Arch Neurol. 1996 Feb;53(2):141-7.
To test the utility of a new, easy to administer instrument for assessing activities of daily living in patients with amyotrophic lateral sclerosis (ALS), to validate its accuracy, and to assess its ability to record disease progression in patients with ALS against other functional scales, quantitative isometric muscle testing, and global assessment scales.
Serial assessments of patients who presented to four ALS treatment centers in two multicenter studies.
Study 1 (cross-sectional) evaluated 75 consecutive patients who presented to four ALS treatment centers during a 2-month period. Study 2 (longitudinal) evaluated the progression of 53 patients who were enrolled in a multicenter, phase I-II clinical trial of recombinant human ciliary neurotrophic factor for treatment of ALS.
The ALS Functional Rating Scale (ALSFRS) was compared with quantitative myometry and with other measures of daily function in patients with ALS both cross-sectionally and longitudinally.
The first study of 75 patients evaluated the internal consistency, the test-retest reliability, and the construct validity of the ALSFRS. Internal consistency and test-retest reliability were high. Patient self-rating of upper- and lower-extremity-dependent tasks were highly correlated with measures of upper- and lower-extremity strength, respectively. Thus, the ALSFRS has good construct validity. In the second study, ALSFRS scores declined in tandem with deterioration in motor and pulmonary function, indicating its sensitivity to change.
The ALSFRS is a useful instrument for evaluation of functional status and functional change in patients with ALS. Its results are in close agreement with objective measures of muscle strength and pulmonary function. The ALSFRS may be used as a screening measure for entry into clinical trials, as a surrogate measure of function in situations in which muscle strength cannot be measured directly, or as an adjunct to myometry.
测试一种新型、易于使用的工具评估肌萎缩侧索硬化症（ALS）患者日常生活活动能力的效用，验证其准确性，并评估其与其他功能量表、定量等长肌肉测试及整体评估量表相比，记录ALS患者疾病进展的能力。
在两项多中心研究中，对前往四个ALS治疗中心就诊的患者进行系列评估。
研究1（横断面研究）评估了在2个月期间前往四个ALS治疗中心就诊的75例连续患者。研究2（纵向研究）评估了53例参与重组人睫状神经营养因子治疗ALS的多中心I-II期临床试验患者的病情进展。
将ALS功能评定量表（ALSFRS）与定量肌功能测试以及ALS患者日常功能的其他指标进行横断面和纵向比较。
对75例患者的第一项研究评估了ALSFRS的内部一致性、重测信度和结构效度。内部一致性和重测信度较高。患者对上肢和下肢相关任务的自我评分分别与上肢和下肢力量测量高度相关。因此，ALSFRS具有良好的结构效度。在第二项研究中，ALSFRS评分随着运动和肺功能的恶化而下降，表明其对变化的敏感性。
ALSFRS是评估ALS患者功能状态和功能变化的有用工具。其结果与肌肉力量和肺功能的客观测量结果密切一致。ALSFRS可作为进入临床试验的筛查指标，在无法直接测量肌肉力量的情况下作为功能替代指标，或作为肌功能测试的辅助手段。
