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미토콘드리아 호흡연쇄 복합체 결함이 동반된 난치성 소아 뇌전증에서의 Zonisamide 부가 요법

Other Titles
 Zonisamide Adjunctive Therapy in Intractable Childhood Epilepsy with Mitochondrial Respiratory Chain Complex Defects 
 한정호  ;  이선호  ;  이하늘  ;  김세희  ;  강훈철  ;  이준수  ;  김흥동  ;  이영목 
 Journal of the Korean Child Neurology Society (대한소아신경학회지), Vol.24(3) : 89-94, 2016 
Journal Title
 Journal of the Korean Child Neurology Society  (대한소아신경학회지) 
Issue Date
Zonisamide ; Epilepsy ; Mitochondria ; Respiratory chain ; Free radical
Purpose: 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.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
강훈철(Kang, Hoon Chul) ORCID logo https://orcid.org/0000-0002-3659-8847
김세희(Kim, Se Hee) ORCID logo https://orcid.org/0000-0001-7773-1942
김흥동(Kim, Heung Dong) ORCID logo https://orcid.org/0000-0002-8031-7336
이선호(Lee, Sun Ho)
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
한정호(Han, Jung Ho) ORCID logo https://orcid.org/0000-0001-6661-8127
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