II. Epidemiology

  1. Responsible for 2-3% of childhood Epilepsy
  2. Onset before age 12 months in 90% of patients
  3. Peak onset at age 4-8 months

III. Causes

  1. Tuberous sclerosis
  2. Phenylketonuria (PKU)
  3. Maple syrup urine disease
  4. Aicardi's Syndrome
  5. Agenesis of the Corpus Callosum
  6. Nonketotic Hyperglycemia

IV. Signs

  1. Flexor, extensor, or mixed Posture
  2. Brief head bobs to massive jerks of the body
  3. May occur in clusters
  4. Child drowsy on awakening (postictal)

V. Diagnostics: Electroencephalogram (EEG)

  1. Hypsarrhythmia
    1. Multiple high amplitude multi- focal spikes
    2. Disorganized wave discharges

VI. Differential Diagnosis

  1. Benign infantile Myoclonus
  2. Severe Myoclonus

VII. Management

  1. Difficult to control
  2. Adrenocorticotropic Hormone (ACTH)
  3. Valproate

VIII. Prognosis

  1. Herald ominous neurodevelopmental prognosis
  2. Outcome depends on cause

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