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Surat Tanprawate, MD, FRCP(T) Division of Neurology, Chiang Mai University 11.05.2013
Outline
Clinical symptoms a/o syndromes
mood/affect lability, personality alterations, psychosis (hallucination/illusion), obsessive-compulsive disorder, dissociative disorder, alter sexual behavior/paraphilic disorder disorder of consciousness, delirium and dementia, amnestic disorders, aphasia, alexia, agraphia, apraxia, disorder of visual processing, frontal lobe syndrome
Common diseases
dementia, movement disorder, epilepsy, stroke, headache
Lability of affect
sudden changes in emotional expression eg. pseudobalbar palsy, epileptic seizure
Pseudobulbar palsy
Balbar: LatinBulb: A globular or fusiform anatomical structure or enlargement.Bulbus: swollen root
Bulbar palsy refers to bilateral impairment of function of the cranial V, VII, IX, X and XI, which occurs due to lower motor neuron lesion either at nuclear or fascicular level in the medullar oblongata or from bilateral lesions of the lower cranial nerves outside the brainstem
The Brainstem
Pseudobulbar palsy
Pseudobulbar palsy results from an upper motor neuron lesion to the corticobulbar pathway in the pyramidal tract.
Symptoms: -difficulty chewing, swallowing -slurred speech (often initial presentation) -inappropriate emotional outbursts.
Pathological Laughter
Pathological crying
Emotionalism is crying that is congruent with mood (sadness) but patient is unable to control crying as they would have before stroke. Catastrophic reaction is crying or withdrawal reaction triggered by a task made difficult or impossible by a neurologic deficit (e.g. moving a hemiplegic arm)
associated with post-stroke depression
Epileptic seizure
A gelastic seizure sudden burst of energy, usually in the form of laughing or crying Cause: hypothalamic hamartomas, temporal and frontal lobe lesion
Explosive
Posttraumatic encephalopathy, HD Plascidity
Visual pathway
Eye
Occipital cortex
Optic nerves
Temporal lobe
Geniculocalcarin e projections
Migraine Aura
99% 31%
18%
n=163
Michael B. R. et al. Brain 1996: 119, 355-361
Typical aura
Typical aura consisting of visual and/or sensory and/or speech symptoms. Gradual development, duration no longer than one hour, a mix of positive and negative features and complete reversibility characterize the aura which is associated with a headache that does not fulfill criteria for Migraine without aura.
Visual aura
Somesthetic metamorphopsia. Distortion of body and spaceDid Lewis Carroll draw inspiration from migraine auras?
Alice in Wonderland. By Lewis Carroll
The disease is named after the Swiss philosopher, who described the condition in 1769.
He first documented it in his 89-year-old grandfather who was nearly blind from cataract in both eyes but perceived men, women, birds, carriages, buildings, tapestries, physically-impossible circumstances and scaffolding patterns.
Described a 72-year-old womans visual hallucination of colorfully dressed people and children which occurred at dusk. The hallucinations occurred during normal conscious state and the patients neurological signs were associated with those characteristic of an infarct to the midbrain and pons. Von Bogaert, Lhermittes colleague, named these type of hallucinations peduncular in reference to the cerebral peduncle as well as to the midbrain and its surroundings.
Parkinsonism can be broadly divided as typical parkinsonism(Parkinsons disease) and atypical parkinsonism
Organic cause
less paranoid and thought disorder more insight associated with abnormal physical signs/symptoms more often nocturnal, and are associated with drowsiness
Disorder of consciousness
Clinical symptoms/syndrom es
Arousal and awareness, the two components of consciousness in coma, vegetative state, minimally conscious state, and locked-in syndrome.
Definition
Alert and aware, quadriplegic with lower CN palsy
Lesion
Bilateral anterior pontine
Comment
Similar state:severe polyneuropathy MG, NM blocking agent
Extensive cortical grey Synonyms include apallic and subcortical white syndrome, coma vigil, matter with relative cerebral cortical death preservation of brain stem
Abulia
Severe apathy, patient neither Bilateral frontal medial speak nor moves spontaneously
Severe case resemble akinetic mutism, but patient is alert and aware
Catatonia
Usually psychiatric
Pseudocoma
Feigned coma
Locked in syndrome
Apathy
DDx
Abulia
Motivational circuitry.
Robert S. Marin,. J Head Trauma Rehabil.2005: 4(20)377-388
Phineas P. Gage
Phineas P. Gage (1823 1860) was an American railroad construction foreman now remembered for surviving an accident in which a large iron rod was driven completely through his head, destroying much of his brain's left frontal.
Prefrontal cortex
Prefrontal cortex mediates complex human behavior, and three major behavioral syndromes associated with prefrontal dysfunction have been identified
Organization of the three frontal-subcortical circuits in which lesions produce alterations of cognition and emotion. VA indicates ventral anterior; MD, medial dorsal.
Cummings JL. Arch Neurol. 1993;50:873-880
Orbitofrontal cortex
disinhibition anosmia
Aphasia
Clinical symptoms/syndrom es
Aphasia
Aphasia refers to an impairment in linguistic communication produced by brain dysfunction
It must be distinguished from other disorders of verbal output such as dysarthria, mutism, and the abnormal language production of patients with thought disorder
Language test
Speech fluency
Comprehension Repetition Naming
Writing
Motor aphasia
Sensory aphasia
A: Wernicke's area B: concept center M: Broca's area a--> A -auditory input to Wernicke's area M --> m -motor output from Broca's area A --> M -tract connecting Wernicke's and Broca's areas A --> B -pathway essential for understanding spoken input B --> M -pathway essential for meaningful verbal output.
Conduction aphasia
Aphasia chart
Brocas aphasia
Broca had the opportunity to examine the brain of a language-impaired patient, M. Leborgne, when it came to autopsy. The patient had been capable of very little speech although his comprehension appeared well preserved
Wernickes aphasia
He saw a patient whose comprehension was severely impaired; when the patient came to autopsy, the lesion was discovered in the posterior, superior left temporal lobe Wernicke hypothesized that this area was the locus of storage of "auditory word images," which were necessary for the production as well as the comprehension of speech.
Stooped posture
Rigidity
Tremor
Reduce arm swing Hips and knees slightly flex Short shuffling steps
Parkinsonism
clinical syndrome of bradykinesia, resting tremor, cogwheel rigidity, and postural instability
Parkinsons disease
clinical syndrome of asymmetrical parkinsonism, usually with rest tremor, in association with the specific pathological findings of depigmentation of the SN as a result of loss of melanin-laden dopaminergic neurons containing eosinophilic cytoplasmic inclusions(Lewy bodies)
Primary or idiopathic parkinsonism Group Parkinsonsof disease Parkinsonism Secondary parkinsonism TYPICAL OR hydrocephalus, vascular CLASSIC
parkinsonism, encephalitis, drug induced parkinsonism
Hereditary parkinsonism
Parkinsons disease
Non-motor symptoms
Loss of sense of smell, constipation REM behavior disorder (a sleep
disorder)
Corticobasal degeneration
Common: typical neuroleptic antipsychotic drug, antidopaminergic antiemetic, reserpine (decrease presynaptic) Uncommon: SSRIs, lithium, phenytoin, methyldopa, valproic acid, flunarizine
Sign:
Tremor
Common diseases
Tremor
a rhythmic oscillation of a body part
produced by alternating or synchronous contraction of opposing muscles
other movement clinical symptoms can be act like tremor: dystonic tremor, myoclonic tremor
Terminology for tremor and the hierarchical relation of the terms as indicated by the numbers
Inspection
Frequency
Location
Head: chin, face, tongue, palate Upper extremity: shoulder, elbow, wrist, fingers Trunk Lower extremity: hip, knee, ankle joint, toes
Cerebellar signs
Pyramidal signs Neuropathic signs
Physiologic tremor
Rest
No
Anxiety aggravated
Response to betablocker
Postural, Kinetic
Hyperthyroid, tachycardia
Essential tremor
Postural, Kinetic
No
Parkinsonian tremor
Rest
Neurodegeneration
Cerebellar tremor
Ataxia
May plus with Various cause other neurological affected cerebellar deficit pathway
Essential tremor
Core criteria for identifying ET Bilateral action tremor of the hands and forearms Absence of other neurological signs, with the exception of the cogwheel phenomenon May have isolated head tremor with no abnormal posture
Essential tremor
Achimedes spiral
Treatment ET
First line
Second line
Gabapentin, topiramate, clozapine, long acting benzodiazepine (clonazepam)
Amnestic syndrome
NEJM 2004
Is it pseudo-dementia?
Term applied to apparent cognitive impairment associated with psychiatric disorders, most often depression (50-100%). Four criteria proposed by Caine (1981) for diagnosis
1) intellectual impairment in a patient with a primary psychiatric disorder 2) features of impairment are similar to those seen in CNS disorders 3) the cognitive deficits are reversible 4) there is no known neurological condition to account for the presentation Caine (1981)
depressed affect/mood neurovegetative signs slow, aspontaneous, monotonous speech long response latency frequent "I dont know" responses
Classification of dementia
Based on caused
Cerebral disorder (with or without extrapyramidal feature) VS systemic disorder Reversible-Arrestable VS irreversible disorder
Based on site
Cortical VS subcortical
Other
- Picks disease - Huntingtons chorea - Hereditary ataxia - Wilsons disease
Infarction,
hemorrhage
Endocrine and other organ failureHypothyroidismAdrenal insufficiency and Cushing syndromeHypo- hyperparathyroidismOrgan failure Renal failure, Liver failureToxic/ metabolicAlcoholic dementia , Drug medication and narcotic, Heavy metal intoxication, Dialysis dementia, Organic toxin, Porphyria
Treatable-Arrestable dementia
Tumor
Head trauma and diffuse brain damage Dementia pugilistica, Chronic subdural hematoma, Post anoxia, Post encephalitis
Lab evaluation
AAN guideline (Knopman et al. 2001) Routine screening
Clinical suspicious
Blood screening for syphilis
Others
Genetic testing: Apo E genotype CSF test: beta amyloid, tau, AD7C-NTP
Lab evaluation
AAN guideline (Knopman et al. 2001) Non-contrast CT brain or MRI scan in
most case
No disturbance of consciousness
Onset between ages 40 and 90, most often after age 65 Absence of systemic disorders or others brain diseases that could account for the deficits and progression
Neurology, Vol. 34, pp 939-944
Degenerative disorder
Familial
Sporadic
PD with dementia, Parkinson plus syndrome(PSP, CBD, MSA subtype), Dementia with Lewy bodies(DLB)
Secondary parkinsonism
Group of neurodegenerative dementia of varied etiology, in which the frontal a/o temporal lobes are relatively selectively affected, even into later stages of the disease, and are associated with varying amount of subcortical pathology and degeneration
Mistaken for a psychiatric disorder associated with inappropriate social behavior early affects those middle aged rather than the elder
Seizure:
the clinical manifestation of an abnormal and hypersynchronous discharge of a population of cortical neurons
Epilepsy:
Classification of Epilepsy/Seizure
Seizures (1981)
Based on clinical features and EEG findings. Replaces old terms such as Grand Mal, Petit Mal, psychomotor.
Epileptic disorders with similar signs, symptoms, prognosis and response to treatment. Important for choosing treatment options and counseling patients regarding etiology, genetics and likely prognosis etc.
Generalized Seizures:
Appear to arise simultaneously in both hemispheres
Unclassifiable Seizures:
Focal
Generalised
Undetermined
Special Syndromes
Idiopathic
Symptomatic/Cryptogenic
e.g. - TLE - FLE - PLE - OLE
Idiopathic
Absence vs CPS
Seizure vs Syncope
Epileptic vs Pseudoseizure
Headache disorder
Common diseases
http://ihs-classification.org
Investigation
(-)
(+)
Migraine headache
Cluster headache and other TACs
Focal neurologic s/s other than typical visual or sensory aura Papilledema Neck stiffness
Temporal profile
Worsening headache -Mass lesion, SDH, MOH
Concurrent event
Pregnancy, post partum -Cerebral vein thrombosis, carotid dissection, pituitary apoplexy Headache with cancer, HIV, systemic illness (fever, arteritis, collagen vascular disease)
Provoking activity
Triggered by cough, exertion or Valsava -SAH, mass lesion
Age> 50
Migraine with typical aura needs 2 attacks In children, the attack may last 1-72 hours
ICHD-II Cephalalgia.2004
Migraine management
Patient communication and education
Life style change and avoid trigger factors Acute headache management Prophylactic headache management
neuroleptic
Drugs
Amytriptiline (TCA)
Relative indications
Relative contraindication
Adverse effect
Other pain disorders, Mania, urinary Drowsiness, dry depression, anxiety, retention, heart blocks, mouth, increase insomnia glaucoma appetite, weight gain
Propranolol (Bblocker)
Hypertension, angina
Asthma, depression, CHF, Raynauds disease Obesity, depression, PD Liver disease, bleeding disorder
Fatique, lethargy, nausea, depression, dizziness Drowsiness, weight gain, depression, PD Nausea dyspepsia, sedation, increase appetite, weight gain Paresthesia, weight loss, alter taste, language disturbance
Topiramate (AED)