Epidemiological Surveillance of Amyotrophic Lateral Sclerosis: A Review

Background: Registries and clinical databases are important tools to systematically record and collect information about individuals with rare diseases and to monitor disease patterns in populations. Through a review of the published literature on strategies used for surveillance of Amyotrophic Lateral Sclerosis (ALS), our objective was to better delineate the varied approaches used to monitor ALS at a population level. Further, we sought to determine the potential of registries to enhance knowledge on the epidemiology of ALS using a case study comparing epidemiological outputs from registries in the United States, United Kingdom, and Italy. Summary: We searched Medline, Embase, Global Health, PsycInfo, Cochrane Library, and CINAHL identifying articles published between January 1st, 2010, and May 12th, 2021. Studies describing population registries, cohorts of individuals with ALS, or large-scale studies aimed at systematically identifying people with ALS, were eligible for inclusion. 1,447 publications were found, of which 141 were selected for full text review, and 41 of those were selected for data extraction. We identified ALS registries and pertinent databases in 4 continents (North America, Europe, Asia, and Oceania). Stated objectives of the registries/databases shaped their framework, methodology, and follow-up. The US National Registry demonstrates substantial research outputs and methodological strengths, producing many descriptive epidemiological outputs (n=5 studies) and several methodological papers (n=12 studies). The UK and Italy overall each produced a similar number of studies (albeit with fewer methodological papers), across several different registries and regions. Key Messages: Due to challenges inherent to the surveillance of rare diseases, registries are a vital tool in determining and assessing the global impact of ALS. Nevertheless, the development and implementation of registries is not feasible everywhere in the world. There are advantages and drawbacks to structuring registries at a national or regional level, often dictated by funding availability, resources and health care infrastructure, and research objectives. To fully assess the epidemiological burden of ALS globally, collaborative initiatives are needed to fill gaps in knowledge, and there is a critical need to harmonize and optimize the development, collection, and sharing of data across registries.