Newman Christopher John, Walsh Michael, O'Sullivan Rory, Jenkinson Ann, Bennett Damien, Lynch Bryan, O'Brien Timothy
Central Remedial Clinic, Vernon Avenue, Clontarf, Dublin 3, Ireland.
Gait Posture. 2007 Jun;26(1):120-7. doi: 10.1016/j.gaitpost.2006.08.006. Epub 2006 Sep 28.
Certain typical gait characteristics such as foot-drop and foot supination are well described in Charcot-Marie-Tooth disease. These are directly related to the primary disease and due to the weakness of ankle dorsiflexors and everters characteristic of this hereditary neuropathy. We analysed 16 subjects aged 8-52 years old (11 with type I, 5 with type II Charcot-Marie-Tooth disease) using three-dimensional gait analysis and identified kinematic features previously unreported. These patients showed a combination of tight tendo achillei, foot-drop, failure of plantar flexion and increased foot supination, but also presented with excessive internal rotation of the knee and/or tibia, knee hyperextension in stance, excessive external rotation at the hips and decreased hip adduction in stance (typical of a broad based gait). These proximal features could have been an adaptation to or consequence of the disrupted ankle and foot biomechanics, however a direct relation to the neuropathy is also possible since sub-normal muscle power was observed at the proximal levels in most subjects on both manual testing and kinetic analysis. Gait analysis is a useful tool in defining the characteristic gait of patients with Charcot-Marie-Tooth disease.
某些典型的步态特征，如足下垂和足内翻，在夏科-马里-图斯病中有详细描述。这些特征与原发性疾病直接相关，是由这种遗传性神经病变所特有的踝背屈肌和外翻肌无力导致的。我们使用三维步态分析对16名年龄在8至52岁之间的受试者（11例I型、5例II型夏科-马里-图斯病患者）进行了分析，并确定了此前未报告的运动学特征。这些患者表现出跟腱紧张、足下垂、跖屈功能障碍和足内翻增加的综合症状，同时还存在膝关节和/或胫骨过度内旋、站立时膝关节过度伸展、髋关节过度外旋以及站立时髋关节内收减少（典型的宽基步态）。这些近端特征可能是对踝关节和足部生物力学紊乱的一种适应或结果，然而，由于在大多数受试者的近端水平上，通过手动测试和动力学分析均观察到肌肉力量低于正常水平，因此也有可能与神经病变直接相关。步态分析是确定夏科-马里-图斯病患者特征性步态的有用工具。
