Schizencephaly: An Analysis of Clinical and Neuroradiologic Findings.
- Author:
Kwang Deog JO
1
;
Youn Mee HWANG
;
Myoung Chong LEE
Author Information
1. Department of Neurology, College of Medicine, Ulsan University, Korea.
- Publication Type:Original Article
- MeSH:
Arteriovenous Malformations;
Brain;
Cell Movement;
Corpus Callosum;
Dilatation;
Humans;
Intellectual Disability;
Lateral Ventricles;
Lip;
Magnetic Resonance Imaging;
Malformations of Cortical Development*;
Neuronal Migration Disorders;
Seizures;
Septo-Optic Dysplasia
- From:Journal of the Korean Neurological Association
1993;11(3):404-414
- CountryRepublic of Korea
- Language:Korean
-
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.
