A RATING-SCALE FOR AMYOTROPHIC-LATERAL-SCLEROSIS - DESCRIPTION AND PRELIMINARY EXPERIENCE
A RATING-SCALE FOR AMYOTROPHIC-LATERAL-SCLEROSIS - DESCRIPTION AND PRELIMINARY EXPERIENCE
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DOI：
10.1002/ana.410220308
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发表时间：
1987-09-01
影响因子：
11.2
通讯作者：
APPEL, SH
中科院分区：
文献类型：
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作者：
APPEL, V;STEWART, SS;APPEL, SH
已开发出一种评级量表，用于定量评估肌萎缩侧索硬化症 (ALS) 的临床状态和疾病进展。该量表包括吞咽、言语和呼吸功能以及上肢和下肢肌肉组织的力量和功能的评估。该评估执行起来相对简单，并且可以生成 ALS 总得分和每组测试功能的得分的可重复数据。 30分是正常的； 164分表示功能障碍最大。在随访至少一年的 74 名患者中，每一位患者的 ALS 总评分均呈线性增加。在患者中，疾病进展速度相差二十倍，从最慢到最快的病程连续分布。 34% 的患者在一年内表现出超过 48 点的快速变化，预测在不到两年的时间内进展到末期； 19% 的患者在一年内表现出小于 13 分的缓慢变化，预计至少需要五年时间才能进展到末期。该 ALS 评分系统应该能够更准确地评估临床试验中的药物疗效以及进展率与临床变量的相关性。
A rating scale has been developed to provide a quantitative estimate of clinical status and disease progression in amyotrophic lateral sclerosis (ALS). This scale includes assessment of swallowing, speech, and respiratory function, and both strength and function of upper and lower extremity musculature. The evaluation is relatively simple to perform and yields reproducible data for both a total ALS score and a score for each group of functions tested. A score of 30 points is normal; 164 points indicates maximal dysfunction. The total ALS score increased in a linear fashion in each of 74 patients followed for at least one year. Among patients, the rate of disease progression varied twenty-fold, with a continuous distribution from the slowest to most rapid course. Thirty-four percent of patients exhibited a rapid change of greater than 48 points in the year, predicting progression to a terminal stage in less than two years; 19% of patients exhibited a slow change of less than 13 points in one year, predicting progression to a terminal stage over at least five years. This ALS scoring system should permit more accurate assessment of drug efficacy in clinical trials and correlation of rates of progression with clinical variables.