Short-term developmental and seizure outcomes were assessed in 7 children, aged <12 months, with cryptogenic West syndrome treated with a short-duration (7-12 days) and low-dose synthetic ACTH at Nagoya City Hospital, Japan. All patients had received a trial of one or two AEDs before ACTH. Daily single dose of ACTH was 0.022-0.027 mg/kg/day (mean, 0.96 IU/kg), and total dose was 0.17-0.28 mg/kg (mean, 9.0 IU/kg). Interval between onset of spasms and initiation of ACTH was 12-105 days (median 14 days). Tonic spasms were controlled in all patients with no serious side effects. Spasms disappeared within 1 week, and hypsarrhythmia resolved within 2 weeks. EEG abnormalities persisted in 2. IQ or developmental quotients of 6 patients were 79-110 at age 2 to 6 years. One patient with a long treatment lag had a developmental quotient of 60. [1]

COMMENT. Vitamin B6 and AEDs, including clonazepam, valproic acid, and zonisamide, are used before initiating ACTH, because of possible adverse effects of hormone therapy. Delay in intiation of ACTH may result in poor outcome, and a favorable response to very low dose-short duration therapy should favor a recommendation for early trial of ACTH. Previous trials of ACTH using relatively low dose regimens have proven effective without serious side effects [2, 3], whereas high-dose, long-term regimens are associated with frequent serious side effects. [4]