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Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency

Authors
 Hoon-Chul Kang  ;  Heung Dong Kim  ;  Young Mok Lee  ;  Si Hoon Han 
Citation
 PEDIATRIC NEUROLOGY, Vol.35(2) : 158-161, 2006 
Journal Title
 PEDIATRIC NEUROLOGY 
ISSN
 0887-8994 
Issue Date
2006
MeSH
Child, Preschool ; Electron Transport Complex I/deficiency* ; Female ; Humans ; Landau-Kleffner Syndrome/complications* ; Landau-Kleffner Syndrome/diagnosis ; Landau-Kleffner Syndrome/therapy ; Mitochondrial Diseases/complications* ; Mitochondrial Diseases/diagnosis ; Mitochondrial Diseases/therapy
Abstract
Landau-Kleffner syndrome is characterized by epileptic aphasia associated with electrical status epilepticus of slow wave sleep. A 5-year-old female, who had manifested normal developmental progress, was referred with principal complaints of fluctuating sensory aphasia and bizarre behavior during the preceding 4 months. Landau-Kleffner syndrome was confirmed by clinical and electroencephalographic features; in addition, the patient’s mitochondrial respiratory chain-complex I deficiency was confirmed by fibroblast culture with the evidence of energy metabolism disorder. This patient’s seizures were intractable to many antiepileptic drugs, adrenocorticotrophic hormone, and intravenous immunoglobulin, with catastrophic cognitive and behavioral decline, but the seizures were successfully controlled by ketogenic diet with supplementary mitochondrial cocktail including coenzyme Q10, riboflavin, l-carnitine, and high-dose multivitamins. The patient finally regained fully normal cognitive functioning. Landau-Kleffner syndrome with mitochondrial respiratory chain-complex I deficiency was controlled in this case by ketogenic diet and supplementary mitochondrial cocktail therapy.
Full Text
http://www.sciencedirect.com/science/article/pii/S088789940600083X
DOI
10.1016/j.pediatrneurol.2006.01.012
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/109327
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