Preliminary report of a multi-center study on the West syndrome

Abstract

During a 12-month period, 54 infants with the West syndrome (10 idiopathic, 44 symptomatic) referred to 10 major children's hospitals for initial treatment were evaluated to obtain comprehensive data on clinical findings and current treatment modalities. Prominent features included prevalence of prenatal and perinatal etiologies, severe neurological deficits and disturbed psychomotor development as well as patient-specific spectrum of seizure manifestations. Characteristic behavioural abnormalities before onset of spasms are an early indicator for the West syndrome. Therapeutic management varied considerably. Response to ACTH/steroid regiments was more favourable than to non-ACTH/steroid regimens. The most frequent serious adverse reactions during the initial treatment period were arterial hypertension and infections. Improved therapeutic strategies based on detailed initial patient assessment and systematic monitoring of beneficial effects and adverse reactions are necessary for future trials.