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Current neurology and neuroscience reports
Sep, 2003;3 (5): 433-41.

Developmental genetic malformations of the cerebral cortex.

Volney L Sheen, Christopher A Walsh
Author Information
  1. Volney L Sheen: Department of Neurology, Beth Israel Deaconess Medical Center, HIM 816, 4 Blackfan Circle, Boston, MA 02115, USA.
PMID: 12914687 DOI: 10.1007/s11910-003-0027-8

Abstract

Cortical malformations give rise to severe clinical manifestations such as epilepsy and mental retardation, but sometimes to more subtle problems like dyslexia. From a clinical standpoint, such structural abnormalities are diagnosed by radiographic and histologic findings, with disease classifications often based on these observations. Using this categorization, many of the responsible genes have been determined and now provide a means of understanding the molecular basis of the neurologic disorders. This review discusses the known genetic developmental syndromes in the context of the observed cortical malformations, the expression and function of the responsible genes, and their potential roles during the various stages of central nervous system development.

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Grants

  1. K08 MH063886/NIMH NIH HHS

MeSH Term

Animals
Cerebral Cortex
Humans
Nervous System Malformations
Syndrome
Journal Article Review

Word Cloud

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