Myotonic dystrophy type 1 (DM1) is a multisystemic disease characterized by progressive muscle weakness. The aim of this project is to evaluate the effects of a 12-week lower limb strength training program in 11 men with DM1. Maximal isometric muscle strength, 30-second sit-to-stand, comfortable and maximal 10-m walk test (10 mwt) were evaluated at baseline, 6 and 12 weeks, and at 6 and 9 months. The one-repetition maximum strength evaluation method of the training exercises was completed at baseline, 6 and 12 weeks. Muscle biopsies were taken in the vastus lateralis at baseline and 12 weeks to evaluate muscle fiber typing and size (including atrophy/hypertrophy factors). Performance in strength and functional tests all significantly improved by week 12. Maximal isometric muscle strength of the knee extensors decreased by month 9, while improved walking speed and 30 second sit-to-stand performance were maintained. On average, there were no significant changes in fiber typing or size after training. Further analysis showed that individual abnormal hypertrophy factor at baseline could explain the different changes in muscle size among participants. Strength training induces maximal isometric muscle strength and lasting functional gains in DM1. Abnormal hypertrophy factor could be a key component to identify high and low responders to hypertrophy in DM1.

中文翻译：

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力量训练可有效缓解 1 型强直性营养不良的骨骼肌损伤

肌强直性营养不良 1 型 (DM1) 是一种以进行性肌无力为特征的多系统疾病。该项目的目的是评估为期 12 周的下肢力量训练计划对 11 名患有 DM1 的男性的影响。在基线、第 6 周和第 12 周以及第 6 和 9 个月时评估了最大等长肌肉力量、30 秒从坐到站、舒适和最大 10 米步行测试 (10 mwt)。训练练习的一次重复最大力量评估方法在基线、6和12周完成。在基线和 12 周时对股外侧肌进行肌肉活检，以评估肌纤维类型和大小（包括萎缩/肥大因素）。力量和功能测试的表现在第 12 周都有显着改善。膝伸肌的最大等长肌肉力量在第 9 个月下降，同时保持提高的步行速度和 30 秒从坐到站的性能。平均而言，训练后的纤维类型或大小没有显着变化。进一步的分析表明，基线时的个体异常肥大因子可以解释参与者肌肉大小的不同变化。力量训练可在 DM1 中诱导最大等长肌肉力量和持久的功能增益。异常肥大因子可能是确定 DM1 肥大反应高和低的关键组成部分。力量训练可在 DM1 中诱导最大等长肌肉力量和持久的功能增益。异常肥大因子可能是确定 DM1 肥大反应高和低的关键组成部分。力量训练可在 DM1 中诱导最大等长肌肉力量和持久的功能增益。异常肥大因子可能是确定 DM1 肥大反应高和低的关键组成部分。