Sturge-Weber 증후군에시의 간질성 발작의 유형 및 경과
Features and Outcomes of Epileptic Seizure in Sturge-weber Syndrome
김원섭, 김종신, 김현미, 조강호, 김기중, 박호진, 황용승,
소속 상세정보
김원섭 ( Kim Won-Seop )
서울대학교 의과대학 소아과학교실
김종신 ( Kim Jong-Shin )
서울대학교 의과대학 소아과학교실
김현미 ( Kim Hyun-Mi )
서울대학교 의과대학 소아과학교실
조강호 ( Cho Kang-Ho )
서울대학교 의과대학 소아과학교실
김기중 ( Kim Ki-Joong )
서울대학교 의과대학 소아과학교실
박호진 ( Park Ho-Jin )
서울대학교 의과대학 소아과학교실
황용승 ( Hwang Yong-Seung )
서울대학교 의과대학 소아과학교실
KMID : 0877219970010010125
Abstract
Purpose : It Is well known that various types of epilepsy can occur in the patients with Sturge-Weber syndrome We reviewed the clinical features and outcomes of epileptic seizures in the children with Sturge-Weber syndrome.
Methods : Medical records of the 10 epileptic patients (male 5, female 5) among the 11 children with Sturge-Weber syndrome were reviewed in regard to seizure types. age at onset of seizure, electroencephalographic findings, clinical course and outcomes.
Result : Ten epileptic patients were noted to have 2 simple partial seizure, 2 complex partial seizure and 6 generalized seizure. The background activity of EEG showed focal delta slowings in 8 and diffuse slowings in 2 cases. Multifocal spike discharges were noted in 2 cases. After administration of appropriate entiepileptic drugs, 7 became seizure free. The response rates according to seizure type were as follows simple partial seizure 100%, generalized seizure 83.3 %, complex partial seizure 0%.
Conclusion : The patients with complex partial seizure were less responsive to the medical treatment. Sturge-Weber syndrome showed epilepsies in infancy or early childhood. As lesions of CNS progressed, epilepsy might remain as intractable and defects of CNS progressed, epilepsy might remain as intractable and defects of CNS were aggravated. So, early diagnosis anti treatment aye needed.
키워드
간질성 발작; 스터지-웨버 증후군
Epileptic seizure; Sturge-Weber syndrome
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