Giancarlo Logroscino1, Marco Piccininni2, Caroline Graff3, Orla Hardiman4, Albert Ludolph5, Fermin Moreno6, Markus Otto5, Anne Remes7, James Rowe8, Harro Seelaar9, Eino Solje10, Elka Stefanova11, Latchezar Traykov12, Vesna Jelic13, Melissa Thaeri Rydell3, Niall Pender14, Sarah Anderl-Straub5, Myriam Barandiaran6, Alazne Gabilondo6, Johanna Kruger7, Alexander Murley8, Timothy Rittman15, Emma L van der Ende10, John Van Swieten10, Paivi Hartikainen16, Gorana Mandic Stojmenovic17, Shima Meherabian12, Luisa Benussi18, Antonella Alberici19, Maria Teresa Dell'Abate1, Chiara Zecca1, Barbara Borroni19
1Center for Neurodegenerative Diseases and the Aging Brain, Pia Fondazione Cardinale Giovanni Panico, University of Bari-Aldo Moro, Bari, Italy, 2Institute of Public Health, Charité – Universitätsmedizin Berlin, Berlin, Germany, 3Department NVS, Division of Neurogeriatrics, Karolinska Institutet, Solna, Sweden, 4Trinity Biomedical Sciences Institute, 5Department of Neurology, University Hospital Ulm, Ulm, Germany, 6Cognitive Disorders Unit, Department of Neurology, Hospital Universitario Donostia, San Sebastian, Spain, 7Unit of Clinical Neuroscience, Neurology, University of Oulu, Oulu, Finland, 8Department of Clinical Neurosciences, MRC Cognition and Brain Sciences Unit, and Cambridge University Hospitals NHS Cambridge, United Kingdom, 9Erasmus University Medical Center, 10Department of Neurology and Alzheimer Center Erasmus MC, Erasmus MC University Medical Center, Rotterdam, the Netherlands, 1119. Faculty of Medicine, Neurology Clinic, University Clinical Center, University of Belgrade, Serbia, 12UH Alexandrovska, Department of Neurology, Medical University Sofia, Sofia, Bulgaria, 13Theme Inflammation and Aging, Medical Unit Aging Brain, Karolinska Universtity Hospital Huddinge, Solna, Sweden, 14Department of Neurology, Beaumont Hospital, Dublin, Ireland, 15Department of Clinical Neurosciences, 16Kuopio University Hospital, 17Faculty of Medicine, Neurology Clinic, University Clinical Center, University of Belgrade, Serbia, 18Molecular Markers Laboratory, IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy, 19Centre for Neurodegenerative Disorders, Neurology Unit, ASST Spedali Civili Brescia and University of Brescia, Italy
Objective:
To assess FTLD epidemiology across Europe. The FRONTotemporal dementia Incidence European Research Study (FRONTIERS) was established for this purpose.
Background:
Diagnostic incidence data for syndromes associated with Frontotemporal Lobar Degeneration (FTLD) in multinational studies is urgent in light of upcoming therapeutic approaches.
Design/Methods:
Retrospective population-based study conducted from June 1st, 2018 to May 31st, 2019 in thirteen tertiary FTLD research clinics from the UK, Netherlands, Finland, Sweden, Spain, Bulgaria, Serbia, Germany, and Italy. All new FTLD-associated cases (hereafter: incident FTLD) during the study period were counted, with a combined catchment population of over 11,000,000 person-years. 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 age±standard deviation=66.7±9.02; female=41.6%), the annual incidence rate for FTLD in Europe was 2.36 per 100,000 person-years (95% Confidence Intervals [CI]=1.59-3.51). There was a progressive increase in FTLD incidence across age, reaching its peak at the age of 71. Overall, the incidence was higher among men (2.84 per 100,000 person-years; 95% CI=1.88-4.27) than among women (1.91 per 100,000 person-years; 95% CI=1.26-2.91). Behavioural variant frontotemporal dementia was the most common phenotype (40.07%), followed by primary progressive aphasia (28.46%) and extrapyramidal phenotypes (25.84%). Frontotemporal dementia with amyotrophic lateral sclerosis was the rarest phenotype (5.62%). Almost 35% of FTLD patients presented a family history of dementia. The estimated number of new FTLD cases in Europe is approximately 12,000 people per year.
Conclusions:
FTLD-associated syndromes are more common than previously recognized, and diagnosis should be considered at any age. Improved knowledge of FTLD epidemiology may contribute to appropriate health and social care planning, and in the design of future clinical trials.