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Infantile spasms

Eric H Kossoff1

  • 1Department of Neurology, The John M. Freeman Pediatric Epilepsy Center, The Johns Hopkins Hospital, Baltimore, MD 21287, USA. ekossoff@jhmi.edu

The Neurologist
|March 12, 2010
PubMed

Insights

Infantile spasms (West syndrome) is a serious epilepsy affecting 1 in 2000 infants. While established treatments exist, newer therapies offer promise with fewer side effects for this rapidly evolving condition.

Area of Science:

  • Pediatric Neurology
  • Epileptology

Background:

  • Infantile spasms (West syndrome) is a rare epilepsy affecting 1 in 2000 infants.
  • Associated with significant developmental and cognitive delays.
  • Limited effective, tolerable, and available therapies since 1841.

Purpose of the Study:

  • To review clinical features, EEG findings (hypsarrhythmia), prognostic factors, and treatment options for infantile spasms.
  • To discuss current and emerging therapeutic strategies.

Main Methods:

  • Review of existing guidelines, surveys, and practice parameters.
  • Discussion of established and novel treatment options.
  • Inclusion of recent advances in animal models for treatment research.

Main Results:

  • Adrenocorticotropin hormone and vigabatrin are established treatments; vigabatrin is indicated for tuberous sclerosis.
  • Emerging therapies include high-dose oral prednisolone, ketogenic diet, and topiramate, potentially with fewer side effects.
  • Development of animal models facilitates new treatment testing.

Conclusions:

  • The field of infantile spasms research is experiencing rapid advancements.
  • Timely intervention and exploration of new treatments are crucial for affected infants.
Abstract

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