TREATMENT AND PROGNOSIS OF WEST SYNDROME

From 1969 to 1990, 180 cases with West syndrome (WS) were admitted to our hospital. We investigated the long-term outcome of 109 WS cases followed for over 3 years. Seizure remission and normal mentality were obtained in 58.7% and 23.9%, respectively. Both seizure and mental prognoses were significantly more favorable in the idiopathic group than in the symptomatic group. High-dose pyridoxal phosphate (PAL-P) therapy was tried in 170 patients and was effective in 20 patients (11.8%). The long-term mental and seizure prognoses for the PAL-P-effective group were favorable. Synthetic adrenocorticotropic hormone therapy (mean maximum dose/day, 0.03 mg/kg; mean treatment duration, 26.7 days) was tried in 114 patients and was effective in 101 (88.6%). Seizure remission and normal mentality were obtained in 55.8% and in 20.8% of patients at the time of follow-up. The therapy duration was shorter, and total dosage for suppression of seizures was lower than those reported in previous studies. From 1987, high-dose valproate therapy was tried in 15 PAL-P-ineffective cases and was effective in 6 (40.0%), with the initial effect maintained in all responsive cases at the time of follow-up.