Neurology 2002;58:1615-1621
© 2002 American Academy of Neurology
The prevalence of frontotemporal dementia
E. Ratnavalli, MD DM,
C. Brayne, MD FRCP,
K. Dawson, RGN and
J. R. Hodges, MD FRCP
From the Department of Neurology (Drs. Ratnavalli and Hodges, and K. Dawson), Addenbrooke’s Hospital, and Institute of Public Health (Dr. Brayne), University of Cambridge; MRC Cognition & Brain Sciences Unit (Dr. Hodges), Cambridge, United Kingdom; and Department of Neurology (Dr. Ratnavalli), National Institute of Mental Health and Neurosciences, Bangalore, India.
Address correspondence and reprint requests to Prof. John Hodges, MRC Cognition & Brain Sciences Unit, 15 Chaucer Road, Cambridge CB2 2EF, UK; e-mail: john.hodges{at}mrc-cbu.cam.ac.uk
Objective: To estimate the prevalence of frontotemporal dementia (FTD) and other degenerative early-onset dementias in a geographically defined population.
Background: Early-onset dementia (at age <65 years) results in high psychiatric morbidity and caregiver burden. Prevalence figures are available for early-onset AD but not for FTD, a dementia that is almost invariably of early onset.
Methods: Case ascertainment was by review of case records of three specialist clinic databases and inpatient admissions at a university hospital in Cambridge, United Kingdom, for patients with dementia who were <65 years of age, living in Cambridge City or East or South Cambridgeshire (population 326,019) on May 30, 2000. All the relevant health services in the area were also contacted for potential cases. Diagnosis of various dementias was based on published criteria. All patients with potential FTD were examined by the study investigators and underwent structural neuroimaging. The 1998 population estimates for the area were used to calculate age and sex prevalence with confidence intervals for AD, FTD, and other causes of dementia.
Results: A total of 108 patients (66 men and 42 women) with dementia with onset before they were 65 years of age were identified, of whom 60 were <65 years on the census date, giving an overall prevalence of 81 (95% CI, 62.8 to 104.5) per 100,000 in the 45- to 64-year age group. The prevalences of early-onset FTD and AD were the same: 15 per 100,000 (8.4 to 27.0) in the 45- to 64-year-old population. The mean age at onset of FTD was 52.8 years and there was a striking male preponderance (14:3). It is possible case ascertainment methods resulted in a relative underrepresentation of some forms of dementia.
Conclusions: Frontotemporal dementia is a more common cause of early-onset dementia than previously recognized and appears to be more common in men.
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V. M. Van Deerlin, L. H. Gill, J. M. Farmer, J. Q. Trojanowski, and V. M-Y. Lee
Familial Frontotemporal Dementia: From Gene Discovery to Clinical Molecular Diagnostics
Clin. Chem.,
October 1, 2003;
49(10):
1717 - 1725.
[Abstract]
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R J Harvey, M Skelton-Robinson, and M N Rossor
The prevalence and causes of dementia in people under the age of 65 years
J. Neurol. Neurosurg. Psychiatry,
September 1, 2003;
74(9):
1206 - 1209.
[Abstract]
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S. M. Rosso, L. D. Kaat, T. Baks, M. Joosse, I. de Koning, Y. Pijnenburg, D. de Jong, D. Dooijes, W. Kamphorst, R. Ravid, et al.
Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study
Brain,
September 1, 2003;
126(9):
2016 - 2022.
[Abstract]
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T B Gislason, M Sjogren, L Larsson, and I Skoog
The prevalence of frontal variant frontotemporal dementia and the frontal lobe syndrome in a population based sample of 85 year olds
J. Neurol. Neurosurg. Psychiatry,
July 1, 2003;
74(7):
867 - 871.
[Abstract]
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P. M. Stanford, C. E. Shepherd, G. M. Halliday, W. S. Brooks, P. W. Schofield, H. Brodaty, R. N. Martins, J. B. J. Kwok, and P. R. Schofield
Mutations in the tau gene that cause an increase in three repeat tau and frontotemporal dementia
Brain,
April 1, 2003;
126(4):
814 - 826.
[Abstract]
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M. Broe, J.R. Hodges, E. Schofield, C.E. Shepherd, J.J. Kril, and G.M. Halliday
Staging disease severity in pathologically confirmed cases of frontotemporal dementia
Neurology,
March 25, 2003;
60(6):
1005 - 1011.
[Abstract]
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Y. A.L. Pijnenburg, N. S.N.M. Schoonenboom, P. Scheltens, M. Riemenschneider, S. Wagenpfeil, A. Kurz, and H. Forstl
Tau and A{beta}42 protein in CSF of patients with frontotemporal degeneration
Neurology,
January 28, 2003;
60(2):
353 - 354.
[Full Text]
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What Is the True Prevalence of Early-Onset Frontotemporal Dementia?
Journal Watch Neurology,
August 22, 2002;
2002(822):
6 - 6.
[Full Text]
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D. Galasko and K. Marder
Picking away at frontotemporal dementia
Neurology,
June 11, 2002;
58(11):
1585 - 1586.
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