Sainte-Justine UHC Research Center, Centre de Réadaptation Marie Enfant, Montreal, QC, Canada.
Department of Physical Activity Sciences, Université du Québec à Montréal (UQAM), Montreal, QC, Canada.
J Neuromuscul Dis. 2022;9(2):311-320. doi: 10.3233/JND-210639.
Myotonic dystrophy type 1 (DM1) is characterized by progressive and predominantly distal muscle atrophy and myotonia. Gait and balance impairments, resulting in falls, are frequently reported in this population. However, the extent to which individuals with DM1 rely more on a specific sensory system for balance than asymptomatic individuals (AI) is unknown.
Evaluate postural control performance in individuals with DM1 and its dependence on vision compared to AI.
20 participants with DM1, divided into two groups based on their diagnosis, i.e. adult and congenital phenotype, and 12 AI participants were recruited. Quiet standing postural control was assessed in two visual conditions: eyes-open and eyes-closed. The outcomes measures were: center of pressure (CoP), mean velocity, CoP range of displacement in anteroposterior and mediolateral axis, and the 95% confidence ellipse's surface. Friedman and Kruskal-Wallis analysis of variance were used to compare outcomes between conditions and groups, respectively.
Significant group effect and condition effect were observed on postural control performance. No significant difference was observed between the two DM1 groups. The significant differences observed between the AI group and the two DM1 groups in the eyes-open condition were also observed in the eyes-closed condition.
The result revealed poorer postural control performance in people with DM1 compared to AI. The DM1 group also showed similar decrease in performance than AI in eyes-closed condition, suggesting no excessive visual dependency.
1 型肌强直性营养不良(DM1)的特征是进行性和主要是远端肌肉萎缩和肌强直。该人群常报告步态和平衡受损,导致跌倒。然而,DM1 个体相对于无症状个体(AI)在多大程度上依赖特定的感觉系统来维持平衡尚不清楚。
评估 DM1 个体的姿势控制表现及其与 AI 相比对视觉的依赖程度。
招募了 20 名 DM1 患者,根据诊断分为成人和先天性表型两组,以及 12 名 AI 参与者。在两种视觉条件下评估安静站立姿势控制:睁眼和闭眼。结果测量指标包括:中心压力(CoP)、平均速度、前后轴和左右轴的 CoP 位移范围以及 95%置信椭圆的表面积。采用 Friedman 和 Kruskal-Wallis 方差分析分别比较条件和组之间的结果。
在姿势控制表现方面观察到显著的组效应和条件效应。两个 DM1 组之间没有观察到显著差异。在睁眼条件下,AI 组与两个 DM1 组之间观察到的显著差异也在闭眼条件下观察到。
结果表明,与 AI 相比,DM1 患者的姿势控制表现较差。DM1 组在闭眼条件下的表现也与 AI 相似,表明对视觉的依赖性不大。