Center for Motion Analysis, Connecticut Children's Medical Center, 399 Farmington Ave. Farmington, CT, USA.
Center for Motion Analysis, Connecticut Children's Medical Center, 399 Farmington Ave. Farmington, CT, USA; Division of Orthopedics, Connecticut Children's Medical Center, 399 Farmington Ave. Farmington, CT, USA.
Neuromuscul Disord. 2020 Oct;30(10):825-832. doi: 10.1016/j.nmd.2020.08.359. Epub 2020 Aug 21.
The purpose of this study is to assess how Charcot-Marie-Tooth disease, a group of inherited peripheral neuropathies that result in distal weakness, affects walking velocity over time in comparison to age-matched controls. Comprehensive gait analysis of 57 children (mean age 12.0, SD 3.7 years) compared to 76 age-matched controls (mean age 10.1, SD 3.4 years) demonstrated slower walking velocity (p<0.001) due to both shorter stride length (p<0.001) and diminished cadence (p=0.01). There was higher walking velocity (p<0.001), stride length (p=0.002) and cadence (p<0.001) in patients with dorsiflexor strength ≥3 and higher walking velocity (p=0.001) and cadence (p=0.03) in patients plantar flexor strength ≥4. Analysis of Charcot-Marie-Tooth type 1 and type 2 subgroups showed that walking velocity increased significantly with age in controls (p=0.001) but did not increase in children with either subtype (p>0.54). Stride length increased significantly with age in all groups (p<0.001) but at a slower rate in type 1 and 2 compared to controls. These differences contributed to increasing deficits in walking velocity and stride length with age in type 1 and 2 in comparison to controls, with deficits appearing earlier in type 2. Since the slower walking velocity in children with Charcot-Marie-Tooth disease is primarily due to short stride length, treatments that enable improved stride length, such as plantar flexor strengthening and bracing, may improve walking velocity and associated gait function.
本研究旨在评估遗传性周围神经病——腓骨肌萎缩症（Charcot-Marie-Tooth disease），其特征为远端肌无力，如何随时间影响步行速度，与年龄匹配的对照组进行比较。对 57 名儿童（平均年龄 12.0 岁，标准差 3.7 岁）和 76 名年龄匹配的对照组（平均年龄 10.1 岁，标准差 3.4 岁）进行全面步态分析，结果显示腓骨肌萎缩症患儿的步行速度较慢（p<0.001），原因是步幅较短（p<0.001）和步频降低（p=0.01）。腓骨长肌力量≥3 的患者具有更高的步行速度（p<0.001）、步幅（p=0.002）和步频（p<0.001），而跖屈肌力量≥4 的患者具有更高的步行速度（p=0.001）和步频（p=0.03）。腓骨肌萎缩症 1 型和 2 型亚组分析显示，对照组的步行速度随年龄显著增加（p=0.001），但 1 型和 2 型患儿的步行速度并未增加（p>0.54）。所有组的步幅都随年龄显著增加（p<0.001），但 1 型和 2 型的增加速度较对照组较慢。这些差异导致 1 型和 2 型患儿的步行速度和步幅随年龄增长而逐渐出现缺陷，2 型患儿的缺陷出现更早。由于腓骨肌萎缩症患儿的步行速度较慢主要是由于步幅较短，因此，改善步幅的治疗方法，如跖屈肌力量增强和支具固定，可能会改善步行速度和相关步态功能。
