Global Epidemiology of Amyotrophic Lateral Sclerosis: A Systematic Review of the Published Literature
Open accessAbstract
Background: Amyotrophic lateral sclerosis (ALS) is relatively rare, yet the economic and social burden is substantial. Having accurate incidence and prevalence estimates would facilitate efficient allocation of healthcare resources. Objective: To provide a comprehensive and critical review of the epidemiological literature on ALS. Methods: MEDLINE and EMBASE (1995-2011) databases of population-based studies on ALS incidence and prevalence reporting quantitative data were analyzed. Data extracted included study location and time, design and data sources, case ascertainment methods and incidence and/or prevalence rates. Medians and interquartile ranges (IQRs) were calculated, and ALS case estimates were derived using 2010 population estimates. Results: In all, 37 articles met the inclusion criteria. In Europe, the median incidence rate (/100,000 population) was 2.08 (IQR 1.47-2.43), corresponding to an estimated 15,355 (10,852-17,938) cases. Median prevalence (/100,000 population) was 5.40 (IQR 4.06-7.89), or 39,863 (29,971-58,244) prevalent cases. Conclusions: Disparity in rates among ALS incidence and prevalence studies may be due to differences in study design or true variations in population demographics such as age and geography, including environmental factors and genetic predisposition. Additional large-scale studies that use standardized case ascertainment methods are needed to more accurately assess the true global burden of ALS.
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“6 cases per 100,000 people every year [19], and is particularly high in Europe, with 15,000 new cases per year [20], [21].”
Background
- Authors
- Chiò, A., Logroscino, G., Traynor, B. J., Collins, J., Simeone, J. C., Goldstein, L. A., & White, L. A.
- Affiliations
- University of Turin (IT)
- University of Bari Aldo Moro (IT)
- National Institutes of Health (US)
- Biogen (United States) (US)
- Venue
- Neuroepidemiology
- Publisher
- S. Karger AG
- Published
- 2013
- Volume
- 41
- Issue
- 2
- Pages
- 118-130
- ISSN
- 0251-5350 (ISSN-L)
- 1423-0208
- PMC
- PMC4049265
- OpenAlex
- W2072204165
- Semantic Scholar
- 1b0c210d33ed778a844e814d1250568995f5e3a8
- Cited by
- 862
- References
- 78
- Citations by year
- 2026: 49 · 2025: 52 · 2024: 64 · 2023: 85 · 2022: 80 · 2021: 97 · 2020: 89 · 2019: 61 · 2018: 79 · 2017: 64 · 2016: 64 · 2015: 42 · 2014: 28 · 2013: 6 · 2012: 1
- FWCI
- 6.08
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Neuroepidemiology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- Funders
- Biogen
- National Institutes of Health
- National Institute on Aging (ZIAAG000933)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
- Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
- Keywords
- amyotrophic lateral sclerosis, incidence, prevalence, global burden of disease, case ascertainment, population-based study, rare diseases, healthcare resource allocation
- MeSH terms
- Amyotrophic Lateral Sclerosis, Humans, Global Health
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
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