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Myoclonus
AdeWijaya, MD – November 2019
Introduction
• Sudden, brief, shock-like involuntary movements, associated
with bursts of muscular activity (positive myoclonus) or
silencing of muscular activity (negative myoclonus)
• It may be present at rest, during voluntary movement (action-
induced) or due to provoking stimuli such as sensory, visual,
auditory or emotional cues
• Typically, myoclonus presents as short (10–50 ms, rarely more
than 100 ms), non-rhythmic jerks, often without any
discernible pattern
Caviness, J.N. Myoclonus. Mayo Clin. Proc. 1996, 71, 679–688.
Shibasaki, H. Neurophysiological classification of myoclonus. Neurophysiol. Clin. 2006, 36, 267–269
Epidemiology
• The average annual incidence: 1 per 100,000 person-years
• Lifetime prevalence: less than 10 cases per 100,000 population
• The rate increased with advancing age and was consistently
higher in men
• Neurodegenerative and dementing diseases were the most
common cause of symptomatic myoclonus (about 70%)
Caviness, J.N.; Alving, L.I.; Maraganore, D.M.; Black, R.A.; McDonnell, S.K.; Rocca, W.A. The incidence and prevalence of myoclonus in olmsted county, minnesota. Mayo Clin. Proc. 1999, 74, 565–569.
Caviness, J.N.; Brown, P. Myoclonus: Current concepts and recent advances. Lancet Neurol. 2004, 3, 598–607.
Classification
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Cortical Myoclonus
• Myoclonus in the Context of Epilepsy
• Neurodegenerative and Mitochondrial Disorders
• Cerebrovascular Disease with Probable Cortical Sources
• Acute post-hypoxic myoclonus
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Subcortical Myoclonus
• Myoclonus Dystonia
• Orthostatic Myoclonus
• Chronic Posthypoxic Myoclonus
• Cerebrovascular Disease with Probable Subcortical Sources
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Others
• Segmental and Peripheral Myoclonus
• Propriospinal Myoclonus
• Negative Myoclonus
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Metabolic Causes of Myoclonus
• Uremic encephalopathy
• Hepatic encephalopathy
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Autoimmune Conditions with Myoclonus
• Opsoclonus-Myoclonus Syndrome
• Steroid-Responsive Encephalopathy with Autoimmune
Thyroid Disease
• Stiff-Person Syndrome and Progressive Encephalomyelitis
with Rigidity
• Limbic encephalitis
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Infectious und Para-Infectious Causes of
Myoclonus
• HIV infection
• Whipple disease
• Viral (VZV, HSV-1, EBV, measles, mumps, WNV, St. Louis
encephalitis, EEE (Eastern equine encephalitis) virus,WEE
(Western equine encephalitis) virus, Dengue virus), bacterial
(Leptospira, Mycoplasma pneumonia), parasitic (cerebral
malaria) or slow virus infections (subacute sclerosing
panencephalitis)
Cardoso, F. Hiv-related movement disorders: Epidemiology, pathogenesis and management. CNS Drugs 2002, 16, 663–668.
Louis, E.D.; Lynch, T.; Kaufmann, P.; Fahn, S.; Odel, J. Diagnostic guidelines in central nervous system whipple’s disease. Ann. Neurol. 1996, 40, 561–568.
Zutt, R.; van Egmond, M.E.; Elting, J.W.; van Laar, P.J.; Brouwer, O.F.; Sival, D.A.; Kremer, H.P.; de Koning, T.J.; Tijssen, M.A. A novel diagnostic approach to patients with myoclonus. Nat. Rev. Neurol. 2015, 11, 687–697.
Medication andToxin-Induced Myoclonus
• Opioids
• Antidepressants
• Classic or atypical antipsychotics
• Antiepileptic drugs
• Antibiotics
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Treatment
• Cortical myoclonus: levetiracetam, valproic acid, clonazepam
or piracetam
• Subcortical myoclonus: clonazepam
• Deep brain stimulation
Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
Summary
• Sudden, brief, shock-like involuntary movements
• Positive vs negative myoclonus
• Cortical vs subcortical myoclonus
• Various etiologies
• Treatment: anticonvulsants; deep brain stimulation
THANKYOU

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Myoclonus

  • 2. Introduction • Sudden, brief, shock-like involuntary movements, associated with bursts of muscular activity (positive myoclonus) or silencing of muscular activity (negative myoclonus) • It may be present at rest, during voluntary movement (action- induced) or due to provoking stimuli such as sensory, visual, auditory or emotional cues • Typically, myoclonus presents as short (10–50 ms, rarely more than 100 ms), non-rhythmic jerks, often without any discernible pattern Caviness, J.N. Myoclonus. Mayo Clin. Proc. 1996, 71, 679–688. Shibasaki, H. Neurophysiological classification of myoclonus. Neurophysiol. Clin. 2006, 36, 267–269
  • 3. Epidemiology • The average annual incidence: 1 per 100,000 person-years • Lifetime prevalence: less than 10 cases per 100,000 population • The rate increased with advancing age and was consistently higher in men • Neurodegenerative and dementing diseases were the most common cause of symptomatic myoclonus (about 70%) Caviness, J.N.; Alving, L.I.; Maraganore, D.M.; Black, R.A.; McDonnell, S.K.; Rocca, W.A. The incidence and prevalence of myoclonus in olmsted county, minnesota. Mayo Clin. Proc. 1999, 74, 565–569. Caviness, J.N.; Brown, P. Myoclonus: Current concepts and recent advances. Lancet Neurol. 2004, 3, 598–607.
  • 4. Classification Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 5. Cortical Myoclonus • Myoclonus in the Context of Epilepsy • Neurodegenerative and Mitochondrial Disorders • Cerebrovascular Disease with Probable Cortical Sources • Acute post-hypoxic myoclonus Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 6. Subcortical Myoclonus • Myoclonus Dystonia • Orthostatic Myoclonus • Chronic Posthypoxic Myoclonus • Cerebrovascular Disease with Probable Subcortical Sources Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 7. Others • Segmental and Peripheral Myoclonus • Propriospinal Myoclonus • Negative Myoclonus Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 8. Metabolic Causes of Myoclonus • Uremic encephalopathy • Hepatic encephalopathy Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 9. Autoimmune Conditions with Myoclonus • Opsoclonus-Myoclonus Syndrome • Steroid-Responsive Encephalopathy with Autoimmune Thyroid Disease • Stiff-Person Syndrome and Progressive Encephalomyelitis with Rigidity • Limbic encephalitis Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 10. Infectious und Para-Infectious Causes of Myoclonus • HIV infection • Whipple disease • Viral (VZV, HSV-1, EBV, measles, mumps, WNV, St. Louis encephalitis, EEE (Eastern equine encephalitis) virus,WEE (Western equine encephalitis) virus, Dengue virus), bacterial (Leptospira, Mycoplasma pneumonia), parasitic (cerebral malaria) or slow virus infections (subacute sclerosing panencephalitis) Cardoso, F. Hiv-related movement disorders: Epidemiology, pathogenesis and management. CNS Drugs 2002, 16, 663–668. Louis, E.D.; Lynch, T.; Kaufmann, P.; Fahn, S.; Odel, J. Diagnostic guidelines in central nervous system whipple’s disease. Ann. Neurol. 1996, 40, 561–568. Zutt, R.; van Egmond, M.E.; Elting, J.W.; van Laar, P.J.; Brouwer, O.F.; Sival, D.A.; Kremer, H.P.; de Koning, T.J.; Tijssen, M.A. A novel diagnostic approach to patients with myoclonus. Nat. Rev. Neurol. 2015, 11, 687–697.
  • 11. Medication andToxin-Induced Myoclonus • Opioids • Antidepressants • Classic or atypical antipsychotics • Antiepileptic drugs • Antibiotics Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 12. Treatment • Cortical myoclonus: levetiracetam, valproic acid, clonazepam or piracetam • Subcortical myoclonus: clonazepam • Deep brain stimulation Eberhardt O, Topka H. Myoclonic disorders. Brain sciences. 2017 Aug;7(8):103.
  • 13. Summary • Sudden, brief, shock-like involuntary movements • Positive vs negative myoclonus • Cortical vs subcortical myoclonus • Various etiologies • Treatment: anticonvulsants; deep brain stimulation