Schizencephaly

Findings:

Multiple MR images show a gray matter lined cleft extending from the sylvian fissure into the left lateral ventricle, associated with focal dilatation of the ventricle and dimpling of the ependymal surface.

Differential Diagnosis:

Open or closed lipped schizencephaly, remote insult with encephalomalacia (would not be grey matter lined)

Discussion:

nMigrational anomaly occurring at approximately 6 weeks gestation.


  -other migrational anomalies may be associated: lissencephaly, polymicrogyria, pachygyria


  -infarct vs. developmental, TORCH


  ->50% have septo-optic dysplasia


  -less common with temporal or occipital clefts.

n  -80% rule- bilateral, open, symmetric

 

nType I- closed lipped, near normal clinically vs. seizures and hemiparesis

 

nType II- open lipped, mental retardation/developmental delay, seizures, spasticity


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