31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies.

Abstract

Two patients with mitochondrial encephalomyopathy due to complexes I and IV deficiencies received 150 mg/d of coenzyme Q10 (CoQ). We studied them with a bicycle ergometer exercise test and 31P NMR spectroscopy before and after 10 months of treatment. Before treatment, we observed a low phosphocreatine/inorganic phosphate (PCr/P(i)) resting value along with abnormally high resting lactate concentration. During exercise, there was a pronounced acidosis with delayed kinetics of postexercise recovery for blood lactate, pH, PCr, and PCr/P(i) ratio. Oxygen uptake during exercise was reduced while the lowering of the ventilatory threshold indicated an early activation of glycolysis. After treatment, the bicycle ergometer exercise test indicated a significant improvement with a decrease in resting blood lactate level, an increase in oxygen consumption during exercise, and an increase in the kinetics of lactate disappearance during the recovery period. A shift of the ventilatory threshold to higher workload was present. 31P NMR spectroscopy confirmed the improvement, showing a significant increase in the PCr/P(i) ratio at rest and in the kinetics of recovery for pH, PCr, and PCr/P(i) ratio following exercise in patient 1. For patient 2, we observed a less pronounced acidosis correlated with a lesser amount of Pi produced during exercise. These observations indicate an improvement of mitochondrial function and a shift from high to low glycolytic activity in both patients consequent to CoQ treatment.

• Authors: D Bendahan, C Desnuelle, D Vanuxem, S Confort-Gouny, D Figarella-Branger, J F Pellissier, G Kozak-Ribbens, J Pouget, G Serratrice, P J Cozzone
• Journal: Neurology (Neurology) Vol. 42 Issue 6 Pg. 1203-8 (Jun 1992) ISSN: 0028-3878 [Print] United States
• PMID: 1603348 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Ubiquinone
• Phosphorus

Topics

• Adolescent
• Adult
• Exercise Test
• Humans
• Magnetic Resonance Spectroscopy
• Male
• Mitochondria, Muscle
• Muscles (metabolism)
• Muscular Diseases (diagnosis, drug therapy, metabolism)
• Oxidation-Reduction
• Phosphorus
• Ubiquinone (therapeutic use)