Long-term follow-up study of West syndrome: Differences of outcome among symptomatic etiologies.

Abstract

To evaluate the outcome of West syndrome and to elucidate the differences in the outcome related to the timing of brain injury. Medical records of 60 patients who were followed regularly for more than seven years were reviewed. The following clinical features were assessed: onset, seizure evolution, electroencephalography and intelligence. Those variables were compared among five groups: cryptogenic, prenatal, preterm, term, and postnatal groups. The onset ages of the postnatal group were later than those of the others (P<.05). The relapse after adrenocorticotropic hormone therapy of the preterm group was the earliest among the groups (P<.05). Regarding encelphalography, the ratio of patients with focal discharges was higher in the postnatal group than in the prenatal group (P<.05). The ratios of patients in whom focal epilepsy developed were higher in the term and postnatal group than in the cryptogenic and prenatal group (P<.05). The term group showed similar characteristics to those of the postnatal group. Seven of the 60 had normal intelligence, including three girls with tuberous sclerosis. The diverse outcomes of West syndrome depending on etiology seemed to be related to the timing of brain injury and brain development.