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Movement Disorders Professor Tim Anderson Neurologist University of Otago Christchurch 11:00 - 11:55 WS #91: Shakes Jerks and Spasms - Recognition and Differential Diagnosis 12:05 - 13:00 WS #102: Shakes Jerks and Spasms - Recognition and Differential Diagnosis (Repeated) Shakes, Jerks and Spasms Recognition and differential diagnosis Tim Anderson Christchurch Phenomenology hyperkinetic hypokinetic disorders disorders ▪ tremor ▪ bradykinesia ▪ jerks ▪ spasms ▪ rigidity ▪ stereotypy ▪ postural instability ▪ akithisia Types of Movement Disorders Tremor Jerks Spasms Types of Movement Disorders ✓ Tremor Jerks Spasms Tremor “rhythmical oscillation of a body part” ▪ tremor frequency is usually not relevant ▪ diagnosis of cause is on the basis of: the type of tremor the associated features Tremor - Classification ▪ Rest ▪ Action Tremor - Classification ▪ Rest ▪ Action Postural Kinetic (tremor during movement) Simple kinetic (non goal directed) Intention (goal directed) Task specific isometric Tremor types (Rest) ▪ Rest Tremor ✓The only true rest tremor is Parkinsonian Parkinson’s Disease Bradykinesia (slowness) Parkinson’s Disease Tremor Types (postural) ▪ Rest tremor ▪ ActionTremor Postural Kinetic Postural Tremor • Essential tremor • Enhanced Physiological • Drugs • hyperthyroidism • brainstem/red nucleus • Wilson's disease “Tremor present on sustained posture “ Tremor Types (intention) ▪ Rest ▪ Action Postural Kinetic Simple kinetic (non goal directed) Intention (goal directed) Task specific isometric Intention Tremor ✓ Brainstem disease, or ✓ Cerebellar disease • Multiple sclerosis • Cerebellar degeneration “ Tremor during target-directed movements” Tremor Syndromes Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes: enhanced physiologic tremor classical essential tremor (ET) primary orthostatic tremor task- and position-specific tremors dystonic tremor tremor in Parkinson's disease cerebellar tremor Holmes' tremor palatal tremor drug-induced and toxic tremor tremor in peripheral neuropathies psychogenic tremor. Classical Essential Tremor Essential Tremor ▪ 50% hereditary (no specific gene established) ▪ 50% alcohol responsive ▪ always involves the hands (postural) symmetrically then head, tongue, voice (rarely jaw or legs) head tremor usually side-to-side ▪ no other neurologic features ▪ note! can get "cogwheeling" at wrists (due to superimposed tremor) Louis EL. Lancet Neurology 2005; 4:100-10 Essential Tremor Treatment oral medications ▪ Gabapentin ▪ Propranolol 300mg, 1200– 3600mg 10mg/day = start dose 160–320mg = normal ▪ Topiramate daily dose 25mg, 200–400mg ▪ Primidone ▪ Alprazolam 62·5mg, 62·5–1000mg 0·75mg, 0·75– 2·75mg ▪ Nimodipine 120mg, 120mg Essential Tremor Treatment Other therapies ▪ Botulinum toxin Into forearm muscles Reduces tremor but no functional improvement Better for head tremor ▪ Thalamotomy ▪ Thalamic deep brain stimulation (DBS Tremor Syndromes Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes: enhanced physiologic tremor classical essential tremor (ET) primary orthostatic tremor task- and position-specific tremors dystonic tremor tremor in Parkinson's disease cerebellar tremor Holmes' tremor palatal tremor drug-induced and toxic tremor tremor in peripheral neuropathies psychogenic tremor. primary orthostatic tremor Treatment: clonazepam, L-dopa, gabapentin Tremor Syndromes Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes: enhanced physiologic tremor classical essential tremor (ET) primary orthostatic tremor task- and position-specific tremors dystonic tremor tremor in Parkinson's disease cerebellar tremor Holmes' tremor palatal tremor drug-induced and toxic tremor tremor in peripheral neuropathies psychogenic tremor. task and position-specific tremors e.g. vocal tremor and writing tremor Tremor Syndromes Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes: enhanced physiologic tremor classical essential tremor (ET) primary orthostatic tremor task- and position-specific tremors dystonic tremor tremor in Parkinson's disease cerebellar tremor Holmes' tremor palatal tremor drug-induced and toxic tremor tremor in peripheral neuropathies psychogenic tremor. dystonic tremor Tremor Syndromes Additional data from a medical history and the results of a neurologic examination can be combined into one of the following clinical syndromes: enhanced physiologic tremor classical essential tremor (ET) primary orthostatic tremor task- and position-specific tremors dystonic tremor tremor in Parkinson's disease cerebellar tremor Holmes' tremor palatal tremor drug-induced and toxic tremor tremor in peripheral neuropathies psychogenic tremor. cerebellar tremor Types of Movement Disorders Tremor ✓ Jerks Spasms Types of Jerk ▪ Chorea “continuous flow of brief jerky movements which flit from one part of the body to the next in random fashion” Chorea “continuous flow of brief jerky movements which flit from one part of the body to the next in random fashion” Types of Jerk ▪ Chorea ▪ Huntington’s disease ▪ Sydenham’s chorea ▪ Drugs Levodopa Antipsychotics Anticholinergics ▪ Stroke Treatment Tetrabenazine 12.5mg od max 50mg tds athetosis from stroke Chorea from L-dopa Jerks ✓ chorea myoclonus tics “continuous flow of brief jerky movements which flit from one part of the body to the next in random fashion” Types of Jerk ▪ Chorea ▪ Myoclonus “rapid shock-like muscle jerks, often repetitive, sometimes rhythmic” Myoclonus “rapid shock-like muscle jerks, often repetitive, sometimes rhythmic” Types of Jerk ▪ Chorea ▪ Can be due to many brain ▪ Myoclonus and spinal cord disorders ▪ After cardiac arrest ▪ drugs Treatment 1. clonazepam 0.25mg od 2mg tds 2. sodium valproate 200mg od 500mg tds 3. levetiracetam 500mg 1000mg bd Types of Jerk ▪ Chorea ▪ Myoclonus ▪ Tics “repetitive, stereotyped jerks which can be mimicked voluntarily, and briefly held in check” Types of Jerk ▪ ▪ Predilection around face/head ▪ ▪ Single (simple) or multiple ▪ Common in childhood – transient ▪Tics ▪ Tourettes Syndrome Start in childhood Multiple motor tics Sonic tics Improves in adulthood Types of Jerk ▪ Chorea ▪ Myoclonus Treatment (if necessary) • tetrabenazine ▪Tics • clonidine • haloperidol • dopamine agonists • CBT (esp. teens) Types of Movement Disorders Tremor Jerks ✓ Spasms = Dystonia Dystonia (spasms) "sustained contraction of voluntary muscles causing twisting and repetitive movements or abnormal posture” Treatment ▪ anticholinergics e.g. procyclidine, benztropine ▪ botulinum toxin Dystonia - classification Focal Segmental ▪ spasmodic torticollis (cervical ▪ Meige syndrome dystonia) ▪ cranio-cervical dystonia ▪ blepharospasm ▪ oromandibular dystonia Generalised ▪ idiopathic torsion dystonia ▪ spasmodic dysphonia (laryngeal dystonia) Hemidystonia ▪ dystonic writers cramp ▪ post stroke ▪ Other structural lesions ▪ limb dystonia focal and segmental dystonia Hemidystonia and generalised dystonia stereotypies Huntington’s disease Intellectually impaired stereotypies – post stroke.
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