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Pathophysiology of massive infantile spasms: Perpective on the putative role of the brain adrenal axis
Author(s) -
Baram Tallie A.
Publication year - 1993
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410330302
Subject(s) - adrenocorticotropic hormone , corticotropin releasing hormone , pathophysiology , hormone , medicine , neuropeptide , endogeny , convulsant , endocrinology , neuroscience , psychology , receptor
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 supressed by ACTH/Steroids, is implicated. Evidence for the age‐specific vonvulsant properties of CRH is presented, and a putative secenario in which a stressinduced enhancement of endogenous CRH‐mediated seizures is discussed. Clinical resting of the CRH‐Excess theory and its therapeutic implication are suggested.