Shake It Off: Recognizing & Treating Movement Disorders

Transcription

1 Ooi Phaik Yee Annual scientific meeting College of Medicine, Academy of Medicine of Malaysia 12 th November 2017 Shake It Off: Recognizing & Treating Movement Disorders

2 MOVEMENT DISORDER A group of symptoms or syndromes characterised by involuntary or abnormal movements

3 Movement disorders:too fast or too slow

4 Hyperkinesias: excessive, abnormal, involuntary movements Tremor Chorea Myoclonus Dystonia Tics Abdominal dyskinesias Akathisic movements Asynergia, ataxia Athetosis Ballism Dysmetria Hemifacial spasm Hyperekplexia Myorhythmia Paroxysmal dyskinesias Restless legs Stereotypy Jumpy stumps Moving toes &/or fingers myokymia

5 Hypokinesias: decreased amplitude, slowness, loss of movement Akinesia, bradykinesia (parkinsonism) Apraxia Blocking (holding) tics Cataplexy & drop attacks Catatonia, psychomotor depression, & obsessional slowness Freezing phenomenon Hesitant gait Hypothyroid slowness Rigidity Stiff muscles

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7 APPROACH

8 Approach The clinical approach to movement disorders. Abdo, W.F. et al. Nat. Rev. Neurol. 2010

9 First and foremost: Establish phenomenology?hypokinesia? Hyperkinesia Which movement/s? Pattern recognition Localization focal, segmental, hemifocal, multifocal, generalized Characteristics Rhythmic/ non-rhythmic Speed amplitude In resting position/ evoked by action or increasing with action Spontaneous/ provoked Elementary/ complex Suppressibility paroxysmal

10 Tremor unintentional, rhythmic, regular, sinusoidal oscillation of a body part in a fixed plane. results from contractions of agonist & antagonist muscles At rest/ posture/ movement

11 Causes of tremor Rest tremor Parkinson s disease Drug induced parkinsonism Vascular parkinsonism Multiple system atrophy Spinocerebellar ataxia psychogenic Postural tremor Enhanced physiological tremor Drugs Toxins ( mercury, toluene, solvents) Metabolic : hyperthyroidism, Cushing s syndrome) Essential tremor Neuropathy Dystonia Task-specific tremors Parkinson s disease Orthostatic tremor psychogenic Kinetic tremor Cerebellar disease Holme s tremor Wilson s disease psychogenic Head tremor Essential tremor Dystonia Cerebellar disease 3 rd ventricular cysts Spasmus nutans Labyrinthine fistula Tic disorder Chin tremor Hereditary geniospasm Parkinson s disease Jaw tremor Parkinson s disease Essential tremor dstonia Palatal tremor Essential symptomatic

12 Parkinsonism Cardinal features Bradykinesia Rigidity Tremor Postural/ gait disturbance

13 Causes of parkinsonism Heredodegenerative Parkinson s disease Parkinson plus: progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration Alzheimer s disease Huntington s disease Parkinsonism-ALS-dementia complex of Guam Frontotemporal dementia with parkinsonism linked to chromosome 17 X-linked dystonia Parkinsonism Fahr s syndrome Fragile X tremor ataxia syndrome Dopa responsive dystonia Neuroacanthocytosis DRPLA SCA 3 Secondary causes Drugs ( dopamine receptor blocker) Toxins ( managanase, MPTP, mercury) Vascular disease Post-encephalitis (eg JE) Neurosyphilis Anoxic brain injury Traumatic brain injury Basal ganglia lesions Metabolic disorders eg Wilson s disease, GM1 gangliosidosis) Hydrocephalus Basal ganglia calcification

14 myoclonus Sudden, brief, shock-like involuntary movements caused by muscular contraction ( positive myoclonus) or inhibition ( negative myoclonus)

15 Causes of myoclonus Primary myoclonus Hiccups, hypnic jerks Benign infantile myoclonus with feeding/slep Essential myoclonus Myoclonus dystonia Cortical myoclonus Myoclonus with epilepsy Benign myoclonic epilepsy of infancy Juvenile myoclonic epilepsy Early infantile myoclonic encephalopathy West s syndrome Lennox-gastaut syndrome Epilepsia partialis continua Progressive myoclonic epilepsy with ataxia Lafora body disease Unverricht Lundborg disease Neuronal ceroid lipofuscinosis Sialidosis Myoclonic epilepsy with ragged red fibers Dentatorubropallidoluysian atrophy (DRPLA) Symptomatic myoclonus with encephalopathy Liver failure Renal failure Drug intoxication ( alcohol, lithium) Toxins ( lead poisoning) Post hypoxic ( Lance Adam syndrome) Progressive encephalomyelitis with rigidity Symptomatic myoclonus without encephalopathy Plus dementia: Alzheimer s disease, dementia with lewy bodies, Creutzfeld Jakob disease Plus parkinsonism: corticobasal degeneration, multiple system atrophy, spinocerebellar ataxias Focal/segmental: spinal cord/root/ plexus injury Palatal myoclonus Others: Whipple disease, coeliac disease, paraneoplastic, drugs

16 chorea involuntary, brief, abrupt, irregular, unpredictable, nonstereotyped movements may appear purposeful, fidgety & clumsy Flow randomly from one part of body to another may occur with athetosis & ballism

17 Inherited or degenerative Huntington s disease HD-like syndrome Wilson s disease Neuroacanthocytosis McLeod s syndrome Dentatorubropallidoluysian atrophy Lesch-Nyhan syndrome Benign hereditary chorea Ataxia telangiectasia Amino acid disorders Spinocerebellar ataxia (SCA 17) Autoimmune/ postinfectious Systemic lupus erythematosus Antiphospholipid syndrome Bechet s syndrome Coeliac disease Hashimoto s thyroiditis Vasculitis Syndenham chorea PANDAS Infectious HIV Abscesses in the basal ganglia from any cause Creutzfeld Jakob disease (nvcjd) Causes of chorea Drugs Dopamine receptor blocking drugs Levodopa Stimulants Oral contraceptive pills Anticonvulsants Anticholinergics Ca channel blocker Structural lesions Lesions in the basal ganglia from any cause ( usually caudate nucleus) Metabolic Disorders of thyroid, parathyroid, glucose, sodium, calcium, magnesium Chorea gravidarum Paroxysmal Paroxysmal kinesigenic dyskinesia Paroxysmal non-kiesigenic dyskinesia Others Post cardiac bypass (post pump syndrome)

18 dystonia sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both typically patterned, twisting, & may be tremulous Typically happens in action Athetosis: slow, writhing, continuous movements predominantly of distal extremities Sensory trick: geste antagoniste

19 Causes of dystonia Primary dystonia Young onset: primary torsional dystonia Adult onset Task speficic Focal Rare primary dystonias Dystonia plus syndromes Dopa responsive dystonia Myoclonic dystonia Secondary dystonia Brain injury ( putamen) Drugs: acute and delayed ( tardive) Post encephalitis Paroxysmal dystonia Paroxysmal exercise induced dystonia Paroxysmal kinesigenic dyskinesia Paroxysmal non-kinesigenic dyskinesia Heredo-degenerative Parkinson s disease Corticobasal degeneration Progressive supranuclear palsy Multiple system atrophy Huntington s disease Wilson s disease Spinocerebellar ataxias Neuronal brain iron accumulation syndrome DRPLA Neuroacanthocytosis X-linked dystonia parkinsonism (Lubag) Rapid onset dystonia parkinsonism Mohr Tranebjaerg syndrome ( deafness dystonia) Metachromatic leukodystrophy GM1/GM2 ganglosidosis Amino acidemias Niemann pick type C Ataxia telangiectasia Psychogenic dystonia

20 Power of observation Knowing what you are looking for Conclusion Pattern recognition Focused, systematic approach investigational work-up can be greatly simplified and focused once the type of movement disorder has been defined properly

21 Thank you