Peds

Infantile Spasms

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Infantile Spasms

  • See Also
  1. Seizure
  2. Pediatric Spell
  • 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
  • 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
  • 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)
  1. Hypsarrhythmia
    1. Multiple high amplitude multi- focal spikes
    2. Disorganized wave discharges
  • Differential Diagnosis
  1. Benign infantile Myoclonus
  2. Severe Myoclonus
  • Management
  1. Difficult to control
  2. Adrenocorticotropic Hormone (ACTH)
  3. Valproate
  • Prognosis
  1. Herald ominous neurodevelopmental prognosis
  2. Outcome depends on cause