Pathophysiology of massive infantile spasms: perspective on the putative role of the brain adrenal axis

Ann Neurol. 1993 Mar;33(3):231-6. doi: 10.1002/ana.410330302.

Abstract

Massive infantile spasms are an age-specific seizure syndrome of infancy. Uniquely, the spasms respond to hormonal manipulation using adrenocorticotropic hormone (ACTH) or glucocorticoids. A hypothesis explaining the efficacy of hormonal therapy, age-specificity, multiple causative factors, and spontaneous resolution of infantile spasms is presented. Corticotropin-releasing hormone (CRH), an excitant neuropeptide suppressed by ACTH/steroids, is implicated. Evidence for the age-specific convulsant properties of CRH is presented, and a putative scenario in which a stress-induced enhancement of endogenous CRH-mediated seizures is discussed. Clinical testing of the CRH-excess theory and its therapeutic implications are suggested.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adrenocorticotropic Hormone / metabolism*
  • Brain / metabolism*
  • Corticotropin-Releasing Hormone / metabolism*
  • Corticotropin-Releasing Hormone / pharmacology
  • Glucocorticoids / metabolism
  • Humans
  • Infant
  • Spasms, Infantile / metabolism
  • Spasms, Infantile / physiopathology*

Substances

  • Glucocorticoids
  • Adrenocorticotropic Hormone
  • Corticotropin-Releasing Hormone