Infantile spasms: Criteria for an animal model

Carl E. Stafstrom, Gregory L. Holmes

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Infantile spasms is an epilepsy syndrome with several distinctive features, including age specificity during infancy, characteristic semiology (epileptic spasms), specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression), and responsiveness to the adrenocorticotropic hormone (ACTH). There is no adequate animal model of infantile spasms, perhaps due to these clinically unique features, that is specific for the developing human brain. An informative animal model would provide insights into the pathophysiology of this syndrome and form the basis for the development of innovative therapies. This chapter considers criteria for an "ideal" animal model of infantile spasms, as well as "minimal" criteria that we consider essential to yield useful information. Two animal models of infantile spasms have been described in rodents: seizures induced by corticotropin-releasing factor and N-methyl-d-aspartic acid. Neither of these models conforms exactly to the human analog, but each possesses intriguing similarities that provide testable hypotheses for future investigations.

Original languageEnglish (US)
Title of host publicationEpilepsy, Infantile Spasms, and Development Encephalopathy
PublisherAcademic Press Inc.
Pages391-411
Number of pages21
ISBN (Print)0123668492, 9780123668493
DOIs
StatePublished - 2002
Externally publishedYes

Publication series

NameInternational Review of Neurobiology
Volume49
ISSN (Print)0074-7742

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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