[P3-42] Low-dose ACTH therapy for infatile spasms in the patient with Down syndrome
Purpose: It was reported that the patients of Down syndrome with infantile spasms (IS) responded to adrenocorticotropic hormone (ACTH) therapy. However, Treatment effect of low-dose synthetic ACTH therapy for infantile spasms developed in Down syndrome was remained unknown. This study aimed to assess effect of low-dose synthetic ACTH therapy for IS in Down syndrome. Methods: We investigated the rate of IS in 6 patients (3 boys and 3 girls) treated with ACTH therapy. The age at onset of IS and at the start of ACTH therapy ranged from 6 to 18 months (median, 6.5 ± 3.61 months) and from 7 to 18 months (median, 10 ± 10 months), respectively. Three patients underwent ACTH therapy as initial therapy. Two patients who did not respond to valproic acid underwent ACHT therapy after valproic acid monotherapy. One patient who did not respond to vitamin B6 underwent combination ACTH therapy with valproic acid after vitamin B6 monotherapy. We investigated the response rate of IS treated with ACTH therapy. Results: One (14.3%) of the 7 patient who did not respond to valproic acid showed cessation of IS for more than 1 year. After ACTH therapy, IS ceased by administration of antiepileptic drugs in the 4 patients. IS ceased by topiramate in 3 of 4 patients, and by lamotrigine in one of the 4 patients. In the remaining a patient, IS was drug resistant, and has not ceased until now. Conclusion: Low-dose synthetic ACTH therapy might be ineffective for IS treatment in Down syndrome.