Objective: To assess the utility of novel measures derived from a rapid syllable repetition task (i.e. oral dysdiadochokinesis [DDK]) in early stratification of fast and slow progressive bulbar amyotrophic lateral sclerosis (ALS) and prediction of bulbar disease progression rate. Methods: Fifty-four individuals with ALS were tracked longitudinally on their oral DDK and global bulbar/speech performance (i.e. bulbar subscore on the ALS Functional Rating Scale-Revised [ALSFRS-R]; articulation rate during passage reading) for a four-month average duration. Based on the bulbar deterioration rate over the tracked period, the participants were stratified as 14 fast bulbar progressors and 40 slow bulbar progressors using a posteriori classification approach. To determine if oral DDK performance predicts the differential bulbar disease progression trajectories in these individuals during the early stages of the tracked period (prior to significant bulbar/speech signs), twenty-two measures of lip motor performance in an oral DDK task were derived to (1) differentiate fast and slow bulbar progressors using the Receiver Operating Characteristic analysis and (2) predict bulbar disease progression rates across all individuals using linear regressions. Results: Movement jitter, a measure of temporal variability of alternating lip movement during DDK, showed 80% sensitivity and 95% specificity in differentiating fast and slow bulbar progressors early in the disease, and outperformed the ALSFRS-R bulbar subscore and articulation rate. Movement jitter also predicted bulbar disease progression rates across participants. Conclusion: Findings provided preliminary validation of the clinical value of movement jitter during oral DDK in patient stratification and bulbar disease prognosis.

中文翻译：

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延髓肌萎缩性侧索硬化的快进和慢进的早期分层的言语测量：唇部运动抖动。

目的：评估源自快速音节重复任务的新措施（即口服dysdiadochokinesis [DDK]）在快，慢进行性延髓肌萎缩性侧索硬化症（ALS）的早期分层以及预测延髓疾病进展率中的实用性。方法：对54名ALS患者进行了纵向跟踪，追踪了他们的口服DDK和总体延髓/语音表现（即经ALS功能评定量表修订的延髓子评分[ALSFRS-R]；通过阅读时的发音率），持续了四个月平均持续时间。根据所跟踪时期的延髓退化率，采用后验分类方法将参与者分为14个快速延髓进展者和40个缓慢延髓进展者。为了确定口服DDK的表现是否可以预测这些个体在所追踪的早期阶段（明显的球形/语音迹象之前）的差异性延髓疾病进展轨迹，推导了22种口服DDK任务中唇运动表现的测量指标， （1）使用接收器操作特征分析区分快速和缓慢的延髓进展者，（2）使用线性回归预测所有个体的延髓疾病进展率。结果：运动抖动是DDK期间嘴唇交替运动随时间变化的一种量度，在区分疾病早期的快，慢延髓进展过程中显示80％的敏感性和95％的特异性，优于ALSFRS-R延髓子评分和关节运动率。运动抖动还可以预测参与者的延髓疾病进展速度。结论：这些发现为口服DDK期间运动分层对患者分层和延髓疾病预后的临床价值提供了初步的验证。