Dementia and Geriatric Cognitive Disorders

Original Research Article

Dementia in First-Degree Relatives of Patients with Frontotemporal Dementia

A Family History Study

Gräsbeck A.a,b · Horstmann V.c · Nilsson K.a · Sjöbeck M.d · Sjöström H.a · Gustafson L.a

Author affiliations

aDepartment of Psychogeriatrics, bSwedish National Institute of Health Care and Sciences, cUnit of Gerontology and Care for the Elderly and dDepartment of Pathology, Lund University, Lund, Sweden

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Dement Geriatr Cogn Disord 2005;19:145–153

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Article / Publication Details

First-Page Preview
Abstract of Original Research Article

Received: June 18, 2004
Published online: February 11, 2005
Issue release date: February 2005

Number of Print Pages: 9
Number of Figures: 3
Number of Tables: 4

ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)

For additional information: https://www.karger.com/DEM

Abstract

Several studies have found a clustering of dementia in relatives of patients with frontotemporal dementia (FTD). This study analysed the familial aggregation of FTD specifically as well as the occurrence of dementia in general in first-degree relatives of patients with FTD. A family history study was carried out on 478 first-degree relatives of 74 index patients suffering from FTD. Cases of organic dementia and of FTD were diagnosed according to internationally accepted diagnostic criteria. Age- and sex-specific incidences of organic dementia and of FTD were calculated as was the proportion of FTD in relation to organic dementia in general; comparisons with clinical and population studies were made. There was a tenfold increase in the incidence of FTD in the first-degree relatives of FTD patients compared with the incidence of FTD in a population study. The proportion of FTD in relation to all types of organic dementia was much higher in relatives of FTD patients compared to the corresponding proportions in clinical and population-based studies. There was a small, non-significant difference between the present family history study and the population studies as regards the incidence of organic dementia. The findings suggest that hereditary and/or shared environmental factors are strongly involved in the aetiology of FTD. There were no indications of familial clustering of organic dementia in general in relatives of FTD patients.

© 2005 S. Karger AG, Basel




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References

  1. Brun A, Englund E, Gustafson L, Passant U, Mann D, Neary D, Snowden J: Consensus on clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry 1994;57:416–418.
  2. Foster, NL, Wilhelmsen K, Sima AF, Jones MZ, D’Amato CJ, Gilman S, Conference Participants: Frontotemporal dementia and parkinsonism linked to chromosome 17: A consensus conference. Ann Neurol 1997:41:706–715.
  3. Neary D, Snowden JS, Gustafson L, et al: Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology 1998;51:1546–1554.
  4. European Concerted Action on Pick’s Disease (ECAPD) Consortium. Provisional clinical and neuroradiological criteria for the diagnosis of Pick’s disease. Eur J Neurol 1998:5:519–520.
    External Resources
  5. McKhann GM, Albert MS, Grossman M, Miller B, Dickson D, Trojanowski JQ: Clinical and pathological diagnosis of frontotemporal dementia. Report of the work group on frontotemporal dementia and Pick’s disease. Arch Neurol 2001;58:1803–1809.
  6. Rosso SM, Donker Kaat L, 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, Heutnik P, van Swieten JC: Frontotemporal dementia in the Netherlands: Patient characteristics and prevalence estimates from a population-based study. Brain 2003;126:2016–2022.
  7. Ratnavalli E, Brayne C, Dawson K, Hodges JR: The prevalence of frontotemporal dementia. Neurology 2002;58:1615–1621.
  8. Gislason TB, Sjögren M, Larsson L, Skoog I: 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 2003;74:867–871.
  9. Andreasen N, Blennow K, Sjödin C, et al: Prevalence and incidence of clinically diagnosed memory impairments in a geographically defined general population in Sweden. Neuroepidemiology 1999;18:144–155.
  10. Sanders J, Schenk VWD, Van Veen P: A family with Pick’s disease. Verhandelingen der Koninklijke Nederlandsche Akademie van Wetenschappen 1939, section 2, part 38, no 3:1–124.
  11. Schenk VWD: Re-examination of a family with Pick’s disease. Ann Hum Gen 1959;23:325–333.
  12. Gustafson L: Frontal lobe degeneration of non-Alzheimer type. 2. Clinical picture and differential diagnosis. Arch Gerontol Geriatr 1987;6:209–223.
  13. Knopman DS, Mastri AR, Frey WH, Sung JH, Rustan T: Dementia lacking distinctive histologic features: A common non-Alzheimer degenerative dementia. Neurology 1990;40:251–256.
  14. Passant U, Gustafson L, Brun A: Spectrum of frontal lobe dementia in a Swedish family. Dementia 1993;4:160–162.
  15. Stevens M, van Duijn CM, Kamphorst W: Familial aggregation in frontotemporal dementia. Neurology 1998;50:1541–1545.
  16. Neary D: Classification of the dementias. Rev Clin Gerontol 1999;9:55–64.
    External Resources
  17. Wilhelmsen KC, Lynch T, Pavlou E, Higgins M, Nygaard TG: Localization of disinhibition-dementia-parkinsonism-amytrophy complex to 17q21–22. Am J Hum Genet 1994;55:1159–1165.
  18. Heutink P, Stevens M, Rizzu P, Bakker E, Kros JM, Tibben A, Niermeijer MR, Van Duijn CM, Oostra BA, Van Swieten JC: Hereditary frontotemporal dementia is linked to chromosome 17q21-q22: A genetic and clinico-pathological study of three Dutch families. Ann Neurol 1997;41:150–159.
  19. Hong M, Zhukareva V, Vogelsber-Ragaglia V, Wszolek Z, Reed L, Miller BL, Geschwind DH, Bird TD, McKeel D, Goate A, Morris JC, Wilhelmsem KC, Schellenberg GD, Trojanowski JQ, Lee VM: Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. Science 1998;282:1914–1917.
  20. Hutton M, Lendom CL, Rizzu P, Baker M, Froelich S, Houlden H, Pickering-Brown S, Chakraverty S, Isaacs A, Grover A, Hackett J, Adamson J, Lincoln S, Dickson D, Davies P, Petersen RC, Stevens M, de Graaff E, Wauters E, van Baren J, Hillebrand M, Joosse M, Kwon JM, Nowotny P, Heutnik P, et al: Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 1998;393:702–705.
  21. Hosler B, Siddique T, Sapp P, Sailor W, Huang MC, Hossain A, Daube JR, Nance M, Fan C, Kaplan J, Hung WY, McKenna-Yasek D, Haines JL, Pericak-Vance MA, Horvitz HR, Brown RH Jr: Linkage of familial amyotrophic lateral sclerosis with frontotemporal dementia to chromosome 9q21-q22. JAMA 2000;284:1664–1669.
  22. Gydesen S, Brown JM, Brun A, Chakrabati L, Gade A, Johannsen P, Rossor M, Thusgaard T, Grove A, Yancopoulou D, Spillantini MG, Fisher EM, Collinge J, Sorensen SA: Chromosome 3 linked frontotemporal dementia (FTD-3). Neurology 2002;59:1585–1594.
  23. Rosso SM, van Swieten JC: New developments in frontotemporal dementia and parkinsonism linked to chromosome 17. Curr Opin Neurol 2002;15:423–428.
  24. Froelich-Fabre S: Genetic studies of frontotemporal dementia; thesis, Stockholm, 2001.
  25. Morris HR, Kahn N, Janssen JC, Brown JM, Perez-Tur J, Baker M, Ozansoy M, Hardy J, Hutton M, Wood NW, Lees AJ, Revesz T, Lantos P, Rossor M: The genetic and pathological classification of familial frontotemporal dementia. Arch Neurol 2001;58:1813–1816.
  26. Poorkaj P, Grossman M, Steinbart E, Payami H, Sadovnick A, Nochlin D, Tabira T, Trojanowski JQ, Borson S, Galasko D, Reich S, Quinn B, Schellenberg G, Bird TD: Frequency of tau gene mutations in familial and sporadic cases of non-Alzheimer dementia. Arch Neurol 2001;58:351–352.
  27. Tolnay M, Probst A: Frontotemporal lobar degeneration – Tau as a pied piper? Neurogenetics 2002;4:63–75.
  28. Ostojic J, Axelman K, Lannfelt L, Froelich-Fabre S: No evidence of linkage to chromosome 9q21–22 in a Swedish family with frontotemporal dementia and amyotrophic lateral sclerosis. Neurosci Lett. 2003;340:245–247.
  29. Zekanowski C, Peplonska B, Styczynska M, Gustaw K, Kuznicki J, Barcikowska M: Mutation screening of the MAPT and STH genes in Polish patients with clinically diagnosed frontotemporal dementia. Dement Geriatr Cogn Disord 2003;16:126–131.
  30. World Health Organization: International Statistical Classification of Diseases and Related Health Problems, ed 10. Geneva, World Health Organization, 1992.
  31. Gräsbeck A, Horstmann V, Englund E, Passant U, Gustafson L: Evaluation of predictors of mortality on frontotemporal dementia. J Geriatr Psychiatry 2003;18:586–593.
  32. Andreasen N, Endicott J, Spitzer L, Winokur G: The family history method using diagnostic criteria. Reliability and validity. Arch Gen Psychiatry 1977;34:1229–1235.
  33. Endicott J, Andreasen N, Spitzer RL: Family History-Research Diagnostic Criteria. New York, Biometrics Research, New York State Psychiatric Institute, 1975.
  34. Hachinski VF, Iliff LD, Zilhka E, du Boulay GH, McAllister VL, Marshall J, Ross Russel RW, Symon L: Cerebral blood flow in dementia. Arch Neurol 1975;32:632–637.
  35. Gustafson L, Nilsson L: Differential diagnosis of presenile dementia on clinical grounds. Acta Psychiatr Scand 1982;65:194–209.
  36. Brun A, Gustafson L: The Lund longitudinal dementia study: A 25-year perspective on neuropathology, differential diagnosis and treatment; in Corain B, Nicolini M, Winblad B, Wisniewski H, Zatta P (eds): Alzheimer’s Disease: Advance in Clinical and Basic Research. New York, Wiley, 1993, pp 4–18.
  37. Barber R, Snowden JS, Craufurd D: Frontotemporal dementia and Alzheimer’s disease: Retrospective differentiation using information from informants. J Neurol Neurosurg Psychiatry 1995;59:61–70.
  38. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, ed 3. Washington, American Psychiatric Association, 1980.
  39. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, ed 3 revised. Washington, American Psychiatric Association, 1987.
  40. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, ed 4. Washington, American Psychiatric Association, 1994.
  41. Hagnell O, Lanke J, Öjesjö L, Rorsman B: The Incidence of Mental Illness over a Quarter of a Century. Stockholm, Almqvist & Wicksell International, 1990.
  42. Fratiglioni L, Launer LJ, Andersen K, Breteler MMB, Copeland JRM, Dartigues J-F, Lobo A, Martinez-Lage J, Soininen H, Hofman A, for the Neurologic Diseases in the Elderly Research Group: Incidence of dementia and major subtypes in Europe: A collaborative study of population-based cohorts. Neurology 2000:54(suppl 5):10–15.
  43. Neary D, Snowden JS, Northen B, Goulding P: Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry 1988;51:353–361.
  44. Giannakopoulos P, Hof PR, Bouras C: Dementia lacking distinctive histopathology: Clinicopathological evaluation of 32 cases. Acta Neuropathol 1995;89:346–355.
  45. Barker WW, Luis CA, Kashuba A, Luis M, Harwood DG, Lowensten D, Waters C, Jimison P, Shepherd E, Sevush S, Graff-Radford N, Newland D, Todd M, Miller B, Gold M, Heilman K, Doty L, Goodman I, Robinson B, Pearl G, Dickson D, Duara R: Relative frequencies of Alzheimer’s disease, Lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the State of Florida Bank. Alzheimer Dis Assoc Disord 2002;16:203–212.
  46. Hokoishi K, Ikeda M, Maki N, Nebu A, Shigenobu K, Fukuhara R, Komori K, Tanabe H: Frontotemporal lobar degeneration: A study in Japan. Dement Geriatr Cogn Disord 2001;12:393–399.
  47. Stevens T, Livingston G, Kitchen G, Manela M, Walker Z, Katona C: Islington study of dementia subtypes in the community. Br J Psychiatry 2002;180:270–276.
  48. Varma AR, Snowden JS, Lloyd JJ, Talbot PR, Mann DMA, Neary D: Evaluation of the NINCDS-ADRDA criteria in the differentiation of Alzheimer’s disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry 1999;66:184–188.
  49. Faraone SV, Tsuang MT: Methods in psychiatric genetics; in Tsuang MT, Tohen M, Zahner GEP (eds): Textbook in Psychiatric Epidemiology. New York, Wiley, 1995, pp 81–134.
  50. Silverman JM, Smith CJ, Marin DB, Mohs RC, Propper CB: Familial patterns of risk in very late-onset Alzheimer disease. Arch Gen Psychiatry 2003;60:190–197.
  51. Sjogren T, Sjogren H, Lindgren AG: Morbus Alzheimer and morbus Pick; a genetic, clinical and patho-anatomical study. Acta Psychiatr Neurol Scand 1952;82:1–152.
  52. Gustafson L: Frontal lobe degeneration of non-Alzheimer type. 2. Clinical picture and differential diagnosis. Clinical picture of frontal lobe degeneration of non-Alzheimer type. Dementia 1993;4:3–4.
    External Resources
  53. Andreasen N, Rice J, Endicott J, Reich T, Coryell W: The family history approach to diagnosis: How useful is it? Arch Gen Psychiatry 1986;43:421–429.
  54. Rorsman B, Hagnell O, Lanke J: Prevalence and incidence of senile and multi-infarct dementia in the Lundby study: A comparison between the time periods 1947–1957 and 1957–1972. Neuropsychobiology 1986;15:122–129.
  55. Hagnell O, Franck A, Gräsbeck A, Öhman R, et al: Senile dementia of the Alzheimer type in the Lundby study. 1. A prospective, epidemiological study of incidence and risk during the 15 years 1957–1972. Eur Arch Psychiatry Clin Neurosci 1991;241:159–164.
  56. Henderson AS, Jorm AF, Mackinnon A, Christensen H, Scott LR, Korten AE, Doyle C: A survey of dementia in the Canberra population: Experience with ICD-10 and DSM-III-R criteria. Psychol Med 1994;24:473–482.
  57. Erkinjuntti T, Østbye T, Steenhus R, Hachinski V: The effect of different diagnostic criteria on the prevalence of dementia. N Engl J Med 1997;337:1667–1674.

Article / Publication Details

First-Page Preview
Abstract of Original Research Article

Received: June 18, 2004
Published online: February 11, 2005
Issue release date: February 2005

Number of Print Pages: 9
Number of Figures: 3
Number of Tables: 4

ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)

For additional information: https://www.karger.com/DEM


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