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We calculated age\u2010 and sex\u2010specific incidence in 2009 according to multiple, progressively more stringent case definitions. Our most inclusive definition required one ALS code, whereas the most restrictive definition required at least one additional ALS code more than 6\u00a0months after the first code, including one from a neurologist. We identified associated imaging studies and electrodiagnostic testing and followed all cases through the end of 2014 to determine survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The overall incidence for our most inclusive definition was 22.84 per 100 000 person\u2010years for men and 16.05 per 100 000 person\u2010years for women. The overall incidence was 5.72 per 100 000 person\u2010years for men and 3.99 per 100 000 person\u2010years for women for our most restrictive definition. For our most inclusive definition, fewer than 39.7% of cases ever had an ALS diagnosis from a neurologist, more than 50% had an electrodiagnostic test or imaging study, and 40.1% survived less than 1 year after diagnosis, with 25.5% of these cases surviving no more than 6\u00a0months. Cases not meeting the most restrictive definition were more likely than those who did meet the restrictive definition to be older, black, or Asian.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The oldest and marginalized Medicare beneficiaries diagnosed with ALS are less likely to be included in epidemiological studies with restrictive definitions, but future studies will need to assess the accuracy of diagnosis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27652","type":"journal-article","created":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T07:30:45Z","timestamp":1654759845000},"page":"289-296","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Incidence of amyotrophic lateral sclerosis in older adults"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6314-8553","authenticated-orcid":false,"given":"Alejandra","family":"Camacho\u2010Soto","sequence":"first","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7768-4736","authenticated-orcid":false,"given":"Susan","family":"Searles Nielsen","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irene M.","family":"Faust","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C.","family":"Bucelli","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy M.","family":"Miller","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brad A.","family":"Racette","sequence":"additional","affiliation":[{"name":"Department of Neurology Washington University School of Medicine  St. Louis Missouri United States"},{"name":"School of Public Health, Faculty of Health Sciences University of the Witwatersrand  Johannesburg South Africa"},{"name":"Department of Neurology Barrow Neurological Institute  Phoenix Arizona"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,6,24]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2010.235952"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000365850"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2014-007359"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.3109\/21678421.2015.1019516"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24488"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.47.2.571"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1080\/14660820052415862"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10654-018-0392-x"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.59.2.280"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1991.00530180041015"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00391-020-01734-1"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(94)90191-0"},{"key":"e_1_2_9_14_1","unstructured":"University of Washington Rural Health Research Center.Rural\u2010urban commuting area (RUCA) zip code data version 2.0. 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