A model of symptomatic infantile spasms syndrome

Morris H. Scantlebury, Aristea S. Galanopoulou, Lenka Chudomelova, Emmanuel Raffo, David Betancourth, Solomon L. Moshé

Research output: Contribution to journalArticle

78 Scopus citations

Abstract

Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.

Original languageEnglish (US)
Pages (from-to)604-612
Number of pages9
JournalNeurobiology of Disease
Volume37
Issue number3
DOIs
Publication statusPublished - Mar 1 2010

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Keywords

  • Animal model
  • Autism
  • Doxorubicin
  • Epilepsy
  • Infantile spasms
  • Lipopolysaccharide
  • Neonatal rat
  • West syndrome
  • p-Chlorophenylalanine

ASJC Scopus subject areas

  • Neurology

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