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. 2023 Mar 1;80(3):279-286.
doi: 10.1001/jamaneurol.2022.5128.

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries

Giancarlo Logroscino  1 , Marco Piccininni  2   3 , Caroline Graff  4   5 , Orla Hardiman  6   7 , Albert C Ludolph  8   9 , Fermin Moreno  10   11 , Markus Otto  8   12 , Anne M Remes  13   14   15 , James B Rowe  16 , Harro Seelaar  17 , Eino Solje  18   19 , Elka Stefanova  20 , Latchezar Traykov  21 , Vesna Jelic  22   23 , Melissa Taheri Rydell  4 , Niall Pender  6   7 , Sarah Anderl-Straub  8 , Myriam Barandiaran  10   11 , Alazne Gabilondo  10   11 , Johanna Krüger  13   14   24 , Alexander G Murley  16 , Timothy Rittman  16 , Emma L van der Ende  17 , John C van Swieten  17 , Päivi Hartikainen  19 , Gorana Mandic Stojmenovic  20 , Shima Mehrabian  21 , Luisa Benussi  25 , Antonella Alberici  26 , Maria Teresa Dell'Abate  1 , Chiara Zecca  1 , Barbara Borroni  26 ; FRONTIERS group
Collaborators, Affiliations

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries

Giancarlo Logroscino et al. JAMA Neurol. .

Abstract

Importance: Diagnostic incidence data for syndromes associated with frontotemporal lobar degeneration (FTLD) in multinational studies are urgent in light of upcoming therapeutic approaches.

Objective: To assess the incidence of FTLD across Europe.

Design, setting, and participants: 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.

Main outcomes and measures: Random-intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.

Results: 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.

Conclusions and relevance: 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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Conflict of interest statement

Conflict of Interest Disclosures: Dr Logroscino reported receiving grants from Regione Puglia during the conduct of the study. Dr Piccininni reported receiving funding from Novartis Pharma and a research grant from the Center for Stroke Research Berlin (private donations) and being an associate editor for Frontiers in Epidemiology. Dr Hardiman reported receiving personal fees for serving as editor of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration outside the submitted work. Dr Moreno reported receiving grants from the Instituto de Salud Carlos III and the Tau Consortium outside the submitted work. Dr Otto reported receiving grants from the German Federal Ministry of Education and Research (BMBF)–German Consortium for Frontotemporal Lobar Degeneration and BMBF–Genfi-Prox during the conduct of the study. Dr Remes reported receiving grants from the Academy of Finland during the conduct of the study. Dr Rowe reported receiving grants from the National Institute for Health Research (NIHR), the Medical Research Council, and the Cambridge Centre for Parkinson-Plus during the conduct of the study; grants from Wellcome Trust and Dementias Platform UK (with indirect funding from Janssen, GSK, Lilly, and AstraZeneca) outside the submitted work; and personal fees from Asceneuron, Astex, SV Health, CuraSen, and UCB Pharma Ltd outside the submitted work. Dr Stefanova reported receiving personal fees from Roche for lecturing and conference fees from Teva Actavis. Dr Pender reported receiving personal fees (speaking and honoraria) from the Dementia Academy and Novartis and grants from the Huntington’s Disease Association of Ireland outside the submitted work. Dr Murley reported receiving grants from the Holt Fellowship during the conduct of the study. No other disclosures were reported.

Figures

Figure.
Figure.. Sex- and Age-Specific Incidence Rates of Frontotemporal Lobar Degeneration in Europe per 100 000 Person-Years
Estimates were derived from the fixed effects of a random-intercept Poisson model including age (cubic polynomial) and sex as covariates and a random intercept for residence area. Shading indicates 95% bootstrapped CIs.
See this image and copyright information in PMC

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