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Disease Lissencephaly

Name Lissencephaly
Synonyms Agyria | Agyrias | Broad Gyri of Cerebrum | ILS | Large Gyri of Cerebrum | LIS1 | Lissencephalia | Lissencephalias | Lissencephalies | LISSENCEPHALY 1 | LISSENCEPHALY, CLASSIC SUBCORTICAL LAMINAR HETEROTOPIA, INCLUDED | LISSENCEPHALY SEQUENCE, ISOLATED | Macrogyria | Macrogyrias | Pachygyria | Pachygyrias | SBH, INCLUDED | SCLH, INCLUDED | SUBCORTICAL BAND HETEROTOPIA, INCLUDED
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.
Categories Congenital abnormality | Nervous system disease
MeSH® ID D054082
OMIM® ID 607432
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Top ↑ Ancestors

1. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Nervous System Malformations Has associated chemicals Has associated genes Has associated exposure references Malformations of Cortical Development Has associated chemicals Has associated genes Malformations of Cortical Development, Group II Has associated chemicals Has associated genes Lissencephaly Has associated chemicals Has associated genes
2. DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities Has associated chemicals Has associated genes Has associated exposure references Congenital Abnormalities Has associated chemicals Has associated genes Has associated exposure references Nervous System Malformations Has associated chemicals Has associated genes Has associated exposure references Malformations of Cortical Development Has associated chemicals Has associated genes Malformations of Cortical Development, Group II Has associated chemicals Has associated genes Lissencephaly Has associated chemicals Has associated genes

Top ↑ Descendants

Lissencephaly Has associated chemicals Has associated genes
  Fryns-Aftimos Syndrome
  Kuzniecky syndrome
  Lissencephaly 3 Has associated chemicals Has associated genes
  Lissencephaly, Familial, with Cleft Palate and Cerebellar Hypoplasia
View node Classical Lissencephalies and Subcortical Band Heterotopias Has associated chemicals Has associated genes
View node Cobblestone Lissencephaly Has associated chemicals Has associated genes
  Lissencephaly Type III and Bone Dysplasia
  Massa Casaer Ceulemans syndrome
  Norman Roberts lissencephaly syndrome Has associated chemicals Has associated genes
  Pachygyria, frontotemporal
  PACHYGYRIA WITH MENTAL RETARDATION, SEIZURES, AND ARACHNOID CYSTS Has associated chemicals Has associated genes
  Partial lissencephaly
  Winter Harding Hyde syndrome