Summary

• An epilepsy syndrome, typically presenting in infancy, with a varying aetiology.
• Spasms may be flexor, extensor, mixed flexor-extensor, symmetrical, or asymmetrical, and typically occur in clusters.
• A hypsarrhythmic EEG is characteristic but not universally present at all times.
• A mortality rate of 5% to 30% is reported. Among survivors, developmental delay ensues in 80% to 90%, and 50% to 70% later develop other seizure types.
• Treatment options include hormonal therapy (ACTH or corticosteroids) or vigabatrin.