Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College, Dublin, Ireland.
Department of Neurology, Beaumont Hospital, Beaumont, Ireland.
J Neurol Neurosurg Psychiatry. 2017 Jul;88(7):557-563. doi: 10.1136/jnnp-2016-314495. Epub 2017 Mar 11.
Prospective population based-registers of amyotrophic lateral sclerosis (ALS) have operated in Europe for over two decades, and have provided important insights into our understanding of ALS. Here, we review the benefits that population registers have brought to the understanding of the incidence, prevalence, phenotype and genetics of ALS and outline the core operating principles that underlie these registers and facilitate international collaboration. Going forward, we offer lessons learned from our collective experience of operating population-based ALS registers in Europe for over two decades, focusing on register design, maintenance, identification and management of bias and the value of cross-national harmonisation and integration.
前瞻性基于人群的肌萎缩侧索硬化症（ALS）登记处已经在欧洲运行了二十多年，为我们理解 ALS 提供了重要的见解。在这里，我们回顾了人群登记处对理解 ALS 的发病率、患病率、表型和遗传学所带来的益处，并概述了这些登记处的核心运作原则，这些原则促进了国际合作。展望未来，我们将借鉴欧洲二十多年来运营基于人群的 ALS 登记处的集体经验，重点介绍登记处的设计、维护、偏倚的识别和管理，以及跨国协调和整合的价值。
