Cedarbaum J M, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A
Regeneron Pharmaceuticals, Inc., 777 Old Saw Mill River Rd., Tarrytown, NY 10591, USA.
J Neurol Sci. 1999 Oct 31;169(1-2):13-21. doi: 10.1016/s0022-510x(99)00210-5.
The ALS Functional Rating Scale (ALSFRS) is a validated rating instrument for monitoring the progression of disability in patients with amyotrophic lateral sclerosis (ALS). One weakness of the ALSFRS as originally designed was that it granted disproportionate weighting to limb and bulbar, as compared to respiratory, dysfunction. We have now validated a revised version of the ALSFRS, which incorporates additional assessments of dyspnea, orthopnea, and the need for ventilatory support. The Revised ALSFRS (ALSFRS-R) retains the properties of the original scale and shows strong internal consistency and construct validity. ALSFRS-R scores correlate significantly with quality of life as measured by the Sickness Impact Profile, indicating that the quality of function is a strong determinant of quality of life in ALS.
肌萎缩侧索硬化功能评定量表（ALSFRS）是一种经过验证的评定工具，用于监测肌萎缩侧索硬化（ALS）患者的残疾进展情况。最初设计的ALSFRS的一个缺点是，与呼吸功能障碍相比，它对肢体和延髓功能障碍给予了不成比例的权重。我们现已验证了ALSFRS的修订版，其中纳入了对呼吸困难、端坐呼吸和通气支持需求的额外评估。修订后的ALSFRS（ALSFRS-R）保留了原量表的特性，并显示出很强的内部一致性和结构效度。ALSFRS-R评分与疾病影响量表所衡量的生活质量显著相关，表明功能质量是ALS患者生活质量的一个重要决定因素。
