- Responsible for 2-3% of childhood Epilepsy
- Onset before age 12 months in 90% of patients
- Peak onset at age 4-8 months
- Tuberous sclerosis
-
Phenylketonuria (PKU)
- Maple syrup urine disease
- Aicardi's Syndrome
- Agenesis of the Corpus Callosum
- Nonketotic Hyperglycemia
- Flexor, extensor, or mixed Posture
- Brief head bobs to massive jerks of the body
- May occur in clusters
- Child drowsy on awakening (postictal)
- Hypsarrhythmia
- Multiple high amplitude multi- focal spikes
- Disorganized wave discharges
- Benign infantile Myoclonus
- Severe Myoclonus
- Herald ominous neurodevelopmental prognosis
- Outcome depends on cause
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