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Malformations of cortical development and epilepsy
Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pre...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Les Laboratoires Servier
200
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3181860/ https://www.ncbi.nlm.nih.gov/pubmed/18472484 |
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author | Leventer, Richard J. Guerrini, Renzo Dobyns, William B. |
author_facet | Leventer, Richard J. Guerrini, Renzo Dobyns, William B. |
author_sort | Leventer, Richard J. |
collection | PubMed |
description | Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures, A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etioiogic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD, The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaiy, classical iissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented. |
format | Online Article Text |
id | pubmed-3181860 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 200 |
publisher | Les Laboratoires Servier |
record_format | MEDLINE/PubMed |
spelling | pubmed-31818602011-10-27 Malformations of cortical development and epilepsy Leventer, Richard J. Guerrini, Renzo Dobyns, William B. Dialogues Clin Neurosci Basic Research Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures, A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etioiogic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD, The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaiy, classical iissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented. Les Laboratoires Servier 2008 -03 /pmc/articles/PMC3181860/ /pubmed/18472484 Text en Copyright: © 2008 LLS http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Basic Research Leventer, Richard J. Guerrini, Renzo Dobyns, William B. Malformations of cortical development and epilepsy |
title | Malformations of cortical development and epilepsy |
title_full | Malformations of cortical development and epilepsy |
title_fullStr | Malformations of cortical development and epilepsy |
title_full_unstemmed | Malformations of cortical development and epilepsy |
title_short | Malformations of cortical development and epilepsy |
title_sort | malformations of cortical development and epilepsy |
topic | Basic Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3181860/ https://www.ncbi.nlm.nih.gov/pubmed/18472484 |
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