Infantile spasms1

Abstract

West's syndrome is an age-specific set of symptoms consisting of myoclonic epilepsy (infantile spasms), an interictal electroencephalographic pattern (hypsarrhythmia), and moderate-to-severe mental retardation. Infantile spasms were first reported in 1841, but all three characteristics were not described until 1951. The basic pathophysiology of and ideal therapy for the seizures remain undefined. The author reviews the historical aspects, clinical manifestations, etiology, diagnostic evaluation, and treatment of West's syndrome.