Aerodigestive Research Core Laboratory, University of Florida, Gainesville, Florida, USA.
Department of Speech, Language, and Hearing Sciences, University of Florida, Gainesville, Florida, USA.
Muscle Nerve. 2023 Sep;68(3):296-302. doi: 10.1002/mus.27923. Epub 2023 Jun 21.
INTRODUCTION/AIMS: Given the widespread use of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) to measure disease progression in ALS and recent reports demonstrating its poor sensitivity, we aimed to determine the sensitivity and specificity of the ALSFRS-R bulbar subscale and speech item to detect validated clinical ratings of dysarthria in individuals with ALS.
Paired ALSFRS-R and validated Speech Intelligibility Test (SIT) data from individuals with ALS were analyzed. Trained raters completed duplicate, independent, and blinded ratings of audio recordings to obtain speech intelligibility (%) and speaking rate (words per minute, WPM). Binary dysarthria profiles were derived (dysarthria ≤96% intelligible and/or <150 WPM). Data were obtained using the Kruskal-Wallis test, receiver-operating characteristic (ROC) curve, area under the curve (AUC), sensitivity and specificity percentages, and positive/negative predictive values (PPV/NPV).
A total of 250 paired SIT and ALSFRS-R data points were analyzed. Dysarthria was confirmed in 72.4% (n = 181). Dysarthric speakers demonstrated lower ALSFRS-R bulbar subscale (8.9 vs. 11.2) and speech item (2.7 vs. 3.7) scores (P < .0001). The ALSFRS-R bulbar subscale score had an AUC of 0.81 (95% confidence interval [CI] 0.75 to 0.86). A subscale score of ≤11 yielded a sensitivity of 86%, specificity of 57%, PPV of 84%, and NPV of 60% to correctly identify dysarthria status. The ALSFRS-R speech item score demonstrated an AUC of 0.81 to detect dysarthria (95% CI 0.76 to 0.85), with sensitivity of 79%, specificity of 75%, PPV of 89%, and NPV of 58% for a speech item cutpoint of ≤3.
The ALSFRS-R bulbar and speech item subscale scores may be useful, inexpensive, and quick tools for monitoring dysarthria status in ALS.
介绍/目的：鉴于肌萎缩侧索硬化功能评定量表修订版（ALSFRS-R）被广泛用于测量肌萎缩侧索硬化症的疾病进展，以及最近有报道表明其敏感性较差，我们旨在确定 ALSFRS-R 球部亚量表和言语项目的敏感性和特异性，以检测肌萎缩侧索硬化症患者经验证的言语障碍临床评分。
对肌萎缩侧索硬化症患者的 ALSFRS-R 和经过验证的言语可懂度测试（SIT）数据进行了分析。经过培训的评估员完成了音频记录的重复、独立和盲目的评估，以获得言语可懂度（%）和说话速度（每分钟单词数，WPM）。得出了二元构音障碍谱（构音障碍≤96%可懂度和/或<150 WPM）。使用 Kruskal-Wallis 检验、受试者工作特征（ROC）曲线、曲线下面积（AUC）、敏感性和特异性百分比以及阳性/阴性预测值（PPV/NPV）来获得数据。
共分析了 250 对 SIT 和 ALSFRS-R 数据点。72.4%（n=181）确认存在构音障碍。构音障碍患者的 ALSFRS-R 球部亚量表（8.9 分比 11.2 分）和言语项目（2.7 分比 3.7 分）评分较低（P<.0001）。ALSFRS-R 球部亚量表评分的 AUC 为 0.81（95%置信区间 [CI] 0.75 至 0.86）。亚量表评分≤11 可使辨别的敏感性为 86%，特异性为 57%，阳性预测值为 84%，阴性预测值为 60%，以正确识别构音障碍状态。ALSFRS-R 言语项目评分的 AUC 为 0.81，用于检测构音障碍（95%CI 0.76 至 0.85），言语项目切点≤3 时，敏感性为 79%，特异性为 75%，阳性预测值为 89%，阴性预测值为 58%。
ALSFRS-R 球部和言语项目亚量表评分可能是监测肌萎缩侧索硬化症构音障碍状态的有用、廉价和快速工具。
