Schizencephaly : An Analysis of Clinical and Neuroradiologic Findings

J Korean Neurol Assoc > Volume 11(3); 1993 > Article

Journal of the Korean Neurological Association 1993;11(3): 404-414.

Schizencephaly의 임상적 및 신경방사선 소견에 관한 고찰

조광덕, 황연미,이명종

울산대학교 신경과.

Schizencephaly : An Analysis of Clinical and Neuroradiologic Findings

Kwang-Deog Jo, M.D., Youn-Mee Hwang, M.D., Myoung-Chong Lee, M.D.

Department of Neurology, College of Medicine Ulsan University

Abstract

Schizencephaly is a congenital brain anomaly, a disorder of cell migration characterized by gray matter-lined clefts, which is caused by faulty migration of the subependymal neuroblasts. The cleft may be closed (Type I) or open by a cavity communicating with the lateral ventricle (Type II). We identified the characteristic features of schizencephaly on CTs in one patient and on MRIs in 15 patients. Age at detection ranged from 16 months to 37 years. Three patients had bilateral clefts and 13 patients had unilateral cleft. Twelve patients had closed lip cleft whlle four patients had open lip cleft. Small sized unilateral closed cleft, the most comrnon type, was found in ten patients. Three patients had multiple clefts. Clinically these patients presented with seizures in 15 patients, motor disturbance in six patients, speech impairment in five patients, developemental delay in four patients, and mental retardation in three patients. The presence and severity of these symptoms correlated with the size and location of the lesions. Associated cerebral anomalies included ventricular dilatation in seen patients, agenesis of septum pallucidum in seven patients, other neuronal migration disorder in four patients, agenesis or dysgenesis of corpus callosum in three patients, septo-optic dysplasia in two patients, abnormal extension of svlvian fissure in one patient, and arteriovenous malformation in one patient.