Pediatric Epilepsy: Animal Models of Catastrophic Epilepsies of Childhood

C. E. Stafstrom, L. Velíšek

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The catastrophic epilepsies are among the most devastating neurologic disorders affecting young children. Catastrophic epilepsies are age-specific epilepsy syndromes that are refractory to medical treatment and are associated with encephalopathy and cognitive decline. Examples include infantile spasms and Lennox-Gastaut syndrome. Catastrophic epilepsies of childhood occur within a specific age window and are characterized by numerous etiologies. Attempts to develop animal models of catastrophic epilepsies have been quite limited, as rodents and other experimental systems lack clinical and electrographic features found in human syndromes. In this article, we describe a new model of infantile spasms in infant rats, a '2-hit' model produced by prenatal exposure to betamethasone and postnatal seizures induced by . N-methyl-. d-aspartic acid. This model replicates the developmentally-specific flexion seizures, interictal electroencephalographic abnormalities, ictal EEG changes, and adrencorticotrophic hormone responsiveness of human infantile spasms.

Original languageEnglish (US)
Title of host publicationEncyclopedia of Basic Epilepsy Research
PublisherElsevier Inc.
Pages1034-1038
Number of pages5
ISBN (Print)9780123739612
DOIs
StatePublished - Jan 1 2009
Externally publishedYes

Keywords

  • Epileptic encephalopathy
  • Infantile spasms
  • Lennox-Gastaut syndrome
  • NMDA

ASJC Scopus subject areas

  • Medicine(all)
  • Neuroscience(all)

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    Stafstrom, C. E., & Velíšek, L. (2009). Pediatric Epilepsy: Animal Models of Catastrophic Epilepsies of Childhood. In Encyclopedia of Basic Epilepsy Research (pp. 1034-1038). Elsevier Inc.. https://doi.org/10.1016/B978-012373961-2.00182-X