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

Giancarlo Logroscino; Marco Piccininni; Caroline Graff; Orla Hardiman; Albert C. Ludolph; Fermin Moreno; Markus Otto; Anne M. Remes; James B. Rowe; Harro Seelaar; Eino Solje; Elka Stefanova; Latchezar Traykov; Vesna Jelic; Melissa Taheri Rydell; Niall Pender; Sarah Anderl-Straub; Myriam Barandiaran; Alazne Gabilondo; Johanna Krüger; Alexander G. Murley; Timothy Rittman; Emma L. Van Der Ende; John C. Van Swieten; Päivi Hartikainen; Gorana Mandić Stojmenović; Shima Mehrabian; Luisa Benussi; Antonella Alberici; Maria Teresa Dell'abate; Chiara Zecca; Barbara Borroni

doi:10.1001/jamaneurol.2022.5128

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

Giancarlo Logroscino^*, Marco Piccininni, Caroline Graff, Orla Hardiman, Albert C. Ludolph, Fermin Moreno, Markus Otto, Anne M. Remes, James B. Rowe, Harro Seelaar, Eino Solje, Elka Stefanova, Latchezar Traykov, Vesna Jelic, Melissa Taheri Rydell, Niall Pender, Sarah Anderl-Straub, Myriam Barandiaran, Alazne Gabilondo, Johanna KrügerAlexander G. Murley, Timothy Rittman, Emma L. Van Der Ende, John C. Van Swieten, Päivi Hartikainen, Gorana Mandić Stojmenović, Shima Mehrabian, Luisa Benussi, Antonella Alberici, Maria Teresa Dell'abate, Chiara Zecca, Barbara Borroni

^*Corresponding author for this work

Neurology

Research output: Contribution to journal › Article › Academic › peer-review

51 Citations (Scopus)

35 Downloads (Pure)

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 11023643 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 100000 person-years (95% CI, 1.59-3.51 cases per 100000 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 100000 person-years; 95% CI, 1.88-4.27 cases per 100000 person-years) than among women (1.91 cases per 100000 person-years; 95% CI, 1.26-2.91 cases per 100000 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 12057. 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..

Original language	English
Pages (from-to)	279-286
Number of pages	8
Journal	JAMA Neurology
Volume	80
Issue number	3
DOIs	https://doi.org/10.1001/jamaneurol.2022.5128
Publication status	Published - 13 Mar 2023

Bibliographical note

Funding Information:
Funding/Support: Dr Logroscino was partially supported by grant B84I18000540002 from the Regione Puglia and CNR for Tecnopolo per la Medicina di Precisione, and by the Research Center of Excellence for Neurodegenerative Diseases and Brain Aging (CIREMIC). Dr Graff was partially supported by the Schörling Foundation (Swedish FTD Initiative), Swedish Demensfonden, ALF–Region Stockholm, Vetenskapsrådet Dnr 2018-02754 and JPND 2019-02248 from the Swedish Research Council, and Gamla Tjänarinnor. Dr Moreno was partially supported by the Tau Consortium. Dr Remes was partly supported by grant 315460 from the Academy of Finland. Dr Rowe was partially supported by grant SUAG/051 G101400 from the Medical Research Council, by the Cambridge Centre for Parkinson-Plus, by a Holt fellowship, and by grant BRC-1215-20014 from the National Institute for Health Research Cambridge Biomedical Research Centre. Dr Seelaar was partially supported by grant 733050513 from the Netherlands Organisation for Health Research and Development, Alzheimer Nederland, and the Bluefield Project to Cure Frontotemporal Dementia. Dr Solje was partially supported by the Sigrid Jusélius Foundation, Instrumentarium Science Foundation, Orion Research Foundation, and Finnish Brain Foundation. Dr Stefanova was partially supported by N175090 from the Ministry of Education and Science, Republic of Serbia. Dr Traykov was partially supported by KP-06-N53/3 and DN 03/12 from the Bulgarian National Science Fund. Dr Benussi was partially supported by the Italian Ministry of Health–Ricerca Corrente.

Publisher Copyright:
© 2023 American Medical Association. All rights reserved.

Access to Document

10.1001/jamaneurol.2022.5128Licence: CC BY-NC-ND

Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European CountriesFinal published version, 231 KBLicence: CC BY-NC-ND

Cite this

Logroscino, G., Piccininni, M., Graff, C., Hardiman, O., Ludolph, A. C., Moreno, F., Otto, M., Remes, A. M., Rowe, J. B., Seelaar, H., Solje, E., Stefanova, E., Traykov, L., Jelic, V., Rydell, M. T., Pender, N., Anderl-Straub, S., Barandiaran, M., Gabilondo, A., ... Borroni, B. (2023). Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries. JAMA Neurology, 80(3), 279-286. https://doi.org/10.1001/jamaneurol.2022.5128

@article{4acd72b22eb94d82a67c43c379429e58,

title = "Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries",

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 11023643 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 100000 person-years (95% CI, 1.59-3.51 cases per 100000 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 100000 person-years; 95% CI, 1.88-4.27 cases per 100000 person-years) than among women (1.91 cases per 100000 person-years; 95% CI, 1.26-2.91 cases per 100000 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 12057. 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..",

author = "Giancarlo Logroscino and Marco Piccininni and Caroline Graff and Orla Hardiman and Ludolph, {Albert C.} and Fermin Moreno and Markus Otto and Remes, {Anne M.} and Rowe, {James B.} and Harro Seelaar and Eino Solje and Elka Stefanova and Latchezar Traykov and Vesna Jelic and Rydell, {Melissa Taheri} and Niall Pender and Sarah Anderl-Straub and Myriam Barandiaran and Alazne Gabilondo and Johanna Kr{\"u}ger and Murley, {Alexander G.} and Timothy Rittman and {Van Der Ende}, {Emma L.} and {Van Swieten}, {John C.} and P{\"a}ivi Hartikainen and Stojmenovi{\'c}, {Gorana Mandi{\'c}} and Shima Mehrabian and Luisa Benussi and Antonella Alberici and Dell'abate, {Maria Teresa} and Chiara Zecca and Barbara Borroni",

note = "Funding Information: Funding/Support: Dr Logroscino was partially supported by grant B84I18000540002 from the Regione Puglia and CNR for Tecnopolo per la Medicina di Precisione, and by the Research Center of Excellence for Neurodegenerative Diseases and Brain Aging (CIREMIC). Dr Graff was partially supported by the Sch{\"o}rling Foundation (Swedish FTD Initiative), Swedish Demensfonden, ALF–Region Stockholm, Vetenskapsr{\aa}det Dnr 2018-02754 and JPND 2019-02248 from the Swedish Research Council, and Gamla Tj{\"a}narinnor. Dr Moreno was partially supported by the Tau Consortium. Dr Remes was partly supported by grant 315460 from the Academy of Finland. Dr Rowe was partially supported by grant SUAG/051 G101400 from the Medical Research Council, by the Cambridge Centre for Parkinson-Plus, by a Holt fellowship, and by grant BRC-1215-20014 from the National Institute for Health Research Cambridge Biomedical Research Centre. Dr Seelaar was partially supported by grant 733050513 from the Netherlands Organisation for Health Research and Development, Alzheimer Nederland, and the Bluefield Project to Cure Frontotemporal Dementia. Dr Solje was partially supported by the Sigrid Jus{\'e}lius Foundation, Instrumentarium Science Foundation, Orion Research Foundation, and Finnish Brain Foundation. Dr Stefanova was partially supported by N175090 from the Ministry of Education and Science, Republic of Serbia. Dr Traykov was partially supported by KP-06-N53/3 and DN 03/12 from the Bulgarian National Science Fund. Dr Benussi was partially supported by the Italian Ministry of Health–Ricerca Corrente. Publisher Copyright: {\textcopyright} 2023 American Medical Association. All rights reserved.",

year = "2023",

month = mar,

day = "13",

doi = "10.1001/jamaneurol.2022.5128",

language = "English",

volume = "80",

pages = "279--286",

journal = "JAMA Neurology",

issn = "2168-6149",

publisher = "American Medical Association",

number = "3",

}

Logroscino, G, Piccininni, M, Graff, C, Hardiman, O, Ludolph, AC, Moreno, F, Otto, M, Remes, AM, Rowe, JB, Seelaar, H, Solje, E, Stefanova, E, Traykov, L, Jelic, V, Rydell, MT, Pender, N, Anderl-Straub, S, Barandiaran, M, Gabilondo, A, Krüger, J, Murley, AG, Rittman, T, Van Der Ende, EL, Van Swieten, JC, Hartikainen, P, Stojmenović, GM, Mehrabian, S, Benussi, L, Alberici, A, Dell'abate, MT, Zecca, C & Borroni, B 2023, 'Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries', JAMA Neurology, vol. 80, no. 3, pp. 279-286. https://doi.org/10.1001/jamaneurol.2022.5128

TY - JOUR

T1 - Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries

AU - Logroscino, Giancarlo

AU - Piccininni, Marco

AU - Graff, Caroline

AU - Hardiman, Orla

AU - Ludolph, Albert C.

AU - Moreno, Fermin

AU - Otto, Markus

AU - Remes, Anne M.

AU - Rowe, James B.

AU - Seelaar, Harro

AU - Solje, Eino

AU - Stefanova, Elka

AU - Traykov, Latchezar

AU - Jelic, Vesna

AU - Rydell, Melissa Taheri

AU - Pender, Niall

AU - Anderl-Straub, Sarah

AU - Barandiaran, Myriam

AU - Gabilondo, Alazne

AU - Krüger, Johanna

AU - Murley, Alexander G.

AU - Rittman, Timothy

AU - Van Der Ende, Emma L.

AU - Van Swieten, John C.

AU - Hartikainen, Päivi

AU - Stojmenović, Gorana Mandić

AU - Mehrabian, Shima

AU - Benussi, Luisa

AU - Alberici, Antonella

AU - Dell'abate, Maria Teresa

AU - Zecca, Chiara

AU - Borroni, Barbara

N1 - Funding Information: Funding/Support: Dr Logroscino was partially supported by grant B84I18000540002 from the Regione Puglia and CNR for Tecnopolo per la Medicina di Precisione, and by the Research Center of Excellence for Neurodegenerative Diseases and Brain Aging (CIREMIC). Dr Graff was partially supported by the Schörling Foundation (Swedish FTD Initiative), Swedish Demensfonden, ALF–Region Stockholm, Vetenskapsrådet Dnr 2018-02754 and JPND 2019-02248 from the Swedish Research Council, and Gamla Tjänarinnor. Dr Moreno was partially supported by the Tau Consortium. Dr Remes was partly supported by grant 315460 from the Academy of Finland. Dr Rowe was partially supported by grant SUAG/051 G101400 from the Medical Research Council, by the Cambridge Centre for Parkinson-Plus, by a Holt fellowship, and by grant BRC-1215-20014 from the National Institute for Health Research Cambridge Biomedical Research Centre. Dr Seelaar was partially supported by grant 733050513 from the Netherlands Organisation for Health Research and Development, Alzheimer Nederland, and the Bluefield Project to Cure Frontotemporal Dementia. Dr Solje was partially supported by the Sigrid Jusélius Foundation, Instrumentarium Science Foundation, Orion Research Foundation, and Finnish Brain Foundation. Dr Stefanova was partially supported by N175090 from the Ministry of Education and Science, Republic of Serbia. Dr Traykov was partially supported by KP-06-N53/3 and DN 03/12 from the Bulgarian National Science Fund. Dr Benussi was partially supported by the Italian Ministry of Health–Ricerca Corrente. Publisher Copyright: © 2023 American Medical Association. All rights reserved.

PY - 2023/3/13

Y1 - 2023/3/13

N2 - 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 11023643 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 100000 person-years (95% CI, 1.59-3.51 cases per 100000 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 100000 person-years; 95% CI, 1.88-4.27 cases per 100000 person-years) than among women (1.91 cases per 100000 person-years; 95% CI, 1.26-2.91 cases per 100000 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 12057. 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..

AB - 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 11023643 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 100000 person-years (95% CI, 1.59-3.51 cases per 100000 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 100000 person-years; 95% CI, 1.88-4.27 cases per 100000 person-years) than among women (1.91 cases per 100000 person-years; 95% CI, 1.26-2.91 cases per 100000 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 12057. 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..

UR - http://www.scopus.com/inward/record.url?scp=85150072198&partnerID=8YFLogxK

U2 - 10.1001/jamaneurol.2022.5128

DO - 10.1001/jamaneurol.2022.5128

M3 - Article

C2 - 36716024

AN - SCOPUS:85150072198

SN - 2168-6149

VL - 80

SP - 279

EP - 286

JO - JAMA Neurology

JF - JAMA Neurology

IS - 3

ER -

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

Abstract

Bibliographical note

Access to Document

Other files and links

Fingerprint

Cite this