Korean Journal of Pediatrics 2009;52(4):458-463.
Published online April 15, 2009.
Clinical manifestations and neuroimaging findings of schizencephaly in children
Jae Rang Lee, Seung Kim, Young Mock Lee, Joon Soo Lee, Heung Dong Kim
Department of Pediatrics, Severance Children`s Hospital, Yonsei University Collegy of Medicine, Seoul, Korea
소아 뇌갈림증의 신경영상학적 소견 및 임상 양상
이재랑, 김 승, 이영목, 이준수, 김흥동
연세대학교 의과대학 소아과학교실
Correspondence: 
Young Mock Lee, Email: ymleemd@yumc.yonsei.ac.kr
Abstract
Purpose
: Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly.
Methods
: The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively.
Results
: The mean age at diagnosis was 9.08¡¾2.67 months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures.
Conclusion
: Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.
Key Words: Schizencephaly, Epilepsy, Children, MRI


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