The course of West syndrome

Abstract

West syndrome (infantile spasms) is an unusual but generally devastating epileptic syndrome presenting in infancy. Although occasionally one component of an underlying disorder (such as chromosomal derangements or metabolic diseases), the disorder is idiopathic (“cryptogenic”) in a number of children. Much of our contemporary knowledge of West syndrome comes from work by Hamano et al in Japan. In the current issue of The Journal, this group addresses the question of factors that might predict the developmental outcome of children with cryptogenic West syndrome. These workers reviewed the records of 32 patients with an average age of over 8 years. Specifically, they looked for differences between children with a normal developmental outcome and those with delays. Children with normal developmental outcome had a shorter lag between the onset of spasms and the institution of treatment (a variety of agents, including anticonvulsants, intravenous gamma globulin, and ACTH). Delayed children were also more likely to have other seizures types associated with their infantile spasms, and to have EEGs that evolved to show frontal lobe activity. Given the size of the group studied, as well as the careful data collection by this group, this should be a valuable paper for the clinician who is counseling a family with a child in whom this frightening diagnosis is made. Although it is not clear from this study whether the “short interval” observation is a surrogate for something else or a direct effect of early treatment, the study also suggests that this is a diagnosis that should be pursued vigorously once suspected. Developmental Outcomes of Cryptogenic West SyndromeThe Journal of PediatricsVol. 150Issue 3PreviewTo elucidate factors affecting the developmental outcome of cryptogenic West syndrome. Full-Text PDF