title: Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes.
abstract: To estimate the lifetime risk, prevalence, incidence, and mortality of the principal clinical syndromes associated with frontotemporal lobar degeneration (FTLD) using revised diagnostic criteria and including intermediate clinical phenotypes.
  
  
  
  Multisource referral over 2 years to identify all diagnosed or suspected cases of frontotemporal dementia (FTD), progressive supranuclear palsy (PSP), or corticobasal syndrome (CBS) in 2 UK counties (population 1.69 million). Diagnostic confirmation used current consensus diagnostic criteria after interview and reexamination. Results were adjusted to the 2013 European standard population.
  
  
  
  The prevalence of FTD, PSP, and CBS was 10.8/100,000. The incidence and mortality were very similar, at 1.61/100,000 and 1.56/100,000 person-years, respectively. The estimated lifetime risk is 1 in 742. Survival following diagnosis varied widely: from PSP 2.9 years to semantic variant FTD 9.1 years. Age-adjusted prevalence peaked between 65 and 69 years at 42.6/100,000: the age-adjusted prevalence for persons older than 65 years is double the prevalence for those between 40 and 64 years. Fifteen percent of those screened had a relevant genetic mutation.
  
  
  
  Key features of this study include the revised diagnostic criteria with improved specificity and sensitivity, an unrestricted age range, and simultaneous assessment of multiple FTLD syndromes. The prevalence of FTD, PSP, and CBS increases beyond 65 years, with frequent genetic causes. The time from onset to diagnosis and from diagnosis to death varies widely among syndromes, emphasizing the challenge and importance of accurate and timely diagnosis. A high index of suspicion for FTLD syndromes is required by clinicians, even for older patients.
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany. itsc2@medschl.cam.ac.uk.
authors.forename: Ian T S
authors.initials: IT
authors.lastname: Coyle-Gilchrist
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Katrina M
authors.initials: KM
authors.lastname: Dick
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Karalyn
authors.initials: K
authors.lastname: Patterson
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Patricia
authors.initials: P
authors.lastname: Vázquez Rodríquez
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Eileen
authors.initials: E
authors.lastname: Wehmann
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Alicia
authors.initials: A
authors.lastname: Wilcox
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Claire J
authors.initials: CJ
authors.lastname: Lansdall
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Kate E
authors.initials: KE
authors.lastname: Dawson
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Julie
authors.initials: J
authors.lastname: Wiggins
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Simon
authors.initials: S
authors.lastname: Mead
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: Carol
authors.initials: C
authors.lastname: Brayne
authors.affiliation: From the Department of Clinical Neurosciences (I.T.S.C.-G., K.P., P.V.R., A.W., C.J.L., K.E.D., J.W., J.B.R.) and Cambridge Institute of Public Health (C.B.), University of Cambridge; Dementia Research Centre (K.M.D.) and MRC Prion Unit and Department of Neurodegenerative Disease (S.M.), University College London, UK; and University Medical Center Eppendorf (E.W.), University Hamburg, Germany.
authors.forename: James B
authors.initials: JB
authors.lastname: Rowe
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D001037:Aphasia / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D057174:Frontotemporal Lobar Degeneration / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D015995:Prevalence; D012306:Risk; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D016019:Survival Analysis; D013577:Syndrome; D006113:United Kingdom / Q000453:epidemiology