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The proposal of a clinical protocol to assess central and peripheral fatigue in myotonic dystrophy type 1.
Archives Italiennes De Biologie ( IF 0.8 ) Pub Date : 2017-7-18 , DOI: 10.12871/000398292017125
S Baldanzi 1 , G Ricci , M Bottari , L Chico , C Simoncini , G Siciliano
Affiliation  

DM1 is an autosomal-dominant disorder characterized by muscle weakness, myotonia, and multisystemic involvement. According to current literature fatigue and daytime sleepiness are among the main symptoms of DM1. Oxidative stress has been proposed to be one of the pathogenic factors of fatigue consequent to DM1. In this study, we investigated the dimensions of experienced fatigue and physiological fatigue in a sample of 26 DM1 patients (17 males, 9 females, mean age 41.6 years, SD±12.7); experienced fatigue has been studied through Fatigue Severity Scale (FSS), and physiological fatigue was measured through an intermittent incremental exercise of the forearm muscles using a myometer; oxidative stress balance markers trend during aerobic exercise test have been collected. The occurrence of central fatigue in the sample means that central activation worsens during the motor contraction; interestingly FSS score was significantly correlated to MVC (before and after the effort, r-before=-0.583, p<0.01, r-after= -0.534, p<0.05), and to motor disability measured by MRC (r=-0.496, p<0.05); moreover we found a strong tendency towards significance in the association to lactate baseline (r=0.378, p=0.057).Results are discussed to define whether or not, based on clinical and laboratory grounds, such exercise training protocol may be suitable for proper management of DM1 patients; proper assessment of fatigue should be included in algorithms for data collection in DM1 patient registries.

中文翻译:

建议评估1型强直性肌营养不良症中枢和周围疲劳的临床方案。

DM1是一种常染色体显性疾病,其特征在于肌肉无力,肌强直和多系统受累。根据当前文献,疲劳和白天嗜睡是DM1的主要症状。已经提出氧化应激是DM1导致的疲劳的病因之一。在这项研究中,我们调查了26名DM1患者(17名男性,9名女性,平均年龄41.6岁,SD±12.7)的样本中经历的疲劳和生理性疲劳的大小;已通过疲劳严重程度量表(FSS)研究了经历过的疲劳,并使用肌张力计通过前臂肌肉的间歇性增量运动来测量生理疲劳。有氧运动测试过程中的氧化应激平衡标记趋势已被收集。样本中出现中央疲劳,意味着中央收缩在运动收缩过程中恶化;有趣的是,FSS评分与MVC显着相关(在努力前后,r-before = -0.583,p <0.01,r-after = -0.534,p <0.05),以及通过MRC测量的运动障碍(r = -0.496) ,p <0.05);此外,我们发现了与乳酸基线相关的显着趋势(r = 0.378,p = 0.057)。讨论了根据临床和实验室的依据来定义这种运动训练方案是否适合适当管理的结果DM1患者;DM1患者注册表中用于数据收集的算法中应包括对疲劳的正确评估。r-before = -0.583,p <0.01,r-after = -0.534,p <0.05),通过MRC测得的运动障碍(r = -0.496,p <0.05);此外,我们发现了与乳酸基线相关的显着趋势(r = 0.378,p = 0.057)。讨论了根据临床和实验室的依据来定义这种运动训练方案是否适合适当管理的结果DM1患者;DM1患者注册表中用于数据收集的算法中应包括对疲劳的正确评估。r-before = -0.583,p <0.01,r-after = -0.534,p <0.05),通过MRC测得的运动障碍(r = -0.496,p <0.05);此外,我们发现了与乳酸基线相关的显着趋势(r = 0.378,p = 0.057)。讨论了根据临床和实验室的依据来定义这种运动训练方案是否适合适当管理的结果DM1患者;DM1患者注册表中用于数据收集的算法中应包括对疲劳的正确评估。这种运动训练方案可能适合于DM1患者的适当治疗;DM1患者注册表中用于数据收集的算法中应包括对疲劳的正确评估。这种运动训练方案可能适合于DM1患者的适当治疗;DM1患者注册表中用于数据收集的算法中应包括对疲劳的正确评估。
更新日期:2020-08-21
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