Cortical dysplasia and epilepsy: Animal models

Philip A Schwartzkroin, Steven N. Roper, H. Jurgen Wenzel

Research output: Contribution to journalArticle

30 Citations (Scopus)

Abstract

Cortical dysplasia syndromes - those conditions of abnormal brain structure/organization that arise during aberrant brain development - frequently involve epileptic seizures. Neuropathological and neuroradiological analyses have provided descriptions and categorizations based on gross anatomical and cellular histological features (e.g., lissencephaly, heterotopia, giant cells), as well as on the developmental mechanisms likely to be involved in the abnormality (e.g., cell proliferation, migration). Recently, the genes responsible for several cortical dysplastic conditions have been identified and the underlying molecular processes investigated. However, it is still unclear how the various structural abnormalities associated with cortical dysplasia are related to (i.e., "cause") chronic seizures. To elucidate these relationships, a number of animal models of cortical dysplasia have been developed in rats and mice. Some models are based on laboratory manipulations that injure the brain (e.g., freeze, undercut, irradiation, teratogen exposure) of immature animals; others are based on spontaneous genetic mutations or on gene manipulations (knockouts/transgenics) that give rise to abnormal cortical structures. Such models of cortical dysplasia provide a means by which investigators can not only study the developmental mechanisms that give rise to these brain lesions, but also examine the cause-effect relationships between structural abnormalities and epileptogenesis.

Original languageEnglish (US)
Pages (from-to)145-174
Number of pages30
JournalAdvances in Experimental Medicine and Biology
Volume548
StatePublished - 2004

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Malformations of Cortical Development
Epilepsy
Brain
Animals
Animal Models
Genes
Lissencephaly
Teratogens
Gene Knockout Techniques
Cell proliferation
Giant Cells
Cell Movement
Rats
Seizures
Research Personnel
Cell Proliferation
Irradiation
Mutation

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Cortical dysplasia and epilepsy : Animal models. / Schwartzkroin, Philip A; Roper, Steven N.; Wenzel, H. Jurgen.

In: Advances in Experimental Medicine and Biology, Vol. 548, 2004, p. 145-174.

Research output: Contribution to journalArticle

Schwartzkroin, Philip A ; Roper, Steven N. ; Wenzel, H. Jurgen. / Cortical dysplasia and epilepsy : Animal models. In: Advances in Experimental Medicine and Biology. 2004 ; Vol. 548. pp. 145-174.
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