Department of Human Movement and Sport Sciences, University of Rome Foro Italico, Rome, Italy.
Neuromuscul Disord. 2011 Jan;21(1):52-7. doi: 10.1016/j.nmd.2010.09.008. Epub 2010 Nov 12.
The study aimed at quantifying the walking energy cost of a group of Charcot-Marie-Tooth 1A patients (CMT1A), with low severity of walking impairment, in comparison with healthy individuals. Oxygen uptake was measured in 8 patients (age-range 20-48 years; Barthel >90; Tinetti >20) and 8 healthy individuals, matched for age and gender, when walking on a circuit for 5-min at their self-selected speeds ("slow", "comfortable" and "fast"). Both comfortable and fast speeds were lower in patients than in the control group (0.92±0.16 vs 1.16±0.22 and 1.27±0.27 vs 1.61±0.22 m s⁻¹, respectively; P<0.05), whereas walking energy cost per unit of distance was higher in patients than in the control group (P<0.05) at both "comfortable" (2.27±0.35 vs 1.92±0.21 J kg⁻¹m⁻¹) and "fast" speed (3.05±0.35 vs 2.37±0.42 J kg⁻¹m⁻¹). CMT1A patients, therefore, choose to walk slower but with higher metabolic cost compared to healthy individuals, despite no clinically evident walking impairment, which is likely due to altered walking patterns.
本研究旨在量化一组运动神经元病 1A 型（CMT1A）患者的步行能量消耗，这些患者的步行障碍程度较轻。研究比较了 8 名 CMT1A 患者（年龄 20-48 岁；巴氏指数>90；Tinetti 量表>20）和 8 名健康对照者在以自身选择的速度（“慢”、“舒适”和“快”）在电路上行走 5 分钟时的耗氧量。与对照组相比，患者在舒适和快速速度下的速度均较低（分别为 0.92±0.16 比 1.16±0.22 和 1.27±0.27 比 1.61±0.22 m s⁻¹；P<0.05），而患者在舒适（2.27±0.35 比 1.92±0.21 J kg⁻¹m⁻¹）和快速（3.05±0.35 比 2.37±0.42 J kg⁻¹m⁻¹）速度下每单位距离的步行能量消耗更高（P<0.05）。因此，尽管 CMT1A 患者没有明显的临床步行障碍，但他们选择走得更慢，但代谢成本更高，这可能是由于行走模式的改变所致。
