Congenital Abnormality
lissencephaly
lissencepha·ly
Subclass of:
Malformations of Cortical Development, Group II
Definitions related to lissencephaly:
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A brain malformation characterized by smooth folds and grooves in the brain.NICHD Pediatric TerminologyU.S. National Cancer Institute, 2021
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A rare genetic brain malformation characterized by smooth folds and grooves in the brain. There are approximately 20 different types of lissencephaly that are identified by various symptoms.NCI ThesaurusU.S. National Cancer Institute, 2021
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A smooth brain malformation of the CEREBRAL CORTEX resulting from the abnormal location of developing neurons during corticogenesis. It is characterized by an absence of normal convoluted indentations on the surface of the brain (agyria), or fewer and shallower indentations (pachygryia). There is a reduced number of cortical layers, typically 4 instead of 6, resulting in a thickened cortex, and reduced cerebral white matter that is a reversal of the normal ratio of cerebral white matter to cortex.NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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A spectrum of malformations of cortical development caused by insufficient neuronal migration that subsumes the terms agyria, pachygyria and subcortical band heterotopia. See also neuropathological definitions for 2-, 3-, and 4-layered lissencephaly.Human Phenotype Ontology (HPO)The Human Phenotype Ontology Project, 2021
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The term lissencephaly covers a group of rare malformations sharing the common feature of anomalies in the appearance of brain convolutions (characterised by simplification or absence of folding) associated with abnormal organisation of the cortical layers as a result of neuronal migration defects during embryogenesis.National Center for Advancing Translational Sciences
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