P-31 NMR-SPECTROSCOPY AND ERGOMETER EXERCISE TEST AS EVIDENCE FOR MUSCLE OXIDATIVE PERFORMANCE IMPROVEMENT WITH COENZYME-Q IN MITOCHONDRIAL MYOPATHIES
P-31 NMR-SPECTROSCOPY AND ERGOMETER EXERCISE TEST AS EVIDENCE FOR MUSCLE OXIDATIVE PERFORMANCE IMPROVEMENT WITH COENZYME-Q IN MITOCHONDRIAL MYOPATHIES
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DOI：
10.1212/wnl.42.6.1203
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发表时间：
1992-06-01
期刊：
影响因子：
9.9
通讯作者：
COZZONE, PJ
中科院分区：
文献类型：
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作者：
BENDAHAN, D;DESNUELLE, C;COZZONE, PJ
2例因复合体I和IV缺乏所致的线粒体脑肌病患者服用辅酶Q10(CoQ)150 mg/d。我们在治疗前和治疗10个月后进行了自行车运动试验和P-31核磁共振波谱的研究。治疗前，我们观察到低的磷酸肌酸/无机磷(PCr/P(I))静息值以及异常高的静息乳酸浓度。在运动过程中，存在明显的酸中毒，运动后血乳酸、pH、聚合酶链式反应和聚合酶链式反应/P(I)比值恢复的动力学延迟。运动中的摄氧量减少，而呼吸阈值的降低表明糖酵解的早期激活。治疗后，自行车运动试验显示静息血乳酸水平下降，运动耗氧量增加，恢复期乳酸消失动力学增强。出现了换气阈值向更高工作量的转变。P-31核磁共振波谱证实了这一改善，显示患者1的安静状态下的Pcr/P(I)比值以及运动后pH、Pcr和Pcr/P(I)比值的恢复动力学显著增加。对于患者2，我们观察到较不明显的酸中毒与运动中产生的较少的P(I)有关。这些观察表明，辅酶Q治疗后，两名患者的线粒体功能都得到了改善，糖酵解活性从高到低。
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 P-31 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. P-31 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 P(i) 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.