Serrao Mariano, Ranavolo Alberto, Casali Carlo
Department of Medical and Surgical Sciences and Biotechnologies, University of Rome Sapienza, Latina, Italy.
Department of Occupational and Environmental Medicine, Epidemiology and Hygiene, Istituto Nazionale per l'Assicurazione contro gli Infortuni sul Lavoro, Rome, Italy.
Handb Clin Neurol. 2018;154:299-303. doi: 10.1016/B978-0-444-63956-1.00018-7.
Beyond the classic clinical description, recent studies have quantitatively evaluated gait and balance dysfunction in cerebellar ataxias by means of modern motion analysis systems. These systems have the aim of clearly and quantitatively describing the differences, with respect to healthy subjects, in kinematic, kinetic, and surface electromyography variables, establishing the basis for a rehabilitation strategy and assessing its efficacy. The main findings which characterize the gait pattern of cerebellar patients are: increased step width, reduced ankle joint range of motion with increased coactivation of the antagonist muscles, and increased stride-to-stride variability. Whereas the former is a compensatory strategy adopted by patients to keep the center of mass within the base of support, the latter indicates the inability of patients to maintain dynamic balance through a regular walking pattern and may reflect the primary deficit directly related to cerebellar dysfunction and the consequent lack of muscle coordination during walking. Moreover, during the course of the disease, with the progressive loss of walking autonomy, step length, and lower-limb joint range of motion are drastically reduced. As to the joint coordination defect, abnormal intralimb joint coordination during walking, in terms of both joint kinematics and interaction torques, has been reported in several studies. Furthermore, patients with cerebellar ataxia show a poor intersegmental coordination, with a chaotic coordinative behavior between trunk and hip, leading to increased upper-body oscillations that affect gait performance and stability, sustaining a vicious circle that transforms the upper body into a generator of perturbations. The use of motion analysis laboratories allows a deeper segmental and global characterization of walking impairment in these patients and can shed light on the nature of both the primary specific gait disorder and compensatory mechanisms. Such deeper understanding might reasonably represent a valid prerequisite for establishing better-targeted rehabilitation strategies.
除了经典的临床描述外,最近的研究还通过现代运动分析系统对小脑共济失调患者的步态和平衡功能障碍进行了定量评估。这些系统旨在清晰、定量地描述小脑共济失调患者与健康受试者在运动学、动力学和表面肌电图变量方面的差异,为康复策略奠定基础并评估其疗效。小脑共济失调患者步态模式的主要特征包括:步宽增加、踝关节活动范围减小且拮抗肌共同激活增加、步幅间变异性增加。前者是患者采用的一种补偿策略,用于将重心保持在支撑面内,而后者则表明患者无法通过规律的行走模式维持动态平衡,可能直接反映了与小脑功能障碍相关的主要缺陷以及行走过程中随之而来的肌肉协调不足。此外,在疾病过程中,随着行走自主性的逐渐丧失,步长和下肢关节活动范围会大幅减小。关于关节协调缺陷,多项研究报告了行走过程中肢体内部关节在运动学和相互作用扭矩方面的异常协调情况。此外,小脑共济失调患者表现出节段间协调性差,躯干和髋部之间存在混乱的协调行为,导致上身摆动增加,影响步态表现和稳定性,形成一个恶性循环,使上身成为干扰的产生源。使用运动分析实验室可以更深入地对这些患者的行走障碍进行节段性和整体性特征描述,并能够阐明原发性特定步态障碍和代偿机制的本质。这种更深入的理解可能合理地成为制定更具针对性的康复策略的有效前提。