Infantile Spasms

Abstract

SINCE West¹ vividly described the syndrome of infantile spasms with progressive mental deterioration 125 years ago, the condition has been known under a variety of names, such as nodding spasms, salaam seizures, jack-knife seizures, "lightning majors," and massive myoclonic epilepsy. In 1954 Gibbs and associates² coined the terms, infantile spasms and hypsarhythmia, to denote respectively the type of seizure and the characteristic electroencephalographic pattern associated with the seizures. These authors also emphasized the unsatisfactory response to treatment and the poor prognosis for mental development (87% of their 237 patients were left mentally defective). New hope for better treatment and a better outlook in infantile spasms was evoked in 1958, when Sorel and Dusaucy-Bauloye³ reported dramatic results with use of corticotropin (ACTH). These observations were quickly confirmed in the United States.^4,5 Subsequently, however, studies on children observed for longer periods after treatment with corticotropin or corticosteroids, revealed