Infantile Spasms

Research output: Chapter in Book/Report/Conference proceedingChapter

3 Scopus citations

Abstract

West syndrome is an infantile epileptic encephalopathy with infantile spasms that are characteristic seizures associated with electrodecremental responses, hypsarrhythmia, and intellectual disabilities. There is a need to develop better therapies for West syndrome. Several rodent models of infantile spasms have been developed recently to elucidate the pathogenesis, and help identify new treatments. These are acute models, in which spasms occur only immediately after induction, and chronic models of infantile spasms that recapitulate many features of the syndrome. In this chapter, we are summarizing the main features and findings stemming from these models, as well as discuss their utility in the development of treatments for infantile spasms, other associated seizures, and their comorbidities.

Original languageEnglish (US)
Title of host publicationModels of Seizures and Epilepsy
Subtitle of host publicationSecond Edition
PublisherElsevier Inc.
Pages977-993
Number of pages17
ISBN (Print)9780128040669
DOIs
StatePublished - Jan 1 2017

Keywords

  • APC
  • ARX
  • Adenopolyposis colon
  • Aristaless X linked homeobox
  • Chronic early stress
  • Down syndrome
  • Electrodecremental response
  • Epilepsy
  • Hypsarrhythmia
  • Infantile spasms
  • Multiple-hit model of infantile spasms
  • NMDA
  • Prenatal betamethasone
  • Stress
  • Tetrodotoxin
  • Tuberous sclerosis
  • West syndrome

ASJC Scopus subject areas

  • General Neuroscience

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