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Approach to Movement Disorders Approach to Movement Disorders u Voluntary or Involuntary u Suppressible u Psychogenic movement disorders u Hyperkinetic or Hypokinetic or Mixed u Characteristics & Natural History u Phenomenology Hyperkinetic Movement Disorders u Onset, duration, aggravating/ relieving factors Refresher Course 2562 u Distribution u Progression Assoc. Prof. Praween Lolekha, MD., MSc. u Associated Features, Medications Neurology division, Department of Internal Medicine, Thammasat University Presence of ≥ 1 movement disorders? Presence of ≥ 1 movement disorders? Identify all subtype of movement disorders? Dystonia, parkinsonism, tremor, ataxia Define the dominant Identify associated Identify associated Depression, anxiety, Pyramidal tract type of movement neurological non-neurological Dystonia cardiomyopathy, KF-rings, symptom disorder features features chronic hepatitis Clinical based syndrome Wilson’s Disease Diagnosis Wilson’s Disease Diagnostic work-up Ceruloplasmin, 24-hoururine copper Diagnosis Wilson’s Disease Tremor Defining tremors Rest Tremor Action Tremor u A rhythmic mechanical oscillation of at least one functional body region that is produced by alternating or synchronous contractions of Postural Tremor Kinetic Tremor opposing muscles. Parkinson’s Disease Drug-Induced Enhanced physiologic Simple kinetic u The most common movement disorders in Wilson’s Disease Drug Induced Intention Tremor adults. Severe Essential Tremor Essential Tremor (target-direct ) Neuropathy Task-specific Isometric Physiologic tremor High frequency tremor > 7 Hz - Physiologic tremor - ET u Present in every normal subject during postural/action - OT u Low amplitude, high frequency (6-12 Hz) u Enhanced physiologic tremor (EPT): u Easy visibility, predominant postural, high-frequency, PD 4-7 Hz of tremor <2 years, reversible Low frequency tremor u Endogenous/ exogenous intoxication < 4 Hz - Cerebellar tremor u Stress, anxiety - Holmes tremor u Hyperthyroidism - Palatal tremor u Caffeine u Drugs-induced tremor: valproate, AMT, lithium Movement Disorders, Vol. 13. Supplement 3. 1998 New Criteria of ET Fulfils consensus criteria for definite (classic) ET Tremor Parkinson’s Disease Essential Tremor Hands, legs, chin, lip, jaws Hands (usually symmetric) Distribution (usually asymmetric) head, voice u Hereditary ET Behavioral setting Resting tremor Postural +/- kinetic tremor u Family history of at least 1 first-degree affected relative Frequency 4-6 Hz 8-12 Hz u Onset of both must be before 65 years May lessen/ disappear with Amplitude Worsens ET progresses u Sporadic ET treatment, advanced PD Any age, common with u Does not have immediate family member with ET Age Typically onset in 60-70 advancing age u Onset is before 65 years May have positive, usually Family history Often positive (50%) u Senile ET negative Bradykinesia, rigidity, u Onset is after 65 years Associated features Absent as a rule shuffling gait u Family history may or may not be present Alcohol, beta-blockers, Responsive Levodopa antiepileptic Mov Disord 2009;24:2033-2041 Task/Position specific Holmes (midbrain) tremor tremor / Rubral tremor u The most common: primary writing tremor (PWT) u Rest and action (intension, postural) tremor u A variant of ET or writer’s cramp u Slow frequency (<4.5 Hz) and not regular u A non-progressive condition u More proximal tremor, sometime lower limb u Sporadic or dominantly inherited u Other brain stem signs: CN, nystagmus, dysarthria u Treatment: u Involvement of contralateral red nucleus and u Medication: response rate 50% cerebellar outflow tract (cerebellothalamic) u Anticholinergics, beta-blockers, primidone u Ischemic, hemorrhagic, mass, post traumatic u Levodopa, topiramate, benzodiazepines u Rx: Levodopa (30%) u Botulinum toxin injections u Thalamotoy , DBS (Vim) Palatal tremor (PT) Therapeutic options for tremor Palatal myoclonus (1-3Hz) ET OT Task Dystonic PD Cerebellar Holmes Neuro Propranolol XXXXXXXX u u Essential PT (25%) Symptomatic PT (75%) Primidone* XXXXX u Rhythmic contractions u Focal brainstem lesion: stroke, Gabapentin XX of tensor veli palatini encephalitis, demyelinating disease, Topiramate X (CN v3) mass, degenerative disease CBZ XX u Ear clicking u Guillain–Mollaret triangle (dentate (opening/closing nucleus-red nucleus - inferior olivary) Clonazepam XXXXX Eustachian tube) u Contractions of levator veli palatini Alprazolam X u Disappears with sleep (CN9,10,7) Levodopa XXXX u Frequency <2 Hz u Elevation of corneas/ soft palate, DA XXX nystagmus, face, tongue, neck, Anticholinergic XXXX diaphragm Botulinum XXX u Persist in sleep Tetrabenazine* X u Unilateral or bilateral involvements Clozapine X XX Neurosurgery XXXXXX Dystonia u Sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal postures u +/- dystonic tremor & dystonic jerk u Sensory tricks in localized dystonia (Alleviating maneuvers) Movement Disorders, Vol. 28, No. 7, 2013 Treatment Myoclonus u Focal dystonia u Generalized dystonia u Sudden, brief, jerky involuntary u Botulinum toxin u Levodopa movements , involving face, trunk, and extremities u Anticholinergic u Anticholinergic u Positive (muscular contraction) u u Baclofen u Baclofen Negative (muscular inhibitions) u e.g. asterixis, postural lapses u Benzodiazepines u Benzodiazepines/ u Surgery u Surgery Myoclonus Drug-induced Myoclonus u Physiologic myoclonus u Symptomatic myoclonus u Psychiatric medications u Sleep jerks, hiccup u Infection, postinfectious syndromes u SSRIs, MAOI, lithium, tricyclic u Essential myoclonus u Electrolyte disorders, u Drug withdrawal u Epileptic myoclonus renal/ hepatic failure u Calcium channel blockers u Drug induced syndromes u Narcotics u Post hypoxia (Lance- Adams syndrome) u Anticonvulsants u Paraneoplastic u Contrast media u Opsoclonus-myoclonus syndrome Myoclonus vs. seizure vs. tremor Treatment Myoclonus Seizure (EPC) Tremor Distribution Generalized or Focal , Unilateral or multifocal area Jacksonian bilateral limbs, u Treat the underlying disorder > focal spread or 2 nd head, chin u Correction of metabolic abnormalities Distal generalized u Rhythmicity Arrhythmic > Rhythmic Rhythmic Removal of an offending drug rhythmic u Symptomatic treatment Speed Fast, usually 10- 0.1-6Hz Slow, usually 4-8 u Consider antimyoclonic agents 50ms up to Hz 100ms Enhancing Postural, Spontaneous, Rest, postural, factors stimulus, action action, stimulus action EPC: epilepsia partilalis continua Antimyoclonic Agents Chorea & Ballism Myoclonus Treatment u Chorea : Cortical myoclonus Sodium valproate Clonazepam u Involuntary hyperkinetic movement disorder Piracetam consisting of sudden, irregular , flowing , purposeless Levetiracetam movements that are distally prominent Zonisamide u Athetosis : Brainstem myoclonus Clonazepam u A continuous stream of slow, sinuous movements, typically of the hands and feet Spinal myoclonus Clonazepam u Ballism : Peripheral myoclonus Botulinum toxin (for hemifacial spasm) u Proxima l, high amplitude, high velocity movements Chorea: Classification Chorea: treatment u Primary (genetic) u Secondary u Huntington’s disease u Vascular chorea u Depend on etiology u Neuroacanthocytosis u Autoimmune chorea: u Hyperglycemia : control blood sugar u McLeod syndrome Sydenham’s chorea u Sydenham’s chorea : observe, self limited u Benign hereditary chorea u Metabolic chorea: u Drug induced : stop medication hyperglycemia u Wilson’s disease u u Dentatorubralpallidoluysi Drug-induced chorea u Medication an atrophy (DRPLA) u Infectious chorea u Neuroleptic : Haloperidol u Atypical neuroleptics eg. Olanzapine, Risperidone Tics & Tourette Dyskinesia u Spontaneous, purposeless , u Complex involuntary movement: simple and complex Chorea(most), tremor, ballism, dystonia, tics, movements or myoclonus vocalizations that abruptly u Commonly used for drug induced abnormal interrupt normal motor movements activities u Drugs: Levodopa u Temporarily suppressible u Urge u Multiple body regions eg. Face, neck, shoulder, eye Tardive dyskinesia Paroxysmal dyskinesias u Usually Chorea u Abnormal involuntary movements that u Commonly Orobuccolingual are intermittent or episodic in nature , with sudden onset and with no change in u Risks: Old female consciousness u >3months dopaminergic blocking u Dystonia, chorea, ballismus, complex u Neuroleptic, antiemetic drugs combination of movements u idiopathic (primary) or secondary (symptomatic) Paroxysmal dyskinesias Secondary PK PKD PNKD PED PHD Duration Very brief 30 min – 1 hr (4hr) 2min- 2hrs 30-60sec Sudden Alcohol, coffee, Prolonged or Triggering movement: tobacco, sustained NREM sleep factor speed, force, emotional, hunger, exercise strength fatiuge 7-15 years Age at onset 2-79 years 2-30 years Adolescence (6mo-33yr) Sex M > F F > M Benzodiazepine, CBZ, PHT, anticonvulsant, Gabapentin, CBA, PHT, Treatment acetazolamide acetazolamide, L- L-dopa (20%) acetazolamide Topiramate, PB dopa Chr. 16p11 (60- 15q24, 20q13.2- Gene Chr.2q33-35 (AD) Chr.16p11 70%) 13.3 KD: kinesigenic dyskinesia, NKD: nonkinesigenic dyskinesia, ED: exercise-induced dyskinesia, HD: hypnogenic (noctunal) dyskinesia Movement Disorders, Vol. 17, No. 4, 2002 Thank you!.
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