Busse Monica E, Hughes Gareth, Wiles Charles M, Rosser Anne E
Department of Physiotherapy, School of Healthcare Studies, Cardiff University, Ty Dewi Sant, Heath Park, Cardiff, CF14 4XN, UK.
J Neurol. 2008 Oct;255(10):1534-40. doi: 10.1007/s00415-008-0964-x. Epub 2008 Aug 2.
Sub-clinical muscle involvement, including myopathic changes and mitochondrial dysfunction of skeletal muscle, has been reported in people with Huntington's disease (HD). Muscle strength was evaluated using a hand-held dynamometer. Reliability and validity in people with HD were determined.
Isometric muscle strength of 6 lower limb muscle groups was measured in 20 people with HD and matched healthy controls. People with HD were evaluated with the Unified Huntington's Disease Rating Scales (UHDRS). Within session reliability using intra-class correlation coefficients (ICC) was calculated. Discriminant and convergent validity was also evaluated.
UHDRS motor scores of people with HD ranged from 28 to 80. Reliability of strength testing was excellent (ICC 0.86 to 0.98). People with HD had on average about half the strength of healthy matched controls. UHDRS motor scores and strength scores were significantly correlated (convergent) providing a further indication of validity of strength testing.
The hand-held dynamometer is a reliable and valid measurement tool to detect strength differences between people with HD and a matched control group. There is significant reduction in lower limb muscle strength in HD which does not appear to have been described previously.
据报道，亨廷顿舞蹈症（HD）患者存在亚临床肌肉受累情况，包括骨骼肌的肌病性改变和线粒体功能障碍。使用手持测力计评估肌肉力量。确定其在HD患者中的可靠性和有效性。
对20例HD患者及相匹配的健康对照者测量6组下肢肌肉的等长肌力。HD患者采用统一亨廷顿舞蹈病评定量表（UHDRS）进行评估。计算组内相关系数（ICC）以评估测试过程中的可靠性。同时评估判别效度和聚合效度。
HD患者的UHDRS运动评分范围为28至80分。力量测试的可靠性极佳（ICC为0.86至0.98）。HD患者的平均力量约为相匹配健康对照者的一半。UHDRS运动评分与力量评分显著相关（聚合效度），进一步表明力量测试的有效性。
手持测力计是一种可靠且有效的测量工具，可检测HD患者与相匹配对照组之间的力量差异。HD患者下肢肌肉力量显著降低，这一点此前似乎未见报道。
