Tramonti Caterina, Dalise Stefania, Bertolucci Federica, Rossi Bruno, Chisari Carmelo
Unit of Neurorehabilitation, Department of Neuroscience, University Hospital of Pisa , Italy.
Eur J Transl Myol. 2014 Dec 24;24(4):4726. doi: 10.4081/ejtm.2014.4726. eCollection 2014 Nov 28.
Myotonic Dystrophy type 1 (DM1) is a dominantly inherited disease comprehending multiple features. Fatigue and exhaustion during exercise often represent significant factors able to negatively influence their compliance to rehabilitation programs. Mitochondrial abnormalities and a significant increase in oxidative markers, previously reported, suggest the hypothesis of a mitochondrial functional impairment. The study aims at evaluating oxidative metabolism efficiency in 18 DM1 patients and in 15 healthy subjects, through analysis of lactate levels at rest and after an incremental exercise test. The exercise protocol consisted of a submaximal incremental exercise performed on an electronically calibrated treadmill, maintained in predominantly aerobic condition. Lactate levels were assessed at rest and at 5, 10 and 30 minutes after the end of the exercise. The results showed early exercise-related fatigue in DM1 patients, as they performed a mean number of 9 steps, while controls completed the whole exercise. Moreover, while resting values of lactate were comparable between the patients and the control group (p=0.69), after the exercise protocol, dystrophic subjects reached higher values of lactate, at any recovery time (p<0,05). These observations suggest an early activation of anaerobic metabolism, thus evidencing an alteration in oxidative metabolism of such dystrophic patients. As far as intense aerobic training could be performed in DM1 patients, in order to improve maximal muscle oxidative capacity and blood lactate removal ability, then, this safe and validate method could be used to evaluate muscle oxidative metabolism and provide an efficient help on rehabilitation programs to be prescribed in such patients.
1型强直性肌营养不良症(DM1)是一种具有多种特征的显性遗传病。运动过程中的疲劳和疲惫往往是对康复计划依从性产生负面影响的重要因素。先前报道的线粒体异常和氧化标志物显著增加提示线粒体功能受损的假说。本研究旨在通过分析静息状态和递增运动试验后的乳酸水平,评估18例DM1患者和15名健康受试者的氧化代谢效率。运动方案包括在电子校准跑步机上进行次最大递增运动,保持主要为有氧状态。在静息状态以及运动结束后5、10和30分钟评估乳酸水平。结果显示,DM1患者在运动早期出现疲劳,因为他们平均走了9步,而对照组完成了整个运动。此外,虽然患者和对照组之间的静息乳酸值相当(p=0.69),但在运动方案后,营养不良受试者在任何恢复时间的乳酸值都更高(p<0.05)。这些观察结果提示无氧代谢早期激活,从而证明此类营养不良患者的氧化代谢存在改变。那么,就DM1患者可以进行高强度有氧训练以提高最大肌肉氧化能力和血乳酸清除能力而言,这种安全且经过验证的方法可用于评估肌肉氧化代谢,并为这类患者制定的康复计划提供有效帮助。