Neuromuscular Disorders Reference Centre, Lille University Medical Center, University of Lille 2, Lille, France.
Neuromuscul Disord. 2012 Aug;22(8):720-7. doi: 10.1016/j.nmd.2012.04.011. Epub 2012 Jun 8.
Several authors have reported alterations in vasodilation during effort in patients with dystrophinopathies, in which a lack of neuronal NO synthase is thought to lead to functional muscle ischemia. In order to determine changes in muscle oxygenation during effort in patients with Becker muscular dystrophy (BMD) and assess the parameters' links with disease severity and functional status, 10 BMD patients and 10 age-matched controls performed isokinetic, constant-load knee extension exercises at (i) 20% of their extensors' peak torque (i.e. the same relative load) and (ii) the same absolute load (20 Nm). Muscle oxygenation was evaluated noninvasively using near-infrared spectroscopy (NIRS), with the time course of deoxygenation as the main criterion. As expected, BMD patients displayed a lower peak torque than controls (-62%). During both types of exercise, initial muscle deoxygenation was faster (by 27-41%) in BMD patients than in controls. Greater disease severity (according to the Motor Function Measure) and functional impairment (walking endurance) were associated with a faster second deoxygenation phase (τ). The validity and relevance of muscle deoxygenation parameters and the alteration of vasodilatation by nNOS deficiency in dystrophinopathies should be assessed by further studies.
几位作者报道了在神经原型一氧化氮合酶缺乏导致功能性肌肉缺血的肌营养不良症患者中,运动时血管舒张的改变。为了确定 Becker 型肌营养不良症(BMD)患者运动时肌肉氧合的变化,并评估这些参数与疾病严重程度和功能状态的关系,10 名 BMD 患者和 10 名年龄匹配的对照者在(i)20%的伸肌峰值扭矩(即相同的相对负荷)和(ii)相同的绝对负荷(20 Nm)下进行等速、恒负荷膝关节伸展运动。使用近红外光谱(NIRS)非侵入性地评估肌肉氧合,脱氧的时间过程是主要标准。正如预期的那样,BMD 患者的峰值扭矩比对照组低(-62%)。在这两种运动中,BMD 患者的初始肌肉脱氧速度比对照组快(快 27-41%)。更大的疾病严重程度(根据运动功能测量)和功能障碍(行走耐力)与更快的第二次脱氧阶段(τ)相关。进一步的研究应评估肌肉脱氧参数的有效性和相关性,以及神经原型一氧化氮合酶缺乏对肌营养不良症中血管舒张的改变。