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晚发庞培病成年人的神经肌肉表现的表征:一个对照病例横截面研究研究

Théo Maulet¹, Céline Bonnyaud², Pascal Laforêt³

¹Laboratory End: icap, Inserm Unit 1179, UVSQ, 2 Av. de la Source de la Bièvre, 78180 Montigny-le-Bretonneux France; Paris-Saclay University, Research Unit ERPHAN, UVSQ, 2 Av. de la Source de la Bièvre, 78180 Montigny-le-Bretonneux France; Movement analysis laboratory, Functional exploration unit, APHP, Raymond Poincaré hospital, 104 Bd Raymond Poincaré, 92380 Garches, France.

Neuromuscular disorders : NMD

|November 21, 2023

概括

患有晚发佩病 (aLOPD) 的成年人经历了最大肌肉力量的减少,特别是在部肌肉中. 然而,它们的肌肉激活,爆炸性强度和耐疲劳能力与健康个体相当.

科学领域:

神经肌肉生理学神经肌肉生理学
罕见疾病罕见疾病
肌肉生物学肌肉生物学

背景情况:

晚期佩病 (aLOPD) 涉及逐渐的肌肉恶化.
在aLOPD患者中神经肌肉性能仍然不完全理解.

研究的目的:

在aLOPD患者和匹配的对照者之间比较最大的肌肉力量,激活,爆炸力和神经肌肉疲劳.
研究LOPD对神经肌肉功能各方面的影响.

主要方法:

评估了20名LOPD患者和20名对照人群.
对部,膝盖和脚肌肉的测量异比最大自愿收缩 (MVC) 扭矩.
在持续的收缩过程中评估自愿激活水平 (VAL),扭矩发展速度 (RTD) 和神经肌肉疲劳.

主要成果:

与对照组相比,aLOPD患者在所有测量肌肉群中表现出明显较低的最大肌肉强度 (MVC扭矩).
最受影响的肌肉是部延伸肌肉,其次是部引肌肉和引肌肉,表明近距离到远距离的弱势梯度.
在正常化的RTD,VAL或神经肌肉疲劳水平中,没有发现群体之间的显著差异.

结论:

最大肌肉力量是LOPD中主要神经肌肉特征受损的.
在LOPD患者中,剩余肌肉组织的功能效率保持不变,与对照个体的功能效率相当.
研究结果表明,专注于力量维护的有针对性的干预措施可能对管理LOPD有益.

关键词:

爆炸强度的爆炸强度.仪器评估是指使用仪器进行评估.晚期发病的庞贝病晚期发病的庞贝病最大肌肉力量最大的肌肉力量.肌肉激活肌肉激活神经肌肉疲劳 - 神经肌肉疲劳

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