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Lissencephaly

More information in Books or onNLM PubMed
Definition: A "smooth brain" malformation of the CEREBRAL CORTEX resulting from 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.  note specifics; MICROLISSENCEPHALY is also available   
Examples Classical Lissencephalies and Subcortical Band Heterotopias; Cobblestone Lissencephaly
Other names Pachygyrias; Lissencephalies; Lissencephalias; Macrogyria; Lissencephalia; Large Gyri of Cerebrum; Broad Gyri of Cerebrum; Agyrias; Pachygyria; Agyria
 
SubstanceCAS Registry & nameCategoriesSource
Pachygyria, frontotemporal  0   *Lissencephaly.
Kuzniecky syndrome  0   *Epilepsy *Intellectual Disability *Lissencephaly.
Norman Roberts lissencephaly syndrome  0   *Lissencephaly.
Winter Harding Hyde syndrome  0   *Abnormalities, Multiple *Contracture *Facies *Lissencephaly.
Partial lissencephaly  0   *Lissencephaly.
Massa Casaer Ceulemans syndrome  0   *Arthrogryposis *Lissencephaly.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012

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