Eric H Kossoff1. 1. Department of Neurology, The John M. Freeman Pediatric Epilepsy Center, The Johns Hopkins Hospital, Baltimore, MD 21287, USA. ekossoff@jhmi.edu
Abstract
BACKGROUND: Infantile spasms (West syndrome) is an epilepsy condition affecting 1 in 2000 infants. Perhaps no more worrisome neurologic disorder exists because of its frequent association with delayed development and cognition at such a young age. Despite its existence in the literature since 1841, proven therapies are limited because of efficacy, tolerability, at times even availability. REVIEW SUMMARY: In this review, the clinical features, electroencephalogram (EEG) findings (hypsarrhythmia), prognostic factors, and myriad of treatment options for this condition will be discussed. Guidelines, surveys, and practice parameters have judged adrenocorticotropin hormone and vigabatrin to be the most proven treatments, with the latter indicated for tuberous sclerosis. However, potentially helpful therapies with fewer side effects have recently emerged including high-dose oral prednisolone, ketogenic diet, and topiramate. Additionally, advances in the past several years include the creation of viable animal models for testing new treatments. CONCLUSIONS: At no other time since its first description in 1841 has the field of infantile spasms research been so rapidly changing. For the thousands of infants faced with this potentially devastating disorder, there is no time like the present.
BACKGROUND:Infantile spasms (West syndrome) is an epilepsy condition affecting 1 in 2000 infants. Perhaps no more worrisome neurologic disorder exists because of its frequent association with delayed development and cognition at such a young age. Despite its existence in the literature since 1841, proven therapies are limited because of efficacy, tolerability, at times even availability. REVIEW SUMMARY: In this review, the clinical features, electroencephalogram (EEG) findings (hypsarrhythmia), prognostic factors, and myriad of treatment options for this condition will be discussed. Guidelines, surveys, and practice parameters have judged adrenocorticotropin hormone and vigabatrin to be the most proven treatments, with the latter indicated for tuberous sclerosis. However, potentially helpful therapies with fewer side effects have recently emerged including high-dose oral prednisolone, ketogenic diet, and topiramate. Additionally, advances in the past several years include the creation of viable animal models for testing new treatments. CONCLUSIONS: At no other time since its first description in 1841 has the field of infantile spasms research been so rapidly changing. For the thousands of infants faced with this potentially devastating disorder, there is no time like the present.
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