II. Epidemiology
- Responsible for 2-3% of childhood Epilepsy
- Onset before age 12 months in 90% of patients
- Peak onset at age 4-8 months
III. Causes
- Tuberous sclerosis
- Phenylketonuria (PKU)
- Maple syrup urine disease
- Aicardi's Syndrome
- Agenesis of the Corpus Callosum
- Nonketotic Hyperglycemia
IV. Signs
- Flexor, extensor, or mixed Posture
- Brief head bobs to massive jerks of the body
- May occur in clusters
- Child drowsy on awakening (postictal)
V. Diagnostics: Electroencephalogram (EEG)
- Hypsarrhythmia
- Multiple high amplitude multi- focal spikes
- Disorganized wave discharges
VII. Management
- Difficult to control
- Adrenocorticotropic Hormone (ACTH)
- Valproate
VIII. Prognosis
- Herald ominous neurodevelopmental prognosis
- Outcome depends on cause