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소아 뇌갈림증의 신경영상학적 소견 및 임상 양상

Other Titles
 Clinical manifestations and neuroimaging findings of schizencephaly in children 
Authors
 이재랑  ;  김 승  ;  이영목  ;  이준수  ;  김흥동 
Citation
 Korean Journal of Pediatrics, Vol.52(4) : 458-463, 2009 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2009
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+/-2.67 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
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DOI
10.3345/kjp.2009.52.4.458
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
김흥동(Kim, Heung Dong) ORCID logo https://orcid.org/0000-0002-8031-7336
이영목(Lee, Young Mock) ORCID logo https://orcid.org/0000-0002-5838-249X
이준수(Lee, Joon Soo) ORCID logo https://orcid.org/0000-0001-9036-9343
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/105153
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