Department of Rehabilitation and Regenerative Medicine, Columbia University Irving Medical Center, New York, New York.
Departments of Biostatistics and Computational Biology and Neurology, University of Rochester, Rochester, New York.
Muscle Nerve. 2023 Jul;68(1):81-84. doi: 10.1002/mus.27765. Epub 2022 Dec 26.
INTRODUCTION/AIMS: Fatiguability and perceived fatigue are common unrelated symptoms in ambulatory individuals with spinal muscular atrophy (SMA). Ratings of perceived exertion (RPE) measures the sense of effort during an activity and has been used as a proxy for fatigue. Relationships between perceived fatigue, fatiguability, and RPE have been described in healthy populations, but the relationship in SMA has not been examined.
Eighteen ambulatory individuals with SMA and 16 age-matched controls (age, 13 to 57 years; 26 [76.5%] males) performed the 6-minute walk test (6MWT) and cardiopulmonary exercise tolerance test (CPET) and completed the International Physical Activity Questionnaire---short form (IPAQ). RPE was collected during the CPET and 6MWT. Fatiguability was measured during the 6MWT. Physical activity (PA) volume was calculated using the IPAQ. Wilcoxon rank-sum tests were used to compare groups. Spearman correlation coefficients evaluated associations between variables. SMA subgroups were predetermined using 6MWT distances of over or under 300 meters.
There were no significant associations between fatiguability and RPE or PA in SMA (P > .05). PA was strongly associated with 6MWT RPE (r = 0.71) in SMA individuals who walked fewer than 300 meters (n = 7). There were no significant associations between any variables in controls (P > .05).
RPE is not associated with fatiguability in SMA. The possible association of PA and RPE may reflect the increased intensity of the 6MWT in weaker patients. RPE represents a sense of effort during exercise and should not be used as a substitute for fatiguability but may be a measure of patient experience during exercise.
简介/目的：在患有脊髓性肌萎缩症（SMA）的门诊患者中，疲劳和感知疲劳是常见的、不相关的症状。感知用力程度（RPE）可衡量活动过程中的用力感，可作为疲劳的替代指标。在健康人群中已经描述了感知疲劳、疲劳性和 RPE 之间的关系，但在 SMA 中尚未进行研究。
18 名患有 SMA 的门诊患者和 16 名年龄匹配的对照者（年龄 13 至 57 岁；26 [76.5%] 名男性）进行了 6 分钟步行测试（6MWT）和心肺运动耐力测试（CPET），并完成了国际体力活动问卷-短表（IPAQ）。CPET 和 6MWT 期间采集 RPE。6MWT 期间测量疲劳性。使用 IPAQ 计算体力活动（PA）量。采用 Wilcoxon 秩和检验比较组间差异。Spearman 相关系数评估变量间的相关性。根据 6MWT 距离（大于或小于 300 米）将 SMA 亚组预先确定。
SMA 患者中疲劳性与 RPE 或 PA 之间无显著相关性（P >.05）。在 6MWT 距离小于 300 米的 SMA 患者（n=7）中，PA 与 6MWT RPE 高度相关（r=0.71）。在对照组中，各变量之间无显著相关性（P >.05）。
RPE 与 SMA 中的疲劳性无相关性。PA 和 RPE 之间的可能相关性可能反映了较弱患者 6MWT 的强度增加。RPE 代表运动过程中的用力感，不应将其用作疲劳性的替代指标，但可能是运动过程中患者体验的指标。
