Closed Lipped Schizencephaly
Findings:
Multiple MR images demonstrate an abnormally vertical and deep configuration of the left sylvian fissure. A subtle gray matter lined cleft extends to the level of the left lateral ventricle, associated with subtle dimpling of the ventricular margin.
Differential Diagnosis:
Schizencephaly, remote infarct, cortical dysplasia.
Discussion:
Schizencephaly is in the spectrum of migrational anomalies and is differentiated from a zone of neuronal loss and gliosis by the presence of gray matter lining the tract from cortical surface to the ventricle where a dimpled margin is often seen. Pathologically, it is thought to result from disruption of radial glial fibers and/or disordered developmental migration of neurons from the ependymal region to their final position in the peripheral cortex. The cleft may be completely closed or open to variable degrees, with the width of the cleft generally proportionate to clinical sequelae which may range from mild hemiparesis or asymptomatic to severe spasticity and mental retardation and developmental delay with seizure disorders. A remote infarct will not be lined with gray matter.