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1 Department of Rehabilitation Sciences, Katholieke Universiteit Leuven, Leuven. 2 Dominiek Savio Institute, DC GID(t)S Gits. 3 Department of Paediatric Neurology, University
Hospitals Leuven, Leuven. 4 Clinical Motion Analysis Laboratory Leuven, University Hospital Pellenberg, Leuven. 5 Department of Neurology, Hpital Universitaire des Enfants
Reine Fabiola, Brussels. 6 Faculty of Medicine, Universit libre de Bruxelles, Brussels, Belgium. 7 Department of Child and Adolescent Studies, Utrecht University, Utrecht, the
Netherlands. 8 Centre for Developmental Disabilities, University Hospitals Leuven, Leuven. 9 Faculty of Medicine, Katholieke Universiteit Leuven, Leuven, Belgium.
Correspondence to Mr Elegast Monbaliu at Dominiek Savio Instituut, Koolskampstraat 24 8830 Gits, Belgium. E-mail: elegast.monbaliu@faber.kuleuven.be
PUBLICATION DATA AIM The aim of this study was to examine the reliability and validity of the Dyskinesia Impairment
Accepted for publication 25th September 2011. Scale (DIS). The DIS consists of two subscales: dystonia and choreoathetosis. It measures both
Published online 16th January 2012. phenomena in dyskinetic cerebral palsy (CP).
METHOD Twenty-five participants with dyskinetic CP (17 males; eight females; age range 522y;
ABBREVIATIONS mean age 13y 6mo; SD 5y 4mo), recruited from special schools for children with motor disorders,
BADS BarryAlbright Dystonia Scale were included. Exclusion criteria were changes in muscle relaxant medication within the previous
DIS Dyskinesia Impairment Scale 3 months, orthopaedic or neurosurgical interventions within the previous year, and spinal fusion.
ICC Intraclass correlation coefficient Interrater reliability was verified by two independent raters. For interrater reliability, intraclass
MDD Minimal detectable difference correlation coefficients were assessed. Standard error of measurement, the minimal detectable
SCPE Surveillance of Cerebral Palsy in
difference, and Cronbachs alpha for internal consistency were determined. For concurrent validity
Europe
of the DIS dystonia subscale, the BarryAlbright Dystonia Scale was administered.
RESULTS The intraclass correlation coefficient for the total DIS score and the two subscales
ranged between 0.91 and 0.98 for interrater reliability. The reliability of the choreoathetosis
subscale was found to be higher than that of the dystonia subscale. The standard error of the
measurement and minimal detectable difference values were adequate. Cronbachs alpha values
ranged from 0.89 to 0.93. Pearsons correlation between the dystonia subscale and BarryAlbright
Dystonia Scale was 0.84 (p<0.001).
INTERPRETATION Good to excellent reliability and validity were found for the DIS. The DIS may
be promising for increasing insights into the natural history of dyskinetic CP and evaluating
interventions. Future research on the responsiveness of the DIS is warranted.
Cerebral palsy (CP) is worldwide the most common neuromo- due to sustained muscle contractions. Choreoathetosis in CP
tor disorder in children, with an incidence of 2 to 3 per 1000 is dominated by hyperkinesia and tone fluctuation (but mainly
live births.1,2 CP can be categorized into spastic, dyskinetic, decreased). Chorea refers to rapid, involuntary, jerky, often
and ataxic groups. Dyskinetic CP is further differentiated into fragmented movements. Athetosis means slower, constantly
dystonia and choreoathetosis.1,3 changing, writhing, or contorting movements.6,7 These SCPE
Spastic CP is by far the most common type of CP, with a descriptions are in accordance with the recently published
prevalence of approximately 80%,4 and is followed by dyski- definitions of dystonia, chorea, and athetosis by the Taskforce
netic CP with a prevalence between 6.5%5 and 14.4%.4 on Childhood Movement Disorders.8,9 The Taskforce defines
According to the Surveillance of Cerebral Palsy in Europe dystonia as a movement disorder in which involuntary sus-
(SCPE),6 dyskinetic CP is characterized by involuntary, tained or intermittent muscle contraction causes twisting and
uncontrolled, recurring, occasionally stereotyped movements, repetitive movements, abnormal postures, or both,8 chorea as
in which the primitive reflex patterns predominate and muscle an ongoing, randomly appearing sequence of (one or more)
tone varies.6 The SCPE described dystonia in CP as domi- discrete involuntary movements or movement fragments, and
nated by abnormal postures that may give the impression of athetosis as a slow continuous, involuntary writhing move-
hypokinesia and muscle tone that is fluctuating (but with easily ment that prevents maintenance of a stable posture.9 The defi-
elicitable tone increase). Characteristics are involuntary move- nitions of the SCPE and the Taskforce describe dystonia and
ments, distorted voluntary movements, and abnormal postures choreoathetosis in a very similar way and are essentially
278 DOI: 10.1111/j.1469-8749.2011.04209.x The Authors. Developmental Medicine & Child Neurology 2012 Mac Keith Press
descriptive, based on consensus emerging from experts from What this paper adds
different clinical and basic fields of science. Good to excellent reliability and validity was found for a new clinical scale
Over the last few years, there has been continuing develop- evaluating dyskinesia in cerebral palsy.
ment of interventions in children with dyskinetic CP, includ- This is the first scale that independently measures dystonia and choreoathetosis
ing intrathecal baclofen,1012 deep brain stimulation,13,14 oral in dyskinetic cerebral palsy.
The reliability of the choreoathetosis subscale was found to be higher than
medication,1517 ventral rhizotomies,18 and botulinum toxin that of the dystonia subscale.
injections.19 However, objective evidence supporting these
interventions is only preliminary. Specific assessment of dysto- ited or no differentiation between action and rest or duration
nia has mostly relied on the BarryAlbright Dystonia Scale and amplitude, and combined several dyskinesia characteristics
(BADS).20 Operationally, the BADS has become a criterion within one score, which may limit the sensitivity of the scales
standard for scoring dystonia in CP, but several studies1017 (see Table I). Additionally, we explored the content and scale
have reported the difficulty of measuring dystonia reliably construct of the Toronto Western Spasmodic Torticollis Rat-
and or questioned the sensitivity of the BADS. ing Scale25 and the Unified Parkinsons Disease Rating Scale26
In a recent study,21 the reliability and validity of the BADS (Movement Disorder Society). Based on this analysis and the
was reassessed and special attention was given to the sensitivity SCPE definitions of dystonia, choreoathetosis, and dyskinetic
of the scale. This study showed reliability results similar to CP,3,6,7 the DIS was developed according to the methodologi-
those of Barry et al.20 but also revealed limitations in the sensi- cal framework of Kirshner and Guyatt.27 Its content was thor-
tivity of the BADS. oughly discussed with a clinical expert team (EO, JD, HF, PD,
Content analysis showed that the BADS included several and FR) from the Cerebral Palsy Reference Centre (University
dystonia characteristics over eight body regions. However, the Hospital Pellenberg, Leuven, Belgium).
items are a combination of several different dystonia charac- In a second step, the interrater reliability of this scale was
teristics within one score (e.g. duration and amplitude) and no assessed in a pilot study. Four physical therapists with exten-
differentiation is made between rest and activity. Also, for the sive clinical experience of children with CP (UH, IV, ES, and
first time, the measurement error of the BADS was assessed ED) underwent a training session with the reference and train-
and a high standard error of measurement (SEM) and minimal ing DVD of the SCPE6 and were instructed on how to use the
detectable difference (MDD) were found, respectively 6% and preliminary constructed scale. They then scored 10 videotaped
18%. In clinical practice, this means that a score difference of children with dyskinetic CP independently. Afterwards, the
18% is necessary to ascertain that true improvement has content of the scale, the included items, and the scoring crite-
occurred, as lower values might be ascribed to measurement ria were discussed with these four raters and the clinical expert
errors. Also in this study,21 two primary dystonia scales were team. Subsequently, the discussion together with (1) the num-
evaluated in dyskinetic CP, namely the BurkeFahnMarsden ber of participants able to accomplish the task, (2) the reliabil-
Movement Scale22 and the Unified Dystonia Rating Scale.23 ity of the item scores, and (3) the participants clinical
For these scales, even higher MDDs were found, 27% and experience, ensured that an item reduction was obtained and
25% respectively.21 Finally, several groups have emphasized that the scoring criteria and instructions were revised.
that dystonia and choreoathetosis often occur concurrently in The final DIS (Appendix I, supporting information pub-
dyskinetic CP9,21,24 However, to our knowledge no standard- lished online) consists of two subscales, one for dystonia and
ized tools for measuring choreoathetosis in CP have been one for choreoathetosis (see Fig. 1). Both subscales evaluate
validated. duration and amplitude in 12 body regions including the eyes,
For these reasons, we have strived to develop a new assess- mouth, neck, trunk, and limbs. For the limbs, a distinction is
ment tool to score dystonia and choreoathetosis at rest and made between the proximal and distal region and between the
during activity in individuals with dyskinetic CP. We right and left side. For each of the assessed body regions, the
attempted to enhance the sensitivity of this tool in comparison duration refers to the amount of time that dyskinesia is pres-
with the commonly used dystonia scales. In this paper, we ent, whereas the amplitude aspects refer to the range of
describe how we developed the DIS and assessed its reliability motion of the dyskinetic movements. All body regions are
and validity. scored during two activities (action) and one resting posture
(rest). Summation of the region scores gives a total action
METHOD score (range 0192) and a total rest score (range 096) for both
Development of the dyskinetic impairment scale subscales. The action and rest scores add up to a total
One of the first steps in the development of the DIS consisted score for dystonia and choreoathetosis, each with a range from
of a content analysis of the three available secondary and pri- 0 to 288. The total DIS score is the sum of the dystonia and
mary dystonia scales.20,22,23 In accordance with Sanger et al.,9 choreoathetosis subscale.
movements can be described by the context in which they
occur, for example postural, rest, action, or associated with spe- Reliability and validity
cific tasks.9 Dyskinesia characteristics can be assessed at rest Participants
and during activity and in terms of duration, amplitude, and This study included 25 participants aged between 5 and 22
influence on functional activities. From this point of view, con- years (17 males; eight females; mean age 13y 6mo; SD 5y
tent analysis revealed that the three scales analysed made lim- 4mo). All participants were diagnosed by a paediatric neuro-
Discrimination
BFMS + ) ) 9 + ) ) )
UDRS + ) ) 14 + ) + +
BADS ) + ) 8 + ) ) )
DIS ) + + 12 + + + +
DIS
logist and were recruited from special schools for children the presence of their own physiotherapist. The duration of
with motor disabilities. Individual participant characteristics videotaping was similar to the duration in other dystonia
are presented in Appendix II (supporting information pub- scales (e.g. Unified Dystonia Rating Scale, BADS, Burke
lished online). Exclusion criteria were changes in muscle relax- FahnMarsden Movement Scale), with a maximum of 30
ant medication within the previous 3 months, orthopaedic or minutes. The passive range of motion of the upper and lower
neurosurgical interventions within the previous year, and spine limb joints was measured with a goniometer to serve as a
fusion. Ethical approval was obtained from the Ethical Com- baseline for the amplitude assessment of the DIS. Afterwards,
mittee of the Katholieke Universiteit Leuven. All participants a video montage was made in accordance with the scoring
and or their parents provided informed consent. order of the DIS.
To assess interrater reliability, two physical therapists (EM,
Procedure JV) scored all videos in series within 15 days. The two raters
Based on the recommendations of the Dystonia Study had experience in discriminating dystonia and choreoathetosis
Group,23 the 25 participants were videotaped (by ES and in CP and were trained in scoring with the DIS.
MV) according to a standard video protocol. It contained all To assess concurrent validity, the second rater (JV)
postulated activities and rest postures of the DIS (see Appen- scored the BADS for all 25 participants. The BADS evalu-
dix III, supporting information published online). An effort ates dystonia over eight body regions on a five-point ordi-
was made to provide relaxing surroundings. All participants nal scale. The video protocol was also used to assess the
were filmed in their habitual environment at school and in BADS scale.
Table II: Interrater reliability: intraclass correlation coefficients (ICC) with 95% confidence intervals (CI) between raters for the Dyskinesia Impairment Scale
Active Rest
P P
Duration (D) Amplitude (A) (D+A) Duration (D) Amplitude (A) (D+A)
ICC 95% CI ICC 95% CI ICC 95% CI ICC 95% CI ICC 95% CI ICC 95% CI
Dystonia subscale
1 Eyes 0.50 0.140.74 0.55 0.210.76 0.63 0.310.82 0.75 0.520.88 0.84 0.660.93 0.79 0.590.90
2 Mouth 0.73 0.480.87 0.78 0.570.90 0.86 0.700.93 0.75 0.500.88 0.67 0.380.84 0.75 0.510.88
3 Neck 0.50 0.140.75 0.66 0.370.84 0.61 0.290.81 0.56 0.220.78 0.64 0.34-.083 0.65 0.350.83
4 Trunk 0.49 0.120.74 0.48 0.110.73 0.54 0.190.77 0.72 0.450.86 0.81 0.620.91 0.78 0.570.90
5 Arm RP 0.62 0.300.81 0.47 0.110.73 0.51 0.150.75 0.88 0.740.94 0.81 0.610.92 0.87 0.730.94
6 Arm LP 0.86 0.710.94 0.70 0.430.86 0.79 0.570.90 0.91 0.800.96 0.83 0.660.92 0.88 0.750.95
7 Arm RD 0.98 0.970.99 0.90 0.780.95 0.99 0.970.99 0.81 0.610.91 0.88 0.750.95 0.87 0.720.94
8 Arm LD 0.99 0.991.00 0.99 0.981.00 1.00 0.991.00 0.69 0.410.85 0.86 0.700.93 0.79 0.570.90
9 Leg RP 0.64 0.330.82 0.47 0.100.73 0.60 0.280.80 0.55 0.210.77 0.71 0.440.86 0.64 0.340.82
10 Leg LP 0.62 0.300.81 0.47 0.100.73 0.56 0.220.78 0.68 0.390.84 0.67 0.380.84 0.80 0.600.90
11 Leg RD 0.70 0.430.86 0.65 0.350.83 0.75 0.510.98 0.70 0.43-.086 0.87 0.720.94 0.81 0.620.91
12 Leg LD 0.77 0.550.89 0.61 0.300.81 0.78 0.580.90 0.46 0.080.72 0.75 0.500.80 0.64 0.400.82
Total score 0.87 0.730.94 0.87 0.720.94 0.88 0.740.94 0.90 0.790.96 0.94 0.870.97 0.850.97
Choreoathetosis subscale
1 Eyes 0.60 0.280.80 0.50 0.130.74 0.57 0.230.78 0.71 0.440.86 0.48 0.110.73 0.67 0.380.84
2 Mouth 0.87 0.720.94 0.71 0.440.86 0.81 0.620.92 0.90 0.800.97 0.84 0.680.93 0.93 0.850.97
3 Neck 0.81 0.620.91 0.76 0.530.89 0.85 0.690.93 0.83 0.650.92 0.80 0.600.91 0.84 0.680.93
4 Trunk 0.87 0.720.94 0.64 0.500.90 0.80 0.600.91 0.81 0.610.91 0.77 0.550.89 0.81 0.620.91
5 Arm RP 0.89 0.740.94 0.92 0.820.96 0.91 0.810.96 0.85 0.690.93 0.87 0.720.94 0.95 0.790.96
6 Arm LP 0.91 0.800.96 0.88 0.740.94 0.92 0.820.96 0.84 0.680.93 0.89 0.760.95 0.86 0.710.94
7 Arm RD 0.81 0.610.91 0.89 0.760.95 0.89 0.750.95 0.85 0.700.93 0.94 0.860.97 0.94 0.870.97
8 Arm LD 0.89 0.770.95 0.86 0.720.94 0.89 0.760.95 0.94 0.880.98 0.86 0.710.94 0.94 0.870.97
9 Leg RP 0.81 0.620.91 0.81 0.620.91 0.81 0.620.91 0.96 0.900.98 0.73 0.480.87 0.90 0.790.96
10 Leg LP 0.73 0.730.94 0.78 0.560.90 0.86 0.700.93 0.83 0.650.92 0.75 0.520.88 0.86 0.710.94
11 Leg RD 0.85 0.690.93 0.73 0.470.87 0.81 0.620.91 0.88 0.750.95 0.74 0.500.88 0.85 0.690.93
12 Leg LD 0.59 0.280.79 0.60 0.280.80 0.64 0.330.82 0.92 0.830.96 0.79 0.580.90 0.88 0.740.94
Total score 0.97 0.920.98 0.94 0.860.97 0.96 0.920.98 0.96 0.920.98 0.93 0.850.97 0.96 0.920.98
RP, right proximal; LP, left proximal; RD, right distal; LD, left distal.
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