A Programme for ALS Care in Europe (ALS-CarE)

肌萎缩侧索硬化症(ALS)是一种进行性神经退行性疾病，发病于壮年。目前还没有有效的疾病调节疗法来治疗ALS，死亡通常发生在症状出现后3年内。管理是治标不治本的，旨在最大限度地提高生活质量，最大限度地减少疾病负担。ALS的复杂性和快速发展的本质要求建立在可靠的疾病分期和基于证据的症状管理基础上的响应性多学科护理系统。ALS护理的目的是将从人群来源获得的详细临床信息纳入到响应性护理计划中。将验证标准化的分期系统，并将测量从诊断到生命末期的疾病阶段的生活质量和患者体验，并优化管理。卫生经济分析将确定资源利用方面的关键差异，并将有助于新疗法的药物经济学分析。完成的项目将为肌萎缩侧索硬化症提供一个用户友好的最佳实践框架，可以修改该框架以管理其他神经退行性疾病。

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that strikes in the prime of life. There are currently no effective disease modifying therapies for ALS and death usually occurs within 3 years of symptom onset. Management is palliative and is aimed at maximising quality of life and minimising the burden of disease. The complexity and rapidly progressive nature of ALS requires a responsive multidisciplinary care system that that is built on reliable disease staging and evidence based symptom management.The purpose of ALS-CarE is to incorporate detailed clinical information drawn from population based sources into a responsive care programme. A standardised staging system will be validated, and quality of life and patient experiences will be measured and management optimised across disease stages from diagnosis to end of life. Health economic analysis will identify key differences in resource utilisation and will be useful for pharmaco-economic analyses of new therapeutics. The completed project will provide a user-friendly best practice framework for ALS that can be modified for management of other neurodegenerative diseases.