title: Long-term coenzyme Q10 therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency: a 31P NMR study.
abstract: For 2 years we administered high doses of coenzyme Q10 (CoQ) to a patient having mitochondrial encephalomyopathy with cytochrome c oxidase deficiency. Abnormal elevation of the serum lactate per pyruvate ratio and the increased concentration of serum lactate plus pyruvate induced by exercise decreased with CoQ treatment. This therapeutic effect continued for 2 years. 31P nuclear magnetic resonance spectroscopy showed acceleration of the postexercise recovery of the ratio of phosphocreatine to inorganic phosphate in muscle during CoQ treatment. These observations support the beneficial effect of CoQ on the impaired mitochondrial oxidative metabolism in muscle. Also, impaired central and peripheral nerve conductivities consistently improved during CoQ treatment. These results indicate that CoQ has clinical value in the long-term management of patients with mitochondrial encephalomyopathies, even though there are clinical limitations to the effects of this therapy.
authors.affiliation: Second Department of Internal Medicine, Osaka University Medical School, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Nishikawa
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: S
authors.initials: S
authors.lastname: Yorifuji
authors.forename: Y
authors.initials: Y
authors.lastname: Nakamura
authors.forename: S
authors.initials: S
authors.lastname: Ueno
authors.forename: S
authors.initials: S
authors.lastname: Tarui
authors.forename: T
authors.initials: T
authors.lastname: Kozuka
authors.forename: T
authors.initials: T
authors.lastname: Nishimura
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D030401:Cytochrome-c Oxidase Deficiency; D005073:Evoked Potentials, Somatosensory; D005080:Exercise Test; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008931:Mitochondria, Muscle; D009135:Muscular Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D010758:Phosphorus; D014451:Ubiquinone / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D014451:Ubiquinone; D010758:Phosphorus