An age-specific endogenous-convulsant hypothesis implicating corticotropin-releasing hormone (CRH), an excitant neuropeptide suppressed by ACTH/steroids, is proposed for the pathophysiology of massive infantile spasms in a report from the University of Southern California, Los Angeles, CA. Stated briefly, abnormally increased CRH synthesis and activity results in neuronal hyperexcitability. Deranged CRH-responsive brain-stem circuits may explain the spasms. ACTH treatment controls spasms and modifies the EEG by suppressing CRH synthesis. [1]
COMMENT. The author proposes that specific receptor blockers of CRH will arrest infantile spasms and may prove safer than ACTH and of some benefit to cognitive function.
The results of ACTH treatment in 21 children with intractable seizures other than infantile spasms at the Children’s Hospital, Camperdown NSW, Sydney, were disappointing. Only 24% had a good response. Hypokalemia, hypertension, and infection complicated treatment in 43%, 33%, and 19%, respectively [2]. More favorable results have been reported by some.