BackgroundThis update of the guideline on the management of amyotrophic lateral sclerosis (ALS) was commissioned by the European Academy of Neurology (EAN) and prepared in collaboration with the European Reference Network for Neuromuscular Diseases (ERN EURO‐NMD) and the support of the European Network for the Cure ALS (ENCALS) and the European Organization for Professionals and Patients with ALS (EUpALS).MethodsGrading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to assess the effectiveness of interventions for ALS. Two systematic reviewers from Cochrane Response supported the guideline panel. The working group identified a total of 26 research questions, performed systematic reviews, assessed the quality of the available evidence, and made specific recommendations. Expert consensus statements were provided where insufficient evidence was available.ResultsA guideline mapping effort revealed only one other ALS guideline that used GRADE methodology (a National Institute for Health and Care Excellence [NICE] guideline). The available evidence was scarce for many research questions. Of the 26 research questions evaluated, the NICE recommendations could be adapted for 8 questions. Other recommendations required updates of existing systematic reviews or de novo reviews. Recommendations were made on currently available disease‐modifying treatments, multidisciplinary care, nutritional and respiratory support, communication aids, psychological support, treatments for common ALS symptoms (e.g., muscle cramps, spasticity, pseudobulbar affect, thick mucus, sialorrhea, pain), and end‐of‐life management.ConclusionsThis update of the guideline using GRADE methodology provides a framework for the management of ALS. The treatment landscape is changing rapidly, and further updates will be prepared when additional evidence becomes available.

中文翻译：

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欧洲神经病学学会 (EAN) 与欧洲神经肌肉疾病参考网络 (ERNEURO-NMD) 合作制定肌萎缩侧索硬化症治疗指南

背景本次肌萎缩侧索硬化症 (ALS) 治疗指南更新是由欧洲神经病学研究院 (EAN) 委托，与欧洲神经肌肉疾病参考网络 (ERN EURO-NMD) 合作编写，并得到了欧洲神经肌肉疾病协会 (ERN EURO-NMD) 的支持。治愈 ALS 网络 (ENCALS) 和欧洲 ALS 专业人员和患者组织 (EUpALS)。方法使用建议评估、制定和评估分级 (GRADE) 方法来评估 ALS 干预措施的有效性。来自 Cochrane Response 的两名系统评审员为指南小组提供了支持。工作组共确定了 26 个研究问题，进行了系统回顾，评估了现有证据的质量，并提出了具体建议。在证据不足的情况下，提供了专家共识声明。结果指南绘制工作仅揭示了另一项使用 GRADE 方法的 ALS 指南（国家健康与护理卓越研究所 [NICE] 指南）。对于许多研究问题来说，现有的证据很少。在评估的 26 个研究问题中，NICE 建议可针对 8 个问题进行调整。其他建议需要更新现有的系统评价或从头评价。就目前可用的疾病缓解治疗、多学科护理、营养和呼吸支持、沟通辅助、心理支持、常见 ALS 症状（例如肌肉痉挛、痉挛、假性延髓影响、浓稠粘液、流涎、疼痛）的治疗以及结论 使用 GRADE 方法对指南进行的更新为 ALS 的管理提供了一个框架。治疗环境正在迅速变化，当有更多证据可用时，将准备进一步的更新。