Don Romildo, Serrao Mariano, Vinci Paolo, Ranavolo Alberto, Cacchio Angelo, Ioppolo Francesco, Paoloni Marco, Procaccianti Rita, Frascarelli Flaminia, De Santis Fabio, Pierelli Francesco, Frascarelli Massimo, Santilli Valter
Department of Physical Medicine and Rehabilitation, Movement Analysis Laboratory, La Sapienza University of Rome, Italy.
Clin Biomech (Bristol). 2007 Oct;22(8):905-16. doi: 10.1016/j.clinbiomech.2007.06.002. Epub 2007 Aug 7.
To describe the temporal, kinetic, kinematic, electromyographic and energetic aspects of gait in Charcot-Marie-Tooth patients with foot drop and plantar flexion failure.
A sample of 21 patients fulfilling clinical, electrodiagnostic and genetic criteria for Charcot-Marie-Tooth disease were evaluated by computerized gait analysis system and compared to a group of matched healthy subjects. Patients were classified as having isolate foot drop (group 1) and association of foot drop and plantar flexion failure (group 2).
While it was impossible to detect a reliable gait pattern when the group of patients was considered as a whole and compared to healthy subjects, we observed two distinctive gait patterns when patients were subdivided as group 1 or 2. Group 1 showed a gait pattern with some characteristics of the "steppage pattern". The complex motor strategy adopted by this group leads to reduce the swing velocity and to preserve the step length in spite of a high energy consumption. Group 2 displayed a "clumsy pattern" characterized by very slow gait with reduced step length, a broader support area and great reduction in the cadence. This group of patients is characterized by a low energy consumption and greater energy recovery, due above all to the primary deficit and the various compensatory mechanisms.
Such between-group differences in gait pattern can be related to both primary motor deficits and secondary compensatory mechanisms. Foot drop and plantar flexion failure affect the overall gait strategy in Charcot-Marie-Tooth patients.
描述伴有足下垂和跖屈功能障碍的夏科-马里-图思病患者步态的时间、动力学、运动学、肌电图及能量学方面的特征。
采用计算机化步态分析系统对21例符合夏科-马里-图思病临床、电诊断及遗传学标准的患者进行评估,并与一组匹配的健康受试者进行比较。患者被分为单纯足下垂组(第1组)和足下垂合并跖屈功能障碍组(第2组)。
当将患者组作为一个整体与健康受试者进行比较时,无法检测到可靠的步态模式,但当将患者细分为第1组或第2组时,我们观察到两种不同的步态模式。第1组表现出具有“跨阈步态模式”某些特征的步态模式。尽管能量消耗较高,但该组采用的复杂运动策略导致摆动速度降低并保持步长。第2组表现出“笨拙步态模式”,其特征为步态非常缓慢,步长缩短,支撑面积增大,步频大幅降低。该组患者的特点是能量消耗低且能量恢复能力强,这主要归因于原发性缺陷和各种代偿机制。
步态模式的组间差异可能与原发性运动缺陷和继发性代偿机制有关。足下垂和跖屈功能障碍会影响夏科-马里-图思病患者的整体步态策略。