간질발작을 주 증상으로 하는 회백질 이소증을 동반한 Schizencephaly 1례

A Case of Schizencephaly with Heterotopic Gray Matter that was only manifested a Seizure Attack

We experienced a case of schizencephaly with heterotopic gray matter that was only manifested a seizure attack. The clinical manifestation of this patient was only seizure without mental retardation, which was controlled by anticonvulsants. The term schizencephaly was introduced by Yakovlev and Wadsworth in 1946 to describe bilateral, nearly symmetrical and full-thickness clefts, which were characterized by an infolding of cortical gray matter along the clefts. The most widely accepted theory for the pathogenesis of this anomaly is the failure of formation of a segment of the germinal matrix or segmental failure of neuronal migration. Heterotopic gray matter is frequently found within and near the cleft. Patients who have these condition present clinically with seizures and developmental delay, and abnormal motor skills are noted in the more severly affected cases. Recognition is important for planning proper treatment and for genetic counseling