West syndrome: early remission is not assurance of normal final outcome

Abstract

West syndrome (WS), an age-specific epilepsy of infancy and early childhood, considered of poor prognosis, may have remission. With the objective of describing the clinical features, treatment and outcome of the children with WS followed in a Latin American country, we devised a retrospective study. Infants with recurrent spasms, and typical or atypical hypsarrhythmia on electroencephalography (EEG) were included, and their medical report reviewed with particular interest on therapy (anti-epileptic drug, steroids) and outcome. Short-term (remission of spasms up to 6 months after the beginning) and long-term outcome (development and seizure status over the age of 2 years) were focused. Of the 37 selected infants, most (29/37, 78.3%) were classified as symptomatic, and neonatal history of hypoxia (10/29) was the most frequent etiology. The majority had a typical hypsarrhythmia EEG pattern. Most used a combination of anti-epileptic drugs and steroids. A favourable short-term outcome occurred in 20 infants, but only three had normal development and were seizure free after the age of 2 years. Early seizure remission is not assurance of normal final outcome in WS.