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Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes

التفاصيل البيبلوغرافية
العنوان: Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes
المؤلفون: Patricia Vázquez Rodríquez, Eileen Wehmann, Katrina M. Dick, Claire J. Lansdall, Alicia Wilcox, Karalyn Patterson, Kate Dawson, Ian Coyle-Gilchrist, Simon Mead, Carol Brayne, James B. Rowe, Julie Wiggins
المساهمون: Patterson, Karalyn [0000-0003-1927-7424], Brayne, Carol [0000-0001-5307-663X], Rowe, James [0000-0001-7216-8679], Apollo - University of Cambridge Repository
المصدر: Neurology
بيانات النشر: Lippincott Williams & Wilkins, 2016.
سنة النشر: 2016
مصطلحات موضوعية: 0301 basic medicine, Adult, Male, Risk, medicine.medical_specialty, Pediatrics, Population, Disease, Article, Progressive supranuclear palsy, 03 medical and health sciences, 0302 clinical medicine, mental disorders, medicine, Aphasia, Prevalence, Corticobasal degeneration, Humans, Psychiatry, education, Survival analysis, Aged, education.field_of_study, business.industry, Incidence (epidemiology), Incidence, Age Factors, Frontotemporal lobar degeneration, Syndrome, Middle Aged, medicine.disease, Survival Analysis, United Kingdom, 3. Good health, 030104 developmental biology, Phenotype, Female, Neurology (clinical), Supranuclear Palsy, Progressive, Frontotemporal Lobar Degeneration, business, 030217 neurology & neurosurgery, Frontotemporal dementia
الوصف: 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. 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. 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. 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.
وصف الملف: application/pdf
اللغة: English
تدمد: 1526-632X
0028-3878
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::27eddeafa13ab0eb2d943c4cbc50e190
http://europepmc.org/articles/PMC4854589
Rights: OPEN
رقم الانضمام: edsair.doi.dedup.....27eddeafa13ab0eb2d943c4cbc50e190
قاعدة البيانات: OpenAIRE
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