Abstract
Infantile spasms (IS) is a relatively common and severe epilepsy of infants and young children. IS likely contributes to up to 10% of all mental retardation, and it evolves into further epilepsies in about 50% of cases.1 Although this catastrophic epilepsy was first identified more than 160 years ago, its cause and mechanism are poorly understood and treatment is unsatisfactory, particularly in preventing the postulated effects of these seizures on cognitive function.1,2
Highlights
Models for Infantile spasms (IS) proposed to date fit one of two general categories: those that recapitulate a specific cause of IS, and models that attempt to define and recreate a “final common pathway” for all of the causes that elicit IS
The neuropeptide corticotropin-releasing hormone (CRH) is an attractive candidate as a stress-activated convulsant that may contribute to the mechanisms of IS
adrenocorticotropic hormone (ACTH) does not act as a direct anticonvulsant, but rather ameliorates IS by reducing the synthesis and release of endogenous CRH.11
Summary
Models for IS proposed to date fit one of two general categories: those that recapitulate a specific cause of IS (eg, loss of interneurons in the ARX mouse, artificial stroke/ hemispherectomy), and models that attempt to define and recreate a “final common pathway” for all of the causes that elicit IS. This mechanism of action of ACTH is consistent with the time course of its efficacy in infantile spasms,17 where response is often all or none, requires a day or two to start, and often continues even when ACTH treatment is terminated.17
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