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000258104 0247_ $$2doi$$a10.1001/jamaneurol.2022.5128
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000258104 1001_ $$aLogroscino, Giancarlo$$b0
000258104 245__ $$aIncidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries.
000258104 260__ $$aChicago, Ill.$$bAmerican Medical Association$$c2023
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000258104 520__ $$aDiagnostic 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.
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000258104 650_2 $$2MeSH$$aMale
000258104 650_2 $$2MeSH$$aHumans
000258104 650_2 $$2MeSH$$aFemale
000258104 650_2 $$2MeSH$$aAged
000258104 650_2 $$2MeSH$$aFrontotemporal Dementia: epidemiology
000258104 650_2 $$2MeSH$$aIncidence
000258104 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis
000258104 650_2 $$2MeSH$$aRetrospective Studies
000258104 650_2 $$2MeSH$$aFrontotemporal Lobar Degeneration: epidemiology
000258104 650_2 $$2MeSH$$aSyndrome
000258104 650_2 $$2MeSH$$aEurope: epidemiology
000258104 7001_ $$aPiccininni, Marco$$b1
000258104 7001_ $$aGraff, Caroline$$b2
000258104 7001_ $$aHardiman, Orla$$b3
000258104 7001_ $$0P:(DE-2719)2812633$$aLudolph, Albert C$$b4$$udzne
000258104 7001_ $$aMoreno, Fermin$$b5
000258104 7001_ $$aOtto, Markus$$b6
000258104 7001_ $$aRemes, Anne M$$b7
000258104 7001_ $$aRowe, James B$$b8
000258104 7001_ $$aSeelaar, Harro$$b9
000258104 7001_ $$aSolje, Eino$$b10
000258104 7001_ $$aStefanova, Elka$$b11
000258104 7001_ $$aTraykov, Latchezar$$b12
000258104 7001_ $$aJelic, Vesna$$b13
000258104 7001_ $$aRydell, Melissa Taheri$$b14
000258104 7001_ $$aPender, Niall$$b15
000258104 7001_ $$aAnderl-Straub, Sarah$$b16
000258104 7001_ $$aBarandiaran, Myriam$$b17
000258104 7001_ $$aGabilondo, Alazne$$b18
000258104 7001_ $$aKrüger, Johanna$$b19
000258104 7001_ $$aMurley, Alexander G$$b20
000258104 7001_ $$aRittman, Timothy$$b21
000258104 7001_ $$avan der Ende, Emma L$$b22
000258104 7001_ $$avan Swieten, John C$$b23
000258104 7001_ $$aHartikainen, Päivi$$b24
000258104 7001_ $$aStojmenovic, Gorana Mandic$$b25
000258104 7001_ $$aMehrabian, Shima$$b26
000258104 7001_ $$aBenussi, Luisa$$b27
000258104 7001_ $$aAlberici, Antonella$$b28
000258104 7001_ $$aDell'Abate, Maria Teresa$$b29
000258104 7001_ $$aZecca, Chiara$$b30
000258104 7001_ $$aBorroni, Barbara$$b31
000258104 7001_ $$agroup, FRONTIERS$$b32$$eCollaboration Author
000258104 7001_ $$aBelezhanska, Diyana$$b33$$eContributor
000258104 7001_ $$aBianchetti, Angelo$$b34$$eContributor
000258104 7001_ $$aBinetti, Giuliano$$b35$$eContributor
000258104 7001_ $$aCotelli, Maria$$b36$$eContributor
000258104 7001_ $$aCotelli, Maria Sofia$$b37$$eContributor
000258104 7001_ $$aDreharova, Irena$$b38$$eContributor
000258104 7001_ $$aFilardi, Marco$$b39$$eContributor
000258104 7001_ $$aFostinelli, Silvia$$b40$$eContributor
000258104 7001_ $$aGhidoni, Roberta$$b41$$eContributor
000258104 7001_ $$aGnoni, Valentina$$b42$$eContributor
000258104 7001_ $$aNacheva, Genoveva$$b43$$eContributor
000258104 7001_ $$aNovaković, Ivana$$b44$$eContributor
000258104 7001_ $$aPadovani, Alessandro$$b45$$eContributor
000258104 7001_ $$aPopivanov, Ivo$$b46$$eContributor
000258104 7001_ $$aRaycheva, Margarita$$b47$$eContributor
000258104 7001_ $$aStockton, Katherine$$b48$$eContributor
000258104 7001_ $$aStoyanova, Katya$$b49$$eContributor
000258104 7001_ $$aSuhonen, Noora-Maria$$b50$$eContributor
000258104 7001_ $$aTainta, Mikel$$b51$$eContributor
000258104 7001_ $$aToncheva, Draga$$b52$$eContributor
000258104 7001_ $$aUrso, Daniele$$b53$$eContributor
000258104 7001_ $$aZlatareva, Dora$$b54$$eContributor
000258104 7001_ $$aZulaica, Miren$$b55$$eContributor
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