Schizencephaly: a Clinical and CT Study

Schizencephaly is a primary developmental defect of the brain, presumably caused by failure of formation of the cerebral mantle in the regions of the cerebral fissures. Identification has usually been at autopsy, in association with severe neurologic abnormalities. We identified the characteristic features of schizencephaly on CTs in 11 patients. Age at detection ranged from an infant at 8 months to a 30-year-old adult. Clinical abnormalities varied from mild to severe, including developmental delays and retardation, microcephaly, focal or generalized motor abnormalities, and seizures. CT findings included cerebral clefts, infolding of cortical gray matter along the clefts, an abnormal ventricular system, and other associated cerebral anomalies.