Validation of the DYALS (Dysphagia in Amyotrophic Lateral Sclerosis)

Dysphagia is a common symptom during the trajectory of ALS, and it can significantly impact on the quality of life and prognosis of patients. Nowadays, no specific tool for the screening of dysphagia in ALS is validated, and the approach is heterogeneous across the Italian Centres. To validate the DYALS (Dysphagia in Amyotrophic Lateral Sclerosis) questionnaire, adapting the DYMUS (Dysphagia in Multiple Sclerosis) questionnaire, for the assessment of dysphagia in ALS patients, in order to uniform the evaluations across the Italian ALS network. We included 197 patients diagnosed with ALS following the El Escorial criteria, in sixteen Italian ALS Centres between 1st December 2019 and 1st July 2020. For each patient, we collected clinical and demographic data, and obtained ALSFRS-r score, ALSAQ-5 score, DYMUS score, and EAT-10 score. Across the 197 patients, the ratio M/F was 113/84, and the median age was 64 years (IQR 56-72.5). Bulbar patients were 20%, and spinal patients 80%. The median ALSFRSr total score of patients was 35 (IQR 28-39). DYALS score was statistically higher in bulbar ALS than in spinal ALS (median=6, IQR 4.5-9 vs median=1, IQR 0-5, z =6.253, p&lt;0.0001). DYALS questionnaire showed a high internal consistency (Cronbach’s alpha=0.88). There was a statistically significant correlation between DYALS and EAT-10 (rho=0.90, p&lt;0.0001). DYALS scale is reliable, manageable, and easily usable for the screening of dysphagia in ALS. It can be shared with all the Italian ALS Centres in order to collect uniform data for therapeutic strategies and clinical trials.吞咽障碍（Dysphagia）是肌萎缩侧索硬化症（Amyotrophic Lateral Sclerosis, ALS）病程中常见的临床症状，可显著影响患者的生活质量与预后。目前尚无经过验证的ALS吞咽障碍专用筛查工具，且意大利各ALS诊疗中心的筛查方案存在显著异质性。为统一意大利ALS诊疗网络内的吞咽障碍评估标准，本研究以多发性硬化吞咽障碍（Dysphagia in Multiple Sclerosis, DYMUS）问卷为基础进行改编，开发并验证用于ALS患者吞咽障碍评估的DYALS（Dysphagia in Amyotrophic Lateral Sclerosis）问卷。研究于2019年12月1日至2020年7月1日期间，纳入意大利16家ALS诊疗中心的197例符合艾司库里尔（El Escorial）诊断标准的ALS患者。研究人员收集了所有患者的临床资料与人口学特征数据，并获取其肌萎缩侧索硬化功能评定量表-修订版（Amyotrophic Lateral Sclerosis Functional Rating Scale-revised, ALSFRS-r）评分、肌萎缩侧索硬化生活质量问卷-5项版（Amyotrophic Lateral Sclerosis Assessment Questionnaire-5, ALSAQ-5）评分、DYMUS评分与进食评估问卷-10项（Eating Assessment Tool-10, EAT-10）评分。197例入组患者中，男女性别比为113:84，年龄中位数为64岁（四分位数间距IQR：56~72.5）；其中球部起病患者占20%，脊髓起病患者占80%。患者的ALSFRS-r总分中位数为35（IQR：28~39）。球部起病ALS患者的DYALS评分显著高于脊髓起病患者（中位数：6，IQR：4.5~9 vs 中位数：1，IQR：0~5，z=6.253，p<0.0001）。DYALS问卷具有良好的内部一致性（克朗巴赫α系数=0.88）。DYALS评分与EAT-10评分存在显著统计学相关性（秩相关系数rho=0.90，p<0.0001）。综上，DYALS量表具备良好的信度、可操作性与易用性，可用于ALS患者的吞咽障碍筛查。该问卷可在意大利所有ALS诊疗中心推广使用，以收集统一的临床数据，为治疗策略制定与临床试验开展提供支持。