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MYOCLONUS PRESENTED BY MATTHEW EBRIGHT PGY-2. MYOCLONUS - OUTLINE Definition Pathophysiology Classification Etiologies Specific Examples Evaluation Treatment.

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Presentation on theme: "MYOCLONUS PRESENTED BY MATTHEW EBRIGHT PGY-2. MYOCLONUS - OUTLINE Definition Pathophysiology Classification Etiologies Specific Examples Evaluation Treatment."— Presentation transcript:

1 MYOCLONUS PRESENTED BY MATTHEW EBRIGHT PGY-2

2 MYOCLONUS - OUTLINE Definition Pathophysiology Classification Etiologies Specific Examples Evaluation Treatment Videos

3 DEFINE MYOCLONUS What is it? Rapid Brief Involuntary Contraction One or multiple muscles, around a joint Symmetry? Synchrony? Differential Diagnosis?

4 PATHOPHYSIOLOGY Not entirely clear! Reduced intracortical inhibition Hyper-excitable sensorimotor cortex Cerebellar degeneration Striatal lesions

5 HOW TO CLASSIFY MYOCLONUS 1.Anatomic localization 2.Time course 3.Triggers 4.Other general clinical features

6 HOW TO CLASSIFY MYOCLONUS 1.Anatomic localization 2.Time course 3.Triggers 4.Other general clinical features

7 LOCALIZATION Is it… Focal Segmental Multi-focal Diffuse/generalized

8 LOCALIZATION Cortical Diffuse vs focal Sensorimotor cortex Subcortical Cortical-subcortical and subcortical-nonsegmental Segmental Brainstem Spine

9 HOW TO CLASSIFY MYOCLONUS 1.Anatomic localization 2.Time course 3.Triggers 4.Other general clinical features

10 TIME COURSE Non-progressive Spinal, benign rolandic epilepsy, JME, post-hypoxic, iatrogenic, metabolic Progressive Neurodegenerative, epilepsia partialis continua Rapid onset or progression CJD, SSPE, post-infectious, anti-NMDA, metabolic, psychogenic

11 HOW TO CLASSIFY MYOCLONUS 1.Anatomic localization 2.Time course 3.Triggers 4.Other general clinical features

12 TRIGGERS 1.Spontaneous 2.Action-induced 3.Sensory-induced

13 HOW TO CLASSIFY MYOCLONUS 1.Anatomic localization 2.Time course 3.Triggers 4.Other general clinical features

14 CLINICAL GROUPING Primary Secondary

15 PRIMARY MYOCLONUS Physiologic Essential/hereditary Myoclonus-dystonia syndrome Hyperekplexia Epileptic EPC, benign rolandic epilepsy, juvenile myoclonic epilepsy, familial cortical tremor with epilepsy Primary progressive myoclonus of aging

16 SECONDARY MYOCLONUS Progressive myoclonic encephalopathies and storage diseases Lafora body MERRF Lipofuscinosis Unverricht disease Ramsay Hunt Krabbe Tay Sachs Sandhoff Sialidosis (cherry-red spot myoclonus) Gaucher type III Dentatorubrual-pallidoluysian atrophy

17 SECONDARY MYOCLONUS Neurodegenerative diseases Alzheimer dementia Parkinson disease Dementia with Lewy bodies Corticobasal degeneration Multiple system atrophy Frontotemporal dementia

18 SECONDARY MYOCLONUS Infectious/Inflammatory Encephalitis (NMDA encephalitis) Post-infectious SSPE Opsoclonus-myoclonus syndrome

19 SECONDARY MYOCLONUS Metabolic Liver, renal, dialysis, sodium, glucose, Celiac, Whipples Drugs/Toxins Anti-convulsants, anti-psychotics, anesthetics, morphine, levodopa, contrast, heavy metals Nervous System Damage Post-hypoxic, traumatic, stroke, spinal cord lesions, demyelinating disease Psychogenic

20 SPECIFIC EXAMPLES Myoclonus-dystonia Syndrome of essential myoclonus Action-induced multifocal myoclonus Cervical dystonia or writer’s cramp Autosomal dominant Alcohol-responsive

21 SPECIFIC EXAMPLES Post-Hypoxic Myoclonus (Lance-Adams Syndrome) Subcortical, multifocal Action-induced, exquisitely Days to weeks after recovery

22 SPECIFIC EXAMPLES Neurodegenerative Diseases PD – levodopa, amantadine, severe late disease Corticobasal degeneration – action induced and stimulus sensitive MSA – low-amplitude, irregular, arrhythmic, hands and fingers, face LBD CJD – aggressive, progressively multifocal

23 SPECIFIC EXAMPLES Opsoclonus-myoclonus ataxia syndrome Chaotic myoclonic eye movements Oscillopsia Multifocal, action-related myoclonus Paraneoplastic or post-infectious

24 SPECIFIC EXAMPLES Hyperekplexia Autosomal dominant Stiff babies Episodic tonic spasms Nocturnal myoclonus Exaggerated startle responses Brainstem localization Non-habituating Overly-sensitive to stimuli May persist through life

25 SPECIFIC EXAMPLES Canine myoclonus Distemper - RNA virus Multi-system disease Encephalomyelitis Myoclonus, seizures, rapid neurodegeneration

26 EVALUATION Localize! Labs, CSF? Imaging EMG EEG-EMG

27 TREATMENT

28 EXAMPLE VIDEOS Opsoclonus-Myoclonus - https://www.youtube.com/watch?v=zX4j0IsFbAkhttps://www.youtube.com/watch?v=zX4j0IsFbAk Propriospinal Myoclonus - https://www.youtube.com/watch?v=W15D8N0wlyYhttps://www.youtube.com/watch?v=W15D8N0wlyY Generalized Myoclonus in SSPE - https://www.youtube.com/watch?v=pfCQP7IL2XY https://www.youtube.com/watch?v=pfCQP7IL2XY Myoclonic Dystonia - permalink/cont/a/cont_19_5_2013_08_08_zadikoff_190510_sdc3.mp4 permalink/cont/a/cont_19_5_2013_08_08_zadikoff_190510_sdc3.mp4 Post-hypoxic Myoclonus (Lance-Adams) - permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc4.mp4 permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc4.mp4 Amantadine-induced - permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc6.mp4 and permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc7.mp4 permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc6.mp4 permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc7.mp4

29 VIDEOS Corticobasal Degeneration - s/permalink/cont/a/cont_19_5_2013_08_08_duker_190510_sdc11.mp4 s/permalink/cont/a/cont_19_5_2013_08_08_duker_190510_sdc11.mp4 Unverricht-Lundborg - s/permalink/cont/a/cont_19_5_2013_08_08_morgante_190510_sdc12.mp4 s/permalink/cont/a/cont_19_5_2013_08_08_morgante_190510_sdc12.mp4 Hyperekplexia - s-literally-scared-STIFF-time-s-startled-sudden-noise-taken-surprise.htmlhttp://www.dailymail.co.uk/health/article /The-father- s-literally-scared-STIFF-time-s-startled-sudden-noise-taken-surprise.html Focal Cortical Myoclonus - https://www.youtube.com/watch?v=BHeCok19jWohttps://www.youtube.com/watch?v=BHeCok19jWo Palatal Myoclonus – https://www.youtube.com/watch?v=GlnxI93PrMM Canine Myoclonus – https://www.youtube.com/watch?v=MHQhoH5gYWohttps://www.youtube.com/watch?v=MHQhoH5gYWo

30 IN SUMMARY Myoclonus: -History -Exam -Localize -Time course -Clinical features -Diagnostics -Treat -Neurology all in one special!


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