Transcript Myoclonus - UMMS Wiki

MYOCLONUS
PRESENTED BY MATTHEW EBRIGHT PGY-2
MYOCLONUS - OUTLINE
Definition
Pathophysiology
Classification
Etiologies
Specific Examples
Evaluation
Treatment
Videos
DEFINE MYOCLONUS
What is it?
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Rapid
Brief
Involuntary
Contraction
One or multiple muscles, around a joint
Symmetry?
Synchrony?
Differential Diagnosis?
PATHOPHYSIOLOGY
Not entirely clear!
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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
HOW TO CLASSIFY
MYOCLONUS
1. Anatomic localization
2. Time course
3. Triggers
4. Other general clinical features
LOCALIZATION
Is it…
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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
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Progressive myoclonic encephalopathies and storage
diseases
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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
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Alzheimer dementia
Parkinson disease
Dementia with Lewy bodies
Corticobasal degeneration
Multiple system atrophy
Frontotemporal dementia
SECONDARY MYOCLONUS
• Infectious/Inflammatory
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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
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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
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Chaotic myoclonic eye movements
Oscillopsia
Multifocal, action-related myoclonus
Paraneoplastic or post-infectious
SPECIFIC EXAMPLES
• Hyperekplexia
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Autosomal dominant
Stiff babies
Episodic tonic spasms
Nocturnal myoclonus
Exaggerated startle responses
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Brainstem localization
Non-habituating
Overly-sensitive to stimuli
May persist through life
SPECIFIC EXAMPLES
• Canine myoclonus
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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=zX4j0IsFbAk
Propriospinal Myoclonus - https://www.youtube.com/watch?v=W15D8N0wlyY
Generalized Myoclonus in SSPE https://www.youtube.com/watch?v=pfCQP7IL2XY
Myoclonic Dystonia http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4s/
permalink/cont/a/cont_19_5_2013_08_08_zadikoff_190510_sdc3.mp4
Post-hypoxic Myoclonus (Lance-Adams) http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4s/
permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc4.mp4
Amantadine-induced http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4s/
permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc6.mp4 and
http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4s/
permalink/cont/a/cont_19_5_2013_08_08_espay_190510_sdc7.mp4
VIDEOS
Corticobasal Degeneration http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4
s/permalink/cont/a/cont_19_5_2013_08_08_duker_190510_sdc11.mp4
Unverricht-Lundborg http://wolterskluwer.http.internapcdn.net/wolterskluwer_vitalstream_com/MP4
s/permalink/cont/a/cont_19_5_2013_08_08_morgante_190510_sdc12.mp4
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=BHeCok19jWo
Palatal Myoclonus –
https://www.youtube.com/watch?v=GlnxI93PrMM
Canine Myoclonus – https://www.youtube.com/watch?v=MHQhoH5gYWo
IN SUMMARY
Myoclonus:
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History
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Exam
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Localize
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Time course
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Clinical features
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Diagnostics
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Treat
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Neurology all in one special!