Abstract
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.
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Received: 15 October 1997 Received in revised form: 6 February 1998 Accepted: 20 March 1998
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Chan, A., Reichmann, H., Kögel, A. et al. Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy. J Neurol 245, 681–685 (1998). https://doi.org/10.1007/s004150050267
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DOI: https://doi.org/10.1007/s004150050267