MYOCLONUS PRESENTED BY MATTHEW EBRIGHT PGY2 MYOCLONUS OUTLINE

MYOCLONUS PRESENTED BY MATTHEW EBRIGHT PGY-2

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

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

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

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

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

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

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

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

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

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

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

CLINICAL GROUPING Primary Secondary

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

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

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

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

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

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

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

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

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

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

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

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

TREATMENT

EXAMPLE VIDEOS Opsoclonus-Myoclonus - https: //www. youtube. com/watch? v=z. X 4 j 0 Is. Fb. Ak Propriospinal Myoclonus - https: //www. youtube. com/watch? v=W 15 D 8 N 0 wly. Y Generalized Myoclonus in SSPE https: //www. youtube. com/watch? v=pf. CQP 7 IL 2 XY Myoclonic Dystonia http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/ permalink/cont/a/cont_19_5_2013_08_08_zadikoff_190510_sdc 3. mp 4 Post-hypoxic Myoclonus (Lance-Adams) http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/ permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc 4. mp 4 Amantadine-induced http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/ permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc 6. mp 4 and http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/ permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc 7. mp 4

VIDEOS Corticobasal Degeneration http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/permalink/cont/a/cont_19_5_2013_08_08_duker_190510_sdc 11. mp 4 Unverricht-Lundborg http: //wolterskluwer. http. internapcdn. net/wolterskluwer_vitalstream_com/MP 4 s/permalink/cont/a/cont_19_5_2013_08_08_morgante_190510_sdc 12. mp 4 Hyperekplexia - http: //www. dailymail. co. uk/health/article-2767882/The-fathers-literally-scared-STIFF-time-s-startled-sudden-noise-taken-surprise. html Focal Cortical Myoclonus - https: //www. youtube. com/watch? v=BHe. Cok 19 j. Wo Palatal Myoclonus – https: //www. youtube. com/watch? v=Glnx. I 93 Pr. MM Canine Myoclonus – https: //www. youtube. com/watch? v=MHQho. H 5 g. YWo

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