Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy

J Neurol. 1998 Oct;245(10):681-5. doi: 10.1007/s004150050267.


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.

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Case-Control Studies
  • Coenzymes
  • Exercise Test
  • Female
  • Humans
  • Male
  • Middle Aged
  • Mitochondrial Encephalomyopathies / drug therapy*
  • Sensitivity and Specificity
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / therapeutic use


  • Coenzymes
  • Ubiquinone
  • coenzyme Q10