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dc.contributor.authorCoyle-Gilchrist, Ian TSen
dc.contributor.authorDick, Katrina Men
dc.contributor.authorPatterson, Karalynen
dc.contributor.authorVázquez, Rodríquez Patriciaen
dc.contributor.authorWehmann, Eileenen
dc.contributor.authorWilcox, Aliciaen
dc.contributor.authorLansdall, Claireen
dc.contributor.authorDawson, Kate Een
dc.contributor.authorWiggins, Julieen
dc.contributor.authorMead, Simonen
dc.contributor.authorBrayne, Carolen
dc.contributor.authorRowe, Jamesen
dc.date.accessioned2016-03-15T10:38:25Z
dc.date.available2016-03-15T10:38:25Z
dc.date.issued2016-04-01en
dc.identifier.citationCoyle-Gilchrist et al. Neurology (2016)en
dc.identifier.issn0028-3878
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/254475
dc.description.abstract$\textbf{Objectives:}$ 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. $\textbf{Methods:}$ 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. $\textbf{Results:}$ 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. $\textbf{Conclusions:}$ 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.
dc.description.sponsorshipThis study was funded by the NIHR Cambridge Dementia Biomedical Research Centre and Biomedical Research Unit in Dementia; the Wellcome Trust (103838), the PSP Association; Alzheimer Research UK; The Evelyn Trust.
dc.languageEnglishen
dc.language.isoenen
dc.publisherWolters Kluwer
dc.rightsAttribution 4.0 International
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rightsAttribution 4.0 Internationalen
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/en
dc.subjectfrontotemporal dementiaen
dc.subjectcorticobasal degenerationen
dc.subjectdementia aphasiaen
dc.subjectall epidemiologyen
dc.subjectprogressive supranuclear palsyen
dc.titlePrevalence, characteristics, and survival of frontotemporal lobar degeneration syndromesen
dc.typeArticle
dc.description.versionThis is the final version of the article. It first appeared from Wolters Kluwer via http://dx.doi.org/10.​1212/​WNL.​0000000000002638en
prism.endingPage1743
prism.publicationDate2016en
prism.publicationNameNeurologyen
prism.startingPage1736
prism.volume86en
dc.rioxxterms.funderNIHR
dc.rioxxterms.funderWellcome Trust
dc.rioxxterms.projectid103838
dcterms.dateAccepted2016-01-21en
rioxxterms.versionofrecord10.1212/WNL.0000000000002638en
rioxxterms.versionVoR
rioxxterms.licenseref.urihttp://creativecommons.org/licenses/by/4.0/en
rioxxterms.licenseref.startdate2016-04-01en
dc.contributor.orcidPatterson, Karalyn [0000-0003-1927-7424]
dc.contributor.orcidBrayne, Carol [0000-0001-5307-663X]
dc.contributor.orcidRowe, James [0000-0001-7216-8679]
dc.identifier.eissn1526-632X
rioxxterms.typeJournal Article/Reviewen
pubs.funder-project-idDepartment of Health (via National Institute for Health Research (NIHR)) (unknown)
pubs.funder-project-idWELLCOME TRUST (103838/Z/14/Z)
pubs.funder-project-idEvelyn Trust (46722)
pubs.funder-project-idMedical Research Council (MC_U105597119)
pubs.funder-project-idCambridge University Hospitals NHS Foundation Trust (CUH) (unknown)
pubs.funder-project-idPSP Association (unknown)
pubs.funder-project-idEvelyn Trust (17/09)
pubs.funder-project-idMEDICAL RESEARCH COUNCIL (MR/J009482/1)
pubs.funder-project-idWellcome Trust (093875/Z/10/Z)
cam.orpheus.successThu Jan 30 12:54:54 GMT 2020 - The item has an open VoR version.*
rioxxterms.freetoread.startdate2100-01-01


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Attribution 4.0 International
Except where otherwise noted, this item's licence is described as Attribution 4.0 International