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A case of Ohtahara syndrome with mitochondrial respiratory chain complex I deficiency

Authors
 Joo Hee Seo  ;  Young Mock Lee  ;  Joon Soo Lee  ;  Se Hoon Kim  ;  Heung Dong Kim 
Citation
 Brain & Development, Vol.32(3) : 253-257, 2010 
Journal Title
 Brain & Development 
ISSN
 0387-7604 
Issue Date
2010
Abstract
Ohtahara syndrome (OS) is known as an intractable epileptic syndrome in neonatal and early infantile period, differentiated from early myoclonic encephalopathy (EME) in its etiology. We report a patient with OS associated with mitochondrial respiratory chain complex (MRC) I defect. With ketogenic diet and mitochondrial cocktail therapy, seizures were completely controlled and suppression-burst patterns disappeared 3 months after starting treatment. It is suggested that OS could be caused by specific metabolic disorder such as MRC defect and the intensive therapies including ketogenic diet, vitamin and coenzyme therapy and antioxidant treatment might be helpful for some patients.
Full Text
http://www.sciencedirect.com/science/article/pii/S0387760408003057
DOI
10.1016/j.braindev.2008.12.020
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Se Hoon(김세훈) ORCID logo https://orcid.org/0000-0001-7516-7372
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Seo, Joo Hee(서주희)
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/101223
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