From the Service de Médecine Physique et de Réadaptation, CHU Clermont-Ferrand, INRA, Université Clermont Auvergne, Clermont-Ferrand, France (VR, CM, PG, EC); Service de neurologie, CHU Clermont-Ferrand, INSERM Clermont Auvergne Université, Clermont-Ferrand, France (PC, FT); Clinique Charcot, Sainte-Foy-lès-Lyon, France (CC-C); and Direction recherche clinique et Innovation, CHU Clermont-Ferrand, France (BP).
Am J Phys Med Rehabil. 2019 May;98(5):422-425. doi: 10.1097/PHM.0000000000001084.
Charcot-Marie-Tooth disease type 1A is the most common hereditary neuropathy. Affected individuals have a distal motor deficit, initially affecting the lower limbs and impairing walking performance. Isokinetic dynamometry can be used to objectively assess muscle strength of patients with neuromuscular disorders. No studies have evaluated the effect of muscle strength deficits of knee extensors and flexors on walking parameters for patients with Charcot-Marie-Tooth disease type 1A. The purpose of this study was to determine correlations between the isokinetic muscular strength of knee flexors and knee extensors and walk parameters for patients with Charcot-Marie-Tooth disease type 1A. isokinetic muscular strength of the knee was assessed on an isokinetic dynamometer (Cybex) and walking by instrumented walkway analysis (GaitRite). We included 33 patients (23 females, mean ± SD age 46.7 ± 13.3 yrs, mean ± SD body mass index 25.7 ± 4.6 kg/m). We found a correlation between walking speed and isokinetic muscular strength of knee extensors for the entire population and between walking speed and isokinetic muscular strength of knee extensors and knee flexors for patients younger than 50 yrs. Isokinetic dynamometry can provide objective measures of knee muscle strength, which is correlated with walking speed but not cadence or step/stride length of patients with Charcot-Marie-Tooth disease.
腓骨肌萎缩症 1A 型是最常见的遗传性周围神经病。受影响的个体存在远端运动缺陷，最初影响下肢并损害行走能力。等速测力法可用于客观评估神经肌肉疾病患者的肌肉力量。目前尚无研究评估腓骨肌萎缩症 1A 型患者膝伸肌和屈肌的肌力缺陷对行走参数的影响。本研究旨在确定腓骨肌萎缩症 1A 型患者的膝屈肌和伸肌等速肌力与行走参数之间的相关性。使用等速测力仪（Cybex）评估膝关节等速肌力，使用步态分析系统（GaitRite）进行步行测试。共纳入 33 名患者（23 名女性，平均年龄±标准差为 46.7±13.3 岁，平均 BMI±标准差为 25.7±4.6kg/m2）。我们发现，整个研究人群的步行速度与膝伸肌等速肌力之间存在相关性，而 50 岁以下患者的步行速度与膝伸肌和屈肌等速肌力之间也存在相关性。等速测力法可以提供膝关节肌肉力量的客观测量，与步行速度相关，但与腓骨肌萎缩症患者的步频或步长/跨步长无关。
