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Vol. 17, No. 4, 2004
Issue release date: June 2004
Dement Geriatr Cogn Disord 2004;17:253–257

Natural History of Frontotemporal Dementia: Comparison with Alzheimer’s Disease

Pasquier F. · Richard F. · Lebert F.
Memory Clinic, University Hospital, EA 2691, and INSERM U 508, Lille, France

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Introduction: Frontotemporal dementia (FTD) is a more common cause of dementia than previously recognised. Few data are available regarding the natural course of FTD in terms of survival, nursing home admission and causes of death. Methods: An observational study of all consecutive patients referred to the memory centre of Lille, France, between 1995 and 1999, and examined at least twice in this centre, with a diagnosis of FTD (frontal or behavioural variant) or of Alzheimer’s disease (AD) was performed. Kaplan-Meyer analysis allowing for delayed entry was used to compare the survival functions in FTD and AD. Results: 552 patients were included, of whom 49 (8.9%) were lost to follow-up at 3 years. FTD patients were younger (mean age at onset 59 years), had more often a family history of psychiatric disorders (20%), had a longer delay between first symptoms and first visit (5.9 years) and a higher Mini-Mental State Examination (MMSE) score at first visit (24.5) than patients with AD (19.9). The mean annual MMSE score decline was 0.9 point in FTD vs. 2.0 points in AD (p < 0.0004). Fewer patients with FTD than with AD entered an institution (RR: 0.20, 95% CI 0.05–0.81). After adjustment for sex, age at first visit, level of education and MMSE score at first visit, survival rates in FTD and AD did not differ significantly. Patients with FTD often had a sudden death, the cause of which could not be found. The earlier the first visit after onset, the longer the survival rate, whatever the diagnosis (RR: 0.76, 95% CI 0.67–0.86, p < 0.0001 per year of earlier first visit). Conclusion: This large study showed that the mean duration of FTD was 2 years longer than that of AD, but the risk of death after adjustment for age and sex was similar in FTD and in AD. Sudden and unexplained causes of death were frequent and need further study. Early management increases the life span of demented patients.

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  1. Harvey RJ, Rossor MN, Skelton-Robinson M, Garralda E: Young onset dementia: Epidemiology, clinical symptoms, family burden, support and outcome. 1998. Available at:
  2. Ratnavalli E, Brayne C, Dawson K, Hodges JR: The prevalence of frontotemporal dementia. Neurology 2002;58:1615–1621.
  3. Rosso SM, Kaat LD, Baks T, Joosse M, de Koning I, Pijnenburg Y, de Jong D, Dooijes D, Kamphorst W, Ravid R, Niermeijer MF, Verheij F, Kremer HP, Scheltens P, van Duijn CM, Heutink P, van Swieten JC: Frontotemporal dementia in The Netherlands: Patients characteristics and prevalence estimates from a population-based study. Brain 2003;126:2016–2022.
  4. Gustafson L, Brun A, Passant U: Frontal lobe degeneration of non-Alzheimer type; in Rossor MN (ed): Unusual Dementias. London, Baillière, 1992, pp 559–583.
  5. Snowden JS, Neary D, Mann DMA: Fronto-Temporal Lobar Degeneration: Fronto-Temporal Dementia, Progressive Aphasia, Semantic Dementia. New York, Edinburgh, Churchill Livingstone, 1996.
  6. Brun A, Englund E, Gustafson L, Passant U, Mann DMA, Neary D, Snowden JS: Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994;57:416–418.
  7. McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan EM: Clinical diagnosis of Alzheimer’s disease: Report of the NINCDS-ADRDA Work Group under the auspices of the Department of Health and Human Services Task Forces on Alzheimer’s disease. Neurology 1984;34:939–944.
  8. Pasquier F, Lebert F, Petit H: Consultations et centres de la mémoire. Marseille, Solal, 1997.
  9. Pasquier F, Lebert F, Petit H: Organisation des centres de la mémoire et perspectives. Rev Neurol (Paris) 1999;155:4S83–4S92.
  10. Edwards-Lee T, Miller BL, Benson DF, Cummings JL, Russell GL, Boone K, Mena I: The temporal variant of frontotemporal dementia. Brain 1997;120:1027–1040.

    External Resources

  11. Dal Forno G, Carson KA, Brookmeyer R, Troncoso JC, Kawas CH, Brandt J: APOE genotype and survival in men and women with Alzheimer’s disease. Neurology 2002;58:1045–1050.
  12. Wolfson C, Wolfson DB, Asgharian M, M’Lan CE, Ostbye T, Rockwood K, Hogan DB, for the Clinical Progression of Dementia Study Group: A reevaluation of the duration of survival after the onsert of dementia. N Engl J Med 2001;344:1111–1116.
  13. Klein JP, Moeschberger ML: Survival Analysis: Techniques for Censored and Truncated Data. New York, Springer, 1997.
  14. Chow TW, Miller BL, Hayashi VN, Geschwind DH: Inheritance of frontotemporal dementia. Arch Neurol 1999;56:817–822.
  15. Hodges JR, Davies R, Xuereb J, Kril J, Hallyday G: Survival in frontotemporal dementia. Neurology 2003;61:349–354.
  16. Stevens M, van Duijn CM, Kamphorst W, de Knijff P, Heutink P, van Gool WA, Scheltens P, Ravid R, Oostra BA, Niermeijer MF, van Swieten JC: Familial aggregation in frontotemporal dementia. Neurology 1998;50:1541–1545.
  17. Jost BC, Grossberg GT: The natural history of Alzheimer’s disease: A brain bank study. J Am Geriatr Soc 1995;43:1248–1255.
  18. Brookmeyer R, VCorrada MM, Curriero FC, Kawas C: Survival following a diagnosis of Alzheimer disease. Arch Neurol 2002;59:1764–1767.
  19. Pasquier F, Lebert F, Lavenu I, Guillaume B: The clinical picture of frontotemporal dementia: Diagnosis and follow-up. Dement Geriatr Cogn Disord 1999;10(suppl 1):10–14.
  20. Han L, Cole M, Bellavance F, McCusker J, Primeau F: Tracking cognitive decline in Alzheimer’s disease using the Mini-Mental State Examination: A meta-analysis. Int Psychogeriatr 2000;12:131–247.

    External Resources

  21. Mungas D, Reed BR, Ellis WG, Jagust WJ: The effects of age on rate of progression of Alzheimer disease and dementia with associated cerebrovascular disease. Arch Neurol 2001;58:1243–1247.
  22. Pasquier F, Richard F, Lebert F: Diagnosis and management of frontotemporal dementia; in Iqbal K, Winblad B (eds): Alzheimer’s Disease and Related Disorders: Research Advances. Bucarest, Anna Aslan International Academy of Aging, 2003, pp 149–158.
  23. Keene J, Hope T, Fairburn CG, Jacoby R: Death and dementia. Int J Geriatr Psychiatr 2001;16:969–974.
  24. Helmer C, Joly P, Letenneur L, Commenges D, Dartigues JF: Mortality with dementia: Results from a French prospective community-based cohort. Am J Epidemiol 2001;54:642–648.
  25. Lebert F, Delacourte A, Pasquier F: Treatment of frontotemporal dementia; in Gauthier S, Cummings J (eds): Alzheimer’s Disease and Related Disorders. London, Martin Dunitz, 2002, pp 169–180.

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