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.吞咽困难是肌萎缩侧索硬化症（Amyotrophic Lateral Sclerosis, ALS）病程中常见的症状，可显著影响患者的生活质量与预后。当前尚无经过验证的ALS吞咽障碍筛查专用工具，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流程，本研究改编多发性硬化吞咽困难（DYMUS, Dysphagia in Multiple Sclerosis）问卷，开发并验证用于ALS患者吞咽障碍评估的DYALS（肌萎缩侧索硬化吞咽困难，Dysphagia in Amyotrophic Lateral Sclerosis）问卷。 本研究于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例符合埃斯科里亚尔标准（El Escorial criteria）确诊的ALS患者。收集所有患者的临床与人口学资料，记录其ALSFRS-r（肌萎缩侧索硬化功能评定量表-修订版）评分、ALSAQ-5（肌萎缩侧索硬化生活质量问卷-5项版）评分、DYMUS评分与EAT-10（进食评估问卷-10项）评分。 197例患者中，男女比例为113:84，中位年龄为64岁（四分位数间距Interquartile Range, IQR：56~72.5）；延髓起病患者占比20%，脊髓起病患者占比80%。患者的ALSFRS-r总评分中位值为35（IQR：28~39）。 延髓起病ALS患者的DYALS评分显著高于脊髓起病患者（中位值6，IQR：4.5~9 vs 中位值1，IQR：0~5，z=6.253，P<0.0001）。DYALS问卷具有较高的内部一致性（克朗巴赫α系数Cronbach’s alpha=0.88），且与EAT-10评分呈显著正相关（rho=0.90，P<0.0001）。 本研究开发的DYALS量表具备良好的信度、可操作性与易用性，可用于ALS患者吞咽障碍的筛查。该问卷可在意大利所有ALS诊疗中心推广应用，为治疗策略制定与临床试验收集统一标准化的数据。