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Approach myoclonus


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Approach myoclonus

  1. 1. Approach to Myoclonic seizures in Childhood
  2. 2. Myoclonus • Myoclonus is a brief, involuntary muscle contraction (jerk) that may represent • (1) a seizure manifestation, as in infantile spasms; • (2) a physiological response to startle or to falling asleep; or • (3) an involuntary movement either alone or in combination with tonic-clonic seizures
  3. 3. CLINICAL AND ETIOLOGIC CLASSIFICATION • Marsden and colleagues • Physiologic • Essential • Epileptic • Secondary (symptomatic)
  4. 4. Physiologic myoclonus (normal subjects) • A. Sleep jerks (eg, hypnic jerks) • B. Anxiety-induced • C. Exercise-induced • D. Hiccough (singultus) • E. Benign infantile myoclonus with feeding
  5. 5. Essential myoclonus (primary symptom, nonprogressive history) • A. Hereditary (autosomal dominant) • B. Sporadic
  6. 6. Epileptic myoclonus • Fragments of epilepsy • Myoclonic epilepsy syndromes • Familial cortical myoclonic tremor with epilepsy
  7. 7. Fragments of epilepsy • Isolated epileptic myoclonic jerks • Epilepsia partialis continua (focal status epilepticus) • Idiopathic stimulus-sensitive myoclonus • Photosensitive myoclonus • Absences with a minor myoclonic component • Epilepsy with myoclonic absences
  8. 8. Myoclonic epilepsy syndromes • Infantile spasms (West syndrome) • Severe myoclonic epilepsy of infancy (Dravet syndrome) • Benign myoclonic epilepsy of infancy • Lennox-Gastaut syndrome • Myoclonic astatic epilepsy (Doose syndrome) • Cryptogenic myoclonus epilepsy (Aicardi) • Juvenile myoclonic epilepsy (Janz syndrome) • Familial cortical myoclonic tremor with epilepsy
  9. 9. Familial cortical myoclonic tremor with epilepsy • Autosomal dominant inheritance • Adult onset • Distal action tremor and myoclonus • Infrequent, secondarily generalized tonic- clonic seizures • Relatively benign course, typically with normal cognition • Responsiveness to anticonvulsants.
  10. 10. Symptomatic (secondary) myoclonus • Post–central nervous system injury • Basal ganglia degenerations • Drug-induced • Storage disorders • Viral encephalitis
  11. 11. Early infantile epileptic encephalopathies • Early myoclonic infantile encephalopathy • Early epileptic infantile encephalopathy (Ohtahara syndrome)
  12. 12. Epilepsy syndromes with myoclonic seizures • West syndrome • Lennox-Gastaut syndrome • Myoclonic astatic epilepsy (Doose syndrome) • Severe myoclonic epilepsy of infancy (Dravet syndrome)
  13. 13. West syndrome • Starts between the ages of 2 and 12 months • Triad of infantile spasms that usually occur in clusters (particularly in drowsiness or upon arousal), developmental regression, and a typical EEG picture – hypsarrhythmia. • Either cryptogenic or symptomatic • ARX gene (ambiguous genitalia) • Medical emergency (critical period of 3 weeks)
  14. 14. Lennox-Gastaut syndrome • Between the age of 2 and 10 years • Triad of developmental delay, multiple seizure types and 1-2 Hz spike–and-slow waves, polyspike bursts in sleep, and a slow background in wakefulness.
  15. 15. Myoclonic astatic epilepsy (Doose syndrome) • Onset of seizures is between the ages of 2 and 5 years • Similar to but milder than Lennox-Gastaut syndrome • Does not have tonic seizures or polyspike bursts in sleep • Prognosis is more favorable
  16. 16. Dravet syndrome • Most severe of the phenotypic spectrum of febrile seizures plus • Onset is in the 1st year of life, characterized by febrile and afebrile unilateral clonic seizures recurring every 1 or 2 months.
  17. 17. Progressive myoclonic epilepsies • Progressive dementia and worsening myoclonic and other seizures. • Type I or Unvericht Lundborg disease is more slowly progressive than the other types and usually starts in adolescence. • Type II or Lafora body disease can have an early childhood onset but usually starts in adolescence, is more quickly progressive, and is usually fatal.
  18. 18. Treatment - General • Patients with benign forms of myoclonic epilepsy often respond well to valproic acid or clonazepam. • The duration of treatment is usually approximately 5 years. • Second-line medications include ethosuximide, zonisamide, and topiramate.
  19. 19. Treatment - Specific • West syndrome is best treated with ACTH. • Vigabatrin - approved by the FDA for use in children with infantile spasms. • Treatment of seizures in Lennox-Gastaut syndrome varies according to the preponderant seizure type.
  20. 20. Treatment - Specific • Dravet syndrome is usually treated with valproate and benzodiazepines such as clonazepam. • Lamotrigine has been reported to exacerbate seizures in Dravet syndrome and other myoclonic epilepsies • Stiripentol and ketogenic diet may have a niche role in treating Dravet syndrome.