{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T14:58:26Z","timestamp":1790261906422,"version":"4.1.0"},"reference-count":29,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,11,19]],"date-time":"2021-11-19T00:00:00Z","timestamp":1637280000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003840","name":"Irish Institute of Clinical Neuroscience","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003840","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>This study evaluates the incidence, prevalence and survival trends of motor neurone disease (MND) in Northern Ireland from 2015 to 2019.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A capture\u2013recapture analysis was performed using five independent data sources. Incidence and prevalence rates were standardized to the European Standard Population. Survival outcomes were analysed using Kaplan\u2013Meier curves and Cox regression analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Amongst 254 total cases of MND, capture\u2013recapture analysis estimated three missing cases (case ascertainment 98.8%). Age standardized incidence of captured cases was 3.12 per 100,000 (2.73, 3.50) and standardized prevalence ranged from 9.45 to 6.49 per 100,000 from 2015 to 2019. Standardized incidence and prevalence rates in 2006 were 1.4 and 3.3 per 100,000 respectively. Of identified cases, 133 (52.4%) were male; 94.5% had amyotrophic lateral sclerosis; median age of onset was 67\u00a0years; median time to diagnosis was 12\u00a0months (95% confidence interval 11.2, 12.8); survival from diagnosis was 12\u00a0months (95% confidence interval 10.6, 15.4); 25 (9.8%) reported a family history of MND or frontotemporal dementia; and a known MND\u2010associated genetic mutation was identified in 7.9% of total cases, of which the most common was <jats:italic>C9orf72<\/jats:italic> (5.7% of all patients). Factors associated with improved survival were younger age at onset, longer time to diagnosis, attendance at regional MND clinic, and initial neurology presentation as outpatient (all <jats:italic>p<\/jats:italic>\u00a0&lt;\u00a00.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The incidence and prevalence of MND in Northern Ireland has increased over the last 10\u00a0years, in line with increasing rates reported from other European countries. Improved survival was associated with younger age at onset, longer time to diagnosis, attendance at a regional MND clinic and outpatient presentation to a Neurology Department.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15172","type":"journal-article","created":{"date-parts":[[2021,11,8]],"date-time":"2021-11-08T17:38:33Z","timestamp":1636393113000},"page":"707-714","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Epidemiology and survival trends of motor neurone disease in Northern Ireland from 2015 to 2019"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1008-598X","authenticated-orcid":false,"given":"Gavin","family":"McCluskey","sequence":"first","affiliation":[{"name":"Department of Neurology Royal Victoria Hospital Belfast UK"},{"name":"Northern Ireland Centre for Stratified Medicine Altnagelvin Hospital Campus Ulster University Derry 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