Idebenone improves cerebral mitochondrial oxidative metabolism in a patient with MELAS.
We report a 36-year-old man with MELAS in whom a 5-month course of high-dose oral idebenone, a derivative of coenzyme Q10, increased markedly cerebral metabolic ratio of oxygen and oxygen extraction fraction without increased cerebral blood flow with PET. The results indicate that idebenone improves mitochondrial oxidative metabolism in the brain and suggest a therapeutic potential of idebenone for MELAS.
Ikejiri
Y
Y
Division of Clinical Neurosciences, Hyogo Institute for Aging Brain and Cognitive Disorders, Himeji, Japan.
Mori
E
E
Ishii
K
K
Nishimoto
K
K
Yasuda
M
M
Sasaki
M
M
D000328:Adult; D016227:Benzoquinones / Q000627:therapeutic use*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D017241:MELAS Syndrome / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism*; D014055:Tomography, Emission-Computed; D014451:Ubiquinone / Q000031:analogs & derivatives
D016227:Benzoquinones; D014451:Ubiquinone; C036619:idebenone