Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy.
We used a standardized bicycle ergometry protocol with a stepwise increasing workload (30-100 W) to evaluate various metabolic factors for the diagnosis and metabolic monitoring of mitochondrial encephalomyopathies. All patients (n = 9) showed pathological venous lactate/pyruvate (L/P) ratios, which normalized in three patients after 6 months of coenzyme Q10 (CoQ) therapy. Thus, the L/P ratio proved to be the clinically most useful parameter in the evaluation and monitoring of mitochondrial diseases, showing higher sensitivity than lactate measurements only. CoQ may exert a favourable effect in some patients with mitochondrial diseases.
Neurologische Universitätsklinik, Würzburg.
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Chan
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Reichmann
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Kögel
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Beck
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Gold
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D016022:Case-Control Studies; D003067:Coenzymes; D005080:Exercise Test; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017237:Mitochondrial Encephalomyopathies / Q000188:drug therapy*; D012680:Sensitivity and Specificity; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
D003067:Coenzymes; D014451:Ubiquinone; C024989:coenzyme Q10