Incidence and Prevalence of Amyotrophic Lateral Sclerosis in Canada: A Systematic Review of the Literature

Background: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disease of unknown etiology. Although known to be rare, precise information on the frequency of ALS is essential to anticipate future demands on health resources and as baseline information for epidemiological studies. As part of a new ALS epidemiological initiative in Canada, we conducted a systematic review of published incidence and prevalence research in Canada. Methods: Electronic searches and bibliographic reviews of pertinent publications were conducted. Results: We identified 6 published studies from 4 Canadian provinces conducted between 1974 and 2004; 2 were available only as abstracts. Reported annual incidence rates were similar and study quality was generally good, but there was insufficient detail to adequately assess the methodological quality of 3 of the studies. The most recent studies reported an annual ALS age-adjusted incidence of 2.13 per 100,000 in Nova Scotia (2003-2004) and a crude mean annual incidence of 2.4 per 100,000 in Newfoundland and Labrador (2000-2004). Conclusions: There are limited data on the frequency of ALS in Canada. We found no studies from 6 of the Canadian provinces or from the territories. Future research is needed to estimate the frequency of occurrence of ALS in Canada. Copyright (C) 2009 S. Karger AG, Basel