Chiò Adriano, Canosa Antonio, Calvo Andrea, Moglia Cristina, Cicolin Alessandro, Mora Gabriele
'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.
Neurology I, Azienda Ospedaliera Città Della Salute E Della Scienza of Turin, Turin, Italy.
Expert Rev Neurother. 2021 Dec;21(12):1419-1440. doi: 10.1080/14737175.2021.1984883. Epub 2021 Oct 21.
The burden of non-motor symptoms is a major determinant of quality of life and outcome in amyotrophic lateral sclerosis (ALS) and has profound negative effect also on caregivers.
Non-motor symptoms in ALS include cognitive impairment, neurobehavioral symptoms, depression and anxiety, suicidal ideation, pain, disordered sleep, fatigue, weight loss and reduced appetite, and autonomic dysfunctions. This review summarizes the measures used for the assessment of non-motor symptoms and their properties and recaps the frequency and progression of these symptoms along the course of ALS.
Non-motor symptoms in ALS represent a major component of the disease and span over several domains. These symptoms require a high level of medical attention and should be checked at each visit using ad hoc questionnaires and proactively treated. Several instruments assessing non-motor symptoms have been used in ALS. Specific screening questionnaires for non-motor symptoms can be used for monitoring patients during telehealth visits and for remote surveillance through sensors and apps installed on smartphones. Novel trials for non-motor symptoms treatment specifically designed for ALS are necessary to increase and refine the therapeutic armamentarium. Finally, scales assessing the most frequent and burdensome non-motor symptoms should be included in clinical trials.
非运动症状的负担是肌萎缩侧索硬化症（ALS）患者生活质量和预后的主要决定因素，对护理人员也有深远的负面影响。
ALS的非运动症状包括认知障碍、神经行为症状、抑郁和焦虑、自杀意念、疼痛、睡眠障碍、疲劳、体重减轻和食欲减退以及自主神经功能障碍。本综述总结了用于评估非运动症状的方法及其特性，并概述了这些症状在ALS病程中的发生频率和进展情况。
ALS的非运动症状是该疾病的主要组成部分，涉及多个领域。这些症状需要高度的医疗关注，每次就诊时都应使用专门的问卷进行检查并积极治疗。已有多种评估ALS非运动症状的工具。针对非运动症状的特定筛查问卷可用于远程医疗就诊期间对患者的监测，以及通过安装在智能手机上的传感器和应用程序进行远程监测。有必要开展专门针对ALS的非运动症状治疗的新试验，以增加和完善治疗手段。最后，评估最常见和最具负担的非运动症状的量表应纳入临床试验。
