Efficacy of idebenone for respiratory failure in a patient with Leigh syndrome: a long-term follow-up study.
Respiratory failure can be the direct cause of death in patients with Leigh syndrome. Unfortunately, no effective treatment strategy is available. Here, we report successful treatment of a patient with Leigh syndrome using idebenone, a derivative of coenzyme Q-10. The patient's brainstem function, especially respiratory function, improved after idebenone treatment. Idebenone may be worth trying in patients with Leigh syndrome.
Haginoya
Kazuhiro
K
Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai 982-0241, Japan. khaginoya@silk.ocn.ne.jp
Miyabayashi
Shigeaki
S
Kikuchi
Masahiro
M
Kojima
Akira
A
Yamamoto
Katsuya
K
Omura
Kiyoshi
K
Uematsu
Mitsugu
M
Hino-Fukuyo
Naomi
N
Tanaka
Soichiro
S
Tsuchiya
Shigeru
S
D000293:Adolescent; D001933:Brain Stem / Q000187:drug effects / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000187:drug effects; D005500:Follow-Up Studies; D006801:Humans; D019344:Lactic Acid / Q000134:cerebrospinal fluid; D007888:Leigh Disease / Q000150:complications*; D008297:Male; D017286:Polysomnography; D012119:Respiration / Q000187:drug effects; D012131:Respiratory Insufficiency / Q000188:drug therapy* / Q000209:etiology; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
D014451:Ubiquinone; D019344:Lactic Acid; C036619:idebenone