induced therapy failure occurred.
(r=0.480; p=0.001 and r=0.261; p=0.007), duration of PD Conclusion: TD and II were generally stable with long-
(r=0.499; p=0.001 and r=0.359; p=0.001) and H&Y stage term BT application, but could be influenced by treatment
(r=0.607; p=0.001 and r=0.556; p=0.001) in both YOPD delays, CD fluctuations or SUTF. ET indicated that patients
and LOPD groups, respectively. In the multivariate linear were treated suboptimally for ~23% of their treatment
regression model, in the YOPD group only the H&Y stage cycle, supporting a reduction of II. BT formulations
of the disease appeared to be a significant predictor of an associated with low antigenicity may be useful if II is
increasing number of NMS, while in the LOPD group reduced to 12 weeks.
significant contributors were also age at onset and duration
of PD.
Conclusions: NMS were less frequent in our YOPD
patients and had different predictors than in LOPD group.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


306 Posters, Sunday 9 September

P1567 P1568
Dysautonomic dysfunction in Parkinsons A meta-analysis of cognitive deficits in
disease evaluated by SCOPA-AUT scale clinical subtypes of multiple system
V. Arnao, S. Realmuto, F. Valentino, G. Famoso, atrophy
S. Mastrilli, A. Cinturino, V. Perini, P. Aridon, E. Lyros1,2, A. Tsapanou3, L. Messinis4,
P. Ragonese, M. DAmelio, G. Savettieri P. Papathanasopoulos5, K. Fassbender6
Dipartimento di Biomedicina Sperimentale e Neuroscienze 1Neurology, University of Saarland, Homburg, Germany,
Cliniche, Universit degli Studi di Palermo, Italy 2Neurology, Medical School, University of Patras,
Introduction: Non-motor symptoms of PD are common, 3University of Athens Medical School, Athens, 4University of

occur across all stages of PD, and are under-reported. Patras, 5University of Patras Medical School, Patras,
Dysautonomic symptoms generally associated with more Greece, 6University of Saarland, Homburg, Germany
advanced Hoehn and Yahr (H&Y) stages, further aggravate Dementia and severe cognitive impairment are considered
quality of PD patients life. as non-supporting features for the diagnosis of multiple
Objective: To assess frequency of dysautonomic symptoms system atrophy (MSA). Several studies however suggest
in PD patients and to correlate to PD related characteristics. that neuropsychological deficits are frequent in this
Method: All consecutive non-demented PD patients who condition. It would also be interesting to see whether the
underwent an extensive evaluation including UPDRS, and magnitude of these deficits would be any different among
Scale for Outcomes in PD for autonomic symptoms MSA with predominant parkinsonism (MSA-P) and MSA
(SCOPA-AUT) were enrolled. SCOPA-AUT evaluates the with predominant cerebellar ataxia (MSA-C). Therefore we
involvement of gastro-intestinal, urinary, cardiovascular, conducted a meta-analysis to increase power by pooling
thermoregulatory, pupillomotor and sexual dysfunction. data from individual relevant studies based on a Pubmed
Supine to standing position blood pressure and cardiac database search (studies that qualified had to include both
frequency changes were measured. Orthostatic hypotension patient groups or clearly defined only MSA-P or MSA-C
(OH) was diagnosed according to consensus statement on group, a control group, a battery of diverse neuro
the definition of OH (Freeman, Clin Auton Res. 2011). psychological tests, statistics that were convertible to affect
Results: 89 PD patients were included (mean age at size g and be written in English). Eligible studies reached
interview was 67.7). Patients were stratified according to the number of 7. The overall mean effect size for each
the medium score of SCOPA-AUT scale (17.1). Those with cognitive domain was computed using a random effects
higher SCOPA-AUT score had more frequently an older model and adjusted for sample size. Our preliminary results
age at PD onset (77%; 62.4 years), higher UPDRS IV score indicate that significant decrements in neuropsychological
(61%; 30.1) and longer disease duration (64%; 5.2 years). performance loading on executive functions are present in
Mean SCOPA-AUT score was significantly higher both patients groups with their magnitude however being
(21.88.2) among those patients (5; 5.6%) who showed OH greater for the MSA-P group. Reasoning, psychomotor
compared to those without (16.930.2). speed and memory recognition were less severely impaired.
Conclusion: Our study remarks the role of autonomic It is implied that dysfunction in the frontostriatal circuits but
dysfunction in PD. In our population characterized by mild also of its cerebellar connections may underlie the observed
to moderate disease severity, in most of patients the deficits.
autonomic system was involved. In these symptoms
frequency increases with duration and severity of disease,
might be present also at onset, suggesting therefore
evaluating their presence also in the earliest stage.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 307

P1569 P1570
Validation study of genetic factors Leukoaraiosis as a predictor of cognitive
contributing to Parkinsons disease in decline in patients with Parkinsons
Spanish and Latin American populations disease submitted to STN-DBS
A. Sesar1, P. Cacheiro2,3, M. Camia-Tato3, M. Lopez4, F. Sousa1, J. Rocha1, H. Costa2, C. Sousa3, J. Lima3,
B. Quintns2,3, E. Alonso5, E. Cebrin6, A. Castro1, R. Figueiredo4, C. Reis4, J. Massano5, M. Basto4,
A. Carracedo2,3, M.J. Sobrido2,3 P. Linhares5, R. Vaz5, M.J. Rosas5
1Neurology, Santiago de Compostela Clinical Hospital. 1Neurology Department, Hospital de Braga, 2Neurology

University of Santiago de Compostela, 2Neurogenetics Department, 3Neuropsychology Unit, 4Neuroradiology


Group, Fundacion Publica Galega de Medicina Xenomica- Department, 5Movement Disorders Unit - Functional
SERGAS, 3Genomic Medicine Group, School of Medicine- Surgery, Hospital de So Joo, Porto, Portugal
University of Santiago de Compostela, Spain, 4Biological Introduction: Subthalamic nucleus deep brain stimulation
Systems, Universidad Autonoma Metropolitana-Xochimilco, surgery (STN-DBS) may lead to selective cognitive decline.
5Neurogenetics, Instituto Nacional de Neurologa y
Leukoaraiosis is a cognitive decline predictor in patients
Neurociruga Manuel Velasco Surez, Mexico DF, Mexico, with cerebrovascular disease. The aim of the study is to
6Neurology, Complexo Hospitalario Universitario de
evaluate the influence of leukoaraiosis on cognitive decline
Pontevedra, Santiago de Compostela, Spain
after STN-DBS in Parkinsons disease (PD).
Introduction: In past years some genes have been Methods: From October 2002 and December 2010,
associated with predisposition to Parkinsons disease (PD) neuropsychological studies pre- and post-DBS (12-18
. The aim of this study was to analyze several candidate months) were prospectively registered and Fazekas
genes as susceptibility factors to PD in populations from classification of leukoaraiosis in pre-DBS MR imaging was
Spain and Latin America. evaluated in patients with PD undergoing STN-DBS.
Methods: PD cases defined by the Gelb criteria were Demographics, disease duration, educational level, Mini
compared with controls selected after family history and Mental State Examination (MMSE), Frontal Assessment
clinical exam. SNPs in 12 previously reported genes and Battery (FAB), categorical (Fvcat) and phonemic (Fvfon)
intergenic regions were genotyped by SNPlex. After quality verbal fluency tests were compared.
control by genotyping rates, minor allele frequency and Results: 91 patients were enrolled, 64 with leukoaraiosis.
Hardy-Weinberg equilibrium - no significant population In this group there was a decrease in MMSE (27.94 to 26.94
substructure is known in the Galician population; 71 SNPs p<0.05), FAB (13.02 to 11.80 p<0.05), Fvcat (13.76 to
were analyzed in 268 patients and 257 controls. Quality 10.77 p<0.05) and Fvfon (18.48 to 13.86 p<0.05). In
control and association tests were carried out with the R patients without leukoaraiosis, FAB (13.62 to 13.33
package SNPassoc. Imputation of additional markers was p<0.05), Fvcat (14.73 to 11.81 p<0.05) and Fvfon (20.56 to
performed using HapMap panels. 14.48 p<0.05) decreased, but not MMSE (27.74 to 27.89
Results: Nominally significant associations (P<0.05) were p<0.05). The mean value of MMSE and FAB post-DBS was
found for SNPs belonging to MAPT, SNCA and SYT11 lower in subjects with leukoaraiosis (p<0.05). There were
genes. Only the MAPT association remained significant no differences in score variations between Fazekas 0-1 and
after multiple testing correction (False Discovery Rate). 2-3, progression to dementia or educational level.
These results were also checked in a Mexican cohort. Conclusion: These results confirm the trend for cognitive
Conclusions: Genetic variation in MAPT and SNCA have decline in the evaluated areas after DBS. Patients with
been associated with PD in independent populations. The leukoaraiosis have lower scores on evaluated domains, but
replication of MAPT and SNCA association confirms the only MMSE and FAB differences were significant.
participation of these genes in susceptibility to PD while
serving as a quality indicator of our sampling protocol.
Regarding SYT11 there is only a meta-analysis of GWAS
suggesting association with PD. Our data, together with
previous results, suggest that SYT11 encoding
synaptotagmin XI, substrate for parkin may play a so far
under-recognized role in the pathogenesis of PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


308 Posters, Sunday 9 September

P1571 P1572
What is the diagnosis behind patients CSF uric acid as an anti-oxidant in
with parkinsonian syndrome and normal Parkinsons disease
123I-Ioflupane SPECT? A clinical follow-up M. Pohja1, K. Murros2
study Helsinki University Central Hospital, 2Helsinki University
1

Central Hospital, Jorvi Hospital, Espoo, Finland


M. Menndez1,2, F. Tavares1, M. Martnez1,
A. Lpez-Muiz2, J.A. Vega2 Oxidative stress is thought to be an important factor in the
Hospital lvarez-Buylla, Mieres, 2Morfologa y Biologa
1 pathogenesis of Parkinsons disease (PD). Low serum uric
Celular, Universidad de Oviedo, Spain acid (UA) has been associated with faster clinical
Introduction: Making the diagnosis of Parkinsons disease progression of the disease. UA is an antioxidant having
(PD) is a common clinical situation faced by neurologists, interactions with another important antioxidant, ascorbic
geriatricians, and general physicians. The Food and Drug acid (AA). We studied if UA or AA or total antioxidant
Administration (FDA) recently approved [123I]ioflupane capacities in plasma and CSF are altered in PD. 16 PD
([123I]-fluoropropyl CIT, DaTSCAN), a dopamine patients (52-73 yrs, 50% males, mean disease duration 4
transporter (DAT) radioligand, for SPECT to assist in the years, mean UPDRS motor score 21) were recruited. 16
evaluation of adult patients with suspected parkinsonian gender- and age-matched healthy subjects served as
syndromes (PS). We aimed what is the diagnosis of patients controls. UA in CSF was statistically lower in PD patients
with PS and normal DaTSCAN. than in controls (19.6 umol/l vs. 25.6umol/l, p=0.03). In
Methods: A series of 30 patients with parkinsonism and plasma, the corresponding difference was trend-like
normal 123 I-Ioflupane SPECT. They were followed up for (p=0.07). The CSF/plasma ratio was the same in both
at least 2 years; and then a second DaTSCAN was groups but strong correlation between serum and CSF UA
performed. The diagnosis was reconsidered at endpoint. was seen in PD but not in the control group (0.77, p=0.01
Results: The second DaTSCAN changed from normal to vs. 0.42, p=0.10). In PD group, AA was higher both in
abnormal only in 4 patients. Final diagnosis included cases plasma (77umol/l vs. 56umol/l, p=0.03 ) and in CSF (206
of essential tremor (4), SWEDD (2), FTAX (1), iatrogenic umol/l vs. 156umol/l, p=0.01) suggesting higher vitamin C
parkinsonism (1), vascular parkinsonism (2), corticobasal intake but no difference in active transport mechanism
degeneration (2), multisystem atrophy (4) and PD (8). The across BBB. Antioxidant capacities in serum and CSF
diagnosis remained uncertain in 6 cases. estimated by TRAP were similar in both groups. Our
Conclusion: DaTSCAN can help the diagnostic process in novelty finding was that in PD patients CSF uric acid
certain situations but it should not be relied on as a substitute concentrations are lower than in healthy, gender- and age-
for careful, experienced clinical assessment and follow up. matched controls. In addition, plasma and CSF uric acid
There is a list of alternative diagnoses to consider when a concentrations are more tightly coupled suggesting
PD patient presents with normal DaTSCAN. Following potentially different or altered UA regulation mechanism in
cases of uncertain diagnosis with DaTSCAN studies does PD.
not seem to be useful.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 309

P1573 P1574
Does Rivastigmine improve balance Correlation between severity of cognitive
control in Parkinsons dementia? deficit and motor symptoms in patients
E. Pourcher1,2, A. Nadeau3, M. Jaime3, P. Corbeil4,5 with Parkinsons disease
1Clinique Sainte-Anne, 2Department of Medicine, Faculty of S. Tomic1, M. Vladetic1, K. Solic2, S. Misevic1, S. Juric1,
Medicine, Laval University, 3Department of Kinesiology, L. Knezevic Poljak1, S. Butkovic Soldo1
Laval University, 4Groupe de Recherche en Analyse du 1University Hospital Center Osijek, 2Medical School
Mouvement et Ergonomie, Faculty of Medicine, Laval University of J.J. Strossmayer in Osijek, Croatia
University, 5Centre de Recherche FRSQ, Centre Hospitalier
Affili Universitaire de Qubec, QC, Canada Aim: To determine the existence of cognitive deficits in
patients with idiopathic Parkinsons disease (PD) compared
Introduction: According to Allcock et al. (2009), impaired to a control group and to find a correlation between the
attention predicts falling in Parkinsonian patients, however severity of cognitive deficits and motor symptoms in order
the contribution of attention improvement with to prove influence of dopamine on cognition.
cholinesterase inhibitors on balance control in Parkinsons Patients and methods: Demented and depressed PD
disease has never been formally explored. patients were excluded from the study. Patients with PD
Methods: 20 patients with idiopathic Parkinsons disease were scored with UPDRS III and both groups made tests of
and mild to moderate dementia (PDD) were randomized to cognitive function: Dementia Rating Scale (DRS), Raven
receive either Rivastigmine trans-dermal patches or oral progressive coloured matrices (RPCM) and Mill Hill
capsule for 24 weeks. Postural control using a force Vocabulary (MHV) test. To analyse data we used the
platform was assessed at baseline and 24 weeks under four methods of descriptive statistics, Student T-test, Mann-
conditions: eyes open/ eyes closed with static support Whitney U-test, Pearson and Spearman correlation
surface/ sway referenced to body sway form support co-efficient with p<0.05.
surface. Other outcomes included scores of the MATTIS Results: We found statistically significant differences in
Dementia Rating Scale (global and attention sub-scores) MHV test and the frequency of pathological tests of
and the UPDRS motor sub-section. construction and conceptualization (DRS subscales)
Results: At 24 weeks, there was no difference in measures between PD patients and control groups. We found no
of postural control at rest, with a static support surface, correlation between the results of cognitive tests and
however, a significant reduction was observed in sway UPDRS part III in the PD patients group. There are no
velocity when the support surface was sway-referenced to connections between cognitive deficit and duration of
body sway (p<0.01). Participants UPDRS III scores did not disease and type of PD (rigid or tremorous form). There is
differ. Better performances on the MATTIS Dementia a possible connection between damaged visual-spacial
Rating Scale global scores were observed after 24 weeks perception and lesion of right side of brain.
(p=0.052). However neither the global score, nor the Discussion: Patients with PD more often had cognitive
attention sub-score of the MATTIS correlated with deficit (dysexecutive syndrome, synonym finding and
improvement in sway velocity. No difference was observed impaired visual-spatial perception) when compared to the
in balance performance between patients on patches and control group. There is no correlation between cognitive
patients on oral form. deficits and severity of motor symptoms in PD patients.
Conclusion: In PDD, Rivastigmine improves adaptive Conclusion: These results suggest that cognitive deficits
balance control independently of an improvement on the are not only due to dopamine depletion, but etiology is
attention MATTIS score. This result tends to support an probably more complex.
improvement of balance driven by a sub-cortical cholinergic
modulation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


310 Posters, Sunday 9 September

P1575 P1576
The Rome tremor scale: an easy Expanding the spectrum of chorea
instrument for the evaluation of tremor associated with immune-mediated
F. Puledda, L. Troilo, G. Lenzi diseases: two case reports
Neurology and Psychiatry, University of Rome Sapienza, E. Unti1, S. Mazzucchi1, D. Martino2, F. Vanelli1,
Rome, Italy R. Ceravolo1
Background: Tremor is a movement disorder that affects Department of Neuroscience, University of Pisa,
1

more than 10% of the general population. However, few Department of Neurological and Psychiatric Sciences,
2

clinical scales are available for tremor evaluation: the University of Bari, Italy
Clinical Tremor Rating Scale (CTRS) and the UPRS. The Introduction: Wegener Granulomatosis (WG) and psoriasis
CTRS needs 30-90 minutes, and a fully compliant patient to are diseases in which neurological involvement is quite
be carried out. The UPRS is utilised in PD, with just a few rare; up to our knowledge, there are no cases associated
of the 42 evaluations concerning tremor. with movement disorders in literature.
Objectives: In the planning of a clinical trial on tremor, we First case: A 72-year-old male, with a diagnosis of WG
designed a user-friendly tremor scale, the Rome Tremor since 1999. Since 2003 he presented simple verbal tics, four
Scale (RTS) and tested it against the CTRS. limbs involuntary movements, and an amnestic mild
Materials and methods: RTS consists of six items: 0=no cognitive impairment. No steroids were given. Hyperkinetic
tremor; 1=tremor present in emotional situations, not syndrome improved with low dosages of tetrabenazine
invalidating; 2=tremor present, affects writing; 3=tremor Second case: A 67-year-old woman presented with choreic
present, affects daily activities; 4=tremor present, patients movements mainly in the left limbs, slight dysarthria and
require constant help; 5=disabling tremor. painful tumefaction of left wrist. Neuropsychological
Results: 40 outpatients attending our Clinic because of evaluation showed a mild frontal dysfunction. Within four
neurological problems including tremor completed both months diagnosis of psoriasis was made. Methylprednisolone
CTRS and RTS. The mean score was 15.851.33 for CTRS was given with improvement of chorea.
and 2.0250.094 for RTS. Correlation between CTRS and Methods: Genetic tests for HD, HDL2, SCA-17 and
RTS shows r=0.943. By dividing patients into two cohorts, research for acanthocytis were negative. FDG-PET showed
PD and essential tremor, correlation remains highly right caudo-putaminal hypermetabolism in the first patient
significant, r=0.971 and r=0.907 respectively. and bilateral basal ganglia hypermetabolism in the second
Conclusions: The high correlation between RTS and CTRS patient. Brain MRI did not show any significant alterations.
indicates that RTS appears to be a valid and easy instrument CSF examination, iron and copper sieric values were in the
for the clinical evaluation of tremor in an out-patient normal range. Sieric anti-basal ganglia antibodies (ABGA)
neurological setting, both in PD patients and patients showed auto-antigen of 45 kDa.
affected by tremor due to other causes. Conclusions: Evidence of striatal hypermetabolism on
FDG-PET with chorea associated with SLE and primary
antiphospholipid syndrome has been reported probably due
to auto-antibodies versus basal ganglia neurons. The ABGA
positivity previously reported in autoimmune movement
disorders, does not imply a pathogenetic relationship,
however along with the striatal hypermetabolism in both
and the good response to steroids in the second, one might
extend the spectrum of chorea associated with autoimmune
diseases.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 311

P1577 P1578
Frequency distribution of orthostatic A randomized, double-blind, placebo-
hypotension, nocturnal hypertension and controlled trial of hydrogen water in
postprandial hypotention in patients with Parkinsons disease
Parkinsons disease: a case-control study A. Yoritaka1,2, M. Takanashi2, M. Hirayama3, S. Ohta4,
A. Chitsaz N. Hattori2
Neurology, Isfahan University of Medical Sciences, Isfahan, 1Neurology, Juntendo Koshigaya Hospital, Koshiaya-shi,
Iran 2Neurology, Juntendo University School of Medicine,
Bunkyo-ku, 3Pathophysiological Laboratory Science, Nagoya
Background: Etiology of orthostatic hypotension include
University Graduate School of Medicine, Nagoya,
sympathetic denervation and treatment with dopaminergic 4Biochemistry and Cell Biology, Development and Aging
drugs.
Sciences, Graduate School of Medicine, Nippon Medical
Methods: The current study was conducted as a case- School, Tokyo, Japan
control study in the second half of 2008 and first half of
2009. The study population included Parkinsonian patients Background: The major pathological hallmarks of
who received levodopa, >500mg/day for over 6 months. Parkinsons disease (PD) are cellular energy depletion and
Patients who had not received any levodopa until the time oxidative stress leading to cellular dysfunction and death.
of the study were in the control group. Exclusion criteria Molecular hydrogen (MH) selectively reduces the hydroxyl
included having a concomitant disease or taking medications radical, the most reactive oxygen species, and can thereby
that cause orthostatic hypotension. Study variables included effectively protect cells. MH water has been reported to
demographic characteristics, severity of disease, orthostatic prevent dopaminergic cell loss in rat models of PD.
hypotension, postprandial hypotension and nocturnal Method: To determine that intake of MH water is safe and
hypertension. Using the mercury barometer, the presence or a disease-modifying treatment of PD, we conducted a
absence of orthostatic hypotension was determined in all double-blind, placebo-controlled trial of MH water in
patients. The postprandial hypotension and nocturnal levodopa-treated patients with PD. The subjects prepared
hypertension were identified using the portable device of MH water (1.6ppm) by using Aquela blue (ecomo
ambulatory blood pressure monitoring. International) and consumed 1,000ml of MH water divided
Findings: The frequency distribution of orthostatic per day for 48 weeks.The primary end-point was the change
hypotension was 12% and 20% in the case and control from baseline in the total score of the Unified Parkinsons
group, respectively. The frequency distribution of disease Scale (UPDRS). Additional analysis is the change
postprandial hypotension was 12% and 24% in the case and of part of II and III of UPDRS and Hoehn and Yahr stage
control group, respectively. The frequency distribution of after 8, 24, 48 weeks and 8 weeks after treatment compared
nocturnal hypertension was 40% and 56% in the case and with the data at baseline.
control group, respectively. Result: 18 subjects (7 male, 11 female) were enrolled in the
Conclusion: Although the prevalence of orthostatic treatment protocol. The changes in the total UPDRS score
hypotension between the case and the control group was will be calculated with Mann-Whitney U-test after blinding
different, the difference was not meaningful. will be opened after all subjects have finished protocol.
Conclusion: No doubt on safety of MH water intake has
been found until now. We will demonstrate if MH may be
applicable or not for the therapy in Parkinsons disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


312 Posters, Sunday 9 September

P1579 P1580
The prevalence of non-motor symptoms Transcranial sonography in differential
and restless legs syndrome in Parkinsons diagnosis of restless legs syndrome
disease: correlation with quality of life M. Budisic, Z. Trkanjec, V. Supanc, A. Lovrencic-Huzjan,
K.A. Abdul Manaf1, W.N.N. Wan Yahya1, H.J. Tan1, V. Bai-Kes
R. Azman Ali1, H. Othman2, S. Azhar3, University Hospital Sestre Milosrdnice, Zagreb, Croatia
N. Mohamed Ibrahim1 Background: Restless legs syndrome (RLS) has a
1Internal Medicine, 2Pathology, 3Community Health, prevalence of around 10% in the general population. Still, it
University Kebangsaan Malaysia Medical Centre, Kuala is one of the most underdiagnosed neurological disorders.
Lumpur, Malaysia Non-idiopathic RLS is more frequently found in patients
Introduction: The non-motor symptoms (NMS) are with anaemia. Recent transcranial sonography studies have
important determinants of health and quality of life (QoL) shown that substantia nigra (SN) hypo-echogenicity appears
in Parkinsons disease (PD). It occurs early and may even to be a frequent finding in RLS, just as it is increased blood
predate the diagnosis of PD. NMS are not well recognized flow velocity in a. cerebri media (ACM), measured by
in clinical practice despite their impact. TCCS, in anaemic patients. Aim of this study was to
Aims: This study was conducted to determine the evaluate the usage of combined TCS/TCCS imaging in
prevalence of NMS and restless legs syndrome (RLS) in PD differential diagnosis of idiopathic and secondary RLS.
and their impact on QoL. Patients and methods: 30 patients with RLS symptoms
Methods: This was a cross-sectional study involving (diagnosis was made according to IRLSSG criteria), 20
patients with idiopathic PD. Prevalence of NMS using the patients with anaemia (haemoglobin values <12g/dl) and
NMS questionnaires was determined. The QoL and severity RLS symptoms, and 30 controls underwent neurological
of NMS were assessed with PDQ-39 questionnaires and and sonographic examination.
NMS Assessment Scale, respectively. Patients who met the Results: Bilateral mean SN area measured on TCS was
criteria of International RLS Study Group criteria for the significantly lower in idiopathic RLS patients versus
diagnosis of RLS were identified and blood samples to test controls ( 0.090.01cm2 vs. 0.180.02 cm2; p<0.01). Values
serum iron were taken. of mean blood flow velocity measured in ACM by TCCS
Results: A total of 113 patients consisting of 60 males and were also higher in RLS patients (123.247.33 vs.
53 females were recruited. The mean age was 64.89.0 80.76.55; p<0.01) with anaemia, with normal finding of
years. The median duration of illness was 5.0 (2.0-8.0) SN area.
years. 97.3% of patients reported the presence of NMS. The Conclusion: TCCS in combination with TCS is a useful
most common symptoms were gastro-intestinal symptoms tool in diagnosing idiopathic RLS, just as it is in
(76.1%) followed by neuropsychiatric symptoms (72.6%) differentiating idiopathic from secondary RLS due to
and autonomic dysfunction (64.6%). 11 patients (9.7%) had anaemia.
RLS. Patients with PD/RLS had a younger age of PD onset
(p=0.023) and lower serum ferritin levels (p=0.616). NMS
affected the QoL significantly in all dimensions of PDQ-39.
Each dimension of PDQ-39 correlated strongly with the
severity of NMS.
Conclusions: NMS was highly prevalent in our patients.
The presence and severity of NMS adversely affected the
QoL.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 313

P1581 P1582
XCiDaBLE, a phase IV, prospective Evaluating the efficacy of rasagiline on
observational trial evaluating depressive symptoms in Parkinsons
incobotulinumtoxinA (XEOMIN) for disease patients without dementia: the
cervical dystonia or blepharospasm: ACCORDO study (ongoing clinical trial)
preliminary baseline data for subjects P. Barone, for the ACCORDO Study Investigators
with blepharospasm Centro per le Malattie Neurodegenerative, University of
Salerno, Italy
M.S. LeDoux1, J. Jankovic2,
K. Sethi3, A. Verma3,
E.J. Pappert3, H.H. Fernandez4 Objective: In Parkinsons disease (PD), the presence of
1University of Tennessee Health Science Center, Memphis, depression is associated with worse cognitive function,
TN, 2Baylor College of Medicine, Houston, TX, 3Merz functional ability, and patients quality of life. Accumulating
Pharmaceuticals, LLC, Greensboro, NC, 4Cleveland Clinic, evidence suggests that, in addition to its established motor
Cleveland, OH, USA benefits, treatment with rasagiline may also be beneficial in
Introduction: While repeated injections of botulinum the management of certain non-motor symptoms of PD;
toxin-A are the established treatment for blepharospasm, however little is known about its effects on depression. This
there is a lack of prospectively acquired data on treatment ongoing study aims to evaluate the efficacy of rasagiline on
outcomes in real-world clinical practice. depression and explore the relationship between depressive
Methods: XCiDaBLE is an open-label, prospective, symptoms and cognitive function in PD patients without
observational US study. Subjects are followed for two dementia.
cycles of incobotulinumtoxinA treatment with flexible Methods: This is a multicenter, randomized, double-blind,
dosing and injection intervals determined by the investigator. placebo-controlled study, enrolling ~136 PD patients
Subject-reported outcomes (recorded via Interactive Voice/ (Hoehn & Yahr I-III) with depression (Beck Depression
Web Response) include the Jankovic Rating Scale (JRS). Inventory (BDI-IA) score 15) but not dementia (MMSE
Investigators assess the baseline Clinical Global Impressions <26). Patients are randomized (1:1) to rasagiline 1mg/day
(CGI)-Severity. An employment questionnaire, work or placebo for 12 weeks; other anti-PD medications are
history and the SF-12v2 (0=worst, 100=best) are used to permitted at a stable level. The primary efficacy measure is
evaluate work productivity and quality of life (QoL). change from baseline to week 12 in BDI-IA total score.
Results: By 01 September 2011, 184 subjects with Secondary measures include a neuropsychiatric cognitive
blepharospasm were enrolled (27.7% male, mean age 64.9 test battery, PD Quality of Life Questionnaire, Apathy Scale
years, mean age at onset of blepharospasm 53.9 years, and Unified Parkinsons Disease Rating Scale Parts II and
97.8% previously treated with botulinum toxin). At baseline, III. Safety is assessed through adverse events (AEs).
subjects CGI-Severity was: 32.8% normal-to-mild, 35.5% Results: As of February 2012, 121 patients have been
moderate, 22.4% marked, and 9.3% severe; mean JRS randomized. Mean age is 66.7 years and mean time from
Sumscore was 5.0. Baseline mean SF-12v2 scores were diagnosis is 4.3 years. So far, 4 patients have withdrawn
43.1 (Physical QoL) and 49.1 (Mental QoL). Most subjects early due to AEs (worsening of dyskinesia, vertigo, left
(105) were employed at onset of blepharospasm, but at trunk flexion due to PD, nausea).
study baseline 19 subjects reported their employment status Conclusion: ACCORDO is the first prospective study to
was affected by blepharospasm and 33 subjects in evaluate the efficacy of rasagiline on depression. Full
employment found that their productivity was reduced results are expected in Q4 2012.
(82.8% mean estimated productivity; normal=100%). 17
subjects received or were seeking employee disability
benefits due to blepharospasm.
Conclusions: Blepharospasm has a negative impact on
QoL, employment status and work productivity. XCiDaBLE
will provide valuable insight into the dosing and injection
frequency of incobotulinumtoxinA in subjects with
blepharospasm and treatment outcomes in real-world
clinical practice.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


314 Posters, Sunday 9 September

P1583 P1584
Quality of life in patients with different Differentiation of parkinsonian disorders
forms of primary dystonia based on mineralization pattern of the
N.S. Basurovic1, M. Svetel2, T. Pekmezovic3, V.S. Kostic2 deep grey matter by susceptibility-
1Special Hospital for Cerebrovascular Diseases St. Sava, weighted magnetic resonance imaging
2Institute for Neurology, Clinical Centre Serbia, 3Institute for
M. Modi1, S. Thakur2
Epidemiology, Clinical Centre Serbia, Belgrade, Serbia 1 Neurology, 2Radiodiagnosis, PGIMER, Chandigarh, India
Introduction: Dystonia is frequently associated with
Objectives: There are multiple causes of
depression, anxiety, social phobia and poor quality of life.
parkinsonism;idiopathic Parkinsons disease (IPD) is most
Aim: Defining differences in quality of life in patients with
common (80-85%) and has to be differentiated from
primary focal and segmental dystonia.
atypical parkinsonian disorders (APD), i.e., progressive
Patients and methods: The study included 157 patients
supranuclear palsy (PSP), multiple system atrophy (MSA)
with primary focal dystonia (PFD) and 28 patients with
and corticobasal denegation (CBD). The aim was to study
primary segmental dystonia (PSD). Patients were treated at
mineralization pattern of the deep grey matter by
the Institute for Neurology KCS (1997-2008). All patients
susceptibility-weighted (SWI) magnetic resonance imaging
completed the Serbian translation of SF-36 which was used
to differentiate Parkinsons disease and atypical
as an outcome measure for health-related quality of life
parkinsonian disorders.
(HR-Qol).
Methods: A total of 30 subjects was enrolled for the study
Results: The mean age at onset of dystonia in patients with
of which 10 were in the control group and 20 patients
PFD was 43.211.5 years, and in patients with PSD it was
presented with features of parkinsonism. MR imaging
43.317.3 years. The mean duration of disease in patients
examinations were performed with a 18-channel 3.0T whole
with PFD was 7.3 years, and in patients with PSD it was 9.9
body imager (TIM MAGNETOM Verio; Siemens). First,
years. The ratio man/women in the group with PFD was
routine MR imaging of the brain including T1WI, T2WI
64:9, and in group with PSD was 17:11. Patients with PSD
and FLAIR sequences were done, followed by SWI. The
scored significantly worse in bodily pain (PSD 30.628.2
hypo-intensity of post lateral putamen, red nucleus,
vs. PFD 68.431.4) (p<0.01), role functioning emotional
substantia nigra, and dentate nucleus in all groups were
(PSD 38.144.2 vs. PFD 58.244.9) (p<0.01) and social
measured in comparison with SI of the CSF and vein of
functioning (PSD 50.013.6 vs. PFD 64.624.9) (p<0.05),
Galen.
while the same patient group scored significantly better in
Results: In the conventional MRI, presence of midbrain
general health (PSD 64.615.7 vs. PFD 53.918.8)
atrophy, superior cerebellar peduncle signal changes and
(p<0.05). There were no significant differences between the
atrophy differentiated PSP patients from MSA-P and PD
groups regarding physical functioning, role functioning
patients. The increased hypointensity scores of red nucleus
physical, vitality and mental health.
on SWI in PSP patients differentiated it from PD and MSA-
Conclusions: The quality of life in patients with dystonia,
P. The increased hypointensity score of substantia nigra in
has been defined by the complex interaction between
PD patients supports the site of pathology and differentiated
distribution and/or severity of disease, characteristics of
them from atypical parkinsonism patients.
patients personality and the influence of social environment.
Conclusion: Among the routine MRI techniques available
to differentiate PD and APD patients, we observed SWI as
a simple and advanced modality.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 315

P1585 P1586
Does neuro-inflammation affect Prevalence and characteristics of non-
occurrence of dementia in patients with motor symptoms in Korean PD patients:
Parkinsons disease? study using a non-motor symptoms
I.-U. Song, J.-W. Park, K.-S. Lee, J.-S. Kim questionnaire
Department of Neurology, The Catholic University of Korea, J.-Y. Kim
Seoul, Republic of Korea Neurology, Inje University Paik Hospital, Seoul, Republic of
Background and objective: The clinical value of high Korea
sensitivity C-reactive protein (hs-CRP) in patients with PD We investigated the frequencies and characteristics of non-
is poorly defined yet. Therefore, we conducted this study to motor symptoms (NMS) in Korean PD patients using a non-
analyze association between hs-CRP levels in PD and those motor symptoms questionnaire (NMSQ). We performed a
in PD with dementia. hs-CRP is an exquisitely sensitive semistructured interview including NMSQ in 57 consecutive
systemic marker of inflammation and tissue damage and patients who visited the movement disorder clinic of Seoul
increased level of hs-CRP is strongly associated with Paik Hospital from May 2011 to October 2011. The
inflammatory reactions. Microglia-mediated neuro demographic data, Hoehn and Yahr (HY) stage, and daily
inflammation has been hypothesized to play an important levodopa equivalent dose (LEDD) were also checked. Mean
role in the pathogenesis of idiopathic Parkinsons disease age and duration of disease were 70.8 and 4.6 years. Mean
(PD). Furthermore, many reports have suggested that high HY stage was 2.7 and LEDD was 793.4mg/day. We found
concentration of hs-CRP is associated with increased risk of that the mean number of NMS per patient was 14.3 and all
cardiovascular disease, stroke, and cognitive impairment patients had non-motor symptoms (range: 5-25). The most
including dementia. common items were: nocturia (87.7%), anxiety (73.7%),
Methods: We examined 60 patients with PD without constipation (71.9%), remembering (70.2%), urgency
dementia and 40 patients with PD with dementia and 50 (66.7%), and insomnia (66.7%). The most common domains
normal control subjects, and investigated differences of were: the urinary (77.2%), depression/anxiety (65.7%), and
hs-CRP among these 3 groups. sleep disorders (58.3%). The total number of NMS was
Result: Comparing the 3 groups, there was no significant correlated with HY stage but not with age and disease
difference between PD and PDD groups for mean hs-CRP duration. In women, the mean number of NMS per patient
values, but these two groups demonstrated significantly was significantly high than in men (15.2 vs. 12.3, p=0.017).
higher mean hs-CRP values than the control group. There was a marked difference in frequencies of sleep,
Conclusions: The pathogenesis of PD is currently unknown, depression/anxiety, and apathy/memory domains, which
but significant microglial inflammation is observed in the were significantly higher in women. We suggest that NMS
region of dopaminergic degeneration. Furthermore, it is is more prevalent in Korean PD patients compared with the
known that inflammation plays a role in the pathogenesis of results of other previous studies. Women might be more
PD and dementia. However, we suggest in our study that, vulnerable to NMS than men especially in sleep, depression/
although neuroinflammation plays a role in anxiety, and apathy/memory. Cultural differences might
neurodegenerative disease including PD and dementia, influence the frequencies or characteristics of NMS.
neuroinflammation did not contribute to the pathogenesis of
PD with dementia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


316 Posters, Sunday 9 September

P1587 P1588
The examination of relationships between The functional interhemispheric
voice abnormalities and the respiratory asymmetry, clinical asymmetry and
functions in patients with Parkinsons clinico-neuropsychological features in
disease patients with Parkinsons disease
H. Nakamura1,2, E. Sugawara1, M. Endou1, T. Takahashi1, M.A. Bykanova, N.V. Pizova
Y. Kuroiwa2 Yaroslavl State Medical Academy, Yaroslavl, Russia
1Neurology, National Hospital Organization Yokohama Purpose: To identify the relationship between clinical
Medical Center, 2Neurology, Yokohama City University, lateralization of Parkinsons disease, individual profile of
Yokohama, Japan functional interhemispheric asymmetry (IPFA) and their
Introduction: Parkinsons disease (PD) is well known to influence on the neurophysiological features of patients
exhibit voice impairments. The aim of this study is to with Parkinsons disease (PD).
evaluate objective changes in voice quality in patients with Methods and materials: To examine 70 patients (28 male
PD. and 42 female) from 1.5 to 3 stages of PD by using the
Methods: 32 PD patients (16 male and 16 female; mean following scales: UPDRS, HOEHN and YAHR. The IPFA
ageSD: 70.89.01; Hoehn and Yahr: 2.340.70; Unified was defined using the special questionnaire with 48 jobs. 32
Parkinsons Disease Rating Scale (UPDRS) part III: patients were examined using EEG study (Brain Loc).
15.038.87) and control group (14 male and 15 female; Results: Left-sided onset was defined significantly more
mean ageSD: 69.87.58) were assessed. The analysed frequently in patients with mixed and left IPFA than in
objective voice parameters were voice volume and patients with the right IPFA (p<0.05). Right-sided onset was
maximum phonation time (MPT). All patients in the PD observed significantly more frequently in patients with right
group were examined by respiratory function test (vital IPFA than in patients with mixed and left profile (p<0.05).
capacity and forced expiratory volume). Slow-wave activity in the form of generalized bilaterally
Results: There were significant differences in MPT which synchronous bursts was observed in 27 persons (84.3%).
was significantly shorter in the PD group than in the control The lateralization of abnormal slow-wave activity with a
group (12.84 seconds and 17.58 seconds, respectively) and lack of response activation was identified in 21 (65.6%)
in voice volume, which was significantly smaller in the PD patients. Generator of pathological activity in right-sided
group than in the control group (91.99 and 98.60dB, onset was detected predominantly in the left hemisphere,
respectively). In the PD group, %VC was 96.7716.13 and while left-sided debut - on the right hemisphere mainly at
FEV1% was 80.876.49. The correlations between the the thalamostrionigral structures. A clear lateralization EEG
UPDRS motor score and the MPT, and between the UPDRS activity was observed in 18 (85.7%) from 21 patients with
motor score and the voice volume were not significant at the predominant involvement in the pathological process of the
0.05 level. dominant hemisphere.
Conclusion: The voice of PD patients is shorter and smaller Conclusions: During the EEG study (Brain Loc) generator
than that of the control group. Voice abnormalities in PD of pathological slow wave activity was detected on the
patients are not correlated with respiratory function or opposite side of the affected limb, and a clear lateralization
motor function. was observed with primary lesion of the dominant
hemisphere.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 317

P1589 P1590
Clinical correlates of depression and Urinary and respiratory tract infections in
anxiety in patients with Parkinsons people with Parkinsons disease
disease A. Fisher1, J. English1, W. Gray2, G. Tan2, G. Idle2,
H. Tachibana1, T. Yamanishi1, M. Oguru1, K. Matsui1, R. Walker2
1Medical School, Newcastle University, Newcastle upon
M. Hashimoto1, K. Matsubara1, N. Takenaka1,
S. Matsutani1, T. Oku1, D. Danno1, K. Kawabata1, Tyne, 2Medicine, Northumbria Healthcare NHS Foundation
B. Okuda2, K. Toda3, N. Oka4 Trust, North Shields, UK
1Department of Internal Medicine, Hyogo College of Background: Bacterial infections can be a common
Medicine, Nishinomiya, 2Department of Neurology, Ehime complication in people with Parkinsons disease (PD).
Prefectural Central Hospital, Matsuyama, 3Toda Internal Respiratory and urinary tract infections can result from non-
Medicine-Rehabilitation Clinic, Akashi, 4Department of motor symptoms such as impaired swallowing and urinary
Rehabilitation, NHO South Kyoto Hospital, Jouyou, Japan dysfunction. We aimed to audit patients urine and sputum
Introduction: Although depression and anxiety are culture results to investigate how common such infections
important determinants of quality of life (QOL) in patients are.
with Parkinsons disease (PD), it is unclear whether Methods: Within Northumbria Healthcare NHS Foundation
depression and anxiety are associated with similar Trust, UK, microbiology archives were used to locate PD
demographic and clinical features. patients with positive growth on culture during the ten year
Objective: To clarify whether depression and anxiety are period from June 2001-June 2011. Organism was noted and
similar in terms of their associations with other clinical the case notes were reviewed for clinical details.
variables. Results: Of 643 eligible PD patients, only 12 (1.9%) had
Methods: 117 patients with PD participated in this study. positive sputum growths. Of those who had positive sputum
Patients were associated clinically using assessment samples 75% were male and 58% were in early stage of
instruments comprising both neurologist-administered disease (Hoehn and Yahr stage I). In contrast, there were
rating scales and self-administered questionnaires. The 101 (15.7%) positive urine cultures, although 48% were
former assessments were the modified Hoehn and Yahr asymptomatic. The most common urinary infections were
Scale (HY), the Unified Parkinsons Disease Rating Scale E. Coli (33%) and Coliforms (29%).
(UPDRS), and the Mini-Mental State Examination Conclusions: Despite the fact that pneumonia is a common
(MMSE). The latter scales included State-Trait Anxiety complication in people with PD, particularly in the later
Inventory (STAI), Beck Depression Inventory (BDI), an stage of disease, there were remarkably few positive sputum
apathy scale (AS), and 2 QOL instruments: the EuroQoL samples during the ten-year period. Interestingly, over half
(EQ-5D) and the 39-item Parkinsons disease questionnaire of the samples were in people with early stage of PD; this
(PDQ-39). may reflect the difficulty of those in later stage disease to
Results: Anxiety (STAI 41 for men or 42 for women) expectorate. Positive urine cultures were much more
was diagnosed in 52% of the patients and 55% were common, although this may reflect the relative ease of
diagnosed to have depression (BDI 14). 14% of the total obtaining a sample. Whether these findings reflect the
sample had anxiety without depression, whereas 17% had pattern of infection in people with PD is not clear.
depression without anxiety. Both STAI and BDI scores were Comparison with the general population is required.
negatively correlated with 2 QOL scales and positively
associated with AS score. BDI scores were associated with
age at onset, duration of illness, HY stage, and UPDRS
scores, whereas STAI scores were not associated with these
variables.
Conclusions: These findings suggest that anxiety and
depression may be separable in PD, although both are
common in patients with PD and are associated with QOL.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


318 Posters, Sunday 9 September

P1591 P1592
Epidemiology of restless legs syndrome in Ultrasonographic and clinical correlates
Croatia in patients with idiopathic Parkinsons
M. Relja1, V. Mileti2 disease: a prospective study
1Department Neurology, Medical School University of
K. Lauckaite1, D. Rastenyte1, D. Surkiene1, A. Vaitkus1,
Zagreb, 2Department Neurology, Zagreb University, Zagreb, R. Gleizniene2
Croatia Neurological, 2Radiological, Lithuanian University of
1

Introduction: Restless legs syndrome (RLS) is a common Health Sciences, Kaunas, Lithuania
and frequently underdiagnosed neurological disorder. RLS Introduction: Among the new techniques, transcranial
may be associated with sleep disturbances and may have a sonography (TCS) has been drawing attention as an easily
negative impact on quality of life. Reported prevalence accessible and inexpensive imaging method in Parkinsons
estimate of RLS have varied widely (from 2.5 to 29%). disease (PD), despite its value is still disputable.
Aims of study: To estimate the prevalence and to evaluate Methods: The PD patients for prospective TCS
the characteristics of RLS in Croatia. investigation were referred from the Neurological
Patients and methods: A cross-sectional survey included Department of the Hospital of Lithuanian University of
1432 randomly chosen subjects aged 18 to 81 years. A Health Sciences in 2011. A PA2-5 transducer on the
neurologist and/or family physician conducted interviews ultrasound system Voluson 730 Expert (GE Healthcare,
using a questionnaire based on diagnostic criteria developed Austria) was used for TCS. A total of 113 patients were
by the International Restless Legs Syndrome Study Group enrolled for further analysis, after excluding 19 (14.4%)
(IRLSSG). with bilateral temporal insufficiency. Diagnosis was
Results: A total of 245 (17.6%) examinees answered established according to the UK brain bank criteria. The
positively, fulfilling minimal criteria for RLS. Idiopathic clinical, demographic data, UPDRS-III, HAD, HamD scale
form of RLS was observed in 136 (55.5%) of 245 patients results were collected.
indicating the estimated prevalence of 9.5% in the Croatian Results: The mean age of PD patients (SD) was
population. Underlying conditions associated with 63.512.0yrs, UPDRS-III 12.85.4 points, and stage
secondary RLS were diabetes mellitus (50%), iron according to Hoehn-Yahr (H-Ys) 1.80.7. Tremor-dominant
deficiency (37.5%) and use of medications (11.5% - SSRIs, PD was diagnosed in 39 (34.5%), postural-instability-gait-
antidepressants). The prevalence was higher in women and disorder-dominant (PIGD) in 27 (23.9%), mixed type in 47
increased with age. (41.6%). The subgroups did not differ according to age, sex,
Conclusions: This is the first epidemiologic study of RLS H-Ys. The biggest substantia nigra (SN) plots were in PIGD
in Croatia showing the 9.5% prevalence of idiopathic RLS. (Kruskal-Wallis Chi2=46.9, p<0.001). The significant
Only 4.5% complained of severe symptoms that needed correlations were detected between diameter of the third
treatment. The results found in this study are similar to the ventricle (V3) and age (Spearman r=0.5, p<0.001), V3 and
findings of other European countries. H-Ys (r=0.2, p=0.05), HamD and H-Ys (r=0.6, p=0.003),
Supported by the Croatian Ministry of Science PD subtype and SN plot (r=0.3, p=0.002), H-Ys and SN
plot (r=0.3, p=0.004), UPDRS-III and SN plot on the left
(r=-0.6, p=0.028), H-Ys and nuclei raphe changes (r=-0.2,
p=0.049), H-Ys and lateral ventricles diameter (r=0.3,
p=0.023).
Conclusions: Multiple correlates between ultrasonographic
and clinical parameters were detected in PD patients, which
add valuable clinical information.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 319

P1593 P1594
Intravascular platelet activation in Total numbers of neurons and glial cells in
patients with hepatolenticular the basal ganglia of brains from patients
degeneration with multiple system atrophy
M. Melnikova1, S. Lobzin1, T. Fedorova1, E. Panina1, B. Haugen1, K. Winge2,3, L. Salvesen1,2, T. Agander1,4,
L. Vedyukova1, G. Sisoeva1, L. Tarkovskaya2, B. Pakkenberg1
T. Morozova2 1Research Laboratory for Stereology and Neuroscience,
1North-Western State Medical University named after I.I. 2Bispebjerg Movement Disorders Biobank, 3Department of
Mechnikov, 2Russian Research Institute of Hematology and Neurology, Bispebjerg University Hospital, Copenhagen,
4Department of Pathology, Roskilde University Hospital,
Transfusiology, Saint-Petersburg, Russia
Introduction: Hepatolenticular degeneration (HLD) is an Roskilde, Denmark
autosomal recessive inherited disorder of copper Introduction: MSA is a sporadic, progressive
metabolism, characterized by hepatic and neurological neurodegenerative disorder characterized by varying
dysfunction. Accumulated amounts of copper produce severity of parkinsonism, cerebellar ataxia and autonomic
angiotoxic and haemolytic effects, which can significantly failure. MSA is histopathologically characterized by glial
influence haemostasis. However, the platelet cytoplamic inclusions and selective neurodegeneration. The
morphofunctional characteristics in HLD were not details on the pathogenesis are still unknown.
investigated before. Material and methods: The material comprises 10 brains
Methods: Platelet count and intravascular platelet activation from patients with MSA and 10 control subjects. The total
(IPA) were evaluated in 48 patients with HLD. 23 patients number of neurons and glial cells in Substantia Nigra, the
were examined before chelating therapy and 25 on different Subthalamic Nucleus (StN) and the Red Nucleus (RN) of
stages of treatment. All patients were divided into groups on brains from patients with MSA were estimated using design
the base of the prevalence of hepatic or neurological based stereological methods providing quantitative data on
manifestations, and different severity of hepatic impairment. brains affected by a neuronal loss. Data were analyzed using
Severity of the disease was evaluated by the Unified Sigma Stat 11.0, Systat Software Inc., San Jose, California,
Wilsons Disease Rating Scale. A control group included 25 USA.
normal individuals. Results: The MSA brains were estimated to have about
Results: All patients had thrombocytopenia and significant 40% fewer pigmented neurons in Substantia Nigra
increase of IPA parameters: total amount of active forms of (40.1*104 versus 91.8*104, p0.001). In the Subthalamic
platelets and number of platelets, involved in aggregation. Nucleus and the Red Nucleus the total number of neurons,
Severity of the disease and degree of hepatic impairment oligodendrocytes and astrocytes were the same in the two
influenced only the platelet count, but not IPA. Level of groups, whereas microglia were more abundant in the MSA
cytolysis and cholestasis did not influence IPA (p<0.05). In brains in both StN (20.4*104 versus 82.6*103, p=0.026)
patients receiving chelating therapy parameters of IPA were and RN (10.2*104 versus 33, 1*104, p0.001).
reduced significantly, but remained higher than in controls. Conclusion: In terms of cell numbers the Subthalamic
Conclusion: In HLD patients, activation of platelet Nucleus and the Red Nucleus are not severely affected in
haemostasis is observed. Parameters of IPA remain high patients with MSA compared to the control subjects. The
during the chelating therapy. Impairment of morphological increased microglia level could indicate inflammation in
and functional properties of platelets contributes to severity MSA. The high loss of neurons in Substantia Nigra supports
of chronic brain and liver diseases and requires treatment. the neurodegenerative nature of MSA.
Thus, some additional factors can modify the activation and
aggregation of platelets in HLD and be the goal for further
research.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


320 Posters, Sunday 9 September

P1595 P1596
A case of orthostatic tremor associated Study of osteoprotegerin concentrations
with Graves disease and level of 25(OH)D in blood serum of
S. Mazzucchi, D. Frosini, M. Giuntini, E. Del Prete, patients with Parkinsons disease,
R. Ceravolo ischemic stroke and other neurological
Department of Neuroscience, University of Pisa, Pisa, Italy diseases
Introduction: Orthostatic tremor (OT) is a rare disorder U. Fiszer1, M. Piacik-Gromada1, B. Parafinuk2,
characterized by high frequency lower limbs tremor and G. Korczak-Kowalska3, K. Bocian3, T. Szatanowski1
unsteadiness on standing with remission during sitting or Medical Center of Postgraduate Education, 2The Childrens
1
lying. Memorial Health Institute, 3University of Warsaw, Poland
A 70-year-old woman presented with a sense of unsteadiness
and tremor in her legs mainly during standing and walking, Osteoporosis and decrease of the level of vitamin 25(OH)D
which gradually worsened over a year without any benefit was described in patients with PD. Concentration of
after clonazepam. Neurological exam showed tetra osteoprotegerin (OPG) has been associated with the risk of
hyperreflexia, paraparetic gait and inability to perform osteoporosis and vascular pathology, however, it has not yet
tandem gait. She also reported intolerance of heat, excessive been examined in PD patients.
sweating and recent loss of weight with increased appetite, Methods: The study group included 95 patients, within this
dysphagia and dysphonia. group 45 suffered from PD. A - with initial PD (20 persons),
Methods: Electromyographic recordings from vastus B - with advanced PD >5 years (25 persons), C - with stroke
medialis and tibialis anterioris performed during standing (30 persons), D - with other neurological disease (OND) (20
showed a rhythmic contractile activity at about 8Hz. After persons). Osteoprotegerin concentration measured by the
prolonged supine rest, a rhythmic contractile activity at sandwich ELISA, and the level of 25(OH)D in blood serum
lower amplitude re-appeared in both lower legs. Due to was obtained by the automatic chemiluminescence method.
atypical clinical and EMG activity and low frequency of Results: Osteoprotegerin concentration in serum was
tremor, a symptomatic origin was hypothized. Brain and (mean): A - 5.50pg/ml, B - 6.80pg/ml, C - 7.59pg/ml, D -
cervico-dorsal MRI and motor evoked potentials were 5.68pg/ml. The results demonstrate an increased
normal. Her thyroid function exams revealed severe osteoprotegerin concentration in stroke patients (C v A, U
hyperthyroidism (thyroid stimulating hormone (TSH) Mann Whitney test p<0.03). The mean level of 25(OH)D in
<0.004U/ml [0.400-4.000U/ml], fT3 9.25pg/ml [1.80- groups was: A - 15.45ng/ml, B - 13.80ng/ml, C - 11.12ng/
4.80pg/ml], fT4 5.05ng/dl [0.80-1.80ng/dl]) and positivity ml, D - 17.48ng/ml. Normal level of 25(OH)D in adults is
of TSH receptor antibodies. A chest and neck CT scan between 30 and 80ng/ml. Results showed decrease of level
revealed an increase in thyroid size involving the upper of 25(OH)D in patients with initial and advanced PD. It was
mediastinum and affecting the left side of the oesophagus. also seen in patients with OND, and particularly with
Result: A diagnosis of Graves disease was performed and ischemic stroke (C v D, p<0.03, t-Student test).
she was started on Carbimazole with dramatic improvement Conclusion: No increase of OPG in initial PD was found,
of tremor. as opposed to stroke patients. The results also showed
Conclusion: Graves syndrome has been recently described insufficient supply of vitamin D in patients with different
as a possible cause of OT, and should be taken into account neurological diseases.
mainly when OT presents with atypical phenotype.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 321

P1597 P1598
Chronic pain in Parkinsons disease - Motor phenotype of LRRK2-associated
characteristics and association with bone Parkinsons disease: a longitudinal study
mineral density F. Nabli, S. Ben Sassi, E. Hentati, I. Lakhdhar, H. Nahdi,
H.-J. Jung1, S.-M. Choi1, E.-S. Park2, S.-Y. Lee2, R. Amouri, F. Hentati
B.-H. Cho2, B.-C. Kim2, M.-K. Kim2, M.-S. Park2, National Institute of Neurology, Tunis, Tunisia
S.-H. Lee2 Background and aims: Mutations in the Leucine-rich
Neurology, Chonnam National University Hwasun Hospital,
1
repeat kinase 2 gene (LRRK2) showed to be a significant
Hwasun-gun, 2Neurology, Chonnam National University cause of autosomal dominant forms of Parkinsons disease
Hospital, Gwangju, Republic of Korea (PD). In order to determine the motor characteristics of
Background and purpose: Chronic pain and low bone LRRK2-related disease, we conducted a longitudinal study
mineral density (BMD) in Parkinsons disease (PD) are of 58 LRRK2-associated PD patients and compared them
increasingly recognized as a major cause of reduced health- with idiopathic PD patients.
related quality of life. But, there have been few studies Patients and methods: 58 patients diagnosed with
concerning chronic pain and relationship between PD-related LRRK2 G2019S mutation were included in the
osteoporosis in PD, even though patients with PD have a study and compared to 59 sporadic PD patients with
high incidence of falls. The aim of this study was to negative tests for LRRK2 G2019S, PINK1, SNCA, PRKN
investigate the prevalence and the characteristics of chronic et DJ1 mutations. Patients were assessed at baseline and
pain and its associations with BMD in patients with PD. after a follow-up period of six years. Collected data included
Patients and methods: 91 patients diagnosed with the Movement Disorder Society-unified Parkinsons
idiopathic PD were interviewed with semi-structured pain Disease rating scale (MDS-UPDRS), the Hoehn and Yahr
questionnaire. The severity of PD judged using Unified PD stage and the Schwab and England scale.
Rating Scale, Hoehn and Yahr staging. BMD in the lumbar Results: The LRRK2-associated PD patients had a mean
spine and proximal femur were evaluated by dual X-ray age at baseline of 68.4511.82 years, a mean age of onset
absorptiometry. Patients were divided into three groups of 56.2512.05 years and in most cases (61%) a PIGD
according to chronic pain, acute pain and no pain. We then phenotype. The mean annual decline in the UDRS motor
investigated the clinical correlation between severity of PD score and the Hoehn and Yahr staging were of 1.3% and
and BMD. 2%, respectively. Motor severity correlated with disease
Results: 73 (80.2%) out of 91 patients reported pain and 68 duration and PIGD phenotype, and tremor dominant
(74.7%) patients had chronic pain. Patients with chronic phenotype predicted slower progression of motor
pain were more prevalent in females (p=0.02) and old age impairment. PD motor phenotype and motor scores were
(p=0.042) and higher UPDRS II (p=0.016), NMSAS similar in the LRRK2-associated PD group and in the
(p=0.028), and the Gait and Falls questionnaire (p=0.01). idiopathic PD group with no significant differences in the
BMD in patients with chronic pain were significantly lower progression rate of motor impairment.
femur BMD. Conclusion: Motor phenotype seems to be similar in
Conclusions: Pain is common in patients with PD and LRRK2-related PD and idiopathic PD
associated with poor ADL, higher frequency of falling and
lower BMD. Patients with chronic pain should be carefully
examined and screened for osteoporosis to prevent bone
loss and associated disability due to falling.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


322 Posters, Sunday 9 September

P1599 P1600
Is the nigrostriatal dysfunction a possible Glucocerebrosidase L444P mutation
predictive marker of visual hallucinations confers a genetic risk factor for
in Parkinsons disease? Evidence from a Parkinsons disease in central China
123I-FP-CIT SPECT study N. Xiong, Y. Wang, C. Chen, J. Huang, T. Wang
L. Kiferle, R. Ceravolo, V. Nicoletti, P. De Feo, Department of Neurology, Union Hospital, Tongji Medical
D. Volterrani, U. Bonuccelli College, Huazhong University of Science and Technology,
Department of Neuroscience, University of Pisa, Italy Wuhan, China
Introduction: The pathogenesis of visual hallucinations Introduction: Mutations of the glucocerebrosidase (GBA)
(VH) in Parkinsons disease (PD) has been considered gene have been reportedly associated with various ethnic
multifactorial. In the pathophysiology of VH, a combination Parkinsons disease (PD) populations. However, whether or
of impaired visual processing and attention was reported. not there is any association of different sequence variants of
Imaging studies evidenced a role of the primary visual GBA with PD in central China remains unknown. The
system and visual association areas as well as a dysfunctional agriculture-related central China area should be drawn
activation of frontal areas. Moreover, longitudinal particular attention to, as recent epidemiological studies
neuropsychological studies showed an association of VH suggest an association of environmental toxins such as
with baseline frontal dysfunction, suggesting verbal fluency rotenone and other pesticides with the higher incidence of
as a potential predictor of VH within 2 years. Due to the sporadic PD in rural areas.
functional connections between basal ganglia and frontal Methods: Here, we conducted a case control study in a
areas, a role of basal ganglia and of the frontostriatal circuits cohort of 208 central Chinese PD patients and 298 control
in the pathogenesis of VH may be postulated. subjects for three GBA mutations (L444P, N370S and
Objective: To unveil whether a worse nigrostriatal R120W) by Polymerase chain reaction-restriction fragment
dysfunction at baseline may predict the development of VH length polymorphism and DNA sequence.
in PD. Results: Our data suggested a significant higher frequency
Methods: 16 non-demented VHPD and 14 non-demented of L444P mutation in GBA gene of PD cases (3.4%)
non-VHPD patients, matched for age of onset of disease, compared with the controls (0.3%) (p=0.007, OR=10.34,
disease duration and severity, levodopa equivalent dose, 95% CI=1.26-84.71). As for gender, there was no significant
underwent a 123I-FP-CIT SPECT at disease onset. Striatal difference between mutation carriers and non-carriers.
uptake values in the two groups were investigated. Additionally, the average age at onset of PD carriers showed
Results: The group of VH had a significant reduction no significant difference between carriers and non-carriers.
(p<0.05) of right caudate uptake values at baseline when Specifically, the frequency of L444P mutation was higher in
compared with non-VHPD. No significant differences were late onset PD (LOPD) cases compared with that in control
present between groups in left caudate and putaminal uptake subjects. The N370S and R120W mutations experienced no
values. difference between the PD group and the control group.
Conclusions: The frontal impairment reported in VHPD Conclusions: Our observations demonstrated that the GBA
may be due to a right caudate dysfunction, as it is connected L444P mutation confers a potential risk factor for PD,
to the frontal brain areas via neuronal loops. Our data could especially LOPD, in the central China area.
suggest the early caudate dysfunction as a potential
predictive marker of VH in PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 323

P1601 P1602
Is the cold hands sign useful in Reasons for discontinuation of botulinum-
differentiating multiple system atrophy toxin treatment of focal hand dystonia
from Parkinsons disease? M. Balaz1,2, T. Gajda3, M. Bares1,2
1Department of Neurology, Masaryk University, St Annes
M. Asahina1, D.A. Low2, C.J. Mathias2, Y. Fujinuma1,
A. Katagiri1, Y. Yamanaka1, J. Shimada1, A. Poudel1, University Hospital, 2CEITEC - Central European Institute
S. Kuwabara1 of Technology, MU Brno, 3Medical Faculty, Masaryk
1Neurology, Chiba University School of Medicine, Chiba, University, Brno, Czech Republic
Japan, 2Autonomic & Neurovascular Medicine Unit, Objective: The goal of the study was to assess the reasons
Imperial College London at St. Marys Hospital, London, for discontinuation of long-term treatment of writers cramp
UK in patients treated with botulinum toxin (BTX). We also
Aim: A previous study on a small number of patients observed the long-term BTX treatment outcomes.
showed that low skin temperature of the hands with skin Introduction: Botulinum toxin A is considered to be an
colour changes, the so-called "cold hands sign", may be effective treatment for the patients suffering from focal
useful for distinguishing multiple system atrophy (MSA) hand dystonia (such as writers cramp) but its long-term
from Parkinsons disease (PD). Therefore, low skin usefulness may be limited by frequent discontinuation of
temperature of the hand may be a characteristic of MSA the treatment for various reasons.
rather than PD. We evaluated the skin colour and Methods: We reviewed the records of 54 patients who had
temperature of the hand in a larger number of MSA and PD more than 400 applications of BTX. We also conducted
patients. surveys via mail or telephone interviews with the patients
Methods: Skin temperature on the centre of the palm was who were lost to regular follow-up.
measured in 50 MSA patients (age, 64.46.0 years; duration, Results: Each patient had at least 2 application sessions,
2.91.7 years), 50 PD patients (67.57.7 years; 8.17.6 median treatment duration was 82 months. 83% of treated
years), and 25 healthy subjects (age, 64.711.4 years). All patients reported at least a partial improvement of their
participants were Japanese by ethnicity. condition. There were no serious adverse events. Most
Results: Temperatures of <28C were observed in 3 MSA frequent side effect was the weakness of muscles adjacent
patients (6%) and none of the PD patients and controls. We to the injection site. Altogether 30 (55%) patients chose to
could not find any skin colour changes on the hand, such as discontinue the treatment.
dusky and violaceous, in any participants. Palm skin Conclusions: A high proportion of the patients chose to
temperature was significantly lower in MSA patients discontinue the treatment with BTX. Main reasons for
(32.02.7C) than in controls (34.10.9C, p=0.0002), but discontinuation were side effects (33%), low efficacy
was not different compared with the PD group (32.91.8C, (30%), relocation (6.5%), change of writing habits (6.5%),
p=0.06). There was no significant difference between the new symptoms and change of treatment (6.5%), other and
PD and control groups. unknown reasons (17.5%). While BTX remains an effective
Conclusions: Measurement of skin temperature is easy and treatment, drop-out rate may be higher than in other focal
non-invasive and the cold hands (<28C) sign may be dystonias, especially due to the side effects and subjectively
useful for distinguishing MSA from PD. However, the assessed low efficacy in some patients.
sensitivity appears to be low.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


324 Posters, Sunday 9 September

P1603 P1604
Association between falls and urinary Pain and its clinical types in Parkinsons
disturbance in Parkinsons disease disease
K. Sakushima1, S. Yamazaki2, Y. Hayashino2, J. Harsany1, M. Hanakova1, P. Valkovic1,2
S. Fukuhara2, I. Yabe1, H. Sasaki1 1Second Department of Neurology, School of Medicine,
1Department of Neurology, Hokkaido University Graduate Comenius University, 2Institute of Normal and Pathological
School of Medicine, Sapporo, 2Department of Healthcare Physiology, Slovak Academy of Sciences, Bratislava, Slovak
Epidemiology, Graduate School of Medicine and Public Republic
Health, Kyoto, Japan Introduction: Pain is an important and distressing symptom
Introduction: Falling is one of the most common and in Parkinsons disease (PD). A frequently overlooked
serious incidents among public health problems. It can clinical feature of PD, pain may be severe enough to
cause injuries such as sprains and fractures. Hospitalization overshadow the motor symptoms of the disorder. Aim of
may be required when it is a serious injury. Patients with this study was to determine frequency and types of pain in
Parkinsons disease have a higher risk of falls. Urinary patients with PD. Moreover, we correlated pain
incontinence is also known as a risk factor of falls for the characteristics with demographic data, quality of life, and
elderly. However, the risk of falling associated with other depression.
symptoms of urinary disturbance is unclear. The purpose of Methods: This is a single-centre cross-sectional study. 35
this study is to identify the association between falls and consecutive subjects with PD participated. Demographic
urinary disturbance in Parkinsons disease. data, severity and types of pain were assessed using
Methods: A prospective cohort study at a single institution structured questionnaire and Beck Depression Inventory,
with six months observation period was conducted. Subjects respectively. Pain was divided based on Fords classification
were ambulatory patients with Parkinsons disease. (Mov Disord. 2010;25 Suppl 1:S98-103): musculoskeletal,
Assessment included patient demographics, disease severity dystonic, central neuropathic, low back/radicular, and not-
measured by Hoehn and Yahr Scale, and urinary disturbance classified pain. Severity of average pain during the last
measured by overactive bladder symptom score (OABSS). week was rated on 10-point visual analogous scale.
Falls were reported by a self-documented falls record. Results: In our set of PD subjects, the presence of pain
Results: A total of 86 patients (33 males, 53 females) were types was as follows: musculoskeletal pain 34.3%, dystonic
included. Mean age was 71.07.5. Hoehn and Yahr Scale pain 8.6%, central parkinsonian pain 17.1%, low back /
was 2.61.1. 39 patients (45%) had at least one fall (faller) radicular 54.3%, and not-classified pain 14.3%. No pain
and 28 patients (33%) had two or more falls in six months. referred 20% of patients, one type 48.8%, two types 20.0%,
In OABSS, urinary urgency and urgency incontinence were three types, 5.7%, and four types 5.7%. A significant
more common in fallers than in non-fallers (55% vs. 30% correlation was found between PD duration and number of
and 58% vs. 35%, respectively). However, daytime pain types (rho=0.53018, p=0.0011), and number of pain
frequency and night-time frequency is not associated with types and average pain severity (rho=0.63984, p<0.0001)
falls. Conclusion: Pain in PD is present in 80% of patients. The
Conclusion: Falls in patients with Parkinsons disease were most frequent type of pain is the low/back radicular pain.
not associated with urinary frequency but associate with Our results stress the importance of pain type determination
urinary urgency and incontinence. because of different treatment approaches.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 325

P1605 P1606
Non-motor symptoms in Parkinsons Defective iron-handling and altered
disease: a correlation with depression cellular oxidative status in skin fibroblasts
and quality of life from pantothenate kinase associated
M. Hanakova1, J. Harsany1, P. Valkovic1,2 neurodegeneration patients
1Second Department of Neurology, School of Medicine,
S. Levi1,2, A. Campanella1, D. Privitera1, M. Guaraldo1,
Comenius University, 2Institute of Normal and Pathological C. Barzaghi3, A. Cozzi2, P. Santambrogio2, B. Garavaglia3
Physiology, Slovak Academy of Sciences, Bratislava, Slovak 1Vita-Salute San Raffaele University, 2Division of
Republic Neuroscience, San Raffaele Scientific Institute, 3Fondazione
Introduction: Non-motor symptoms (NMS) of Parkinsons IRCCS-Istituto Neurologico Carlo Besta, Milan, Italy
disease (PD) are not well recognized in clinical practice, Introduction: Pantothenate Kinase Associated
and are frequently missed during routine. Aim of this study Neurodegeneration (PKAN) is the most prevalent form of
was to determine frequency and severity of NMS in patients neurodegeneration involving iron accumulation in the brain.
with PD. Moreover, we correlated NMS scores with It is characterized by progressive impairments in movement,
demographic data, quality of life, and depression. speech and cognition. The disease is inherited in a recessive
Methods: This is a single-centre cross-sectional study. manner due to mutations in the Pantothenate Kinase-2
NMS severity was determined by using the Non-Motor (PANK2) gene that encodes a mitochondrial protein
Symptom assessment scale for PD (NMSS; Chaudhuri KR, involved in Co-enzyme A synthesis.
et al. Mov Disord. 2007;22(13):1901-11.) and self- Aim: To elucidate the molecular mechanism leading to iron
completed NMS-Questionnaire for PD ( the NMSQuest; homeostasis dysfunction in Pank2 gene defect cells.
Chaudhuri KR, et al. Mov Disord. 2006;21(7):916-23.). Patients and methods: We analyzed, in primary skin
Quality of life was assessed by PDQ-8 questionnaire, and fibroblasts from three PKAN patients and three unaffected
depression was scored by means of Beck Depression subjects, the oxidative status and their ability to respond to
Inventory (BDI). iron.
Results: Data from 35 consecutive patients, mean age Results: In basal conditions, PKAN fibroblasts show an
65.27.8 years, duration of disease 5.54.5 years, and all increase in carbonylated proteins and altered expression of
stages of PD were collected. The mean NMSS score was antioxidant enzymes with respect to the controls. After iron
39.624.6 (range: 9-110). The mean NMSQuest score was supplementation, the PKAN fibroblasts had a defective
10.54.7 (range: 2-22). We found significant correlation response to the additional iron. Under these conditions,
between NMSS score and NMS-Quest score (rho 0.71; ferritins were up-regulated and Transferrin Receptor 1
p<0.0001), NMSQuest and duration of PD (rho=0.55, (TfR1) was down-regulated to a minor extent in patients
p=0.0007), NMS-Quest and BDI (rho 0.69; p<0.0001), compared to the controls. Analysis of Iron Regulatory
NMSS and BDI (rho 0.56; p=0.0005), and PDQ-8 score and Proteins (IRPs) reveals that, with respect to the controls,
BDI (rho=0.51, p=0.0017) . PKAN fibroblasts have a reduced amount of membrane-
Conclusion: NMS are significantly frequent across all associated mRNA-bound IRP1, which responds imperfectly
stages of PD. This study proves the efficiency of screening to iron. This accounts for the defective expression of ferritin
questionnaire the NMS-Quest, as well as of NMSS. Scores and TfR1 in patients cells. The inaccurate quantity of these
of both instruments highly correlate. Moreover, NMS proteins produced a higher bioactive labile iron pool and
correlate with depression that correlates with quality of life consequently increased iron-dependent ROS formation.
in PD. Our results stress the importance for active detection Conclusions: Our results suggest that Pank2 deficiency
of NMS in PD patients. promotes an increased oxidative status that is further
enhanced by the addition of iron, potentially causing
damage in cells.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


326 Posters, Sunday 9 September

P1607 P1608
Fatigue in Parkinsons disease Montreal cognitive assessment as a new
T. Torgan screening tool for dementia in
Perm State Medical Academy, Perm, Russia Huntingtons disease: a validation study
Introduction: It is not clear whether fatigue in patients with P. Moskal1, K. Banaszkiewicz2, E.M. Klimiec1,
Parkinsons disease is the primary or the secondary A. Starowicz3, M. Blaz1, A. Szczudlik1
symptom. 1Department of Neurology, Jagiellonian University Medical

Objective: To investigate whether fatigue is associated with College, 2Krakowska Akademia Neurologii, 3Department of
depression, autonomic disturbances, the severity of Medical Psychology, Jagiellonian University Medical
movement disorders and sleep disturbance in patients with College, Krakow, Poland
Parkinsons disease. Background and aims: Cognitive disturbances are often
Methods: The Unified Parkinsons Disease Rating Scale, the first manifestation of Huntingtons disease (HD) and are
Hoehn and Yahr scale, the Multidimensional Fatigue an important factor contributing to patients disability.
Inventory (MFI-20), the Hospital Anxiety and Depression Neuropsychological assessment is the most relevant, but
Scale (HADS), the Epworth Sleepiness Scale (ESS), Veyn time consuming, method of cognitive evaluation. The
Questionnaire for autonomic disturbances were used. Montreal Cognitive Assessment (MoCA) is a new screening
Results: 55 patients (16 males, 39 female) with Parkinsons instrument which detects early cognitive impairment.
disease without dementia were studied. Mean age was 64 However, MoCA has not been compared with
(60-70) years, the duration of the disease was 5 (2-5) years. neuropsychological tests in HD. The aim of this study was
Patients received different dopaminergic therapy and were to find a cut-off for MoCA that sensitively screens for
examined in off period. The control group consisted of 19 dementia in HD patients.
persons of the same age and sex. The mean rate of fatigue Patients and methods: 22 HD patients were examined with
in patients with Parkinsons disease (37; 25-46 points) was MoCA and a battery of neuropsychological tests which
higher (=0.000) than in the control group (15; 9-19 points). assessed 5 cognitive domains: Stroop Test and Perception
It did not depend on sex and age of the patients. 38 patients and Attention Test for attention; Benton Visual Retention
had more than 36 points on MFI-20. The rate of fatigue in Test for memory; Trail Making Test, Wisconsin Card
this group did not correlate with th duration (R=-0,11, Sorting Test and Similarities from Wechsler Adult
p=0.644) and the severity of the disease (R=0.23, =0.308), Intelligence Scale (WAIS) for executive functions; Verbal
the severity of motor disorders (R=0.11, p=0.642) and Fluency Test for language; Block Design from WAIS for
depression (R=-0.02, p=0.924). The autonomic (R=-0.41, visuospatial abilities. Dementia was defined as an
p=0.070) and sleep disturbances (R=0.02, p=0.093) did not impairment in at least two cognitive domains. Receiver
influence the fatigue. operating characteristics analysis was used to find a
Conclusion: Fatigue in patients with Parkinsons disease is sensitive cut-off in MoCA to diagnose dementia.
not the secondary symptom due to depression or sleep Results: According to neuropsychological assessment 15
disturbances, but is a primary phenomenon with its own patients had dementia. The most sensitive MoCA cut-off
pathogenesis. score was 26, with 100% sensitivity and 71% specificity.
Another 2 patients were impaired in only one cognitive
domain and scored 26 on MoCA, the other 5 patients
without any cognitive disturbances scored >26.
Conclusion: MoCA cut-off score 26 allows for sensitive
screening for dementia in HD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 327

P1609 P1610
Spasticity and "spastic" gait in children The impact of non-motor symptoms on
with hereditary spastic paraplegias health-related quality of life in patients
I.U. Isaias1,2, A. Marzegan1, E. Todeschini1, P. Cavallari1, with atypical parkinsonism
J. Volkmann2, P. Crenna1 S.-B. Koh, C.-N. Lee
1Dipartimento di Fisiologia Umana, Laboratorio per Neurology, Korea University College of Medicine, Seoul,
lAnalisi del Movimento (LAMB) P. & L. Mariani, Universit Republic of Korea
degli Studi di Milano, Italy, 2Neurologische Klinik und
Background: Atypical parkinsonism is less common and
Poliklinik, Universittsklinik Wrzburg, Germany
has more severe symptoms than Parkinsons disease.
Introduction: Hereditary spastic paraplegias (HSPs) are a Relatively little is known about the characteristics of non-
clinically and genetically heterogeneous group of conditions motor symptoms (NMS), which could affect to health
characterized by the presence of lower limb spasticity and related quality of life (QoL) in multiple systemic atrophy
pyramidal weakness. We aimed to address spastic muscle (MSA) and progressive supranuclear palsy (PSP). We report
behaviour under dynamic conditions (unperturbed the characteristics of NMS and the impact of NMS on
overground walking) in children with HSP. health-related QoL of MSA and PSP.
Patients and methods: 10 children (3 male; age range: Methods: Out of 58 patients with a diagnosis of atypical
4-13 years) were enrolled in the study and evaluated parkinsonism, 32 patients had multiple systemic atrophy
clinically and by means of brain MRI, BAER, SSEP and parkinsonian subtype (MSA-P), 14 patients had multiple
MEP. Spastic muscle (soleus) behaviour was quantitatively systemic atrophy cerebellar dysfunction subtype (MSA-C),
characterized by the level of current EMG activity as a 12 patients had PSP. We assessed the Unified Parkinsons
function of the muscle lengthening velocity (muscle Disease Rating Scale part III (UPDRS-III), the modified
kinematics was estimated by geometrical models). Hoehn & Yahr scale (H&Y), the Parkinsons Disease
Results: Four different patterns of spastic muscle behaviour Questionnaire (PDQ-39) and the non-motor symptom scale
were described. Pattern-I was defined by the presence of (NMSS).
hyper-synchronous activity in the post heel-contact phase Results: There were no differences of sex ratio, disease
with equal or higher amplitude with respect to the following duration, UPDRS-III score, and H&Y stage in the three
push-off phase (and no activity pause between these two groups. In the MSA-P, MSA-C and PSP group, PDQ-39
phases). We classified pattern-II or -III when the hyper- scores are correlated with NMSS scores, not UPDRS-III
synchronous activity in the post heel-contact phase (as in scores. Concerning the correlations of domains in NMSS
pattern-I) was paused by Post Synchronous Discharge scores and PDQ-39 scores, PDQ-39 scores of MSA-P group
Silent Periods (PSDSPs) >70ms (pattern-III if PSDSPs are correlated with domains of mood/cognition,
were >3). Pattern-IV was characterized by hyper- cardiovascular inducing falls, and urinary dysfunction. In
synchronous activity also in the swing phase. Each child MSA-C group, PDQ-39 scores are correlated with domains
showed a different combination of these patterns. Overall, of sleep/fatigue, mood/cognition, urinary dysfunction. In
pattern-II was the most frequent (30%) followed by pattern-I PSP group, PDQ-39 scores are correlated with domain of
(14.5%) and pattern-III and -IV (< 10%). mood/cognition.
Conclusion: A dynamic evaluation allowed a detailed Conclusion: Non-motor symptoms are associated with QoL
profiling of spasticity in children with HSP. This might be in the three groups, and the mood/cognition domain is a
useful to determine functional severity of lower limb very important part of NMS. When we treat MSA, PSP
spasticity at follow-ups and for proper medical treatment. patients, we must consider non-motor symptoms, especially
mood/cognition.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


328 Posters, Sunday 9 September

P1611 P1612
White matter lesions and depression in MAPT rs242562 and GSK3 rs334558 are
patients with Parkinsons disease associated with Parkinsons disease in a
V. Markovi1, I. Petrovi1, E. Stefanova1, D. Kozic2, central Chinese cohort
R. Semnic2, V. Kosti1 N. Xiong, L. Yu, J. Xiong, J. Huang, T. Wang
1Movement Disorders Department, Neurology Clinic Clinical Department of Neurology, Union Hospital, Tongji Medical
Center of Serbia, Belgrade, 2Diagnostic Imaging Center, College, Huazhong University of Science and Technology,
Institute of Oncology, Sremska Kamenica, Serbia Wuhan, China
Introduction: The incidence of Parkinsons disease (PD) Background: MAPT is a neuronal protein involved in the
increases with age, and white matter changes (WMHs) pathogenesis of several neurodegenerative diseases
present as signal hyperintesities on T2W MRI studies are including PD. GSK3 catalyzes phosphorylation in multiple
commonly observed in older adults. Severity of deep sites of tau protein. These results have driven out a research
WMHs was also correlated with depression. hotspot which is about the gene-gene interaction between
Objective: To investigate whether WMHs were associated MAPT gene and GSK3 gene and their respective links in
with depression in PD patients. several diseases. Previous data have indicated that the
Methods: We included 34 patients with (PD-D) and 25 genetic alteration of GSK3 and its interaction with MAPT
without depression (PD-nD) with PD onset above 60yrs, haplotype are collectively related to PD morbidity rate in a
and 30 healthy controls. Diagnosis of depression was Greek cohort. However, the association among the GSK3
established via Structured Clinical Interview for DSM-IV gene alteration, MAPT haplotype and PD have not been
Axis I disorders and quantified using Hamilton Depression explored previously in the Chinese population.
Rating Scale (HDRS). Patients underwent brain 1.5-T MRI. Methods: Here, we performed a case-control association
WMHs were rated using T2W images and classified using study in a Chinese cohort of 211 PD patients and 279
the semiquantitative visual rating scale of Scheltens. matched controls from central China by Polymerase chain
Results: Comparing controls and PD patients as a group reaction-restriction fragment length polymorphism.
there were no differences in WMHs in any examined region. Results: Our data showed that allele frequency of MAPT
However, PD-D group had significantly higher WMHs rs242562 G/G polymorphism was significant higher in
scores for total deep WM and BG regions when compared controls than that in PD patients while G/A genotype of
to controls as well as for frontal deep WM and total MAPT rs242562 revealed higher frequency in PD subjects
periventricular regions WMHs comparing with the PD-nD compared to controls. The genotype frequency of GSK3
group. There were no differences in WMHs between PD-nD rs334558 C/C was over-represented in matched female
patients and controls in any of the examined regions. The controls compared to female PD patients. Compared with
multivariate linear regression analysis was carried out with female PD patients, the genotype frequency of GSK3
HDRS score as a dependent variable and lobar (frontal, rs334558 T/T polymorphism was higher in male PD
temporal, parietal, occipital), periventricular and BG patients.
WMHs, age, education and cerebrovascular risk factors as Conclusions: We conclude that G/G genotype of MAPT
independent variables. Significance was only shown for rs242562 and C/C genotype of GSK3 rs334558 are
periventricular WMHs total score (p=0.04), explaining the associated with PD and may serve as protective genetic
39% of the variance in the HDRS. factor for PD in the central Chinese population.
Conclusion: Our study confirms the role of WMHs in
depression associated with PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 329

P1613 P1614
The mechanisms of movement control Predictors of quality of life of patients
and time estimation in cervical dystonia with Parkinsons disease
patients: a behavioural study M. Gomez-Gallego, S. Oliveira, M. Leon, C. Albert
P. Filip1,2, I. Husrov1, M. Bare1,2 Catholic University of Murcia, Spain
1First Department of Neurology, Faculty of Medicine Introduction: Current treatments for Parkinsons disease
Masaryk University and St. Annes Teaching Hospital, are mainly symptomatic and aimed at improving patients
2Central European Institute of Technology, CEITEC MU,
quality of life (QoL). The objective of this study is to
Brno, Czech Republic determine which clinical factors are the most relevant for
Introduction: The pathophysiology of cervical dystonia is patients QoL.
still relatively unknown. The functional imbalance of Methods: 39 PD patients were administered the QoL scale
respective muscle groups is thought to be caused by PDQ-39. A complete neurological examination, including
neurochemical abnormities in basal ganglia and recently, the Hoehn & Yahr Scale (H-Y), the Barthel Index (BI), and
there has been emerging substantial evidence of considerable both part I and part III of Unified Parkinsons Disease
cerebellar involvement. This project aims to affirm the Rating Scale (UPDRS) was performed. The associations
function of the above mentioned structures in precise between sociodemographic and clinical variables (years of
movement timing. disease, treatment, H-Y stage, BI and UPDRS domains
Methods: The participants were asked to perform a series scores) with PDQ-39 domains were tested using ANOVA
of simple computer tasks where the parameters of a moving tests and correlational analyses. Multiple regression
object (speed, acceleration, partially the movement analyses were carried out for each domain of PDQ-39.
direction) were generated randomly. As the object moved Results: PDQ-39 total score was significantly associated
on the computer screen from left to right, the participant had with marital status, UPDRS part I score, BI, postural tremor
to press a button in an optimal time window to launch a score, axial function score, and both left and right
fireball from the bottom of the screen that was supposed bradykinesia scores. BI and UPDRS part I scores explained
to hit the moving target. The fireball travelled with a 63% of the model variance. Higher BI scores and taking
constant speed and trajectory. However, as the speed was L-DOPA predicted lower PDQ-mobility scores (R2=70.2).
not sufficient to ignore the launch-interception time, The predictors of PDQ-activities of daily living were BI,
anticipatory reaction according to changing parameters of axial function score and H-Y stage (R2=66). PDQ-39-
the object was essential. We evaluated the hit ratio and communication was predicted only by BI (R2 =15.4); PDQ-
percentage of early and late errors. The results were 39-cognition by UPDRS part I score (R2=25.8); PDQ-39-
compared to the healthy subjects data. bodily comfort by rigidity score (R2 =14.4); and PDQ-39-
Results: The overall hit ratio in the healthy subjects was emotional well-being by UPDRS-part I score (R2=18.8).
significantly higher than in the cervical dystonia group Conclusions: Disability is the strongest predictor of QoL in
(42.07%9.18% versus 34.91%8.23%). Also the reaction PD. Treatment for PD should include strategies to improve
time in cervical dystonia patients was significantly longer. functional independence.
Conclusions: Our data suggest that cervical dystonia
patients have a substantial problem with predictive motor
timing. The results imply that cerebellum and basal ganglia
participate in the integration of visual information with
motor output.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


330 Posters, Sunday 9 September

P1615 P1616
CSF levels of chromogranin-A in the early Quality of life in Huntingtons disease and
stage of Parkinsons disease its association with psychopathology
M. Kaiserov1, K. Mensikova1, D. Stejskal2, P. Kanovsky1 J. Fernandes1, I. Moreira1, S. Cavaco1,2, J. Damsio3,
Department of Neurology, Palack University and
1
R. Loureiro3, M. Magalhes3
University Hospital, Olomouc, 2Center of Laboratory 1Unidade Multidisciplinar de Investigao Biomdica,

Medicine, Prostejov, Czech Republic Instituto de Cincias Biomdicas da Universidade do Porto


Background: Chromogranin-A levels in the cerebrospinal (UMIB, ICBAS-UP), 2Unidade de Neuropsicologia, 3Servio
fluid have been shown to be significantly reduced in late de Neurologia, Centro Hospitalar do Porto (CHP), Porto,
stages of Parkinsons disease (PD). There are only few data Portugal
referring to its level in the early disease stage; its significance Introduction: Huntingtons disease (HD) is a hereditary
as a potential marker in the differential diagnoses of PD and neurodegenerative disorder without cure or treatments
could not be established yet. that can alter the course of disease progression. Health-
Objectives: To establish the level of chromogranin-A in a related quality of life (QoL) in HD patients is an important
pilot cohort of early stage, treatment-nave PD patients. target of clinical intervention.
Methods: 10 patients (4 males, 6 females) and 10 gender- Aim: To explore the association between HD patients QoL
and age-matched controls have been examined for the levels and their demographic, clinical, and psychopathologic
of chromogranin-A in the cerebrospinal fluid; the control characteristics.
subjects were those patients suffering from either low back Method: The Short Form Health Survey (SF-36) was used
pain or tension-type headache. to assess QoL in 24 HD patients (9 males, 15 females; mean
Results: The mean CSF level of chromogranin-A in PD age of 48.813.3 yrs; 5.62.2 yrs of education; mean
patients was 74.8 (range 41.9 - 123.8) g/l, in the control disease duration of 6.24.5 yrs; mean CAG repeat of
group it was 143.9 (range 116 - 181.3) g/l. The statistical 43.64.7) and 24 demographically-matched healthy
analysis showed a difference at the significance level of subjects. The Unified Huntington Disease Rating Scale
p0.005. (UHDRS), Hamilton Rating Scale for Depression (HRSD),
Conclusions: Our pilot study showed that chromogranin-A Beck Depression Inventory (BDI) and Dementia Rating
levels in CSF of early stage PD patients is significantly Scale-2 were also applied to HD patients. Mann-Whitney
reduced; it probably deserves further research with regard and Pearsons correlations were used for data analyzes.
to its role as a potential biomarker in the differential Results: Statistically significant reductions in HD patients
diagnosis of PD. QoL were observed in all SF-36 dimensions (Physical
Functioning, p=0.008; Role Functioning-Physical, p=0.006;
General Health, p=0.001; Vitality, p=0.008; Social
Functioning, p=0.018; Role Functioning-Emotional,
p=0.002; Mental Health, p=0.018), except Bodily Pain.
Among HD patients, females had worse SF-36 Role
Functioning-Physical (p=0.014) and Vitality (p=0.003). The
decrease in QoL (all except Body Pain) was significantly
related (r>-0.42 to -0.73) to severity of psychopathological
symptoms (UHDRS Behaviour Subscale, BDI, and HRSD).
Relatively modest associations were found with other
demographic and clinical variables.
Conclusion: These results point to the importance of
depressive symptoms in HD patients QoL and reinforce the
need for an integrated and multidisciplinary clinical
approach in HD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 331

P1617 P1618
Cognitive functions, autonomic and Selegiline rescues gait deficits and
sensorial involvements in patients with dopaminergic cells in a subacute MPTP
essential tremor mouse model of Parkinsons disease
M. Korkmaz1, Y. Ktk1, M.A. Dikililer2, M. Ycel1, Q. Zhao1, Y. Bai1, D. Cai2
H. Akgn1, M.T. Kak1, Z. Odaba1 1Department of Neurology, Putuo Hospital,Shanghai
1Department of Neurology, Glhane Medical Academy, University of Traditional Chinese Medicine, 2Department of
2Department of Family Medicine, rnak Military Hospital, Integrative Medicine, Zhongshan Hospital, Fudan
Ankara, Turkey University, Shanghai, China
Introduction: Essential tremor (ET) is one of the most Monoamine oxidase type-B (MAO-B) inhibitors have been
frequent movement disorders in neurology practice. In often recommended as the first-line Parkinsons disease
recent years, there are many discussions about ET being a treatment. Clinical studies showed that they had limited
neurodegenerative disease. symptomatic improvements which seem to be associated
Aim: Neurodegenerative diseases appear with an insidious with enhancement of neurotransmission. Besides selective
onset and are usually progressive. Their incidence increases inhibition of MAO-B activity and elevating endogenous
parallel to age. Neuronal loss frequently is seen in dopamine concentration many experiments found that they
pathological materials. These characteristic features are also possessed neuroprotective effects and a potential of disease-
valid for essential tremor. Therefore ET may be accepted as modification in PD. It may be one of the significant
a neurodegenerative disease. To research this hypothesis we strategies for supporting neuronal survival in PD to augment
aimed to evaluate cognitive functions, autonomic and neurotrophic factor activity, especially glial cell line-
sensorial affects in patients with ET. derived neurotrophic factor (GDNF) and brain-derived
Methods: 60 patients diagnosed with essential tremor neurotrophic factor (BDNF). In this study, we investigated
according to Washington Heights- Inwood Genetic Study of the effects of selegiline on rescuing motor dysfunction and
Essential Tremor (WHIGET) Essential Tremor Diagnose dopaminergic function in 1-methyl-4-phenyl-1, 2, 3,
Criteria were included in our study. Patients are separated 6-tetrahydropyridine (MPTP)-lesioned mice. We found that
in two groups as severe and mild according to results of oral administration of selegiline (1.0mg/kg/day for 14 days)
performance subscale of Essential Tremor Rating to MPTP-treated mice, commencing after impairment of the
Assessment Scale (TETRAS). nigrostriatal system, suppressed the reduction of nigral
Results: No difference was seen in Sympathetic Skin dopaminergic neurons and striatal fibres of MPTP-lesioned
Response and R-R intervals between groups. Lower animals (p<0.001). At the 7th and 14th days of low dose
standard mini mental test scores, longer cortical selegiline-treatment, the gait disorder showed obvious
somatosensory evoked potentials latencies and higher improvement. Furthermore there was a significant elevation
serum total copper levels were observed in the severe group in expression of GDNF and BDNF mRNA (2.10 and 2.75-
compared to the mild group. fold) and proteins (143.53% and 157.05%) in selegiline-
Conclusion: We also observed cognitive and sensorial treated mice compared with saline-treated MPTP-lesioned
involvement in electrophysiological tests. In addition, high mice. In addition, the Bax/Bcl-2 ratio of gene and protein in
levels of serum total copper were correlated with disease MPTP-lesioned mice obviously increased, and this effect
severity. Detailed investigation about copper metabolism in could be reversed by selegiline. Correlation analysis
more patients should be made to uncover this functional revealed that both the gait measurement and GDNF/BDNF
correlation. were positively correlated with the number of dopaminergic
neurons. These findings demonstrate that selegiline has
neurorescue effects probably associated with induction of
NTFs and anti-apoptotics.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


332 Posters, Sunday 9 September

P1619 P1620
The evolution of complexity in Does brain degeneration in Wilsons
transcranial magnetic stimulation induced disease involve not only copper but also
surface EMG: a possible illustration of iron accumulation?
plasticity-like changes M. Skowronska1, T. Litwin1, K. Dzieyc1,
M. Cukic1,2, A. Kalauzi3, M. Ljubisavljevic4, A. Wierzchowska1, A. Czlonkowska1,2
12nd Department of Neurology, Institute of Psychiatry and
N. Jorgovanovic2, V. Kostic1
1Institute for Neurology, Belgrade University, Belgrade, Neurology, 2Department of Experimental and Clinical
2Department for Signals, Systems and Automatic Control, Pharmacology, Medical University of Warsaw, Poland
University of Novi Sad, Novi Sad, 3Institute for Introduction: Wilsons disease (WD) is an autosomal
Multidsciplinary Studies, University of Belgrade, Serbia, recessive inherited disorder of copper metabolism. Clinical
4Department of Physiology, Faculty of Medicine and Health
manifestations of WD include neurologic, hepatic and
Sciences, UAE University, Al Ain, United Arab Emirates psychiatric symptoms. Changed MR results are observed in
Introduction: The aim of this study was to determine most WD patients with the neuropsychiatric form, and in
whether a single-pulse TMS induces changes in complexity some with the hepatic and presymptomatic forms. In
of surface EMG immediately after the stimulation. previous studies showed that T2* and susceptibility-
Methods: We analyzed changes in Fractal Dimension (FD) weighted imaging (SWI) are highly sensitive to brain iron
in FDI muscle of the dominant hand in 9 healthy subjects, accumulation, which causes decreased signal intensity.
while exerting three intensities of voluntary activation weak Brain abnormalities in WD patients have been also
(<30% of MVC), medium (30-60%) and strong (>60%); demonstrated with transcranial sonography (TCS).
control group were 5 healthy volunteers sustaining the same Aim: Our aim was to investigate whether Wilsons disease
three levels of contraction without presentation of TMS. FD patients have MR changes typical of neurodegeneration
was calculated using Higuchis algorithm, of EMG with brain iron accumulation, using T2* and blood
immediately after a TMS-induced silent period and oxygenation level dependent imaging protocols. We also
compared with those calculated from preceding TMS EMG investigated TCS lesions in basal ganglia in WD patients.
sections; FD with moving window was used to depict the Methods: MRI with established protocol and TCS was
curves of complexity changes, and then we performed FFT performed in admitted, clinically stable, and treated
of the data. All analysis were performed in response to three patients.
intensities of TMS stimulation set at 1.1 x MT, 1.2 x MT Results: Hypo-intensity in the lenticular nucleus (LN) was
and 1.3 x MT. Ten single TMS stimuli were delivered in observed on T2* images of 78% neurologic, 50%
each series using a figure-of-eight coil positioned over the presymptomatic, and no hepatic patients (p<0.05) In SWI
optimal spot on the skull to elicit MEP from FDI muscle. images we found hypointense signal in LN in all neurologic,
Results: The FD of the EMG after TMS fell in the majority 83% presymptomatic, and 46% hepatic patients. No
of examined series of recordings (in 72 out of 90 series), acoustic window was found in 2 patients. Hyperechogenicity
and cyclic-like changes were observed in all the curves in TCS in LN was found in 87.5% neurologic, 83%
constructed. Statistically significant changes in FD of series presymptomatic and 75% hepatic patients. SWI is more
before and after were more prominent for recordings sensitive in detecting a paramagnetic signal than
during sustaining mild and medium MVC, than in strong conventional T2 and T2* sequences, especially for Wilsons
MVC (p<0.05). disease hepatic patients.
Conclusion: It appears that single pulse TMS of the motor Conclusions: Magnetic resonance data suggests brain iron
cortex induces plasticity-like changes in the voluntary EMG accumulation in Wilsons disease. TCS is also a valid tool
signal. for detection of basal ganglia metal accumulation in WD
patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 333

P1621 P1622
Progressive supranuclear palsy-like International studies on Huntingtons
syndrome as a relatively common feature diseases in Poland
of different neurodegenerations D. Zielonka
R. Matj1, R. Rusina2,3 Poznan University of Medical Sciences Poland, Poznan,
1Pathology and Molecular Medicine, 2Neurology, Thomayer Poland
Hospital, 3Neurology, Institute for Postgraduate Education Introduction: Huntingtons disease (HD) is a progressive
in Medicine, Praha, Czech Republic neurodegenerative disorder where the conditioning factor is
Introduction: Progressive supranuclear palsy (PSP) is a dynamic mutation in HTT gene. Being rare, it is difficult
characterized by early gait disturbances and falls, axial to collect a large enough cohort for significant interventional
rigidity, vertical gaze palsy, and subcortical dementia. PSP and environmental studies.
is considered to be a tauopathy; however, a PSP-like Materials and methods: Conducted in Poland, HD
syndrome has been associated with different research initiatives were reviewed in an archive of the HD
neurodegenerative entities. We present a series of 10 research Coordination Centre for Poland located at the
patients with a clinical presentation evoking PSP and Department of Social Medicine of Poznan University of
neuropathological findings of different neurodegenerative Medical Sciences in Poland, Clinical Trials - a service of the
entities. U.S. National Institutes of Health (www.clinicaltrials.gov),
Methods: Patients fulfilling the clinical and radiological EMBASE database, and the U.S. National Library of
diagnostic criteria for possible or probable PSP were post Medicine National Institutes of Health (PubMed).
mortem neuropathologically examined. The detailed Results: Two observational and three clinical studies
histopathological assessment included immuno regarding HD were conducted in Poland. The core study for
histochemical analysis using a spectrum of antibodies such research initiatives is REGISTRY; a prospective,
against different proteins associated with observational, longitudinal study on the rate of HD
neurodegenerations. progression collecting a large number of data on patients
Results: We found in 5 patients frontotemporal lobar during annual visits. Participants of the HD studies as well
degeneration (FTLD-TDP); 2 patients had a synucleino as number of cross-sectional and clinical studies increased
pathy (multiple system atrophy and diffuse Lewy body quickly in the last years. Based on REGISTRY and other
disease, respectively), 1 case was neuro pathologically studies, database as well as HD patients association
closed as a genetic form of Creutzfeldt-Jakob disease and 2 participants are easily and quickly collected for any other
cases were Alzheimers disease. studies. 643 participants underwent all HD studies in Poland
Conclusion: Our observations support previous data up to date.
suggesting that the clinical picture of PSP-like syndrome Conclusions: Due to its large population Poland has
seems to be related to preferential localization of become a target country for rare diseases research.
pathogenetic inclusions and neuronal cell loss rather than to REGISTRY was formed as a novel approach, to overcome
specific pathological mechanisms of the disease itself. difficulties in patient collection for Huntingtons disease
Moreover, our observation confirms the importance of interventional and environmental studies, to effectively
neuropathological verification in patients clinically increase the number of enrolled study participants.
diagnosed as atypical PSP.
Acknowledgement: This study was supported by grant IGA
NT12094-5/2011 from the Czech Ministry of Health.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


334 Posters, Sunday 9 September

P1623 P1624
Normal 0 21 primary episodic lingual Cerebral amyloid and hypertensive
protrusion dystonia angiopathy as the cause of diminished
R. Manso-Caldern level of consciousness and complex
Neurology, Hospital Universitario de Salamanca, movement disorders
Salamanca, Spain
R.C. Ginestal, A. Herranz, P. Garca-Ruiz, J. del Val,
Introduction: Lingual protrusion dystonia (LPD) is a S. Bellido, M.A. Aranda, I. Navas, B. Gonzalez-Giraldez
disabling form of cranial dystonia which varies from Neurology, Fundacin Jimnez Daz, Madrid, Spain
repetitive and/or episodic to sustained tongue protrusion,
Introduction: Amyloid and hypertensive angiopathies can
and can also be action-induced with speaking or eating.
infrequently cause transient focal neurological episodes.
LPD often occurs in association with oromandibular
These episodes use to be sensitive (paresthesias), motor
dystonia but can be isolated. Both idiophatic and secondary
seizure-like episodes or visual disturbances.
cases exist. Secondary causes include tardive dystonia, head
Patient: A 84-year-old woman, with a chronic respiratory
injury, electrical injury, heredodegenerative diseases (neuro-
condition and a chronic renal insufficiency due to
acanthocytosis, pantothenate kinase-associated
hypertension, previously treated with two courses of
neurodegeneration, neuroferritinopathy, Wilsons disease)
antibiotics for a respiratory infection, developed a
or varicella infection. However, primary episodic LPD is
pneumonia. She was treated with iv cefepime. 24 hours
rare.
after admission, she started to be unresponsive. 48 hours
Methods: A 17-year-old woman presented with a one-year
later, left upper limb coreic and ballistic movements,
history of episodic tightening of her tongue while speaking
together with lower limb ballistic movements and
and eating, causing difficulty in articulation and swallowing.
generalized myoclonias appeared. The CT scan showed an
Each attack lasted less than 1 minute. This symptom started
extensive chronic microangiopathic angiopathy. The CSF
after experiencing a cervical spine trauma. There was no
exam did not disclose any sign of infection or haemorrhage.
family history of neurological disorders. She did not take
The EEG exam demonstrated a diffuse encephalopathy with
neuroleptics or other medications. On examination she had
no signs of epileptic activity. We started treatment with iv
episodic speech-induced tongue protrusion associated with
valproic acid and tiapride. The antibiotic therapy was
mild dysarthria. The remainder of the neurological and
adapted to the renal insufficiency. An MRI was performed
general examination was normal.
showing an extensive and severe microangiopathic
Results: Routine haematological and biochemical
angiopathy. Several haemosiderin deposits related with past
evaluation were completely normal, as well as thyroid
microbleedings were seen both in supratentorial and
function, copper and ceruloplasmin, number of
infratentorial locations. There were no signs of acute
acanthocytes, autoimmune screen and serologies. Magnetic
haemorrhage or ischaemia. After 4 days with the new
resonance imaging of the brain and cervical spine was
antibiotic scheme and under valproic acid and tiapride, the
normal. EEG recorded during an episode of tongue spasm
patient started to improve. First, the fever and the movement
was normal. Interictal EMG of the tongue showed no
disorders disappeared. Then, the level of consciousness
evidence of denervation or myokymia. Two trials of
returned to normal. She was discharged 27 days after
treatment with clonazepam and carbamazepine were
admission with no signs of neurological impairment.
ineffective. The patient refused further trials of medication.
Discussion: We present a very infrequent case of acute
Conclusion: In contrast to most other paroxysmal
multifocal choreic, myoclonic and ballistic related to
movement disorders, episodic LPD does not appear
amyloid and hypertensive angiopathy.
responsive to anti-epileptic drugs.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 335

P1625 P1626
Improvement of tremor in a Parkinsons Effects of Nigella sativa oil on haloperidol
case after thalamic infarction induced movement deficits in a rat model
A. Kksal1, M. ztrk1, N.H. Stpideler Kksal2, T. Malik1, D.J. Haleem1, S. Pervez2, S. Hasan2, T. Fatima3
B. Mutluay1, A. Ceyhan Dirican1, F. Aysal1, S. Bayba1 1Neurochemistry & Biochemical Neuropharmacology Unit ,
1Neurology, Bakrky Training and Research Hospital for Biochemistry Department, The University of Karachi,
Mental Health and Neurological Sciences, 2Neurosurgery, Karachi, 2Department of Pathology and Microbiology,
3Department of Biological Biomedical Sciences, The Aga
Istanbul Educational and Research Hospital, Istanbul,
Turkey Khan University Hospital, Karachi, Pakistan
Introduction: The medical treatment of Parkinsons disease The neuropathological status of haloperidol (HP) induced
includes dopamin agonists, enzyme inhibitors and extrapyramidal symptoms (EPS) remains unclear, but
neuroprotective agents together with L-dopa. These drugs several lines of evidence suggest that persistent neuronal
are all symptomatic and do not affect the course of the alterations in the basal ganglia cause EPS by HP provoked
disease and motor complications may arise during medical oxidative stress. The objective of this study was to evaluate
treatment. Thus, surgery is also performed for the the possible protective effects of the antioxidative agent
symptomatic treatment of this disease. Surgery is observed Nigella sativa (NS) oil on HP induced neuropathological
to be more effective on tremor rather than the bradykinesia alterations and related motor symptoms in the rodent
and rigidity of Parkinsons disease. striatum.
Case report: A 76-year-old male Parkinsons patient is Methods: HP was administered alone and with NS oil. EPS
followed in our outpatient clinic since 2003. He had right was monitored in the HP treated groups and the animals
side dominant bradykinesia, rigidity and resting tremor. He treated with NS only and placebo.
was Hoehn&Yahr Stage 2 and had a UPDRS score of 16 Results: The HP treated group displayed a high degree of
points. His symptoms were partially under control with a motor impairment (p<0.00) shown on rota rod experiment,
combination of L-dopa, benserazide and entacapone. The vacuous chewing movement (p<0.00) shown grossly
patient was admitted to our emergency clinic in May 2007 disturbed the large fraction of the cytoarchitectonic pattern
with difficulty in speaking and right-sided weakness. (p<0.05), histopathology with nerve cell depletion
Neurological examination revealed dysarthria and slight concomitant shrunken cytoplasm, nuclear membrane
right-sided hemiparesis involving the face. Diffusion- breakdown and chromatin disorganization. Scarring was
weighted cranial MRI showed an acute infarction at the also a prominent feature owing profusion of astrogliosis in
ventromedial part of the thalamus. the dorso- and ventrolateral regions of the caudate putamen
Results: On the 15th and 30th day, neurological examination and in the core of nucleus accumbens. Moderate levels of
revealed a complete recovery of dysarthria and hemiparesis halo and pyknotic neurons were also observed in HP treated
as well as the tremor of Parkinsons disease. Bradykinesia rodents. The morphological HP induced neuronal changes
and rigidity were unaffected. were almost absent in the HP plus NS treated groups
Conclusion: Though the possibility of recovery of tremor (p<0.00). However minor astrogliosis was observed with no
in Parkinsons disease after a thalamic infarction is obvious indication of cell loss and 82% normal neuronal
theoretically known, it is a very rare clinical entity. We densities were observed using a quantitative, analytic
aimed to present this rare case with thalamotomy-like approach in the NS plus HP treated striatum. We conclude
effects of thalamic infarction and also to discuss surgery in that NS therapy has preventive effects on HP induced
Parkinsons disease in the light of this case. neuronal degeneration in the striatum.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


336 Posters, Sunday 9 September

P1627 P1628
Cardiovascular factors in hospitalized Social isolation and quality of life in
patients with idiopathic Parkinsons Parkinsons disease
disease: a retrospective analysis J.Y. You
K. Lauckaite1, G. Zemgulyte2, I. Sniokaite2, D. Surkiene1, Neurology, Hanil General Hospital, Seoul, Republic of
D. Rastenyte1, A. Vaitkus1 Korea
Neurological, 2Lithuanian University of Health Sciences,
1 Background: Parkinson patients have many difficulties in
Kaunas, Lithuania mobility, cognition and psychiatric symptoms such as
Introduction: Evidence suggests that cardiovascular depression, anxiety and suicidal idea. Of course many
abnormalities, co-morbid presence of white matter lesions physical problems disable patients, but recent progress
(WML) may exacerbate or contribute to some motor and including medication and surgical methods have improved
cognitive deficits in Parkinsons disease (PD). progressively. So physical disabilities improved rapidly.
Methods: A retrospective study was carried out at the But psychosocial disabilities have been undertreated and
Hospital of the Lithuanian University of Health Sciences. missed. So this study aims to find other psychosocial
We have analyzed case histories of 129 (55 males, 74 support that can change the QoL of Parkinson patient.
females) PD patients hospitalized in 2011. Diagnosis was Method: 20 Parkison patients who live alone or with an old
established according to the UK brain bank criteria. The spouse were selected. Inclusion criteria were no regular job
clinical, demographic data, computed tomography (CT), and social club. So socially isolated patient were selected.
electrocardiogram, carotid ultrasound (ECCS), Barthel The patients QoL was measured and assessed by the
index (BI), MMSE results were collected. Parkinsons disease quality of life (PDQL) and the Beck
Results: The mean age of PD patients (SD) was Depression inventory (BDI). We divided this group. One
68.68.9yrs, and stage according to Hoehn-Yahr (H-Ys) group (No=12) is newly attending the day care unit or
2.20.9. Tremor-dominant PD (TD) was diagnosed in 39 rehabilitation center providing the physical activity and
(30.2%), postural-instability-gait-disorder-dominant cognitive rehabilitation. The other group (No=8) is socially
(PIGD) in 58 (45%), mixed type (MT) in 25 (19.4%), 7 isolated, as before. Baseline, 3 months, 6 months PDQL and
(5.4%) were unclassified. The most advanced H-Ys BDI were checked.
(p=0.027) and the lowest MMSE points (p=0.099) were in Result: The first group who newly joined social activities
MT and PIGD groups. Mostly TP accounted for the urgent showed improvement in BDI and PDQL. The improvement
admission (37.5%, Chi2=9.1, p=0.028). H-Ys correlated difference gap is increased to time.
negatively with BI (r=-0.4, p<0.001), systolic BP (r=-0.2, Conclusion: Socially isolated state due to physical activity,
p=0.03) and QRS interval (r=-0.3, p=0.014). Brain CT was depression or other socio-economic state aggravated
performed in 43 (33.3%), with WML detected in 22 Parkinson patients QoL. So physicians must consider the
(51.2%). The highest rate of WML was in PIGD (Chi2=8.3, social isolation. If physicians consider this problem, the
p=0.004) and in the elderly (p=0.002). Linear velocities on family and social support will be increased. Recently in
ECCS (n=46, 35.7%) were lower in common or external Korea, government supporting social nursing insurance is
carotid arteries at stages 2 vs. 1 (p<0.05). started. And this system is expected to improve the
Conclusions: WML were associated with an age and PIGD Parkinson patients QoL in aspects of social isolation.
PD. The highest motor and cognitive impairment was
detected in MT and PIGD. According to cardiovascular
factors, stage 2 and later is an advanced disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 337

P1629 P1630
Parkinsons disease (PD) epidemiology High intake of folate provides anti-
and clinical presentation in Lugansk, parkinsonism effects: no role for plasma
eastern Ukraine level of homocysteine
I. Pepenina H. Haghdoost-Yazdi1, N. Fraidouni2, A. Faraji1,
Out-Patient, Hospital N10, Lugansk, Ukraine M. Sarookhani1
1Cellular and Molecular Research Center, Qazvin University
PD is a common extrapyramidal disorder in Ukraine which
is vastly misdiagnosed. Low awareness of PD differential of Medical Sciences, Qazvin, 2Tarbiat Moalem University,
diagnosis, as well as low access to drugs and neurosurgical Tehran, Iran
interventions seriously affect management efficiency. Several lines of evidence show that homocysteine (Hcy)
Study objective: To analyze PD prevalence in Lugansk levels are increased in blood and CSF of patients with
(Eastern Ukraine). Parkinsons disease. Folate is necessary for Hcy metabolism
Methods: The retrospective follow-up study of PD and there is generally an inverse relationship between
prevalence in the region has been performed. Diagnosis of plasma folate and Hcy levels. In the present study, effect of
PD was based on Hughes et al. criteria (1992), for staging folate supplementation on the severity of 6-hydroxydopamine
Hoehn-Yahr scale (1967) was used. (6-OHDA)-induced parkinsonism was investigated. Rats
Results: Prevalence of PD in different age groups varies were nourished with different doses of folate supplements
significantly from 4.8 per 100,000 at the age below 55 to from 1 month before stereotaxic injection of 6-OHDA to the
178.0 per 100,000 in older patients. Clinical disease end of experiments. Plasma Hcy was measured at the end of
peculiarities have been analyzed in 71 cases. Patients in experiments to identify its association with parkinsonism.
stages 2-4 predominated (21 males, 28 females). Tremor Our results indicate that folate supplementation attenuates
prevailed in 22 patients (8 males, 14 females), akinetic-rigid severity of parkinsonism. The best effect was seen in rats
syndrome in 21 patients (13 males, 8 females), mixed receiving folic acid at 10-folds of normal MEM (minimum
symptoms were found in 28 patients (14 males, 14 females). essential medium). In this group, number of apomorphine-
Right-side onset was established in 42 cases (18 males, 24 induced rotations in the post-surgery tests was more than
females), left-side in 29 (17 males, 12 females). Rapid 60% lower than that in the control group. These rats also
progression was more frequent in 29 cases (16 males, 13 showed significant better performances in rotarod test and
females), whereas moderate and slow type were detected in had learning patterns similar to healthy rats. 5-fold
19 cases (11 males, 8 females) and 18 cases (7 males, 11 supplementation of folate also remarkably improved the
females), respectively. In the disease management mostly rotarod performances but had modest effect on the rotational
L-dopa, NMDA-antagonists, and dopamine-receptor behaviour. 2-fold supplementation had no effect. The levels
agonists are being used (in 56, 34 and 27 cases, respectively). of Hcy in rats receiving moderate and low doses of folate
6 patients use MAO-B inhibitors. supplementations were near to that in the control group.
Conclusion: PD in Lugansk is being mostly detected in However, in rats treated by high dose of folate, Hcy was
advanced stage seriously hampering the life quality in older significantly higher than that in the control group. Our
patients. results indicate that folate supplementation provides anti-
parkinsonism effect, in a dose dependent manner, but this
effect is not mediated by lowering plasma Hcy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


338 Posters, Sunday 9 September

P1631 P1632
Rasagiline for the treatment of gait The role of the activity of NO-system in
disturbances in patients with Parkinsons the development of depression in
disease (PD): an open label study Parkinsons disease
N. Van Blercom, G. Linazasoro R.J. Matmurodov, K.M. Khalimova, M.M. Raimova
Centro Investigacin Parkinson Policlnica Gipuzkoa, San Tashkent Medical Academy, Tashkent, Uzbekistan
Sebastian, Spain Some non-motorized manifestations such as disorders of
Background: Gait disturbances are a major cause of smell, constipation, depression, pain syndromes occur
disability in patients with advanced PD. Their management before the development of the classic motor symptoms of
is difficult and the classical dopaminergic treatment is often Parkinsons disease (PD).
unsuccessful. Rasagiline has shown some beneficial effects Objective: To study the role of the activity of NO-system in
against gait disorders. the development of depression in patients with PD.
Aim: To study the symptomatic effect of rasagiline 1mg/ Methods: A total of 58 (35 of them with depression and 23
day on gait and mobility in advanced PD. non-depressed) patients with PD. The average age was
Methodology: 15 non-demented PD patients (mean age 54.34.5 years. For the estimation of NO-system studied in
74.65.8 y.o; mean PD duration 9.36.1 years; mean Hoehn the concentration of red blood cells NOx, the activity of
& Yahr stage 3.140.8) were included in this prospective, NADPH-dependent NO, the level of peroxynitrite
open study of 8 weeks duration. They showed clinically (ONOO-), the activity of NO-synthase (NOS). The level of
significant gait disturbances as determined by the Timed Up depression was determined using the Zung scale.
and Go test (TUaG), (TUaG >10 seconds in ON and 14 in Results: The results of this analysis showed that the NO-system
OFF). They were receiving a stable dose of antiparkinsonian parameters in patients without depression comprise: NO
medications. Besides TUaG (primary variable), patients 22.70.55mmol/l, eNOS 13.90.263mol/min/l, NADPH-HP
were assessed by using the UPDRS, freezing of gait and 1.020.03mmol/min/l, ONOO- 0.080.005mmol/l. In patients
new freezing of gait questionnaires (FOG-Q and NFOG- Q) with mild depression up: NO-30.80.34, eNOS- 9.710.13,
and PDQ-39. NADPH-NR-1.460.02, ONOO- 0.0210.003. In patients
Results: 4 patients were withdrawn due to early mild side with depression of moderate severity parameters of NO-up:
effects. TUaG was significantly improved (basal 26.618.7, NO-38.50.45mmol/l, eNOS 8.30.3mol/min/l , NADPH-HP
final 2014; p<0.05). UPDRS, PDQ39, FOG and NFOG 1.790.1mmol/min/l and ONOO- 0.350.02mmol/l. Our data
questionnaires remained unchanged though a trend towards indicate that the degree of growth depression in PD is
improvement in the last two questionnaires was observed. accompanied by a worsening imbalance of the NO-system and
Conclusion: Rasagiline improved some parameters related the progression of the clinical course.
to gait performance, mainly TUaG. Rasagiline is a Conclusion: Thus, the degree of depression in PD depends
therapeutic option in this clinical situation. on the activity of the NO-parameter system. In this
The study was sponsored by Lundbeck. connection, the imbalance in the NO-system can be
considered as a factor in the progression of depression in
PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 339

P1633 P1634
Parkinsons syndrome in chronic Muscle testing and loss of ambulation in
neuroborreliosis Friedreichs ataxia
N.S. Baranova, M.A. Bykanova C. Mignard-Moydelacroix1, A. Boufferet2, L. Bonnet2,
Yaroslavl State Medical Academy, Yaroslavl, Russia D. Mignard-Moydelacroix2, Centre de Rfrence
Purpose: To identify the occurrence of Parkinsons Maladies Neurologiques Rares
syndrome (PS) in patients with chronic neuroborreliosis
1CHR de La Reunion, 2Neurologie-Maladies Rares, CHU de
(CNB). La Reunion, Saint-Pierre, Reunion
Materials and methods: We explored 164 patients (115 Many genetic diseases are observed in Reunion Island, a
female and 49 male) with diagnosis of CNB, average age French island in the Indian Ocean. Late-onset Friedreichs
49.616.27 years. CNB was diagnosed by the criteria: the ataxias are frequent. Many adult people are walking yet in
presence of neurologic symptoms within 6 months from the our experience.
appearance of erythema migrans and/or a tick bite or Aims: The neurological disorder affected mainly the
symptoms persisting for more than 6 months; the presence cerebellar tracts. Initially, the muscles are not weak. The
in serum or CSF of diagnostically significant elevated titres muscle testing changes over time were compared with the
of antibodies to Borrelia burgdorferi; the presence of walking status to clarify the cause of the loss of ambulation.
clinical improvement from specific antibiotic therapy and/ Materials and methods: 44 patients for 12 years were
or decrease in antibody titres to Borrelia burgdorferi in the classified into three categories: walkers (16), standing (11)
dynamic study of blood serum; the exclusion of other and non-walkers (17). 175 muscle testings were achieved
causes, which could explain development of existing since 2008: The average score showed muscle damage for
symptoms. They also used the Diagnostic criteria UK each muscle group. Next, in six patients who lost the ability
Parkinsons disease (PD) Society Brain Bank (1992), to walk (6) since 2008: the testing done before, during and
Schwab and England (1969) scale, MRI. after the loss of ambulation were reviewed .
Results: PS was diagnosed in 5 (3%) patients with CNB. Results: The walkers had a mean score between 4 and 5
The combination of PD and CNB was observed in 3 (1.8%) for all muscle groups; the standing up had poorer
patients (1 male and 2 female). Slight deterioration was outcomes (between 3 and 4) with elective involvement (3/5)
noted (no more than 10% of Schwab and England) and the on three muscle groups, (psoas, gluteus and adductors);
persistence of symptoms of PD after antibiotic treatment of non-walkers scores showed the same involvement. In the
neuroborreliosis. PS due to CNB was defined in 2 (1.2%) 6 patients who lost ambulation since 2008, 6 months before
female. Symptoms of PS due to CNB significantly the loss of ambulation, no patient had a wholesome muscle
decreased after antibiotic therapy. Levodopa, dopamine score (all<5). Three muscle groups were more impaired
agonists were not effective. than others (3/5): the gluteus, psoas and adductors.
Conclusion: PS as a manifestation of neuroborreliosis Conclusion: The loss of ambulation in patients with
occurred in 1 patient from 82 cases of CNB. Antibiotic Friedreichs ataxia is correlated markedly and significantly
therapy significantly reduced the severity of parkinsonian with muscle weakness of psoas, gluteus and adductors.
symptoms. Preventive muscle strengthening on these muscles, could
delay the disease progression.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


340 Posters, Sunday 9 September

P1635 P1636
Exposure of multiple system atrophy Medication use in patients with multiple
(MSA) patients with or without orthostatic system atrophy or Parkinsons disease
hypotension (OH) to potentially compared to a group of patients
hypotensive drugs consulting a general practitioner
S. Perez-Lloret1, M.V. Rey1, A. Pavy-Le Traon2, S. Perez-Lloret1, M.V. Rey1, A. Pavy-Le Traon2,
W. Meissner3, F. Ory-Magne2, C. Brefel-Courbon2, W. Meissner3, F. Ory-Magne2, C. Brefel-Courbon2,
N. Fabre2, F. Tison3, O. Rascol1 N. Fabre2, F. Tison3, O. Rascol1
1 Clinical Pharmacology, 2Neurology, CHU Toulouse, 1 Clinical Pharmacology, 2Neurology, CHU Toulouse,
3 Neurology, CHU Bordeaux, France 3 Neurology, CHU Bordeaux, France
Background: OH is a hallmark of MSA. Objective: To compare drug utilization between MSA,
Objective: To assess the exposure of MSA patients with or Parkinsons disease (PD) patients or unselected patients
without OH to drugs that can potentially induce OH. consulting a general practitioner (GP).
Methods: Patients were assessed at the French MSA Methods: 147 MSA patients (according to Gilman criteria)
reference Centers between 2008 and 2011. Blood pressure were assessed at the MSA reference Center between 2008
(BP) was measured 5 min after lying down and every min and 2011. 653 PD patients (according to UKPDSBB
during 10 min after standing up. According to Gilmans criteria) and 98 patients visiting a GP for reasons not related
criteria, OH was defined as systolic/diastolic BP fall to PD or MSA were recruited from the same geographical
30/15mmHg during the first 3min after standing. Exposure area. Data were analyzed by chi-square test followed by
to drugs commonly associated with OH, such as insulin, pair wise comparisons by bonferroni-adjusted z-test for
antihypertensives of any class, peripheral vasodilators, proportions.
drugs for heart-disease, alpha1-adrenergic receptor Results: MSA patients were younger than PD or GP patients
antagonists, dopaminergic drugs and antidepressants, was (651 vs. 681 and 711 years p<0.001). Proportion of
recorded. males was similar in the 3 groups (MSA: 50% vs. PD: 49%
Results: 131 MSA patients were included in the study (age and 46%, p=0.9). MSA patients were more frequently
64.70.7, 50% males, 61% MSA-P, UMSARS II score exposed to drugs for bowel disorders (MSA: 19% vs. PD:
25.30.7). OH was detected in 76 (58%) patients. 84% of 6% and GP: 10% p<0.001), to urinary antispasmodics
patients were exposed to at least 1 potentially hypotensive (MSA: 18% vs. PD: 2% and GP: 1% p<0.001) to drugs used
drug. These patients were exposed to a mean of 21 for orthostatic hypotension such as midodrine or
potentially hypotensive drugs. Patients with OH were less fludrocortisone (MSA: 42% vs. PD: 3% vs. GP: 0%,
frequently exposed to antihypertensives (16% vs. 31%, p<0.001) to antihypertensives (MSA: 22% vs. PD: 40% vs.
p<0.01), levodopa (58% vs. 78%, p<0.01) or dopamine GP: 58% p<0.001). MSA patients were less frequently on
agonists (9% vs. 36%, p<0.01). Results remained significant antiparkinsonians as compared to PD (73% vs. 88%
after adjusting for demographic or disease-related factors p<0.05). Finally, MSA patients were more frequently on
by logistic regression analysis. antidepressants (MSA: 48% vs. PD: 18% and GP: 10%
Conclusion: Our study showed that patients with OH were p<0.001).
less frequently exposed to antihypertensives, levodopa or Conclusions: Medication use patterns differ in patients
dopamine agonists. As it is possible that treating physicians with MSA, PD and those visiting a GP.
may have avoided exposing MSA patients to these drugs,
drug exposure may not be a major factor connected with
OH in MSA.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 341

P1637 P1638
Medication use in the patients of the Features of the basal metabolism in
French multiple system atrophy (MSA) patients with Parkinsons disease
reference center R. Bogdanov1, A. Bogdanov2, E. Manannikova1,
M.V. Rey1, S. Perez-Lloret1, A. Pavy-Le Traon2, B. Kaganov2, S. Kotov1
1Department of Neurology, Moscow Regional Scientific
W. Meissner3, F. Ory-Magne2, C. Brefel-Courbon2,
N. Fabre2, F. Tison3, O. Rascol1 Research Clinical Institute n.a. M. F. Vladimirsky, 2The
1 Clinical Pharmacology, 2Neurology, CHU Toulouse, Institute of Nutrition Russian Academy of Medical Science,
3 Neurology, CHU Bordeaux, France Moscow, Russia
Objective: To describe medication use in MSA patients and Aim: To study basal metabolic rate in patients with
to relate it with different characteristics of the disease. Parkinsons disease.
Methods: Patients were assessed at the French MSA Material and method: 30 patients with a diagnosis of
reference Center. The following variables were collected: Parkinsons disease were examined and compared with 30
MSA diagnosis (probable vs. possible according to control subjects. None of the patients were receiving any
Gilman criteria), disease duration, autonomic dysfunction anti-parkinsonian treatment before the examination. We
(SCOPA-Aut), disease severity (UMSARS I+II), clinical studied basal metabolic rate and oxidation rates of
subtype (MSA-P vs. MSA-C) and any medication use macronutrients (protein, fat and carbohydrates) by indirect
(coded by ATC). Data were analyzed by chi-square test; respiratory calorimetry in all patients.
only significant differences are reported. Results: The average basal metabolic rate was defined. It
Results: 147 MSA patients were recruited (mean age was increased by 33.9% in the patients with Parkinsons
65.30.7; 50% males, 61% MSA-P; 82% probable MSA; disease compared to the control group. The separated
mean UMSARS-score 48.91.3; mean disease duration analysis of the average rate of oxidation of the
5.10.2).Overall, MSA patients received 8.20.4 macronutrients in patients with Parkinsons disease showed
medications. 73% of patients received at least one that the average rate of oxidation of the fats was increased
antiparkinsonian (mainly levodopa: 67%), 33% midodrine by 94%, average rate of oxidation of the carbohydrates was
and 10% fludrocortisone. More severely affected patients increased by 8.6%, and there were no statistical significant
(UMSARS>47) more frequently received antithrombotics changes for proteins, compared to the control group.
(27% vs. 14%; p<0.05) antidepressants (61% vs. 38%; Conclusion: Our results show that the increased basal
p<0.01,) or drugs for bowel disorders (30% vs. 10%; metabolic rate of patients with Parkinsons disease, in the
p<0.01). More patients with MSA-P (versus -C) received first place, was caused by increased basal oxidation of the
antiparkinsonian (90% vs. 46%; p<0.01), antihypertensive fats, and in the second place it was caused by increased
(28% vs. 12%; p<0.05) or analgesic (19% vs. 4%; p<0.01) basal oxidation of the carbohydrates. This result may be
medications. More patients with probable MSA (versus used for diet therapy optimization of patients with
possible) received midodrine (39% vs. 7%; p<0.01) and Parkinsons disease.
less alpha-blockers (5% vs. 19%; p<0.02). Patients with
SCOPA-Aut score >22 were more frequently on
fludrocortisone (18% vs. 3%; p<0.01) or antidepressants
(57% vs. 41%; p<0.04). Patients with disease duration >5
years were more frequently on alpha-adrenergic blockers
for urinary problems (14% vs. 3%; p<0.01).
Conclusion: In MSA, medication use significantly differs
according to disease characteristics.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


342 Posters, Sunday 9 September

P1639 P1640
Using an endophenotype to evaluate the Drug-induced parkinsonism treated with
effect of environmental factors in disease Yokukansan in elderly patients
penetrance of adult onset primary torsion J.Y. Park, Y.H. Sim, E.C. Lee, K.H. Cho
dystonia Department of Cardiovascular & Neurologic Diseases,
College of Korean Medicine, Kyung Hee University, Seoul,
A. Molloy1, O. Kimmich1, D. Bradley1, R. Reilly2,
Republic of Korea
S. O Riordan1, M. Hutchinson1
1St Vincents University Hospital, 2Trinity Centre for Introduction: Drug-induced parkinsonism (DIP) is a
Bioengineering, Trinity College Dublin, Ireland common cause of parkinsonism in elderly people. Many
different types of medication, not only neuroepileptics, may
Objective: We aim to investigate whether significant
induce or exacerbate parkinsonism. Older people are more
differences exist in environmental exposures among
exposed to developing DIP, because they are on multiple
manifesting individuals with adult onset primary torsion
medications for chronic senile diseases. DIP is considered
dystonia (AOPTD) and their unaffected first degree
to be reversible after stopping the offending drugs within 4
relatives with abnormal temporal discrimination, indicating
months. However in some cases, it may persist. The
abnormal gene carriage, to determine reasons for the
managements with anticholinergic agents or dopaminergic
reduced penetrance of AOPTD.
drugs seemed to have additional drug side effects especially
Background: AOPTD is a poorly penetrant autosomal
in elderly people. Yokukansan (YKS) is a herbal formula
dominant disorder. Most (85-90%) gene carriers for AOPTD
used to treat Parkinsons disease in oriental medicine. We
are non-manifesting despite having reached an adequate age
introduce cases of DIP treated with Yokukansan in old
for penetrance. The temporal discrimination threshold
patients.
(TDT) is abnormal in patients with AOPTD and 50% of
Case report: We describe 4 cases of DIP possibly caused
their unaffected first degree relatives. An abnormal TDT is
by medication for common senile diseases, which were
a mediational endophenotype indicating gene carriage in
dibenzodiazepine, SSRIs for depression, flunarizine for
unaffected relatives. It is hypothesized that genetic,
vertigo, and levosulpiride for dyspepsia. All patients were
epigenetic and environmental factors may exert protective
female and above 70 years of age. They presented postural
or deleterious effects affecting penetrance of AOPTD. By
upper limb tremor and bradykinesia about 5 months after
examining environmental exposure history in both AOPTD
the initiation of offending drugs. 2 of them concurrently had
patients and their similarly aged unaffected siblings with
oral-buccal dyskinesia.
abnormal TDTs we may determine the role of the
Results: Symptoms completely improved several weeks or
environment in disease penetrance.
months after discontinuing the causative drugs and being
Methods: This is a case-control single centre prospective
treated with YKS. None of the patients suffered from any
study that will be performed using a standardised
side effects attributable to YKS.
questionnaire. The questionnaire will collect information on
Conclusions: DIP adversely affects the quality of life in
demographics and on a range of past environmental
older patients. Through careful medication history taking
exposures in patients with AOPTD and their unaffected
and avoidance of any potentially offending medication, it
siblings. A total of 60 AOPTD probands with cervical
can be often reversible. Furthermore, we propose that YKS
dystonia and one or more of their unaffected siblings with
might be helpful to the management of DIP.
abnormal TDTs will be recruited. All unaffected siblings
will be over 40 years of age, and age and gender matched to
the proband.
Results: Results of this study will be available at the
congress.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 343

P1641 P1647
Non-motor disturbances in patients with The effect of homocystein and MTHFR
Parkinsons disease gene mutation in Parkinsons disease
S.B. Sattarova1, Y.N. Madjidova2, D.B. Sattarova2 treatment
1Tashkent State University, Nezami, 2Tashkent Medical
P. Nurkan1, H.A. Idrisoglu2, A. Sazci2, N. Polat2
Academy, Tashkent, Uzbekistan 1Merd Company, 2Department of Neurology, Medical

Faculty of Istanbul, Turkey


P1642
Hemifacial spasm due to posterior fossa P1648
lipoma: a case report Association of Parkinsons disease and
S. Nazarbaghi essential tremor
Neurology Department, URMIA University of Medical V. Razdorskaya, G. Yudina, O. Voskresenskaya
Sciences, Urmia, Iran Saratov State Medical University, Saratov, Russia

P1643 P1649
Apomorphine in treatment of Parkinsons Development and validation of a quality of
disease: initiation without discontinuation life measure for carers of people with
or reduction of dopaminergic therapy Parkinsons disease
M.F. Oztekin1, N. Oztekin2, F. Ak2 C. Jenkins, S. Dummett
1Neurology, MOH Ankara Yildirim Beyazit Education and University of Oxford, Health Services Research Unit, UK
Research Hospital, 2Neurology, MOH Ankara Numune
Education and Research Hospital, Ankara, Turkey
P1650
P1644 The combination of SPECT-CT and
Subjective evaluation of emotional visual functional MR imaging in the diagnosis of
stimuli in patients with Parkinsons neuro-acanthocytosis: a case report
disease: a pilot study N. Xiong, Q. Zhu, Z. Liang, C. Liu
Department of Neurology, Union Hospital, Tongji Medical
R. Berlot, B. Dolenc, D. Georgiev, J. Bon, M. Trot, College, Huazhong University of Science and Technology,
Z. Pirtoek Wuhan, China
Department of Neurology, University Medical Centre
Ljubljana, Slovenia
P1651
P1645 Aphasia as primary symptom in
corticobasal degeneration
Life quality improvement in Parkinsons
C. Balla, J.L. Pepin
disease treated with foetal stem cells CHR Citadelle, Lige, Belgium
N. Sych, M. Klunnyk, O. Ivankova
Embryonic Tissues Center EmCell, Kiev, Ukraine
P1652
P1646 Overnight switching from pramipexole to
ropinirole CR in patients with Parkinsons
Strategy of starting levodopa treatment in
disease: an open preliminary trial in Korea
Parkinsons disease patients in Armenia
M.Y. Park, H.J. Park
Z. Tavadyan1,2, H. Bakunts1 Neurology, Yeungnam University College of Medicine,
Department of Angioneurology, Yerevan State Medical
1
Daegu, Republic of Korea
University after Mkhitar Heratsi, 2Somnus Sleep and
Movement Disorders Clinic, Yerevan, Armenia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


344 Posters, Sunday 9 September

P1653 P1659
Treatment of patients with writers cramp Paroxistic non-kinesiogenic and
by injections of botulinum toxin-A hipnogenic dyskinesia (Lugaresi disease):
V. Zmachynskaya, S. Likhachev, T. Charnukha a case report
Republican Research and Clinical Center of Neurology and M.A. Sierra-Beltrn1, C.M. Hernndez-Crdenas2,
Neurosurgery, Minsk, Belarus H. Sentes-Madrid3
1CIDyT, Mdica Sur, 2Critical Care Medicine, 3Neurology

P1654 Department, INCMNSZ, Mexico City, Mexico

Comorbidities of fatigue syndrome in


patients with Parkinsons disease P1660
V. Datieva, O. Levin New approach to gait disorders therapy in
Russian Medical Academy of Postgraduate Education, late stages of Parkinsons disease
Moscow, Russia I. Litvinenko1, I. Krasakov1,2, R. Khalimov1, A. Trufanov1
1Department and Clinic of Neurology, Military Medical

P1655 Academy named after S.M. Kirov, Saint-Petersburg, 2The


Center of Extrapyramidal Disorders, The Nikiforov Russian
Investigation of the prevalence of Center of Emergency and Radiation Medicine, Saint
essential tremor in individuals aged 18-60 Petersburg, Russia
in Erzurum
L. zel1, R. Demir1, G. zdemir1, E. zyldrm2, P1661
U. Avar3, H. Ulvi1, R. Aygl1 The effectiveness of cognitive & reflexive
1Department of Neurology, 2Department of Public Health,
3Family Medicine, Medical School of Atatrk University, saccade measures in discriminating
Erzurum, Turkey Huntingtons disease from healthy
controls
P1656 E.A. Toh1,2, M.R. MacAskill1,2,
J.C. Dalrymple-Alford1,2,3, D.J. Myall2, L. Livingston1,2,
The heterogenity of hypersomnia in J. Ross2, T.J. Anderson1,2
Parkinsons disease 1Department of Medicine, University of Otago, 2New

M. Nodel, N.N. Yakhno Zealand Brain Research Institute, 3Department of


Neurology, Moscow Medical Academy, Moscow, Russia Psychology, University of Canterbury, Christchurch, New
Zealand

P1657
Recently diagnosed chronic liver disease
presenting with parkinsonian symptoms
T. Kasikci1, S. Bek2, E. Tokgoz2, Y. Kutukcu2,
Z. Odabasi2
1 Neurology, 2Gulhane Medical Faculty, Ankara, Turkey

P1658
Post-infectious cerebellar atrophy
C. Silva, D. Neutel, M. Coelho, L. Albuquerque
Hospital Santa Maria, Lisbon, Portugal

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 345

Multiple sclerosis 1 P1663


Depression in multiple sclerosis patients
P1662 treated with IFN1b - who is to blame?
The relationship between age and long- C. Gavan1, E. Terecoasa1, S. Gheorghevici2,
term evolution in multiple sclerosis A. Poalelungi1, C. Tiu1
1University Hospital Bucharest, 2University of Medicine and
A. Scalfari1, A. Neuhaus2, M. Daumer2, G. Ebers3,
Pharmacy Carol Davila, Bucharest, Romania
P. Muraro4
1Centre of Neuroscience, Division of Experimental Medicine, Introduction: Multiple Sclerosis (MS) is a progressive,
Department of Medicine, Imperial College London, UK, disabling disease which affects mainly young adults.
2Sylvia Lawry Centre, Munich, Germany, 3Oxford University, Depression is common during the course of this disease and
Oxford, 4Imperial College London, UK has a multifactorial etiology.
Background: Age has been indicated as a possible factor Methods: We performed a cross-sectional study on 100
influencing multiple sclerosis (MS) disease evolution. patients treated with IFN1b s.c. As this treatment can
Objectives and methods: Among 1023 patients from the favour the onset or the worsening of depression, the purpose
LO Ontario database, Kaplan-Meier and Cox regression of our study was to analyze which factors are associated
analyses investigated the impact of age at disease onset, age with depression and if a longer period of treatment is linked
at onset of progression and current age on long-term to a higher frequency of depression. Each patient was
prognosis. clinically evaluated with a complete neurologic examination
Results: Growing older significantly affected disability and EDSS score was calculated. The presence of depression
accumulation (HR per additional year: DSS 6=1.06; DSS was evaluated using the Hamilton Depression Rating Scale
8=1.04) in relapsing remitting (RR) patients only. Age at (HDRS); cut-off point for depression 8. The patients were
onset of primary progression (PP) did not influence disease divided in two subgroups according to the presence or
evolution. However, age at onset of RR phase, predicted absence of depression. Patients with depression were further
risk of conversion to secondary progression (SP) and of classified as mild, medium and severe
attaining DSS levels. Onset at age 40 (HR=4.22) and at age Results: Depression was found in 41% of the patients. The
50 (HR=6.04), respectively, doubled and tripled risks of duration of treatment was not statistically significantly
developing SP vs. onset at age 20 (HR=2.05). Older onset associated with the presence of depression. Female sex
meant shorter times to disability levels but secondary to (OR=2.46; 95%CI=1.04-5.81) and age >40 years (OR=4.14;
shortened SP latency. Mean age at SP and PP onset was 95%CI=1.76-9.72) were factors associated with depression.
similar (40.2 vs. 38.6 years; p=0.096) and the progressive EDSS parameters statistically significantly associated with
course, preceded or not by relapses was uniform, unaffected depression were: sensitive (p=0.02), cerebellar (p=0.009),
by age at RR onset or by age at SP onset. Among PP and and bowel and bladder (p=0.002). Impaired pyramidal
RR/SP patients median ages at attainment of DSS 6 (49 vs. function was not associated with the presence of depression.
48), DSS 8 (58 vs. 58) and DSS 10 (78 vs. 78) were Conclusion: EDSS parameters associated with depression
strikingly similar. are: sensitive, cerebellar, and bowel and bladder. Duration
Conclusions: Ageing was associated with progressive of treatment is not associated with depression.
phenotypes and with shorter latency to SP, but did not affect
the progressive course. Age independently affects disability
development primarily by acting on probability and latency
of SP.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


346 Posters, Sunday 9 September

P1664 P1665
Grey matter damage predicts the Circadian rhythm genes and multiple
accumulation of disability and cognitive sclerosis (MS)
impairment 13 years later in patients with P. Lavtar1, G. Rudolf1, A. Maver1, L. Lovrei1, S. Risti2,
multiple sclerosis N. Starevi izmarevi2, M. Kapovi2, J. Sepi3,
M. Filippi1, P. Preziosa1, M. Copetti2, G. Riccitelli3, A. Stankovi4, M. ivkovi4, E. Dini5, R. Raievi5,
R. Messina1, G. Comi4, M.A. Rocca1 S. ega Jazbec6, Z. Klemenc Keti7, K. Peterlin Potisk8,
1Neuroimaging Research Unit and Department of Neurology, B. Peterlin1
1Institute of Medical Genetics, Division of Obstetrics and
Vita-Salute San Raffaele University and San Raffaele
Scientific Institute, Milan, 2Biostatistics Unit, IRCCS- Gynaecology, University Medical Center Ljubljana,
Ospedale Casa Sollievo della Sofferenza, San Giovanni Slovenia, 2Department of Biology and Medical Genetics,
3Postgraduate School, School of Medicine, University of
Rotondo, 3Neuroimaging Research Unit, 4Department of
Neurology, Vita-Salute San Raffaele University and San Rijeka, Croatia, 4Laboratory of Radiobiology and Molecular
Raffaele Scientific Institute, Milan, Italy Genetics, Institute of Nuclear Sciences Vina, 5Department
of Neurology, Military Medical Academy, Belgrade, Serbia,
Introduction: We assessed the value of conventional and 6Department of Neurology, University Medical Center
magnetization transfer (MT) MRI quantities of normal- Ljubljana, 7Department of Family Medicine, Medical School,
appearing white matter (NAWM) and grey matter (GM) University of Ljubljana, 8University Rehabilitation Institute,
damage and their 12-month changes in predicting the Ljubljana, Slovenia
accumulation of disability and cognitive impairment in MS.
Background: Multiple sclerosis is a chronic inflammatory
Methods: Brain conventional and MT MRI scans were
demyelinating disease of the central nervous system (CNS).
obtained at baseline and after 12 months in 73 patients,
Evidence from epidemiological studies indicates that
followed clinically for 13 years. At 13-year follow-up,
prevalence of MS varies with geographic latitude, increasing
neuropsychological assessment was also performed
with distance from the equator on both hemispheres. The
whenever possible. At baseline and 12 months,
circadian clock has a bidirectional relationship with diverse
T2-hyperintense and T1-hypointense lesion volume, GM,
physiological processes, including metabolism, CNS
WM, and thalamic fraction, average lesion MT ratio (MTR),
functions, and immune responses. Therefore we
average GM MTR, average NAWM MTR and thalamic
hypothesized that differences in epidemiology of MS
MTR were measured. A multivariate analysis, adjusted for
related to geographical latitude might be associated with
follow-up duration, was performed to identify the predictors
chronobiology. Thus, gene variability in circadian rhythms,
of long-term neurological deterioration and cognitive
genes might be a risk factor for multiple sclerosis.
impairment.
Methods: A total of 900 (82 familial cases) Caucasian
Results: At 13-year follow-up, EDSS rating was performed
patients and 1024 healthy unrelated ethnically matched
in 67 patients and neuropsychological evaluation was done
controls without family history of MS, were included in the
in 31 of them. 42 patients (63%) showed a disability
study. Diagnosis of MS was established according to
worsening (death=7 patients) and 30% were cognitively
McDonalds criteria. Altogether, 8 SNP were included in
impaired. The multivariate model included baseline GM
our study, 4 in CLOCK gene: rs6811520, rs6850524,
MTR (p=0.01, OR=0.97), GM MTR percentage change
rs11932595 and rs13124436; and 4 in ARNTL gene:
after 12 months (p=0.005, OR=1.22), and percentage
3789327, rs1481892, rs4757144 and rs12363415.
reduction of GMF after 12 months (p=0.1, OR=0.91) as
Results: Significant difference in genotype distribution in
independent predictors of disability worsening at 13 years
ARNTL rs3789327 was observed in MS patients when
(r2=0.22, C-index=77%). Baseline GM MTR (p=0.06,
compared to controls (p0.000001).The same statistical
OR=0.83) and disease duration (p=0.04, OR=1.97) were
significance was reached when relapsing-remitting MS and
associated to cognitive impairment (p=0.06, OR=0.83,
patients with the non-familial form of MS were separately
r2=0.86, C-index=99%).
compared to controls. Also, in CLOCK gene rs6811520
Conclusions: GM damage is one of the key factors
statistically significant difference in genotype distribution
associated with long-term accumulation of disability and
(p0.005) was found in all MS patients and non-familial
cognitive impairment in MS.
form of MS when compared with controls.
This study was partially supported by a grant from
Conclusion: We provide evidence for association between
Fondazione Italiana Sclerosi Multipla (FISM2010/R/18)
genetic variation in circadian rhythms genes and MS.
Further studies are required to substantiate the significance
of these genetic variations.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 347

P1666 P1667
Treatment of bladder dysfunctions with MRI findings in Ehlers Danlos syndrome
sacral nerve modulation in a series of misdiagnosed as multiple sclerosis
multiple sclerosis patients R. Peres1, C. Hamonet2, D. Fredy3, D. Ducreux4, F. Pico1
1Neurology Department and Stroke Center, Mignot Hospital,
C. Zuliani1, E. Ostardo2, L. Limido3, M. Pastorello4,
E. Andretta5 Versailles, 2Rehabilitation Medicine Department, Hotel-Dieu
1Neurology, General Hospital, Mirano, 2Urology, General Hospital, 3Neuroradiology Department, Sainte-Anne
Hospital Santa Maria degli Angeli, Pordenone, 3Medtronic, Hospital, 4Neuroradiology Department, Bictre Hospital,
Milan, 4Urology, Sacro Cuore Hospital, Negrar, 5Urology, Paris, France
General Hospital, Dolo, Italy We report a case of a 38-year-old woman who presented a
Introduction: Sacral nerve modulation (SNM) has been complex neurological story first labelled as a primary
recently used in neurogenic bladder dysfunction progressive multiple sclerosis (MS). The major event in her
unresponsive to conventional treatments; aim of our study medical history was a severe traumatic brain injury.
was to evaluate the effectiveness and safety of this treatment Thereafter, she reported various symptoms: a left
in MS patients. hemiparesis with progressive worsening over 5 years, an
Methods: 17 subjects who fulfilled inclusion criteria, 13 episode of transient tetraplegia, sphincter dysfunctions,
females and 4 males (10 RR, 2 SP and 5 PP), mean age vertigos, transient visual disturbance and chronic
49.810.9 years, mean MS duration 13.59.2 years, mean widespread pain. Neurological examination revealed a
EDSS score 5.81.8, underwent surgical unilateral tetra-pyramidal syndrome and ataxia. Evoked potentials
implantation of the InterStim System (Medtronic Inc.), with objectified motor weakness in the left hemicorpus. Cerebral
electrical parameters individually programmed. MRI was normal but cervical spine MRI showed several
Demographic and MS related data were collected and each hyperintense lesions on T2 weighted sequences. Magnetic
patient filled out a questionnaire about the perceived resonance diffusion tension imaging with fibre tracking
changes in micturition symptoms and the improvement in revealed an interruption in both cervical corticospinal tracts
QoL. The indications for SNM were voiding (24%), storage with no activity pattern. Methylprednisolone infusions were
(41%) and mixed (35%) symptoms. The mean follow-up inefficient. After several years of wandering diagnosis, this
was 5226 months. case revealed to be a hypermobile Ehlers Danlos Syndrome
Results: 100% of patients with storage, 75% with voiding (EDS type III). With the appropriate symptomatic therapy,
and 40% with mixed symptoms reported subjective the patient obtained a better quality of life. The clinical
improvements. Statistically significant changes were: in presentation of EDS including pseudo paralysis due to
storage and mixed symptoms a reduction in subjective proprioceptive dysfunction may contribute to erroneous MS
urgency level (p=0.016), in the number of micturitions/die diagnosis. This case underlines the importance to consider
(p=0.012) and pads/die (p=0.033); in voiding and mixed an EDS when faced with an atypical MS, especially when
symptoms a reduction in number of catheterisations/die polyalgias are present, in order to guide patients towards
(p=0.017), residual volumes (p=0.018) and increase in adequate treatment. In addition, the central nervous system
voided volumes (p=0.016). lesions revealed by MRI in this case are consistent with
Conclusion: Our study indicates that SNM could be a MRI findings in other EDS cases (lesions in the corticospinal
therapeutic option in very selected MS patients with storage tracts, the corpus callosum, the arcuate fasciculus...) which
and voiding symptoms refractory to conservative treatments, suggests these lesions could be considered typical of EDS.
while it should be avoided in mixed symptoms. Finally
long-term efficacy of such treatment needs further research,
because MS is typically progressive and hence may have a
variable response over time.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


348 Posters, Sunday 9 September

P1668 P1669
Cerebrospinal fluid biomarkers of amyloid Evaluation of fampridine response by
-metabolism in multiple sclerosis multivariate analysis of mobility in
M. Axelsson1, K. Augutis2, E. Portelius3, G. Brinkmalm3, multiple sclerosis
U. Andreasson3, M. Gustavsson3, C. Malmestrm3, T. Schulthei, S. Sobek, R. Kempcke, T. Ziemssen
J. Lycke3, K. Blennow3, H. Zetterberg3, N. Mattsson3 Department of Neurology, Technical University Dresden,
1Department of Neurosciences, Sahlgrenska Academy, Germany
Institute of Neuroscience and Physiology, Gothenburg,
2Institute of Neuroscience and Physiology, Department of
Introduction: Walking impairment can often be found in
MS. Mobility problems are associated with negative effects
Psychiatry and Neurochemistry, The Sahlgrenska Academy
on daily and occupational life. Fampridine is approved to
at University of Gothenburg, Mlndal, 3Sahlgrenska
improve walking in MS patients with walking disability.
University Hospital, Gothenburg, Sweden
The present study aimed to investigate fampridine response
Background: Amyloid precursor protein (APP) and on walking impairment in a setting of multivariate analysis.
amyloid (A) peptides are intensely studied in Methods: 117 patients (82 women, 35 men) of the MS
neuroscience, and cerebrospinal fluid (CSF) measurements centre Dresden were examined. Initial investigation (V0)
may be used to track metabolic pathways of APP in vivo. was followed by fampridine therapy for 14 days and a
Reduced CSF levels of A and the soluble fragments second investigation (V1) for analysis of treatment effects
-sAPP and -sAPP have been reported in inflammatory afterwards. Quantitative (T25FW, 2-MWT, MSWS-12,
diseases, including multiple sclerosis (MS). However, the FAP-Score of GAITRite system) and qualitative gait
precise pathways of APP degradation in MS are unclear, and parameters (SCGI) were assessed.
it is not known if they may be affected by treatment. Results: 87 patients (74.4%) were classified as responders,
Objective: To characterize CSF biomarkers for APP 30 patients (25.6%) as non-responders. At V0 no significant
catabolism in MS, including the effects of disease modifying differences in all gait parameters could be found between
treatment. responders and non-responders. At V1, after two weeks on
Methods: CSF samples from 90 MS patients and 28 healthy fampridine, responders and non-responders improved non-
controls were analyzed for -sAPP, -sAPP, AX-38/40/42 significantly in T25FW (10.5 vs. 15.4 sec) and 2-MWT
and A1-42 by immunoassays. A subset of samples was (108.6 vs. 90.3 meters). Responders had a marked
analyzed for A isoforms by immunoprecipitation and improvement on FAP score (V0 78.6 vs. V1 81.8; p<0.001).
matrix-assisted laser desorption-ionization time-of-flight MSWS-12 (54.4 vs. 73.9) and SCGI (1.21 vs. -0.14)
mass spectrometry (IP-MS). CSF from relapsing remitting differed significantly between both groups (p<0.001).
(RRMS) (N=29) and progressive patients (N=12), treated Conclusion: Our results confirm positive short-term effects
with natalizumab or mitoxantrone, respectively, were of fampridine on various gait variables in a multidimensional
analyzed at baseline and at follow-up after one to two years setting. Responders improved in all parameters compared to
of treatment. non-responders. Statistical significance could be
Results: CSF -sAPP, -sAPP and A peptide levels were demonstrated in two out of six outcome parameters
reduced in patients compared to controls, but increased (MSWS-12 and SCGI).
towards normal levels after natalizumab treatment. A Overall, using multivariate gait analysis revealed a high rate
multivariate model of IP-MS measured A isoforms of treatment responders (74.4%). Multidimensional
separated progressive patients from controls, with RRMS operationalization of mobility may help identifying a larger
patients having intermediate levels. proportion of fampridine responders.
Conclusions: For the first time, we show that
immunomodulation by natalizumab therapy may counter-
act the altered metabolism of APP in MS. When analyzed
by mass spectrometry, the CSF A isoform distribution was
distinct in progressive patients as compared to controls with
RRMS patients showing less clear alterations.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 349

P1670 P1672
Improving the sensitivity of the criteria for CSF cytology in clinically isolated
the diagnosis of primary progressive syndrome may predict progression to
multiple sclerosis multiple sclerosis
S.B. Kelly, K. Kinsella, M. Duggan, N. Tubridy, Y. Motuzova1, F. Marnetto2, L. Granieri2, P. Valentino2,
C. McGuigan, M. Hutchinson A. Sala2, G. Amadore3, A. Bertolotto2
Neurology, St. Vincents University Hospital, Dublin, Ireland 1Department of Neurology and Neurosurgery, Belorussian

Background and aims: The 2010 McDonald revisions to State Medical University, Minsk, Belarus, 2Neurologia 2 -
the diagnosis of primary progressive MS (PPMS) have Centro Riferimento Regionale Sclerosi Multipla, 3S.C.D.U.
amended the criteria for dissemination in space (DIS) in the di Radiodiagnostica, Azienda Sanitaria Ospedaliera San
brain and spinal cord. Either may be replaced by the Luigi, Orbassano, Italy
presence of CSF oligoclonal bands (CSFOBs). The aim of Introduction: The usefulness of lumbar puncture (LP) at
this study was to evaluate the importance of CSFOBs and the time of clinically isolated syndrome (CIS) is matter of
to analyse the sensitivity of the diagnostic criteria for the debate. Besides, the prognostic role of white blood cell
PPMS. (WBC) count in cerebrospinal fluid (CSF) is not deeply
Patients and methods: Retrospective review of all patients studied. We assessed the value of WBC count and Ig
with a diagnosis of PPMS from 1990-2010. Age and mode oligoclonal bands (IgOB) in CSF of patients with CIS for
of presentation, results of brain and spinal cord MRI and predicting conversion to clinically definite multiple
CSF were documented. The sensitivities of these sclerosis (CDMS).
investigations and of Thompsons and the McDonald Methods: 58 patients with CIS who underwent LP between
Criteria (2001, 2005, 2010) was determined. January 2006 and January 2010 were screened. CSF
Results: Of 95 PPMS patients with a mean age at onset of analysis included WBC count and IgOB. The diagnosis of
41 years, 90% presented with progressive spastic paraparesis CIS and CDMS was made according to the McDonald
or brainstem syndromes. The sensitivity of Thompsons and criteria 2010.
2001 McDonald criteria was 64%; 74% for the 2005, 81% Results: During a median follow-up of 26 months, 41
for the 2010 McDonald criteria. The sensitivity was higher (70.7%) patients converted to CDMS. CSF analysis revealed
for CSF and brain 16/49 than for CSF and cord 3/49 (33% more than 4 WBC/mm3 in 28 (48.3%) patients and CSF
vs. 6%). When criteria requiring two of: 1 MRI brain IgOB in 42 (72.4%) patients. Median WBC count in patients
lesion in two areas typical for demyelination, 1 T2 spinal with CIS who developed CDMS was 5 (range, 0-45) WBC/
lesion or CSFOBs was applied to our cohort, sensitivity mm3, and in patients who did not have CDMS at the end of
increased to 84%. follow-up was 2 (range, 1-12) WBC/mm3 (p=0.0034).
Conclusion: Altering the diagnostic criteria for PPMS so All CIS patients (n=24) with both WBC>5/mm3 and IgOB
that two of: MRI brain with 1 lesion in two areas typical positive developed CDMS after a median period of 9.4
for demyelination, 1 spinal cord plaque or CSFOBs would months (range, 3.4-17.4) after CIS. In the group of patients
increase the sensitivity of the criteria for the diagnosis of who had WBC<5/mm3 and IgOB negative (n=12) only 2
this disease. patients developed CDMS. Only 3/28 patients with
WBC>5/mm3 and 4/42 patients with positive IgOB
remained CIS patients during the follow-up period.
P1671 Conclusion: LP with assessment of WBC and IgOB at the
Inbreeding of MS in the general Faroese time of CIS may play an important role in predicting
population CDMS.
P. Joensen
National Hospital Faroe Island, Torshavn, Faeroe Islands
To estimate the inbreeding in all native Faroese diagnosed
with MS when they were residing on the islands, all patients
diagnosed with MS in the period 1943-2007 are included in
the study, after exclusion of patients of foreign origin, the
inbreed coefficients of 58 patients were estimated and for
these patients an average inbreeding coefficient of 0.0067
was revealed. For 10 control persons without any
neurological disease an average inbreed coefficient of
0.0081 was estimated. Therefore the results point to, that
the inbreeding seems not to be more common between MS
patients than in the general population, however the small
control group must be taken into account.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


350 Posters, Sunday 9 September

P1673 P1674
Visual habituation deficit in patients with Fibrinogen depleting agent batroxobin
multiple sclerosis (final results) has a beneficial effect on experimental
L.S. Bir, E. Degirmenci, C. Erdogan auto-immune encephalomyelitis
Neurology, Pamukkale University, Denizli, Turkey Y. Yang, S.-J. Tian, L.-F. Chen, L. Wu, D.-H. Huang,
Introduction: Visual habituation studies were performed W.-P. Wu
on neurological conditions like migraine, familial General Hospital of Chinese PLA, Beijing, China
hemiplegic migraine and photosensitive epilepsy but as far Objective: Multiple sclerosis (MS) was characterized with
as we know there is no study in multiple sclerosis. In this widespread demyelination and axonal loss. Fibrinogen
study we aimed to investigate the habituation of pattern- (fibrin) deposition was considered as one of the pathogenesis
reversal visual evoked potentials (PR-VEP) in patients with of MS. In the present study, we explored the capacity of
relapsing remitting multiple sclerosis (RRMS). prophylactic and therapeutic potential of fibrin depletion by
Methods: Ten blocks of 1024 responses were analyzed in batroxobin to affect the inflammatory demyelination
terms of peak latencies and peak-to-peak amplitudes of the process in experimental auto-immune encephalomyelitis
maximal negative and positive deflections determined by (EAE) mice model.
visual inspection and habituation was analyzed as the Methods: In MOG-induced EAE, batroxobin was
percentage of amplitude change between the 1st and 2nd to separately injected after immunization immediately
10th blocks. Eyes of patients without any history of optic (prevention) and after the appearance of clinical
neuritis and with P100 latency longer than 110ms were manifestations (suppression). At the time of
classified as group 1 and the eyes of patients with history of postimmunization (p.i.) day 30, 40, 60, spinal cords and
optic neuritis and P100 latency longer than 110ms were cerebellum tissue samples from EAE mice were collected
classified as group 2. A third group consisted of healthy for histopathological and immunohistochemical methods.
control eyes. Molecular biological methods such as Western-blot and
Results: PR-VEP amplitudes showed a significant clear-cut Real-time PCR were supplied to detect MBP, p-Akt, MHC-I
habituation after the first block in the control group but and t-PA.
patient groups did not show any significant decrease in the Results: We observed that treatment with batroxobin
PR-VEP amplitudes of all blocks. significantly ameliorated clinical severity of EAE, reduced
Conclusion: Our electrophysiological study has confirmed inflammatory cell infiltration, demyelination, axonal
the lack of habituation in RRMS patients even if there is no damage and suppressed the activation of astrocytes and
optic neuritis history before. This result would be important macrophages/microglia comprising the CD11b+ population.
for the evaluation of visual system involvement of patients The expression of p-Akt appeared to reduce, while that of
in whom standard VEP examinations are within normal MBP seemed to increase in batroxobin-treated mice as
limits. compared to control mice. In addition, our results showed
that in vitro batroxobin reversed the dentric-like formation
of macrophages irritated by fibrinogen under inflammatory
conditions.
Conclusion: Therefore, strategy targeting fibrin as a
potential therapy for EAE may be beneficial for the
treatment of MS patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 351

P1675 P1676
The influence of host genetics on Epstein- Short-term evolution of spinal cord
Barr virus specific antibody levels in damage in multiple sclerosis: a diffusion
multiple sclerosis patients and controls tensor imaging study
E. Sundqvist1, P. Sundstrm2, I. Lima Bomfim1, M. Thaudin1,2, G. Saliou3, K. Deiva4, C. Denier1,
J. Hillert1, L. Alfredsson3, I. Kockum1, T. Olsson1 D. Adams1, D. Ducreux2,3
1Dept. Clinical Neuroscience, Karolinska Institutet, 1Neurologie, CHU Bictre, 2UMR 788, Facult de Mdecine

Stockholm, 2Ume University Hospital, Ume, 3Institute for Paris Sud, 3Neuroradiologie, 4Neurologie Pdiatrique, CHU
Environmental Medicine, Karolinska Institutet, Stockholm, Bictre, Le Kremlin-Bictre, France
Sweden Introduction: The potential of DTI to detect spinal cord
Introduction: We have previously analysed the association abnormalities in patients with multiple sclerosis has already
of IgG antibodies against Epstein-Barr Nuclear Antigen 1 been demonstrated.
(EBNA1) and EBNA1 385-420 amino acid epitope with Objectives: The objective of this study was to apply DTI
multiple sclerosis (MS). The aim of this study was to techniques to multiple sclerosis patients with a recently
analyse the influence of HLA-A and HLA-DRB1 genotype diagnosed spinal cord lesion, in order to demonstrate a
on EBNA1 IgG and EBNA1:385-420 IgG titres in incident correlation between variations of DTI parameters and
MS cases, and matched population based controls, recruited clinical outcome, and to try to identify DTI parameters
throughout Sweden. predictive of outcome.
Methods: Anti-EBNA1 IgG and EBNA1: 385-420 IgG Methods: A prospective single-centre study of patients with
antibodies were measured in serum, and dichotomised spinal cord relapse treated by intravenous steroid therapy.
based on the median among the controls. The influence of Patients were assessed clinically and by conventional MRI
HLA-alleles was analysed using logistic regression on 987 with DTI sequences at baseline and at 3 months.
controls and 911 MS cases. Alleles with an allele frequency Results: 16 patients were recruited. At 3 months, 12 patients
of over 10% in EBNA IgG low individuals were grouped had clinically improved. All but one patient had lower FA
into HLA-A*X and DRB1*X, respectively. A stepwise values than normal subjects in either inflammatory lesions
selection was applied, adjusting for age at sampling, sex and or Normal-Appearing Spinal Cord and either at baseline or
area of residence, and for EBNA1: 385-420 IgG for a batch- at follow-up. Patients who improved at 3 months presented
effect in ELISA measurements. a significant reduction in the mean ADC (p=0.002) and a
Results: Among MS cases, DRB1*15 was associated to significant increase in FA values (p=0.02) in Normal-
EBNA IgG titres, OR 1.29 (1.0-1.6 95%CI) p=0.02. A*03 Appearing Spinal Cord during the follow-up period.
(OR 1.5, 1.1-2.1, p=0.01), A*24 (OR 1.7, 1.0-2.8, p=0.04), Patients in whom the ASIA sensory score improved at 3
and DRB1*04 (OR 0.6, 0.4-0.8, p=0.001) were associated months showed a significantly higher FA in inflammatory
to EBNA1: 385-420 IgG titres. Among controls, DRB1*03 lesion at baseline (p=0.009) compared to patients with no
(OR 0.7, 0.6-1.0, p=0.03) and DRB1*04 (OR 0.7, 0.6-0.9, improvement.
p=0.003) were associated to EBNA1: 385-420 IgG titres. Conclusion: DTI MRI detects more extensive abnormalities
We observed no association to EBNA1 IgG titres among the than conventional T2 MRI. These abnormalities are
controls. correlated with functional clinical outcome, especially those
Conclusions: We have shown that there is a genetic observed in normal-appearing spinal cord. A less marked
influence on EBNA1 IgG and EBNA1:385-420 IgG titres. decrease in FA value inside the inflammatory lesion was
There are also differences between MS cases and controls, associated with better outcome.
supporting a role for EBV in MS pathogenesis.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


352 Posters, Sunday 9 September

P1677 P1678
Association of promoter resistin gene Impact of OCB status and HLA-DRB1 risk
polymorphism with multiple sclerosis: a alleles on lesion load in MS patients at
possible link to the inflammatory profile age 40
and the age of onset V. Karrenbauer1, R. Prejs2, T. Masterman1, J. Hillert1,
S. Emamgholipour1, A. Hossein-Nezhad2, M.A. Sahraian3 A. Glaser1, K. Imrell1
1Department of Clinical Biochemistry, Tehran University of Clinical Neuroscience, Karolinska Institutet, Stockholm,
1

Medical Sciences, Faculty of Medicine Tehran, 2Tehran Department of Radiology, Karolinska University Hospital,
2

University of Medical Sciences, 3Department of Neurology, Huddinge, Sweden


Sina MS Research Center, Sina Hospital, Tehran University Approximately 95% of Nordic MS patients harbour
of Medical Sciences, Tehran, Iran oligoclonal IgG bands (OCB) in the cerebrospinal fluid.
Introduction: The adipocytokines might participate in the OCB-positive and OCB-negative patients have been
regulation of immunity and inflammation. Considering that reported to be similar with regard to clinical characteristics
immune and inflammatory mechanisms have been but immunogenetically different.
implicated in the pathogenesis of multiple sclerosis (MS), Objective: To address the question if being positive for
resistin as an adipocytokine may provide important insights OCB, carrying HLA-DRB1*15 or carrying HLA-DRB1*04
into underlying mechanisms of the disease. Moreover, have an impact on the risk of belonging to higher lesion
polymorphism in the resistin promoter region, is associated load groups in different neuro-anatomical compartments, at
with alteration of serum resistin. This study investigates the a stage of disease where hypothetical entities may have
association between the polymorphism of RETN-420 C/G reached maximal phenotypically diversity. From a cohort of
(rs1862513) and MS susceptibility, disease behaviour and 2094 MS patients we were able to retrieve 40 OCB-negative
levels of inflammatory mediators (including CRP, IL-1 well characterized patients and a corresponding set of 60
and TNF-) in an Iranian population. OCB-positive patients, who had undergone similar magnetic
Patients and methods: We included a total of 282 resonance (MR) examinations close to their 40th birthday.
participants, 132 of them suffering from three different Lesion load in different brain compartments was manually
types of MS, namely relapsing remitting-MS (RR-MS) calculated by a radioneurologist (co-author RP). Patients
secondary progressive-MS (SP-MS) and primary were assigned to one of four or five categories regarding
progressive MS (PP MS). The RETN-420C/G polymorphism juxtacortical, infratentorial and periventricular lesion load,
and the serum levels of inflammatory mediators IL-1, total amount of T2 lesions and total amount of T1 lesions.
TNF, and CRP were measured. Proportional ordinal logistic regression was used for
Results: The C-allele frequency was higher among the MS evaluating associations.
patients compared with controls, although this difference Results: Being positive for OCB, carrying HLA-DRB1*15
was not significant. Also, MS patients with C/C genotype or carrying HLA-DRB1*04 did not significantly increase
had the lowest age of onset and the highest level of the risk of belonging to higher lesion load groups. However,
inflammatory mediators. In addition, an increase in severity a trend was seen for OCB positive patients experiencing a
of MS was observed in patients with C-allele, after double risk of belonging to infratentorial higher lesion load
clustering patients with PP-MS and SP-MS as a single categories (2.03 (0.91-4.57), uncorrected p-value 0.09).
group. Conclusion: In clinically active patients there seem to be
Conclusion: These data suggest that C-allele of RETN similar risks of belonging to higher lesion load categories
rs1862513 may represent a genetic risk factor for MS in the whether or not patients have OCBs, carry HLA-DRB1*15
Iranian population. Further studies are needed to elucidate or HLA-DRB1*04.
the underlying mechanisms.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 353

P1679 P1680
Overweight as a risk factor for multiple Callosal atrophy in multiple sclerosis is
sclerosis related to cognitive speed
K. Wesnes1,2, T. Riise2, M. Pugliatti3, K.-M. Myhr4 G. Bergendal1, J. Martola2, L. Stawiarz3,4,
Dept. of Neurology, St Olavs Hospital, Trondheim,
1
M. Kristoffersen-Wiberg2, S. Fredrikson3, O. Almkvist5,6
University of Bergen, Norway, 3Institute of Clinical
2 1Division of Clinical Geriatrics, Department of

Neurology, University of Sassari, Italy, 4Neurology and the Neurobiology, Care Sciences and Society, Karolinska
National MS Competence Center, Haukeland University Institutet, 2Department of Clinical Science, Intervention and
Hospital, Bergen, Norway Technology at Karolinska Institutet, Division of Medical
Introduction: It is generally accepted that environmental Imaging and Technology, 3Division of Neurology,
factors contribute to the risk of multiple sclerosis (MS). Department of Clinical Neuroscience, Karolinska Institutet,
4SMILE Image Lab, Karolinska University Hospital-
Some recent studies have shown that increased body size at
age 18-20 gives a twofold risk of MS. We wanted to study Huddinge, Karolinska University Hospital, 5Division of
this association in a large case-control study analyzing body Alzheimer Neurobiology Center, Department of
size at different ages. Neurobiology, Care Sciences and Society, Karolinska
Methods: A population based sample of 959 cases (673 Institutet, 6Department of Psychology, Stockholm University,
Stockholm, Sweden
women and 286 men) and 1718 controls (1256 women and
462 men) in Norway reported their body size using body- Introduction: Long-term changes regarding corpus
silhouettes from 1-9 where 9 was the largest at age 5, 10, 15, callosum area (CCA) and information processing speed in
20, 25, 30 and current age. We analyzed women and men cognitive and sensory-motor tasks have rarely been studied
separately and compared the groups using T-tests and in multiple sclerosis (MS).
logistic regression using body size 3 as a reference and Objective and methods: Information processing speed in
adjusting for smoking and education. cognitive (Symbol Digit Modalities Test, SDMT), sensory
Results: Cases reported larger mean body size than the (Visual and Auditory Reaction Time) and motor (Finger-
controls at all ages except at current age where the opposite Tapping speed, FT; right and left hand) tasks as well as
trend was found. Among men we found an odds ratio (OR) auditory inter-hemispheric transfer (verbal dichotic
1.44 (95% CI 0.65-3.18, p-trend 0.001) for large body size listening, VDL) were related to CCA, measured by MRI at
(6-9) at age 20 and OR 2.01 (95% CI 1.04-3.90, p-trend baseline and at follow-up after nine years in 22 MS patients.
0.005) at age 25. Among women we also found increasing Possible confounding by demographic (age, gender and
OR for increasing body size up to body size 5 where the OR education), clinical (symptom onset, duration, severity of
was 1.43 (95% CI 1.00-2.05, p-trend 0.02) at age 20 and OR disease) and relative brain volume (RBV) as well as lesion
1.31 (95% CI 0.93-1.84, p-trend 0.01) at age 25. load, was taken into account.
Conclusion: Our data confirm that overweight in young Results: The smaller the CCA at baseline, the slower was
adults is a risk factor for MS, especially among men. SDMT performance at baseline. In a similar way, CCA at
follow-up was associated with poor SDMT result at follow-
up. Furthermore, the higher the annual rate of change in
CCA the poorer was the performance in VDL on the left ear
and the more pronounced was the right ear advantage. A
positive relationship between performance in VDL right ear
and annual rate of change in RBV was also seen. Sensori-
motor tests were not significantly associated with CCA.
Lesion load at baseline was associated with FT performance
at baseline. Demographic, clinical and radiological (RBV
and lesion load) characteristics did not confound the
significant relation between CCA and SDMT.
Conclusions: CCA unlike RBV and lesion load was
associated with SDMT, which indicated a marked cognitive
rather than perceptual-motor component.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


354 Posters, Sunday 9 September

P1681 P1682
Impact of fampridine on fatigue, The social and economic costs of a
depression and cognition in multiple multiple sclerosis relapse
sclerosis S. Kelly, M. Duggan, L. Buckley, K. Kinsella,
S. Sobek, R. Kempcke, T. Ziemssen, T. Schulthei C. McGuigan, M. Hutchinson, N. Tubridy
Department of Neurology, Technical University Dresden, Neurology, St. Vincents University Hospital, Dublin, Ireland
Germany Introduction: Disease modifying therapies (DMTs)
Introduction: Fatigue and depression are independent modestly reduce relapse rates in relapsing MS at a mean
predictors of life quality in MS. Fatigue is a main reason for annual drug cost of 15,000. There are few data on the cost
invalidity and early retirement. Cognitive dysfunctions of MS relapses.
cause substantial occupational and social problems in Aim: We identified the costs and socio-economic impact
patients with MS. The present study aimed to investigate associated with mild, moderate and severe relapses.
the influence of fampridine on fatigue, depression and Methods: Patients with an MS relapse from March 2011
cognition in patients with MS. until January 2012 completed a questionnaire detailing
Methods: 117 patients (82 women, 35 mean, median age length of time and earnings lost from work and other
50.1 years) of the MS centre Dresden were examined. The indirect expenses. The overall direct medical costs of mild
initial investigation (V0) was followed by taking fampridine (no treatment or oral prednisolone), moderate (outpatient
for 14 days with a second investigation (V1) for analysis of intravenous methylprednisolone) and severe (requiring
treatment effects afterwards. Qualitative parameters for hospitalisation) relapses were calculated.
fatigue, depression and cognition (Weimus, ADS-L, Results: Of 40 relapses in 34 patients (21 women), 4 were
PASAT) were collected. mild, 27 moderate, and 9 severe. 16 (46%) were receiving
Results: 87 patients (74.4%) were classified as responders, DMTs. Mean expanded disability status score was 2.8 and
30 patients (25.6%) as non-responders. At V0, non- increased to 3.3 during the relapse. Only 21/34 (62%) were
responders showed significantly higher values for physical working prior to relapse. The median total direct costs were
(p=0.009*), cognitive (p=0.048*) and total fatigue 5,807. The median direct and indirect cost of a mild relapse
(p=0.012*). No significant differences were evident for was 760, for a moderate relapse the median cost was
ADS-L (p=0.101) and PASAT (p=0.378) at V0. 1,890 and for a severe relapse, the median length of
At V1, differences between responder and non-responder hospital stay was 11 days at a median cost of 4,768 (mean
groups concerning physical (p<0.001*), cognitive 8,000; range 3,000 24,000). All but one patient
(p<0.001*) and total fatigue (p<0.001*) had increased. returned to work after a median of 9 days after the relapse.
Depression also differed significantly between both groups This individual required an invalidity pension. The median
(p<0.001*), whereas no differences for PASAT (p=0.935) loss of earnings for those returning to work was 4,343.
emerged. Analysis of variance revealed significantly Conclusion: MS relapses result in substantial direct and
different changes between groups over time concerning all socio-economic costs but a significant proportion of patients
fatigue dimensions (p<0.001*, respectively) and depression are not working prior to having a relapse.
(p=0.001*).
Conclusion: Responders and non-responders of fampridine
therapy do not just differ regarding motor-related outcomes.
Responders additionally benefit concerning cognitive and
physical fatigue as well as depression, while non-responders
show no change in these dimensions. Cognitive function
does not seem to be influenced by fampridine.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 355

P1683 P1684
Clinical efficiency of high-dosage Effect of BG-12 in subgroups of patients
immuno-ablative therapy with autologous with relapsing-remitting multiple
stem cell transplantation in multiple sclerosis: findings from the CONFIRM
sclerosis (comparator and an oral fumarate in
A. Kartashov1, A. Kuznetsov1, D. Fedorenko2, relapsing-remitting multiple sclerosis)
V. Melnichenko2, A. Novik2 study
1Neurology, 2Hematology, National Pirogov Center of
M. Hutchinson1, R.J. Fox2, D. Miller3, J.T. Phillips4,
Therapy and Surgery, Moscow, Russia
M. Kita5, E. Havrdova6, J. OGorman7, M. Yang7,
A high-dosage immunoablative therapy with autologous M. Novas7, V. Viglietta7, K.T. Dawson7
stem cell transplantation (HDIT+ASCT) is worldwide 1St Vincents University Hospital, Dublin, Ireland, 2Mellen
applied as one of the new methods of treatment of a multiple Center for Multiple Sclerosis Treatment and Research,
sclerosis. Cleveland Clinic, Cleveland, OH, USA, 3University College
Purpose: To examine the clinical efficiency of HDIT+ASCT Londons Institute of Neurology, NMR Research Unit,
in patients suffering from multiple sclerosis. London, UK, 4Multiple Sclerosis Program, Baylor Institute
Methods: 154 patients (75 (49%) men and 9 (51%) women) for Immunology Research, Dallas, TX, 5Virginia Mason
have been surveyed. Average age is 313.85 years. 72 Medical Center, Seattle, WA, USA, 6Department of
(46.8%) of patients had relapsing-remitting multiple Neurology, First Faculty of Medicine, Charles University in
sclerosis, 47 (30.5%) secondary progressive, 27 (14.7%) Prague, Prague, Czech Republic, 7Biogen Idec Inc, Weston,
primary progressive and 8 (5.1%) progressive-relapsing. MA, USA
Average duration of disease was 8.544.76 years. Degree of Introduction: In the Phase 3 CONFIRM study, BG-12
neurologic deficiency was EDSS 3.721.8 on EDSS scale, (dimethyl fumarate) significantly reduced relapses and MRI
80.2413.5 on SCRIPPS scale. The estimation of activity over 2 years, versus placebo. Here we report on the
HDIT+ASCT efficiency was carried out on the basis of an efficacy of BG-12 in subgroups of relapsing-remitting MS
EDSS, SCRIPPS scales, brain RI and indicators of (RRMS) patients from CONFIRM.
immunoregulatory index prior to the beginning of Methods: Patients were randomized 1:1:1:1 to oral BG-12
HDIT+ASCT, in 3, 6 and 12 months after HDIT+ASCT. 240mg BID or TID, placebo, or subcutaneous glatiramer
Results: After the HDIT+ASCT in the neurologic status acetate (GA) 20mg/day (active reference comparator). The
revealed positive dynamics in 35 (22.7%) patients. Clinical primary endpoint was annualized relapse rate (ARR) at 2
stabilization is revealed in 115 (74.6%) patients. Clinical years. The efficacy of BG-12 was evaluated in pre-specified
deterioration is revealed in 4 (2.59%) patients. It is subgroups stratified by baseline demographic and disease
established that positive RI dynamics is revealed in characteristics, including age, relapse history, prior MS
51.6% relapsing-remitting multiple sclerosis and in 38.2% treatment, and Expanded Disability Status Scale score,
progressive multiple sclerosis (reduction of quantity and among others.
activity of demyelinisation focuses). Indicators of Results: The intent-to-treat population comprised 1,417
immunoregulatory index in early terms after HDIT+ASCT patients, of which 363, 359, 345, and 350 received placebo,
are 0.830.72, in late terms 1.460.72. BG-12 BID, BG-12 TID, and GA, respectively. Both doses
Conclusion: Positive dynamics under the HDIT+ASCT by of BG-12 reduced ARR over 2 years, compared with
data of EDSS, SCRIPPS scales, brain I and the indicators placebo, across all subgroups. For example, treatment with
of immunoregulatory index testifies an effective treatment BG-12 BID and TID reduced the ARR versus placebo at
for multiple sclerosis. 2 years by 48% (rate ratio 0.52 [95% CI 0.36-0.73]) and
63% (0.37 [0.25-0.55]), respectively, in patients who had
1 relapse in the year prior to study entry; 34% (0.66
[0.42-1.04]) and 28% (0.72 [0.47-1.11]) in patients who had
2 relapses; 53% (0.47 [0.31-0.69]) and 55% (0.45
[0.30-0.68]) in patients who had prior MS treatment; and
36% (0.64 [0.44-0.95]) and 46% (0.54 [0.36-0.81]) in
treatment-nave patients.
Conclusion: Subgroup analyses from the CONFIRM study
indicate favourable effects of either BG-12 dosages on ARR
across a wide spectrum of RRMS patients.
Supported by: Biogen Idec Inc.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


356 Posters, Sunday 9 September

P1685 P1686
Effect of BG-12 on magnetic resonance Fasudil, a Rho kinase inhibitor, protects
imaging activity in subgroups of patients spinal cords and vascular endothelial
with relapsing-remitting multiple cells against the inflammatory cell
sclerosis: findings from the DEFINE study infiltrations in experimental auto-immune
A. Bar-Or1, R. Gold2, L. Kappos3, D.L. Arnold4, encephalomyelitis mice
G. Giovannoni5, K. Selmaj6, J. OGorman7, M. Stephan7, J.-Z. Yu1, Y.-X. Li1, Y.-T. Liu1, Y.-H. Li1, C.-Y. Liu1,
K.T. Dawson7 H.-F. Zhang1, L. Feng1, Y. Xie1, J. Meng1, B.-G. Xiao1,2,
1Montreal Neurological Institute and Hospital, McGill
C.-G. Ma1,3
University, Montral, QC, Canada, 2St Josef Hospital, Ruhr 1Institute of Brain Science, Shanxi Datong University,
University, Bochum, Germany, 3Neurology, University Datong, 2Institute of Neurology, Huashan Hospital, Institute
Hospital, Basel, Switzerland, 4NeuroRx Research Inc, of Brain Science and State Key Laboratory of Medical
Montral, QC, Canada, 5Queen Mary University of London, Neurobiology, Fudan University, Shanghai, 3Shanxi
Blizard Institute, Barts and the London School of Medicine University of Traditional Chines Medicine, Taiyuan, China
and Dentistry, London, UK, 6Medical University of Lodz,
Purpose: To investigate the therapeutic effect of Fasudil on
Lodz, Poland, 7Biogen Idec Inc, Weston, MA, USA
expression of ROCK-II and occludin in vascular endothelial
Introduction: In the Phase 3 DEFINE study, BG-12 cells of spinal cords in mice affected with EAE.
significantly reduced MRI activity over 2 years vs. placebo Methods: EAE was induced with MOG35-55 in C57BL/6
in relapsing-remitting MS (RRMS) patients. We assessed mice. Fasudil was intraperitoneally injected in a dosage of
the efficacy of BG-12 on MRI endpoints in patient 50mg/kg/d from day 6 post-immunization (p.i.), and
subgroups from DEFINE. followed by 13 consecutive days. Injection of saline into
Methods: Patients were randomized to BG-12 240 mg BID mice was set up as control. Clinical signs were evaluated
or TID or placebo. Effects of BG-12 on MRI endpoints at 2 and body weight was recorded every other day. Mice were
years were evaluated in patient subgroups by baseline sacrificed on days 28-30 p.i. The expression of ROCK-II in
demographic and disease characteristics, including age, spinal cords was detected by immunofluorescence staining.
prior relapses, treatment history, T2 lesion volume, and The extraction of spinal cords was collected for the detection
presence of gadolinium-enhancing (Gd+) lesions. of occludin expression.
Results: The intent-to-treat population comprised 1,234 Results: The differences of incidence, body weight and
patients; MRI evaluations were performed in a subset of mean maximum clinical scores were statistically significant
540 patients. Both BG-12 dosages reduced numbers of T2 between Fasudil-treated and control EAE mice (p<0.05).
hyperintense and Gd+ lesions over 2 years, compared with Typical muff-like inflammatory cell infiltration and
placebo, across all subgroups. For example, in patients who demyelination were detected in spinal cords of control EAE
had 1 relapse in the year prior to study entry, reductions vs. mice, but were obviously decreased after intervention with
placebo in number of new/newly-enlarging T2 lesions were Fasudil. After the intervention with Fasudil, the expression
90% (95% CI 84-94%) for BG-12 BID and 80% (68-87%) of ROCK-II in vascular endothelial cells of spinal cords was
for TID, while in patients with 2 relapses, relative decreased. Further studies showed that Fasudil increased
reductions were 75% (49-88%) and 66% (33-83%), the expression of occludin in spinal cords.
respectively. In patients who had 1 relapse, there were Conclusions: Fasudil, a Rho kinase inhibitor, decreases the
90% (odds ratio 0.10 [95% CI 0.04-0.24]) and 79% (0.21 incidence rate, relieves the clinical severity in EAE mice
[0.11-0.42]) reductions in the odds of having a greater possibly through the inhibition of ROCK-II production on
number of Gd+ lesions vs. placebo, with BG-12 BID and vascular endothelial cells and the up-regulation of the
TID, respectively, while in patients with 2 relapses, expression of tight junction protein occludin in spinal cords,
reductions were 90% (0.10 [0.02-0.43]) and 59% (0.41 which limits infiltration of inflammatory cells into the CNS,
[0.15-1.13]). and ultimately prevents the development of EAE.
Conclusion: Both BG-12 dosages demonstrated benefits on
MRI endpoints across subgroups of RRMS patients with
different disease characteristics.
Supported by: Biogen Idec Inc.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 357

P1687 P1688
The glutathione and S-nitrosothiols Neurological deficit in patients with
interaction within CNS during the acute multiple sclerosis and subclinical
phase in an experimental model of hypothyroidism
multiple sclerosis E. Kiseleva1, N.N. Spirin2
1Neurology, 2Neurology with medical genetics and
S. Ljubisavljevic, I. Stojanovic, R. Pavlovic,
D. Sokolovic, D. Pavlovic Neurosurgery, Yaroslavl State Medical Academy, Yaroslavl,
Faculty of Medicine, University of Nis, Serbia Russia
Objective: Multiple sclerosis (MS) is characterized by Introduction: Thyroid pathology is frequently detected in
inflammatory processes that are associated with nitric oxide multiple sclerosis patients (MS). The aim of this study was
(NO) and its related species production in CNS, which can to evaluate the relationship between the neurological
nitrosylate protein thiols and modulate their structure and features of MS and thyroid dysfunction.
function, also reducing the CNS content of redox active Methods: 65 patients (17 males, 48 females) with clinically
compounds, such as glutathione (GSH). definite relapsing-remitting MS were examined. All patients
Method: We have evaluated the relationships between had clinical remission. The mean age was 36.8 years (19 -
S-nitrosothiols (RSNO) and GSH in an experimental model 61 years). Severity of neurological deficit was assessed
of MS - experimental autoimmune encephalomyelitis according to the EDSS, the Functional Systems Score
(EAE), during treatment with inhibitor of inducible NO (FSS). We have performed ultrasound study of the thyroid
synthase - aminoguanidine (AG) and thiol donor molecule gland, analysis of serum thyroid hormones and antithyroid
- N acetyl cysteine (NAC). EAE was induced by the myelin antibodies. Statistical analysis was performed using
basic protein, dissolved in phosphate buffered saline (PBS) standard statistical methods.
emulsified in the complete Freunds adjuvant (CFA), also, Results: Subclinical hypothyroidism was diagnosed in
with injections of Pertussis toxin. Animals were assigned to: 13.8% of patients with MS, 55.5% had thyroid pathology
control (PBS), EAE, CFA, EAE+AG, AG, EAE+ NAC and without dysfunction. 30.7% of patients without thyroid
NAC group, were scored daily for the clinical signs of EAE. pathology were the comparison group. 66% of patients
RSNO and GSH were evaluated in - whole encephalitic received -IFN-1b in group with subclinical hypothyroidism;
mass (WEM) and cerebellums. in the comparison group, only 20%. The mean FS scores for
Results: The clinical signs of EAE were reduced in EAE pyramidal and sensory functions were higher in patients
treated animals compared to the other groups (p<0.05). with subclinical hypothyroidism, than in the comparison
RSNO concentration was increased in EAE untreated group (pyramidal FSS 3.11.02 and 2.41.08, sensory FSS
animals compared to the EAE AG and NAC treated 2.60.71 and 1.91.12, p<0.05). 77% of patients with
(p<0.05). Also, during treatment with AG and NAC, GSH subclinical hypothyroidism had polyneuropathic type of
concentration was more increased than in untreated animals sensory dysfunction.
(p<0.05). Conclusion: Thus, the occurrence of the polyneuropathic
Conclusion: The finding of our work suggests a potential type of sensory dysfunction in patients with multiple
role of RSNO and GSH in early clinical presentation of sclerosis can be regarded as a relative marker of subclinical
experimental MS that might be also useful as predictive hypothyroidism. Such patients should conduct a screening
parameters for MS treatment based on increased GSH and of the thyroid gland.
thiol pool in CNS.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


358 Posters, Sunday 9 September

P1689 P1690
Neuropathic pain: a disabling symptom of Olfactory dysfunction in patients with
neuromyelitis optica primary progressive multiple sclerosis
K. Mutch, L. Elsone, A. Jacob F. Schmidt, O. Goektas, F. Fleiner, L. Harms
Neurology, Walton Centre Foundation NHS Trust, Liverpool, Charit Universittsmedizin Berlin, Germany
UK Introduction: The olfactory function is diminished in
Background: Neuromyelitis Optica (NMO) is an patients with multiple sclerosis (MS). Recently we could
inflammatory demyelinating disorder of the central nervous detect a correlation between the olfactory bulb and olfactory
system characterised by relapses affecting optic nerves and brain volumes of MS patients with their lesion load. This
longitudinal extensive transverse myelitis resulting in visual study focuses on the olfactory function of patients with
and physical disabilities and often neuropathic pain. Little primary progressive MS (PPMS).
is known regarding the occurrence, type and intensity of Design and methods: 14 patients with PPMS and 14
pain in NMO. matching control persons were examined. Exclusion criteria
Objective: The aim of the study was to investigate the were olfactory dysfunction with a different genesis (post-
frequency and features of neuropathic pain in NMO and its viral, sinunasal, post-traumatic, toxic) and age >65 years.
impact on Quality of life. Olfactory testing was performed using the tripartide TDI-
Method: 58 patients, with a diagnosis of NMO or NMO test measuring olfactory threshold, the discrimination and
spectrum disorder (those with myelitis only) using the identification of different odours.
Wingerchuk Criteria, attending the National NMO clinic Results: 13 of 14 PPMS patients (93%) and none of the
over one year were assessed. 44 patients had aquaporin-4 control persons showed hyposmia. The mean TDI-score of
antibodies. The Brief Pain Inventory (BPI) short form tool patients with PPMS was 27.52.9, the mean Threshold-
was used to assess pain and its effect on quality of life. score (T-score) 5.61.4, the mean Discrimination-score
Results: 33/58 (57%) patients reported neuropathic pain. 10 (D-score) 9.51.9 and the mean Identification-score
(17%) patients described pain as their worst symptom. 25 (I-score) was 12.41.6. For the control persons, the mean
(76%) patients were in constant pain for more than 6 TDI-score was 34.02.2, the T-score 6.92.1, the D-score
months. An increase in pain intensity was associated with a 12.61.6 and the I-score was 14.50.9. There was a
new relapse in 16 patients. Descriptions of pain included significant difference between the TDI-score (p<0.0001),
burning, pricking shooting, nagging and exhausting. Pain the D-subscore (p<0.0001) and the I-subscore (p<0.001) of
interfered with activities and quality of life for 41 (71%) of patients with PPMS compared with the control group.
patients. Activity increased pain in 10 patients and 18 Conclusions: Hyposmia appears frequently in patients with
patients had no pain during sleep or lying still. PPMS. Odor discrimination and identification of PPMS-
Conclusion: Neuropathic pain is a significant symptom of patients seems to be worse than odor threshold compared to
NMO. It is essential to acknowledge, access and manage the healthy control group. Demyelination with axon
patients pain in order to improve their quality of life. destruction results in chronic progredient progression which
might explain the decreased olfactory function of PPMS-
patients.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 359

P1691 P1692
Validity and reliability of Persian minimal Assessment of the efficacy of
assessment of cognitive function in rehabilitation therapy in patients with
multiple sclerosis relapsing-remitting course (RR) of
A. Eshaghi1, S. Riyahi-Alam1, T. Roostaei1, G. Haeri1, multiple sclerosis (MS) and mental
A. Aghsaie1, M.R. Aidi1, H.R. Pouretemad2, M. Zarei3,4, fatigue: an fMRI study
S. Farhang1, R. Saeedi1,2, A. Nazeri1, H. Ganjgahi3, Z. Rozhkova1, O. Omelchenko1, T. Kobys2
A.R. Azimi1, R. Benedict5, M.A. Sahraian1 1Radiology, Medical Clinic BORIS, 2Neurology, National
1Sina MS Research Center, Tehran University of Medical Medical Bogomolets University, Kiev, Ukraine
Sciences, 2Department of Psychology, Shahid Beheshti
University, 3Brain Mapping Research Center, Brain Mapping Objectives: We use movement tasks for assessment of the
Research Center, Tehran University of Medical Sciences, therapy efficacy in patients with RRMS and mental fatigue
Tehran, Iran, 4FMRIB Centre, John Radcliffe Hospital, before and after rehabilitation therapy.
University of Oxford, Oxford, UK, 5Department of Methods: Two groups of patients were studied by fMRI on
Neurology, Buffalo School of Medicine, Buffalo, NY, USA 1.5T SIGNA EXCITE (GE). The 1st group includes 58 right
hand patients (22-43y) with RRMSG, the 2nd group (CG)
Introduction: Cognitive dysfunction is common in multiple
includes 20 volunteers (23-40y). fMRI data were obtained
sclerosis and validated batteries are limited in languages
using block design parametric paradigm, EPI:TR/
other than English.
TE=3000/60ms, matrix 64x64, FoV=24. We used complex
Objective: We aimed to translate, to cross-culturally adapt,
hand movement task, which consisted of finger tapping,
to validate, and assess reliability of Minimal Assessment of
successive fingers tapping, and hand squeezing subtasks
Cognitive Function in MS (MACFIMS) in Persian, which
under following scheme: 2phase DEL-7rest-7finger-7rest-
is a well-constructed battery in MS literature.
7all fingers-7rest-7hand-7rest-7finger-7rest-7all fingers-
Patients and methods: The battery was adapted to Persian
7rest-7hand-7rest-7finger-7rest-7all fingers-7rest-7hand-
in accordance with available guidelines. A total of 158 MS
7rest. All fMRI data are obtained before (as the baseline
patients and 90 controls underwent neuropsychological
fMRI) and after rehabilitation therapy.
assessment. For reliability assessment, the battery was
Results: From analysis of fMRI data (using FSL software
re-administered in a subset of 41 patients after a short
package) we found no differences between the total
interval using alternate forms to mitigate practice effects
MS-lesions load. In the baseline fMRI examination the
(approximately 10 days).
bilateral activation of the sensori-motor cortex, thalami, and
Results: Patients performed significantly worse than
cerebellum was observed. After rehabilitation therapy we
controls in all cognitive tests supporting discriminant
found a statistically significant reduction of the activation
validity of our adapted battery. Approximately half of the
in the ipsilateral primary motor cortex area, in the
patients (46.2%) showed cognitive impairment as defined
contralateral primary motor cortex area, and in the thalami
by the impairment in two or more tests. Symbol Digit
bilaterally. In the cerebellum a significant reduction of the
Modalities Test was the most robust test by ROC analysis.
activation areas was observed.
All tests were acceptable and had a good level of reliability.
Conclusion: fMRI method gives us quantitative approach
Conclusions: This is the first validation of gold-standard
for evaluation of the cortical reorganization during complex
cognitive testing in Persian. Persian MACFIMS shows
hand movement tasks in patients with RRMS. Although the
nearly the same psychometrics as its English counterpart.
pathological findings in the demyelinating plaques are
diverse, the comparison fMRI data in patients with RRMS
and mental fatigue provides us very useful information for
estimation of the efficacy of rehabilitation therapy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


360 Posters, Sunday 9 September

P1693 P1694
Lactonase activity of paraoxonase-1 in Factors relating to unmet needs of Irish
multiple sclerosis patients: a pilot study MS patients
A. Jamroz-Wisniewska1, J. Betowski2, Z. Stelmasiak1, R. Lonergan1, K. Kinsella1, P. Fitzpatrick2, M. Duggan1,
H. Bartosik-Psujek1 S. Jordan1, D. Bradley1, M. Hutchinson1, N. Tubridy1
1Neurology, 2Pathophysiology, Lublin Medical University, Department of Neurology, St. Vincents University Hospital,
1

Lublin, Poland School of Public Health and Population Science, University


2

Background: The lactonase activity of paraoxonase-1 College Dublin, Dublin, Ireland


(PON1), an antioxidant enzyme bound with HDL in serum, Background: Non-pharmacological MS-related needs may
is thought to be the most important in physiology. As the be overlooked during fund allocation. Identification of
role of oxidative stress in the pathogenesis of multiple unmet needs (UN) and associated clinical/demographic
sclerosis (MS) is considered to be significant, we studied factors may inform service planning and guide resource
this physiological activity of PON1 in MS. allocation to optimise care.
Patients and methods: 57 MS patients - 21 patients with a Aim: To identify unmet MS-related needs and associated
relapsing-remitting type of disease (in remission) and 36 factors in Irish MS patients.
patients with a secondary progressive type of disease, and Patients and methods: Observational study in 3 regions:
44 control subjects were involved in the study. Lactonase South Dublin (urban), Donegal and Wexford (rural).
and arylesterase activities of PON1 (homocysteine Patients completed a validated Needs Assessment
thiolactone and phenyl acetate as substrates) were measured Questionnaire (NAQ). Univariate and multivariate analyses
spectrophotometrically. were performed.
Results: There were no statistically significant differences Results: 325 patients completed NAQ: 52.3% reported UN
in mean values for serum lactonase (p=0.8) and arylesterase relating to MS: 1 UN in 27%, 2 in 73%, 5 in 24%.
(p=0.6) activities of PON1 between the studied groups. Univariate analysis: UN related to EDSS >6.5 (p<0.001),
Conclusions: In groups of MS patients, compared to the progressive subtype (p<0.001), increased age (p=0.003),
control group, no statistically significant differences in MS duration (p=0.003). By multivariate analysis: increasing
PON1 lactonase and paraoxonase activities were detected. EDSS and rural residence (p<0.05).
UN included: physiotherapy 57.1%, social 43.5%, financial
33.5%, employment 29.4%, occupational therapy 28.8%,
home adaptations 27.7%.
Discussion: Over 50% reported UN, suggesting that non-
pharmacological needs are suboptimally addressed,
particularly in older, more disabled patients with progressive
MS. Increasing EDSS and rural residence survived
multivariate analysis, highlighting need for increased fund
allocation, especially towards community support and
multidisciplinary service development. Identifying unmet
needs and associated factors may inform service planning
and emphasizes need for improved resources.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 361

P1695 P1696
Bilateral horizontal gaze paralysis and Preparation-specific genetically
facial diplegia due to a pontine tegmental determined risk of developing neutralizing
plaque in multiple sclerosis antibodies towards interferon-beta in
E. Papageorgiou, I. Markakis, V. Koukouni, G. Gekas patients with multiple sclerosis
Neurology, General State Hospital of Piraeus, Nikaia, J. Link1, K. Fink1,2, M. Lundkvist1, B. Brynedal1,
Greece I. Kockum1, A. Fogdell-Hahn1, J. Hillert1,2
Introduction: Brainstem involvement is common during 1Centre for Molecular Medicine, Karolinska Institutet,
the course of multiple sclerosis (MS) and usually manifests 2Department of Neurology, Karolinska University Hospital,
as internuclear ophthalmoplegia, monocular abduction Stockholm, Sweden
deficits, trigeminal symptoms, vestibular signs and Introduction: A significant proportion of patients with
unilateral facial palsy. However, acute multiple cranial multiple sclerosis (MS) receiving interferon-beta (IFN-
nerve palsies have been rarely reported. We describe a beta) develop neutralizing antibodies (NAbs). However,
patient with MS who developed bilateral horizontal NAbs only occur in a subset of patients, which indicates
ophthalmoplegia and facial diplegia due to a demyelinating that factors such as human leukocyte antigen (HLA)
lesion in the caudal pontine tegmentum. genotype could be involved in determining the risk for
Case-report: A 44-year-old woman with a 6-year history of NAbs.
relapsing-remitting MS, presented with complaints of We investigated if certain HLA class I and II alleles are
blurry vision and nausea for one week. Neurological associated with the development of NAbs in a Swedish
examination revealed complete bilateral horizontal gaze cohort of MS patients treated with IFN-beta and whether or
paralysis involving saccades, pursuit and oculocephalic not an association with clinically relevant NAb-titres exists.
reflexes, with preserved convergence and vertical eye Method: 419 patients with known HLA status matched the
movements. One day following admission the patient also strict criteria for NAb-positivity (n=230) or NAb-negativity
developed facial diplegia. CSF had a normal cell count and (n=189). Precise titres were measured with RT-PCR (MxA
protein with positive oligoclonal bands. Brain MRI revealed expression). Cut-off for clinically relevant titres was defined
chronic demyelinating lesions in the periventicular white as an NAb-titre higher than 150TRU/ml. Statistical
matter and cerebellum and an acute lesion in the dorsal dependencies were evaluated with chi square test, logistic
pontine tegmentum involving both facial colliculi. regression and Wilcoxon rank sum test.
Treatment with intravenous methyl-prednisolone led to a Result: The risk of developing NAbs was higher when
fast improvement of facial diplegia; the gaze deficit started treated with Rebif or Betaferon compared to Avonex.
to improve gradually following subsequent trials of plasma Patients, treated with IFNb1-a, were more often positive for
exchange and was completely restored within six weeks of NAbs if they were carriers of DRB1*15, whereas HLA-
relapse onset. A*02 influenced the risk of NAbs in patients treated with
Conclusion: To our knowledge, this is the first reported IFNb1-b. Nevertheless, there was no allele group that
case of complete horizontal ophthalmoplegia associated decreases the risk of NAbs lower than that of Avonex in
with facial diplegia in MS. The lesion topography in our either Betaferon or Rebif treated groups and no allele group
patient suggests bilateral involvement of infranuclear and increased the risk of NAbs on Avonex higher than the risk
intranuclear abducens fibres as well as of the neighbouring in Betaferon or Rebif treated groups.
inner genu of facial nerves. Thus MS should be included in Conclusion: The development of NAbs is influenced by
the differential diagnosis of this rare clinical syndrome. HLA genotypes in some part, but is mainly dependent on
the choice of the IFN-beta preparation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


362 Posters, Sunday 9 September

P1697 P1698
Hiccups and vomiting in neuromyelitis Human endometrial stem cells attain
optica oligodendrocyte features in vitro
S. Oumari, Y. Benmansour, A. Araqi-Houssaini, S. Ebrahimi Barough1, H. Mohseni Kouchesfehania2,
H. Elotmani, B. Elmoutawakil, M.A. Rafai, I. Slassi J. Ai3, B. Younesi1, M. Massumi4
CHU IBN Rochd, Casablanca, Morocco 1Biological Science, 2Tarbiat Moallem University, 3Tissue
Introduction: In addition to optic nerve and spinal cord Engineering, School of Advanced Medical Technologies,
4Department of Animal and Marine Biotechnology, National
pathology, neuromyelitis optica (NMO) has been reported
to have brainstem involvement resulting in intractable Institute of Genetic Engineering and Biotechnology, Tehran,
hiccups with persistent nausea and vomiting. Iran
Methods: We retrospectively studied patients who Introduction: Human Endometrial-derived stem cells
presented vomiting and hiccups during a relapse of NMO (EnSCs) are the abundant and easy available source for cell
spectrum disorders. replacement therapy. Oligodendrocytes are myelinating
Results: Among 31 patients treated for NMO, 4 (13%; 3 cells in the central nervous system (CNS) that form the
women and 1 man; 47 years mean age) were included. 3 myelin sheath of axons to support rapid nerve conduction.
patients had initially consulted a gastroenterologist because In CNS disorders, such as stroke, multiple sclerosis and
these symptoms preceded the neurological deficit. Vomiting spinal cord injury, demyelination of axons contributes to
lasted a median of 47 days (range: 4 days - 4 months). 3 functional deficit.
patients were seropositive for anti-aquaporin-4 and MRI Material and methods: The characterized cells were
showed area postrema involvement in all cases. These coaxed to oligodendrocyte differentiation by induction of
symptoms responded favourably to corticosteroids only bFGF, EGF and PDGF-AA (20ng/ml) signalling molecules
because symptomatic treatments were ineffective. for 21 days and T3 (30ng/ml) for 8 days. Differentiated cells
Discussion: Our data confirm that the hiccups and vomiting were analyzed for expression of neuronal markers by
frequently are the initial symptoms of NMO and the RT-PCR and Immunocytochemistry.
involvement of the area postrema is an important starting Results: The flow cytometric analysis showed that EnSCs
point in pathophysiology of NMO spectrum disorders. were positive for CD90, CD105, OCT4 and were negative
for CD31, CD34. The result showed the expression profile
of oligodendrocyte markers such as Nestin, Olig2, PDGFRa,
CNP in the level of mRNA. The expression of Olig2,
PDGFRa, A2B5 proteins in EnSCs were confirmed 30 days
post-treatment (PT) by Immunocytochemistry.
Conclusion: The EnSCs can response to the signalling
molecules which are usually used for oligodendrocyte
differentiation and can program to oligodendrocyte cells
and may convince to consider these cells as a unique source
for cell therapy of neurodegenerative disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 363

P1699 P1700
Valproic acid induces the expression of Acute psychosis masquerading Susacs
the EAAT3 glutamate transporter in syndrome
human oligodendroglioma cells M.G. enol1, S. Demir1, A. Ayata2
M.G. Bianchi, L. Reia, M. Allegri, R. Sala, M. Chiu,
1Neurology, GATA Haydarpaa Training Hospital,
R. Franchi-Gazzola, J. Uggeri, O. Bussolati
2Neurology, GATA Haydarpaa Eitim Hastanesi, Istanbul,
Dept. of Experimental Medicine, University of Parma, Italy Turkey
Introduction: Glutamate transport has never been studied Purpose: To report clinical findings and course of a patient
in human oligodendroglioma cells, although the neuronal initially presenting acute psychosis, which was recognized
transporter EAAT3 is highly expressed in Oligodendrocyte as Susacs Syndrome in further evaluation.
Precursor Cells. On the other hand, transcriptional Methods: A 25-year-old man has been admitted to a
regulation of EAAT3 is still incompletely characterized and psychiatry clinic with acute psychotic symptoms including
EAAT3 role in oligodendrocytic differentiation unknown. memory loss and amnesia, confusion, poor co-operation
Conflicting evidence has been reported on the effects of and awareness of surroundings. He had been also referred
HDAC inhibitors, such as valproic acid (VPA), on to a neurology clinic because of difficulty in walking. He
myelinating cells and glutamate transporters. has been initially diagnosed as multiple sclerosis due to the
Methods: Gene expression has been studied with RT-PCR suspicious MRI findings and had been referred to our
and Western Blot. EAAT3 activity has been determined neurology department for further investigation. Detailed
from the initial influx of the glutamate analogue D-aspartate. neurologic and ophthalmologic examination, including
Results: EAAT3 expression and activity, barely detectable MRI, fundus fluorescein angiography (FFA) and optical
in human oligodendroglioma Hs683 cells under control coherence tomography (OCT), were performed. According
conditions, are markedly induced by valproic acid (VPA). to the ophthalmic and neurologic findings, the patient was
EAAT3 induction is detected at both mRNA (3x) and diagnosed as Susacs syndrome.
protein levels (10x after 72h of treatment). Under the same Results: His visual acuity and hearing level were not
conditions, VPA does not change EAAT3 expression in evaluated at admission because of low co-operation. MRI
human U373 glioblastoma cells. Transport activity is also revealed multifocal hyperintense callosal lesions. On the
increased by VPA and exhibits typical EAAT3 features, fundoscopic examination multiple peripheral arteriolar
such as stimulation by phorbols and insensitivity to occlusions and related retinal oedema were observed and
dihydrokainate and UCPH. While no saturable glutamate occlusions were confirmed with FFA. OCT imaging also
transport is detectable in control Hs683 cells, VPA-treated confirmed the retinal thickening and oedema. The patient
cells exhibit a high affinity transport activity (Km 7M), the has been treated with high dose (1gr/day) methyl
Vmax of which is increased by phorbol. EAAT3 mRNA prednisolone and none of the symptoms was healed. Steroid
induction is associated with a two-fold increase of PDGFRA therapy was stopped and intravenous IgG (IVIG) was
mRNA, a marker of early Oligodendrocyte Precursor Cells, infused at a dose of 0.5mg/kg daily for five consecutive
while CNP, TUBB3 and GFAP mRNAs remain unchanged. days. The patient responded well to the IVIG infusion and
Conclusions: The VPA-dependent induction of the complete reversal of neurologic and psychiatric symptoms
glutamate transporter EAAT3 in human oligodendroglioma were observed.
cells may represent an indicator of early commitment to Conclusion: Susacs syndrome is a very rare disorder and
oligodendrocytic differentiation. initial presentation may lead to misdiagnosis or cause delay
Aided by FISM, Genova, Italy as happened in this case. Multi-disciplinary approach may
help accurate diagnosis.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


364 Posters, Sunday 9 September

P1701 P1702
Optical coherence tomography (OCT) in Conus medullaris syndrome as a
patients with multiple sclerosis (MS) complication of radio-isotope
Z. Vladimirova, S. Cherninkova cisternography
Neurology, University Hospital Aleksandrovska, Sofia, S.-H. Koh1, H.-J. Yu2, B.-S. Park1, J. Park3, K.-Y. Lee1,
Bulgaria Y. Lee1, J. Kim1
Introduction: OCT is a non-invasive method for 1Hanyang University College of Medicine, Seoul,
examination of the retina that can be used as a tool for 2Neurology, Bundang Jesaeng Hospital, Seongnam,
assessment of the course of neurodegeneration in MS by 3Neurology, Inje University Paik Hospital, Pusan, Republic

measurement of the retinal nerve fibre layer (RNFL) as of Korea


axonal structure (showing loss of axons), and by Objective: Conus medullaris syndrome (CMS) is a clinical
determination of the macular ganglion cell layer volume neurologic syndrome caused by a conus medullaris lesion.
(showing loss of neurons). CMS is a heterogeneous entity with various aetiologies
Aim: To measure the thickness of RNFL and the macular such as trauma or a space-occupying lesion. Multiple cases
ganglion cell layer in MS patients and compare it with of CMS following spinal anaesthesia have been reported,
healthy volunteers. but CMS after radioisotope (RI) cisternography has not yet
Material and methods: We have examined 15 MS patients been reported.
(age 10-54), separated in three groups - with acute optic Methods: We present four patients who developed CMS
neuritis (ON), with ON in the past, and patients who have after RI cisternography.
never had ON. We have compared the findings with those Results: All experienced neurological deficits such as
of 17 healthy volunteers (age 20-44) with no ocular or paraparesis, sensory loss, and urinary incontinence three to
system diseases. four days after RI cisternography. Two showed abnormalities
Results: We found changes in RNFL thickness and in on lumbar magnetic resonance imaging, and three had
macular ganglion cell volume in almost all MS patients, complete symptom resolution within ten weeks.
with most advanced thinning in patients with ON in the past Conclusions: The pathomechanism of CMS is unclear, but
(more than 6 months ago). The patients with long history for we hypothesize that RI neurotoxicity might be responsible.
MS, who have never had ON also showed changes in RNFL It is possible that the use of low-dose 99mTc-DTPA or an
thickness, but not so advanced. The most subtle changes alternative diagnostic tool such as magnetic resonance
were found in patients with acute ON (even the most severe cisternography could help to prevent this complication.
cases, e.g. with only light perception), probably due to the
time needed for development of such atrophic alterations.
Conclusion: The patients with MS show thinning of RNFL
and the ganglion cell layer that are representative for the
P1703
course of neurodegeneration in the whole organism. Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 365

P1704 P1705
Risk of multiple sclerosis after optic Vitamin D3 supplementation in relapsing
neuritis in patients with normal baseline MS: considering the safety issues
MRI S.M. Nabavi1, S. Zari2, D. Morsali3
1Neurology, Shahed University, Tehran, 2Shahed University,
I.B. Marques1, M.C. Macrio1, F. Matias1, E.D. Silva2,
L. Sousa1 Teharn, Iran, 3UCL, London, UK
1Neurology, 2Ophthalmology, Coimbra University Hospital,

Coimbra, Portugal
P1706
Introduction: Optic Neuritis (ON) is the initial presentation
of multiple sclerosis (MS) in approximately 20% of patients. Reversible therapy-related dysplastic
Although abnormal baseline brain MRI is the strongest hematopoiesis following interferon beta
conversion predictor, patients with normal MRI can also therapy in multiple sclerosis patients: 2
develop MS. In these patients predictors of conversion are case reports
still to be determined. Our objective is to estimate MS risk
S.M. Nabavi1, A. Hamzehloo2, J. Shams2
and identify conversion predictors in ON patients with 1 Neurology, 2Shahed University, Teheran, Iran
normal baseline MRI.
Methods: Retrospective chart review of patients with
idiopathic ON and normal MRI, presenting to our hospital P1707
between 2003 and 2010. Demographic, clinical, paraclinical
and treatment data were collected and analyzed. Abstract cancelled
Results: 42 patients were included. 10 patients converted to
MS, seven during the first 2 years and the remaining during P1708
the next 3 years. 5-year MS conversion rate was 23.8%. A
Homocysteine, vitamin B12 and folate
statistically significant increase in MS conversion rate was
associated with retrobulbar ON (p=0.022), a history of levels in Iranian patients with multiple
previous symptoms suggestive of demyelination (p=0.002), sclerosis: a case control study
positive oligoclonal bands in CSF (p=0.004) and incomplete M. Moghaddasi1, H. Razjouyan2, N. Mohebi1,
recovery (p=0.002). Age, sex, ON laterality, other M. Aghaei1, M. Mamarabadi1
neurological exam abnormalities, abnormal visual evoked Tehran University of Medical Sciences, Tehran, Iran,
1

potentials, corticosteroid treatment or ON recurrence were Drexel University, Monmouth Medical Center, Long Branch,
2

not associated with a significant increase in MS conversion NJ, USA


rates.
Conclusion: A considerable proportion of patients with ON
and normal baseline brain MRI will develop MS. The risk P1709
of conversion appears to be higher during the first 2 years, Quality of life assessment in patients with
so follow up, at least during this period, is advisable. Early MS receiving interferon beta-1a:
immunomodulatory treatment may be considered
individually, especially in patients with multiple factors a comparative longitudinal study of
associated with increased MS conversion risk. Avonex and its biosimilar cinnovex
R. Abolfazli1, A. Hosseini2, K. Gholami1, M. Javadi1,
H. Torkamandi1, S. Emami2
Tehran University of Medical Sciences, Tehran, 2Azad
1

University, Tehran, Iran

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


366 Posters, Sunday 9 September

P1710 P1714
Cognitive performance in a cohort of MS Correlation of blink reflex abnormalities
patients in comparison to healthy and magnetic resonance imaging findings
subjects: an assessment with BRB-N from in multiple sclerosis patients
Iran E. Degirmenci, C. Erdogan, L.S. Bir
S.M. Nabavi1, S. Tahbaz2, Z. Behjati2, F. Nourbala2, Neurology, Pamukkale University, Denizli, Turkey
S. Sadeghi3, Z. Saeedi2, MS Research Unit, Mostafa
Center P1715
Neurology, Shahed University, 2Tehran University, 3Shahed
1

University, Tehran, Iran Epidemiological and clinical


characteristics of multiple sclerosis in the
P1711 Tomsk region, Russian Federation
M. Titova, V. Alifirova
An open-label, survey-based, multicenter Neurology and Neurosurgery, Siberian State Medical
study to determine patient satisfaction University, Tomsk, Russia
with single-use prefilled AVONEX PEN
auto-injector in multiple sclerosis patients P1716
M. Morreale1, T. Campbell2,3, V. Migounov4, E. Cass1,
S. Wong1 Development of a T-cell vaccination (TCV)
1Innomar Strategies Inc., AmerisourceBergen Canada, program for chronic progressive multiple
Burlington, ON, 2Capital District Health Authority, sclerosis (CPMS). 18 years of clinical
3Dalhousie University, Halifax, NS, 4Biogen Idec Canada
experience
Inc., Mississauga, ON, Canada
G.A. Moviglia, G. Varela, M.T. Moviglia Brandolino,
N. Blasetti, S. Piccone, G. Albanese, C. Gaeta
P1712 CIITT, Universidad Maimnides, Caba, Argentina
Effects of natalizumab on global oxidative
stress in patient with multiple sclerosis P1717
E. Aguera1,C. Bahamonde1, F. Sanchez-Lopez1, Using the act test for follow-up of an
A.I. Giraldo2, I. Tunez2 attack period in clinically isolated
Neurology, Hospital Universitario Reina Sofa Cordoba,
1

Biochemistry and Molecular Biology, Facultad de


2 syndrome: comparison on the basis of
Medicina, Universidad de Cordoba, Cordoba, Spain pasat in multiple sclerosis patients
B. Piri inar, S. Ozakbas
Dokuz Eylul University, Izmir, Turkey
P1713
The prevalence of neuromyelitis optica
and neuromyelitis optica-IgG
P1718
seropositivity amongst Saudis with Cluster analysis of health-related quality
central demyelinating diseases of life (HRQoL) indices in multiple
A. Al-Khathaami1,2, F. Yunus1, M. Alamgir2, S. Kojan2, sclerosis patients
M. Aljumah3 L. Sokolova, G. Gudzenko
1Department of Epidemiology and Biostatistics, College of Bogomolets National Medical University, Kyiv, Ukraine
Public Health and Health Informatics, King Saud Bin
Abdulaziz University for Health Sciences, 2Department of
Medicine, King Abdulaziz Medical City, 3King Abdullah
International Medical Research Center, King Saud Bin
Abdulaziz University for Health Sciences, Riyadh, Saudi
Arabia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 367

P1719 P1724
Monitoring disease progression and 4-aminopyridine reduces cerebellar
responses to disease-modifying drugs in tremor in a patient with multiple sclerosis
MS with serial MR images and a M. Whr, R. Schniepp
biomarker for structural changes Neurology, University of Munich, Hospital Grosshadern,
Munich, Germany
W.R. Riddle1, S.C. DonLevy2, S. Shokouhi1
1 Radiology and Radiological Sciences, Vanderbilt University,
2 Pediatric Nurse Practitioner, Nashville, TN, USA P1725
Clinical and demographic determinants of
P1720 disease severity in multiple sclerosis
6 months follow-up after natazulimab patients
initiation in acute MS relapse in a S. Baghizadeh1, N. Beladimoghaddam2, M. Sahraian3
19-year-old patient 1Qazvin University of Medical Sciences, Qazvin, 2Shahid
Beheshti University of Medical Sciences, 3Tehran University
D. Taranu, S. Walter, K. Fassbender, S. Behnke
of Medical Sciences, Tehran, Iran
Neurology, Saarland Medical University, Homburg,
Germany
P1726
P1721 Abstract cancelled
Dynamic changes of visual evoked
potentials and brainstem auditory evoked P1727
potentials in patients with multiple Abstract cancelled
sclerosis
V. Ignatova1, L. Haralanov1, L. Todorova2 P1728
1Clinic of Neurology, MHAT-NHH, 2Institute of Biophysics Early symptoms and time to the diagnosis
and Biomedical Engineering, BAS, Sofia, Bulgaria
of multiple sclerosis
H. Nzwalo, F. S, I. Cordeiro, F. Ferreira, C. Basilio
P1722 Neurology, Faro Hospital EPE, Faro, Portugal
Innovative management of MS patients on
natalizumab using MSDS-3D software P1729
T. Schulthei, R. Kempcke, T. Ziemssen The effects of mindfulness-based stress
Multiple Sclerosis Centre at the Department of Neurology,
Technical University Dresden, Germany reduction (MBSR) program on physical
symptoms, quality of life and mental
health in patients with multiple sclerosis
P1723
S.M. Nabavi1, M.A. Besharat2, S. Granmayeh2, A. Imani3,
Balos concentric sclerosis: S. Sadeghi4
pathognomonic MRI and MR 1Neurology, Shahed University, 2Tehran University, Tehran,
spectroscopy findings Iran, 3MBSR, Buffalo, New York, NY, USA, 4Shahed
University, Tehran, Iran
M.I. Yon, A. Gulunay, S. Mungan, Z.N. Oztekin, F. Ak
Neurology, Numune Education and Research Hospital,
Ankara, Turkey

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


368 Posters, Sunday 9 September

P1730 P1736
Demyelinating disorders and visual fields: Prevalence of depression in patients
visual field deficit due to inflammation in suffering from multiple sclerosis in the
the optic chiasm Ukraine
N. Kale1,2, N. Kraus Warner2, S. Yedavally2, O. Kalbus, V. Shkolnyk
E. Eggenberger2 Department of Neurology and Ophthalmology,
Bakirkoy Training and Research Hospital, Department of
1 Dnepropetrovsk State Medical Academy, Dnepropetrovsk,
Neurology, Istanbul, Turkey, 2Michigan State University, Ukraine
Department of Neuro-Ophthalmology, East Lansing, MI,
USA
P1737
CSF findings and follow-up MRI imaging
P1731
features of acute transverse myelitis
Correlation between cognitive functions, J.H. Lee1, S.H. Hwang2, J.H. Park3
fatigue, depression and disability status in 1Neurology, National Health Insurance Corporation Ilsan

a cohort of multiple sclerosis patients Hospital, Goyang-si, 2Neurology, Kangnam Sacred Heart
S.M. Nabavi1, S. Tahbaz2, Z. Behjati2, F. Nourbala2, Hospital, Hallym University, 3Neurology, Sanggyepaik
S. Sadeghi3, Z. Saeedi2, A. Salahesh3, MS Research Unit, Hospital Inje University, Seoul, Republic of Korea
Mostafa Center
1Neurology, Shahed University, 2Tehran University, 3Shahed

University, Tehran, Iran


P1738
Familial cases of multiple sclerosis in the
Novosibirsk region of Russia
P1732
D.S. Korobko1, N.A. Malkova1,2, E.A. Kudryavtseva3,4,
Prevalence heterogeneity of multiple M.L. Filipenko3
sclerosis in the Volyn region (Ukraine) 1Regional MS Centre, State Novosibirsk Regional Clinical

N.V. Bobryk Hospital, 2Novosibirsk State Medical University, 3Institute of


Neurology, Volyn Region Hospital, Lutsk, Ukraine Chemical Biology and Fundamental Medicine, Siberian
Division, Russian Academy of Sciences, 4Novosibirsk State
University, Novosibirsk, Russia
P1733
Autonomic dysfunction in patients with P1739
multiple sclerosis Severe alopecia associated with
H. Tutkan1, H.L. Gl2, O. Karadas3 interferon beta treatment in a multiple
1Neurology, Occupational Diseases Hospital, 2Neurology,

Kartal Research and Education Hospital, Istanbul, sclerosis patient


3Neurology, Erzincan Military Hospital, Erzincan, Turkey G. Genc1, S. Demirkaya2
Maresal Cakmak Hospital, Erzurum, 2Gulhane Military
1

Medical Academy, Ankara, Turkey


P1734
Importance of an intervention program for P1740
the promotion of physical activity in the
3-year safety and efficacy data of
perception of illness in people with
natalizumab in Cypriot patients with
multiple sclerosis
relapsing-remitting multiple sclerosis
L. Pedro1, J. Pais-Ribeiro2, J. Pscoa Pinheiro3
1Physiotherapy, Inst. Politecnic of Lisbon - ESTESL, Lisbon, E. Kkolou, E. Gaglia, J. Toufexis, M. Pantzaris
2Psychology, University of Porto, Porto, 3Medicine Physical The Cyprus Institute of Neurology and Genetics, Nicosia,
Rehabilitation, University of Coimbra, Portugal Cyprus

P1735
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 369

P1741 P1746
Treatment of the emotional-and-affective The positivity rate of CSF oligoclonal
disturbances in patients with relapsing- band in Turkish multiple sclerosis patients
remitting multiple sclerosis and a light A. Altintas, M. Nalbantoglu, B. Zeydan
stage of invalidization Neurology, Istanbul University Cerrahpasa Medical Faculty,
Istanbul, Turkey
T. Yakushina, V. Lizhdvoy
M.F.Vladimirsky Moscow Regional Clinical and Research
Institute, Moscow, Russia P1747
Complementary-alternative medicine in
P1742 multiple sclerosis
Risk of developing multiple sclerosis after T. Basak1, V. Unver1, S. Demirkaya2
clinically isolated optic neuritis in Latvian 1School of Nursing, 2Neurology, Gulhane Military Medical

patients Academy, Ankara, Turkey


L. Vainsteine1, D. Kozica2, G. Karelis1,2, L. Elsone2
Riga Eastern Clinical University Hospital Gailezers,
1 P1748
Neurology and Neurosurgery Department, Riga Stradins
2

University, Riga, Latvia


Switching and escalating therapy in long
lasting multiple sclerosis - not always
necessary
P1743 A.T. Carvalho1,2, M.J. S1,3
Assessment of optic nerve axonal 1Centro Hospitalar de So Joo, 2Centro Hospitalar de Vila
pathology in multiple sclerosis patients Nova de Gaia/Espinho, 3Health Sciences Faculty, University
using optical coherence tomography Fernando Pessoa, Porto, Portugal
D. Pastare1, I. ire2, G. Laganovska2, A. Millers1
1Department of Neurology, 2Department of Ophthalmology, P1749
Pauls Stradins Clinical University Hospital, Riga, Latvia
Antibodies to N-homocysteinylated
albumin in patients with multiple sclerosis
P1744 - preliminary results
Screening for cognitive impairment in A. Jamroz-Wisniewska1, J. Beltowski2, Z. Stelmasiak1,
multiple sclerosis with MOCA test H. Bartosik-Psujek1
1Neurology, 2Pathophysiology, Lublin Medical University,
S. Aksoy, E. Timer, M. Akgn, E. Kvrak, D. Necioglu
Lublin, Poland
Orken
Sisli Etfal Education and Research Hospital, Istanbul,
Turkey P1750
False diagnosis in multiple sclerosis
P1745 F. Khorvash1, B. Asadi2, R. Norouzi3, A. Sonbolestan4,
Correlation of parameters of sideropenic M. Salari3
1Isfahan University of Medical Sciences, Isfahan
anaemia and neurological disability in
Neurosciences Research Center, Isfahan, 2University of AJA,
patients diagnosed with multiple Tehran, 3Isfahan Neuroscience Research Center (INRC),
sclerosis: a retrospective study 4Isfahan University of Medical Sciences, Isfahan, Iran

A. Alajbegovic1, N. Kukavica2, S. Alajebegovic3,


H. Resic2, J. Djelilovic-Vranic1, L. Todorovic1,
N. Jovanovic3
P1751
1Neurology Clinic, Clinical Center of Sarajevo University, Aquaporin 4 abs positive neuromyelitis
2Clinic of Haemodialysis, Clinical Center University of optica in Bulgaria: a clinical case
Sarajevo, 3Cantonal Hospital Zenica, Bosnia-Herzegovina
V. Grozeva, D. Georgiev
MHATNP St. Naum Sofia, Medical University Sofia, Sofia,
Bulgaria

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


370 Posters, Sunday 9 September

P1752 P1756
Natalizumab reduces MRI markers Alterations of the morphofunctional
suggestive of irreversible tissue damage parameters of the peripheral blood
in relapsing-remitting multiple sclerosis lymphocytes in exacerbations of multiple
C. Oreja-Guevara1, A. Royo2, L. Rodriguez De Antonio1, sclerosis
S. Noval3, B. Chamorro1, J. Moreno1, E. Diez-Tejedor1, T. Yakushina, I. Vasilenko, V. Lizhdvoy, S. Kotov
J. Alvarez-Linera4 M.F.Vladimirsky Moscow Regional Clinical and Research
1Neurology, 2Radiology, 3Ophthalmology, University Hospital Institute, Moscow, Russia
La Paz, Idipaz, 4Radiology, Hospital Ruber Internacional,
Madrid, Spain
P1757
P1753 Pulmonary artery hypertension associated
with interferon beta 1a therapy in a
Hypercoagulable state mimics multiple
patient with multiple sclerosis
sclerosis: a case presentation
S. Baghizadeh1, M. Ghahari2, M. Sahraian3
N. Zahra1, S.M. Nabavi2, A. Saadat1 1Qazvin University of Medical Sciences, Qazvin, 2Shaheed
1Baghatallah University, 2Neurology, Shahed University, Beheshti University of Medical Sciences, 3Tehran University
Tehran, Iran of Medical Sciences, Tehran, Iran

P1754 P1758
Fatigue characteristics in multiple Can we speak about a psychiatric attack
sclerosis patients living in the north during multiple sclerosis?
Khorasan province located in the northern A. Ben Mahmoud, S. Hfaiedh, H. Derbali, N. Ben Ali,
east of Iran, a low prevalence area M. Fraj, S. Blel
S.M. Nabavi1, R. Jajvandian2 Hopital Charles Nicolle, Tunis, Tunisia
1Neurology, Shahed University, 2North Khorasan University,
Tehran, Iran
P1759
The effects of combined model of training
P1755
exercise method in function and some
Multiple sclerosis and ankylosing biomarkers of MS patients
spondylitis in an HLA-B27 negative M.R. Kordi1, S.M. Nabavi2, S. Khodadadeh1
patient 1Tehran University, 2Neurology, Shahed University, Tehran,

M. Messelmani, N. Ben Ali, S. Fray, H. Khiari-Mrabet, Iran


A. Mrabet
Neurological Department, Charles Nicolle Hospital, Tunis,
Tunisia

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 371

Muscle and neuromuscular junction P1761


diseases Magnetic resonance imaging evaluation
of calf hypertrophy in Becker muscular
dystrophy
P1760 M. Monforte1, G. Tasca1, E. Iannaccone1, M. Masciullo1,
Development and current status of an EU P. Ottaviani2, F. Laschena2, E. Mercuri3, E. Ricci1
patient registry for Lambert-Eaton 1Institute of Neurology, Catholic University School of

myasthenic syndrome (LEMS) Medicine, 2Department of Radiology, Istituto Dermopatico


dellImmacolata, 3Pediatric Neurology Unit, Catholic
C. Desnuelle1, F. Hanisch2, G. Le Masson3, University School of Medicine, Rome, Italy
R. Mantegazza4, A. Meisel5, J.P. Sieb6
1Centre de Rfrence Maladies Neuromusculaires, Nice, Introduction: Calf muscle hypertrophy is considered a
France, 2Martin-Luther-Universitat Halle-Wittenberg, Halle common clinical feature in dystrophinopathies, and has
(Saale), Germany, 3Centre Hospitalier Universitaires been previously attributed to deposition of fat and
Bordeaux, France, 4Istituto Neurologico C.Besta, Milan, connective tissue. Other evidences also suggested a
Italy, 5Charit Universittsmedizin Berlin, 6Hanse-Klinikum component due to true hypertrophy. The aim of our study is
Stralsund, Germany to use MR imaging to investigate the contribution of these
components to calf hypertrophy in Becker muscular
Introduction: LEMS is a rare autoimmune neuromuscular
dystrophy (BMD) patients.
disorder, frequently associated with small cell lung
Methods: 58 BMD patients aged 3511 years underwent
carcinoma or other malignancy and results in neuromuscular
lower limb MR scans. Patients were subdivided into 4
dysfunction, exertion-induced muscle weakness, and
groups, according to clinical severity (from asymptomatic
autonomic dysfunction. To better understand the natural
to non-ambulant). Cross sectional area (CSA) of lower leg
history and clinical course of LEMS, a voluntary,
posterior compartment muscles was digitally measured and
international, multi-centre, longitudinal, observational
correlated with the sum of the T1 scores (T1 scale) of the
LEMS patient registry was launched in May 2010. The
same muscles. T1 score represented a measure of fat
registry will also track long-term safety and efficacy data on
infiltration of each muscle.
medications used to treat LEMS.
Results: We found a non-significant correlation (rho=0.035
Methods: A protocol of recommended assessments is
p=0.795) between CSA and calf muscle T1 scale. We found
performed at enrolment and at routine follow-up visits to
a significant correlation between T1 scale and clinical
collect information on demographics, medical history,
severity (rho=0.901 and p<0.001), but not between CSA
treatments, signs and severity of neuromuscular dysfunction
and clinical severity (rho=0.012 p=0.927). We also found
(EMG parameters, reflexes, muscle strength, ataxia,
significant correlations between T1 scale and age
mobility/exercise), daily functioning including EQ-5D,
(rho=0.451 and p<0.001) and CSA and age (rho=0.292
autonomic dysfunction, and safety information (adverse
p=0.026).
events, ECG, spirometry, and clinical laboratory tests).
Conclusions: Based on our findings, it is likely that a major
Assessment data are entered through an internet portal
contribution to calf hypertrophy is provided by real
using a validated web-based application developed for this
hypertrophy instead of muscle fatty degeneration in BMD
program (BioMarin Pharmaceutical, USA). Recruitment
patients. Not surprisingly, T1 scale scores increase with age
and observation are planned for 5 years.
and clinical severity, while CSA only correlates with age.
Results: 43 patients (24 males, 19 females, mean age 62.2
yr, SD 12.3, range 27-84 yr) are currently enrolled in the
registry, representing 28.3 patient-years of participation
(mean length of follow-up: 8 months). Statistical summaries
will be compiled and presented annually to provide
descriptive information on the overall registry population,
current treatment practices and safety assessments, health
status and daily functioning ability, and results of
neuromuscular functioning tests.
Conclusion: The recently initiated LEMS EU patient
registry will continue to accrue and report important
information on the clinical course and treatment approaches
to this debilitating and under-recognized disorder.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


372 Posters, Sunday 9 September

P1762 P1763
Clinical impact of persistent hyperCKemia Hypogonadism and erectile dysfunction in
in a Norwegian general population: patients with myotonic dystrophy type-1
a case-control study S. Peric1, D. Lavrnic1, T. Nisic2, I. Basta1, M. Miljkovic1,
H. Lilleng1,2,K. Abeler1, S.H. Johnsen1,2, E. Stensland1,2, S. Popovic2, V. Rakocevic Stojanovic1
S. Lseth1,2, S. Lindal2,3, T. Wilsgaard4, S.I. Bekkelund1,2
1Neurology Clinic, 2Endocrinology Clinic, Clinical Center of
1Department of Neurology and Neurophysiology, University Serbia, Belgrade, Serbia
Hospital of North Norway, 2Department of Clinical Introduction: Myotonic dystrophy type-1 (DM1) is the
Medicine, University of Troms, 3Department of Clinical most common form of muscular dystrophy in adults. It
Pathology, University Hospital of North Norway, affects many organs besides muscle, including
4Department of community medicine, University of Troms,
endocrinological abnormalities (insulin resistance, thyroid
Troms, Norway dysfunction and hypogonadism in men). Aim of this study
Background: The purpose of this study was to assess was to assess frequency of erectile dysfunction (ED) and
clinical impact of persistent hyperCKemia in a Norwegian hypogonadism, the correlation between them and the impact
general population. We also investigated frequency of of ED on quality of life (QoL) in patients with DM1.
neuromuscular disorders and risk factors in a group with Method: This study included 25 men (22-58 years old) with
hyperCKemia compared with controls. diagnosis of DM1. Severity of muscular involvement was
Method: In a double blind case-control study, we compared assessed with Muscular Impairment Rating Scale (MIRS)
the frequency of muscular symptoms, functions and and erectile function with International Index of Erectile
neuromuscular diseases between 120 individuals with Function (IIEF). Levels of FSH, LH and testosterone were
persistent hyperCKemia and 130 age- and sex-matched analyzed. Patients completed SF-36 questionnaire as a
individuals with normal CK values, all recruited from the measure of QoL.
6th survey of The Troms Study. The participants underwent Results: ED was present in 18 (72%) patients. 7 (28%)
a standardized interview, including questions concerning patients were euogonadic, 16 (64%) had compensated
muscle symptoms, leisure physical activity, use of statins hypogonadism and 2 (8%) had primary hypogonadism. ED
and presence of other CK risk factors, prior to clinical was more common in patients with hypogonadism (78% vs.
neurological and neurophysiological examination. Knee 57%, p>0.05). ED was not significantly associated with age
extensor muscle strength was assessed with a Cybex NORM at onset of DM1, duration of disease, age and severity of
dynamometer, and a Martin Vigorimeter was used to muscular impairment at the moment of investigation
measure dominant hand grip strength. (p>0.05). SF-36 mental composite score was lower in
Results: We found no evidence of more muscular symptoms patients with ED (55.722.2 vs. 75.820.9, p<0.05).
or impaired muscular strength in those with persistent Conclusion: More than two thirds of men with DM1 had
hyperCKemia compared to the controls. In men, weight and ED and hypogonadism. ED was more frequent in patients
Body Mass Index (BMI) were significantly higher in the with hypogonadism. Mental aspect of QoL was lower in
group with persistent hyperCKemia. In women, no patients with ED. Development of therapeutic strategies for
differences between the groups were detected. ED in DM1 may have positive impact on quality of life in
Use of statins was similar in cases and controls. We these patients.
diagnosed 3 women with previously unknown myopathy,
all in the group with persistent hyperCKemia.
Conclusion: Only minimal clinical differences were found
between individuals with hyperCKemia and controls. Our
findings support the recently published EFNS guidelines on
diagnostic approach to pauci- or asymptomatic
hyperCKemia.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 373

P1764 P1765
Improved methodology for obtaining large Predictive value of anti-titin antibodies in
quantities of correctly folded recombinant early and late onset myasthenia gravis
N-terminal extracellular domain of the P. Szczudlik1, B. Szyluk1, M. Lipowska1, B. Ryniewicz1,
human muscle acetylcholine receptor for J. Kubiszewska1, M. Dutkiewicz2, H. Kwieciski1,
inducing EAMG in rats A. Kostera-Pruszczyk1
1Department of Neurology, 2Department of General and
C. Sun1, X. Qi2, Z. Li3, H. Zhang4 Nutritional Biochemistry, Medical University of Warsaw,
1Department of Neurology, 2Navy General Hospital, Beijing, Poland
3Tangdu Hospital, The Fourth Military Medical University,
Xian, 4Neurology, First Hospital of Jilin University, Introduction: In 85% of myasthenia gravis (MG) patients
Changchun, China the disease is caused by acetylocholine receptor antibodies
(AChR-MG). Antibodies against other muscle antigens,
Introduction: Human myasthenia gravis (MG) is an
including titin, are also detected in MG patients, and were
autoimmune disorder of the neuromuscular system.
reported as markers of severity of MG in some populations.
Experimental autoimmune myasthenia gravis (EAMG) is a
Anti-titin antibodies are more frequent in thymoma patients.
well-established animal model for MG that can be induced
The study was conducted to estimate the role of anti-titin
by active immunization with the Torpedo Californica-
antibodies in Polish MG patients.
derived acetylcholine receptor (AChR). Due to the high cost
Methods: Anti-titin antibodies were tested in sera of 295
of purifying AChR from Torpedo Californica and the harm
MG patients aged 4-89: 164 with early- (50, EOMG) and
to the animals, the development of an easier and more
131 with late-onset MG (LOMG). Thymic pathology, MG
economical way for inducing EAMG remains critically
severity, need for immunosuppression and treatment results
needed.
at last follow-up were analyzed.
Materials and methods: Full-length cDNA of the human
Results: Thymoma were diagnosed in 17 EOMG and 10
skeletal muscle AChR 1 subunit was obtained from TE671
LOMG patients. 81 MG patients were positive for anti-titin
cells. The DNA fragment encoding the extracellular domain
antibodies, all were AChR-MG. In EOMG 6% of the
(ECD) was then amplified by PCR and inserted into pET-
patients had anti-titin antibodies; its presence had 53%
16b. The reconstructed plasmid was transformed into
positive predictive value (PPV), 99% negative predictive
BL21(DE3)pLysS, which was derived from E. coli.
value (NPV) and 90% sensitivity with 94% specificity for
Isopropyl-beta-D-thiogalacto-pyranoside (IPTG) was used
thymoma diagnosis. In LOMG, anti-titin antibodies were
to induce the expression of the N-terminal ECD. The
detected in 54% of the patients; with 47% PPV for thymoma,
produced protein was purified with immobilized Ni2+
50% NPV, 91% sensitivity and 8% specificity, respectively.
affinity chromatography and refolded by dialysis.
In neither EOMG nor LOMG anti-titin antibodies were
Results: The recombinant protein was efficiently refolded
associated with more severe course of the disease defined
to soluble active protein, which was verified by ELISA.
by maximal MGFA, the need for immunosuppression or
After immunization with the recombinant ECD, all rats
treatment outcome.
acquired clinical signs of EAMG. Importantly, the titre of
Conclusion: Detection of anti-titin antibodies in early
AchR antibodies in the serum was significantly higher in
onset-MG can serve as additional test predicting the
the EAMG group than in the control group, indicating
presence of thymoma. In Polish MG patients it does not
successful induction of EAMG.
indicate a more severe course of the disease.
Conclusion: Here we have described an improved
Study supported by the Polish-Norwegian Research Fund
procedure for refolding recombinant ECD of human muscle
PNRPNRF-204-AI-1/07.
AChR. This improvement allows for the generation of large
quantities of correctly folded recombinant ECD of human
muscle AChR, which provides an easier and more
economical way of inducing the animal model of MG.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


374 Posters, Sunday 9 September

P1766 P1767
Clinical features of anti-MuSK positive Exercise and cold-induced sodium
myasthenia gravis patients channel myotonia with the SCN4A
M. Bisciglia, S. Martignago, E. Albertini, C. Angelini mutation
Neurosciences, University of Padova, Italy K. Fujii1, S. Sakota2, H. Uchikawa1, A. Takagi1, S. Anzai1,
Introduction: Myasthenia gravis (MG) is an autoimmune Y. Kohno1
disorder in which autoantibodies against the nicotinic 1Pediatrics, Chiba University Graduate School of Medicine,

acetylcholine receptor (AChR-Ab) are pathogenetic. About Chiba, 2Neurology, Kagoshima University Schiil of
5 to 30% of MG patients have no detectable AChR-Ab Medicine, Kagoshima, Japan
(seronegative MG), and 50% of them have antibodies Introduction: Myotonia is characterized by a failure of
against a muscle specific tyrosine kinase (MuSK-Ab). The voluntary muscle to relax after contraction ceases and
aim of our study was to describe the clinical features in divided into non-dystrophic and dystrophic types. However,
MuSK-Ab positive MG patients. there exist indistinguishable cases not belonging to either
Methods: A retrospective evaluation of MG patients was types, namely sodium channel myotonia. This study aimed
performed: 207 MG patients at the Clinical Centre for to elucidate the clinical manifestations of sodium channel
Neuromuscular diseases of Padua University (131 females, myotonia with heterozygous SCN4A mutation.
76 males) were screened for anti-MuSK antibodies. 6 of Methods: We investigated clinical manifestations of five
them (18%) were found positive. Patients were evaluated members in one sodium channel myotonia family. We also
using the MGFA (Myasthenia Gravis Foundation of identified the missense mutation as c.3917A (pG1306V) in
America) clinical classification. the SCN4A gene.
Results: Mean age at onset was 30.5 years (range: 22 to 46 Results: The five members with the SCN4A mutation
years). 1 patient was 40 years old, male, with a clinical exhibited diverse clinical manifestations such as muscle
history of bulbar MG. 5 patients were female, 4 with oculo- cramp, muscle stiffness, fatigue, and exercise- and cold-
bulbar MG (66%), 1 with generalised MG. At last follow-up induced myotonia without increment of muscle volume.
4 of them (66%) improved their MGFA score. 1 patient They also exhibited no exacerbation by exercises, drop
(16%) was asymptomatic; 2 were stable. They responded attack, or muscle hypertrophy. These manifestations were
poorly to anticholinesterases and did not benefit from different from those of congenital myotonia and
steroid treatment. They improved with immunosuppressive paramyotonia in view of duration and induction. We treated
agents (Cyclosporine or Azathioprine). 3 patients were these patients with carbamazepine or mexiletine, resulting
thymectomised: histological findings showed thymic in successful rescue of the myotonia symptoms.
hyperplasia (2 patients) and atrophy. Discussion: Sodium channel myotonia shows distinct
Conclusions: MuSK positive patients appear to be a clinical features compared to those of congenital myotonia
different and heterogeneous subgroup of MG patients. They and paramyotonia. Common clinical manifestations of
are clinically more severe, requiring more aggressive sodium channel myotonia were exercise- and cold-induced
treatment. Bulbar involvement is predominant and they myotonia without increment of muscle volume. The
respond poorly to conventional immunosuppression or mutated protein, p.G1306V, of this Japanese family has
thymectomy. Cyclosporine might have a role in their been already reported as G1306E and G1306A in European
management. families. Although phenotypes of them were different as
myotonia permanent and myotonia fluctuans, this amino
acid substitution would be crucial for maintaining the
regular muscle function in humans.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 375

P1768 P1769
Limb girdle muscle dystrophies. Is there a New phenotype and pathology features in
pathological-genetic correlation? MYH7-related distal myopathy
C. Monteiro1, R. Taipa2, I. Alves3, E. Vieira4, G. Tasca1, E. Ricci1, S. Penttil2, M. Monforte1,
M. Oliveira4, M. Melo Pires2, R. Santos4, A. Guimares2 V. Giglio3, P. Ottaviani4, G. Camastra5, G. Silvestri1,
1Neurology Department, 2Neuropathology Unit, Hospital de B. Udd2
Santo Antnio - Centro Hospitalar do Porto, 3Neurology 1Institute of Neurology, Catholic University School of
Department, Hospital de So Sebastio, Santa Maria da Medicine, Rome, Italy, 2Neuromuscular Research Center,
Feira, 4Molecular Genetics Unit, Centro de Gentica Mdica Tampere University and University Hospital, Tampere,
Jacinto Magalhes - INSA, Porto, Portugal Finland, 3Centre for Neuromuscular Diseases, Uildm,
4Radiology, Istituto Dermopatico dellImmacolata,
Introduction: Limb girdle muscle dystrophies (LGMD) are
5Cardiology Division and ICU, Ospedale Madre Giuseppina
disorders characterized by progressive weakness and
atrophy, with variable age of onset. Although genetic test is Vannini, Rome, Italy
the gold standard for diagnosis, muscle biopsy may suggest Introduction: Laing distal myopathy (LDM) is an
a particular disorder. autosomal dominant disease due to mutations in the gene
Objectives: Our aim was to verify the correlation between encoding for the human slow-beta myosin heavy chain,
suggested neuropathological diagnosis and molecular MYH7. Most reports describe it as a mild, early onset
genetics in LGMD, looking for the presence of histo- myopathy with involvement usually restricted to foot
enzymological and immunohistochemical patterns. extensors, hand finger extensors and neck flexors, and
Methods: We selected the cases in which the unspecific findings on muscle biopsy. We identified the first
neuropathological diagnosis was LGMD (from the two Italian families with LDM, harbouring two novel
neuropathology unit database). The genetic result and the mutations in the MYH7 gene.
biopsy of each case were reviewed and correlated. Methods: Clinical, neurophysiological, pathological,
Results: From the 132 biopsies with LGMD diagnosis, only muscle MRI and cardiological investigations were
77 cases have DNA analysis - 18 SGCG, 7 CAPN3, 7 performed on affected members from the two families.
DYSF, 6 SGCA and 6 SGCB; 10 cases have other less Results: Affected members from one family presented a
common mutations and 23 remain without diagnosis. moderate-severe phenotype, together with distal involve
Neuropathological diagnosis was confirmed by genetic test ment and even loss of ambulation at advanced age. One
in 15 gamma-sarcoglycanopathies (from 16 suggested, all patient displayed atypical muscle biopsy findings including
with immunohistochemistry for gamma-sarcoglycan absent cytoplasmic bodies and myofibrillar myopathy-like
or markedly reduced), 6 dysferlinopathies (9 suggested), 5 features. Affected members from the second family shared
alpha-sarcoglycanopathies (5 suggested) and 1 calpaino a very mild phenotype, with weakness largely limited to
pathy (7 suggested). In the 6 beta-sarcoglycanopathies long toe and foot extensors and/or late onset. No patient
genetically confirmed, neuropathological diagnosis was showed any sign of heart involvement.
LGMD-non-specified. The review of those biopsies showed Conclusion: Our study significantly broadens the clinical
that all have at least 3 sarcoglycans markedly reduced or and pathological spectrum of LDM. We suggest that MYH7
absent. Neuropathological suggestion of calpainopathies screening should be considered in undiagnosed late-onset
(7 cases) was done based on lobulated fibres; this diagnosis distal myopathy or cytoplasmic body myopathy patients.
was confirmed in only one case. The other genetically
confirmed calpainopathies did not show lobulated fibres or
inflammatory infiltrates.
Conclusions: Immunohistochemistry was helpful for
diagnosing gamma-sarcoglycanopathies and it could also
have been helpful for beta-sarcoglycanopathies.
Lobulated fibres were misleading for the diagnosis of
calpainopathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


376 Posters, Sunday 9 September

P1770 P1771
Exclusive presynaptic location and Protective effect of melatonin against
coupling to ACh release of the novel TNF- toxicity in the L6 muscle cell line
protein kinase C epsilon isoform in the J.G. Lim1, E.J. Chung2, J.H. Park3, D.K. Song3
adult rat neuromuscular synapse Neurology, Keimyung University Dongsan Medical Center,
1

Neurology, Daegu Medical Center, 3Physiology, Keimyung


2
N. Ortiz1, T.I. Orbis2, N. Besalduch2, N.T. Olguin2,
University Dongsan Medical Center, Daegu, Republic of
M.M. Santaf2, M. Toms2, N. Garcia2, M. Priego2,
Korea
M.A. Lanuza2, J. Toms2
1Hospital Universitari Sant Joan, 2Unitat dHistologia i Introduction: An increasing body of evidence implies that
Neurobiologia (UHN), Facultat de Medicina i Cincies de la TNF- plays a critical role in muscle cell destruction in
Salut, Universitat Rovira i Virgili, Reus, Spain inflammatory muscle diseases. We hypothesize that reactive
oxygen species (ROS) mediate TNF--induced muscle cell
Protein kinase C (PKC) regulates a variety of neural
death and destruction. Recently, melatonin has attracted
functions, including ion channel activity, neurotransmitter
attention because of their free radical scavenging and
release and kinetics of postsynaptic membrane receptors.
antioxidant properties.
Nervous tissues express different isoforms of PKC. This
Aim: The aim of this study is to evaluate the possible
diversity of isoforms and their selective location may be the
protective role of melatonin against TNF--induced cell
cause of differences in the PKCs phosphorylation activity.
death in rat L6 myocytes.
Previous studies from our group suggested that the novel
Methods: To examine the functional significance and
isoform of PKC epsilon (nPKCe) is exclusively presynaptic
detailed morphological characteristics of TNF--induced
in the neuromuscular junction (NMJ). In this study we
muscle proteolysis, L6 myocytes were subjected to
examine the distribution of nPKCe in the adult rat
increasing concentrations of recombinant TNF-, and the
neuromuscular junction and its involvement in the release
viability of myotubes was analyzed. L6 myotubes were
of acetylcholine. Immunohistochemical methods and high
incubated in serum-free, 11.1mM glucose-containing media
resolution confocal microscopy showed that PKCe is
containing TNF- with or without various concentrations of
exclusively localized in the nerve terminals of the NMJ.
melatonin, and then cell viability was evaluated using cell
Electrophysiological experiments have shown that blocking
counting assay. Intracellular ROS were measured by using
the activation of PKCe with the specific inhibitor peptide
cell permeable fluorescent dye, H2DCFDA.
eV1-2 completely prevented the enhancement of ACh
Results: TNF- at concentration of 100 ng/ml induced ROS
release induced by the phorbol ester PMA (phorbol
generation and decreased the viability of L6 myotubes.
12-myristate 13-acetate). Phorbol esters are unspecific
Further analysis of cell death showed that TNF- induced
PKCs activators. The potentiation induced by phorbol esters
apoptosis. Melatonin significantly attenuated TNF--
is a good model for presynaptic facilitation and sustained
induced ROS generation and cell death. Muscle fibre
activation of PKCs, and it is also necessary for the regulation
diameter decreased with increasing TNF- concentrations
of presynaptic neural plasticity. This clearly points to a
and was associated with attenuation of the PI3-K/Akt
relevant and specific role for nPKCe in neurotransmission
pathway as well as significant reductions in differentiation
at the NMJ. The coupling of the PKC family to the
markers. Furthermore, treatment of L6 myotubes with
stimulation of the neuromuscular transmitter release by
melatonin strongly ameliorated TNF--induced muscle
PMA could be completely mediated by the nPKCe.
atrophy and proteolysis.
Conclusion: These results suggest that ROS might be
mediated in TNF--induced muscle proteolysis and
melatonin may be useful as protecting agents for muscle
cell destruction related with inflammatory muscle diseases.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 377

P1772 P1773
Improvement in gastro-intestinal Muscle dystrophy due to dysferlin
symptoms following enzyme replacement deficiency: a phenotype study of 17
therapy in adult-onset Pompes disease Moroccan families
J. Pardo, A. Lpez-Ferreiro, E. Costa-Arpn, N. Birouk1, S. Sefiani2, H. Beladi1, B. Benaaboud1,
M. Santamara-Cadavid, G. Fernndez-Pajarn, B. Kably1, R. Ouazzani1
M. Pardo-Parrado 1Neurophysiologie Clinique, 2Neuropathologie, Facult de

Neurology, Hospital Clnico, Santiago de Compostela, Spain Mdecine-Universit Mohamed V Souissi, Rabat, Morocco
Introduction: Pompes disease is a rare, autosomal Dysferlin deficiency is responsible for different phenotypes
recessive glycogen storage disorder, caused by a deficiency of muscle dystrophies including limb girdle syndrome and
of acid-alpha-glucosidase (GAA). Late-onset Pompes distal myopathy. This work aims to give a phenotype
disease tends to present with progressive proximal weakness description of dysferlin deficient patients belonging to 17
and respiratory insufficiency, but other clinical features are Moroccan families. All patients underwent clinical
still emerging and have not been well documented yet. evaluation for muscle impairment distribution and
Methods: We report a 65-year-old male diagnosed with orthopaedic signs, CK dosage, electroneuromyography and
late-onset Pompes disease and treated with enzyme muscle biopsy with immunocytochemistry for dystrophin,
replacement therapy (ERT) since June 2011. sarcoglycans ( and ) and dysferlin. 18 patients aged
Results: His symptoms began 30 years ago with proximal 14 to 57 years were examined. There were 11 males and 7
bilateral lower extremity weakness. Five years later, he females. Age at onset was 20.97.4 years (range: 12 to 35).
presented respiratory insufficiency that required non- 13 families had an established consanguinity, 9 families had
invasive ventilation. During hospitalization for upper more than 1 affected member and 4 families had isolated
gastro-intestinal bleeding, at the age of 51, he presented cases. The phenotypes were as follows: Myoshi type in 7
acute respiratory failure and since then he has been on patients, limb girdle syndrome was found in 4, associated to
mechanical ventilation. When seen in the Neurological lower limbs distal muscles involvement in 4, to biceps
Department at the age of 64, the patient had undergone three brachialis muscles deficit in 2 and to tibialis anterior
muscle biopsies, with normal results. At this time, the impairment in 1 patient. CK level was 1.5 to 6 times normal
patient complained of deteriorating muscle function, fatigue value. All patients had dystrophic changes on muscle biopsy
and weight loss. During the previous twelve months, with absence of dysferlin whereas dystrophin and
chronic bloating, abdominal pain, with occasional diarrhoea sarcoglycans were normal on immunocytochemistry.
or intestinal subocclusion had been major complaints. Functional disability was variable, 2 patients were wheel
Diagnosis was made after evidence of deficient GAA chair bound at the ages of 23 and 38 years, respectively.
enzymatic activity on dried blood spot testing that was later Dysferlin deficiency was identified in 41.5% of muscle
confirmed in cultured fibroblasts. Molecular studies dystrophy families with limb girdle and/or distal muscles
demonstrated mutations in the GAA gene. After four months impairment in our series. It seems to represent the second
on ERT, his abdominal pain and bloating were resolved. cause of muscle dystrophies in Morocco after
Conclusion: To the best of our knowledge, only three sarcoglycanopathies. A screening for the responsible
patients with late-onset Pompes disease have been reported dysferlin gene mutations is necessary to identify a probable
with improvement in gastro-intestinal function following founder mutation in our population.
ERT. Gastro-intestinal symptoms are unrecognized
manifestations of this rare disease and merit more attention.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


378 Posters, Sunday 9 September

P1774 P1775
Botulinum toxin (BOTOX)
efficacy, safety AChR-Ab and MuSK-Ab positive
in blepharospasm and hemifacial spasm myasthenia gravis: morphological and
M. Cabalar, V. Yayla, T. Yazar, A. Culha, O. Selcuk, ultra structural findings on muscle biopsy
V. Guzel M. Bisciglia, S. Martignago, E. Albertini, M. Fanin,
Bakirkoy Training and Research Hospital, Department of E. Pegoraro, C. Angelini
Neurology, Istanbul, Turkey Neurosciences, University of Padova, Italy
Introduction: Botulinum toxin (Botox) is most widely Introduction: Myasthenia gravis (MG) is an autoimmune
used for blepharospasm (BPS) and hemifacial spasm (HFS). disorder: 80% of patients have antibodies against the
We aimed to evaluate the efficacy of the drug and to present nicotinic acetylcholine receptor (AchRAb), 50% of
our results of 4 years experience. seronegative patients have antibodies against a muscle
Methods: We present 15 BPS patients and 67 HFS patients specific receptor tyrosin kinase (MuSK-Ab). The task of
who have been treated with Botox. The average dose of our study was to compare the histological features and
Botox performed for an optimal response was 30-35 MU muscle fibre atrophy in muscle biopsy of AChR-Ab and
for BPS, 25-30 MU for HFS. MuSK-Ab positive MG patients.
Results: The therapeutic effect started after 3 to 5 days and Methods: We analysed skeletal muscle biopsy of 13 MG
the mean total duration of response, initial repetitive patients, 7 positive to AChR-Ab (AChR+) and 6 to MuSK-
efficacy intervals (REI) were longer in BPS (7.463.98 Ab (MuSK+). MuSK+ patients were all females with a
months) and HFS (6.333.92 months) than last REI in BPS mean age of 39 years, AChR+ patients were 2 females and
(5.950.17 months) and HFS (3.280.64 months). There 5 males aged 62 years on average.
was a significant statistical difference between the second Results: Mean muscle fibre atrophy factor was higher in
REI (BPS 14.454.13 and HFS 6.824.04 months) and the AChR+ MG than MuSK+ MG (normal range 0-150), in
last REI (BPS 5.950.17 and HFS 3.280.64 months) type I fibres (494 versus 210) and particularly in type II
(p<0.05). The mean efficacy of treatments was 3.330.72 fibres (1023 versus 300). Morphological analysis showed
(0=no effect, 4=marked improvement) in BPS and HFS. presence of type I and type II fibre grouping in AChR+
Adverse effects occurred in 7.29% of injection series. cases that demonstrate a post-synaptic action of AChR-Ab.
Conclusion: Botulinum toxin is an agent effective and safe A correlation with corticosteroid therapy is excluded:
when suitable technique and material are used in treatment biopsies were done before steroid treatment. COX-negative
of BPS and HFS. But, long-term therapy is sometimes fibres were found in MuSK+ cases. Pycnotic nuclei and
associated with therapy failure following repeated injections myofibrillar disarray were found in AChR+ MG, where
of the neurotoxin, presumably due to specific antibody mitochondria were disposed perpendicularly to the
production. Therefore, we suggest that a shift from one sarcomeres. Disruption of mitochondrial cristae and
product (Botox vs. Dysport) to another should be swelling of mitochondria were present in MuSK+.
considered in case of failure with one product. Conclusion: Our findings suggest muscle atrophy of
MuSK+ and AChR+ patients to be different. AChR-Ab
cause marked muscle atrophy and neurogenic aspects.
MuSK-Ab is associated with myopathic signs and
mitochondrial abnormalities. Mitochondrial impairment
could explain the oculo-bulbar involvement in MuSK+ MG.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 379

P1776 P1777
Investigation of HLA-DRB1 as a risk allele Anti MuSK positive myasthenia gravis.
for familiar auto-immune myasthenia Pitfalls of diagnosis and therapy
gravis management in the Czech Republic
J. Gamez1,2, J.M. Ponseti1,3, M. Canela1,4, M. Jakubkov, J. Piha, M. Tblov, I. Novkov
M.J. Herrero-Mata5, M. Salvad-Figueras1, Department of Neurology and Center of Clinical
E. Palou-Rivera5 Neuroscience, Charles University in Prague, 1st Faculty of
1Myasthenia Gravis Unit, Hospital Universitari Vall d Medicine and General University Hospital Prague, Czech
Hebron, 2Institut de Recerca, VHIR, 3Surgery Department, Republic
Clinica Teknon, 4Thoracic Surgery Department, Hospital Introduction: MG is an autoimmune disease marked by
Universitari Vall d Hebron, 5Laboratori de Immunobiologia neuromuscular transmission failure at the neuromuscular
per Recerca i les Aplicacions Diagnostiques, Banc de Sang i junction. Antibodies against acetylcholine receptors are
Teixits (LIRAD-BST), Barcelona, Spain found in about 85% of all patients. In the year 2001
Auto-immune myasthenia gravis (MG) is usually a sporadic antibodies against muscle specific tyrosine kinase (MuSK)
disease. After observing MG in a woman whose sister had have been discovered also as their pathogenetic role in
died of the disease, Oppenheim suggested the possible neuromuscular transmission.
involvement of genetic factors in 1898. We aimed to Material and methods: In our center investigation of
investigate the prevalence of familiar forms in a cohort of antibodies to MuSK was introduced in the year 2007 for all
267 auto-immune MG patients, and the HLA-DRB1 gene newly diagnosed seronegative patients with clinically
as a possible risk for familial MG. We identified 8 definite diagnosis of MG. By the method of radio
individuals from 5 unrelated families. The average age of immunoassay (RIA) total of 226 serum samples were
onset was 49.8 years (range 31-69). The MGFA types were examined.
I (1), IIa (3) IIb (1), IIIa (1) and IIIb (2). Five had been Results: 9 patients were identified, 6 women and 3 men.
thymectomized (hyperplasia 3, thymoma 2). The type of The mean age at first symptoms was 24.3 years (8-49) in
inheritance suggested an autosomal dominant pattern in 2 women, and 40.3 in men (20-57). In 5 patients, the first
families and a recessive pattern in the other 3. The symptom was diplopia or ptosis, bulbar weakness in 3
DRB1*03:01 genotype was identified in 6 patients, patients, shortness of breath in 1 patient. In 4 patients low-
belonging to four of the families studied. The frequency of frequency repetitive stimulation and single fibre EMG was
familial forms of MG in the population studied is 2%, negative. The mean level of anti-MuSK was 1.167nmol/l
similar to that observed in other studies. We found no (0.335 to 1.89). In 5 patients more than 1 relapse (an average
association with any common HLA haplotype. However, of 5.4 relapses) was observed. All patients have been treated
the prevalence of DRB1*03:01 in our series was 50% for all by corticosteroids, 8 of them by immunosuppressants,
the genotyped alleles (9 patients). This could be a candidate 1 woman was treated by rituximab. Currently, the
risk factor for myasthenia gravis, as the normal prevalence quantitative myasthenia gravis score is 10.1 on average.
of this phenotype among tissue donors in Spain ranges Conclusion: We demonstrated a low sensitivity of
between 11% and 19%. Genetic risk factors for familial electrophysiological testing and poor response to
myasthenia other than the HLA-DRB1 gene may be cholinesterase inhibitors in our patients. There is no clear
involved. The different inheritance patterns, phenotypes, correlation between levels of anti MuSK with the number
antibody types and histological characteristics described in of relapses. In all patients we achieved clinical remission.
the families suggest genetic heterogeneity.
Acknowledgements: PI 10/01070 FIS-FEDER.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


380 Posters, Sunday 9 September

P1778 P1779
Myasthenia gravis: interactions of new Lack of association of toll-like receptor 9
acetylcholinesterase inhibitors with polymorphisms with myasthenia gravis in
nicotinic receptors Han Chinese
V. Sepsova1, J. Krusek2, L. Drtinova1, M. Pohanka3, H. Li, Y. Hong, T. Liu, X. Gao
J.Z. Karasova4 Neurology, Affiliated Hospital of Medical College, Qingdao
1Toxicology, University of Defence, Faculty of Health University, Qingdao, China
Sciences, Hradec Krlov, 2Institute of Physiology, Academy Introduction: Toll-like receptor 9 (TLR9) is a kind of
of Sciences of the Czech Republic, Prague, 3Centre of pattern recognition receptor which can recognize bacterial
Advanced Studies, 4Public Health, University of Defence, and virus genomic DNA. A recent study shows that TLR9
Faculty of Health Sciences, Hradec Krlov, Czech Republic may play an important role in the induction and perpetuation
Reversible inhibitors of acetylcholinesterase (AChE) are of autoimmune diseases. The aim of our study was to
used in the treatment of the autoimmune disorder - investigate whether the polymorphisms of TLR9 variants
myasthenia gravis. The mechanism of action is based on the were associated with myasthenia gravis (MG).
inhibition of AChE active site that leads to an increase of Methods: A total of 132 MG patients and 212 matched
acetylcholine concentration and its prolonged presence in healthy control samples in a northern Han Chinese
the synaptic cleft compensate for the pathologic reduction population were included in this study. The genomic DNA
of neuromuscular receptors. Moreover, certain AChE was extracted and subjected into PCR-RFLP analysis. Two
inhibitors are able to act directly as nicotinic receptor tagging SNPs (rs352140 and rs187084) that span TLR9
modulators. In our study we have focused on the unknown gene were selected by haploview software. Genotypes,
non-acetylcholinesterase effects of several AChE inhibitors. allele frequencies and haplotypes of patients and controls
Measurements were performed using whole-cell patch- were evaluated for the SNPs using the online software
clamp technique. Drugs were applied using a rapid perfusion SNPStats. Subgroups of MG patients were classified by
system. Two new peripherally acting AChE inhibitors (K sex, age, thymoma and severity of MG.
298, K 524) were compared with two standards Results: Both rs352140 and rs187084 showed
(Edrofonium, BW284c51). All experiments were performed polymorphism in Han Chinese population and the
on TE671 cell line expressing human embryonic muscle distribution of genotypes meets the Hardy-Weinberg
acetylcholine receptor. Values of 50% inhibition (IC50) equilibrium. But no significant differences in the frequency
were calculated by fitting the data to the Hill equation. All of alleles, genotypes, and haplotypes in the MG cases were
tested compounds show a comparatively high reversible detected compared with healthy controls. There was also no
inhibition effect (IC50). When tested compounds were pre- association within subgroups of MG patients.
applied for 5s and then co-applied with the agonist Conclusion: We did not find an association between the
acetylcholine (100M), responses were inhibited in two tagging SNPs within TLR9 gene and myasthenia
concentration dependent manner. New synthesized inhibitor gravis. TLR9 gene may not be a major genetic determinant
K 524 shows the lowest IC50 value 5705nM that is of MG.
approximately one or two order of magnitude lower
compared to standards. Mechanism of nicotinic receptor
inhibition is still unknown and will be discussed within our
contribution. Supported by a grant of the Ministry of
Education, Youth and sports, Czech Republic: Specific
research: Determination of biochemical and
pharmacokinetics parameters of acetylcholinesterase
inhibitors is gratefully acknowledged, AV0Z 50110509.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 381

P1780 P1781
Successful treatment of hypokalemic LGMD-2B: clinical phenotype and
periodic paralysis with topiramate diagnosis 50 years after the first
J. Pardo1, T. Garca-Sobrino1, F. Barros2, B. Quintns3,4, symptoms
M.J. Sobrido3,4,5, A. Lpez-Ferreiro1 J. Moreira Martins1,2, L. Santos2,3
1Neurology, Hospital Clnico, 2Unidad de Medicina 1Neurology, Hospital Pedro Hispano, Matosinhos,
Molecular, Fundacin Pblica Galega de Medicina 2Neurology, Hospital de Egas Moniz - CHLO, Lisbon,
Xenmica, 3Hospital Clnico, Santiago de Compostela, 3Neurology, Hospital Fernando Fonseca, Amadora, Portugal
4Center for Biomedical Network Research on Rare Diseases
Introduction: Determining the subtype and etiology of
(CIBERER). Institute of Health Carlos III, Madrid,
5Fundacin Pblica Galega de Medicina Xenmica, muscle disease in patients with LGMD phenotype is
fundamental for genetic counselling, cardiac/respiratory
Santiago de Compostela, Spain
monitoring and further research and therapy. We report a
Introduction: Hypokalemic Periodic Paralysis (hypoPP) is case whose diagnosis was made 50 years after the first
the most common form of periodic paralysis. Most patients symptoms, with appropriate selection of the muscle to
have a mutation in the CACNA1S (hypoPP type I) or in the biopsy.
SCN4A (hypoPP type II) gene. Preventive treatment with Clinical case: A lady aged 75, who initiated proximal
carbonic anhydrase inhibitors or potassium supplementation paresis of lower limbs at the age of 25 years, becoming
is usually needed. In a recent case report, treatment with dependent on a wheelchair at 30. At 55 proximal paresis of
topiramate was found to be effective in two 11-year-old upper limbs began and three years later, ventilatory
twins with hypoPP. dysfunction due predominantly to diaphragmatic paresis
Methods: We present a 36-year-old man diagnosed with progressing; years later, to severe restrictive pattern needing
hypoPP type I, with episodes of transient weakness since BiPAP. EMGs showed a myopathic pattern. Two previous
the age of 14. biopsies gave inconclusive results. At present she has
Results: Since his first spell of generalized transient relatively spared extensor muscles of the wrist and fingers,
weakness lasting several hours, the patient was treated with hip abductors, and toes flexors, with severe muscle atrophy
daily oral potassium. In the next years, the frequency of and weakness of most of the other muscles. A new muscle
attacks increased and acetazolamide was added, with initial biopsy was performed 50 years after the first symptoms
benefit. In 2001, acetazolamide was stopped because of (forearm extensor digitorum communis) showing dystrophic
increasing frequency of attacks and dichlorphenamide was findings, absence of immunohistochemical labelling for
introduced, with a decrease in severity of attacks in the dysferlin, positive MHC-I, perimysium and perivascular
following months. Two years later, because his episodes inflammatory infiltrates. The mutation DYSF: c.610C>T
went up again both in frequency and severity, (p.Arg204X) was found, apparently in homozygosity in
dichlorphenamide was stopped and the patient was put exon 6.
again on acetazolamide. During the last year, the patient had Discussion and conclusions: This case illustrates the
attacks every two weeks, and acetazolamide was switched selective relative sparing of certain muscle groups even
to topiramate in June 2011. Since then, he only had two after 50 years of clinical progression of LGMD-2B
minor attacks upon initiation of the drug, and he remained allowing, even then, a diagnosis by muscle biopsy. The
asymptomatic nine months later. presence of inflammatory findings is described in
Conclusion: To our knowledge, this is the second report of dyspherlinopathies, but could pose diagnostic uncertainty
the therapeutic benefit of topiramate in cases of refractory and change the treatment options.
hypoPP. A clinical trial with this drug is warranted as an
additional treatment option for hyoPP.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


382 Posters, Sunday 9 September

P1782 P1783
Video hand opening time (vHOT) in Early manifestations of Eaton-Lambert
myotonic dystrophy type 1 (DM1): data myasthenic syndrome in the structure of
from STOPP DM paraneoplastic disorders of the nervous
A. Puwanant, J.M. Statland, K.J. Eichinger, N. Dilek, system
R.T. Moxley, C.A. Thornton Y.G. Dubenko
Neurology, University of Rochester Medical Center, Neurology, Kharkiv National Medical University, Kharkiv,
Rochester, NY, USA Ukraine
Introduction: Delayed release of the handgrip in DM1 Introduction: Detection of early stages of paraneoplastic
reflects myotonia and finger extensor weakness. Previous disorders of the nervous system is an insufficiently explored
methods to quantify myotonia required expensive issue, especially as far as the pathology of neuromuscular
equipments and extensive evaluator training. Video transmission is concerned. This causes frequent diagnostic
recording is an accepted procedure to assess motor mistakes.
performance in clinical trials. Object and method: Neurologic, immunologic and EMG
Objective: To evaluate test-retest reliability and validity of examination of 147 patients diagnosed with small cell lung
vHOT. cancer was performed. A group of 39% exhibited weakness
Methods: vHOT recordings were collected in 20 DM1 in proximal lower limbs with the tendency towards
subjects on 2 consecutive days and reviewed by 2 blinded decreased tendon reflexes. 28% of the examined group had
raters. On each day 3 trials of 5-second maximal handgrips started experiencing weakness 9-15 months before the
followed by rapid full extension of the fingers were recorded oncological pathology was diagnosed. Only 21% of the
with 10 minutes rest between trials. Time for maximal hand examined patients exhibited autonomic imbalances
opening was measured separately for the thumb and long (decreased salivation and tear production, orthostatic
finger. vHOT was compared to 90% to 5% handgrip hypotension), 7% of the patients exhibited oculogyric
relaxation times made, using a computerized handgrip malfunctions. Thus, increasing, sometimes varying leg
myometer and automated software on the same day. weakness in part of the patients was the only clinical
Results: The mean vHOT was 4.65.6 seconds. vHOT was implication of the disease, with the oncological pathology
longer for the thumb than the long finger. The correlation of not yet diagnosed.
vHOT between trials on the same day (r=0.94-0.97) was Results: Only immunological tests (showing the presence
better than for trials on separate days (r=0.88). There is no of Anti VGCC and Anti PCA-2) and EMG (at high
evidence for warm-up in successive trials. The ICC for frequency rhythmic stimulation the test registered increased
test-retest concordance for the same rater on 2 successive summery potential, while during low frequency stimulation
days was 0.71. The ICC for concordance between 2 raters the summary potential decreased to the myasthenic typical
was 0.96. The correlation between mean vHOT and 90% to low) allowed identifying the pattern of Eaton-Lambert
5% handgrip relaxation times was 0.75. syndrome early manifestation.
Conclusions: vHOT is a simple, reliable, low-cost Conclusion: Early clinical forms of Eaton-Lambert
alternative to computerized handgrip myometry for syndrome do not always have typical clinical evidence; yet,
assessing myotonia and finger extension in disease specific immunological tests and EMG make it possible to
progression and therapeutic trials in DM1. diagnose right and explore the likely oncological pathology
that lacks clear manifestation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 383

P1784 P1785
Limb-girdle muscular dystrophies with Clinical difference between myasthenia
autosomal-dominant inheritance gravis patients with an onset age of 50-64
T. Adyan, O. Ryzkova, A. Polyakov and 65
Medical Genetics Research Center, Moscow, Russia W. Sakai1,2, N. Matsui1, Y. Izumi1, R. Kaji1
Limb-girdle muscular dystrophies (LGMD) are an example 1Neurology, Tokushima University Hospital, Tokushima,
of both clinical and genetic heterogeneity. Clinically, by the 2Neurology, Nagasaki Kawatana Medical Center, Nagasaki,
description of non-LGMD phenotypes associated with Japan
LGMD genes and of LGMD phenotypes associated with Introduction: The incidence of elderly-onset myasthenia
originally non-LGMD disease genes; and genetically, by the gravis (MG) has been increasing all over the world. With an
description of new LGMD genes that further increase the aging population the management of elderly-onset MG is
diagnostic complexity. In these diseases genetic consultation becoming an important and challenging aspect of medical
and prevention becomes complicated by this fact. By means practice.
of direct automatic sequencing we analyzed coding Methods: We retrospectively analyzed 47 MG patients with
sequences and adjacent introns of the MYOT, LAMIN and onset age at 50 years and above. We compared the clinical
CAV3 gens, causing LGMD 1A, 1B, 1C, respectively, and characteristics of two groups, the late-onset MG (50-64
DES, CRYAB gens, causing myofibrillar myopathies, years; LOMG) and the elderly-onset MG (65years;
among a sample of 18 unrelated patients with signs of EOMG).
progressing limb-girdle muscular weakness (7 men, 11 Results: 20 EOMG and 27 LOMG patients were enrolled
women). In all subjects vertical segregation of the disease in our study. The EOMG group had more frequent incidence
in the family was observed. LMNA gene mutations were of generalization (p<0.05) and it happened over a shorter
identified in 4 patients (22.22%), one patient had MYOT period. The EOMG group had a lower prevalence of anti-
mutation (5.55%) and one patient had DES mutation acetylcholine receptor antibody and its titres. Both groups
(5.55%). Revealed some features of the clinical picture of received immunomodulatory therapy which mainly
patients with mutations in the analyzed genes. The data consisted of prednisolone and calcineurin inhibitors (CNIs).
obtained may be the basis for the algorithm of molecular 55.6% of LOMG patients and 75.0% of EOMG patients
genetic diagnosis of LGMD with autosomal-dominant received CNIs. The EOMG group had a better response
inheritance. under the lower dose of prednisolone than the LOMG group
after one year of therapy.
Conclusions: The EOMG patients more frequently
experience generalization than the LOMG patients. The
EOMG patients should receive immunotherapy in the early
stage of the disease course.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


384 Posters, Sunday 9 September

P1786 P1787
Stiff person syndrome associated with Response of refractory generalized
Graves disease and thymoma myasthenia gravis to treatment with
S.S. Yoon1, K.J. Hwang1, J.T. Woo2, S.S. Cho3 rituximab: first report from Kingdom of
1Neurology, 2Endocrinology, Kyung Hee University Hospital,
Bahrain
Seoul, 3Pohang, Republic of Korea
I. Ahmed Alsharoqi
Background: Stiff person syndrome (SPS) is a rare Clinical Neuroscience, Salmaniya Med Complex, Manama,
neurologic disorder, in which muscle rigidity and cramps Bahrain
result from immune-mediated inhibition of GABAergic Objective: Myasthenia gravis (MG) is the most frequently
neuron function. SPS is associated with autoimmune encountered neuromuscular disorder of an autoimmune
diseases and paraneoplastic conditions. pathology. Current therapies available, which are mainly
Case: A 50-year-old woman presented with progressive immune modulators or suppressors, include acetyl
stiffness of the trunk muscles and painful spasms in both cholinesterase inhibitors, corticosteroids, azathioprine,
legs. She had difficulty in standing and walking. She was methotrexate, cyclophosphamide, mycophenolate mofetil,
diagnosed with Graves disease at an endocrine clinic and intravenous immunoglobulin (IVIg), plasmapheresis and
had no other significant medical history. Her TSH-binding thymectomy. Many of these therapies carry significant side
inhibiting immunoglobulin, antimicrosomal, anti-glutamic effects and in many instances not a satisfactory
acid decarboxylase (GAD), and anti-acetylcholine receptor improvement.
antibody levels were high. On electromyography, both of Methods: We report two patients with generalized MG who
her lower extremities showed continuous motor unit presented with a rapid down spiral clinical course. Both
activities. A chest CT showed a thymoma, for which she female patients were in a myasthenic crisis with severe
underwent an extended thymectomy. After the thymectomy, bulbar weakness and on mechanical ventilation with
she was treated with diazepam. quadriparesis and facial diplegia and ophthalmoplegia. Both
Conclusion: SPS is a rare disorder that might have various were seropositive, however, they were Musk negative. One
aetiologies. Many immunological possibilities might be of them had malignant thymoma grade 4. Both patients
considered for SPS pathogenesis. received IVIg, plasmapheresis and were on mycophenolate
mofetil in addition to corticosteroids but with poor response.
Results: We initiated rituximab with a mean follow-up of
2.5 years (375mg/m2, on days 1, 8, 15, 28 during the first
month and then one dose every 6 months). The response
was remarkable. In 3 weeks they were off ventilator and
they were then fully independent. After 2.5 years of follow-
up, both patients stopped acetyl cholinesterase inhibitors
and corticosteroids. One of the patients died from the
complication of malignant thymoma.
Conclusion: Rituximab, an anti-CD20 monoclonal
antibody is an effective therapy for refractory MG, including
patients with malignant thymoma, being well tolerated by
patients. This is the first report from Bahrain regarding the
use of Rituximab in Arab patients. We also frequently use it
for other immune related neurological disorders.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 385

P1788 P1790
Dysferlinopathy presenting as Stiff-person syndrome associated with
asymptomatic hyperCKemia invasive thymoma, pemphigus and
M.T. Brum, A.M. Romeiro, P. Santos, R.M. Guerreiro, myasthenia gravis: a case report
J. Pinto Marques X. Gao, X. Zheng, H. Li
Department of Neurology, Centro Hospitalar de Setbal, Neurology, Affiliated Hospital of Medical College, Qingdao
Portugal University, Qingdao, China
Introduction: Dysferlinopathies are autosomal recessive Case report: A 57-year-old woman has suffered from
muscular dystrophies caused by mutations in the dysferlin weakness of four limbs for 1 year and stiffness and rigidity
(DYSF) gene. There are 2 main phenotypes: Miyoshi of her lower limbs for 11 years before presenting to us. The
myopathy (MM) and limb-girdle muscular dystrophy type rigidity was relieved by sleeping, relaxation, and oral
2B (LGMD2B). Both entities show onset in young clonazepam treatment. Repetitive nerve stimulation
adulthood, a slow course and massive increase in serum revealed decrement response when she first felt weakness.
creatine kinase (CK) level. Other rarer phenotypes have A CT scan of the thorax revealed invasive thymoma. 1
been described, expanding the spectrum of clinical years ago, she presented with erythema and blister on her
manifestations and prognosis of dysferlinopathies, in skin, and pemphigus was diagnosed pathologically.
particular the form of asymptomatic hyperCKemia. Pemphigus was treated and significantly improved. Before
Methods: In a female, 24-year-old, Caucasian we the patient first presented with fatigability, the signs of
performed anamnesis, neurological examination and pemphigus had disappeared. Neurological examination
diagnostic tests such as determination of serum CK level, after rest revealed no ptosis or diplopia, normal motor and
EMG, muscle biopsy and genetic study. sensory function. But muscle fatigue was revealed after
Results: The daughter of non-consanguineous parents with sustained exertion. Laboratory tests showed significantly
minor thalassemia and epilepsy since being 11 years old; elevated anti-AchR antibody titre, elevated blood
mother with hyperCKemia without muscular complaints; sedimentation rate, positive anti-nuclear antibody,
no family history of epilepsy. After a seizure, high CK level significantly elevated anti-GAD (glutamic acid
in serum (>10x upper limit of normal) was detected. The decarboxylase) antibody level and positive anti-
patient denied myalgia, cramps or other symptoms; amphiphysin antibody. Nerve conduction studies and needle
observation showed normal strength, without muscle electromyography were normal and repetitive nerve
atrophy or hypertrophy and negative Gowers manoeuvre. stimulation was consistent with MG. A positive result of
EMG was normal. Muscle biopsy showed absent labelling neostigmine test supported the diagnosis of MG. She was
for dysferlin on immunohistochemistry. The genetic study treated with pyridostigmine bromide and prednisolone, and
identified two heterozygous mutations in DYSF gene: the symptoms relieved. Clonazepam 2mg/d was also
c.4003G> A at exon 37 and exon 50 in c.5594delG, administered during that time to relieve her stiffness and her
confirming the diagnosis of LGMD2B. myasthenic symptoms did not worsen.
Conclusion: In addition to typical MM and LGMD2B, the Conclusion: MG, stiff person syndrome and pemphigus in
dysferlinopathies are a clinically heterogeneous group, the same patient, who presents with invasive thymoma, is
ranging from asymptomatic to severe functional disability. thought to be the result of generalized abnormal
In this case only follow-up will clarify, whether this is an immunoregulation due to thymoma.
asymptomatic hyperCKemia or a pre-symptomatic stage.
The dysferlinopathies must be considered in the differential
diagnosis of asymptomatic hyperCKemia.

P1789
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


386 Posters, Sunday 9 September

P1791 P1792
The value of systematic clinical Clinical course of late- and very-late
examinations in determining the involved onset MG
muscles and clinical classification of B. Szyluk1, P. Szczudlik1, M. Lipowska1,
myasthenia gravis patients J. Kubiszewska1, M. Dutkiewicz2, A. Kostera-Pruszczyk1
1Neurology, 2General and Nutritional Biochemistry, Medical
H. Li, P. Liu, X. Gao, T. Liu
Neurology, Affiliated Hospital of Medical College, Qingdao University of Warsaw, Poland
University, Qingdao, China Objectives: In aging societies the incidence of late (>50)
Introduction: The roles of history-taking, fatigue test and myasthenia gravis (MG) increases. The aim of the study
neostigmine test in determining muscle involvement and was to provide clinical characteristics of late MG in Poland.
clinical classification of myasthenia gravis (MG) is Material and methods: Single center register of
unknown. consecutive 424 MG patients, aged 2-89. The clinical
Methods: 64 MG patients were assessed by physician A features were compared between three groups of patients:
with history-taking, fatigue test, neostigmine test and 222 with early-onset MG (EOMG, 15-50), 79 with LOMG
repetitive nerve stimulation (RNS) successively. The value (50.1-65) and 73 with very-late onset MG (VLOMG >65).
of each item mentioned above in determining the involved Results: The female:male ratio was 5:1 in EOMG, 1:2 in
muscles and clinical classification of MG was evaluated by LOMG and 3:4 in VLOMG. Acetylcholine receptor
comparing each step. The results were sent to four other antibody-seropositive patients constituted, respectively 81,
physicians with diverse experience in managing MG. 87 and 92% of the groups. Ocular presentation of MG was
Agreement among different physicians was analyzed with twice more frequent in VLOMG than in EOMG (30
the kappa test. vs.15%). Severe disease course complicated by myasthenic
Results: After step 2, the determination about muscle crisis was observed in 15, 18 and 7% of patients,
involvement was revised in 35 patients, and the Osserman respectively. MG was the only disease in 68% of EOMG,
classification in 11 patients and MGFA classification in 21 23% of LOMG and 13% VLOMG. Thymoma was
patients. After step 3, the scope of muscle involvement was diagnosed in 8% of early-onset, 13% of late-onset and 1.5%
reduced in 9 cases, and classification revised in 6 cases. of very late-onset MG. History of neoplasm (other than
After step 4, the scope of muscle involvement was increased thymoma) had 0.4% of EOMG, 7.6% of LOMG and 12%
in 25 cases, and classification revised in 10 cases. Kappa of VLOMG patients. Maximal MGFA severity was
values among physicians were greater than 0.5 (p<0.001 for significantly milder in patients older than 50.
all comparisons) in each step. Kappa values of Osserman Conclusion: In Polish MG patients older than 50 the
classification were higher than those of MGFA classification clinical course of the disease is more favourable than in the
in all steps. early-onset group, despite high frequency of concomitant
Conclusion: Fatigue test is most important in identifying diseases. Supported by Polish-Norwegian Research Fund
potential muscle involvement and determining clinical Grant PNRF-204-AI-1/07
classification accurately. The neostigmine test improves the
specificity for determining the involved muscles. However,
neither neostigmine test nor RNS influences the
determination of clinical classification. There is relatively
good agreement in clinical classification among physicians.
The agreement of the Osserman classification is better than
that of MGFA classification.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 387

P1793 P1794
The serum level of a proliferation inducing Collagen type-VI related myopathies
ligand (APRIL) in patients with myasthenia spectrum: genotype-phenotype
gravis correlation in two patients
A. Safari, A. Borhani Haghighi, D. Niknam, M. Ghodsi, A.M. Carrilho1, I.M. Cordeiro2, I. Conceio3,
E. Kamali Sarvestani T. Evangelista3
Shiraz University of Medical Sciences, Shiraz, Iran 1Neurology, Hospital de So Bernardo, CHS, EPE, Setbal,
Background: Myasthenia gravis is the most common
2Neurology, Hospital de Faro, Faro, 3Neurology, Centro
immune-mediated disorder of neuromuscular junction. Hospitalar de Lisboa Norte, Hospital de Santa Maria,
About 85% of myasthenic patients have auto antibodies Lisbon, Portugal
against the skeletal muscle acetylcholine receptor (AChR). Introduction: COL6A1, COL6A2, COL6A3 gene
B-cell accumulation in the thymus is a hallmark of mutations cause collagen type-VI (ColVI) myopathies, a
myasthenia gravis and it seems that some B-cell stimulating clinical spectrum at each end represented by Ullrich
factors such as a proliferation inducing ligand (APRIL), congenital muscular dystrophy (UCMD) and Bethlem
could be considered as a therapeutic target in Myasthenia myopathy. Less well-defined intermediate phenotypes are
gravis. described in between. Clinical overlap between spectrum
Methods: The level of A-proliferation inducing ligand phenotypes makes differential diagnosis difficult.
(APRIL) was assessed in the study population (myasthenia Cases report:
gravis patients: 35; healthy controls: 35) using ELISA Case 1: Female, 39 years, full-term born, no abnormal
method. findings at the time of birth. At 4 months of life she reported
Results: Mean serum level of APRIL was 9.625.77pg/mL scoliosis and right hemiatrophy with progressive generalized
in patients and 2.65.58pg/mL in controls; the difference weakness, but had no delay in motor milestones. At the age
was not statistically significant (P value: 0.28).Furthermore of 33, examination revealed gait unassisted, scoliosis, right
the levels of APRIL in various stages of myasthenia gravis elbow and long finger flexors, left Achilles tendons
were significantly differed (P value: 0.94) in our study contractures, cavus feet and proximal muscle weakness.
population. Normal values of CK and Aldose were found, muscle
Conclusion: Against some other autoimmune disorders biopsy showed signs of congenital myopathy. A new
such as systemic lupus erythematosus and rheumatoid alteration on gene COL6A1 was found (c.1352A>G (p.
arthritis, the level of APRIL in the serum of patients with Gln451Arg, exon 20, heterozygoty), with undetermined
myasthenia gravis was not considerably increased and significance.
APRIL might not be the proper treatment target of this Case 2: Female, 29 years, full-term born with neonatal
disorder. hypotonia, congenital torcicollis. No delay in motor
milestones, however, since infancy she reported
osteoarticular deformities, progressive weakness over time
with hyperlaxity of the distal fingers. At the age of 29,
examination revealed gait unassisted, right winging scapula,
scoliosis, equino-varis deformity bilaterally and proximal
weakness. Laboratorial studies were unremarkable, signs of
congenital myopathy on biopsy. DNA examination revealed
three COL6A2 gene mutations [exon 12, c.1105C>T
(p.Gln369X), exon 18, c.1466G>A (p.Arg486Gln) and an
intronic variant, c.1970-9G>A] which confirmed the
diagnosis of UCMD.
Conclusion: Genotype-phenotype correlation studies on
ColVI related myopathies reveal the presence of a
continuous spectrum of phenotypic heterogeneity and
severity, reflecting the various molecular pathogenic
mechanisms.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


388 Posters, Sunday 9 September

P1795 P1796
Creatine kinase elevation, lactacidemia, Pompes disease in South America:
and metabolic myopathy in adult patients a review
with diabetes mellitus O.J. Nascimento, L. Azevedo, C. Pupe, G. Quintanilha,
M. Frank1, J. Finsterer2 F. Naylor, A. Fernandes
1 KAR, Vienna, 2Danube University Krems, Austria Federal Fluminense University, Rio de Janeiro, Brazil
Objective: To determine the frequency of elevated creatine Pompes disease (PD) is related to defects in the acid alpha-
kinase (CK) levels among patients with diabetes mellitus glucosidase enzyme, leading to an intracellular glycogen
and to determine how often elevated CK in these patients is accumulation, mainly in muscles. Two clinical presentation
attributable to primary myopathy. forms are described: infantile and late-onset. In both forms
Methods: In this prospective study, we investigated how the clinical presentation includes progressive proximal
often CK, aspartate aminotransferase, alanine amino weakness, muscular hypotonia, hypertrophic cardiomyopathy
transferase, and resting lactate were elevated among and respiratory complaints. This phenotype is not completely
consecutive diabetic patients attending our clinic. Those with defined and a better knowledge of the clinical presentation
elevated CK values were offered a neurologic work-up. spectrum of PD is needed. The early diagnosis is crucial
Results: 99 patients with diabetes mellitus, aged 19 to 87 facing the actual possibility of treatment. The prevalence of
years, were assessed between May 2008 and April 2010. this condition in some areas over the world is not well known.
7 patients had type 1 diabetes and 92 patients had type To contribute to the knowledge of PD we performed a review
2 diabetes. CK, aspartate aminotransferase, alanine of all cases published in South America.
aminotransferase, and resting lactate were elevated in 19 of Method: A search was made in Pubmed and Lilacs.
99, 25 of 99, 22 of 99, and 24 of 98 patients, respectively. Results: From the 64 reported cases, 30 were classified as
11 of the patients with increased CK were self-injecting infantile and 34 as late-onset PD. In the infantile group, the
insulin. 10 of the 24 patients with elevated serum lactate mean age of onset of symptoms was 1 month. 17 infants
took metformin. 7 of the 19 patients with elevated CK (56%) had symptoms since birth. The mean age at diagnosis
consented to neurologic work-up. 2 of these 7 patients had was 5 months. Most patients had an early death, at a mean
elevated resting lactate. In all 7 patients, the findings from age of 11 months. 5 patients received ERT, and were still
neurologic investigation were indicative of a metabolic alive. PD diagnosis is difficulty due to the rarity of the
myopathy and further diagnostic work-up was initiated. disease and the heterogeneity of clinical manifestations.
Conclusions: In diabetic patients attending our clinic, Conclusion: As an early diagnosis is critical to optimizing
elevated CK levels occur in one-fifth and lactacidemia disease management we can speculate that this number of
occurs in one-quarter. Elevated CK levels are attributable to PD cases in South America is underestimated, and a better
a primary metabolic myopathy in most cases. Elevated CK phenotypic identification is needed in order to avoid
levels in the setting of diabetes mellitus require further misdiagnosis of the disease.
neurologic work-up.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 389

P1797 P1798
Post-operative mortality in thymoma Childhood onset myasthenia gravis with
associated with myasthenia gravis thymoma
L. Ionescu1, D.I. Cuciureanu2,3 A. Nikolic1, D. Nikolic2,3, B. Medjo2, P. Ivanovski2,3,
Surgery, University of Medicine and Pharmacy Gr.T. Popa,
1
I. Basta1,3, D. Lavrnic1,3
Neurology, University of Medicine, 3Neurology, Emergency
2 1Department for Neuromuscular Disorders, Clinical Center

Hospital Prof. Dr. N. Oblu, Iasi, Romania of Serbia, Clinic for Neurology, 2University Childrens
Introduction: Myasthenia gravis (MG) associated with Hospital, 3University of Belgrade, School of Medicine,
thymoma is a real challenge for the therapeutic team as its Belgrade, Serbia
course is unpredictable and refractory. Aggravation of MG Introduction: Juvenile myasthenia gravis is acquired,
post-operatively may lead to death and this can be explained autoimmune myasthenia gravis occurring before 16 years of
by tumour recurrence or exacerbation of extratimic age. Thymoma are exceedingly rare in children, especially
autoimmunity. associated with juvenile myasthenia gravis. We report a
Aims: Assessment of post-operative death rate in operated case of a 14-year-old boy with juvenile myasthenia gravis
patients. and thymoma.
Material and methods: 21 patients with thymoma Case report: Our patient presented with difficulties in
associated with MG underwent complete thymectomy from chewing and swallowing, nasal speech and fluctuating
1990 to 2010. 6 patients died between 3 months and 16 weakness of the leg muscles. Neurological examination
years post-operatively. revealed masticatory and bulbar muscle weakness with
Results: All patients who died had an invasive thymoma nasal speech, proximal muscle weakness and fatigability of
associated with Osserman clinical form of MG, III and IV. the arms and legs and distal muscle weakness of the legs,
Two deaths were unrelated with the operated disease but 4 MGFA form of the disease IIIB. Diagnosis of juvenile
died due to aggravation of MG and acute respiratory failure. myasthenia gravis was confirmed by positive neostigmine
The overall mortality rate was 24% and MG related caused test, decremental response on repetitive nerve stimulation
mortality rate was 18%. The 2 patients who died of unrelated and increased titre of serum anti-acetylcholine receptor
MG cause, survived 9 and 16 years post-operatively. 1 antibodies. The patient was treated with anticholinesterases,
patient died due to acute cardiac failure following a tight corticosteroids, Azathioprine and thymectomy. Patho
constrictive pericarditis presumed to be a side effect of post- histological analysis of the thymus gland showed thymoma,
operative radiotherapy. The second patient presented at 10 Masaoka grade II. After two years of unstable course of the
years post-operatively a pleural recurrence which was disease remission was achieved.
removed and survived another 6 years till he died of acute Conclusion: Since there are only ten published thymoma-
myocardial infarction. 4 patients who died due to associated myasthenia gravis cases in the paediatric
aggravation of myasthenia gravis, had an Osserman clinical population so far, this paper gives an important contribution
stage III and IV on admission. All had an invasive thymoma. to better understanding of this rare association.
Conclusions: Aggravation of myasthenia gravis is the first
cause of death in this series in spite of intensive anti-
autoimmunity treatment. Tumour recurrence is rare in
complete resected cases.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


390 Posters, Sunday 9 September

P1799 P1800
Quality of life in myasthenia gravis Thyrotoxic periodic paralysis
patients in Belgrade (Serbia) J. Neumann1, J. Pouzar1, J. Kubk2, P. Jeil3
I. Basta1,
T. Pekmezovic2,S. Peric1,
D. Kisic Tepavcevic3,
1Neurology and Neuro ICU, 2Neurology, 3Internal Medicine
V. Rakocevic Stojanovic1, Z. Stevic1, A. Nikolic4, and Cardiology, Regional Hospital Chomutov, Czech
M. Milicev4, D. Lavrnic1 Republic
1Neurology Clinic, 2Neurology Clinic, Institute of Introduction: Thyrotoxic periodic paralysis (TPP) is a rare
Epidemiology, Clinical Center of Serbia, School of Medicine, complication associated with hyperthyroidism. TPP is more
University of Belgrade, 3Institute of Epidemiology, School of frequent in young patients, especially males. TPP is well-
Medicine, University of Belgrade, 4Neurology Clinic, known in the Asian population compared to other ethnic and
Clinical Center of Serbia, Belgrade, Serbia racial groups.
Objectives: The aim of this study was to assess factors that Case report: We report a case of TPP in a 40-year-old
might influence the health-related quality of life (HRQoL) Caucasian male with a history of hyperthyroidism. The
in patients with myasthenia gravis (MG). patient presented acutely to our department with 5 hours
Material and methods: A cross-sectional study was history of severe muscle weakness and pain. On admission
performed including 230 consecutive patients with MG. he presented with symmetric flaccid paralysis of the lower
Severity of the disease was estimated according to the limbs without sensory symptoms or signs. His initial
MGFA classification and QMG score. HRQoL was assessed potassium level was 1.8mmol/L (3.6-5.1) with normal acid-
by SF-36 questionnaire. Depressive and anxiety symptoms base status. Laboratory investigation on admission revealed
were assessed using the Hamilton rating scales for mild hypophosphatemia and hypomagnesemia. An
depression and anxiety, respectively. Social support was electrocardiogram showed sinus tachycardia and mild
measured by the Multidimensional Scale of Perceived depression ST in lower wall. His free T4 level 64.9pmol/L,
Social Support (MSPSS), while acceptance of the disease free T3 level 18.66pmol/L and TSH <0.005mU/L. He was
by the Acceptance of illness scales (AIS). given intravenous and oral potassium supplements
Results: Our results revealed that HRQoL of MG patients (100mmol over 15 hours) and the paralysis resolved rapidly
was reduced, not only in the domains related to physical with normalization of serum potassium. While in the
health, but also in psychosocial aspects of the HRQoL. The hospital he was treated with thiamazol and metoprolol. He
significant demographic predictors of the worse HRQoL in was discharged in good condition on day 3.
MG patients were older age (p=0.025) and lower education Conclusion: TPP is a rare condition in Caucasians that can
(p=0.012). Among clinical features, significant independent be overlooked and misdiagnosed on presentation. TPP is an
contributing factors of worse HRQoL were more severe alarming and potentially fatal complication of thyrotoxicosis
form of the disease according to MGFA (p=0.001) and characterized by progressive muscle paralysis and
higher QMG score (p=0.001). Finally, psychosocial hypokalemia due to a massive intracellular shift of
predictors of worse quality of life were lower MSPSS score potassium. In TPP, hypokalemia is associated with
(p=0.001), poor acceptance of the disease (p=0.001), as hypophosphatemia and mild hypomagnesemia. Early
well as higher levels of anxiety and depression (p=0.001). diagnosis and immediate therapy prevent serious
Conclusion: Our study revealed that the HRQoL in patients cardiopulmonary complications and may hasten the
with MG is similarly reduced in its psychological and recovery of muscle paralysis.
physical aspects. These results may have a practical
implication pointing out that different aspects of
psychosocial support should be added to the regular
therapeutic protocols.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 391

P1801 P1802
Association between vitamin D receptor Myasthenia gravis patients quality of life
gene polymorphism and myasthenia in Belarus
gravis in Han people from northern China S. Kulikova, H. Osos, S. Likhachev, A. Astapenko
H. Li1, Z. Wang2, X. Gao1, L. Tao1, H. Yu1 Neurology, The Republican Research and Clinical Center of
Neurology, Affiliated Hospital of Medical College, Qingdao
1 Neurology and Neurosurgery, Minsk, Belarus
University, 2Neurology, The Third Peoples Hospital of Myasthenia gravis (MG) is a chronic neuromuscular
Qingdao, China disease. Advances in medical therapy have increased the
Introduction: Vitamin D plays an immuno-modulating role life expectancy of MG patients, without completely curing
via vitamin D receptor (VDR), which has been shown in the disease. To analyze quality of life (QoL), an Belarus
many autoimmune diseases. MG cohort was investigated.
Methods: VDR FokI and ApaI polymorphism was Material and methods: 83 patients with MG and 48
determined by PCR-RFLP method. The frequencies of healthy people were asked with questionnaire QoL SF-36.
genotype and alleles were compared among different The SF-36 consists of eight scaled categories: physical
subgroups classified by gender, age of onset, association of functioning, physical role, bodily pain, general health,
thymoma, Osserman types and maximal severity in the emotional role, mental health, vitality and social functioning.
follow-up. The relationship between the genotype and For the statistical analysis, Statistica 6.0, Mann-Whitney
severity of MG and short-term (three months after treatment test, Spearman correlation analysis were used.
initiation) therapeutic effects of glucocorticoid were also Results: The average age of the patients was 44.216.4
explored. years, 10.8% of them were 65 and older. In most cases
Results: There were no significant differences in generalized MG was diagnosed (80.7%). 75.9% of the
frequencies of genotypes and a1leles between MG group patients had been treated with immunomodulating therapy.
and control group, and among subgroups of MG (p>0.05). In 88% of cases the disease duration was longer than one
The distribution of FokI genotypes and alleles showed no year. 34.9% of the patients had constant disability. All the
statistical difference between the patients with better and figures of SF-36, except for bodily pain, were significantly
worse therapeutic effects of glucocorticoid (p>0.05). The lower than in healthy people (p<0.001). The lowest indicator
frequency of ApaI alleles in the patients with better was found in terms of physical role [25.0(0.0;75.0)] despite
therapeutic effects of glucocorticoid differed from the the fact that physical functioning was disrupted to less
worse group (c2=4.400, p=0.036), while ApaI genotypes extent [60.0(37.5;85.0)]. Physical health was negatively
were not associated with the therapeutic effects of associated with the patients age (R=-0.29; p<0.005),
glucocorticoid. The frequency of ApaI A in the patients with disease duration (R=-0.26; p<0.01), severity of disease
better therapeutic effects group (29.6%) was higher than in (R=-0.4; p<0.0001), therapy with immunomodulating drugs
the worse group (14.6%) (OR=2.46, 95% CI=1.04~10.43, (R=-0.21; p<0.05), disability (R=-0.34; p<0.001). Mental
p=0.036). The patients with the genotype AA or Aa were health was negatively associated with severity of disease
more prevalent in the better therapeutic effects group (R=-0.23; p<0.05).
(51.6%) than in the worse group (29.2%) (OR=2.59, 95% Conclusion: Low health-related QoL of patients with MG
CI=0.98~14.60). requires further analysis to improve therapy of this disease.
Conclusion: VDR FokI and ApaI polymorphism was not
found to be related with the pathogenesis of MG. MG
patients with VDR ApaI A allele may have better short-term
efficiency of glucocorticoid.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


392 Posters, Sunday 9 September

P1803 P1804
Conns syndrome presenting as periodic Tetraparesis as a form of presentation of
paralysis associated with diuretics dermatomyositis
I. Son1, M. Yi1, Y. Kim2, J. Jeong3 M. Leito Marques, J.N. Carvalho, J. Fortuna,
1Department of Neurology and Inam Neuroscience Research A. Morgadinho
Center, Sanbon Medical Center, Wonkwang University, Centro Hospitalar e Universitrio de Coimbra, Portugal
Gunpo-si, 2Neurology, Sungae General Hospital, 3Neurology, Objectives: To describe a case of sub-acute tetraparesis.
Gangnam Severance Hospital, Yonsei University, Seoul, Methods: Monitoring and study of a patient for 20 months
Republic of Korea and case description.
Introduction: Primary hyperaldosteronism or Conns Results: A women, 82 years old, went to Neurology
syndrome featured arterial hypertension, suppressed plasma department due to weakness of gradual onset within one
renin activity, and increased plasma aldosterone month and urinary incontinence. She denied pain, dysphagia
concentration. In most cases (>90%) primary or other sensory complaints. History of flu-like syndrome
hyperaldosteronism attributed to bilateral adrenal 2-3 weeks before. She had a cervical spine MRI showing
hyperplasia or aldosterone-producing adenoma. degenerative changes, without compromise of the spinal
Case: A 51-year-old man presented with bilateral limb cord. Neurological examination revealed tetraparesis,
weakness. He had a history of hypertension medication 5 hyperreflexia, cutaneous plantar responses in flexion. The
years ago, and added hydrochlorthiazide 12.5mg 2 months cranial nerve and sensory exam was normal. Blood analysis
ago. As a first manifestation, the patient cannot raise arms showed CPK of 11,000, increased transaminases, and
but was not disturbed when he gripped. The symptoms increased erythrocyte sedimentation rate. The serum
prolonged for 3~4 days and improved partially. After 1 autoimmunity study showed positive ANAs.
week, bilateral limb tingling sensation and weakness Electromyography was consistent with an acquired
developed, so he could not go upstairs. These symptoms myopathy. In this context, we exhaustively researched a
improved also in 3~4 days. These transient paralyses were neoplasia with complementary exams, which revealed no
repeated of improvement and aggravation. EKG showed positive findings. The patient started therapy with
QT prolongation. The motor grade was proximal grade IV/ metilprednisolone 1g (5 days), and was discharged with
IV, distal V/V. Plasma serum K was 2.3mmol/L and CPK prednisolone 60mg id. At that time, the most likely diagnosis
was 1815U/L. EMG findings are moderate denervation was polymyositis. Later, the patient came back to the
potentials but normal MUAP in bilateral FCR, FDI, hospital with pain on muscle mass palpation, easy fatigue
gastrocnemius (medial), tibialis anterior, vastus lateralis and dysphagia. Muscle biopsy was compatible with
muscles. Baseline plasma renin (0.14ng/ml/hr) decreased dermatomyositis.
than normal and aldosterone (531.9pg/ml) level was Conclusions: The muscle diseases constitute a broad range
elevated. Abdominal CT showed the right adrenal 1.1cm of diagnosis. This case describes a less typical presentation
sized mass. Oral potassium agent was taken, the patient of a (rare) disease. The rapid progression of symptoms
recovered quadriparesis approximately 10 days after. demanded an efficient response and diagnosis. In a patient
Conclusion: Periodic hypokalemic paralysis can show a aged 80, female, who presents with an acute/sub-acute
first manifestation of primary aldosteronism and diuretics muscle disease, it was mandatory to exclude a paraneoplastic
can aggravate hypokalemia. This is a case of hypokalemic syndrome. The atypical clinical picture, like the absence of
myopathy proceed primary aldosteronism after diuretics cutaneous rash, made this an unexpected diagnosis.
medication and presented bilateral limb weakness is worth
of notice. Relatively young patients with uncontrolled blood
pressure consider of secondary hypertension and should
take detailed history taking and evaluation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 393

P1805 P1810
MELAS syndrome: a report of two cases A polymyositis case with cardiac
and review of the literature involvement as the first symptom
M. Lahmar, H. Derbali, H. Khaled, C. Ben Cheikh, E. Matsutani1, H. Mori1, K. Goto2, K. Shindo1
M. Mansour, J. Zaouali, R. Mrissa 1 Neurology, Kurashiki Central Hospital, Kurashiki,
Neurology, Military Hospital, Tunis, Tunisia 2 Neurology, Omuta Hospital, Omuta, Japan

P1806 P1811
Internuclear ophthalmoplegia revealing Phenotypic features in the first Greek
myasthenia gravis: a case report patient with desmin mutation
M. Lahmar, H. Derbali, A. Baatout, C. Ben Cheikh, P. Manta1, G. Papadimas1, C. Papadopoulos1, S. Penttil2,
J. Zaouali, R. Mrissa A. Areovimata1, B. Udd2,3
1Department of Neurology, Aeginition Hospital, National and
Neurology, Military Hospital, Tunis, Tunisia
Kapodistrian University of Athens, Greece, 2Neuromuscular
Research Unit, Department of Neurology, University
P1807 Hospital and University of Tampere, 3Folkha Lsan Institute
Different patterns of dystrophin gene of Genetics and Department of Medical Genetics, Haartman
Institute, University of Helsinki, Finland
expression in an Egyptian family suffering
from muscular dystrophy
G.A. Achmawi1, N. Elsawy2, H. El Assi3, H. Kassem3, P1812
I. Talaat4, S. El Guendi4 A first case of oculopharyngeal muscular
1Neurology Department, 2Physical Medicine, Rheumatology dystrophy in the Slovak Republic
and Rehabilitation Department, 3Genetics Department,
4Pathology Department, Faculty of Medicine, Alexandria J. Martinkova1, P. Spalek2, V. Hancinova2, M. Cingelova1,
University, Alexandria, Egypt J. Benetin2
12nd Department of Neurology, Comenius University, 2Centre
for Neuromuscular Diseases, Department of Neurology,
P1808 Slovak Medical University, Bratislava, Slovak Republic
Non-coronary myocardial infarction (MI)
caused by pyridostigmine treatment in P1813
myasthenia gravis (MG): a case report Two siblings with myotonic dystrophia,
P. Zis1, E. Kontogeorgi1, D. Karakalos1, S. Dimopoulos2, one of them with tetraventricular
A. Tavernarakis1 hydrocephalus and hyperprolactinemia
1Department of Neurology, Evangelismos General Hospital,
21st
Critical Care Medicine Department, Evangelismos S.M. nar, L. zden Erkutlu, S. Bilge, R. Karahan
General Hospital, National Kapodistrian University of zcan, M. Beer
Athens, Greece Taksim Training Hospital, Istanbul, Turkey

P1809 P1814
Cardiac assessment in boys with The value of psychological and
Duchenne muscular dystrophy degenerative changes of vertebral column
D. Vojinovic1, V. Milic Rasic1, T. Ilisic2, A. Kosac1, in sympathetic intrafusal tension
S. Todorovic1 syndrome
1Clinic of Neurology and Psychiatry for Children and Youth,
I. Andronati1, S. Plesca1, M. Sangheli2, I. Zabrian2
Faculty of Medicine, University of Belgrade, 2University 1Department of Back Pain, Institute of Neurology and

Childrens Hospital, Belgrade, Serbia Neurosurgery, 2State Medical and Pharmaceutical


University, Chisinau, Moldova

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


394 Posters, Sunday 9 September

Neuroepidemiology P1817
Age-specific incidence, comorbidities,
P1815 and mortality in patients with alcohol
Neurology on-call coverage in a Spanish dependence syndrome in Taiwan:
tertiary academic hospital: a prospective a nation-wide population study
one-year study C.-J. Lu, Y. Sun
P.M. Rodriguez Cruz, J.R. Perez Sanchez, J.P. Cuello, Neurology, En Chu Kong Hospital, New Taipei City, Taiwan
G. Vicente Peracho, P. Sobrino Garcia, F. Cordido, R.O.C.
C. Alarcon Morcillo, B. De La Casa Fages, D. Mateo, Introduction: Alcohol abuse is one of the main causes of
A. Garca Pastor, F. Diaz Otero, D. Ezpeleta repeated admission with various medical complications. We
Hospital General Universitario Gregorio Maraon, Madrid, investigated the sex-, age-specific incidence, prevalence,
Spain comorbidities and all-cause mortality in people with
Objectives: To analyze the features of neurological alcoholism.
emergency care of a tertiary hospital (referral area: 750,000 Methods: This retrospective cohort study was based on the
inhabitants; 1700 acute-beds) during a one-year period. To medical claims data of the Bureau of National Health
compare this data with the whole medical emergency room Insurance from 1997 to 2008, which contains 1,000,000
(ER). randomly selected beneficiaries. Patients with diagnoses of
Background: Prospective data about neurological patients alcohol dependence syndrome (ICD-9: 303) or alcohol
attended in ER are scarce. Its knowledge may help to abuse (305.0) on outpatient claims and/or hospitalization
optimize resources. records were enrolled. We estimated the annual incidence of
Methods: Prospective and descriptive study, using a alcohol dependence syndrome and related medical
computer database, of the patients assisted by the neurology complications. Cox proportional hazard model was
ER team from July 2010 to July 2011. performed to compare the risk of comorbidities and the
Results: After debugging the database, 290 days were survival rates for each group.
considered valid to analyze. 3,236 patients were included. Results: A total of 4334 patients (3263 men, 75.3%)
Average: 11.15 patients/day. Men 48%. Time distribution: diagnosed with alcohol dependence syndrome or alcohol
8am-3pm=30%, 3pm-10pm=46%, 10pm-8am=23%. The abuse were identified with mean age 40.513.1 years and
neurology team evaluated at first instance 35% of patients. followed up for 57.037.4 months. The overall annual
The remaining 65% were initially evaluated by other incidence is 2.20 in men and 0.74 in women per thousand
specialists. Most common pathologies were stroke 34%, person years, with prevalence highest in subjects aged
seizures 16%, headache 8%, demyelinating 5%, neuro- 45-54 years old with (19.74 per 1000). As compared to sex-
ophthalmology 5%, altered level of consciousness and and age-matched control group, people with alcoholism
dizziness 6%, neuromuscular 3%, movement disorders 3%. have significantly higher risk of dementia, epilepsy, liver
We performed 58 intravenous thrombolyses. Average time cirrhosis and gastric ulcer (all p<0.001). The mortality rates
in ER: 7.17 hours/patient. Destination: admission 28%, in alcoholism and control group are 46.5 and 17.4 per 1000
discharge 25%, outpatients 30%, other departments 7%, person-years, respectively (p<0.001).
intensive care unit 2%, death 1%. In 2009, 73,701 patients Conclusions: People with alcohol abuse and dependence
were evaluated in the medical-ER. Our data reveal that have higher risk of dementia, epilepsy, liver cirrhosis, and
neurology on-call coverage represents 4.40% from the all-cause mortality, affecting predominantly subjects
medical-ER assistance and 5.39% from the total number of younger than 55 years.
urgent admissions.
Conclusion: Neurological disorders in ER are frequent,
wide and complex. Neurological evaluation is frequently
requested by other specialists. Stroke, seizures and headache
prevail in our area. The admission rate in the neurology
department is higher than the rest. These data support the
provision of neurology teams on-call within ER.

P1816
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 395

P1818 P1819
Oral antiplatelet and oral anticoagulant Prevalence of adolescence and adult
drugs used in prevention of onset ataxias in the Cypriot population
cardiovascular diseases in the elderly in K. Christodoulou1, C. Votsi1, P. Nicolaou1, A. Georghiou1,
Poland M. Pantzaris1, S. Papacostas1, K. Kleopa1, Y.-P. Christou1,
B. Labuz-Roszak1, K. Pierzchala1, M. Skrzypek2, G.A. Tanteles1, V. Anastasiadou1,2, T. Kyriakides1,
A. Machowska-Majchrzak1, PolSenior Study Group E. Zamba-Papanicolaou1
1Dept of Neurology, Medical University of Silesia, Zabrze, The Cyprus Institute of Neurology and Genetics, 2Makarios
1

2Medical University of Silesia, Katowice, Poland Hospital, Nicosia, Cyprus


Introduction: The aim of this study was to evaluate the Introduction: The epidemiology of ataxias in Cyprus has
frequency of use of oral antiplatelet (OAP) and anticoagulant not been reported. A single report describing a high
(OAC) drugs among older people in Poland and to assess its frequency of Friedreichs ataxia (FRDA) in the western part
association with cardiovascular risk factors. of the country was published in 1988. The Cyprus Institute
Material and methods: The study was based on data of Neurology and Genetics (CING) is the national referral
collected during the implementation of the multicenter, centre for ataxias and other neurological diseases.
publicly funded research project PolSenior. Methods: Clinical and genetic data on adolescence and
Results: The study group consisted of 4,979 people in the adult onset ataxia patients were collected from the CING
average age of 79.358.69 years. Among them, 1,787 registry. At the last census (01 October 2011) the total
people (35.9%) used at least one drug for prevention of number of inhabitants under the Cyprus Government
cardiovascular diseases. OAP were used regularly by 1,648 control area of the country was 838,897.
(33.1%), OAC by 165 respondents (3.3%). Aspirin was Results: On prevalence day (28 March 2012), 99
most often administered (32.2%). Frequency of examined adolescence and adult onset ataxia patients were alive. 9 of
drugs use depended significantly on age, sex, place of them were excluded as they live in the Turkish area where
residence, level of education and personal income. Among the total number of inhabitants is unknown. Total prevalence
all the respondents treated with OAP, the therapy was was 10.7 per 100,000 inhabitants. The prevalence rate was
applied as secondary cardiovascular prevention in 717 1.5 for molecularly confirmed FRDA, 0.5 for molecularly
respondents (43.5%). Aspirin as the primary prevention was confirmed ataxia with oculomotor apraxia 2 [AOA2, 1
used by 705 people (42.8%). Among the respondents treated family], 3.0 for autosomal recessive cerebellar ataxias (both
with OAC, there were 117 (71%) with history of atrial FRDA and AOA2 excluded), 0.2 for autosomal dominant
fibrillation. Secondary cardiovascular prevention should be ataxias and 5.5 for late onset sporadic ataxias.
considered in further 482 respondents (15.1% of untreated Discussion: The prevalence of adolescence and adult onset
ones), primary in 1447 respondents (45.3%). ataxias in our population is similar to that recorded in other
Conclusions: Aspirin was the most commonly used drug populations, but notably, autosomal dominant ataxias are
for the prevention of cardiovascular diseases in the elderly not as prevalent [2 patients in 1 family] as in other
in Poland, but it seemed to be underutilized in high-risk populations.
patients. Among the elderly with atrial fibrillation, OAC are
used too rarely. It is necessary to develop educational
programs among general practitioners concerning current
recommendations for the use of OAP and OAC.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


396 Posters, Sunday 9 September

P1820 P1821
Study of the influence of depression on Personal anxiety and risk of development
the risk of stroke at men aged 25-64 of stroke in a population of men, 25-64
during 14 years in Russia (WHO program years old during 24 years in Russia
MONICA-psychosocial) (epidemiological research of program
V. Gafarov1,2, E. Gromova1,2, A. Gafarova1,2, I. Gagulin1,2 WHO MONICA)
Collaborative Laboratory of Epidemiology of
1
V. Gafarov1,2, E. Gromova1,2, A. Gafarova1,2, I. Gagulin1,2
Cardiovascular Diseases SB RAMS, 2Institute of Internal 1Collaborative Laboratory of Epidemiology of
Medicine SB RAMS, Novosibirsk, Russia Cardiovascular Diseases SB RAMS, 2Institute of Internal
Background and purpose: We sought to examine the Medicine SB RAMS, Novosibirsk, Russia
relationship between depression symptoms and the risk Objective: To evaluate an influence of personal anxiety on
development of stroke among men aged 25 to 64 years. risk of development of stroke in men aged 25-64.
Methods and materials: Within the framework of program Study design and methods: Within the framework of
WHO MONICA-MOPSY a representative sample of men program WHO MONICA-MOPSY a representative sample
25-64 years old (in the year 1994) was examined. Total of men 25-64 years old (in the years 1984, 1988, 1994) was
sample was 657 persons. Depression symptoms were examined. The total sample was 2149 persons. The period
measured at baseline with the use of the MONICA - of supervision was 24 years. We counted as outcome all
psychosocial Interview Depression scale. The incidence of cases of stroke which had arisen for the first time. We used
new strokes was ascertained under systematic surveillance Spielbergers scale for estimation of personal anxiety (PA).
the 14-year follow-up. Cox - proportional regression model Cox-proportional regression model was used for estimation
was used for an estimation of relative risk (RR). of relative risk (RR).
Results: Prevalence of depression in a cohort of men with Results: 72.2% men with developed stroke had HLA (high
stroke was 70.6%. The relative risk of ischemic stroke level anxiety) and 27.8% ALA (average level anxiety). The
incident was 6.4 (95% CI=3.97-7.58) for those who showed relative risk of developing stroke within 5 years in a group
symptoms of depression compared with those who did not. of men with HLA in comparison with a group of men with
Within 10 years RR of developing stroke RR=5.2 (95% CI, ALA was 6.4 times higher (95%CI=3.088-13.308,
3.6-6.91). Within 14 years RR for stroke 1.4 (95%CI=0.01- (p<0.0001) for stroke. Within 10 years, RR of stroke
1.78 p>0.05).MONICA development increased 3.8 times (95%CI=1.677-8.754,
Conclusion: Depression is a predictor of stroke in middle- p<0.001). Within 15 years, RR of stroke -2.9 times
aged men. (95%CI=1.07-7.952). Within 20 years the tendency to RR
decrease for development of stroke (RR=1.6) was observed.
Within 24 years (RR=1.04) p>0.05.
Conclusion: The data indicate that high level of personal
anxiety may predict higher stroke risk in middle-aged men.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 397

P1822 P1823
The medical economic cost of patients Nutritional effects on depressive
with alcohol dependence syndrome in symptoms in Parkinsons disease patients
Taiwan T. Fukushima1, X. Tan2, Y. Luo3, P. Wang3, J. Song3,
Y. Sun, C.-J. Lu H. Kanda1, T. Hayakawa1, T. Kakamu1, M. Tsuji1
1Hygiene & Preventive Medicine, Fukushima Medical
En Chu Kong Hospital, New Taipei City, Taiwan R.O.C.
University, Fukushima, Japan, 2Wuhan University, Wuhan,
Introduction: Alcohol abuse has been identified as an 3Xiangfan No.1 Hospital, Xiangfan, China
important risk factor for chronic disease and injury. In spite
of the huge burden of their repeated admission, morbidities Introduction: The factors associated with depression in
and social problems, the medical cost of patients with Parkinsons disease, especially lifestyle or nutrition is
alcohol dependence syndrome in Taiwan has not been poorly studied. Our study was conducted to investigate
thoroughly investigated. nutritional factors associated with depression in Parkinsons
Methods: This retrospective cohort study enrolled patients disease.
with diagnoses of alcohol dependence syndrome (ICD-9: Methods: The subjects comprised 82 Parkinsons disease
303) or alcohol abuse (305.0) from 1997 to 2008 based on patients and sex- and age-matched controls recruited from
the medical claims data of 1,000,000 randomly selected the outpatient clinic of Xiangfan No. 1 Peoples Hospital in
beneficiaries from the Bureau of National Health Insurance. Hubei, China between 2006 and 2008. The Parkinsons
We compared the overall medical cost of alcoholic patients disease patients were divided into two groups, 24
with and without medical complications of dementia, Parkinsons disease with depression and 58 Parkinsons
epilepsy, liver cirrhosis or gastric ulcer, as well as the costs disease without depression. DSM-IV criteria were used for
of an age- and sex-matched control group. the primary diagnosis of depressive disorders. The severity
Results: We enrolled 4334 alcoholic patients and 4334 of depression in patients was assessed by the criteria of the
subjects of the control group. In both groups, subjects Hamilton Depression Rating Scale (HAMD-17), and the
complicated either with dementia, epilepsy, liver cirrhosis, score of 14 was defined as depression. Eating habits and
gastric ulcer or gastritis have much higher monthly medical other factors were obtained by recalling on previous state
costs than those without medical morbidities (all p<0.001). before diagnosis of Parkinsons disease and depression.
Besides, among patients with at least one aforementioned Results: Compared with the Parkinsons disease patients
medical disease, those with underlying alcohol dependence without depression and the controls, the Parkinsons disease
syndrome have significantly higher overall medical costs patients with depression showed relatively low income and
than those not used to be alcoholics (p<0.001), with cost low education. Fat and vitamin B1 intake was significantly
differences mostly significant in women or young people higher in the Parkinsons disease patients with depression
aged less than 45 years (all p<0.001). than in both the Parkinsons disease patients without
Conclusions: People with alcohol abuse and dependence depression and the controls. Bean intake was significantly
have significantly higher overall medical costs, especially lower in the Parkinsons disease patients with depression
in women or people younger than 45 years. than in both the Parkinsons disease patients without
depression and the controls.
Conclusion: Low fat and vitamin B1, and high bean intake
might be associated with lower risk for depression in
Parkinsons disease patients with a better educational
background and income bracket.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


398 Posters, Sunday 9 September

P1824 P1825
Different neurological conditions between Trends and seasonal variation in stroke
urban and rural samples from central hospitalization rates in Aragon (Spain)
Colombia: the role of chronic diseases from 1998 to 2010
M.E. Habeych Sanmiguel1, J.M. Garcia2, A. Gimenez-Muoz1, J.R. Ara2, J.M. Abad3,
R.C. Castilla-Puentes3 I. Campello-Morer1, J. Marta2, J.I. Lpez-Gastn2
1Neurological Surgery, University of Pittsburgh, PA, USA, 1Department of Neurology, Royo Villanova Hospital,
2Internal Medicine, Fundacion Cardiovascular, 2Department of Neurology, Miguel Servet Hospital,
Bucaramanga, Colombia, 3Epidemiology, Temple University, 3Department of Health and Consumer Affairs, Government

Philadelphia, PA, USA of Aragon, Zaragoza, Spain


Introduction: Despite availability of neuroepidemiological Background: While stroke mortality in Spain has decreased
data from developing countries including Colombia, urban- significantly in the last decade, trends in stroke
rural differences on neurological conditions have never hospitalization and seasonal variation are less known.
been informed. In countries or places where information Objectives: To evaluate seasonal variation and trends in
cannot be collected otherwise, hospital samples from hospitalization for stroke in a Spanish region (Aragn) over
tertiary centres are acceptable. Thus, our objective is to a period of 13 years.
identify differences in the spectra of neurological encounters Methods: We identified retrospectively all stroke patients
between two samples, one urban and one rural from central admitted to acute care hospitals in Aragon between January
Colombia. 1, 1998, and December 31, 2010, through the Minimum
Material and methods: We compared the relative Basic Data Set - Health Service of Aragon. The major
frequencies of neurological encounters in third level inclusion criterion was an admission with a principal
services, from an urban sample published from Bogot diagnosis of acute stroke as identified through the ICD-
(N=2.932), to our rural sample from Tunja (N=2.664), 9CM system (430, 431, 433.x1, 434.x1, and 436).
collected both circa to 2.000. The classification of Negative binomial regression models were performed for
neurological conditions used is based on the 9th revision of trends analysis. A simple moving average was used to
the International Classification of Diseases, clinical examine monthly variation of stroke hospitalization rates.
modification (ICD-09-CM). A clustered sampling was used. Results: We recorded 21,581 (77%) ischemic strokes, 5,314
The information was collected in a format designed for this (19%) intracerebral haemorrhages and 1,127 (4%)
specific purpose in the United Kingdom, and already tested subarachnoid haemorrhages. Age and sex standardized
in Colombia. hospitalization rates for ischemic stroke showed a
Results: Highly significant statistical differences (df:1,1; significant decrease (9.7% in men and 5.4% in women).
p<0.0001) were found for cerebrovascular disease, seizure Intracerebral haemorrhage rates were highest in 2001 in
disorder, headache, Parkinsons disease and other movement men and in 2006 in women, with a subsequent decrease till
disorders, and, inflammatory (infectious) conditions. 2010. All stroke subtypes were more frequent in winter.
Neuro-developmental disorders (df:1,1; p=0.0029), Conclusions:
dizziness and balance problems (df:1,1; p=0.0018), and - There has been a declining trend in stroke hospitalization
neuropathies (df:1,1; p=0.0007), also showed statistical rates in Aragon in the period from 1998 to 2010.
significant differences. - There was a temporal variation of stroke hospitalization
Discussion and conclusions: Our study showed significant rates. These findings may help in developing strategies for
differences in all categories and diagnostics tested between planning health care.
urban and rural samples. Cerebrovascular disease, although
the most frequent reason of neurological consultation in our
rural sample, could be confounded by the concomitant
presence of cardiovascular and/or pulmonary conditions in
the rural, aged population of this Colombian area.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 399

P1826 P1827
Is the co-operation between healthcare Epidemiology of brain and spinal cord
participants more difficult in a poor social tumours leading to disability in Belarus in
environment associated with a higher risk 2006-2011
of stroke? N.A. Yakushina, T.P. Dubovik, T.S. Golubeva,
A. Folyovich1, I. Vastagh2, A. Kri2, A. Majoros1, A.V. Kopytok
K.L. Kovcs2, A. Ajtay2, Z. Laki1, B. Gunda2, K. Erdei1, Scientific Informational and Organizational Support,
L. Lenti2, Z. Dnos1, D. Bereczki2 National Science and Practice Centre of Medical Assessment
1 Neurology and Stroke Center, Szent Jnos Hospital, and Rehabilitation, Gorodische, Belarus
2 Neurology, Semmelweis University, Budapest, Hungary
Introduction: Low standard of living plays a crucial role in P1828
poor morbidity and mortality rates of cerebrovascular
diseases. It is associated with a lower education level and The use of electronic databases for
health awareness of the population, and a looser relationship epidemiology of movement disorders
of people with the healthcare system. By investigating in the Siberian region of the Russian
stroke patients in two districts of Budapest with extremely Federation
different life circumstances, our team has demonstrated that
M. Nikitina1, I. Zhukova1, O. Izhboldina1, N. Zhukova1,
stroke occurs at a significantly earlier age (67.1514.77 vs.
V. Alifirova1, D. Gutorova2, O. Gileva3, M. Zhestikova4
74.1211.47 years) in a poorer (VIII.) district. In this study, 1Neurology and Neurosurgery, Siberian State Medical
we investigated the role of the quality of relationship
University, 2Neurology, Tomsk Regional Clinical Hospital,
between stroke center, specialist outpatient department, GPs Tomsk, 3Neurology, Neurosurgery and Medical Genetics,
and ambulance service. State Educational Institution of Higher Professional
Patients and method: Our anonymous database consisted Education, Kemerovo, 4Neurology, M.G. Zhesstate
of residents of the two districts, who suffered a stroke in Educational Institution of Postgraduate Professional
2007. Pre-stroke history, risk factors, treatment after the Education, Novokuznetsk, Russia
cerebrovascular accident and adherence to therapy were
also included. At the same time, data for 2010 were
evaluated. We investigated the rate of patients referred to P1829
hospital by GPs with a diagnosis of stroke/TIA. Acute poliomyelitis: a retrospective data
Result: The rate of patients with a diagnosis of cerebrovascular
disease referred to hospital by GPs or a specialist outpatient analysis
department was significantly higher in (the wealthier) L. Bertolasi1, L. Werhagen2
district XII with a more favourable stroke epidemiology 1F Neurological, Morphological and Motor Sciences,

(85.21 vs. 58.85%), whereas the ratio of referrals with University of Verona, Verona, Italy, 2Karolinska Institut at
completed stroke vs. TIA was significantly lower (1.20 vs. Danderyds Hospital, Division of Clinical Sciences,
2.96). Stockholm, Sweden
Conclusion: At a lower standard of living, the efficacy of
cerebrovascular prevention, access to acute stroke care and
patient follow-up is decreased. There is a looser relationship
P1830
between the patient and his/her GP. The Budapest Districts 8-12 project.
Follow-up of stroke patients in two
districts of the Hungarian capital. The role
of local environmental factors in stroke
epidemiology
K.A. Bres-Molnr1, A. Folyovich1, I. Vastagh2, A. Ajtay2,
A. Kri2, A. Majoros1, Z. Laki1, K. Erdei1, K.L. Kovcs2,
B. Gunda2, L. Lenti2, K. Vadasdi1, Z. Dnos1,
D. Bereczki2
1 Neurology and Stroke Center, Szent Jnos Hospital,
2 Neurology, Semmelweis University, Budapest, Hungary

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


400 Posters, Sunday 9 September

Neuroimaging/ -sonology P1832


Whole brain ADC histogram in monitoring
P1831 small vessel disease: major scanner
Fronto-temporo-insula grey matter effects in a large CADASIL cohort
alterations of first-episode, drug-nave B. Gunda1,2, R. Porcher3, E. Jouvent2, D. Herv2,
and very late-onset panic disorder M. Duering4, M. Dichgans4,5, H. Chabriat2
patients
1Neurology, Semmelweis University, Budapest, Hungary,
2Department of Neurology, Hopital Lariboisire, APHP,
C.-H. Lai1,2, Y.-T. Wu3 University Denis Diderot, 3Hopital St Louis APHP, Paris,
1Department of Psychiatry, 2Brain Image Research Unit, France, 4Institute for Stroke and Dementia Research,
Buddhist Tzu-Chi General Hospital, Taipei Branch, Taipei, Ludwig-Maximilians-University, 5German Center for
Taiwan, New Taipei City, 3Institute of Brain Science, Neurodegenerative Diseases, Munich, Germany
National Yang Ming University, Taipei, Taiwan R.O.C.
Objective: DTI derived histogram metrics - as global
Objective: There is no voxel-based morphometry study for measures of ultrastructural tissue integrity- have been
first-episode, drug-nave and very late-onset panic disorder widely reported to correlate significantly with clinical
patients. Besides, differences of onset age might represent parameters in various diffuse cerebral pathologies. The aim
different clinical subgroups. Therefore we designed this of our study was to evaluate whether histograms from the
study to investigate grey matter deficits in this subgroup of widely available DWI sequence with technical updates and
patients. without significant operator dependent post-processing, can
Method: 30 patients and 21 normal controls were enrolled be used similarly to DTI histograms in follow-up studies of
in our study. They all received 3 Tesla magnetic resonance CADASIL, a model of cerebral small vessel disease.
imaging acquisitions for the structural imaging of the brain. Methods: Clinical and MRI data from a large cohort of
All the structural images were processed and analyzed to CADASIL patients were evaluated at baseline and during 3
estimate the differences of grey matter volumes between years of follow-up. We compared DWI-derived apparent
patients and controls. We utilized optimized voxel-based diffusion coefficient (ADC) histogram parameters to those
morphometry function implemented in the FSL (FMRIB of the reference method (DTI Mean diffusivity (MD)) and
Software Library) with agoraphobia, global brain volume, evaluated the effect of cerebrospinal fluid (CSF) suppression
age, gender and duration of illness as covariates. We also and artefact removal on results. A mixed effects model was
performed the voxel-wise linear regression between clinical used to evaluate the random MRI scanner effect on the
rating scale scores and grey matter volumes of brain to parameters.
confirm results of optimized voxel-based morphometry and Results: We found excellent correlation between ADC and
significant region for physiopathology of very late-onset MD parameters especially for mean value, unchanged by
PD. CSF suppression. Correction of image artefacts did not alter
Results: First-episode, drug-nave and very late-onset panic ADC parameters significantly. In contrast, the magnitude of
disorder patients had lower grey matter volumes in the left the random scanner effect on ADC parameters was high and
orbitofrontal cortex, left inferior frontal cortex, left superior larger than that of clinical scores, sex and age.
temporal gyrus and right insula when they were compared Conclusion: DWI derived histogram parameters without
to controls (corrected p<0.005, multiple comparisons, much post-processing (CSF suppression, artefact removal)
cluster threshold: 30 voxels). A negative correlation appear promising for monitoring small vessel disease such
between PDSS and GMV was observed in the right insula as CADASIL. However, given the huge scanner effect on
using general linear model voxel-wise analysis with age and parameters, the use of different scanners including technical
gender corrected. updates may have major impact on the results and should be
Conclusion: Fronto-temporo-insula grey matter deficits evaluated in a multicentre longitudinal trial.
might represent the structural pathophysiology of first-
episode, drug-nave and very late-onset panic disorder.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 401

P1833 P1834
1H-MR spectroscopy only shows elevated 3T high b-value diffusion-weighted MR
water content in adult onset autosomal imaging in hyperacute ischemic stroke
dominant leukodystrophy (ADLD) with M. Lettau1, M. Laible2,3
autonomic symptoms 1Neuroradiology, 2Neurology, University of Freiburg Medical
School, Freiburg im Breisgau, 3Neurology, University of
J. Finnsson1, A. Melberg2, R. Raininko1
Heidelberg Medical School, Heidelberg, Germany
1Radiology, 2Neurology, Uppsala University, Uppsala,
Sweden Background and purpose: In patients with hyperacute
Introduction: Adult onset ADLD with autonomic ischemic stroke, early demonstration of infarction is
symptoms is a rare disease with a clinical course somewhat essential. Diffusion-weighted imaging (DWI) is the key
similar to chronic progressive MS but with very different method for detecting hyperacute infarction. The value of
imaging findings: extensive white matter changes in the high b-value DWI in hyperacute ischemic stroke is
cerebrum and cerebellar peduncles. Patients usually present controversial at 1.5T, and is unknown at 3T.
in the fourth to sixth decade with autonomic symptoms, The aim of this study is to explore the value of high b-value
later developing symptoms from the pyramidal tracts and versus standard b-value DWI at 3T in hyperacute stroke
ataxia. with quantitative and qualitative analysis.
Objective: Present MRS findings in this disease. Materials and methods: This study prospectively included
Patients and methods: 15 members from two non-related 104 consecutive patients with hyperacute stroke. At 3T,
families affected by the disease were studied with MR conventional MR sequences and DWI were performed. The
imaging and single voxel MRS and genetic tests. The MRS examination included a standard DWI (b =1,000s/mm2) and
results were compared to those of healthy controls. two high b-value DWI (b=3,000s/mm2 and b=5,000s/mm2).
Clinically, the family members ranged from asymptomatic Qualitative and quantitative analysis was performed.
to wheelchair-bound. Results: With increasing b-value, DW images appeared
Results: 14/15 family members had the genetic linkage to noisier. The number of detected lesions was significantly
the disease. 12 family members had extensive typical higher on b=3,000 images than on the other DW images and
pathological findings on MRI. The metabolite concentrations higher on b=5,000 images than on b=1,000 images. The
of tCr, tCho and tNAA measured in mM, using internal number of lesions >1cm was not significantly different.
water as a reference, were significantly lower in family Lesion conspicuity was higher, boundary better seen, lesion
members compared to controls and we found linear extent bigger, and estimation of final infarct size was better
correlations between all these metabolite levels. When tCr on high b-value than on standard b-value DWI. Contrast-to-
was used as a reference we found no difference between the noise-ratio (CNR) and signal-to-noise-ratio (SNR)
family members and the controls. No measurable lactate decreased and contrast ratio (CR) increased on high b-value
was detected. DWI compared to standard b-value DWI.
Conclusion: The decreased metabolite concentrations Conclusions: At 3T, high b-value DWI was superior to
measured using internal water as a reference are most likely standard b-value DWI in detection of hyperacute infarction
due to increased water content in the tissues, diluting all and prediction of final infarct size in spite of increasing
metabolites to a similar degree. This is also consistent with imaging artifacts.
the high signal intensity the patients exhibit in the white
matter on T2-weighted MR images and with the reported
histopathological findings of vacuolated myelin.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


402 Posters, Sunday 9 September

P1835 P1836
Gray matter deficits of major depressive Changes in regional cerebral blood flow
disorder: a meta-analysis of voxel-based during light intensity exercise of cycle
morphometry studies ergometery: a PET study
C.-H. Lai1,2 M. Hiura1, T. Nariai2, K. Ishii3, M. Sakata3, K. Oda3,
1Department of Psychiatry, 2Brain Image Research Unit, J. Toyohara3, K. Ishiwata3
Buddhist Tzu Chi General Hospital Taipei Branch, New 1Faculty of Sports and Health Studies, Hosei University,
Taipei City, Taiwan R.O.C. 2Department of Neurosurgery, Tokyo Medical and Dental
Objective: We designed this study to generate conclusive University, 3Tokyo Metropolitan Institute of Gerontology,
grey matter deficits in major depressive disorder (MDD) Tokyo, Japan
through the meta-analysis by signed differential mapping Background and aims: During exercise cerebral blood
(SDM) toolbox. flow (CBF) would be affected by changes in cardiovascular
Method: The Pubmed, ScienceDirect and Scopus databases system and metabolites from working muscles, however
were searched and only studies published or published few investigations have elucidated the changes in CBF
online before November 2010 would be enrolled. Twenty during dynamic exercise. The aim of the present study is to
voxel-based morphometry (VBM) studies of adult MDD investigate the changes in regional CBF (rCBF) during
patients were included for the meta-analysis by SDM cycling exercise and the differences among rCBF
toolbox with the threshold criteria as error probability less performing positron emission tomography (PET)
than 0.00005 and cluster more than 50 voxels. Onset age, Methods: 88 healthy male volunteers were recruited (age;
patient and control number, gender ratio of both groups, 231.9 yr, weight 671.9 kg). CBF was investigated by
ratio of medicated patients, depression rating scores, illness oxygen-15-labelled H2O PET at rest and during low
duration, co-morbidity and corrected p existence were also intensity cycling exercise by autoradiography method in
meta-regressed as covariates to exclude the confounding conjunction with pulmonary oxygen uptake (VO2), cardiac
biases. output (CO) and blood pressure. 57 regions of interest
Results: Voxel-wise meta-analytic results of these 20 VBM (ROI) were defined within the whole brain by circular
studies in MDD patients revealed that grey matter deficits shaped areas.
were observed in right anterior cingulate cortex [Talairach Results: During constant load cycling exercise, VO2 and
coordinates (6, 38, 14), p<0.00005, cluster voxels >50] and CO increased and had a plateau phase of 19.53.6ml/min/
left anterior cingulate cortex [Talairach coordinates (-2, 36, kg and 8.82.1l/min, respectively. Systolic blood pressure
14), p<0.00005, cluster voxels >50] when the patient pool changed from 11410 to 12916mmHg. During exercise
was compared to the control pool. The findings remained rCBF changed from 62.36.6 to 76.79.6ml/min/100g on
largely unchanged in jack-knife sensitivity analyses. Those average. There was a large increase of rCBF in vermis
potential confounding factors had little impact on the (40.2%) and paracentral lobules (35.5%). In brain stem
results. (31.7%), basal ganglia (31.3%) and insular cortex (26.5%),
Conclusion: The meta-analysis suggested the existence of rCBF also increased apparently. These changes were reset
grey matter deficits in bilateral anterior cingulate cortices of to the rest condition while exercise continued for 15minutes.
MDD patients. Conclusion: Changes in rCBF during exercise were
investigated quantitatively with PET. Exercise caused
fluctuation of rCBF in relation with regulation of the
cardiovascular system.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 403

P1837 P1838
Sleeping habits and brain structure: the Identification of symptomatic carotid
volume of the hippocampus is smaller in atheroma plaques. Utility of the grey scale
owls than larks median (GSM) system
D. Kuperczko1, Z. Clemens1, G. Perlaki2, G. Orsi2,3, G. Ruiz-Ares, B. Fuentes, P. Martnez-Snchez,
Z. Kalmar1, A. Schwarz4, N. Kovacs1, B. Faludi1, E. Dez-Tejedor
J. Janszky1 Stroke Unit, Neurology, University Hospital La Paz, Madrid,
Department of Neurology, University of Pecs, 2Pecs
1 Spain
Diagnostic Institute, 3Department of Biology, 4Department of Introduction: To date clinical and subjective
Neurosurgery, University of Pecs, Hungary ultrasonographical criteria are used to identify the
Introduction: Brain morphology is supposed to be related symptomatic plaques but the standardized gray scale
to several life style features. Here we investigated the median (GSM) value could be a useful objective tool for
relationship between volumes of specific brain structures this diagnosis. Our aim is to validate the GSM system as
and sleep duration as well as go-to-bed time which is tool to identify symptomatic carotid atheroma plaques.
considered as a fairly stable trait of the personality. Methods: Prospective study. Acute non-cardioembolic
Methods: 91 healthy young subjects were included in the anterior cerebral circulation ischemic stroke patients with
present study. We used semi-automatic user-independent plaques in internal carotid artery ipsilateral to symptoms
MR volumetry and voxel-based morphometry. We were included. Echogenicity of plaques was measured by a
investigated the relationship between go-to-bed time as well digital and standardized gray scale system in carotid
as sleep duration and volumes of brain structures known to ultrasound B-mode (longitudinal projection) conducted
be involved in sleep regulation (hippocampus, thalamus, within the first week after admission by only one operator.
orbitofrontal cortex and brainstem). Intra-observer variability was measured.
Results: We found significant correlation between go-to- Results: 52 patients were included. GSM system had better
bed time and hippocampal volume; later go-to-bed time was intra-observer variability than subjective criteria.
associated with smaller hippocampal volume. Other Symptomatic plaques showed less echogenicity than
comparisons, including those with sleep duration, did not asymptomatic ones (23 vs. 37; p<0.0001). A Receiver
reach significance. Operation Curve (ROC) analysis pointed to GSM value of
Conclusion: Our results demonstrate that owls (those 29 as the point associated to higher sensitivity (76%) and
who go to sleep late) are at increased risk for having smaller specificity (82%) to identify a plaque as symptomatic
hippocampi compared to larks (those who go to sleep (AUC=0.874; IC 0.779-0.969; p<0.001) which was
early) irrespective of their sleep duration. These results are associated with 13.93-fold increase (IC 3.66-53.01;
consistent with the notion of hippocampal shrinkage p<0.001) to be classified as symptomatic. A probability
associated with higher level of glucocorticoids due to the model to predict the identification of a symptomatic plaque
loss of slow wave sleep mainly present during the first half was defined combining male sex, previous angiotensin
of the night. converting enzyme inhibitors (ACEI) treatment and GSM
<29 (ABC=0.890; IC 0.797-0.984; p<0.001).
Conclusions: GSM system is a useful tool to identify
carotid symptomatic plaques with the 29 value as cut-off
point. The combination of male sex, previous treatment
with ACEI and GSM<29 predicts probability of
symptomatic plaque.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


404 Posters, Sunday 9 September

P1839 P1840
In vivo 1H-MRS and DWI for quantitative Non-invasive pH-weighted magnetic
differentiation of Parkinsons disease resonance imaging
(PD), multiple system atrophy (MSA), and Z. Dai1, G. Xiao1, Z. Shen1, L. Wang1, P.Z. Sun1,2,
progressive supranuclear palsy (PSP) R. Wu3
1Shantou University Medical College, Shantou, China,
Z. Rozhkova1, I. Karaban2, N. Karaban2 2Department of Radiology, Athinoula A. Martinos Center for
Radiology, Medical University Clinic BORIS,
1

Extrapyramidal Disorders, Institute of Gerontology, Kiev,


2 Biomedical Imaging, Massachusetts General Hospital and
Ukraine Harvard Medical School, Charlestown, MA, USA,
3Department of Medical Imaging, 2nd affiliated Hospital of
Purpose: We propose the relaxation times T2M of the main Shantou University Medical College, Shantou, China
cerebral metabolites, and the ADC coefficients in the middle
Introduction: Acid-base balance is the very important part
cerebral peduncles (MCP) for differentiating MSA from PD
of human homeostasis, which is regulated in a dynamic
and PSP.
steady state. Alteration in tissue pH can be an indicator of
Methods: Four groups of patients are studied with 1.5T
many diseases, such as stroke and tumour. Based on the
Signa Excite (GE). The PDG group includes 19 patients
theory of chemical exchange saturation transfer, we set up
with PD, the MSAG group includes 16 patients with MSA,
a new sequence that can realize pH imaging.
the PSPG group consists of 14 patients with PSP, and the
Methods: The new sequence with selective radiofrequency
CG group includes 15 healthy volunteers. For all patients
offset was used at Agilent 7T/160 animal scanner. The pulse
1H spectra are obtained in basal ganglia (BG) and in MCP.
pre-saturation offset was set at -1.87ppm and 1.87ppm. The
From the echo-time dependence of AM (where AM are the
duty cycle was maintained 50%. Other imaging parameters
peak areas of the signals from Cho, Cr and NAA) the T2M
were set as follows: TR=40ms, TE=1.5ms, slice
are calculated. ADC coefficients are determined from EPI.
thickness=2mm, single slice, field of view=6060mm,
Results: The mean T2M values (M = Cho, Cr, NAA) in BG
number of average=5 and acquisition bandwidth=50kHz.
are the following: in PDG (67.1, 40.5, 98.6ms), in MSAG
Total scan time was about 1min. The images were processed
(145.2, 59.3, 207.5ms), in PSPG (210.1, 185.3, 263.4ms),
using Matlab. Other routine MR images were acquired for
in CG (204.2, 198.0, 331.2ms). The mean T2M values in
reference. The pH phantoms were prepared with 3% agar-
MCP are the following: in PDG (63.3, 56.5, 90.2ms), in
gel and 50mmol/L creatine, in which pH value is the one
MSAG (58.2, 49.0, 101.8ms), in PSPG (134.1, 152.3,
and only difference.
233.1ms), in CG (215.0, 114.0, 320.1ms). From analysis of
Results: The intensity of signal is depending on the pH
DWI the ADC values in the BG are: (0.65x10-3), (0.54x10-3),
value, our results show that the lower the pH value is, the
(0.64x10-3), and (0.44x10-3), and in MCP: (0.82x10-3),
weaker the signal intensity is, which has statistical
(0.96x10-3), (0.79x10-3), and (0.81x10-3) in PDG, MSAG,
significance (p<0.05). In addition, the spatial resolution of
PSPG, CG, respectively.
pH imaging is excellent.
Conclusion: We detected in MCP significantly higher
Conclusion: Our pH phantoms proved that we can detect
ADC, and also lower T2M in patients with MSA in
pH value using our new sequence. This finding may provide
comparison with patients with PD and PSP are in vivo
a complement for conventional imaging, and has its
diagnostic markers of MSA.
potential advantages in detecting the acid-base metabolism.
Translated to clinic applications, this technology can be
used to acutely detect the penumbra of stroke at early stage.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 405

P1841 P1842
Botulinum toxin A related changes of Measurement of extracellular-pH based
cortical activity in patients suffering from iopamidol chemical exchange saturation
severe hand paralysis with arm spasticity transfer imaging at 7T
following ischemic stroke L. Wang1, Z. Shen1, Z. Dai1, G. Xiao2, Z. Sun3, R. Wu1
T. Veverka1, P. Hlutk1, Z. Tomov1, P. Hok1,
1Department of Medical Imaging, 2nd affiliated Hospital of
P. Otruba1, M. Krl1, Z. Tds2, J. Zapletalov3, Shantou University Medical College, Shantou, 2Department
R. Herzig1, A. Krobot4, P. Kaovsk1 of Math and Information Technology, Hanshan Normal
1Department of Neurology, 2Department of Radiology, University, Chaozhou, China, 3Martinos Center for
3Department of Biophysics, Biometry and Statistics, Biomedical Imaging, Department of Radiology,
4Department of Physiotherapy, Palack University and Massachusetts General Hospital and Harvard Medical
University Hospital, Olomouc, Czech Republic School, Charlestown, MA, USA
Aim: Investigations were performed to localize and analyze Introduction: pHe is an indicator of many pathological
the botulinum toxin (BoNT-A) related changes of cerebral processes at biosystem. pHe of abnormal tissue, such as
cortex activation in chronic stroke patients suffering from tumour and stroke, is usually lower compared with normal
severe hand paralysis with arm spasticity. tissue. Iopamidol is one of the most common extracellular
Methods: 14 patients (5 males, 9 females, mean age 55.3 contrasts possessing a high number of mobile protons (e.g.
years) suffering from upper limb post-stroke spasticity were amide), which are in exchange with water. In this work, we
investigated. The change of arm spasticity was assessed aim to detect diverse pHe depending iopamidol with a new
using the modified Ashworth scale (MAS). Functional MRI sequence based on the theory of CEST.
(fMRI) sessions were performed before (W0), four weeks Methods: A sample was made of four tubes, one containing
(W4) and 11 weeks (W11) after BoNT-A application. pure water with pH 7.0 and the others containing solutions of
Patients were scanned while performing imaginary iopamidol at the same concentration of 10mM with pH: 5.6,
movement with the impaired hand. 7.3 and 7.6, respectively. All the experiments were performed
Results: BoNT-A treatment was effective in alleviation of on Agilent 7T/160 scanner. The pulse pre-saturation offset of
arm spasticity. Task-related fMRI prior to the treatment MT-GRE sequence was set at -4.3ppm (voff) or 4.3ppm (von)
showed extensive activation of bilateral frontoparietal with respect to the resonance of water protons. Typical
sensorimotor cortical areas, anterior cingulate gyrus, acquisition parameters included bandwidth=50kHz, Flip
pallidum, thalamus and cerebellum. Effective BoNT-A angle=120 deg, FOV=52x52mm2, TR=42ms, TE=2ms, single
treatment (W4) resulted in partial reduction of active slice, averages=10, matrix=128x128. A pH-weighted imaging
network volume in most of the observed areas, whereas was obtained by subtracting the von image from the
BoNT-free data (W11) revealed further volume reduction in corresponding voff one. The z-spectrum was also acquired
the sensorimotor network. On direct comparison, significant with pulsed labelling RF schemes.
activation decreases associated with BoNT-A treatment Results: The signal intensity of each tube containing
were located in areas outside the classical sensorimotor iopamidol depends on the pH value, and the imaging SNR
system, namely, ipsilesional lateral occipital cortex, is well-pleasing. The higher the signal intensity is related
supramarginal gyrus and precuneus cortex. In comparison with the lower pH value (p<0.05, r>0.7).
to W4 and W11, no activation increases were found, instead, Conclusions: Our experiments proved that it is possible to
activation further decreased in ipsilesional insular cortex, detect pHe of abnormal tissue, based iopamidol CEST
contralesional superior frontal gyrus and bilateral frontal imaging with MT-GRE sequence. This finding may help the
pole. non-invasive assessment of the extent and progression of
Conclusions: Whole brain activation patterns during diseases for which pH modifications act as biomarker, as
treatment of post-stroke arm spasticity and further follow well as provide complementary information to conventional
up document predominantly gradual changes both within imaging.
and outside the classical sensorimotor system.
Supported by MHCR grant NS9920-4/2008.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


406 Posters, Sunday 9 September

P1843 P1844
The role of functional MRI in diagnosing Oral somatosensory association with
severe chronic disorders of visual cortex
consciousness N. Narita1, K. Kamiya2
1Neurological Dentistry, Nihon University School of
S.M. Golaszewski1,2,3, M.Seidl1,3,4,
A.B. Kunz1,4,
M. Kronbichler2,5, J. Crone2,5, R. Nardone1,3,6, Dentistry at Matsudo, Mastudo, 2Removable Posthodontics,
E. Trinka1,2,3, F. Gerstenbrand4,7 Nihon University School of Dentistry at Matsudo, Japan
1Department of Neurology, 2Neuroscience Institute, 3Spinal Introduction: Braille reading by blind individuals has been
Cord Injury and Tissue Regeneration Center, Paracelsus reported to cause visual cortex activation (Fujii T, et al.
Medical University Salzburg, 4Karl Landsteiner Institute of Neurosci Res. 2009, 65(2):175-86), while sighted
Neurorehabilitation and Space Neurology, Vienna, 5Institute individuals require training to activate the visual cortex
of Psychology, Paris Lodron University, Salzburg, Austria, (BMC Neuroscience 2006, 7: 79). When considering the
6Department of Neurology, F. Tappeiner Hospital, Merano,
oral region, even though it is naturally impossible to see
Italy, 7Department of Neurology, Medical University inside the oral cavity by oneself or the food bolus while
Innsbruck, Austria chewing, proper chewing is possible without any injury to
Objective: Accurate diagnosis of severe chronic disorders the tongue and cheek. In this study, we attempted to clarify
of consciousness (DOC) after TBI is essential for clinical the specificity between oral tactile discrimination and visual
and rehabilitative care and decision-making. cortex activity.
Neurobehavioral tests, which rely on the patients Methods: 6 healthy subjects participated in this study. For
intellectual and motor ability to communicate, are the most shape discrimination, we used 6 differently shaped test
widely used diagnostic tools, since their advantage over pieces, and the time duration of each discrimination trial in
clinical assessment has been validated. However, with the the mouth was 10 seconds, with 40-second rest intervals.
emergence of modern neuroimaging methods, especially F-NIRS was used to measure occipital cortex activity.
functional MRI, objective physiological markers for Results: Shape discrimination by the mouth activated the
assessing the state of consciousness are available in primary visual cortex, association visual cortex, and
specialized clinics. They are, however not fully integrated somatosensory association cortex. Furthermore, visual
in clinical routine, because their benefit has yet to be cortex activity in incorrect answer was significantly
determined. increased as compared with that in correct answer.
Material and methods: 15 patients in apallic syndrome Conclusion: Primary and association visual cortices are
(AS) and 5 patients in minimally conscious state (MCS) especially involved in shape discrimination in the mouth. In
after TBI and other aetiologies were examined with addition, visual cortex activity during shape discrimination
somatosensory, auditory and event related paradigms in is enhanced during the incorrect task performance, which
fMRI and evoked potentials (EP). The findings were may conceptually coincide with the previous report
compared to the neurobehavioural diagnosis and it was Practice makes perfect: the neural substrates of tactile
analyzed, if additional information from fMRI and EP discrimination.
confirmed or questioned the diagnosis.
Results: 3 out of 15 patients in AS showed fMRI activation
in event related paradigms, suggesting that patients are in
MCS or in beginning remission.
Conclusion: Uncertainty in diagnosis still exists even with
well-established diagnostic assessment scales. As long as
internationally accepted guidelines for assessing patients
with chronic DOC do not exist, every single diagnostic
modality available in each clinical setting should be
performed, to minimize diagnostic error and to find ways,
in terms of perceptive channels, to approach the patients.
FMRI has the potential to bring diagnostics in chronic DOC
forward to the next level.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 407

P1845 P1846
Structural and metabolic correlates of Computer assisted seizure onset zone
neuropsychological dysfunction in localization based on ictal EEG
multiple system atrophy and Parkinsons G. Gritsch1, M. Hartmann1, H. Perko1, F. Frba1,
disease C. Baumgartner2, T. Kluge1
1Department Safety & Security/AIT Austrian Institute of
C. Kobylecki1,2, K. McDonald2,3, J. Thompson2,3,
Technology, 22nd Neurological Department, Hospital Hietzing
J. Anton1, R. Hinz1, A. Gerhard1,2
1Wolfson Molecular Imaging Centre, University of with Neurological Center Rosenhgel, Vienna, Austria
Manchester, Manchester, 2Greater Manchester Neurosciences Introduction: For medical refractory epilepsy patients,
Centre, Salford, 3University of Manchester, UK neurosurgery is often the last chance to become seizure free.
Introduction: Cognitive impairment is known to occur in In order to find the seizure onset zone (SOZ), long-term
patients with multiple system atrophy as well as Parkinsons EEG recordings are performed. Commercial source
disease (PD). However, differences in the cognitive profile localization software can be used to identify the SOZ based
and its underlying substrates between the two conditions are on the recorded EEG. The disadvantage of such tools is that
not clearly understood. We hypothesise that functional and they work on interictal spikes which may not stem from the
structural changes in fronto-subcortical structures underlie SOZ and need a lot of user interaction. Our proposed system
differences in neuropsychological performance in these works on rhythmic ictal EEG without manual interaction of
parkinsonian syndromes. the user.
Methods: Age-matched patients with idiopathic PD (n=5) Methods: The proposed system combines an automatic
and multiple system atrophy-parkinsonism (MSA-P; n=4) seizure detection system (EpiScan) and a source localization
underwent detailed neuropsychological and clinical module. EpiScan measures the strength of rhythmic activity
evaluation. In addition, volumetric T1-weighted MRI and within the EEG signal. If the rhythmicity measure rises
[18F]-fluorodeoxyglucose (FDG) positron emission above a certain threshold, an alarm is reported. For these
tomography (PET) in the resting state were performed. time instances the rhythmic part of the signal is fed into the
Voxel-based analysis of both grey matter volume and FDG- source localization module. The source localization is
PET data was performed using SPM8. performed with an Eigenspace beamformer using a standard
Results: No significant differences in MMSE or Montreal MRI based BEM head model.
Cognitive Assessment (MoCA) scores were found between Results: 4 patients having 19 seizures were investigated.
MSA-P and PD patients. There were no significant EpiScan detected all seizures at a false alarm rate of 0.26
differences in verbal learning or verbal fluency test scores. per hour. In most cases our localization approach was able
Patients with MSA-P had significantly poorer performance to reproduce clinical findings very well. Only where very
on Stroop word (p<0.001) and colour naming (p<0.05), and large artifacts are present, a loss of spatial concentration of
on the Trails B test (p<0.05), but not in the Stroop the SOZ can be observed.
interference condition. In MSA-P compared to PD, grey Conclusion: Our proposed system is capable of
matter atrophy and hypometabolism were identified in the automatically detecting SOZs based on rhythmic ictal EEG.
right anterior cingulate cortex, with additional atrophy in In contrast to other commercial systems no user interaction
the left putamen. is necessary, thus making it a valuable approach for clinical
Conclusion: Our preliminary results indicate that structural practice.
and metabolic changes in frontal areas, such as the anterior
cingulate cortex, are likely to contribute to poorer executive
function in patients with MSA-P compared to PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


408 Posters, Sunday 9 September

P1847 P1848
Is quantitative assessment of cervical Modified CCA+jICA method for
nerve hypertrophy by MRI effective for the neuroimaging data fusion
diagnosis of chronic inflammatory V. Youssofzadeh1, I. Faye1, A.S. Malik1, J.M. Abdullah2,
demyelinating polyradiculoneuropathy? F. Reza2, N. Kamel1
1Electrical and Electronics Engineering, Universiti Teknologi
A single-institution, retrospective
Petronas, Teronoh, 2Neuroscience, Hospital Universiti Sains
case-control study Malaysia, Kota Bharu, Malaysia
K. Tanaka1, N. Mori2, Y. Yokota2, T. Okunomiya1, Introduction: Multi-modal data fusion may provide
D. Kambe1, A. Shinde1, T. Kageyama1, T. Suenaga1 improved analysis compared to that with single modality. In
1Department of Neurology, 2Department of Radiology, Tenri
this paper, EEG and fMRI data are fused to provide better
Hospital, Tenri, Japan
results using data fusion in order to identify functional and
Introduction: Spinal nerve hypertrophy is often seen in dysfunctional activities in the human brain.
patients with chronic inflammatory demyelinating Method: We proposed modification to CCA+jICA
polyradiculoneuropathy (CIDP). The purpose of this study (Canonical Correlation Analysis+ Joint Independent
was to evaluate the effectiveness of quantitative assessment Component Analysis) method by incorporating Full Blind
of cervical nerve hypertrophy by MRI for the diagnosis of Source Separation (FBSS). CCA maximizes inter-subject
CIDP. co-variations across modalities at feature level then JICA
Methods: We retrospectively enrolled 15 consecutive maximizes independency of canonical variants through
patients who had been diagnosed with definite CIDP FBSS, which minimizes entropy of separated sources.
according to the EFNS/PNS diagnostic criteria. 30 controls Results: Using spatiotemporal simulated data, the proposed
matched for sex, age, and MRI systems were also included. scheme is compared with other ICA-based data fusion
A radiologist reviewed the STIR images of cervical MRI models. Table 1 shows the best correlation results for
without prior knowledge of the diagnoses. The diameters of CCA+jICA(FBSS) method. The proposed scheme is also
the C5-C8 cervical nerves were measured. The mean signal tested utilizing concurrent EEG-fMRI data for Auditory
intensities in the nerves and the sternocleidomastoid muscle, Oddball task from 23 healthy subjects. ERP showed
as well as background noise, were measured to calculate the prominent positive (300ms) and negative peaks (100 and
contrast-to-noise ratio (CNR) in each nerve. Receiver 200ms) while fMRI images disclosed higher activity in
operating characteristic analysis was used to determine the bilateral parietal and posterior temporal and lower activity
cut-off values of diameter and CNR means, and the in mid-frontal areas. The obtained results cannot be seen in
diagnostic performance of the assessment procedure was separate analyses of EEG/fMRI.
evaluated. Another radiologist reviewed the MR images in [AVERAGE CORRELATION ANALYSIS]
the same way. The weighed kappa coefficient was calculated Average of Joint ICA CCA + JICA CCA + JICA CCA + JICA CCA + JICA CCA + JICA
to evaluate the interobserver agreement. Correlation
Coefficients
(Infomax)
0.72210.84
(Infomax)
0.88270.42
(EBM)
0.78471.12
(WASOBI) (NC-FastICA)
0.85830.24 0.91170.52
(FBSS)
0.94310.425
Results: The cut-off values of diameter and CNR means Conclusion: The outcomes can be linked to sensory,
were 5.3mm and 38.7, respectively. When the diameter or learning process and expectation based on activations
CNR means exceeded the cut-off values, the sensitivity and produced by CCA+jICA(FBSS). Applying FBSS in
specificity were 53.3% and 93.1%, respectively. The CCA+jICA model demonstrates the possibilities for
weighed kappa coefficient was 0.62, indicating substantial exploring associations in multimodal data and can be used
interobserver agreement. for multi-task applications with improved accuracy.
Conclusion: Quantitative assessment of the cervical nerve
hypertrophy by measuring nerve diameters and CNRs on
the STIR image of MRI was effective for the diagnosis of
CIDP. Moreover, the reproducibility of this assessment
procedure was acceptable.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 409

P1849 P1850
Diffusion measured white matter SPG3A-linked hereditary spastic
disintegration in cluster headache paraplegia associated with cerebral
N. Szab, T.Z. Kincses, E. Tth, G. Csete, B. Tuka, glucose hypometabolism
. Prdutz, L. Vcsei T. Terada1,2, S. Kono1, Y. Ouchi2, K. Yoshida3,
Department of Neurology, Albert Szent-Gyrgyi Clinical Y. Hamaya2, S. Kanaoka2, K. Shirakawa1, T. Konishi1,
Center, University of Szeged, Hungary M. Sakao1, H. Miyajima1
Objectives: In our former study we showed altered 1First Department of Medicine, Hamamatsu University

microstructure of the white matter in migraineurs. It is not School of Medicine, 2Molecular Imaging Frontier Center,
known yet if these changes of MRI diffusion parameters University School of Medicine, 3Department of Molecular
were specific for migraine, or related to the repeated pain Diagnosis, Hamamatsu University School of Medicine,
attacks. Hamamatsu, Japan
Methods: 11 patients with cluster headache and 11 age- SPG3A-linked hereditary spastic paraplegia is an autosomal
matched healthy control subjects were recruited. Diffusion dominant hereditary spastic paraplegia (HSP) caused by a
weighted MRI images with 60 diffusion directions were mutation in the SPG3A gene, and is characterized by
acquired on a 1.5 T GE Signa Excite scanner. Tract based progressive weakness and spasticity in the lower limbs
spatial statistics were used to detect the integrity of white without any neurological abnormalities. We herein report
matter tracts in a skeleton representing the core of the fibre two middle-aged Japanese sisters who were heterozygous
bundles. for an R239C mutation in the SPG3A gene. They both had
Results: Reduced fractional anisotropy was found in a right experienced onset of the disease in childhood, and have a
corticospinal tract, in the corpus callosum and in the anterior pure phenotype of HSP. To investigate cerebral function in
thalamic radiation bilaterally. The axial diffusivity was these patients, [18F]-fluorodeoxyglucose (FDG) positron
bilaterally increased in the anterior thalamic radiation, in emission photography (PET) with three-dimensional
the anterior corona radiate and in the inferior part of the stereotastic surface projection analysis was used for
fronto-occipital fasciculus. The mean and perpendicular measurement of the cerebral metabolism of glucose
diffusivity was increased almost over the whole brain with (CMRGlc). The PET analysis revealed decreased CMRGlc
left sided dominance. in the frontal cortex, and neuropsychological assessments
Discussion: Since the above described white matter showed low scores in the Frontal Assessment Battery. HSP
microstructural alterations, however more extensive, are caused by an R239C mutation has been reported to present
similar to those found in migraine, this points to the a pure phenotype confined to impairment of the corticospinal
assumption that these findings might reflect pain related tract. The decreased CMRGlc in the frontal cortex
alterations that are not specific to either of the diseases. associated with cognitive impairment in the frontal lobe in
these patients may indicate a more widespread
neuropathology from mutations in the SPG3A gene than
previously assumed.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


410 Posters, Sunday 9 September

P1851 P1852
Magnetic resonance imaging is related to Cerebral re-organisation following
the release of neurobiochemical markers traumatic spinal cord injury
of brain damage in chronic cerebral L. Sabre1, T. Tomberg1,2, J. Krv1, J. Kepler3, K. Kepler4,
ischemia . Linnamgi1, T. Asser1
1Department of Neurology and Neurosurgery, University of
Y. Morozova
Russian State Medical University, Moscow, Russia Tartu, 2Radiology Clinic, Tartu University Hospital, Tartu,
3Prnu Hospital, Prnu, 4Institute of Physics, University of
In recent years neurobiochemical markers of brain damage Tartu, Estonia
gained particular attention.
Introduction: The aim of the study was to examine cortical
Aim of study: Correlation between MRI, protein S100 and
re-organisation after traumatic spinal cord injury (TSCI)
TNF- releasing during chronic cerebral ischemia (CCI).
using functional magnetic resonance imaging (fMRI).
108 patients with CCI aged 20 - 60 years. Control group -
Patients and methods: We studied 6 right-handed
20 healthy persons.
tetraplegic TSCI patients at 1, 3 and 12 months after the
All patients were divided in 3 groups:
injury and 12 age- and gender-matched healthy controls at
1 - initial cerebral insufficiency (ICI) - 36.1%;
a single point of time. Individuals performed simple test-
2 - 31.3% - dyscirculatory encephalopathy (DE) of grade 1 and
rest cycles of flexion/extension of the right hand fingers and
3 - 32.2% DE of grade 2. In group 1 MRI was normal in the
flexion/extension of the right ankle during fMRI. The
majority. In group 2 - light and moderate widening of
activation volumes, maximum t-values and centres of
subarachnoid spaces (SS) of convexital surface - 76%;
gravity (COG) were calculated.
moderate widening of SS - 18%. In group 3 -widening of SS
Results: During hand movements the volume of activation
and LV with focal changes of ischemic origin different aged
in the contralateral primary motor cortex (Brodmann area
period (DAP) - 88.6%; widening of LV, multiple focal
(BA) 4) was non-significantly larger in TSCI patients
changes in paraventricular areas DAP of both hemispheres
(3,991.4mm3 vs. 2,433mm3, p=0.11). During the ankle
- 8.6%. In pts with ICI, TNF- was 0.6480.003pg/l, protein
movement their BA4 volume of activation was significantly
S100 - 0.6940.001 unit of optical density (UOD); in
increased (p=0.04). TSCI patients had an increased
patients with DE of grade 1 TNF- was 0.7050.001pg/l
recruitment of associated motor cortex (BA6) during both
and protein S100 0.7400.002 UOD; in patients with DE of
tasks (p=0.01). There was no recovery of neurologic
grade 2 TNF- was 1.2200.0004pg/l and protein S 100-
function in 3 patients and the remaining recovered
0.7640.001 UOD (p<0.001). TNF- in control group
according to American Spinal Injury Association (ASIA)
0.0520.002 pg/l, protein S100- 0.540.002 (UOD),
Impairment Scale. The cortical activations were more
(p<0.001). Changes in MRI correlate with grade of CCI and
increased in the patients who recovered. The mean COGs
TNF- and protein S100 releasing. These data support the
for BA4 during hand movement in TSCI patients showed a
correlation of TNF- and protein S100 with apoptotic or
non-significant shift laterally and anteriorly. During ankle
necrotic neuronal death.
movement there was a statistically significant shift in
activation medially.
Conclusion: Our study found broadening of cortical
activation during the first year after TSCI. To our knowledge
this is the first study to show the dynamic changes of
activation in the acute phase of TSCI.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 411

P1853 P1854
MRI findings in Susacs syndrome: report Posterior reversible encephalopathy
of three cases and review of the literature syndrome (PRES): description of 25 cases
M. Messelmani, H. Khaled, I. Bedoui, M. Mansour, L.C.P. Marrone1, A.C.H. Marrone1, G. Gadonski2,
J. Zaouali, R. Mrissa L.P. Diogo2, C.E. Poli-de-Figueiredo2, J.C. da Costa2
Neurology, Military Hospital, Tunis, Tunisia 1Neurology, Pontificia Universidade Catlica do Rio Grande

Introduction: Susacs syndrome (SS) is a rare disease of do Sul, Porto Alegre, 2Neurology, Pontificia Universidade
unknown pathogenesis. It is caused by a microangiopathy Catlica do Rio Grande do Sul, Brazil
affecting the arterioles of the brain, retina, and cochlea, Posterior reversible encephalopathy syndrome (PRES) is a
giving the classic clinical triad of subacute encephalopathy, clinicoradiologic entity characterized by headaches, altered
visual loss secondary to retinal branch occlusions, and mental status, seizures, and visual loss and is associated
sensorineural hearing loss. with white matter vasogenic oedema predominantly
Results: We report 3 cases of SS. They were all female. affecting the occipital and parietal lobes of the brain. The
Mean age at onset was 40.3 years. Clinical symptoms at cause of PRES is not yet understood. Numerous factors can
onset were neurological in 2 cases and auditory in one case. trigger the syndrome, most commonly: acute elevation of
Neurologic symptoms included headache, memory loss, blood pressure, abnormal renal function and
depression, behavioural disturbance, paraesthesia and immunosuppressive therapy. Other possible aetiologies are
difficulty in walking. Brain MRI showed T2 lesions in the eclampsia, transplantation, neoplasia and chemotherapy
white matter and corpus callosum in the 3 cases. No treatment. The most characteristic imaging pattern in PRES
enhancement after the administration of gadolinium was is the presence of oedema involving the white matter of the
observed. CSF was abnormal in 2 patients showing an posterior portions of both cerebral hemispheres, especially
elevated protein level. They were treated with the parieto-occipital regions, in a relatively symmetric
corticosteroids. pattern that spares the calcarine and paramedian parts of the
Conclusion: SS has been considered rare, but it is probably occipital lobes. We performed a review of clinical data,
more common than previously thought. Due to its neuroimaging and blood basic test of 25 patients with
polymorphism, SS is difficult to diagnose when the clinical PRES, who were followed in Hospital So Lucas-PUCRS
triad is lacking. The MR scans in SS show a rather in the period from March/2007 to September/2011. Our
distinctive pattern of supratentorial white matter lesions that clinical sample consisted of 25 patients (4 men and 21
always involve the corpus callosum. There is often deep women) with mean age 27.84 years (range from 2 to 74).
grey matter, posterior fossa involvement, and frequent The most common causes of PRES reported in this series
parenchymal with occasional leptomeningeal enhancement. were associated with pregnancies in 11 cases (44%),
The central callosal lesions differ from those in followed by lupus in 4 cases (16%). The symptoms more
demyelinating diseases, and should support the diagnosis of commonly reported were headache in 18 patients (72%),
SS in patients with at least two of the three features of the visual disturbance in 17 patients (68%) and seizure in 13
clinical triad. patients (52%). The occipital lobe was the most common
topography of the magnetic resonance abnormality and
occurred in 23 cases (92%), followed by parietal lobe in 15
cases (60%).

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


412 Posters, Sunday 9 September

P1855 P1856
Bi-exponential diffusion signal decay in Detection of CEST MRI using
normal-appearing white matter of multiple magnetization transfer pre-saturation
sclerosis sequence at 1.5 T scan
S. Nagy1, P. Bogner1,2, M. Aradi3, G. Orsi4,5,6, G. Xiao1, Z. Dai2, P.Z. Sun3, R. Wu4
G. Perlaki1,4,5, H. Komaromy1, A. Schwarcz5,7, 1Department of Math and Information Technology, Hanshan

J. Janszky4,5 Normal University, Chaozhou, 2Medical College, Shantou


1Diagnostic Centre of Pcs, 2Department of Radiography, University, Shantou, China, 3Athinoula A. Martinos Center
University of Pcs, Kaposvr, 3Department of Radiology, for Biomedical Imaging, Department of Radiology,
County Hospital of Zala, Zalaegerszeg, 4Department of Massachusetts General Hospital and Harvard Medical
Neurology, University of Pcs, 5MTA-PTE Clinical School, Charlestown, MA, USA, 4Department of Radiology,
Neuroscience MR Research Group, 6Department of Biology, 2nd affiliated Hospital of Shantou University Medical
7Department of Neurosurgery, University of Pcs, Hungary College, Shantou, China
Aims: To characterize bi-exponential diffusion signal Introduction: Solution pH was measured using water
changes associated with reduced white matter myelination proton NMR via chemical exchange dependent saturation
in multiple sclerosis (MS). transfer (CEST) with selected chemical exchange sites. In
Methods: Apparent diffusion coefficients (ADC) were this work, Magnetization Transfer pre-saturation sequence
generated using mono-exponential (0-1,000s/mm2) and was used to realize the CEST imaging.
bi-exponential (0-5,000s/mm2) approaches from 14 patients Methods: The Magnetization Transfer sequence with
with relapsing-remitting type of MS and 14 age- and sex- selective radiofrequency was used on the GE Signa HDX
matched controls after acquiring diffusion-weighted images 1.5T Scanner. The pH samples were prepared with 3% agar-
on a 3-T MRI system. Bilateral region of interest (ROI) gel and 50mmol/L creatine, in which pH value is only
measurements were performed in normal-appearing white difference. Magnetization Transfer pre-saturation pulse was
matter (NAWM) structures. The results were analyzed using set at -121Hz and 121Hz. We used gradient echo to read the
parametric or non-parametric tests and multiple linear MRI. Other imaging parameters were set as follows:
regression models. TR=40ms, TE=4ms, Slice thickness=5mm, single slice,
Results: Mono-exponential ADC increases in controls but field of view=1616mm, NEX=30 and acquisition
decreases in MS as a function of age; nonetheless an bandwidth=31.25KHz. Total scan time was about 7.5min.
elevated ADC is observed with increasing lesion number in The images were processed using Matlab.
patients. Bi-exponential analysis shows that the increased Results: The signal intensity of CEST MRI depended on
ADC is the result of decreased relative volume fraction of the pH value. Our result shows that the lower the pH value
slow diffusing component (fs). However the fast and slow is, the weaker the signal intensity is. In addition, the
diffusion components (ADCf, ADCs) do not change as a diversity of the CEST MRI is approximately at 3%.
function of either age in controls or lesion number and age Conclusion: Our pH samples proved that we can detect pH
in MS patients. value using Magnetization Transfer pre-saturation sequence.
Conclusions: Myelin content of the white matter may affect This finding has its potential advantages in detecting acid-
water diffusion in multiple sclerosis patients. It is suggested based metabolisms.
that these changes are probably due to the reduction of
myelin water fraction.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 413

P1857 P1858
N4U - neuGRID for Users: a web-based MRI in chronic cerebral ischemia (CCI)
gateway to neuroimaging research L. Belova1, Y. Nikitin2, V. Mashin1, N. Belova1,
G. Spulber1,
A. Redolfi2,
L.-O. Wahlund1,
F. Barkhof3, V. Abramova1
R. McClatchey4, C. Finochiaro5, J.-F. Mangin6, Medical Faculty, Ulyanovsk State University, Ulyanovsk,
1

P. Giannakopoulos7, J. Montagnan8, A. Evans9, A. Toga10, Scientific Center for Neurology under Russian Academy of
2

G. Frisoni2 Medical Science (RAMS), Moscow, Russia


1Karolinska Institutet, Stockholm, Sweden, 2IRCCS, Prevention, early diagnosis and treatment of cerebrovascular
Fatebenefratelly, Brescia, Italy, 3VU University Medical diseases, including chronic forms, are an actual problem of
Centre, Amsterdam, The Netherlands, 4University of West of modern neurology. 209 CCI I-III patients divided in two
England, Bristol, UK, 5CFc Consulting, Milan, Italy, groups: 93 with constitutional phlebopathy (CPP) and 116
6Commissariat lEnergie Atomique et aux Energies
without it (NP). Control group - 30 persons. MRI machine
Alternatives, Gif-sur-Yvette Cedex, France, 7Hpitaux Siemens (1.5 tesla) was used. With MR angiography sinus
Universitaires de Genve, Switzerland, 8CNRS, Orsay cedex, sagittalis superior, sinus rectus, sinus transversus cerebri,
France, 9McGill University, Montral, QC, Canada, venae superficiales cerebri, vena cerebri magna and venae
10University of California, Los Angeles, CA, USA
jugulares internae were estimated. In the NP group with
The unprecedented growth, availability and accessibility of CCI progression the following was defined: the increase in
sophisticated image analysis algorithms and powerful linear size and body index of ventriculus lateralis cerebri,
computational resources led to the idea of developing web- increase of patient number with focal brain changes and
based computational infrastructures that could meet users leukoaraiosis. In the CPP group the change of intensity was
new requirements. On the other hand the gap between the the same in different CCI stages (p>0.05). Venae
pace of data generation and the capability to extract superficiales cerebri in patients with CPP were wider, than
clinically or scientifically relevant information is rapidly in the control group and NP in all stages; veins widening
widening. Integration of the power of sophisticated increased in CCI II-III in comparison with CCI I. In CPP
mathematical models, efficient computational algorithms with CCI III sinus rectus and vena magna cerebri sizes
and advanced hardware infrastructure provides the increased. Right and left sinus transversus hypoplasty is
necessary sensitivity to detect, extract and analyze subtle, found in 35 CPP and in 30 NP (p>0.05) patients. One-sided
dynamic and distributed patterns distinguishing one brain sinus transversus thrombosis in 7 CPP patients and 2 NP
from another, and a diseased brain from a normal brain. (p>0.05). The MRI data proved the presence of intracranial
neuGRID is the leading e-Infrastructure where passive venous congestion in CPP patients: the size increase
neuroscientists can find core services and resources for of venae superficiales cerebri, sinus rectus and vena cerebri
brain image analysis. The neuGRID platform makes use of magna. Intracranial sinus thrombosis was found with the
grid services and computing, and was developed with the same rate in CPP and NP patients. Vein tonus and
final aim of overcoming the hurdles that the average functionality could be a reason for intracranial venous
scientist meets when trying to set up advanced experiments discirculation in CCI.
in computational neuroimaging, thereby empowering a
larger base of scientists. Although originally built for
neuroscientists working in the field of AD, the infrastructure
is designed to be expandable to services from other medical
fields (e.g. multiple sclerosis, psychiatric conditions).
neuGRID for Users (N4U) will provide an e-Science
environment by further developing and deploying the
neuGRID infrastructure to deliver a virtual laboratory
offering neuroscientists access to a wide range of datasets
and algorithm pipelines, access to computational resources,
services, and support. Information from this abstract is
intended to make aware researchers working with
neuroimaging of all possibilities when it comes to resources.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


414 Posters, Sunday 9 September

P1859 P1860
Cerebellar and brainstem congenital Magneto-encephalographic spike
defects in Tunisian children identification in insular epilepsy
I. Marouani1, I. Kraoua1, H. Benrhouma1, A. Rouissi1, H.M. Park1, N. Nakasato2
I. Turki1, E.M. Valente2, S. Nagi3, N. Gouider-Khouja1 1Neurology, Gachon University Gil Hospital, Inchon,
1Department of Child and Adolescent Neurology, National Republic of Korea, 2Epileptology, Tohoku University
Institute of Neurology, Tunis, Tunisia, 2CSS-Mendel Institute, Graduate School of Medicine, Sendai, Japan
Rome, Italy, 3Department of Neuroradiology, National
Institute of Neurology, Tunis, Tunisia
Introduction: Cerebellar and brainstem congenital defects
P1861
(CBCD) refer to any structural abnormality of the Presynaptic dopamine transporter SPET
cerebellum or the brainstem, present at birth, and due to scanning for the differential diagnosis of
interrupted or modified development of these structures parkinsonism at the Tartu University
during intra-uterine life, whatever the aetiology. Brain MRI
Hospital
allows accurate description of CBCD and orientation of
genetic diagnosis. H. Lindme1, K. Ulst2, P. Taba3
Objective: To describe the type of CBCD observed in
1University of Tartu, 2Department of Radiology, 3Department
Tunisian children and their clinical and radiological of Neurology and Neurosurgery, University of Tartu, Estonia
features.
Patients and methods: A prospective study (2005-2012) P1862
was conducted in 43 patients with CBCD. Malformations
were classified using a novel classification (Valente et al) CT interpretation by ASPECTS in
based on the determination of the primary cerebellar hyperacute ischemic stroke predicting
diagnosis, then the associated infra- and supratentorial functional outcome
abnormalities.
S. Tiamkao1, B. Sangpetngam2, W. Phuttharak2,
Results: 43 patients (28 males and 15 females) had CBCD.
Northeastern Stroke Research Group
The mean age of patients was 7.1 years. The most frequent 1Division of Neurology, Department of Medicine, 2Radiology,
malformations were molar tooth sign (32.6%), vermian
Faculty of Medicine, Khon Kaen University, Khon Kaen,
hypoplasia (30.2%) and Dandy Walker malformation
Thailand
(14%). Infratentorial and supratentorial abnormalities were
associated in 32.6% and 55.8% of cases, respectively. The
most frequent infratentorial abnormality was cerebellar P1863
hemisphere atrophy (50%) and the most frequent
supratentorial abnormality involved the commissures
Decreased cerebral cortical NAA/Cr
(70.8%). The most frequent clinical presentations observed correlated to prolonged periodicity of
in our patients were psychomotor delay (86%), gait PSD in a patient with Creutzfeldt-Jakob
disturbance (62.7%), visual abnormalities (62.7%) and disease: findings of 1H-MRS and EEG
mental retardation (53.4%).
K. Isonishi1, F. Moriwaka2, T. Kaneko3, S. Kaneko4,
Conclusion: The use of this morphology-based
T. Kashiwaba4
classification is practical and useful since it considers the 1Neurology, Kashiwaba Neurosurgery Hospital, 2Neurology,
totality of the brain and not only the posterior fossa. Clinical Hokuyukai Neurological Hospital, 3Neurophysiology,
and radiological studies of CBCD allow accurate diagnosis 4Neurosurgery, Kashiwaba Neurosurgery Hospital, Sapporo,
and orientate genetic studies in order to propose genetic Japan
counselling.

P1864
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 415

P1865 P1868
Radiologic characteristics of low TIA and NMR
cerebrospinal fluid pressure headache N..R. Gligic, K. Kacar
L. Mohanna1, J. Garcia1, B. Lara1, A. Paipa1, Hospital for Cerebrovascular Diseases, Belgrade, Serbia
C. Homedes1, S. Aixut2, J. Prat1, F. Rubio1
P1869
1Neurology, 2Radiology, Hospital Universitari de Bellvitge,

Barcelona, Spain
MR abnormalities after the first epileptic
fit. Which is the best attitude? Report of
P1866
two cases with different evolution
Dynamic contrast enhanced MRI for the profiles, and review of literature
evaluation of metastatic melanoma E. Ranxha1, G. Brace2, G. Fejzo2, D. Dobi3, A. Rroji4,
Z. Rozhkova, O. Dolia M. Petrela2, G. Kaloshi2
Radiology, Medical Clinic BORIS, Kiev, Ukraine 1Neurology, University Hospital Centre Mother Teresa,
2Neurosurgery, 3Neurology, 4Radiology, UHC Mother
Theresa, Tirana, Albania
P1867
Priority of clinical features in comparison
with MRI DWI of the brain in the peracute P1870
period of ischemic stroke for intravenous Imaging particularities and different
thrombolytic therapy outcomes in cerebral brain thrombosis
I.A. Khasanov1,2, E.I. Bogdanov1 caused by genetic thrombophilia:
1Neurology and Rehabilitation Department, Kazan State a case report series
Medical University, 2Stroke Unit, Republic Clinical Hospital, S. Petrescu1, A. Chirita2, G.D. Vanghelie1, C. Panea1
Kazan, Russia 1Neurology, The Emergency University Elias Hospital,
2Imaging, Regina Maria Medical Center, Bucharest,
Romania

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


416 Posters, Sunday 9 September

Neurological manifestation of systemic P1872


diseases Rendu-Osler-Weber disease with hepatic
systemic shunting may lead to cerebral
P1871 manganese accumulation: clinical,
The effects of oral benfotiamine on neuropsychological and radiological
peripheral nerve function and findings
inflammatory markers in type-1 diabetes: C.J.M. van Boheemen1, E.J.J. Habets1,
a 24-month, double-blind, randomised, G.J. Lycklama Nijeholt2, C.J. Vecht1
Neurology, 2Radiology, Medical Centre The Hague, The
1
placebo-controlled trial Hague, The Netherlands
D.A. Fraser1, L.M. Diep2, I.A.H. Hovden3, K.B. Nilsen3,4, Introduction: Rendu-Osler-Weber (ROW), or hereditary
K.A. Sveen5, I. Seljeflot6,7, K.F. Hanssen7,8 haemorrhagic telangiectasia (HHT) is associated with
1Diabetes Research Centre, 2Oslo University Hospital, arteriovenous malformations (AVM) in various organs,
3Department of Neurology, Section for Clinical including porto-systemic shunting with neurological signs
Neurophysiology, Oslo University Hospital, Oslo,
4Department of Neurosciences, Norwegian University of
and manganese accumulation in the basal ganglia. There are
hardly any reports on the relationship between neurological
Science and Technology, Trondheim, 5Diabetes Research
symptoms with cognitive changes and neuroradiological
Center, 6Center for Clinical Heart Research, Department of
findings of manganese accumulation in ROW.
Cardiology, Oslo University Hospital, 7Faculty of Medicine,
University of Oslo, 8Department of Endocrinology, Oslo
Methods: 2 patients with previously diagnosed ROW were
University Hospital, Oslo, Norway tested on tremor, myoclonus, dystonia, hypo- and
bradykinesia, rigidity, speech and walking difficulties.
Introduction: Short-term studies (3-6 weeks) in humans Extensive neuropsychological testing included Stroop
have suggested that high-dose benfotiamine (up to 600mg/ colour and Word test, subtests of Wechsler Adult Intelligence
day) can improve symptomatic scores in diabetic Scale-III, Number connecting test A and B, Wisconsin card-
polyneuropathy. To assess the efficacy of long-term sorting test and Rey-Osterrieth complex figure. Blood
supplementation, we have carried out a 24-month, chemistry, CT imaging of abdomen and MRI of brain were
randomised, double-blind, placebo-controlled study to performed.
evaluate whether 300mg/day benfotiamine supplementation Results:
can influence peripheral nerve function, soluble markers of Patient #1 showed an abdominal AVM at the inferior
inflammation and other biochemical variables in patients mesenterical vein, and patient #2 had an intrahepatic AVM
with type-1 diabetes. with enlarged portal veins. Detailed neurological
Methods: 67 patients with type-1 diabetes of more than 15 examination revealed no abnormalities in #1; #2 showed a
years duration (mean 31 years) were randomly assigned to mild postural tremor and discrete facial hypokinesia.
receive 24-months benfotiamine (300mg/day) or placebo Neuropsychological testing showed moderate attention
supplementation. Peripheral nerve function and levels of dysfunction in #1 and normal values in #2. Hepatic function
soluble inflammatory variables were assessed at baseline was normal in both patients. MRI markedly showed
and after 24 months. Tests of peripheral nerve function hyperintense T1 signal at the globus pallidus in both patients
included nerve conduction studies (including F-waves) of suggesting manganese accumulation; the pallidal index was
peroneal, tibial, and sural nerves, as well as heart rate 140 and 166, in #1 and #2, respectively.
response to deep breathing. Conclusion: Hepatic AVM leading to cerebral accumulation
Results: 59 patients completed the study. Marked increases of manganese results in a hyperintense T1 signal at the basal
in whole-blood concentrations of thiamine and thiamine ganglia with a variable severity of extrapyramidal, cognitive
diphosphate were found in the benfotiamine group (both p< or behavioural abnormalities. The underlying
0.001 as compared to placebo). However, no significant pathophysiological mechanism of manganese storage in the
differences in changes between the groups in either brain and the effects on neurotransmitter function will be
peripheral nerve function or soluble inflammatory discussed.
biomarkers were observed.
Conclusion: Our findings suggest that high-dose
benfotiamine (300mg/day) supplementation over 24 months
has no significant effects upon peripheral nerve function or
soluble markers of inflammation in patients with type-1
diabetes.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 417

P1873 P1874
Drosophila Parkinsons mutant displays MRI findings and outcome of neuro-
olfactory impairment both to natural and Behets disease: the predictive factors
synthetic compounds S. Farahangiz1, M. Mahmoodi2, S. Sarhadi1, A. Safari3,
S. Poddighe1, M.D. Setzu2, P. Angioni3, P. Solla4, A. Borhani Haghighi4
A. Costa4, F. Marosu4, A. Liscia3 Department of Radiology, 2Health Policy Research Center,
1

1Life and Environmental Sciences, Section of Neuroscience, Research Center for Traditional Medicine and History of
3

Neurological and Cardiovascular Sciences, 2Biomedical Medicine, 4Department of Neurology, Shiraz University of
Sciences, 3Life and Environmental Sciences, Section of Medical Sciences, Shiraz, Iran
Neuroscience, 4Neurological and Cardiovascular Sciences, Introduction: Initial MRI findings of patients with neuro-
University of Cagliari, Italy Behets disease can predict unfavourable outcomes and
Introduction: Parkinsons disease (PD) is the most course of disease in these patients.
common degenerative disorder characterized by the clinical Methods: All the consecutive patients referred from 2002
triad: tremor, akinesia and rigidity. Recently, several studies to 2009 to Behet Clinic at Nemazee Hospital, Shiraz, Iran,
suggested that PD patients, at their very first onset, show who fulfilled ISG criteria for Behets disease and diagnosed
disturbance of olfaction which, since not restrained to PD, as neuro-Behets disease, were enrolled in this study.
has recently received attention in several studies. Characteristics of initial brain MRI were studied in patients
The fruit fly is a powerful model organism for studying with different courses of neuro-Behets disease.
neuronal dysfunction and loss that proceeds from Results: Initial MRIs of 58 patients (31 women) with a
neurodegenerative diseases. The Drosophila genetic model mean SD age of 38.99.7 years were reviewed. 49 (84%)
of PD (Dmel/Pink1 B9) displays the two most important patients had parenchymal and 9 (16%) had non-parenchymal
diagnostic criteria of the disease: rigidity and akinetic neuro-Behets disease. Of those patients with parenchymal
behavioural. neuro-Behets disease, 15 (31%) had monophasic, 13
In this study the olfactory sensitivity of the PD flies has (27%) polyphasic, and 10 (20%) had progressive courses;
been investigated for the first time. 11 (22%) had only headache attributed to Behets disease.
Methods: Electrophysiological recordings have been The most common sites of involvement in patients with
obtained in response to natural volatiles and synthetic parenchymal neuro-Behets disease were periventricular
compounds from groups of PD flies in their early stage of and superficial cerebral white matter, midbrain and pons,
their life cycle (3-10 days) and in a group at the end of it respectively. Of those with parenchymal involvement, 12
(15-30 days). The results have been compared with the (24%) had extension of lesions, 7 (14%) had contrast
same age-groups of wild type flies. enhancement, 12 (24%) had black holes and 5 (10%) had
Results: In both age-groups of PD flies, a decrease in brainstem atrophy. Patients with the progressive course had
olfactory response has been measured in respect to the wild a significantly (p=0.017; OR=18, 95% CI: 1.7-19.1) higher
type flies tested to natural substances and to Hexanol and rate of brainstem atrophy than those with non-progressive
-pinene among the synthetic volatiles. Morphological, an course (monophasic or polyphasic).
analysis of neuro-anatomical correlates is in progress. Conclusion: Presence of brainstem atrophy in the initial
Conclusions: Our result demonstrates that PD flies show MRIs may predict a progressive course in patients with
olfactory impairment and suggest a good tool for a new neuro-Behets disease.
approach for studying Parkinsons disease and may greatly
contributes to better underline the development of
preclinical strategies to treat it.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


418 Posters, Sunday 9 September

P1875 P1876
Thymoma-associated cerebellitis: the Consequences of an impaired cholinergic
early stage of a paraneoplastic cerebellar system in the CNS and at the periphery
degeneration? A. Hrabovska1, E. Krejci2
1Department of Pharmacology and Toxicology, Faculty of
T. Teodoro1, D. Neutel1, P. Pita Lobo1, L. Neto2,
M. Coelho1, L. Albuquerque1 Pharmacy, Comenius University, Bratislava, Slovak
Neurology Department, 2Neuroradiology Department,
1 Republic, 2CNRS UMR 8194, Centre dEtude de la
Hospital de Santa Maria, Lisbon, Portugal Sensorimotricit, Universite Paris Descartes, Paris, France
Background: Thymoma are infrequent causes of Introduction: The cholinergic system in brain is believed
paraneoplastic cerebellar degeneration (PCD). In PCD, an to be in control of some major physiological functions and
initial oedematous phase has rarely been observed. We its dysfunction leads to severe pathologies, e.g., Alzheimers
report a case of subacute cerebellitis associated with a disease, Parkinsons disease and Huntingtons chorea.
thymoma. Moreover, a mechanism by which cholinesterase (ChE)
Case report: A 34-year-old woman was admitted following inhibitors improve cognition in dementia is explained by
a 1-month history of mild fever, headache, dizziness, enhancement of cholinergic transmission in the brain. Here
vomiting and progressive gait imbalance. we present our recent results from genetic studies in mice
She was confused, with a horizontal nystagmus in all gaze that contrast with recent views on the cholinergic system in
positions and mild limb but severe truncal and gait ataxia. the brain.
A traumatic lumbar puncture showed 249 cells/mm3 Methods: Genetically modified mouse models were used in
(predominantly lymphocytes) and 117mg/dL proteins. which acetylcholinesterase is absent in the whole animal
Brain MRI showed diffuse symmetrical swelling of the (AChE-/-), only soluble monomer AChE is present (E5,6-/-),
cerebellum, with cortical-pial enhancement and or ChE are locally omitted predominantly in CNS (PRiMA-/-)
compression of the fourth ventricle. Acyclovir was or at the neuromuscular junction (ColQ-/-). Histochemical
administered, but there was progressive clinical and biochemical analyses of cholinergic markers were
deterioration. PCR analysis for neurotrophic virus in CSF performed. Phenotype and behavioural test results were
was negative. Serum viral serologies were unremarkable. compared with wild-type littermates.
Body CT scan revealed an anterior mediastinal mass, which Results: In AChE-/-, PRiMA-/- and E5,6-/- mice, we
proved to be a thymoma after biopsy. An extensive screening confirmed high levels of acetylcholine in brain resulting
for anti-neuronal antibodies was negative. The patient from similar acetylcholinesterase deficiency in the CNS.
underwent surgical removal of grade II thymoma, with a This was accompanied with severe changes in the number
striking clinical improvement in the following days. A of acetylcholine receptors. AChE-/- mice developed a
follow-up MRI documented radiological resolution. A dramatically changed phenotype (including motor tremor,
second lumbar puncture revealed 30 cells/mm3. lower body weight, seizures and impaired thermoregulation).
Discussion: We report a case of cerebellitis associated with E5,6-/- mice had a similar but less profound appearance.
a thymoma. The impressive clinical and radiological However, PRiMA-/- mice were indistinguishable from the
improvement immediately after tumour removal, and an wild-type littermates. On the other hand, changes in the
extensive investigation negative for infectious causes being phenotype of ColQ-/- mice were severe and resembled the
consistent with an immunological paraneoplastic response. phenotype of AChE-/- and E5,6-/- mice.
The aspect of oedematous cerebellitis possibly corresponds Conclusion: Our results suggest that some actions of the
to an early phase of PCD, previously rarely observed. cholinergic system may arise from the periphery, rather than
Moreover, this patient evolution suggests that this may be a from the CNS as believed nowadays.
still reversible stage of PCD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 419

P1877 P1878
Central nervous system involvement in A retrospective study of neuro-Behets
primary Sjgrens syndrome disease
I. Moreira1, F. Teixeira2, A.M. Silva1, C. Vasconcelos3, F. Imounan1, E.H. Ait Ben Haddou2, W. Regragui3,
F. Farinha3, E. Santos1 A. Benomar2,4, R. Abouqal5, M. Yahyaoui2
1Neurology Department, Hospital Santo Antnio, Centro 1Neurology B and Neurogenetics, Mohammed V University

Hospitalar do Porto, Porto, 2Rheumatology Department, Souissi, 2Neurology B and Neurogenetics, 3Neurology and
Unidade Local de Sade do Alto Minho, Ponte de Lima, Neurogenetics, Hpital des Spcialits, CHU Rabat Sal,
3Clinical Immunology Unit, Hospital Santo Antnio, Centro Mohammed V Souissi University, 4Facult de Mdecine et de
Hospitalar do Porto, Portugal Pharmacie, Centre de Recherch en Epidmiologie Clinique
Introduction: Primary Sjgrens syndrome (pSS) is a et Essais Thrapeutiques (CRECET), 5Facult de mdecine
systemic autoimmune disease characterized by salivary/ et de pharmacie Rabat, Laboratoire de Biostastistiques et de
lacrimal glands lymphocytic infiltration and auto-antibody Recherche en Clinique pidmiologique (LBRCE), Rabat,
secretion, excluding other disorders. The burden of central Morocco
nervous system (CNS) involvement is controversial. We Introduction: Neurological manifestations in Behets
performed an observational retrospective cross-sectional disease represent between 4 to 49% of systemic
case-control study to evaluate prevalence, clinical patterns manifestations and remain the leading cause of morbidity
and outcomes of CNS involvement in a cohort of pSS and mortality.
patients followed in our centre. Aim of study: This report describes clinical features,
Methods: We studied the CNS involvement in 91 pSS therapeutic aspects and follow-up of neuro-Behets
patients (88 females, 3 male). Mean age was 47.6 years. disease.
Diagnosis was established according to the criteria of the Methods: Retrospective series of 56 neuro-Behet cases
American-European Consensus Group. Demographic, fulfilling the International Study Group criteria for Behets
clinical, immunological data were assessed. disease were consecutively recruited over a period from
Results: Nervous system involvement was detected in 24 June 2004 to December 2010. All patients had clinical and
patients (26.4%), of which 12 (13%) had CNS involvement. ophthalmologic examinations; they underwent laboratory
All were women with mean age at disease onset and and imaging investigations. Patients with severe conditions
neurological onset of 40 and 44 years, respectively. (parenchymal involvement and cerebral deep venous
Neurological syndromes found were: seizures in 2 patients, thrombosis) received cyclophosphamide and corticosteroids.
motor and sensory deficits in 2, movement disorders in 2, The other patients received only corticosteroids.
chronic progressive myelitis in 1, chronic progressive Anticoagulant therapy was given to patients with cerebral
myelitis and dementia in 1, aseptic meningitis in 1, and venous thrombosis. Patients follow-up and tolerance to
headache and dementia in 1. From the 24 patients with treatment were analyzed.
neurological involvement, 12 had headaches: 9 migraine Results: The average age at diagnosis was 3412 years,
and 3 tension type headache. From those, only two with with a sex ratio of 1.15. The clinical presentation was
magnetic resonance abnormalities compatible with dominated by meningo-encephalitis (50.9%), cerebral deep
inflammatory disease were included. Neurological venous thrombosis (43.4%) and myelopathy (5.7%). Of the
involvement was the initial manifestation in 8 (75%). The 56, 16 patients were treated by corticosteroids and 40
CNS involvement outcome was good in 10 (83%). patients received cyclophosphamide associated with
Conclusion: Our study showed great heterogeneity of CNS corticosteroids. All patients, despite two aggravated cases,
involvement in pSS which managed to be frequently the evolved positively with clinical improvement and good
initial manifestation of pSS, as had been found in previous tolerance.
studies. The prevalence was much higher than the majority Conclusion: The demographic and clinical aspects of our
of the series in the literature. series are similar to those reported in the literature. In
contrast to previously reported cases of a poor prognosis in
severe neuro-Behets disease, our study suggests that
immediate and aggressive treatment by cyclophosphamide
may ameliorate the prognosis. However, a multicenter study
is needed to confirm the possible efficacy of
cyclophosphamide and to further assess the long-term
tolerance.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


420 Posters, Sunday 9 September

P1879 P1880
Astrocytes are sensitive indicators of Neurological status assessment in
hyperthermia-induced brain oedema in patients after orthotopic liver
normal and in Cu nanoparticles treated transplantation
rats S. Kolomiets1,2, O. Ionova1, E. Borovkova1
1Neurological and Neurosurgical Diseases Department,
H.S. Sharma1, D.F. Muresanu2, M.A. Smith3,
G. Casadesus Smith3, A. Sharma1 Belarusian State Medical University, 2Neurological
1Surgical Sciences, Anaesthesiology & Intensive Care Department, 9 City Hospital, Minsk, Belarus
Medicine, Uppsala University, Uppsala, Sweden, Background: Neurological disorders are the commonest
2Nerosciences, University of Medicine and Pharmacy Cluj-
reason of life quality decreasing in patients after orthotopic
Napoca, Romania, 3Department of Pathology, Case Western liver transplantation (OLT). The actuality of the problem
Reserve University Medical School, Cleveland, OH, USA depends on the increase of the amount of OLT in Belarus.
Whole body hyperthermia (WBH) induces breakdown of Objective: To estimate the neurological disorders intensity
the blood-brain barrier (BBB), oedema formation and brain in patients after OLT.
pathology. However, hyperthermia induced damage to glial Methods: 20 patients after OLT were investigated, treated
cells are still not well documented. The glial cells actively in RSPC Organs and Tissue Transplantation, 10 men and
regulate the brain fluid micro-environment and maintain the 10 women, mean age 39.612 (20-59 years), Body mass
BBB function. Thus, it is likely that hyperthermia will Index (BMI) - 23.14.2kgm2, basic disease duration 3.44.8
influence astrocytic reaction in WBH. Previous studies in a years; the period they were listed 49 months; the follow up
rat model of WBH exhibited profound BBB disruption, - 1211 months.
oedema formation and brain pathology. In the present Neurophysiological testing: Mini-Mental State Examination
investigation, we examined the role of astrocytic activation (MMSE), Frontal Assessment Battery (FAB), Hospital
following WBH in rats using glial fibrillary acidic protein Anxiety and Depression Scale (HADS); Paced Auditory
(GFAP) immunoreactivity. Furthermore, influence of Serial Addition Test 3 (PASAT 3).
copper (Cu) nanoparticles (CuNPs 50-60nm) on WBH The neuropathy intensity was assessed by Neuropathy
induced GFAP immunoreactivity was also investigated. Disability Score (NDS).
Rats subjected to WBH (38C for 4h) resulted in massive Results: The mean indicator on NDS was 6.43.7. The mild
increase in GFAP immunoreactivity in the thalamus cognitive disorders were revealed in 6 patients on MMSE
followed by hypothalamus, pons, brain stem, cerebellum, 24.7+1.8, on FAB 14.41.5. The operative memory decrease
hippocampus, caudate nucleus and cerebral cortex. Pre- (PASAT 3 27.514.1) presented in 8 patients. Depression
treatment with CuNPs (50mg/kg, i.p. for 1 week) and anxiety presented in 13 patients (HADS 10.14.5). The
exacerbated GFAP immunoreactivity in all the brain areas correlation between neurological disorders intensity and
examined. This activation of astrocytes in WBH was largely depression-anxiety manifestations (<0.05), aged >40 y.o.,
seen in areas exhibiting BBB disruption and brain oedema gender (mostly women), BMI >25kg/m2, basic disease
formation in normal or nanoparticle treated rats. These duration >5 years, the period they were listed >6 months,
observations suggest that astrocytes are sensitive indicators the follow-up >5 months were registered.
of thermal brain injury, not reported earlier. Conclusions: In neurophysiological testing prominent
operative memory decreasing, depression and anxiety
presence was revealed. In neurological status the distal
symmetrical sensorimotor polyneuropathy of moderate
stage was present. The obtained results may prove the
neurological disorder progression.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 421

P1881 P1882
Reversible injury of the corticospinal tract Use of body-CT and PET-CT in the
with decreased perfusion-weighted investigation of paraneoplastic
magnetic resonance imaging in a patient neurological syndromes: retrospective
with transient hypoglycaemic hemiparesis audit of practice in a regional
J.-H. Kwon1, W.-J. Kim1, S.-H. Lee2, M.-S. Kim2 neuroscience unit in the UK
Neurology, 2Ulsan University Hospital, Ulsan, Republic of
1
D.Z.J. Lee1, A. Kheder1, M. Forbes1, I. Craven2,
Korea M. Hadjivassiliou1
Profound hypoglycaemia has diverse neurological 1Department of Neurology, 2Department of Radiology,
manifestations from headache to coma and death. Transient Sheffield Teaching Hospitals, Sheffield, UK
hypoglycaemic hemiparesis (THH) is the rarely reported Background: Paraneoplastic neurological syndromes
complication of hypoglycaemia, usually misdiagnosed as (PNS) are characterised by progressive neurological
ischemic stroke. We report a patient with THH whose dysfunction resulting from non-metastatic effects of cancer.
diffusion-weighted image (DWI) and apparent diffusion Positron emission tomography (PET) has emerged as an
co-efficient (ADC) values showed reversible lesions in the effective tool for diagnosis of PNS. An advantage of PET
corticospinal tract from the internal capsule to the midbrain. over conventional imaging modalities such as body-CT
A 75-year-old man with diabetes mellitus and hypertension includes demonstration of metabolically active disease in
was admitted to our hospital complaining of right-sided anatomically normal appearing structures.
weakness and dysarthria. He was diagnosed with type-2 Aims: To analyse and improve use of whole-body CT and
diabetes 10 years ago and took metformin, voglibose and PET-CT in the investigation of PNS in a regional
glimepiride. He was slightly drowsy but communicable. neuroscience unit.
Other neurologic examinations showed right facial palsy Methods: Retrospective review of 42 patients with
and hemiparesis. Initial blood glucose was 34mg/dl. suspected PNS referred for imaging between April 2007
Intravenous dextrose was given to him. Routine laboratory and March 2008 was conducted. The data was presented
tests were all within normal range. Initial DWI performed locally followed by recommendations that a consultant
at 1.5 hour after symptom onset showed high signal neurologist with interest in PNS reviews; each case before
intensity lesions in the left internal capsule and midbrain referral and PET-CT is considered, in cases where body-CT
compatible with the corticospinal tract. ADC values reduced is negative. We re-audited 44 patients referred between July
in the same area. Perfusion-weighted image disclosed 2008 and June 2009 and identified each patients final
delayed mean transit time in the posterior limb of the left diagnosis along with any additional investigations
internal capsule. MR angiography showed no intracranial undertaken.
artery stenosis. Blood sugar was increased to 155mg/dl and Results: In the first cycle, 42 patients underwent whole-
his neurologic deficits were fully recovered after MRI. body CT for investigation of PNS. 4 scans were positive for
DWI, ADC values and FLAIR image taken 5 days after malignancy and 38 were negative, of which only one was
symptom onset revealed completely resolved lesions. In followed up with PET-CT. In the second cycle, whole-body
conclusion, THH seems to be the sequela of reversible CT was performed in 44 patients, of which 11 subsequently
cytotoxic oedema. Physicians should consider the possibility proceeded to PET-CT. 5/44 patients had a final diagnosis of
of THH when MRI reveals the lesion suggesting ischemic PNS, of which only 3 had undergone PET-CT.
stroke in a patient with hypoglycaemia. Conclusion: Early PET scanning in clinically suspected
PNS may prevent expensive and often unnecessary
investigations. Trust guidelines now advise use of PET-CT
rather than conventional body-CT in clinically suspected
PNS. We aim to re-audit our practice in due course.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


422 Posters, Sunday 9 September

P1883 P1884
A rare case of embolic stroke due to Marchiafava-Bignami disease, Wernickes
uterine leiomyosarcoma treated with encephalopathy or metronidazole toxicity
mechanical thrombectomy in a non-alcoholic patient: a diagnostic
A.J. Paipa, R. Gomez, L. Mohanna, P. Cardona, A. Lucia, challenge
H. Quesada, L.M. Cano, F. Rubio A.L. Azevedo1, J. Marques1, J. Passos1, M. Tavares2
Hospital Universitari de Bellvitge, Barcelona, Spain Neurology, 2Neuro-Radiology, Instituto Portugues de
1

Introduction: Stroke as a direct consequence of tumoral Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal
emboli is rare and mainly described in primary cardiac or Introduction: Marchiafava-Bignami disease (MBD) is a
lung cancer. rare entity, usually associated with chronic alcoholism,
Methods: We present the case of a 59-year-old woman with characterized by demyelination and necrosis of the corpus
past medical history of obesity and hypothyroidism who callosum. It has been sporadically reported in non-alcoholic
presented with dysartria, left hemianopsia and left-sided patients, especially in the context of malnutrition or cancer,
hemiparesis, (NIHSS 10). A CT scan showed no and was also reported in patients with concomitant
abnormalities and the angioCT demonstrated a right distal Wernickes encephalopathy (WE). Metronidazole is
internal carotid artery occlusion (TICI 0). Lab test results metabolized into an unstable analogous of thiamine, and
were normal apart from mild leucocytosis. A mechanical can induce an WE-like syndrome.
thrombectomy with a Solitaire device was attempted after Case report: 57-year-old female patient, with mucinous
unsuccessful intravenous thrombolysis. Recanalization of cystadenocarcinoma of the appendix previously submitted
the internal carotid artery was obtained while the middle to right hemicolectomy and small bowel resection. The
cerebral artery remained partially occluded (TICI 1). patient was under prolonged parenteral nutrition due to
Results: The histopathological examination of the thrombus surgical complications and chronically treated with high
was compatible with a mesenchymal sarcomatous tumour. doses of metronidazole. She presented with acute onset of
A transoesophagic echocardiography showed a superior spontaneous vertical nystagmus and dysarthria followed by
right pulmonary vein mass protruding to the left atrium and rapid neurological deterioration, with somnolence, apathy,
a toracoabdominal CT showed a heterogeneous, cystic and disorientation, nystagmus in all directions of gaze, paresis
large (13cm) uterine mass with calcified regions as well as of left eye adduction, bilateral pyramidal signs, ataxia and
disseminated lung nodules compatible with a methastasic limb hypoaesthesia in a stocking-sleeve pattern. T2
leiomyosarcoma. The MRI showed flux absence in the right -weighted MRI showed bilateral symmetric hyperintense
middle cerebral artery as well as deep mild hemorrhagic lesions in corpus callosum (mainly splenium), deep
transformation. No clinical changes were seen at the time of cerebellar white matter, pons, optic chiasm and optic tracts.
discharge (NIHSS 10, mRS 4). EMG showed severe axonal sensory peripheral neuropathy.
Conclusions: Stroke due to tumour emboli is a very rare Replacement therapy with thiamine and folate was started,
complication of systemic cancer. To our knowledge this is with prominent clinical and imagiological improvement.
the first reported case of a leiomyosarcoma presenting as a Conclusions: In this case, the clinical picture and MRI
cerebrovascular tumoral emboli. findings combine different distinctive features of WE, MBD
and Metronidazole toxicity. In selected patients with
malabsorption syndromes, WE and MBD may share similar
pathogenic pathways to metronidazole toxicity. Given the
potential dismal prognosis (especially for MBD), prompt
replacement treatment with thiamine should be initiated.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 423

P1885 P1886
Intracranial plasmacytoma mimicking Sjgrens syndrome and mixed connective
meningioma tissue disease: a case report and a review
M. Holling1, B.R. Fischer1, T. Niederstadt2, W. Stummer1 of literature
1Department of Neurosurgery, 2Department of Clinical M. Lahmar, M. Messelmani, H. Khaled, I. Hmida,
Radiology, University Hospital Mnster, Germany M. Mansour, J. Zaouali, R. Mrissa
Objective: Meningioma is the most common dural Neurology, Military Hospital, Tunis, Tunisia
neoplasm. However, a variety of tumour entities may The occurrence of neurological or psychiatric disturbances
present clinically as meningiomas. Even though a significant in association with rheumatic diseases has been an area of
number of patients with solitary plasmacytomas eventually considerable interest for many years. Mixed connective
develop multiple myeloma, intracranial plasmacytoma is tissue disease (MCTD) is classically considered as an
exceptional and has been rarely described as primary overlap of three diseases, systemic lupus erythematosus,
manifestation of multiple myeloma. scleroderma, and polymyositis that typically have high
Methods: A 61-year-old man presented with a left occipital quantities of antinuclear antibodies (ANAs) and antibodies
skull protrusion. MRI of the head showed a lesion suggestive to ribonucleoprotein (anti-RNP). Sjgrens Syndrome (SS)
for intra-osseous meningioma. Intraoperatively, the tumour is rarely reported in MCTD. We report the case of a 53-year-
imposed as meningioma. Histologically, a tumour of high old woman with no medical history who presented relapsing
cellularity was encountered. Based on typical findings, a remitting neurological manifestations for 20 years. The
neuropathological diagnosis of plasmacytoma was patient presented with memory impairment, gait disorder,
established. This diagnosis prompted complete skeletal paresthesias, arthralgias and worsening of general state. On
radiographs, which revealed an osteolytic lesion of the examination, the patient showed features of cognitive
eighth rib. Before chemotherapy could be initiated, the impairment, frontal lobe syndrome, ataxic and spastic gait
patient died of aspergillus pneumonia. and left hemiparesis. MRI showed multiple hyperintense
Results: Intracranial plasmacytomas are rare and lesions of supra and infra tentorial white and grey matter
infrequently diagnosed by imaging due to their resemblance and subcortical atrophy. Cerebrospinal fluid studies showed
to meningiomas. On MRI, plasmacytomas are often a mild elevation of protein level. Salivary gland biopsy,
indistinguishable from meningioma, with intermediate salivary gland scintigraphy and ophthalmologic examination
signal on T-weighted sequences and isointense signal to showed features consistent with SS. ANAs and anti Scl 70
grey matter on proton density and T2-weighted sequences. antibodies were increased whereas anti SSa/SSb,
The tumour showed typical histological and rheumatoid factor and anti DNA were negative. MCTD
immunohistochemical staining of plasmacytoma with with predominating features of SS was diagnosed. The
positivity for the plasma cell marker CD138 and strong patient received methylprednisone intravenously for three
positivity for kappa light chain. Since additionally CD20- days and cyclophosphamide monthly. A mild improvement
expressing lymphocytic tumour cells were also encountered, in cognitive and motor performances was noticed. MCTD
a diagnosis of lymphocytic plasmacytoma was is a rare autoimmune disorder and cause of treatable
established, which is very rare. dementia. SS is rarely reported in MCTD. Neuropsychiatric
Conclusions: In conclusion, solitary intramedullary features may be the first manifestations of the disease.
plasmacytoma should be considered in the differential Symptoms eventually evolve to become dominated by
diagnosis of meningioma. Conversely, in dural based features of one of the component illnesses.
plasmacytoma lymphocyte rich meningioma must also be
considered as a differential diagnosis. In some cases
differentiation may be difficult.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


424 Posters, Sunday 9 September

P1887 P1888
Catastrophic anti-phospholipid syndrome: Clinical manifestations and imaging
analysis of a clinical and morphological findings in posterior reversible
case encephalopathy syndrome
M. Tviachkovskaya, M. Cvechkovskaya, O. Udina, S.-Y. Bae, S.-J. Lee
M.D. Ponomarev Neurology, Yeungnam University College of Medicine,
Belarusian Medical Academy of Postgraduate Education, Daegu, Republic of Korea
Minsk, Belarus Objectives: Posterior reversible encephalopathy syndrome
Introduction: Catastrophic APS - a rare form of primary (PRES) is typically characterized as symmetric and
anti-phospholipid syndrome (APS) is associated with reversible oedema of the posterior cerebral hemisphere with
autoimmune thrombocytopathies. There are multiple variable symptoms. This study determines the incidence of
thromboses of arteries and veins of different localization. clinical and MRI imaging manifestations of PRES.
Materials and methods: 43 patients with primary APS (26 Materials and methods: We included 16 patients who
female and 17 male, mean age 38.79.7 ) were observed at were diagnosed with PRES, from October 1, 2003, through
the neurological department Hospital 5 from 2001 t February 28, 2012, based on clinical manifestations and
2011. Diagnosis was confirmed accordingly with universally MRI findings. We performed a review of clinical
adopted International Criteria (Sydney, 2006). Macro- and information, including cause of PRES, presenting
microscopic examination was performed in case of autopsy. symptoms, and imaging findings. The lesions were recorded
Results: The main manifestation of primary APS was acute on the basis of FLAIR findings. As atypical imaging
vascular lesions of the brain caused by thrombosis. It is findings, we included gadolinium enhancement, restricted
displayed as stroke (73.1%). In case of a female, 36 years diffusion, haemorrhage, and unilateral or irreversible
old, with an obstetric history (miscarriage) we observed lesions
catastrophic APS, which was affected by multiple Results: The aetiologies of PRES were hypertension,
thromboses: skin lesions (bed sores, haemorrhage), lung 43.8%; chronic kidney disease, 25.0%; eclampsia, 12.5%;
(heartattacks in both lungs), heart (mitral valve vegetation multiple organ dysfunction, 6.2%; and unclear cause,
in the diffuse cardio), oesophagus (multiple erosions and 12.5%. The main presenting symptoms were seizure,
ulcers over), colon (paresis), brain (haemorrhage, and 43.8%; encephalopathy, 68.8%; headache, 31.2%; dizziness,
multifocal infarcts in both hemispheres), which ended 18.8%; and visual disturbance, 18.8%. The incidence of the
lethally. Microvascular thrombi of varying degree of regions of involvement was parieto-occipital, 87.5%;
limitation were found on microscopic examination, a part of posterior frontal, 62.5%; temporal, 50.0%; brainstem,
recanalization without inflammatory infiltration of vessel 37.5%; cerebellar, 43.8%; thalamus, 37.5%; basal ganglia,
walls. 31.2%; and corpus callosum 18.8%. The incidence of
Conclusion: Catastrophic APS develops acutely, manifests atypical MRI manifestations were gadolinium enhancement,
multiple systemic thromboses and has a poor prognosis. 50.0%; restricted diffusion, 31.3%; haemorrhage, 12.5%;
Macro- and microscopic results are crucial in its diagnosis. unilateral lesion, 0%; and irreversible lesion, 41.7%.
Conclusions: Even though it is somewhat hard to accept the
result of this study as an established fact because of the
small number of cases, the result suggests that atypical MRI
manifestations of PRES, contrast enhancement, were more
frequent than that commonly perceived in the previous
literature.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 425

P1889 P1890
Neurological complications of Celiac A case of flaccid paraparesis caused by
disease: a report on 4 patients intestinal Taeniasis
H. Haj Kacem, E. Turki, I. Bouchaima, M. Damak, M. Sabau1, A. Comanescu1, L. Botnaru2
A. Boukhris, I. Feki, M.I. Miladi, C. Mhiri 1County Clinical Emergency Hospital Oradea, University of

Department of Neurology, Habib Bourguiba Hospital, Sfax, Oradea, 2County Clinical Emergency Hospital Oradea,
Tunisia Romania
Introduction: Celiac disease (CD) is an auto-immune Introduction: Taeniasis is an uncommon parasitic infection
gastro-intestinal disorder occurring in genetically in Romania. It usually has unspecific symptoms: abdominal
susceptible individuals triggered by the ingestion of gluten colic, constipation or diarrhoea, anal itching, headache and
and characterized by the presence of anti-transglutaminase nervous hyper excitability.
2 and anti-gliadin antibodies. Clinical manifestations are Objective: We present a case with paraparesis diagnosed as
various. Neurological involvements of CD are rare. The nutrition-related myelopathy and polyneuropathy in a
objective was to determine the clinical, para clinical, nutritional deficiency syndrome caused by intestinal Taenia
therapeutic and the outcome of patients with CD with infection.
neurological involvements. Case presentation: A 62-year-old man reports a long
Methods: A 19-year retrospective study, including all standing diarrhoea of more than 1 year duration, following
patients followed at the neurology departments in Sfax, a vegetarian and hypocaloric nutrition regimen. Marked
Tunisia for CD with neurological involvements. asthenia, as well as pain and numbness in the extremities
Results: We collected 4 patients with a female predominance developed in the following months, while reduction of the
(sex ratio: 0.3). The average age of onset of neurological muscle strength and gait disturbance occurred in the last 4-5
involvements was 20 years (range 7 to 36 years). weeks. Serial and complex medical examination gave
Neurological manifestation was inaugural in one case. It unremarkable findings. Pallor of the skin and of the mucous
was peripheral nerve damage in one case and central membranes, with furfuraceous desquamation was found on
nervous system (CNS) in 3 patients (cerebellar ataxia, physical examination. The gait was unsteady and of
seizures and cerebral venous thrombosis). All patients steppage type, as if walking on pillows. Impairment of all
received a gluten-free diet (GFD) with a favourable types of sensibility, distally distributed in both upper and
outcome. lower limbs, Lhermittes sign, absence of deep tendon
Conclusion: Neurological complications of CD are various reflexes and Babinski sign were found, too. Laboratory
and can especially be revealing CD in adults. Their findings disclosed a mixed type anaemia, an increased
pathophysiological mechanisms are still uncertain. They number of eosinophiles, and decreased serum levels of iron
may affect the CNS (often cerebellar ataxia and/or seizures). and B12 vitamin. Repetitive parasitological examination of
Peripheral neuropathy is common. On brain imaging, the stool was negative for several days, but showed Taenia
calcification and white matter signal abnormalities are most solium infestation after ten successive examinations.
often described. The introduction of a gluten-free diet often Niclosamide, B12 vitamin, folic acid, iron and a well
improves clinical symptoms. balanced nutrition were started, with rapid improvement of
the symptoms.
Conclusion: Malnutrition due to taeniasis resulted in severe
anaemia, nutritional deficiency polyneuropathy and
myelopathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


426 Posters, Sunday 9 September

P1891 P1892
Chronic inflammatory demyelinating Complicated pyloric stenosis with
polyradiculoneuropathy and systemic Wernickes encephalopathy
lupus erythematosus R. Manso-Caldern1, T. Gonzlez2, M.T. Garca3
1Neurology, Hospital Universitario de Salamanca,
M. Zouari, A. Hassine, I. Bedoui, F. Hentati
Salamanca, 2General Surgery, 3Neurology, Hospital Nuestra
Neurology Department, National Institute of Neurology, Seora de Sonsoles, vila, Spain
Tunis, Tunisia
Introduction: Chronic inflammatory demyelinating Introduction: Wernickes encephalopathy (WE) is a serious
polyradiculoneuropathy is often idiopathic. It is presumed but curable neurologic disease caused by thiamine
to be caused by an antibody-mediated immunologic reaction deficiency. The classical clinical triad of ocular
and may be associated with other systemic diseases such as abnormalities, gait ataxia and mental status changes is
systemic lupus erythematosus. A possible causal link present in only a subset of patients. WE is commonly
between these dysimmune diseases is still a matter of associated with alcohol consumption, but any condition
debate. (repeated vomiting, prolonged fasting, etc) that induces
Methods: A woman affected by a chronic inflammatory thiamine deficiency lasting 2 to 3 weeks may lead to WE.
demyelinating polyradiculoneuropathy associated with We present a case of WE due to pyloric stenosis.
systemic lupus erythematosus was selected. Methods: A 55-year-old man developed mental status
Results: We report the case of a 24-year-old woman who changes, including drowsiness, confusion and dizziness
developed within a few days a bilateral brachial weakness within four days. The family denied history of alcohol use,
with paraesthesia in upper limbs. Neurological examination but he had a pyloric stenosis. In the last month, the patient
found a bilateral weakness with distal sensory loss complained of gastric pain with vomiting and fasting.
predominantly in upper limbs and essentially in the Consequently, total parenteral nutrition was required. At
proximal region, associated to areflexia. The CSF contained neurological examination, he had a vertical nystagmus, VI
one white cell with hyperproteinorrachia (1.84g/l). Nerve cranial nerve palsy, signs of cerebellar dysfunction, lethargy
conduction studies found a demyelinating neuropathy. The and general muscle weakness.
diagnosis of a CIDP was retained because the patient was Results: Laboratory values were unremarkable. Brain
fulfilling the criteria of the American Academy of magnetic resonance imaging showed hyperintensive signals
Neurology. During her hospitalization, malar erythema in periventricular areas, thalamus and mamillary bodies.
appeared with lymphopenia and positive tests for antinuclear Even though the patients initial thiamine levels were not
antibody and antinuclear anti-DNA. The diagnosis of determined, WE was diagnosed. Thiamine 300mg daily
systemic lupus erythematosus was retained on the basis of intravenous was started. After 7 days, his symptoms slowly
five criteria of the American College of Rheumatology. The began to improve, with correction of dizziness and
patient had full recovery under steroid therapy. alterations in consciousness. Nystagmus and
Conclusion: We should consider the diagnosis of systemic ophthalmoplegia improved but were not reversed
lupus erythematosus in case of chronic polyradiculoneuropathy completely.
and perform specific immune tests. This case report is Conclusion: The recognition that WE can occur in non-
atypical because of the clinical presentation with brachial alcoholic patients and not always being manifested by the
weakness, which is a rare condition. classical clinical triad is sometimes forgotten. WE is a
neurological emergency and treatment should be initiated
promptly to limit morbidity and mortality.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 427

P1893 P1894
Septo-optic dysplasia and The challenges in establishing a positive
schizencephaly: a case report diagnosis of a seronegative Wegeners
L. Paziente, R. Nardello, S. Mangano granulomatosis with unusual clinical
Dipartimento Materno Infantile di Urologia e Andrologia, presentation: a case report
Unit Operativa di Neuropsichiatria, Palermo, Italy
O.C. Chebut1, R.I. Balasa2, Z. Bajko2, S. Mocan3
Introduction: Septo-optic dysplasia (SOD) is an Neurology I Department, Emergency Clinical County
1

uncommon developmental disorder involving variable Hospital Tirgu-Mures, Tirgu Mures, 2Neurology I
midline brain structures, characterized by optic nerve Department, 3Pathology Department, Emergency Clinical
hypoplasia, dysgenesis of septum pellucidum and pituitary- County Hospital Tirgu-Mures, Romania
hypothalamic dysfunction with consequent endocrine Wegeners granulomatosis/GPA (granulomatosis with
deficits. The association of septo-optic dysplasia and polyangiitis) is a small vessel pauci-immune necrotizing
cortical dysplasia is described as septo-optic dysplasia-plus. vasculitis with granulomatous inflammation that classically
Reports on patients with septo-optic dysplasia-plus have involves the upper respiratory tract, lungs and kidneys and
been rare. Other distinct features, which occur especially rarely the nervous system, gastrointestinal tract and heart. A
when cerebral cortical abnormalities are also present (SOD- 39-year-old woman known with diabetes and obesity was
plus), consist of significant generalized developmental referred to our department because of a right peripheral
delay and/or spastic motor deficits. facial nerve palsy associated with a peripheral right
Methods: We report a 10-year-old boy with septo-optic vestibular syndrome, hearing loss and acute otitis media.
dysplasia-plus syndrome, characterized by septo-optic After a careful assessment of her pathological history we
dysplasia with schizencephaly, significant generalized discovered: a pseudotumor pancreatitis two years before
developmental delay, spastic motor deficits and seizure (chronic pancreatic inflammation with no necrosis in the
intractable. Extraction of DNA for gene EMX2 is in the histological exam), a complete heart block requiring a
process of reporting permanent pacing eighteen months before (considered
Results: Neuroimaging studies revealed schizencephaly cardiac complication of a Lyme disease after an extended
with enormous ex vacuo dilation of the occipital horns of assessment with normal auto-immune serology but positive
the lateral ventricles that involves medium cells up to the Lyme serology) and no pulmonary or renal pathology. She
vertex of the brain; the residual frontal cortex of both sides presented feverish, painful strawberry gums, saddle-nose
develop a pachigiric aspect, especially in parasagittal deformity, raised dark spots on the ankle and halux,
regions; the corpus callosum is represented in all its sections arthralgia and weight loss. Laboratory showed anaemia,
but slightly hypoplastic at the rostrum and the splenium. leukocytosis, elevated ESR and repeated negative ANCA
There are also dislocated left femoral head, optic atrophy, tests. Histological examination of the mastoid biopsy
deficiency determination and alternating exotropia showed a central necrotic granulomatous process with
bilaterally multinucleated giant cells and necrotizing vasculitis. A final
Conclusion: Schizencephaly is a distinct congenital diagnosis of GPA was retained and immunosuppressive
cerebral malformation presenting as transcerebral mantle therapy with cyclophosphamide and steroids was started.
cleft between lateral ventricle and pial surface. Nearly half Three months later she developed oligoanuric acute renal
of the patients with SOD have schizencephaly. When the failure and died two days later. This uncommon GPA
SOD is associated to open-lip schizencephaly, the subjects presentation, diagnosed after cranial neuropathy, can result
have a poor prognosis with respect to psychomotor in diagnostic difficulty and may allow a potentially poor
development and complicating intractable epilepsy. outcome. Cranial neuropathies in GPA occur later in severe
systemic forms. A negative ANCA test does not eliminate a
well-diagnosed GPA.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


428 Posters, Sunday 9 September

P1895 P1897
Sensorineural hearing loss - an Acute-onset of critical illness:
auto-immune sign polyneuropathy and myopathy as a severe
C. Andrade1,2, M. Bernardes2,3, M.J. S1,4, J. Guimares1,2 complication of percutaneous
Neurology, Centro Hospitalar de So Joo, 2Faculty of
1
nephrostolithotomy: a case report
Medicine, University of Porto, 3Rheumatology, Centro
H. Zhang1, L.-M. Wu1, S.-K. Fang1, X.-B. Kong2,
Hospitalar de So Joo, Porto, 4Health Sciences Faculty,
Y. Hou2, R. Zhao2, H.-Y. Li2, H. Li2
University Fernando Pessoa, Porto, Portugal 1Department of Neurology, Jilin University, 2Department of
Introduction: Sensorineural hearing loss (SNHL) is an Urology, China-Japan Union Hospital of Jilin University,
unusual neurological symptom. An auto-immune etiology is Changchun, China
rare and its association with systemic auto-immune diseases
is neurologically relevant. We describe a patient whose
diagnosis of multiple sclerosis (MS) was reviewed after the P1898
onset of episodes of hearing loss, leading to the diagnosis of Convulsive status epilepticus due to
Sjgrens Syndrome (SS).
Case report: A 59-year-old woman who was diagnosed
hypoglycaemia as the initial presentation
with MS at 47, after an episode of vertigo/imbalance and of primary hepatic carcinoma
brain-MRI revealing supratentorial demyelinating lesions L. Wu, H. Zhang, J. Wu
with cerebral atrophy. Interferon-beta1a was started. She Jilin University, Changchun, China
remained clinically and imagiologically stable until, at 55,
relatively sudden episodes of hearing loss began, first
unilateral, then bilateral, initially with spontaneous P1899
recovery, and then only with partial response to steroids. Reversible posterior leuko-encephalopathy
Neurological examination was otherwise unremarkable. syndrome and bilateral renal
Audiogram showed a moderate SNHL. CSF study was
unremarkable (negative oligoclonal bands) and brain and
fibromuscular dysplasia
spinal MRI were unchanged. At 56, she first noticed a dry L. Mohanna1, J. Garcia1, B. Lara1, A. Paipa1,
mouth and red eyes. Schirmer test was subnormal. Retinal C. Homedes1, L.M. Cano1, S. Castaner2, J. Bruna1,
angiography was normal. Salivary gland scintigraphy P. Cardona1, F. Rubio1
showed bilateral enhancement, and minor salivary gland
1Neurology, 2Radiology, Hospital Universitari de Bellvitge,
biopsy, although atypical, showed a lymphocytic infiltrate. Barcelona, Spain
Immunological study was negative, including anti-SSa/SSb
antibodies. After exclusion of secondary causes and a P1900
rheumatologist consultation, SS was assumed, and
azathioprine plus corticosteroids initiated. Abstract cancelled
Conclusion: SNHL may be associated with auto-immune
disorders of CNS. In the presented case, the recurrent P1901
episodes of NSHL became the dominant symptom and thus,
a red-flag that led to a diagnosis review. Based on clinical Cerebral venous thrombosis as the initial
and imagiological features, SS was assumed, a known MS presentation of Behets disease: a case
mimic but with a different therapeutic approach. report
H. Ulvi, N. Aslan, R. Aygul, L. Ozel
Ataturk University, Medical Faculty, Department of
P1896 Neurology, Erzurum, Turkey
Abstract cancelled

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 429

P1902 P1907
Reversible hepatocerebral degeneration Neuromyelitis optica associated with
due to portovenous shunts: a case report primary Sjgrens syndrome
S. Gler1, U. Utku1, A. Tezel2, E. nl3 E. Papageorgiou, E. Papageorgiou, I.E. Markakis,
1Department of Neurology, 2Department of Gastroenterology, G. Gekas
3Department of Radiology, Trakya University, Faculty of
Neurology, General State Hospital of Nikaia, Greece
Medicine, Edirne, Turkey

P1908
P1903 Acute onset cervical myelopathy as first
Posterior reversible encephalopathy manifestation of late onset SLE (systemic
syndrome as presenting form of systemic lupus erythematosus)
sclerosis K. Tsatsou, M. Maria, P. Gouma, V. Myridaki,
M.I. Pedraza1, J. Barbado2, P. Mulero1, E. Rojo1, D. Kravaritis
L. Vega2, S. Herrero1, J. Meja1, C. de la Cruz1, Neurological Department, Evangelismos General Hospital,
A. Guerrero1, R. Fernndez1 Athens, Greece
Neurology Department, 2Internal Medicine Department,
1

University Hospital, Valladolid, Spain


P1909
P1904 Gluten sensitivity and ataxia
A. Ben Mahmoud, H. Derbali, M. Messelmeni,
Central pontine and extrapontine N. Ben Ali, H. Khiari-Mrabet, A. Mrabet
myelinolysis: a case report with Hopital Charles Nicolle, Tunis, Tunisia
assessment of cognitive function
N. Nei, S. Peji P1910
Clinical-Diagnostic Unit, Special Hospital on Addictions,
Belgrade, Serbia Abstract cancelled

P1905 P1911
Fahrs syndrome supports the Wernickes encephalopathy following total
involvement of basal ganglia in the gastrectomy for gastric neoplasm
pathogenesis of Schizophrenia: a case C. Fernandes, L. Pereira, P. Pereira, M. Rodrigues,
J. Proena
report Neurology Department, Hospital Garcia de Orta, Almada,
L. Fonseca1, D. Freitas2, S. Rocha3, . Machado3 Portugal
1 Psychiatry, Alto Ave Hospital Centre, Gumares,
2 Psychiatry, 3Neurology, Braga Hospital, Braga, Portugal
P1912
P1906 A neurological presentation of Prinzmetal
(variant) angina
Restless legs syndrome in haemodialysis
M. Hilli, L. Al Dhahir, W. Al Dhahir
patients: association with anxiety and Barking Havering and Redbridge Trust, London, UK
sexual life
S. Dikici1, A. Bahadir1, D. Baltaci1, H. Ankarali1,
N. Ercan1, M. Erdogan2
1 Duzce University, 2Duzce State Hospital, Dzce, Turkey

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


430 Posters, Sunday 9 September

P1913 P1916
The clinical significance of brain angio- Central and peripheral involvement in a
architectonic features in Alzheimers neurosarcoidosis case
disease S.M. Cnar1, F. Hz1, A. elik2, R. Karahan Ozcan2
D. Izyumov
1 Neurology, 2Taksim Training Hospital, Istanbul, Turkey
Khorezm Regional Multifield Medical Centre, Urgench,
Uzbekistan
P1917
Intracranial vasoreactivity alterations in
P1914 portal-systemic encephalopathy
Polyradiculoneuritis revealing Behets M.A. Sierra-Beltrn1, J.L. Romero-Flores2,
disease A. Torre-Delgadillo3, M. Uribe-Esquivel3
1CIDyT, Mdica Sur, 2The Medical School, Panamerican
I. Bedoui, A. Riahi, H. Khaled, G. Garsallah,
M. Mansour, J. Zaouali, R. Mrissa University, 3Gastroenterology Department, INCMNSZ,
Neurology, Military Hospital, Tunis, Tunisia Mexico City, Mexico

P1915
Analytic tetraparesis
R. Tojal, J. Campillo
Professor Fernando Fonseca Hospital, Amadora, Portugal

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 431

Neuro-ophthalmology/ -otology P1919


Drusen and AMD are associated with
P1918 reduced cognitive function: the Troms
Otolith-ocular responses in patients with study
acute brainstem lesions: ocular H. Lindekleiv1, M.G. Erke1, T. Peto2, K.-A. Arntzen1,
vestibular-evoked myogenic potentials H. Schirmer1, T. Wilsgaard1, G. Bertelsen1, E. Mathiesen1,
S.-Y. Oh1, J.S. Kim2, B.-S. Shin3, M.-W. Seo3 I. Njlstad1, Troms Eye Study Investigators
1University of Troms, Troms, Norway, 2NIHR Biomedical
1Department of Neurology, School of Medicine, Research
Institute of Clinical Medicine of Chonbuk National Research Centre for Ophthalmology, Moorfields Eye
University, Chonbuk National University Hospital, Jeonju, Hospital and University College London, UK
2Department of Neurology, Seoul National University Introduction: Drusen are accumulations of extracellular
Bundang Hospital, Seongnam, 3Department of Neurology, material found in Bruchs membrane of the eye. Drusen are
Chonbuk National University School of Medicine, Research a common finding in the ageing macula and are associated
Institute of Clinical Medicine of Chonbuk National with development of age-related macular degeneration
University-Chonbuk National University Hospital, Jeonju, (AMD). Studies suggest that drusen and AMD are associated
Republic of Korea with neurodegenerative diseases and reduced cognitive
Objective: The ocular vestibular-evoked myogenic function. Population-based studies on the relationship
potential (oVEMP), a recently documented otolith-ocular between AMD and cognitive functions are rare and have
reflex, is considered to manifest the central projections of only reported weak associations. Further studies are
the primary otolithic afferent fibres to the oculomotor warranted to examine the relationship between cognitive
nuclei. The aim of our study is to define oVEMP abnormality function, drusen and AMD.
in patients with acute brainstem lesions and to determine the Methods: We included 2,149 stroke-free participants from
brainstem structures involved in the generation of oVEMPs. the population-based Troms Study in Norway. Retinal
Methods: In response to air-conducted tone burst sound photographs were graded for presence of drusen and AMD.
(ACS), oVEMP was measured in 52 patients with acute Cognitive function was assessed by the word memory test
brainstem lesions. Individualized brainstem lesions were (short verbal memory), digit-symbol coding test (processing
projected to a standard brain mapping template for speed), and the tapping test (psychomotor tempo).
normalization and the probabilistic lesion maps were Results: We found significant decreased score on the digit-
constructed. symbol coding test for participants with soft drusen
Results: More than half (n=28, 53.8%) of the patients with (standardized =-0.15, 95% CI: -0.24 to -0.06) and for
acute brainstem lesions showed abnormal oVEMP. The participants with intermediate drusen (standardized
majority of patients with abnormal oVEMPs showed lesions =-0.20, 95% CI: -0.30 to -0.10). AMD was associated with
in the dorsomedial brainstem that contains the medial significant decreased score on the word memory test
longitudinal fasciculus, the crossed ventral tegmental tract (standardized =-0.26, 95% CI: -0.51 to -0.01).
and the oculomotor nuclei and nerves. Conclusions: The findings suggest that drusen deposition
Interpretation: The otolith-ocular pathway appears to be may share similar pathophysiology with reduced cognitive
located in the dorsomedial brainstem. Complemented to the function or that drusen deposition and reduced cognitive
cervical VEMP for the uncrossed otolith-spinal function, function may be different symptoms of the same underlying
oVEMP to ACS can be applied to evaluate the crossed disease process.
otolith-ocular function in the central vestibulopathy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


432 Posters, Sunday 9 September

P1920 P1921
Selective impairment of high acceleration Clinical characteristics of recurred benign
vestibulo-ocular reflex in spinocerebellar paroxysmal positional vertigo after
ataxia type 6 successful repositioning
J.S. Kim1, Y.E. Huh2, Y.J. Cho3, Y.S. Lee4 B.K. Kim1, K.S. Hong2, J.Y. Ahn3, J. Jung1, J.H. Park4
1Neurology, Seoul National University College of Medicine, 1Eulji University Hospital, 2Ilsan Paik Hospital, 3Seoul

Seoul National University Bundang Hospital, Seongnam-si, Medical Center, 4Paik Hospital, Seoul, Republic of Korea
2Neurology, Seoul National University Bundang Hospital,
Objectives: Benign paroxysmal positioning vertigo (BPPV)
Seongnam, 3Neurology, Ilsan Paik Hospital, Inje University is the most common cause of vertigo and usually easily
College of Medicine, Goyang, 4Neurology, Department of treated with repositioning manoeuvre. Although half of all
Neurology, Seoul National University and Metropolitan, patients eventually have a recurrence, the clinical
Boramae Hospital, Seoul, Republic of Korea characteristics of recurred BPPV were not studied yet.
Spinocerebellar ataxia type 6 (SCA6) may show various Methods: We reviewed the records of 1305 patients with
patterns of abnormal eye movements, primarily due to BPPV from Eulji BPPV registry between May, 1999 and
cerebellar dysfunction. Vestibulo-ocular reflex (VOR) January, 2012. Among 409 patients who experienced
abnormalities include reduced time constants, and increased recurrence during the follow-up period, typical nystagmus
or decreased gain of the VOR during rotation test. However, and vertigo were elicited by provoking manoeuvres (Dix-
selective impairment of high acceleration VOR has not been Hallpike test or roll test in the supine position) in 177
reported. A 63-year-old woman with a history of dizziness patients (34 men and 133 women, mean age was 5713
and imbalance for 15 years underwent evaluation of the years). We compared the involved side and canals of
VOR using bithermal caloric and rotary chair tests, and recurred cases with first attack.
head impulse test (HIT) using a magnetic search coil Results: Among 177 patients, 97 were posterior canal-
technique. She showed spontaneous downbeat nystagmus, BPPV and 80 were horizontal canal-BPPV (calalolithiaisis
gaze-evoked nystagmus in both horizontal and vertical 50, cupulolithiasis 30). The same side of ears was involved
directions, positional nystagmus, saccadic dysmetria, in 142 (79.8%) of 177 recurred patients. 47.2% of cases
impaired smooth pursuit, and positive bedside HIT in involved same canals as first one. The severity of symptoms
addition to dysarthria, dysmetria, and hypo-active deep of recurred cases was milder than in the first attack.
tendon reflexes. The results of bithermal caloric and rotation Conclusions: Most recurrences occurred on the same side
tests were normal, but the head impulse VOR gains were of ear, and recurred cases have less severe symptoms.
markedly decreased for all 6 semicircular canals when
measured using a magnetic search coil technique. Brain
MRI showed diffuse cerebellar atrophy, especially in the
vermal area. She was found to have SCA6 mutation. High
P1922
acceleration. VOR may be selectively impaired in SCA6. Abstract cancelled
Patients with SCA6 should have scrutinized evaluation of
vestibular function over the broad range of acceleration
stimuli.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 433

P1923 P1924
Geotropic central positional nystagmus The prognostic value of vestibular evoked
with hemorrhagic infarction in the myogenic potential in patients with lower-
territory of medial posterior inferior brainstem strokes
cerebellar artery S.-K. Mun
H. Oh1, S.J. Park2, D.-S. Oh3, S.-H. Lee3 Chung-Ang University Hospital, Seoul, Republic of Korea
1Department of Neurology, Gwangju Veterans Hospital, Objective: To find the relationship between the National
2Department of Neurology, Cheomdan Medical Center,
Institutes of Health Stroke Scale (NIHSS) and Vestibular
3Department of Neurology, Chonnam National University
Evoked Myogenic Potential (VEMP) results in central
Hospital, Gwangju, Republic of Korea origin dizziness patients according to status of recovery.
Background: Recurrent positional vertigo and/or Also, the usefulness of VEMP was evaluated as a tool for
nystagmus caused by cerebrovascular disease or other brain predicting recovery.
lesions, so called central positional paroxysmal vertigo Materials and methods: From September 2007 to August
(CPPV), could be encountered in clinical practice even 2010, a prospective study was done involving 18 patients
though the most common diagnosis of recurrent positional who were admitted - and later discharged after recovery -
vertigo syndrome is benign paroxysmal positional vertigo after being diagnosed with central origin dizziness.
(BPPV). To clinicians, the differentiation CPPV from BPPV Examination of neurologic deficits was performed by a
could be a critical issue. physician (a 2nd year Neurology resident) who was not
Case: A 55-year-old man visited our dizziness clinic with familiar with the research. The NIHSS was evaluated four
recurrent positional vertigo and headache. Neuro-otologic times every week, and VEMP was also performed
examinations including supine roll test revealed geotropic identically on both sternocleidomastoid muscles. Both
positional nystagmus. Even though the patient had values were compared and analyzed.
unsteadiness, the initial diagnosis was BPPV involving the Results: In VEMP results according to recovery stages in
lateral semicircular canal. Several repositioning manoeuvres central origin dizziness patients, there was a decrease in
failed to remove the symptoms and signs. Unresponsiveness latency and asymmetry of p13, but there was no difference
to the repositioning manoeuvre, combined with unsteadiness in latency and difference of latency of n23. Upon comparing
and headache led to check brain images. Brain MRI both results, p13 and the level of asymmetry decreased as
revealed acute infarction with hemorrhagic transformation the value of the NIHSS decreased according to recovery of
in the territory of the medial posterior inferior cerebellar symptoms. Correlation between the two groups was
artery, involving the right inferomedial cerebellum statistically significant (p<0.05).
including nodulus. The quality of vertigo and the geotropic Conclusion: Compared to the NIHSS, results of VEMP
nystagmus had improved slightly, but those symptoms were allow for objective evaluation of symptom recovery as well
persistent for the 2-year follow-up period. as consistent results and also may be useful in clinical
Conclusion: It has been known that central positional practice due to its safety.
nystagmus is mostly downbeat or apogeotropic pattern, but
geotropic nystagmus can manifest as a central positional
nystagmus. The most common cause of geotropic nystagmus
is BPPV involving the lateral canal; however, persistent
geotropic nystagmus even after proper repositioning
manoeuvre and combined neurologic signs can point to the
central etiology. In this case, the irritative hemorrhagic
cerebellar lesion involving the nodulus might be a cause of
geotropic central positional nystagmus.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


434 Posters, Sunday 9 September

P1925 P1926
Post-infectious optic neuritis associated Pure upbeat nystagmus in association
with scrub typhus with bilateral internuclear
H.-J. Cho1, K.-D. Choi1, J.-H. Choi2, S.-H. Kim3 ophthalmoplegia
1Neurology, Pusan National University Hospital, Busan, J.-H. Choi1, H.-J. Cho2, S.-H. Kim3, K.-D. Choi2,
2Neurology, Pusan National University Yangsan Hospital, J.S. Kim4
Yangsan, 3Neurology, Dong-A University Hospital, Busan, 1Department of Neurology, Pusan National University
Republic of Korea Yangsan Hospital, Yangsan, 2Department of Neurology,
Introduction: To describe a patient who developed post- Pusan National University Hospital, 3Department of
infectious optic neuritis associated with scrub typhus. Neurology, Dong-A University Hospital, Pusan, 4Department
Methods: An 8-year-old boy was hospitalized for fever, of Neurology, Seoul National University Bundang Hospital,
chills, myalgia, and multiple maculopapular rashes. He was Bundang, Republic of Korea
noted to have an eschar in his body trunk. Extensive testing Background and significance: Upbeat nystagmus occurs
for infectious etiology was negative except for scrub typhus in ventral pontine tegmental lesions affecting the ventral
antibody titre of 1:5120. Fever, chills, and myalgia rapidly tegmental tract carrying signals for upward vestibulo-ocular
resolved after doxycycline treatment. Two weeks later, the reflex (VOR), and in caudal medullary lesions involving the
patient developed painless visual loss in the bilateral eyes. nucleus of Roller, one of the perihypoglossal nuclei which
Results: Upon neurological examination, visual acuity was might contribute to vertical oculomotor integration.
counting fingers at 1 meter in the right eye and he was However, pure upbeat nystagmus in straight ahead position
unable to perceive the light in the left eye. Fundoscopy of gaze has not been described in association with bilateral
revealed optic disc oedema in bilateral eyes. Gadolinium- internuclear ophthalmoplegia (INO) from pontine tegmental
enhanced T1-weighted MRI of the orbit showed bilateral lesion involving the medial longitudinal fasciculus (MLF).
enhancement of the optic nerves indicative of bilateral optic We report pure upbeat nystagmus in a patient with bilateral
neuritis. He was treated with intravenous methylprednisolone INO due to dorsomedial pontine tegmental lesions.
800mg for 5 days, followed by a tapering course of oral Case report: A 66-year-old man developed horizontal
prednisolone. Two months after the onset of his visual diplopia and oscillopsia for three weeks. Examination
symptoms, visual acuity was improved completely. showed bilateral INO and upbeat nystagmus with fixation,
Conclusion: We concluded that the patients optic neuritis which disappeared in darkness. It was enhanced during
was likely associated with the scrub typhus, which has not upgaze and convergence, and decreased in downward gaze.
been reported previously. The timing of visual loss may Vertical head impulse tests were normal. Video-oculography
support an immunological mechanism of scrub typhus- disclosed pure upbeat nystagmus with exponentially
associated optic neuritis. Treatment of post-infectious optic decreasing slow phases. Brain MRI showed enhancing
neuritis with high-dose corticosteroid appears effective in lesions involving bilateral dorsomedial pons extending
our patient. from middle to upper portion. Upbeat nystagmus and
bilateral INO improved with intravenous dexamethasone
administration.
Conclusions: Upbeat nystagmus in our patient may be
attributed to damage of the cell groups of the paramedian
tracts (PMT) or the projections from the interstitial nucleus
of Cajal (INC) to PMT, or disruption of connections
between INC and the nucleus of Roller.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 435

P1927 P1928
Vertigo and psychological distress Intact retinal ganglion cells and visual
M. Mulazzani, C. Mller pathways in patients with progressive
Medical University of Vienna, Austria external ophthalmoplegia due to common
Introduction: With a life time prevalence of 10.3% of the mtDNA deletion
general population, vestibular vertigo is a widespread E.T. Varga1, F. Naghizadeh2, B. Bereznai1, P. Balicza1,
symptom. Furthermore psychiatric disorders are present in V. Remnyi1, M.J. Molnar1, G. Holl2
nearly half of the patients presenting at specialized units for 1Clinical and Research Centre for Molecular Neurology,
dizziness. Therefore the importance of further elucidating Department of Neurology, Semmelweis University,
the connections between vertiginous and psychiatric 2Department of Ophthalmology, Semmelweis University,
symptoms becomes clear. Budapest, Hungary
Methods: 137 healthy adults completed two questionnaires:
Introduction: Mitochondrial (mt) disorders are metabolic
the Symptom-Checklist-90-Revised (SCL-90-R) and the
conditions with multi-organ involvement, often presenting
Vestibular Symptom Scale (VSS). Additional Information
neuro-ophthalmological symptoms, too. The aim of the
on patient history and socio-demographic factors was
study was to investigate the relation between progressive
gathered and assessed in a multivariate regression analysis.
external ophthalmoplegia (PEO), involvement of visual
Results: The global severity of psychological distress (GSI)
pathway and mitochondrial DNA (mtDNA) mutations.
was positively correlated to the self-reported severeness of
Methods: 5 female patients (age between 38 and 54 years),
vertigo and related symptoms (p<0.001), just as each of
suffering from progressive external ophthalmoplegia and
the SCL-90-Rs subscales (p<0.001 for somatisation to
harbouring the common mtDNA deletion were investigated.
0.18 (p=0.039) for psychoticism). Subjects holding a
All eyes underwent a detailed standard clinical evaluation
university degree reported lower psychological distress
via dilated pupil. Visual functions were measured with
compared to subjects with a lower educational level (GSI
determination of best-corrected visual acuity (BCVA),
means: 0.31 vs. 0.42; p=0.019). Women reported higher
standard automated threshold perimetry and VEP. The
levels of depression than men (0.59 vs. 0.41; p=0.008).
quantity of the retinal ganglion cells was measured with
Subjects who declared any kind of disease during the last
Fourier-domain optical coherence tomography; axonal
twelve months scored higher on both vertiginous symptoms
integrity of the retinal ganglion cells was assessed with
and psychological distress (VER means: 0.21 vs. 0.14;
scanning laser polarimetry. Blood samples were screened
p=0.023; GSI means: 0.43 vs. 0.28; p<0.001).
for the most common mtDNA mutations.
Conclusions: These results indicate a clear connection
Result: Neurological investigation revealed PEO with
between psychological distress and vertigo. The existence
variable degree. In 4 of the 5 patients, normal retinal
of psychopathological symptoms aggravates the subjective
ganglion cell thickness, retinal nerve fibre layer thickness
severity of vertigo, and vice versa. This study adds to the
and distribution were found with values within the age-
growing evidence of the bidirectional link between these
related normal range, for both eyes. Visual acuity, threshold
two symptom entities.
perimetry and VEP provided results without definite
pathological changes. Genetic analysis of the mtDNA
revealed the common deletion. One patient had maculopathy
due to vitreoretinopathy unrelated to mtDNA deletion, and
decreased BSCA on both eyes.
Conclusion: In patients younger than 50 years with
progressive external ophthalmoplegia due to the common
mtDNA deletion, the retinal ganglion cells and the visual
pathway may remain intact.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


436 Posters, Sunday 9 September

P1929 P1930
Vestibular function test of dizziness Prevalence of migraine in low tension
patients by vestibular evoked myogenic glaucoma patients
potential M. Abu-Hegazy1, M. Saad1, W.M. Dessouky2
1Neurology, 2Ophthalmology, Mansoura University,
G.-H. Lee
Neurology, Medical College, Dankook University, Cheon-An, Mansoura, Egypt
Republic of Korea Introduction: Some authors reported a significant
Background and objectives: Since the saccule is a frequent relationship between migraine and low tension glaucoma.
site of hydrops formation, we evaluated whether vestibular Objective: To investigate the prevalence of migraine in
evoked myogenic potential (VEMP) responses can reflect patients with low tension glaucoma and to evaluate MRI
the diagnosis and the stage of Menires disease. And we and EEG abnormalities in low tension glaucoma
also studied other vestibular diseases such as vestibular migraineurs.
neuritis and benign paroxysmal positional vertigo. Methods: A prospective study conducted on 40 controls
Materials and methods: Retrospectively, we analyzed the and 39 patients with low tension glaucoma in Mansoura
results of VEMP in 42 patients (20 men and 22 women) Ophthalmology Center diagnosed by fundus examination,
with unilateral definite Menires disease, 21 patients (10 tonometery, and full threshold perimetry. A standardized
men and 11women) with vestibular neuritis, and 21 patients questionnaire was used to indicate migraine positives. Brain
(10 men and 11 women) with benign paroxysmal positional MRI and EEG were done for migraineurs in the control and
vertigo (BPPV). All subjects underwent VEMP testing patient groups.
using ipsilateral 1 KHz-tone burst sound with 105 dB nHL. Results: Migraine was more common in patients with low
Results: VEMP was present in 85% of Menires affected tension glaucoma than in the control group (p=0.03). In
ear. The latency of p13 of affected ears in patients with patients with low tension glaucoma, females were associated
Menires disease, vestibular neuritis (VN), BPPV was not with higher prevalence of migraine than males (20.5 vs.
significantly prolonged than that of normal ears in the 10.3%). Patients with age group ranging from 20 to 25 years
control group except left n23 latency in Menires disease. had a higher incidence of migraine than other age groups.
In patients with Menires disease, the amplitude-ratio was Of the low tension glaucoma migraineurs, 16.7% and 33.3%
statistically (p=0.006) larger than that of the control group. had abnormal brain MRI and EEG, respectively.
Relationship was found in amplitude ratio among groups Conclusion: Migraine is more common in low tension
classified by the stage of Menires disease. glaucoma patients than in controls. It is also more prevalent
Conclusion: This study shows that amplitude ratio of in low tension glaucoma female gender and those with age
VEMP response is a useful method to determine the severity ranging from 20 to 25.
and prognosis of Menires disease. We recommend VEMP
to quantitatively explain to the patient the severity of
Menires disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 437

P1931 P1932
Transient supranuclear gaze palsy Non-traumatic carotid-cavernous fistula
associated with persistent primitive combined with acute brain stem infarction
trigeminal artery resulting in hemiparesis
Y.H. Yun Y.H. Jung
Gwangmyeong Sungae General Hospital, Gwangmyeong-si, Neurology, Chang Won Fatima Hospital, Chang Won,
Republic of Korea Republic of Korea
Primitive trigeminal artery is the most frequent embryonic Background: Carotid-cavernous fistula (CCF) is abnormal
communication between the carotid and vertebrobasilar arteriovenous communications between the carotid and
system with the estimated incidence of 0.1-1.0%. We report cavernous sinus. Direct CCF results from a tear in the
a case presented with recurrent supranuclear vertical gaze intracavernous carotid artery, usually due to trauma or
palsy related to PTA as a symptom of vertebrobasilar intervention. Indirect CCF generally occurs spontaneously
insufficiency. A 57-year-old man was admitted to our and shows more subtle signs. CCF is usually combined with
hospital because of recurrent vertical diplopia for 7 days. aneurysm or trauma. CCF is relatively rare, but may show
On admission he was alert, but presented with conjugated progressive, diverse symptoms such as decreased visual
gaze palsy. In the neuro-ophthalmologic examination, the acuity, exophthalmosis, ophthalmoplegia, and cerebral
vertical vestibulo-ocular reflex and Bells phenomenon was ischemia due to increased venous pressure. However,
preserved. There were no accompanying neurologic deficits hemiparesis has been rarely reported. We report a case of
such as cranial nerve palsies and cerebellar dysfunction. His CCF combined with multiple brainstem infarctions causing
ocular finding prolonged only for 5 hours then disaffeared hemiparesis.
thereafter. We could not find any evidence of acute ischemic Case report: A 74-year-old woman visited our hospital
lesion on the magnetic resonance (MR) imaging. MR with a chief complaint of gait disturbance, headache, and
angiography and digital subtraction angiography, however, repeated vomiting during 10 days. She had a medical history
showed persistent anastomosis from the cavernous segment of diabetes, hypertension, and hyperlipidemia. She had no
of right internal carotid artery (ICA) to the proximal portion traumatic history. A neurological examination revealed mild
of basilar artery (BA). As expected, distal portion of BA dysarthria, right exophthalmos with conjunctival oedema
was hypoplastic. Vertical gaze palsy can be caused by a and ophthalmoplegia, deceased visual acuity of the right
lesion involving the thalamus, rostral interstitial nuclei of eye, left hemiparesis (MRC Grade III) and decreased
medial longitudinal fasciculus, or posterior commissure. pinprick sensation of left upper and lower limbs. Blood tests
Hypoplastic BA might cause transient ischemic symptoms. showed elevated level of total cholesterol (343mg/dl) and
Previous reports dealing with persistent PTA suggested that erythrocyte sedimentation rate(ESR) (30mm/hr). Random
the mechanism of ischemia in the posterior circulation be serum glucose level was elevated to 265mg/dl. Other
embolism from the ICA stenosis or in-situ thrombosis in the studies were within normal limits. A brain MRI and MR
PTA itself. In contrast to the previous studies, our case angiography showed a recent infarction on the right pontine
could show that ischemic symptoms localized to the and medulla oblongata and fistula of the right carotid and
mesodiencephalon might be caused by hemodynamic cavernous sinus.
insufficiency from hypoplastic BA. Conclusion: CCS is usually combined with aneurysm or
trauma, and hemiparesis has rarely been reported. We report
a case of non-traumatic, no aneurysmal CCF combined with
hemiparesis.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


438 Posters, Sunday 9 September

P1933 P1934
Calycopterin induces mitochondrial Central retinal artery obstruction (CRAO) -
biogenesis and autophagy in PC12 clinical and colour Doppler imaging (CDI)
neuron-like cells exposed to H2O2 characteristics
N. Namazi Sarvestani, F. Khodagholi D.C. Jianu1, S.N. Jianu2, L. Petrica3, S.M. Deme4,
Neuroscience Research Center, Tehran, Iran D.F. Muresanu5, S.K. Calomfirescu5, M. Petrica1
1Neurology, Victor Babes University of Medicine and
Introduction: An increase in the intracellular levels of anti-
oxidant agents, and at the same time the removal of already Pharmacy, County Emergency Hospital, 2Ophthalmology,
damaged components, are both part of the oxidative stress Military Emergency Hospital, 3Internal Medicine-
response. Flavones have been considered as one of the Nephrology, Victor Babes University of Medicine and
antioxidants. We determine the neuroprotective effects of Pharmacy, County Emergency Hospital, Timisoara,
4Neurology, West University, Arad, 5Neurology, University of
calycopterin as natural flavones, on H2O2-treated PC12
neuronal cells. Medicine and Pharmacy, Cluj-Napoca, Romania
Methods: PC12 cells treated with 25, 50 and 100M of Introduction: CRAO represents an abrupt diminution of
calycopterin for 3h, followed by adding H2O2 (150M). blood flow through the CRA severe enough to cause
Apoptosis was assessed by MTT test, acridine orange/ ischemia of the inner retina.
ethidium bromide and Hoescht staining and the autophagy Purpose: To assess the role of CDI in the etiological
was determined by Mono Dansyl Cadaverin and acridine diagnosis of CRAO.
stain. Inflammation, ER stress, mitochondrial biogenesis Patients and methods: 4 patients with clinical suspicion of
and autophagy factors were measured by Western blot unilateral CRAO were examined following a protocol
method. including CDI of orbital vessels.
Results: We found that calycopterin protects differentiated Results: They had no emboli visible on ophthalmoscopy.
PC12 cells by inhibiting caspase-dependent pathway of The first patient had no blood flow signal on CDI on a
apoptosis. Calycopterin could decrease ER stress by surface of 2 millimetres behind the left optic disc. B-scan
decreasing calpain and caspase-12 levels. These inhibitions ultrasound evaluation found a small round, moderate
were along with stabilization of Nrf2, phosphorylation of reflective echo within the left optic nerve, 2 millimetres
CREB and decrease of NF-KB levels. The level of behind the optic disc. Left ICA ultrasound examination
inflammatory factors such as NF-KB, TNF and COX-2 found an ulcerated ateromatous plaque, being the source of
was also decreased by calycopterin. Interestingly, cholesterol emboli. The second patient had no detectable
calycopterin promotes mitochondrial biogenesis through flow in the right CRA, due to multiple reverberations
increase of PGC1, NRF1 and TFAM. We also measured determined by calcic arterial emboli, placed 5 millimetres
important factors involved in autophagy such as LC3B, behind the right optic disc. The angio-CT showed an
Atg-7, Atg-12 and P62. Calycopterin could increase unstable aortic plaque, considered as the source of the
autophagy in order to protect PC12 neuronal cells from emboli. The third patient had characteristic CDI findings for
death. Horton disease: low blood velocities, especially end-
Conclusions: We provided documentation of diastolic velocities, and high RI in all retrobulbar vessels, in
neuroprotective effect of a natural flavone, calycopterin, both orbits (severe diminished blood flow velocities in the
against H2O2-induced oxidative stress in differentiated CRA, especially on the affected side). The diagnosis was
PC12 cells by modulating the level of transcription factors, sustained by temporal arteries ultrasound and biopsy. The
increasing the level of antioxidant factors and promoting last patient had very low blood flow velocities in the left
biogenesis of mitochondria and autophagy. CRA, due to an acute left ICA occlusion.
Conclusions: Ultrasound investigation is a valuable
diagnostic tool for identifying potential systemic conditions
associated with CRAO.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 439

P1935
Ophthalmoplegia without ataxia: a case P1938
report Visual evoked potentials in patients with
A.C. Sarilar, M.C. Akbostanci, C. Togay Isikay diabetic neuropathy
Neurology, Ankara University, Ankara, Turkey H. Ulvi, U. Avar, Z. Canseve, Z. Avar, R. Demir,
Acute ophthalmoplegia without ataxia (AOWA) is an L. zel, R. Aygul
extremely rare disease in which acute impairment of ocular Departments of Neurology and Family Medicine and
movements is usually the sole clinical manifestation. We Medical Education, Faculty of Medicine, Atatrk University,
report a patient with isolated acute ophthalmoplegia and Erzurum, Turkey
anti-GQ1b IgG antibody in CSF whose treatment with
intravenous immunoglobulin (IVIg) led to clinical recovery P1939
within four weeks. Case was a 68-year-old woman with
diplopia, bilateral blepharoptosis and dizziness that started Acute myopia induced by administration
one week ago. She had diarrhoea just before her complaints. of 25mg/day single-dose topiramate:
Examination revealed bilateral incomplete ptosis and total a case report
ophthalmoplegia. Pupillary reaction to light was decreased
E. Vardarli1, T. Kocaoglu2, H. Kocaoglu2, D. Mete Uskay3
bilaterally. Other findings like orbital and cranial MRIs 1 Neurology, 2Ophthalmology, Ozel Tinaztepe Hastanesi,
were normal. Biochemistry, blood count, sedimentation, 3 Solo Practice, Izmir, Turkey
C-reactive protein, thyroid hormones, glycolysated
haemoglobin, and thiamine levels were normal. ANA,
ENA, ANCA, anti-dsDNA, anti-acetylcholine receptor P1940
antibodies, HIV, and VDRL was negative. CSF protein and
glucose levels were normal, and there were no cells. Anti
Abstract cancelled
GQ1b IgG antibodies were positive in CSF. Neostigmine
test was negative. Nerve conduction studies were normal. P1941
AOWA was considered and IVIg 0.4g/kg/d for five Neuro-ophthalmologic examination of
consecutive days was started. In the fourth week
examination was normal besides bilateral slight ptosis. stroke patients
AOWA has been designated as an atypical form of Miller- H.H. Bakunts, G.H. Silvanyan, L.K. Egiyan,
Fisher syndrome. The new diagnostic criteria for AOWA, K.F. Baghdasaryan
fulfilled by the presented patient, include acute or subacute Angioneurology, Yerevan State Medical University after
onset of external/internal ophthalmoplegia, absence of other Mkhitar Heratsi, Yerevan, Armenia
neurological symptoms, presence of antiGQ1b IgG
antibodies and exclusion of other identiable causes. Cases
of AOWA successfully treated with IVIg have been
P1942
reported. The disease can have a good prognosis without Abstract cancelled
treatment, though the time to complete recovery is longer.
P1943
Non-invasive alternating current
P1936 stimulation improves vision after
Isolated cranial neuritis due to paranasal traumatic injury of the optic nerve
sinusitis: two case reports A. Fedorov1, Y. Chibisova2, B.A. Sabel1, C. Gall1
C. Angelopoulou1, E. Mavraki1, S. Boutzoni1, 1Institute of Medical Psychology, Otto-von-Guericke

M. Flamouridou1, S. Stathakidou2, O. Tsirogianni2, University of Magdeburg, Germany, 2Department of


M. Riga2, M. Katotomichelakis2, D. Dardabounis3, Neurology, Mechnikov Medical Academy, Saint Petersburg,
S. Lamprakopoulos1 Russia
1Department of Neurology, 2Department of Otolaryngology,
3Department of Ophthalmology, University of Thrace,
Alexandroupolis, Greece P1944
Unusual cause of ptosis
P1937 F. Tokucoglu1, A. Ozdemir2, B. Ozer2
1Ataturk University Department of Neurology Research and
Clinical analysis of hearing loss due to Training Hospital, 2Ataturk Research and Training Hospital,
spontaneous intracranial hypotension Izmir, Turkey
S.-K. Mun
Chung-Ang University Hospital, Seoul, Republic of Korea

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


440 Posters, Sunday 9 September

Neurotoxicology / occupational P1946


neurology Age- and size-dependent neurotoxicity of
engineered nanoparticles from metals
P1945 A. Sharma1, D.F. Muresanu2, R. Patnaik3, H.S. Sharma1
1Surgical Sciences, Anaesthesiology & Intensive Care
Neuroprotective profile of sesamol and
Medicine, Uppsala University, Uppsala, Sweden,
quercetin in experimental paradigm of 2Neurosciences, University of Medicine and Pharmacy Cluj-

QA-induced neurotoxicity: inhibition of Napoca, Cluj-Napoca, Romania, 3Biomaterials, School of


nitrodative-inflammatory cascade Bioengineering, Banaras Hindu University, Institute of
Technology, Varanasi, India
A. Kuhad, S. Singla, V. Arora, K. Chopra
University Institute of Pharmaceutical Sciences, Panjab Chronic administration of engineered nanoparticles from
University, Chandigarh, India metals, e.g., Cu, Ag, or Al (50-60nm, 50mg/kg, i.p. daily for
Introduction: Quinolinic acid (QA), a well known 1 week) induces blood-brain barrier (BBB) disruption, brain
excitotoxin that produces a pharmacological model of oedema formation and brain pathology in rats (age 18 to 22
Huntingtons disease in rats and primates, has been shown weeks). This was most pronounced in Ag followed by Cu
to evoke degenerative events in nerve tissue via NMDA and Al indicating metal specificity in neurotoxicity.
receptor overactivation and oxidative stress to exert its However, size and age dependent neurotoxicity of
neurotoxic actions. nanoparticles are still not well known. In this investigation,
Methods: Rats were intrastriatally administered quinolinic three different sizes of Cu, Ag or Al nanoparticles (20 to
acid and were treated with sesamol (4, 8 and 16mg/kg, i.p) 30nm; 50 to 60nm, or 130 to 150nm) were administered (50
and quercetin (25, 50 and 100mg/kg, i.p) for 14 days before mg /kg, i.p.) in 3 different age groups of rats (9 to 10 weeks;
and 14 days after quinolinic acid administration, with these 18 to 20 weeks or 30 to 35 weeks old). In these animals,
natural antioxidants. breakdown of the BBB to Evans blue albumin (EBA) and
Results: Intrastriatal injection of QA leads to increased radio-iodine, brain water content, neuronal injury, astrocytic
escape latency, impaired locomotor activity, as well as activation, myelin damages were examined using standard
significant increase in immobility time in forced swim test, procedures. We found that brain pathology caused by
This behavioural deficit was integrated with the increased different nanoparticles was inversely related to their sizes.
nitrodative stress markers (increased lipid peroxidation, Thus, smaller nanoparticles from Ag, Cu or Al induced most
raised nitrite concentration and depletion of endogenous pronounced BBB breakdown, brain oedema formation and
anti-oxidants such as catalase, superoxide dismutase and neuronal injuries, glial fibrillary acidic protein (GFAP)
reduced glutathione) along with the significant increase in upregulation as well as myelin vesiculation in youngest
TNF- levels in rat brain suggesting QA mediated oxidative animals. The oldest animals also showed massive brain
and neuro-inflammatory damage. Moreover intrastriatal pathology. The Ag and Cu exhibited greater brain damage
administration of QA resulted in significant decrease in the compared with Al nanoparticles in all age groups regardless
levels of dopamine, serotonin and norepinephrine in the rat of their sizes. The young and elderly age groups exhibited
forebrain. Chronic treatment with sesamol and quercetin greater neurotoxicity to nanoparticles indicating that
attenuated these behavioural, biochemical and perhaps children and elderly are more vulnerable to
neurochemical alterations in the rat brain and these effects nanoparticle-induced brain damage.
were attributed to their strong anti-oxidant and anti-
inflammatory potential.
Conclusions: Conclusively it is suggested that major
features of QUIN-induced neurotoxicity are mediated by
nitrodative stress induced neuro-inflammation and the
neuroprotective role of sesamol and quercetin should be
explored further as effective agents in the management of
Huntingtons disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 441

P1947 P1948
Engineered nanoparticles Ag, Cu and Al Very long chain fatty acid-induced cell
(50-60nm) induce oxidative stress, death with autophagic characteristics on
neuronal nitric oxide synthase oligodendrocytes
upregulation and brain pathology. A. Vejux, K. Ragot, M. Doria, G. Lizard
Neuroprotection by Insulin-like growth Laboratoire Bio-PeroxIL - Biochimie du Peroxysome,
factor1 Inflammation et Mtabolisme Lipidique, Universit de
Bourgogne - Facult des Sciences Gabriel, Dijon, France
A. Sharma1, D.F. Muresanu2, R. Patnaik3, J.V. Lafuente4,
X-linked adrenoleukodystrophy (X-ALD), a peroxisomal
H.S. Sharma1
1Surgical Sciences, Anaesthesiology & Intensive Care leukodystrophy characterized by ABCD1 deficiency, is
Medicine, Uppsala University, Uppsala, Sweden, associated with elevated plasmatic and tissue concentrations
2Neurosciences, University of Medicine and Pharmacy Cluj- of very long chain fatty acids (VLCFA: C24:0, C26:0). As
Napoca, Cluj-Napoca, Romania, 3Biomaterials, School of some dead cells were observed on histological tissue
Bioengineering, Banaras Hindu University, Institute of sections from the brain of X-ALD patients, we determined
Technology, Varanasi, India, 4Neurosciences, Lab Clinical & the type of cell death induced by VLCFA on oligodendrocytes
Experimental Neurosciences (LaCEN), University of Basque (myelin synthesizing cells). Murine oligodendrocytes cells
Country, Bilbao, Spain (158N) were treated for 24-48h with C24:0 or C26:0 (10-
20M) or (C24:0 + C26:0) (5M each). The impact of
The possibility that chronic exposure of nanoparticles
VLCFA on cell viability was evaluated by flow cytometry
causes blood-brain barrier (BBB) breakdown and brain
with propidium iodide (dead cell identification), DiOC6(3)
pathology by inducing oxidative stress and/or nitric oxide
(m measurement) and acridine orange (lysosomal
production was examined in a rat model. Rats treated with
integrity). No apoptotic cells were found. As some
Ag, Cu or Al nanoparticles (50mg/kg, i.p. once daily for 7
lysosomes were observed, autophagy was evaluated by
days) were tested for BBB permeability, brain oedema,
studying LC3 expression and localization by Western
neuronal nitric oxide synthase (nNOS) immunoreactivity
blotting (LC3-II identification as autophagic marker) and
and brain oxidant levels, e.g., myeloperoxidase (MP),
immunofluorescence (transfection with GFP-LC3). On
malondialdehyde (MD) and glutathione (GT) on the 8th day
158N cells, C24:0 and C26:0 alone and combined are able
as compared to saline controls. Cu and Ag but not Al
to induce cell death (loss of cell viability, decrease m,
nanoparticles increased the MP and MD levels by 2-fold in
altered lysosomal integrity) and induce an activation of
the brain, although GH showed 50% decline. At this time
autophagy (presence of LC3-II, punctuated localization of
increase in brain water content and BBB breakdown to
LC3). Thus, VLCFA are able to trigger an autophagic form
protein tracers were seen in areas exhibiting nNOS positive
of cell death in 158N cells. It remains to define whether this
neurons and cell injuries. Pre-treatment with insulin-like
autophagic process can be amplified by ABCD1 deficiency,
growth factor-1 (IGF-1) in high doses (1g/kg,i.v., but not
and whether it contributes to cell death or constitutes a
0.5g/kg daily for 7 days) together with nanoparticles
defence mechanism.
significantly reduced the oxidative stress, nNOS
upregulation, BBB breakdown, oedema formation and cell
injuries. These novel observations demonstrate that (i)
nanoparticles depending on their constitution (Cu, Ag but
not Al) induce oxidative stress and nNOS expression
leading to BBB disruption, brain oedema and cell damage,
and (ii) IGF-1 depending on its doses exerts powerful
neuroprotection against nanoneurotoxicity, not reported
earlier.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


442 Posters, Sunday 9 September

P1949 P1950
Prevalence of alcohol use among seizure Fatal outcome in acute Marchiafava-
patients admitted to the emergency room Bignami in relation to pure alcohol
A. Fedulau1, M. Leone2, A. Ivashynka2, D. Kuzmin1 consumption
1State Medical University, Minsk, Belarus, 2Clinica H. Nzwalo1, B. Banjai2, J. Drago3, F. Ferreira1, J. Soleiro1
Neurologica Maggiore della Carit Hospital, Novara, 1Neurology, 2Internal Medicine, 3Radiology, Faro Hospital
Italy EPE, Faro, Portugal
Both epilepsy and alcohol misuse represent a frequent cause Introduction and methods: Marchiafava-Bignami disease
of admission to the Emergency Room (ER). The number of (MBD) is the symmetrical demyelization of the corpus
people abusing alcohol is increasing in Belarus and callosum rarely seen in chronic alcoholics. There is no
represents a serious public health problem. A history of specific clinical feature of MBD. The diagnosis is based on
alcohol misuse or dependence among patients with seizures the association of clinical and typical Brain MRI findings.
is at least twice as frequent as in the general population in We describe a case of MBD after a relatively short period of
Western European countries. No information is available massive pure alcohol consumption.
for Belarus and most of other east European countries. Results: A 30-year-old woman, was admitted to hospital
Objective: To study the frequency of alcohol history among because of acute confusional state. She had been binge
patients admitted with seizure to the emergency department drinking for one year, but 4 weeks before admission she
of a general hospital in Belarus. started with massive daily intake (250-300ml) of
Materials and methods: Clinical charts of all the commercial pure alcohol. She was disoriented, agitated,
admissions to the ER of the 9th University Hospital in with generalized hypertonicity, and bilateral Babinski sign.
Minsk during one year were evaluated. Data on medical There were no asymmetries or meningeal signs. The
history, general and neurological examination, and extensive laboratory tests were within normal values with
laboratory investigations were collected. exception for mild hypochromic macrocytic anaemia. The
Results: Among 31,658 patients admitted to the emergency electro-encephalogram showed diffuse lentification of
department during 2011, a seizure was diagnosed in 1504 cerebral activity. The brain MRI on T2W, DWI and FLAIR
(4.8%). Two-hundred-fifty-one seizure patients (14%) had images showed diffuse hypersignal of corpus callosum,
a history of alcohol abuse: 35 presented with a new-onset more intense in the splenium, without water restriction. On
seizure, 74 had previous seizures and for 141 without data T1W and on ADC images the lesion appeared hypo-intense.
of epileptic history. Associated pathology included brain On the basis of the history of alcohol abuse in association
injury of varying severity (32 patients), and stroke (1). None with the imaging findings the diagnosis of MBD was made.
of the patients EEG showed focal lesions. She started on supportive measures, corticosteroids, high-
Conclusions: Patients with seizures and a history of alcohol dose intravenous vitamin B complex, including 1000mg/
represent an important burden of neurological emergency. day thiamine, but her condition did not improve. She died a
The prevalence of alcohol abusers among seizure patients week later.
was similar to Western European countries. However, Conclusion: This case of MBD is atypical because of the
ascertainment of a history of alcohol abuse may be preceding short time of alcohol abuse and also the
challenging in the ER because patients often hide their association with pure alcohol consumption which has never
alcoholic history. been described before.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 443

P1951 P1952
Distinct molecular mechanisms are Inhibitory effect of the platelet activating
involved in SK-N-MC cell death in factor on the apoptosis of differentiated
response to hydrogen peroxide and PC 12 cells
superoxide anion D.E. Kim, J.H. Han
M. Moslehi, R. Yazdanparast Neurology, Korea Veterans Hospital, Seoul, Republic of
University of Tehran, Institute of Biochemistry and Korea
Biophysics, Tehran, Iran Objective: Platelet activating factor (PAF) activation
Aims: There is an increasing body of evidence indicating reveals a variable effect on cell death induced by various
that oxidative stress plays a vital role in the pathogenesis of toxic insults. We assessed the PAF effect on MPP+ toxicity
neurodegenerative diseases. Nerve cells are incessantly in relation to the apoptotic process.
exposed to environmental stresses leading to overproduction Methods: The effect of PAF on the MPP+ toxicity was
of some harmful species like reactive oxygen species assessed in PC12 cells differentiated by the nerve growth
(ROS). ROS including hydrogen peroxide and superoxide factor. To assess the MPP+ treatment-induced apoptosis and
anion are potent inducers of various signalling pathways clarify the inhibitory effect of PAF on MPP+ toxicity, we
encompassing MAPKs and JAK-STAT pathways. JNK/p38 investigated the effect of PAF on the nuclear morphological
MAPKs, JAK2 and STAT3 are deemed stress-responsive changes observed in the MPP+-treated cells. The MPP+-
factors involved in oxidative stress-induced cell death. We induced apoptosis was assessed by measuring change in the
scrutinized the effects of hydrogen peroxide and superoxide apoptotic protein levels. We evaluated the effect of PAF on
anion on SK-N-MC neuroblastoma cells to elucidate the the MPP+-induced cytochrome-c released and caspase-3
mechanism by which each oxidant modulated above- activation by performing the ELISA-based quantitative
mentioned pathways leading to SK-N-MC cell death. analysis.
Methods: SK-N-MC cells were exposed to hydrogen Results: PAF exhibited differential effect against MPP-
peroxide and the superoxide anion donor, menadione. induced cell death depending on concentration. Nuclear
Phosphorylation of MAPKs, JAK2 and STAT3 and cell staining with Hoechst 33,258 demonstrated that control
death were investigated. PC12 cells had regular and round-shaped nuclei. In contrast,
Results: Hydrogen peroxide and superoxide anion induced the fragmentation of nuclei was demonstrated in cells
distinct responses in SK-N-MC cells as we showed that treated with 500M MPP+. Treatment with 0.75M PAF or
unlike JAK2 which was activated by both oxidants, STAT3 0.5M cyclosporin-A significantly attenuated the MPP+-
and p38 were activated in response to hydrogen peroxide induced release of cytochrome-c and caspase-3 activation.
and not superoxide radicals in SK-N-MC cells and In contrast, 10M PAF induced cytochrome-c release and
menadione induced JNK-dependent p53 expression and caspase-3 activation, and exhibited an addictive effect
apoptotic cell death which was not detected in H2O2- against the MPP+ toxicity.
induced JNK activation. Conclusions: The results show that PAF at low
Conclusion: ROS type has a key role in selective instigation concentrations, which does not induce a significant toxicity,
of JNK/p38 MAPKs and JAK2-STAT3 pathways in SK-N- may prevent the MPP+ toxicity by suppressing the apoptotic
MC cells. Identifying these differential behaviours and protein activation and mitochondrial membrane
mechanisms of function illuminates therapeutic targets in the permeability change that leads to the cytochrome-c release
prevention or treatment of ROS-induced neurodegenerative and caspase-3 activation.
diseases such as Alzheimers disease.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


444 Posters, Sunday 9 September

P1953 P1954
Does oxaliplatin-induced neurotoxicity Peroneal nerve palsy induced by
affect peripheral small nerve fibres? prolonged squatting: three case reports
M. Buonocore1, A.M. Gatti1, A. Bodini1, G. Amato1, and a review of the literature
A. Milani2, I.T. Lorenzetti2, V. Fregoni2, G.A. Da Prada2 G. Genc, A. Bakr
1Unit of Clinical Neurophysiology, 2Unit of Clinical Medical Maresal Cakmak Hospital, Erzurum, Turkey
Oncology I, Fondazione Maugeri, Scientific Institute of
Introduction: Entrapment neuropathies are common
Pavia, Italy
neuropathies, and the incidence tends to increase in the last
Introduction: Efficacy of oxaliplatin in the treatment of 3 decades. The reasons for this increase are technological
colorectal cancer has been largely demonstrated but its advances in diagnostic tools, mechanization of society and
clinical use is frequently limited by the development of bad working conditions. Three cases of peroneal palsy
neurotoxicity. Large peripheral nerve fibres are involved in caused by prolonged sitting in a specific position are
the development of oxaliplatin induced neurotoxicity; but to described and we aim to emphasize the optimization of
date little is known about a possible damage of peripheral working conditions.
sensory small nerve fibres (SNFs). Aim of the study was to Case Reports: Peroneal nerve entrapment was diagnosed
investigate the effects of oxaliplatin treatment on the in 3 young male patients. 2 of them were working as
Epidermal Nerve Fibre Density (ENFD), the study of which building workers. They had a history of 5 or 6 hours
is an accepted method for the assessment of small fibre working in the building. The other patient was a soldier and
neuropathies. had a history of 2 hours squatting in military sport training.
Methods: 10 patients (5 males, 5 females) completed this The diagnosis was established by clinical and
preliminary study. Two skin biopsies were performed in leg electrophysiological studies. All patients had postural-
and thigh before and after a chemotherapy regimen induced entrapment. Initially, all the patients were treated
containing oxaliplatin. In each patient the treatment was conservatively with a drop-foot splint and vitamin B. 2
repeated every 2 weeks until disease progression, patients responded to treatment. Since detection of no
unacceptable toxicity or a maximum of 12 cycles. clinical and electrophysiological improvement after three
Results: In this preliminary study no significant changes months of conservative treatment, surgical decompression
were observed on ENFD suggesting that the standard was performed in 1 patient, which resulted in a successful
oxaliplatin chemotherapy for colorectal cancer does not outcome.
induce a degeneration of SNFs. Conclusion: Because of nutritional deficiency, metabolic
Conclusions: To the authors knowledge, this is the first factors, or a decrease in protective subcutaneous tissue
report of an investigation on the possible effect of oxaliplatin surrounding the nerve, thin people become more sensitive
on SNFs. This information could be useful for a better to direct pressure and mechanical irritation. We associate
understanding of pathophysiological mechanisms related to the peroneal nerve palsy induced by prolonged squatting of
the development of oxaliplatin-induced neurotoxicity and our cases with this aspect. Since permanent deficits may
possibly permits to assist the advancement of new occur, it is very important to improve the working conditions
neuroprotective strategies. In order to confirm the present of workers who tend to peroneal nerve palsy.
findings and to explore the possibility of an oxaliplatin-
related dysfunction (without degeneration) of SNFs , more
studies on larger populations are warranted.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 445

P1955 P1956
Design, biomolecular modelling and Potential protective roles of retinoic acid
evaluation of surface-engineered in ethanol-induced neurotoxicity
nanoliposomes for the management of P. Dharmasaroja, K. Grataitong
Alzheimers disease Mahidol University, Bangkok, Thailand
M.S. Mufamadi1, Y.E. Choonara1, L.C. du Toit1, Introduction: Imaging studies have shown that excessive
G. Modi2, D. Naidoo3, P. Kumar1, V.M.K. Ndesendo4, ethanol intake can lead to abnormal reduced brain volumes
L. Meyer5, S.E. Iyuke6, V. Pillay1 of grey and white matter across multiple regions. Exposure
1Department of Pharmacy and Pharmacology, 2Department to ethanol leads to a decrease in anti-apoptotic proteins and
of Neurology, 3Department of Neurosurgery, University of an increase in pro-apoptotic proteins in various neuronal
the Witwatersrand, Johannesburg, South Africa, 4School of cells, including Purkinje cells. Retinoic acid (RA) has been
Pharmacy and Pharmaceutical Sciences, St. Johns shown to exert anti-apoptotic effects. This study aimed to
University of Tanzania, Dodoma, Tanzania, 5Central Animal investigate the effects of all-trans RA on ethanol-induced
Services, University of the Witwatersrand, 6School of cell death in human dopaminergic SH-SY5Y neuroblastoma
Chemical and Metallurgical Engineering, University of the cells.
Witwatersrand, Johannesburg, South Africa Methods: Cell viability was revealed using an MTT assay.
Purpose: The purpose of this study was the design, Nuclear morphology was determined by DAPI staining.
modelling and evaluation of the surface engineered Expression of tyrosine hydroxylase (TH) was analyzed
nanoliposomes (NLPs) coupled with chelating ligands using Western blotting and real-time RT-PCR.
[Zinc acetate (ZnAc), Histidine (His) and Results: The effect of ethanol on cell viability was dose-
Ethylenediaminetetraacetic acid (EDTA)] for neuronal cell dependent. In the presence of 10M RA, 200mM ethanol-
protections against A aggregates. induced cell death and nuclear fragmentation were reduced
Methods: NLPs were fabricated from after 24 hours of exposure. Ethanol increased TH protein
disteroylphosphatidylcholine (DSPC), Cholesterol (Chol), expression in a dose- and time-dependent manner, with a
and disteroylphosphatidylethanolamine (DSPE- significant change observed at 200mM ethanol and
mPEG2000) using the reverse phase evaporation technique. following 72 hours of exposure. Western blotting and real-
Chelating ligands were surface engineered onto the surface time RT-PCR analysis showed that 72 hours of treatment
NLPs. Biomolecular modelling analysis was used as with 200mM ethanol significantly increased TH expression
predictive approach in the design of surface-engineered when compared to unexposed controls. In contrast, cells
NLPs to investigate the interaction of metal ions [Cu(II) and cultured with 10M RA and 200mM ethanol displayed
Zn(II)] with A(1-42) peptide resulting in CuA(1-42) or significantly decreased TH expression when compared to
ZnA(1-42) aggregates, and the effect of surface-modified cells treated with ethanol alone.
NLPs in preventing A aggregates. Cyto(neuro)toxicity Conclusions: RA protects against cell death and prevents
studies were performed on the surface-modified NLPs in the biochemical adaptation of ethanol-treated SH-SY5Y
order to assess the effect on neuronal cell proliferation. cells.
Dead cells were assayed using CytoTox-GloCytotoxicity
kit and counted employing a plate reader. The interaction of
DSPC, Chol and DSPE-mPEG2000 during NLPs
formulation and engineering of chelating ligands on the
surface of NLPs was validated by Fourier Transmission
Infrared (FITR) and Scanning Electron Microscopy (SEM).
Results: Analytico-mathematical and geometrical
configuration validated the interaction energy during
protein/metal or metal/chelating ligands complexation. Ex
vivo studies showed about 30-40% cell viability after
exposure to CuA(1-42)/ ZnA(1-42) aggregates.
Furthermore, about 60-80% cell viability was shown after
treatment with surface-modified NLPs with ZnAc, histidine
and EDTA
Conclusions: The in vitro and ex vivo experimental studies
were well corroborated by the in silico molecular
mechanistic studies, thus further confirming the potential of
NLP/chelation therapy for Alzheimers disease intervention.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


446 Posters, Sunday 9 September

P1957 P1958
Tacrolimus neurotoxicity heralded by Cerebrolysin reduces exacerbation of
myoclonus blood-brain barrier breakdown, oedema
A. Nicodme, Y. Serroukh, F. Supiot, T. Gustot, G. Naeije formation, nitric oxide synthase
Universit Libre de Bruxelles, Belgium upregulation and brain pathology after
Introduction: The neurological toxicity of tacrolimus is heat stroke in diabetic and hypertensive
well described, including posterior reversible rats
encephalopathy syndrome (PRES). Myoclonus has not been
reported earlier. We report two cases of patients treated with D.F. Muresanu1, R. Patnaik2, A. Sharma3, H.S. Sharma3
1Neurosciences, University of Medicine and Pharmacy Cluj-
tacrolimus who developed symptoms of PRES heralded by
Napoca, Cluj-Napoca, Romania, 2Biomaterials, School of
myoclonus.
Bioengineering, Banaras Hindu University, Institute of
Interest: 2 patients treated with tacrolimus for liver
Technology, Varanasi, India, 3Surgical Sciences,
transplant due to alcoholic cirrhosis and hepatocarcinoma
Anaesthesiology & Intensive Care Medicine, Uppsala
were hospitalized in neurology. The delay between University, Uppsala, Sweden
transplant and symptoms was five months in both cases.
Case reports: A 68-year-old man presented generalized
myoclonus causing repeated falls one week before P1959
hospitalization. Neurological examination disclosed
generalized rest and action myoclonus, dysarthria, optical
Encephalopathy due to isotretionin
apraxia and a bipyramidal syndrome. Brain imaging and therapy
tacrolimus blood concentration were normal. Tacrolimus H.L. Gl1, O. Karadas2, H. Tutkan3, L. Ak1,
was stopped and the patient recovered. The second case is a U. TurkBoru1
52-year-old man. One week before hospitalization, he 1Neurology, Kartal Research and Education Hospital,
developed generalized myoclonus. Neurological Istanbul, 2Neurology, Erzincan Military Hospital, Erzincan,
examination disclosed incoherent speech, right neglect and 3Neurology, Occupational Diseases Hospital, Istanbul,

severe generalized rest and action myoclonus. Tacrolimus Turkey


blood concentration was also normal. Brain magnetic
resonance imaging (MRI) showed evidence of PRES.
Clinical manifestations and MRI abnormalities disappeared P1960
when tacrolimus was stopped. Opinion making on health status and
Conclusion: Generalized myoclonus caused by tacrolimus ability to run motor vehicles in people
is a severe adverse event and unreported earlier. Myoclonus
after craniocerebral traumas in Poland
may announce PRES symptoms in patients with normal
tacrolimus blood concentration. Clinicians should be aware H. Sinczuk-Walczak
of myoclonus as first manifestation of tacrolimus toxicity. Nofer Institute of Occupational Medicine, Lodz, Poland

P1961
Protective effect of MitoQ against
Dichlorvos induced oxidative stress and
ensuing dopaminergic neuronal cell death
W.Y. Wani, K.D. Gill
Biochemistry, PGIMER, Chandigarh, India

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 447

P1962 P1967
Bortezomib induced severe peripheral Rhabdomyolysis caused by toluene
neuropathy and PRES in a patient with intoxication
multiple myeloma Z. Idrizovic1, S. Vujisic2
1Neurology, General Hospital, Bijelo Polje, 2Neurology,
A. SlassiSennou1, A. Just1, J. Amevigbe1, T. Maisonobe2,
S. Thepot3 Clinical Center Podgorica, Podgorica, Montenegro
1Neurology, Hospital of Beauvais, Beauvais, 2Neurology

Functional Explorations, Piti-Salptrire University


Hospital, 3Hematology, Avicenne Hospital, Paris, France
P1968
Garlic (S-allylcysteine) helps in treatment
of neurodegenerative disorders
P1963
A.P. Singh
GDNF-mediated protection of neurons Special Centre for Molecular Medicine, Jawaharlal Nehru
and glial cells from necrosis and University, New Delhi, India
apoptosis induced by photodynamic
treatment P1969
A. Uzdensky1, G. Fedorenko2, M. Komandirov1
Psychiatric presentation of Marchiafava-
1Biophysics, 2Institute for Neurocybernetics, Southern
Federal University, Rostov-on-Don, Russia Bignami disease: a case report with
typical radiologic findings
A. Galiana-Ivars, S. Fernndez-Menndez,
P1964 L. Redondo-Robles, R. Garca-Santiago,
Chronic methylmercury poisoning B. Cabezas-Delamare, A. Ars-Luque, J. Tejada-Garca,
manifesting as progressive myoclonic E. Rodriguez
epilepsy in a Sudanese family Complejo Asistencial Universitario de Len, Spain
M.T. Obeid, W.M. Malik, M. Homeida
Medicine, The University of Medical Science & Technology, P1970
Khartoum, Sudan
Disulfiram neuropathy: a case report
S.M. Vujisic1, Z. Idrizovic2
P1965 1Neurology, Clinical Centre of Montenegro/Medical Faculty

Autonomic nervous system dysfunction University of Montenegro, Podgorica, 2Neurology, General


Hospital of Bijelo Polje, Montenegro
related with exposure to mixed organic
solvents in shoe-makers
S. Kamisli, U. Teker, O. Kamisli, Y. Kablan, C. Ozcan P1971
Neurology, Inonu University, Malatya, Turkey Effect of Nardostachys jatamansi root
extract on haloperidol induced orofacial
P1966 dyskinesia in rats
Metronidazole-induced reversible R.A. Shaik
Pharmacology, Nizam Institute of Pharmacy, Hyderabad,
encephalopathy in a patient with India
facioscapulohumeral muscular dystrophy
A. Papathanasiou1, V. Zouvelou1, S. Kyriazi2,
M. Rentzos1, C. Moshovos1, E. Kanavakis3,
I. Evdokimidis1
1Department of Neurology, Aeginition Hospital, National and

Kapodistrian University of Athens, 2Department of


Radiology, National and Kapodistrian University of Athens,
3Department of Medical Genetics, Medical School, National

University of Athens, Greece

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


448 Posters, Sunday 9 September

Sleep disorders P1973


Quality of life in patients with restless
P1972 legs syndrome; comparison of the
Mortality and morbidity after sleep influence of pain versus poor sleep quality
disordered breathing Y.W. Cho1, H.-J. Moon1, K.Y. Jung2, W.C. Shin3
1Neurology, Keimyung University Dongsan Medical Center,
P. Jennum1, R. Ibsen2, J. Kjellberg3
1Danish Center for Sleep Medicine, Department of Clinical Daegu, 2Neurology, Korea University College of Medicine,
3Neurology, Kyung Hee University School of Medicine,
Neurophysiology, University of Copenhagen, 2University of
Copenhagen, Glostrup Hospital, Glostrup, 3Danish Institute Seoul, Republic of Korea
for Health Services Research, Copenhagen, Denmark Background: The patients with restless legs syndrome
The long-term prognosis of obstructive sleep apnoea (OSA) (RLS) had a lower quality of life (QOL) than normal
and obesity hypoventilation syndrome (OHS), in controlled controls. However, it is debatable which affects their QOL
settings are incompletely described. Using data from the more, disturbing pain or poor sleep quality. We studied the
Danish National Patient Registry (NPR) (1998-2010), QOL of patients with RLS and compared it to patients with
30,278 individuals with a diagnosis of OSA (23,208 men osteo-arthritis (OA) who were having similar leg discomfort.
and 7,070 women) and 1,562 with a diagnosis of OHS Methods: In total, the scores of the 153 RLS patients were
(1,092 men and 470 women) were identified. Four age-, compared with the scores of 153 OA patients. All subjects
sex- and socio-economic-matched citizens were randomly completed the questionnaires, including the Korean versions
selected from the Danish Civil Registration System, in total of the PSQI and SF-36, the Short-form McGill Pain
120,506 and 6,241 controls, respectively. Morbidity and Questionnaire and VAS for their pain quantification. We
all-cause mortality was extracted from the NPR. The analyzed the related factors in the QOL of RLS patients
10-year survival of treated and untreated patients with OSA through Pearson Correlations.
was 90.7% compared to 92.4% among controls and of Results: The patients with RLS had a higher QOL than
patients with OHS 63.9% compared to 85.5% amongst patients with OA, though, there were no significant
controls (both: p<0.0001). Commonly significant (p<0.01) differences in pain scores between the patients with RLS
morbidities observed in patients with OSA were related to and those with OA and the subjects with RLS even had
respiratory (1.91 (1.82-2.01)), nervous (1.65 (1.55-1.75)), worse sleep quality than those with OA (p<0.001).
endocrine, metabolic, nutrietal (1.53 (1.54-1.75)), ENT: The SF-36 Qol of RLS patients showed a significantly
1.39 (1.30-1.49), circulatory: 1.20 (1.16-1.27), muscu negative correlation with the severity of RLS symptoms
loskeletal: 1.25 (1.20-1.30), digestive illnesses (1.09 (1.03- (r=-0.472, p<0.001), the severity of depression (r=-0.569,
1.14)), and injuries 1.12 (10.8-1.16). Mental disease and p<0.001), pain severity (VAS) (r=-0.394, p<0.001), and
neoplasm showed a lower occurrence: 0.90 (0.81-0.99) and sleep quality (r=-0.290, p<0.001). Step-wise multiple
0.85 (0.80-0.95), respectively. OHS showed higher regressions identified several factors related to SF-36 QoL:
morbidities to respiratory: 4.03 (3,21-5.07), nervous: 3.17 RLS symptom severity (=-0.813, p<0.001), depression
(2.43-4.15), endocrine, metabolic and nutrietal (4.65 (3.67- (=-0.598, p<0.001), and female gender (=-11.983,
5.90)), ENT:1.39 (1.30-1.49), circulatory: 1.84 (1.50-2.26), p<0.001).
musculosceletal: 1.25 (1.20-1.30) and digestive illnesses Conclusions: We found that poor sleep quality related less
(1.09 (1.03-1.14), and injuries 1.12 (10.8-1.16). Neoplasm than pain severity to diminished QOL in patients with RLS.
occurred less often: 0.70 (0.50-0.97). CPAP reduced
mortality in OSA but not in OHS patients due to major
co-morbidities. CPAP treated pts however showed
significantly higher co-morbidity. OSA and especially OHS
present significant morbidity and mortality. CPAP reduced
the excess mortality in OSA.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 449

P1974 P1975
Dynamical structure of NREM sleep Characterizing sleep disorders in geriatric
disclosed by non-linear analysis populations
techniques P. Seibert1,2, J. Valerio2, Y. Rafla2, F. Grimsley3,
L. Sellitti1,2,
L. Priano1, M. Bigoni1, F. Saccomandi3, C. Zimmerman3
1Physician Research Services, Saint Alphonsus Regional
C. Guiot4, A. Mauro1,2
1Dipartimento di Neuroscienze, IRCCS Istituto Auxologico Medical Center, 2Boise State University, 3Saint Alphonsus
Italiano, Universit degli Studi di Torino, Ospedale San Regional Medical Center, Boise, ID, USA
Giuseppe, Verbania, 2Dipartimento di Neuroscienze, Sleep disorders (SD) affect approximately one-third of the
Universit degli Studi di Torino, Ospedale Molinette, world population. The presence of SDs occurs at all ages
3Dipartimento di Neuroscienze, Universit di Torino, INRIM,
although the presentation and subsequent consequences for
4Dip di Matematica Giuseppe Peano, Universit di
an individuals health change in accordance with the natural
Torino, Italy aging process. Currently, evaluation of SD is inadequate
Introduction: Sleep is a dynamic process whose across all age ranges as it is compromised by under reporting
macrostructure may be considered the result of graduations and by relying on self-report rather than professional sleep
of transient EEG activities defining the microstructure of studies (i.e., nocturnal polysomnography (NP) and multiple
sleep. Among EEG activities, peculiar transient sleep latency tests (MSLT)). Moreover, there is a paucity of
synchronized EEG patterns (TSEP) are supposed to play a data specific to older adults. We constructed a 111-item
role in organization and stabilization of NREM sleep. questionnaire to use in conjunction with NP, MSLT, the
Materials and methods: Sleep recordings from 10 healthy Epworth Sleepiness Scale (ESS), and medical chart reviews
subjects (5 males, 5 females; mean age 28.54.8 years; of people referred for evaluation of SDs. We categorized
normal BMI), after an adaptation night, underwent a full- participants into two broad age groups: adult (age 19-65)
night polysomnography in the sleep laboratory. Rough data and geriatric (66-90) to illuminate a characterization of the
of all EEG signals were exported into a MATLAB program geriatric population. Data analysis from 682 people who
specifically written to detect TSEP onsets, and then verified were diagnosed with SD was compared for two groups:
by visual inspection. The time series of TSEP sequences Adults (n=541mean age 46.22; 295 male; 246 female) and
were analysed according to the Recurrence Quantification Geriatric (n=141; mean age 71.59; 83 male; 58 female)
Analysis (RQA) and Recurrence Plot (RP), non-linear revealed a striking pattern of differences between the two
analysis techniques suitable to disclose deterministic groups. For example, the geriatric group had significantly
patterns in complex and apparently chaotic behaviours. greater prevalence of diagnoses of periodic limb movement,
Results: RP and RQA evidence the presence of attractors of poor sleep efficiency, hypersomnolence, and restless leg
different "strength" towards stable sleep. TSEP form a syndrome, and nocturnal hypoxemia. Analysis also
deterministic pseudo-periodic series with an oscillating indicated numerous mental and physical health differences
period which becomes progressively shorter and finally - many of which were unexpected based upon existing
stabilizes its recurrence rate (attractor) until steady slow literature. This investigation provides a characterization of
wave sleep (SWS) is reached. Both several seconds before SD to enhance treatment approaches for geriatric
REM sleep onset and during the last sleep cycles of the populations rather than relying on assumptions of a general
night, the system becomes unstable and inter-TSEP intervals adult SD model.
exhibit higher variability.
Discussion: We conclude that this approach is a suitable
method to describe and quantify the deterministic
neurophysiological processes during the building up of
NREM sleep, so that it may become a novel method to
detect subtle or otherwise unrecognizable conditions of
disturbed sleep.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


450 Posters, Sunday 9 September

P1976 P1977
Obstructive sleep apnoea-hypopnoea and Five Kleine-Levin syndrome cases:
acceptance of nasal CPAP in Korean a systematic report
ischemic stroke patients R. Frandsen1, S. Gammeltoft2, P. Jennum3
1Danish Center of Sleep Medicine, 2Department of Clinical
Y.J. Lee
Neurology, Asan Medical Center, Ulsan University College Biochemistry, University of Copenhagen, Glostrup Hospital,
3Danish Institute for Health Services Research, Copenhagen
of Medicine, Seoul, Republic of Korea
University Hospital, Glostrup, Denmark
Objective: The aims of this study were to investigate the
prevalence of obstructive sleep apnoea-hypopnoea (OSA) Kleine-Levin Syndrome (KLS) is a rare form of episodic
in patients with ischemic stroke and their acceptance rate of hypersomnia with adolescent onset. About 10 cases are
nasal continuous positive airway pressure. reported worldwide yearly. The pathophysiology is poorly
Design and methods: Patients with subacute ischemic understood. We describe five cases of KLS in a concise
stroke were evaluated for the presence of OSA by portable form to aid data collection for further studies of this disease.
polysomnogramme (PSG) or in-lab night PSG. Berlin Methods: Five cases of KLS with clinical symptoms and
questionnaire (BQ) was asked to investigate previous findings are presented with additional systematic reviews of
history of snoring or apnoea. Patients who underwent nasal former cases. Hypocretine measurements, Polysomnography
CPAP titration for OSA were followed up for CPAP and clinical evaluation.
acceptance. Results: We succeeded in measuring hypocretin-1 (hcrt-1)
Results: 94 patients with ischemic stroke were enrolled in patient 2 during an attack and patient 3 at the end of an
(mean age 65.511.4yrs, 58 male). Mean BMI was attack. Hcrt-1 was normal in both patients measured in
23.93.4. 57.1%; and 50% of 59 patients who answered BQ relation to the hypersomnia period. Patient 1 had normal
had a history of persistent snoring and experienced apnoea, Hcrt-1 in the interval between attacks. Hcrt-1 on the last 2
respectively. 87.7% (50/57) of patients with ischemic stroke patients has not been available. PSG results are not similar
who underwent PSG within a month after the onset of to narcolepsy as sleep architecture is normal, and REM
stroke were diagnosed with OSA. The prevalence of mild, sleep does not seem affected like in narcolepsy.
moderate and severe sleep apnoea was 30%, 20%, and 50%, Conclusion: KLS presents a complex clinical picture. KLS
respectively. Mean AHI was 30.919.5/hr. 18 patients does not represent a phenotype like narcolepsy or epilepsy.
underwent nasal CPAP titration. 9 agreed to a trial of nasal Hypocretin does not seem to be involved in the
CPAP. 6 patients (6/9) continued nasal CPAP use with good pathophysiology of KLS.
compliance despite of the fact that some of them remained
quadriplegic.
Conclusions: Our study showed that OSA is highly
prevalent in patients with ischemic stroke. High prevalence
of snoring history suggests that OSA might be a pre-existing
condition rather than the result of stroke. Severe neurologic
deficit does not seem to be an obstacle for nasal CPAP use.
Nasal CPAP use should be encouraged in patients with OSA
and ischemic stroke.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 451

P1978 P1979
Positional obstructive sleep apnoea: Hypertension and OSAS: instrumental-
prevalence, clinical and clinical correlation in a group of patients
polysomnographic characteristics from southern Italy
R.D. Marques1, T. Rodrigues2, F.G. Stelzer3 G. Gervasi1,2, I. Aric1, G. Mento1, R. Silvestri1
1Complexo Hospitalar Santa Casa de Porto Alegre, 1Sleep Medicine Center, Messina Medical School, Messina,
2Neurology, Complexo Hospitalar Santa Casa de Porto 2Neurorehabilitation, Centro Cardinal Ferrari, Istituto Santo
Alegre, Porto Alegre, 3Neurology, Complexo Hospitalar Stefano, Fontanellato, Italy
Santa Casa de Porto Alegre, So Leopoldo, Brazil Introduction: Sleep disordered breathing has been
Introduction: Body position influences the frequency of associated with hypertension. Obstructive sleep apnoea
apnoeas and hypopnoeas in 50 to 60% of obstructive sleep (OSAS) is considered one of the secondary causes of
apnoea (OSA) patients. This group may be treated in hypertension. Aim of this study was to evaluate the eventual
different ways than non-positional OSA (NP-OSA). correlation between the degree of OSAS and related
Objective: To estimate prevalence, clinical and hypertensive status.
polysomnographic factors related to positional OSA Methods: We have retrospectively studied 329 patients
(P-OSA). referred to our Sleep Medicine Center in Messina (Italy),
Design: Retrospective analysis of patients undergone who were evaluated for OSAS through Holter-PSG. They
polysomnography, >18 y.o., with OSA [apnoea-hypopnoea were subdivided into 4 groups according to the Apnoea/
index (AHI) >5/h]. The sample was divided in two groups Hypopnoea index (AHI): C (healthy patients), L (mild
according to presence or absence of P-OSA, defined as total OSAS), M (moderate OSAS), S (severe OSAS). Blood
AHI 5/h with reduction >50% in AHI between supine and pressure (BP) was detected at baseline in all patients.
non-supine positions and AHI <5/h in non-supine position. Statistical significance was set at p<0.05.
Results: In this sample of 314 individuals, P-OSA was Results: In group S both systolic and diastolic BP values
diagnosed in 25.2%. There was no difference related to had a trend toward an increase. Moreover we found a trend
gender, age, daytime sleepiness, and sleep complaints, other to a positive correlation between AHI and BP values, with
than higher rate of witnessed apnoea in NP-OSA (55.1 vs. a Spearmans R-value of 0.16 and 0.14 for systolic and
68.9%; p=0.029). BMI (27.14.6 vs. 31.26.1kg/m2; diastolic values respectively. Higher BP values prevailed in
p<0.0001) was lower in the P-OSA. Allergic rhinitis was males (81.6%) over females (18.4%). However, no
more prevalent in the P-OSA group (43.0 vs. 28.9; p=0.026). correlation between AHI, mean nocturnal SaO2, BMI and
Higher arousal index (29.811.9 vs. 45.719/h; p<0.0001) age was statistically associated with an increased risk for
and less slow wave sleep (29.214.2 vs. 25.613.5%; hypertension.
p=0.050), and trend to more N1 stage (4.53.7 vs. 5.44.2%; Conclusion: Our results confirm a higher prevalence of
p=0.079) were observed in NP-OSA. AHI (17.114.6 vs. hypertension in elderly obese patients with the highest AHI,
39.122.5/h; p<0.0001) was higher in NP-OSA than in mild especially in male subjects. This male preponderance might
OSA (62% vs. 16%; p<0.0001) predominating in P-OSA be related to the overall higher prevalence of male patients
and severe OSA (12.7% vs. 61.3%; p<0.0001) in NP-OSA. in our population, rather than to a real statistical difference
Conclusions: The prevalence of P-OSA in this sample was related to AHI. We were able to detect hypertension in
25.2%, lower than initially expected. P-OSA patients had patients with higher AHI despite no clear association of BP
lower BMI and higher prevalence of allergic rhinitis. values to the OSAS severity degree.
NP-OSA patients have more severe OSA than P-OSA.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


452 Posters, Sunday 9 September

P1980 P1981
Central cholinergic neurotransmitter Issues of clinical and neurophysiological
system - the role in sleep disorders in diagnostics of narcolepsy with cataplexy
patients with Parkinsons disease M. Tormaiov, V. Ha, E. Feketeov
I.V. Krasakov1, I.V. Litvinenko2 Department of Neurology, Medical Faculty of P.J. afrik
1The Center of Extrapyramidal Disorders, The Nikiforov University and Louis Pasteur University Hospital, Koice,
Russian Center of Emergency and Radiation Medicine, Slovak Republic
2Department and Clinic of Neurology, Military Medical
Introduction: Suggestive anamnesis of imperative
Academy named after S.M. Kirov, Saint Petersburg, Russia sleepiness with sudden falls is often insufficient to prove
To evaluate the potential of Galantamine therapy for EDS narcolepsy with cataplexy. Diagnosis is supported by
and RBD in PD patients, we studied 26 patients at the age multiple sleep latency test (MSLT), where average sleep
of 66.67.8 years old with the 3rd stage of PD having latency is shortened under the 8 minutes and SOREM is
subjective complaints of sleep disorders. All the patients present in at least 2 tests. Because of ambiguous results,
were rated with the following scales: MMSE, FAB, PDSS, MSLT must be repeated in some cases to definitely confirm
ESS. 17 patients of this group underwent a complex narcolepsy.
polysomnography (PSG) evaluating the sleep efficacy Methods: 31 patients suspected of narcolepsy with
(TST/TIB100%), sleep latency (LS); REM cycle was also cataplexy were examined, which involved: all-night
studied for behaviour disorders. Galantamine was given in polysomnography with subsequent MSLT during the next
addition to previous therapy 8mg per day for 4 weeks, then day, HLA class II typification, MRI of the brain,
16mg per day for up to 12 weeks. EDS was revealed in 22 psychological as well as psychiatric examination and
patients (84.5%). In 16 (93%) of 17 patients who had had laboratory screening of excessive sleepiness in order to
PSG done a sleep structure disruption as its fragmentation, exclude secondary cause of narcolepsy.
decrease in sleep quality as increasing the wake time during Results: According to the medical history, narcolepsy with
sleeping and, therefore, reduction of its efficacy were cataplexy was presumed in 31 patients. Diagnosis was
determined. In 12 weeks of treatment patients showed confirmed by the first MSLT in 25 subjects (80.6%), and by
reliable decrease in the intensity of daytime sleepiness (ESS repeated MSLT in 4 subjects (12.9%). MSLT was repeated
scale results (p=0.0006)), reduction in sleep fragmentation, in those cases, where REM-sleep was not present in at least
sleep quality (improving the PDSS scale results (p=0.0007)), 2 tests during the first MSLT. Average sleep latency was
sleep efficacy (TST/TIB (p=0.0007)). Against background shortened under 8 minutes in all patients during the first
of the given therapy there were no behaviour disorders in MSLT. Diagnosis was reassessed to depression with
REM sleep revealed. Improvement in hallucination intensity pseudocataplexy in 2 patients (6.5%) after the second
was also noticed: PDSS7 (p=0.001) and cognitive disorders MSLT.
(the MMSE (p=0.001) and FAB (p=0.001) scales). Conclusion: Discrepancy between clinical and
Galantamine therapy induces a significant decrease in neurophysiological findings in diagnostics of narcolepsy
daytime sleepiness intensity and behaviour disorders in with cataplexy may appear. This severe diagnosis should be
REM sleep, reduction in sleep fragmentation and clearly verified, most likely by the repeated MSLT, in case
improvement in sleep quality. that examination of CSL hypocretin level is not available.
Depression was the most common condition imitating the
symptoms of narcolepsy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 453

P1982 P1983
Decreased sleep spindle density in Sleep disorders and risk of stroke in the
patients with idiopathic REM sleep general male population aged 25-64 in
behaviour disorder and patients with Russia (the MONICA-psychosocial
Parkinsons disease study)
J.A.E. Christensen1, P. Jennum2, J. Kempfner1, V. Gafarov1,2, E. Gromova1,2, A. Gafarova1,2, I. Gagulin1,2
M. Zoetmulder3, H. Leonthin4, L. Arvastson5, Collaborative Laboratory of Epidemiology of
1

S.R. Christensen6, H.B.D. Srensen1 Cardiovascular Diseases SB RAMS, 2Institute of Internal


1DTU Electrical Engineering, Technical University of Medicine SB RAMS, Novosibirsk, Russia
Denmark, Lyngby, 2Danish Center for Sleep Medicine, The aim of research: To examine associations between
Department of Clinical Neurophysiology, University of sleep disorders and risk of stroke in the general male
Copenhagen, Glostrup University Hospital, Glostrup, population aged 25-64.
3Department of Neurology, Bispebjerg Hospital,
Materials and methods: Within the framework of WHO
Copenhagen, 4Department of Clinical Neurophysiology, program MONICA-psychosocial in 1994 random
Glostrup University Hospital, Glostrup, 5Biostatistics, representative sample of men at the age 25-64 years,
6Clinical Pharmacology, H. Lundbeck A/S, DK- Valby,
residents in one of the Novosibirsk (Russia) districts were
Denmark investigated. The volume of sample was 657 men. Sleep
Introduction: The objective of this study is to determine disorders have been studied by methodic MOPSY. During
whether sleep spindles (SS) hold the potential of being a the follow up period of 10 years (1995-2004) all first
biomarker for Parkinsons disease (PD). incidents of stroke were investigated in the cohort. Cox
Methods: 12 PD patients with rapid-eye movement (REM) proportional regression model was used for the estimation
Sleep Behaviour Disorder (PD+RBD), 8 PD patients of relative risk.
without RBD (PD-RBD), 10 idiopathic RBD (iRBD) Results: The relative risk of stroke during a follow-up
patients and 13 age-matched controls underwent period of 5 years in subjects estimating their sleep as "bad"
polysomnography (PSG). SS were scored in an extract of or unsatisfactory was 3.9 (p<0.01) times higher than in
data from control subjects. An automatic SS detector using men estimating their sleep as good. During the follow-up
Matching Pursuit (MP) and Support Vector Machine (SVM) period of 10 years risk of stroke was 2.72 (p<0.05) times
was developed and applied to the PSG recordings. The SS higher among men estimating their sleep as bad or
densities in N1, N2, N3, all non-REM (NREM) combined unsatisfactory. The relative risk of stroke development
and REM sleep were obtained and evaluated across the has increased in the group of men having sleep disorders at
groups. the age of 55-64 years and was 8 (p<0.01) times higher than
Results: The SS detector reached a sensitivity of 84.7% and in men estimating their sleep as satisfactory. Risk of
a specificity of 84.5%. On a significance level of =5%, the stroke was higher among men who had sleep disorders with
PD+RBD patients had significantly lowered SS density incomplete secondary-primary education in groups of
compared to the control group in N2, N3 and all NREM. heavy-working and average-working men, among
PD-RBD patients had significantly lowered SS density in pensioners, among divorced and widowed persons.
N2 and in all NREM. The iRBD patients had significantly Conclusion: The received results show that risk of stroke is
lowered SS density in all NREM stages combined. At a associated with sleep disorders in male population aged
significance level of =10%, iRBD had significantly 25-64.
lowered SS density in N2 and N3.
Conclusion: This study suggests that the SS density has
potential of being a biomarker of PD.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


454 Posters, Sunday 9 September

P1984 P1985
Sleep-related eating disorder secondary Excessive daytime sleepiness (EDS):
to zolpidem clinical and polysomnographic findings
H. Nzwalo1, R. Peralta2, C. Bentes2 and relation to obstructive sleep apnoea
1Neurology, Faro Hospital EPE, Faro, 2Neurology, Santa
syndrome (OSA)
Maria Hospital, Lisboa, Portugal
F.G. Stelzer1, T. Rodrigues1, R.D. Marques2
Introduction and methods: Sleep-related eating disorder 1Neurology, 2Pneumology, Complexo Hospitalar Santa Casa
(SRED) is characterized by eating episodes in the first de Porto Alegre, Brazil
period of the night, accompanied by partial loss of Introduction: EDS is a major health issue for modern
consciousness, and amnesia. Although considered society and it is a common complaint associated to OSA.
idiopathic, some drugs can induce SRED. We report a Objectives: To estimate clinical and polysomnographic
video-supported case of zolpidem induced SRED. characteristics of EDS and the association between EDS
Results: A 53-year-old woman came to consultation and OSA.
because of episodes of eating, drinking, cooking, during Design: In a sample of consecutive individuals that
nocturnal sleep time with subsequent amnesia. She was underwent polysomnography (PSG), >18 y.o., we analysed
taking zolpidem for insomnia over 5 years. The history of clinical and PSG data related to EDS (Epworth Sleepiness
neurological, psychiatric, and daytime eating disorder was Scale (ESS) score 10).
negative. She reported awaking with plates and utensils in Results: In this 719 individuals sample, 53.6% had EDS.
her bed. She complained of morning nausea, anorexia, These patients had over weight (8820 vs. 8219kg;
fatigue, and gaining 6kg over the last year. Her sleep time p<0.0001) and BMI (30.56.1 vs. 29.56.6kg/m 2;
was regular. Her Epworth was 9. A home video showed one p<0.0001). Men had more EDS (61.7% vs. 38.3%;
episode. The EEG was normal. One episode of eating on p=0.018). There was no difference related to age, race,
stage N1 of sleep was recorded in the polysomnography. number of nocturnal sleep hours, caffeine consumption.
Her sleep was instable, fragmented, with frequent phase EDS individuals complaint more often of witnessed apnoea
changes, and excessive arousal index. There was no other (64.0 vs. 52.3%; p=0.002); memory (63.9 vs. 47.1%;
abnormal sleep phenomenon, apart from slightly increased p<0.0001) and attention deficit (57.4 vs. 47.1%; p=0.006);
respiratory index disturbance (RDI - 14.7). Zolpidem was morning headache (39.1 vs. 28.3%; p=0.003); sexual
stopped and the night eating episodes ceased. No abnormal dysfunction (31.0 vs. 19.3%; p=0.008). EDS patients smoke
phenomenon was noted in the control polysomnography six more (15.4 vs. 10.1%; p=0.040). Sleep latency was lower
months later. The sleep structure improved including the (13.212.3 vs. 20.432.3min; p<0.0001) and sleep
RDI (11.6). She lost 5kg. efficiency was higher (86.710.6 vs. 83.712.1%;
Conclusion: There are rare cases of zolpidem induced p<0.0001) in the EDS group. Sleep was more fragmented in
SRED. As a rule, SRED usually exists for longer time EDS patients (arousal index: 39.821.1 vs. 36.118.7/h;
before being diagnosed. Clinicians should actively ask p=0.013). There was no association between OSA and EDS,
about symptoms suggestive of SRED to avoid the potential but there was a trend towards EDS in severe OSA (42.8 vs.
deleterious consequences of this sleep disorder. 36%; p=0.075).
Conclusions: Men, obese and non-smokers had more EDS
in this sample. There was no significant association between
OSA and EDS. EDS patients have lower sleep latency,
higher sleep efficiency, and more sleep fragmentation.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 455

P1986 P1987
Achondroplasia with obstructive sleep Catathrenia - a rare but treatable
apnoea successfully treated with nasal parasomnia
CPAP therapy D. Neutel, R. Peralta, C. Bentes
K.H. Noh, S.J. Hwang, K.P. Park, J.W. Cho Hospital de Santa Maria, CHLN, Lisbon, Portugal
Pusan National University Yangsan Hospital, Yangsan, Introduction: Catathrenia is a rare parasomnia that occurs
Republic of Korea most frequently during REM sleep and consists of apnoea
Case report: Achondroplasia is the most common skeletal during end-inspiration and expiratory groaning during
dysplasia in children. The patients usually have a short sleep. The sleep partners who hear the patient describe a
cranial face, midface hypoplasia, small upper airway, which deep breath, hold breath, and exhale with a groan.
might lead to sleep-related respiratory disturbance. We Differential diagnosis is made with other sounds during
describe a case with achondroplasia whose severe sleep, but also with epileptic seizures.
obstructive sleep apnoea was successfully treated with nasal Case report: A 32-year-old male, with a past medical
CPAP (Continuous Positive Airway Pressure) therapy. A history of maxillo-facial surgery (reduction of the lower jaw
7-year-old girl presented with snoring and sleep disturbance. for prognathism) and epilepsy treated with valproate
She could not sleep nearly every day due to severe 1,500mg/day, presented to sleep clinic for a prolonged
respiratory disturbance, therefore she always felt sleepy and groan associated with apnoea, several times a night without
tired during the daytime. Her Epworth Sleepiness Scale snoring. MRI showed cortical perisylvian malformation -
score was 11. She has been diagnosed as achondroplasia areas of polymicrogyria in the margins of the insula and in
several years ago. The patients height was 97 centimetre, the central sulcus predominantly on the right. PSG revealed
and she weighted 24kg. Her neck X-ray and CT showed fragmented and superficial sleep with increased awakenings
adenoid hyperplasia, bilateral flat condyle and narrow and arousals, a predominance of arousals associated with
foramen magnum. Split night polysomnography result respiratory effort, especially in REM sleep, RDI 7.95.
revealed severe obstructive sleep apnoea and severe snoring Home video identified catathrenia. CPAP was initiated with
with high Apnoea Hypopnoea Index (AHI) 177.8 per hour. symptom resolution.
There was no central type apnoea. Arousal index, mostly Discussion: We describe a rare case of an epileptic patient
caused by apnoea or hypopnoea, was 178.3 per hour. There with catathrenia. The differential diagnosis between the two
was only stage 1 sleep and waking state on hypnogram. diseases is not always easy, but essential since the treatment
Nasal CPAP therapy began at 3 oclock in the morning, with non-invasive ventilation can treat this parasomnia and
which gradually decreased apnoea and snoring. Finally, adjustment of anti-epileptic therapy is completely
CPAP at pressure 12mmHg dramatically eliminated apnoea ineffective. Treatment of catathrenia can improve not only
and snoring, and slow wave sleep appeared on hypnogram. the quality of life of patients but also of their partners.
Nasal CPAP therapy is very effective with marked clinical Although classified as a parasomnia, a good response to
improvement to severe obstructive type sleep apnoea of therapies such as CPAP keeps the discussion if catathrenia
achondroplasia patients. CPAP therapy should be considered is indeed a parasomnia or sleep-disordered breathing.
regardless of adenotonsillectomy.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


456 Posters, Sunday 9 September

P1988 P1989
Clinical and polysomnographic Subjective sleepiness in relation to other
characteristics of obstructive sleep sleep complaints and quantified excessive
apnoea (OSA) related to its severity daytime sleepiness in patients with
F.G. Stelzer1, T. Rodrigues1, R.D. Marques2 epilepsy
1Neurology, 2Pneumology, Complexo Hospitalar Santa Casa
S. Khachatryan1,2, T. Stepanyan3, L. Ghahramanyan4,
de Porto Alegre, Brazil I. Shadyan3, H. Amroyan3, Y. Tunyan1
Introduction: OSA is a very common chronic disease in 1Postgraduate Department of Neurology, Yerevan State

adult population. Its manifestations and treatment vary Medical University after Mkhitar Heratsi, 2Somnus Sleep
according to severity. and Movement Disorders Clinic, 3Armenian Republic
Objectives: To estimate clinical and polysomnographic Epilepsy Center Erebouni, 4Clinical Psychology Master
factors associated to OSA severity. Program, Department of Social Psychology, Yerevan State
Design: Retrospective study, included all patients (>18yo), University, Yerevan, Armenia
with apnoea-hypopnoea index (AHI) >5/h, submitted to Aim: To evaluate relationship of subjective sleepiness
polysomnograpy. OSA was classified in mild, moderate, complaint with sleep-disordered breathing (SDB) and
and severe. insomnia complaints and measured excessive daytime
Results: In 630 patient sample, 29.3% had mild OSA; sleepiness (EDS) in patients with epilepsy.
25.2% moderate OSA, 45.6% severe OSA. Severe OSA Methods: Patients with proven diagnosis of epilepsy
patients were older (47.913.3 vs. 5312.7 vs. 54.313.6 attending the Armenian Republic Epilepsy Center
y.o.; p<0.0001), their BMI is higher (28.75.6 vs. 28.75.0 Erebouni were interviewed by trained staff for complaints
vs. 32.26.8kg/m2; p<0.0001). Mild OSA predominated in of sleepiness, sleep-disordered breathing and insomnia.
women (46.5 vs. 37.5 vs. 35.9%; p=0.020). Severe OSA Epworth Sleepiness Scale (ESS) was used for assessment of
patients complain more of witnessed apnoea, mouth EDS. ESS score of 9 and above has been considered a cut-
breathing and nocturia and less of morning headache. off for presence of EDS in this study. Patients were in stable
Prevalence of hypertension was higher in severe OSA (33.5 condition regarding seizure frequency, with no recent
vs. 36.9 vs. 50.5%; p<0.001). Sleep efficiency was lower change in medication. Proportional analysis was used for
(85.911.6 vs. 85.413.3 vs. 83.811.3%; p=0.048) in statistics.
severe OSA. These patients have more N1 (4.64.1 vs. Results: 48 patients with all-cause epilepsy aged 17-64
4.94.1 vs. 6.25.2%; p<0.0001) and N2 stage (49.512.2 years participated in the study (mean age - 36.9 years), of
vs. 49.910.8 vs. 59.813.2%; p<0.0001) and less N3 which 16 (34%) were females. 25 (53.2%) of all patients
(30.012.3 vs. 28.511.2 vs. 20.012.6%; p<0.0001) and had sleepiness complaint, 22 (46.8%) had SDB complaint
REM sleep (166.2 vs. 16.46.0 vs. 13.86.2%; p<0.0001). and insomnia was noted by 25 (53.2%). Of patients
Sleep was more fragmented in severe OSA patients (arousal presenting with sleepiness complaint, only 8 (36%) had
index=25.28.3 vs. 31.37.7 vs. 56.218.1/h; p<0.0001). ESS score 9 and above suggestive for EDS. 12 patients
Oxygen saturation was lower in severe OSA (mean (48%) with sleepiness noted SDB and 16 (64%) of them had
SpO2=94.81.8 vs. 94.51.7 vs. 92.33.2%; p<0.0001). insomnia complaint. SDB accompanied insomnia in 14
Conclusions: Mild OSA predominates in women. Severe patients (63.6%). Mean ESS score in non-sleepy patients
OSA patients are more obese and older. Their sleep is more was 3, in the sleepy 5.7 (p<0.03).
fragmented, they have less slow wave and REM sleep, and Conclusion: The results of our study show that different
lower oxygen saturation during sleep. sleep complaints are prevalent among patients with epilepsy.
They present mostly as sleepiness and insomnia in this
population. EDS seems more to be connected to disrupted
sleep and insomnia than to SDB. Sleepy patients had also
higher ESS scores.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457


Posters, Sunday 9 September 457

P1990 P1991
Augmentation of restless legs syndrome Pathological internet use and risk of sleep
with pregabalin treatment: a case report disorder incidence among adolescents
S. Korkmaz1, T. Bileceoglu2, M. Aksu3 E. Masoudnia
1Neurology Department, Ordu University Medical Faculty, Social Sciences, Yazd Uni, Yazd, Iran
Ordu, 2Neurology Department, Etlik Residency Education
and Research Hospital, Ankara, 3Neurology Department,
Erciyes University Medical Faculty, Kayseri, Turkey P1992
Introduction: Augmentation is described as RLS symptoms New perspectives in the treatment of
becoming more severe, occurring earlier and spreading to insomnia
previously unaffected body areas compared to before A. Tiugan1, C. Tiugan2
treatment. Pregabalin is a treatment option of RLS and 1Psychiatry, University of Medicine and Pharmacy,
presence of augmentation due to pregabalin has not been 2Psychiatry, Emergency Clinical Military Hospital Craiova,
reported in the literature until now. Romania
Case report: A 30-year-old female patient admitted with
tingling and burning complaints in both legs for two years.
These complaints occurred during resting and at night and P1993
relieved by leg movement completely or partially. The
Sleep disorders in patients with chronic
patient was diagnosed RLS regarding to clinical findings;
then pregabalin was administered for RLS treatment. At the cerebral ischemia
beginning of therapy, patients symptoms were highly N. Belova1, L. Belova1, Y. Nikitin2, V. Mashin1,
improved. At the third month of RLS treatment, the patient V. Abramova1, O. Kolotik-Kameneva1, A. Byirina1
reported the reoccurrence of symptoms like initiation of Medical Faculty, Ulyanovsk State University, Ulyanovsk,
1

treatment. At this time, complaints were more severe and Scientific Center for Neurology, Russian Academy of
2

beginning earlier at daytime, being less alleviated by legs Medical Science (RAMS), Moscow, Russia
movement and involved not only legs but also arms. Firstly,
this condition was thought to be RLS progression, and dose
of pregabalin was increased, but worsening of symptoms
P1994
was observed by increasing the dose of pregabalin. Restless leg syndrome can get relief with
Secondly, the dose of pregabalin was decreased and high orgasm: a case study with review of
relief in symptoms was observed. The condition was literature
diagnosed as RLS augmentation due to pregabalin
treatment. R. Bala, Electrophysiology and Sleep Lab Team
Conclusion: This case is the first RLS augmentation due to Medical Universityshakti Neuroscience, Hisar, India
pregabalin usage. Pathophysiology of both RLS and RLS
augmentation are uncertain with the idea that dopaminergic
mechanism is the essential pathology. However, occurrence
of augmentation due to pregabalin that has not a known
relationship with the dopaminergic system is very
interesting.

2012 EFNS European Journal of Neurology 19 (Suppl. 1), 90457

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