The Global Burden of Disease Study 2016 estimated the worldwide all-age prevalence of all motor neuron diseases to be 4.5 per 100,000 people, with an increase in age-standardised prevalence of 4.5% over the period 1990 to 2016. The estimated all-age incidence was 0.78 per 100,000 person-years.[8]GBD 2016 Motor Neuron Disease Collaborators. Global, regional, and national burden of motor neuron diseases 1990-2016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet Neurol. 2018 Dec;17(12):1083-97.
https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(18)30404-6/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/30409709?tool=bestpractice.com
[9]GBD 2016 Neurology Collaborators. Global, regional, and national burden of neurological disorders, 1990-2016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet Neurol. 2019 May;18(5):459-80.
https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(18)30499-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/30879893?tool=bestpractice.com
An earlier analysis of ALS incidence per 100,000 population reported values of 2.1 for Europe, 1.8 in the US, and 0.6 in Asia.[10]Chiò A, Logroscino G, Traynor BJ, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology. 2013;41(2):118-30.
https://www.karger.com/Article/FullText/351153
http://www.ncbi.nlm.nih.gov/pubmed/23860588?tool=bestpractice.com
The mean age of onset of ALS is about 62 years, with a peak incidence between 60 and 75 years.[10]Chiò A, Logroscino G, Traynor BJ, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology. 2013;41(2):118-30.
https://www.karger.com/Article/FullText/351153
http://www.ncbi.nlm.nih.gov/pubmed/23860588?tool=bestpractice.com
Limb-onset ALS occurs in about 70% of patients, and bulbar-onset disease in around 25% of patients.[11]Shoesmith C, Abrahao A, Benstead T, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020 Nov 16;192(46):E1453-68.
https://www.cmaj.ca/content/192/46/E1453
http://www.ncbi.nlm.nih.gov/pubmed/33199452?tool=bestpractice.com
The prevalence of ALS is consistently reported to be higher in men than in women, with a ratio of 1.25:1.[9]GBD 2016 Neurology Collaborators. Global, regional, and national burden of neurological disorders, 1990-2016: a systematic analysis for the Global Burden of Disease Study 2016. Lancet Neurol. 2019 May;18(5):459-80.
https://www.thelancet.com/journals/laneur/article/PIIS1474-4422(18)30499-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/30879893?tool=bestpractice.com
[12]Logroscino G, Traynor BJ, Hardiman O, et al; EURALS. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry. 2010 Apr;81(4):385-90.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2850819
http://www.ncbi.nlm.nih.gov/pubmed/19710046?tool=bestpractice.com
Some studies have reported that this difference disappears with age.[13]Worms PM. The epidemiology of motor neuron diseases: a review of recent studies. J Neurol Sci. 2001 Oct 15;191(1-2):3-9.
http://www.ncbi.nlm.nih.gov/pubmed/11676986?tool=bestpractice.com
There are no definite data regarding the distribution of ALS among people of different ethnicities, but a higher incidence in white people than in Asian, African, and Hispanic groups has been suggested.[10]Chiò A, Logroscino G, Traynor BJ, et al. Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology. 2013;41(2):118-30.
https://www.karger.com/Article/FullText/351153
http://www.ncbi.nlm.nih.gov/pubmed/23860588?tool=bestpractice.com
[14]Cronin S, Hardiman O, Traynor BJ. Ethnic variation in the incidence of ALS: a systematic review. Neurology. 2007 Mar 27;68(13):1002-7.
http://www.ncbi.nlm.nih.gov/pubmed/17389304?tool=bestpractice.com