{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T23:59:17Z","timestamp":1793318357728,"version":"4.5.0"},"reference-count":37,"publisher":"American Medical Association (AMA)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Neurol"],"published-print":{"date-parts":[[2023,3,1]]},"abstract":"<jats:sec id=\"ab-noi220091-4\"><jats:title>Importance<\/jats:title><jats:p>Diagnostic incidence data for syndromes associated with frontotemporal lobar degeneration (FTLD) in multinational studies are urgent in light of upcoming therapeutic approaches.<\/jats:p><\/jats:sec><jats:sec id=\"ab-noi220091-5\"><jats:title>Objective<\/jats:title><jats:p>To assess the incidence of FTLD across Europe.<\/jats:p><\/jats:sec><jats:sec id=\"ab-noi220091-6\"><jats:title>Design, Setting, and Participants<\/jats:title><jats:p>The Frontotemporal Dementia Incidence European Research Study (FRONTIERS) was a retrospective cohort study conducted from June 1, 2018, to May 31, 2019, using a population-based registry from 13 tertiary FTLD research clinics from the UK, the Netherlands, Finland, Sweden, Spain, Bulgaria, Serbia, Germany, and Italy and including all new FTLD-associated cases during the study period, with a combined catchment population of 11\u202f023\u202f643 person-years. Included patients fulfilled criteria for the behavioral variant of frontotemporal dementia (BVFTD), the nonfluent variant or semantic variant of primary progressive aphasia (PPA), unspecified PPA, progressive supranuclear palsy, corticobasal syndrome, or frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS). Data were analyzed from July 19 to December 7, 2021.<\/jats:p><\/jats:sec><jats:sec id=\"ab-noi220091-7\"><jats:title>Main Outcomes and Measures<\/jats:title><jats:p>Random-intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.<\/jats:p><\/jats:sec><jats:sec id=\"ab-noi220091-8\"><jats:title>Results<\/jats:title><jats:p>Based on 267 identified cases (mean [SD] patient age, 66.70 [9.02] years; 156 males [58.43%]), the estimated annual incidence rate for FTLD in Europe was 2.36 cases per 100\u202f000 person-years (95% CI, 1.59-3.51 cases per 100\u202f000 person-years). There was a progressive increase in FTLD incidence across age, reaching its peak at the age of 71 years, with 13.09 cases per 100 000 person-years (95% CI, 8.46-18.93 cases per 100 000 person-years) among men and 7.88 cases per 100 000 person-years (95% CI, 5.39-11.60 cases per 100 000 person-years) among women. Overall, the incidence was higher among men (2.84 cases per 100\u202f000 person-years; 95% CI, 1.88-4.27 cases per 100\u202f000 person-years) than among women (1.91 cases per 100\u202f000 person-years; 95% CI, 1.26-2.91 cases per 100\u202f000 person-years). BVFTD was the most common phenotype (107 cases [40.07%]), followed by PPA (76 [28.46%]) and extrapyramidal phenotypes (69 [25.84%]). FTD-ALS was the rarest phenotype (15 cases [5.62%]). A total of 95 patients with FTLD (35.58%) had a family history of dementia. The estimated number of new FTLD cases per year in Europe was 12\u202f057.<\/jats:p><\/jats:sec><jats:sec id=\"ab-noi220091-9\"><jats:title>Conclusions and Relevance<\/jats:title><jats:p>The findings suggest that FTLD-associated syndromes are more common than previously recognized, and diagnosis should be considered at any age. Improved knowledge of FTLD incidence may contribute to appropriate health and social care planning and in the design of future clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamaneurol.2022.5128","type":"journal-article","created":{"date-parts":[[2023,1,30]],"date-time":"2023-01-30T11:34:46Z","timestamp":1675078486000},"page":"279","source":"Crossref","is-referenced-by-count":85,"title":["Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries"],"prefix":"10.1001","volume":"80","author":[{"given":"Giancarlo","family":"Logroscino","sequence":"first","affiliation":[{"name":"Center for Neurodegenerative Diseases and the Aging Brain, University of Bari-Aldo Moro, Bari at Pia Fondazione Cardinale Giovanni Panico, Tricase, Lecce, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Piccininni","sequence":"additional","affiliation":[{"name":"Institute of Public Health, 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