在 oculopharyngeal 肌肉发育不良的肌肉强度和运动的表征
Jean-Denis Brisson1,2,3,4, Bernard Brais5, Jean Mathieu1,2,3,4
1Centre de recherche du Centre hospitalier universitaire de Sherbrooke (CRCHUS), Université de Sherbrooke, Sherbrooke, Quebec, Canada.
Muscle & nerve
|October 18, 2023
概括
耳肌肉发育不良 (OPMD) 显著影响肌肉力量和运动能力,65岁以下的患者表现出减弱的能力. 这些发现有助于临床指导,并为未来的OPMD临床试验做好准备.
科学领域:
- 神经学 神经学
- 肌肉骨系统疾病 肌肉骨系统疾病
- 康复医学 康复医学 康复医学
背景情况:
- 耳肌肉发育不良 (OPMD) 是一种罕见的遗传疾病,其特征是肌肉逐渐衰弱.
- 在OPMD中,对肌肉强度和运动限制的定量评估是有限的.
- 了解这些缺陷对于患者管理和治疗发展至关重要.
研究的目的:
- 量化评估OPMD成年人的上肢和下肢肌肉力量,上肢功能,疲劳和运动能力.
- 将这些指标与规范参考值进行比较,并在OPMD队列中的不同年龄组中进行比较.
- 研究OPMD患者肌肉强度,疲劳和运动能力之间的关系.
主要方法:
- 一项涉及34名被诊断患有OPMD的参与者的横截面研究.
- 评估定量最大的同位体肌肉强度,抓地力和挤压力.
- 评估疲劳,行走速度,行走耐力,坐立,爬楼梯/下楼的能力.
主要成果:
- 老年参与者 (≥56岁) 与年轻参与者相比,在五个肌肉群中表现出较低的肌肉强度.
- 在老年人中,关键的功能性指标,包括行走耐力,坐站,楼梯移动性和抓握/捏握强度显著低于参考值的80%.
- 在肌肉强度和运动能力 (ρ=0.42-0.80) 之间以及疲劳和肌肉强度或运动能力 (ρ=0.42-0.75) 之间观察到显著的中度至强度的相关性.
结论:
- OPMD显著损害肌肉力量,耐力和功能能力,甚至在65岁之前就明显出现缺陷.
- 这些发现为医疗保健专业人员提供了宝贵的数据,为OPMD患者提供了改进的临床指导.
- 这些结果通过建立基线功能指标和突出显示结果测量验证的必要性,提高了对临床试验的准备.
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