Journal of Molecular Neuroscience

, Volume 45, Issue 3, pp 330–335 | Cite as

Estimating the Number of Persons with Frontotemporal Lobar Degeneration in the US Population

Article

Abstract

There are many challenges for determining the prevalence and incidence of frontotemporal lobar degenerations (FTLD). Consequently, the number of cases of behavioral variant frontotemporal dementia (bvFTD) or primary progressive aphasia (PPA) in the USA is unknown. Our objective was to derive a consensus estimate of bvFTD and PPA prevalence and thereby to estimate the total number of these syndromes in the USA. We identified five prevalence and three incidence studies of FTLD based on passive surveillance and seven studies of survival in FTLD. Data from these studies were used to estimate the number of cases of PPA or bvFTD in the USA. Because prevalence and incidence estimates outside of the 45–64-year age range were either not available or widely divergent, we used data from clinical and pathological series to estimate the proportion of FTLD cases aged <45 or >64 years. The prevalence estimates in the age categories of 45–64 years old have ranged from 15 to 22 per 100,000 person-years in studies where both bvFTD and PPA were identified. The incidence estimates for the same age group ranged from 2.7 to 4.1 per 100,000 person-years. Using a survival rate of 6 to 9 years from onset and rates from the incidence studies, a calculated prevalence estimate (prevalence = incidence × duration) was similar to the previously reported prevalence rates. We estimated that 10% of cases were less than age 45 years and 30% were 65 years and older. We estimate that there are approximately 20,000 to 30,000 cases of the cognitive syndromes of FTLD in the USA. The main threat to the accuracy of the estimates is the difficulty in diagnosing the clinical syndromes that comprise the FTLD group of disorders.

Keywords

Frontotemporal lobar degeneration Prevalence Incidence 

Notes

Acknowledgments

This work is supported by NIH grants U01 AG06786, P50 AG16574, and by the Robert H. and Clarice Smith and Abigail van Buren Alzheimer’s Disease Research Program.

Disclosures

DSK serves on a Data Safety Monitoring Board for Lilly Pharmaceuticals and is an investigator for clinical trials sponsored by Elan Pharmaceuticals, Forest Pharmaceuticals, and Baxter Healthcare. He is deputy editor of Neurology and receives compensation for editorial activities. ROR receives research funding from Abbott Laboratories.

References

  1. Barker WW, Luis CA, Kashuba A, Luis M, Harwood DG, Loewenstein D et al (2002) Relative frequencies of Alzheimer disease, lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the state of Florida brain bank. Alzheimer Dis Assoc Disord 16:203–212PubMedCrossRefGoogle Scholar
  2. Borroni B, Alberici A, Grassi M, Turla M, Zanetti O, Bianchetti A et al (2010) Is frontotemporal lobar degeneration a rare disorder? Evidence from a preliminary study in Brescia county, Italy. J Alzheimers Dis 19:111–116PubMedGoogle Scholar
  3. Chiu WZ, Kaat LD, Seelaar H, Rosso SM, Boon AJ, Kamphorst W et al (2010) Survival in progressive supranuclear palsy and frontotemporal dementia. J Neurol Neurosurg Psychiatry 81:441–445PubMedCrossRefGoogle Scholar
  4. Davies RR, Hodges JR, Kril JJ, Patterson K, Halliday GM, Xuereb JH (2005) The pathological basis of semantic dementia. Brain 128:1984–1995PubMedCrossRefGoogle Scholar
  5. Davies RR, Kipps CM, Mitchell J, Kril JJ, Halliday GM, Hodges JR (2006) Progression in frontotemporal dementia: identifying a benign behavioral variant by magnetic resonance imaging. Arch Neurol 63:1627–1631PubMedCrossRefGoogle Scholar
  6. Forman MS, Farmer J, Johnson JK, Clark CM, Arnold SE, Coslett HB et al (2006) Frontotemporal dementia: clinicopathological correlations. Ann Neurol 59:952–962PubMedCrossRefGoogle Scholar
  7. Garcin B, Lillo P, Hornberger M, Piguet O, Dawson K, Nestor PJ et al (2009) Determinants of survival in behavioral variant frontotemporal dementia. Neurology 73:1656–1661PubMedCrossRefGoogle Scholar
  8. Garre-Olmo J, Genis Batlle D, del Mar Fernandez M, Marquez Daniel F, de Eugenio Huelamo R, Casadevall T et al (2010) Incidence and subtypes of early-onset dementia in a geographically defined general population. Neurology 75:1249–1255PubMedCrossRefGoogle Scholar
  9. Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF et al (2011) Classification of primary progressive aphasia and its variants. Neurology 76:1006–1014PubMedCrossRefGoogle Scholar
  10. Harvey RJ, Skelton-Robinson M, Rossor MN (2003) The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurg Psychiatry 74:1206–1209PubMedCrossRefGoogle Scholar
  11. Hodges JR, Davies R, Xuereb J, Kril J, Halliday G (2003) Survival in frontotemporal dementia. Neurology 61:349–354PubMedGoogle Scholar
  12. Hodges JR, Davies RR, Xuereb JH, Casey B, Broe M, Bak TH et al (2004) Clinicopathological correlates in frontotemporal dementia. Ann Neurol 56:399–406PubMedCrossRefGoogle Scholar
  13. Ikejima C, Yasuno F, Mizukami K, Sasaki M, Tanimukai S, Asada T (2009) Prevalence and causes of early-onset dementia in Japan: a population-based study. Stroke 40:2709–2714PubMedCrossRefGoogle Scholar
  14. Johnson JK, Diehl J, Mendez MF, Neuhaus J, Shapira JS, Forman M et al (2005) Frontotemporal lobar degeneration: demographic characteristics of 353 patients. Arch Neurol 62:925–930PubMedCrossRefGoogle Scholar
  15. Josephs KA, Knopman DS, Whitwell JL, Boeve BF, Parisi JE, Petersen RC et al (2005) Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND. Neurology 65:645–647PubMedCrossRefGoogle Scholar
  16. Knopman D, Petersen R, Edland S, Cha R, Rocca WA (2004) The incidence of fronto-temporal lobar degeneration in Rochester, Minnesota, 1990–1994. Neurology 62:506–508PubMedGoogle Scholar
  17. Knopman DS, Boeve BF, Parisi JE, Dickson DW, Smith GE, Ivnik RJ et al (2005) Antemortem diagnosis of frontotemporal lobar degeneration. Ann Neurol 57:480–488PubMedCrossRefGoogle Scholar
  18. Logroscino G, Traynor BJ, Hardiman O, Chio A, Mitchell D, Swingler RJ et al (2010) Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 81:385–390PubMedCrossRefGoogle Scholar
  19. Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J et al (2009) Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations. Acta Neuropathol 117:15–18PubMedCrossRefGoogle Scholar
  20. Mercy L, Hodges JR, Dawson K, Barker RA, Brayne C (2008) Incidence of early-onset dementias in Cambridgeshire, United Kingdom. Neurology 71:1496–1499PubMedCrossRefGoogle Scholar
  21. Mesulam M, Wicklund A, Johnson N, Rogalski E, Leger GC, Rademaker A et al (2008) Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 63:709–719PubMedCrossRefGoogle Scholar
  22. Nath U, Ben-Shlomo Y, Thomson RG, Morris HR, Wood NW, Lees AJ et al (2001) The prevalence of progressive supranuclear palsy (Steele–Richardson–Olszewski syndrome) in the UK. Brain 124:1438–1449PubMedCrossRefGoogle Scholar
  23. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S et al (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51:1546–1554PubMedGoogle Scholar
  24. Rascovsky K, Salmon DP, Lipton AM, Leverenz JB, DeCarli C, Jagust WJ et al (2005) Rate of progression differs in frontotemporal dementia and Alzheimer disease. Neurology 65:397–403PubMedCrossRefGoogle Scholar
  25. Rascovsky K, Hodges JR, Kipps CM, Johnson JK, Seeley WW, Mendez MF et al (2007) Diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD): current limitations and future directions. Alzheimer Dis Assoc Disord 21:S14–S18PubMedCrossRefGoogle Scholar
  26. Rascovsky K, Hodges JR, Knopman D, Mendez M, Kramer JH, Van Swieten JC et al (2011) Sensitivity of diagnostic criteria for autopsy-confirmed patients with behavioral variant frontotemporal dementia (bvFTD): first report of the international bvFTD criteria consortium (FTDC). Neurology 76(Suppl 4):A262–A263Google Scholar
  27. Ratnavalli E, Brayne C, Dawson K, Hodges JR (2002) The prevalence of frontotemporal dementia. Neurology 58:1615–1621PubMedGoogle Scholar
  28. Roberson ED, Hesse JH, Rose KD, Slama H, Johnson JK, Yaffe K et al (2005) Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology 65:719–725PubMedCrossRefGoogle Scholar
  29. Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y et al (2003) Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain 126:2016–2022PubMedCrossRefGoogle Scholar
  30. The Lund and Manchester Groups (1994) Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 57:416–418CrossRefGoogle Scholar
  31. Xie SX, Forman MS, Farmer J, Moore P, Wang Y, Wang X et al (2008) Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration. J Neurol Neurosurg Psychiatry 79:126–129PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Neurology, College of MedicineMayo ClinicRochesterUSA
  2. 2.Department of Health Sciences ResearchMayo ClinicRochesterUSA

Personalised recommendations