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미토콘드리아 호흡연쇄 복합체 결함이 동반된 난치성 소아 뇌전증에서의 Zonisamide 부가 요법

DC Field Value Language
dc.contributor.author강훈철-
dc.contributor.author김흥동-
dc.contributor.author이선호-
dc.contributor.author이영목-
dc.contributor.author이준수-
dc.contributor.author한정호-
dc.contributor.author김세희-
dc.date.accessioned2017-10-26T07:42:00Z-
dc.date.available2017-10-26T07:42:00Z-
dc.date.issued2016-
dc.identifier.issn1226-6884-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/152397-
dc.description.abstractPurpose: Zonisamide (ZNS) is a broad-spectrum antiepileptic drug (AED). Free radical scavenging is also a possible action mechanism of ZNS. As oxidative stress plays an important role in the pathogenesis of mitochondrial cytopathy, we studied the efficacy and safety of ZNS as possible long-term adjunctive therapy in intractable childhood epilepsy with mitochondrial respiratory chain complex (MRC) defects. Methods: MRC defects were confirmed by biochemical assays with muscle biopsies. We evaluated seizure frequencies and adverse effects in 38 childhood epilepsy patients with MRC defects who were maintained on ZNS for at least 12 months. Patients with more than 50% seizure reduction after 12 months were defined as responders. Results: As for clinical diagnosis of mitochondrial cytopathy, 13 cases (34.2%) were diagnosed as Leigh syndrome and the remaining 25 (65.8%) had uncategorized mitochondrial cytopathy with nonspecific encephalopathy. The response rates for each seizure type after 12 months of ZNS therapy ranged from 23.1 to 55.6%; partial seizures (55.6%; 10/18), absence seizures (50.0%; 2/4), tonic-clonic seizures (40.0%; 2/5), atonic seizures (36.4%; 4/11), spasms (33.3%; 2/6), tonic seizures (31.8%; 7/22) and myoclonic seizures (23.1%; 3/13). The response rate for epileptic syndromes such as West syndrome and Lennox-Gastaut syndrome after ZNS treatment are 33.3% and 55.6% respectively. Somnolence, irritability, anorexia and weight loss were noticed as adverse effects, but all were transient. Conclusion: ZNS could be an effective and safe candidate for adjunctive therapy in intractable childhood epilepsy with MRC defects. It could be considered preferentially for its free radical scavenging effect.-
dc.description.statementOfResponsibilityrestriction-
dc.languageKorean-
dc.publisher대한소아신경학회-
dc.relation.isPartOfJournal of the Korean Child Neurology Society-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.title미토콘드리아 호흡연쇄 복합체 결함이 동반된 난치성 소아 뇌전증에서의 Zonisamide 부가 요법-
dc.title.alternativeZonisamide Adjunctive Therapy in Intractable Childhood Epilepsy with Mitochondrial Respiratory Chain Complex Defects-
dc.typeArticle-
dc.publisher.locationKorea-
dc.contributor.collegeCollege of Medicine-
dc.contributor.departmentDept. of Pediatrics-
dc.contributor.googleauthor한정호-
dc.contributor.googleauthor이선호-
dc.contributor.googleauthor이하늘-
dc.contributor.googleauthor김세희-
dc.contributor.googleauthor강훈철-
dc.contributor.googleauthor이준수-
dc.contributor.googleauthor김흥동-
dc.contributor.googleauthor이영목-
dc.contributor.localIdA01208-
dc.contributor.localIdA04845-
dc.contributor.localIdA02955-
dc.contributor.localIdA03177-
dc.contributor.localIdA05064-
dc.contributor.localIdA00611-
dc.contributor.localIdA00102-
dc.relation.journalcodeJ01815-
dc.identifier.urlhttp://scholar.dkyobobook.co.kr/searchDetail.laf?barcode=4010024981517-
dc.subject.keywordZonisamide-
dc.subject.keywordEpilepsy-
dc.subject.keywordMitochondria-
dc.subject.keywordRespiratory chain-
dc.subject.keywordFree radical-
dc.contributor.alternativeNameKang, Hoon Chul-
dc.contributor.alternativeNameKim, Heung Dong-
dc.contributor.alternativeNameLee, Sun Ho-
dc.contributor.alternativeNameLee, Young Mock-
dc.contributor.alternativeNameLee, Joon Soo-
dc.contributor.alternativeNameHan, Jung Ho-
dc.contributor.alternativeNameKim, Se Hee-
dc.contributor.affiliatedAuthorKim, Heung Dong-
dc.contributor.affiliatedAuthorLee, Sun Ho-
dc.contributor.affiliatedAuthorLee, Young Mock-
dc.contributor.affiliatedAuthorLee, Joon Soo-
dc.contributor.affiliatedAuthorHan, Jung Ho-
dc.contributor.affiliatedAuthorKim, Se Hee-
dc.contributor.affiliatedAuthorKang, Hoon Chul-
dc.citation.volume24-
dc.citation.number3-
dc.citation.startPage89-
dc.citation.endPage94-
dc.identifier.bibliographicCitationJournal of the Korean Child Neurology Society, Vol.24(3) : 89-94, 2016-
dc.date.modified2017-10-24-
dc.identifier.rimsid48604-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers

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