Volume 6, Issue 4 pp. 268-280

Cortical malformations and epilepsy

Philip A. Schwartzkroin

Corresponding Author

Philip A. Schwartzkroin

Department of Neurological Surgery, University of Washington, Health Sciences Center, Seattle, Washington

Department of Neurological Surgery, Health Sciences Center, Room RR-720, University of Washington, Box 356470, 1959 NE. Pacific St., Seattle, WA 98195-6470Search for more papers by this author
Christopher A. Walsh

Christopher A. Walsh

Department of Neurology, Harvard University, Beth Israel Deaconess Medical Center, Boston, Massachusetts

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Abstract

Brain malformations, resulting from aberrant patterns of brain development, are highly correlated with childhood seizure syndromes, as well as with cognitive disabilities and other neurological disorders. The structural malformations, often referred to as cortical dysplasia, are extremely varied, reflecting diverse underlying processes and critical timing of the developmental aberration. Recent studies have revealed a genetic basis for many forms of dysplasia. Gene mutations responsible for such common forms of dysplasia as lissencephaly and tuberous sclerosis have been identified, and investigators are beginning to understand how these gene mutations interrupt and/or misdirect the normal developmental pattern. Laboratory investigations, using animal models of cortical dysplasia, are beginning to elucidate how these structural malformations give rise to epilepsy and other functional pathologies. MRDD Research Reviews 2000;6:268–280. © 2000 Wiley-Liss, Inc.

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