Presentation
The patient presented with developmental delay and recurrent seizures.
Patient Data
A wide open grey matter lined CSF cleft extending from right lateral ventricle to the cerebral cortex is seen. Another grey matter lined cleft with apposed walls is seen extending from left lateral ventricle to the cerebral cortex.
Bilateral polymicrogyria is present.
Septum pellucidum is absent.
MRI features are suggestive of open lip schizencephaly on right and closed lip schizencephaly on left.
Case Discussion
Schizencephaly is a disorder of neuronal migration characterized by a gray matter lined cerebrospinal fluid–filled cleft, which extends from the ependyma to the pial surface of the brain. Multiple abnormalities can be seen in association with schizencephaly such as absence of septum pellucidum, polymicrogyria, dysgenesis of the corpus callous, and arachnoid cysts.
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