258104 20240112171744.0 10.1001/jamaneurol.2022.5128 doi pmid:36716024 pmid pmc:PMC9887528 pmc 2168-6149 ISSN 0003-9942 ISSN 0375-8540 ISSN 1538-3687 ISSN 2168-6157 ISSN 2330-9644 ISSN altmetric:141940202 altmetric DZNE-2023-00566 English 610 Logroscino, Giancarlo 0 Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries. Chicago, Ill. 2023 American Medical Association article DRIVER Output Types/Journal article DataCite Journal Article journal journal PUB:(DE-HGF)16 1692960806_28576 PUB:(DE-HGF) ARTICLE BibTeX JOURNAL_ARTICLE ORCID Journal Article 0 EndNote Diagnostic incidence data for syndromes associated with frontotemporal lobar degeneration (FTLD) in multinational studies are urgent in light of upcoming therapeutic approaches.To assess the incidence of FTLD across Europe.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 11 023 643 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.Random-intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.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 100 000 person-years (95% CI, 1.59-3.51 cases per 100 000 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 100 000 person-years; 95% CI, 1.88-4.27 cases per 100 000 person-years) than among women (1.91 cases per 100 000 person-years; 95% CI, 1.26-2.91 cases per 100 000 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 12 057.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. 353 - Clinical and Health Care Research (POF4-353) G:(DE-HGF)POF4-353 POF4-353 POF IV 0 Dataset connected to CrossRef, PubMed, , Journals: pub.dzne.de Male MeSH Humans MeSH Female MeSH Aged MeSH Frontotemporal Dementia: epidemiology MeSH Incidence MeSH Amyotrophic Lateral Sclerosis MeSH Retrospective Studies MeSH Frontotemporal Lobar Degeneration: epidemiology MeSH Syndrome MeSH Europe: epidemiology MeSH Piccininni, Marco 1 Graff, Caroline 2 Hardiman, Orla 3 Ludolph, Albert C P:(DE-2719)2812633 4 dzne Moreno, Fermin 5 Otto, Markus 6 Remes, Anne M 7 Rowe, James B 8 Seelaar, Harro 9 Solje, Eino 10 Stefanova, Elka 11 Traykov, Latchezar 12 Jelic, Vesna 13 Rydell, Melissa Taheri 14 Pender, Niall 15 Anderl-Straub, Sarah 16 Barandiaran, Myriam 17 Gabilondo, Alazne 18 Krüger, Johanna 19 Murley, Alexander G 20 Rittman, Timothy 21 van der Ende, Emma L 22 van Swieten, John C 23 Hartikainen, Päivi 24 Stojmenovic, Gorana Mandic 25 Mehrabian, Shima 26 Benussi, Luisa 27 Alberici, Antonella 28 Dell'Abate, Maria Teresa 29 Zecca, Chiara 30 Borroni, Barbara 31 group, FRONTIERS 32 Collaboration Author Belezhanska, Diyana 33 Contributor Bianchetti, Angelo 34 Contributor Binetti, Giuliano 35 Contributor Cotelli, Maria 36 Contributor Cotelli, Maria Sofia 37 Contributor Dreharova, Irena 38 Contributor Filardi, Marco 39 Contributor Fostinelli, Silvia 40 Contributor Ghidoni, Roberta 41 Contributor Gnoni, Valentina 42 Contributor Nacheva, Genoveva 43 Contributor Novaković, Ivana 44 Contributor Padovani, Alessandro 45 Contributor Popivanov, Ivo 46 Contributor Raycheva, Margarita 47 Contributor Stockton, Katherine 48 Contributor Stoyanova, Katya 49 Contributor Suhonen, Noora-Maria 50 Contributor Tainta, Mikel 51 Contributor Toncheva, Draga 52 Contributor Urso, Daniele 53 Contributor Zlatareva, Dora 54 Contributor Zulaica, Miren 55 Contributor 10.1001/jamaneurol.2022.5128 Vol. 80, no. 3, p. 279 - PERI:(DE-600)2701924-X 3 279 - 286 JAMA neurology 80 2023 2168-6149 https://jamanetwork.com/journals/jamaneurology/fullarticle/2800415 http://pub.dzne.de/record/258104/files/DZNE-2023-00566.pdf OpenAccess http://pub.dzne.de/record/258104/files/DZNE-2023-00566.pdf?subformat=pdfa pdfa OpenAccess oai:pub.dzne.de:258104 openaire open_access VDB driver dnbdelivery Deutsches Zentrum für Neurodegenerative Erkrankungen I:(DE-588)1065079516 DZNE 4 P:(DE-2719)2812633 DE-HGF Gesundheit Neurodegenerative Diseases G:(DE-HGF)POF4-350 G:(DE-HGF)POF4-353 G:(DE-HGF)POF4 G:(DE-HGF)POF4-300 G:(DE-HGF)POF Clinical and Health Care Research 0 2023 OpenAccess StatID:(DE-HGF)0510 StatID Creative Commons Attribution-NonCommercial-NoDerivs CC BY-NC-ND 4.0 LIC:(DE-HGF)CCBYNCND4 HGFVOC DBCoverage StatID:(DE-HGF)0200 StatID SCOPUS 2023-10-26 DBCoverage StatID:(DE-HGF)0300 StatID Medline 2023-10-26 DBCoverage StatID:(DE-HGF)0199 StatID Clarivate Analytics Master Journal List 2023-10-26 DBCoverage StatID:(DE-HGF)1050 StatID BIOSIS Previews 2023-10-26 DBCoverage StatID:(DE-HGF)0150 StatID Web of Science Core Collection 2023-10-26 DBCoverage StatID:(DE-HGF)1030 StatID Current Contents - Life Sciences 2023-10-26 DBCoverage StatID:(DE-HGF)1110 StatID Current Contents - Clinical Medicine 2023-10-26 JCR StatID:(DE-HGF)0100 StatID JAMA NEUROL : 2022 2023-10-26 IF >= 25 StatID:(DE-HGF)9925 StatID JAMA NEUROL : 2022 2023-10-26 I:(DE-2719)5000077 Clinical Study Center Ulm Clinical Study Center Ulm 0 journal VDB I:(DE-2719)5000077 UNRESTRICTED FullTexts