Frontotemporal Dementia

전두측두엽 치매

  • Jun, Byoung Sun (Department of Psychiatry, Jeju National University Medical School and Jeju National University Hospital) ;
  • Park, Joon Hyuk (Department of Psychiatry, Jeju National University Medical School and Jeju National University Hospital)
  • 전병선 (제주대학교 의학전문대학원 제주대학교병원 정신건강의학교실) ;
  • 박준혁 (제주대학교 의학전문대학원 제주대학교병원 정신건강의학교실)
  • Received : 2016.04.07
  • Accepted : 2016.06.03
  • Published : 2016.08.31

Abstract

Frontotemporal dementia (FTD) is a degenerative disease characterized by the selective frontal and temporal lobe atrophy, and progressive deficits in behavior, executive function, or language. The prevalence and incidence of FTD are 15-22/100000 and 2.7-4.1/100000, respectively, in midlife. Hereditary is an important risk factor for FTD. Although there is some controversy regarding the further syndromatic subdivision of the different types of FTD, FTD is clinically classified into behavioral variant of frontotemporal dementia, semantic dementia and progressive nonfluent aphasia. FTD can be misdiagnosed as many psychiatric disorders because of similarity of the prominent behavioral features. Advances in clinical, imaging, and molecular characterization have increased the accuracy of FTD diagnosis, thus developing for the accurate differentiation of these syndromes from psychiatric disorders. We also discuss about therapeutic strategies for symptom management of FTD. Medications such as serotonin reuptake inhibitors, antipsychotics, and other novel treatments have been used in FTD with various rates of success. Further advanced research should be directed at understanding and developing new diagnostic and therapeutic modalities to improve the FTD patients' prognosis and quality of life.

Keywords

References

  1. Vieira RT, Caixeta L, Machado S, Silva AC, Nardi AE, Arias-Carrion O, et al. Epidemiology of early-onset dementia: a review of the literature. Clin Pract Epidemiol Ment Health 2013;9:88-95. https://doi.org/10.2174/1745017901309010088
  2. Pick A. Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Med Wschr 1892;17:15-17.
  3. Mesulam MM. Slowly progressive aphasia without generalized dementia. Ann Neurol 1982;11:592-598. https://doi.org/10.1002/ana.410110607
  4. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51:1546-1554. https://doi.org/10.1212/WNL.51.6.1546
  5. Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology 2006;66:41-48. https://doi.org/10.1212/01.wnl.0000191307.69661.c3
  6. Mackenzie IR, Neumann M, Bigio EH, Cairns NJ, Alafuzoff I, Kril J, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol 2010;119:1-4. https://doi.org/10.1007/s00401-009-0612-2
  7. WHO. Media centre. Dementia fact sheet [updated 2016 April; cited 2016 May 23]. Available from: http://www.who.int/mediacentre/factsheets/fs362/en/.
  8. Snowden JS, Neary D, Mann DM. Frontotemporal dementia. Br J Psychiatry 2002;180:140-143. https://doi.org/10.1192/bjp.180.2.140
  9. Onyike CU, Diehl-Schmid J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry 2013;25:130-137. https://doi.org/10.3109/09540261.2013.776523
  10. Kim KW, Park JH, Kim MH, Kim MD, Kim BJ, Kim SK, et al. A nationwide survey on the prevalence of dementia and mild cognitive impairment in South Korea. J Alzheimers Dis 2011;23:281-291. https://doi.org/10.3233/JAD-2010-101221
  11. Knopman DS, Roberts RO. Estimating the number of persons with frontotemporal lobar degeneration in the US population. J Mol Neurosci 2011;45:330-335. https://doi.org/10.1007/s12031-011-9538-y
  12. Shinagawa S,Miller BL. Frontotemporal Dementia. In: Rosenberg P, Pascual JM, editors. Rosenberg's Molecular and Genetic Basis of Neurological and Psychiatric Disease. 5th ed. London: Academic Press;2014. p.779-791.
  13. Lee KJ. Clinical Features and Therapeutic Approaches of Frontotemporal Dementia. J Korean Geriatr Psychiatry 2012;16:67-74.
  14. Bang J, Spina S, Miller BL. Frontotemporal dementia. Lancet 2015;386:1672-1682. https://doi.org/10.1016/S0140-6736(15)00461-4
  15. Liu W, Miller BL, Kramer JH, Rankin K, Wyss-Coray C, Gearhart R, et al. Behavioral disorders in the frontal and temporal variants of frontotemporal dementia. Neurology 2004;62:742-748. https://doi.org/10.1212/01.WNL.0000113729.77161.C9
  16. Miller BL, Boeve BF. The behavioral neurology of dementia. Cambridge: Cambridge University Press;2009.
  17. Gregory CA, Serra-Mestres J, Hodges JR. Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests? Neuropsychiatry Neuropsychol Behav Neurol 1999;12:128-135.
  18. Rosen HJ, Gorno-Tempini ML, Goldman WP, Perry RJ, Schuff N, Weiner M, et al. Patterns of brain atrophy in frontotemporal dementia and semantic dementia. Neurology 2002;58:198-208. https://doi.org/10.1212/WNL.58.2.198
  19. Rabinovici GD, Seeley WW, Kim EJ, Gorno-Tempini ML, Rascovsky K, Pagliaro TA, et al. Distinct MRI atrophy patterns in autopsyproven Alzheimer's disease and frontotemporal lobar degeneration. Am J Alzheimers Dis Other Demen 2007-2008;22:474-488. https://doi.org/10.1177/1533317507308779
  20. Davies RR, Kipps CM, Mitchell J, Kril JJ, Halliday GM, Hodges JR. Progression in frontotemporal dementia: identifying a benign behavioral variant by magnetic resonance imaging. Arch Neurol 2006;63:1627-1631. https://doi.org/10.1001/archneur.63.11.1627
  21. Hodges JR. Frontotemporal dementia (Pick's disease): clinical features and assessment. Neurology 2001;56(11 Suppl 4):S6-S10. https://doi.org/10.1212/WNL.56.suppl_4.S6
  22. Seeley WW, Bauer AM, Miller BL, Gorno-Tempini ML, Kramer JH, Weiner M, et al. The natural history of temporal variant frontotemporal dementia. Neurology 2005;64:1384-1390. https://doi.org/10.1212/01.WNL.0000158425.46019.5C
  23. Josephs KA, Duffy JR, Strand EA, Whitwell JL, Layton KF, Parisi JE, et al. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Brain 2006;129(Pt 6):1385-1398. https://doi.org/10.1093/brain/awl078
  24. Rosen HJ, Allison SC, Ogar JM, Amici S, Rose K, Dronkers N, et al. Behavioral features in semantic dementia vs other forms of progressive aphasias. Neurology 2006;67:1752-1756. https://doi.org/10.1212/01.wnl.0000247630.29222.34
  25. Le Ber I, Guedj E, Gabelle A, Verpillat P, Volteau M, Thomas-Anterion C, et al. Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal. Brain 2006;129(Pt 11):3051-3065. dementia. https://doi.org/10.1093/brain/awl288
  26. Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology 1996;4:1-9.
  27. Woolley JD, Khan BK, Murthy NK, Miller BL, Rankin KP. The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. J Clin Psychiatry 2011;72:126-133. https://doi.org/10.4088/JCP.10m06382oli
  28. Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, et al. Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006-1014. https://doi.org/10.1212/WNL.0b013e31821103e6
  29. Claassen DO, Parisi JE, Giannini C, Boeve BF, Dickson DW, Josephs KA. Frontotemporal dementia mimicking dementia with Lewy bodies. Cogn Behav Neurol 2008;21:157-163. https://doi.org/10.1097/WNN.0b013e3181864a09
  30. Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134(Pt 9):2456-2477. https://doi.org/10.1093/brain/awr179
  31. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. Washington, DC: American Psychiatric Publishing;2013. p.614-616.
  32. Zhang Y, Schuff N, Du AT, Rosen HJ, Kramer JH, Gorno-Tempini ML, et al. White matter damage in frontotemporal dementia and Alzheimer's disease measured by diffusion MRI. Brain 2009;132(Pt 9):2579-2592. https://doi.org/10.1093/brain/awp071
  33. Agosta F, Scola E, Canu E, Marcone A, Magnani G, Sarro L, et al. White matter damage in frontotemporal lobar degeneration spectrum. Cereb Cortex 2012;22:2705-2714. https://doi.org/10.1093/cercor/bhr288
  34. Seeley WW, Crawford RK, Zhou J, Miller BL, Greicius MD. Neurodegenerative diseases target large-scale human brain networks. Neuron 2009;62:42-52. https://doi.org/10.1016/j.neuron.2009.03.024
  35. Xia CF, Arteaga J, Chen G, Gangadharmath U, Gomez LF, Kasi D, et al. [(18)F]T807, a novel tau positron emission tomography imaging agent for Alzheimer's disease. Alzheimers Dement 2013;9:666-676. https://doi.org/10.1016/j.jalz.2012.11.008
  36. Maruyama M, Shimada H, Suhara T, Shinotoh H, Ji B, Maeda J, et al. Imaging of tau pathology in a tauopathy mouse model and in Alzheimer patients compared to normal controls. Neuron 2013;79:1094-1108. https://doi.org/10.1016/j.neuron.2013.07.037
  37. Broe M, Hodges JR, Schofield E, Shepherd CE, Kril JJ, Halliday GM. Staging disease severity in pathologically confirmed cases of frontotemporal dementia. Neurology 2003;60:1005-1011. https://doi.org/10.1212/01.WNL.0000052685.09194.39
  38. Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, et al. Selective frontoinsular von economo neuron and fork cell loss in early behavioral variant frontotemporal dementia. Cereb Cortex 2016;26:1843. https://doi.org/10.1093/cercor/bhw012
  39. Sieben A, Van Langenhove T, Engelborghs S, Martin JJ, Boon P, Cras P, et al. The genetics and neuropathology of frontotemporal lobar degeneration. Acta Neuropathol 2012;124:353-372. https://doi.org/10.1007/s00401-012-1029-x
  40. Dickson DW, Hauw JJ, Agid Y, Litvan I. Progressive supranuclear palsy and corticobasal degeneration. In: Dickson DW, Weller Ro, editors. Neurodegeneration: The molecular pathology of dementia and movement disorders. Chichester: Wiley-Blackwell;2011. p.135-155.
  41. Boxer AL, Geschwind MD, Belfor N, Gorno-Tempini ML, Schauer GF, Miller BL, et al. Patterns of brain atrophy that differentiate corticobasal degeneration syndrome from progressive supranuclear palsy. Arch Neurol 2006;63:81-86. https://doi.org/10.1001/archneur.63.1.81
  42. Josephs KA, Hodges JR, Snowden JS, Mackenzie IR, Neumann M, Mann DM, et al. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 2011;122:137-153. https://doi.org/10.1007/s00401-011-0839-6
  43. Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, et al. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology 2010;75:2204-2211. https://doi.org/10.1212/WNL.0b013e318202038c
  44. Mackenzie IR, Foti D, Woulfe J, Hurwitz TA. Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 2008;131(Pt 5):1282-1293. https://doi.org/10.1093/brain/awn061
  45. Borroni B, Alberici A, Archetti S, Magnani E, Di Luca M, Padovani A. New insights into biological markers of frontotemporal lobar degeneration spectrum. Curr Med Chem 2010;17:1002-1009. https://doi.org/10.2174/092986710790820651
  46. Le Ber I. Genetics of frontotemporal lobar degeneration: an up-date and diagnosis algorithm. Rev Neurol (Paris) 2013;169:811-819. https://doi.org/10.1016/j.neurol.2013.07.014
  47. Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, et al. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 1998;393:702-705. https://doi.org/10.1038/31508
  48. Rohrer JD, Lashley T, Schott JM, Warren JE, Mead S, Isaacs AM, et al. Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. Brain 2011;134(Pt 9):2565-2581. https://doi.org/10.1093/brain/awr198
  49. Seelaar H, Kamphorst W, Rosso SM, Azmani A, Masdjedi R, de Koning I, et al. Distinct genetic forms of frontotemporal dementia. Neurology 2008;71:1220-1226. https://doi.org/10.1212/01.wnl.0000319702.37497.72
  50. Chen-Plotkin AS, Martinez-Lage M, Sleiman PM, Hu W, Greene R, Wood EM, et al. Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration. Arch Neurol 2011;68:488-497. https://doi.org/10.1001/archneurol.2011.53
  51. Whitwell JL, Weigand SD, Boeve BF, Senjem ML, Gunter JL, DeJesus-Hernandez M, et al. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain 2012;135(Pt 3):794-806. https://doi.org/10.1093/brain/aws001
  52. Bei Hu, Ross L, Neuhaus J, Knopman D, Kramer J, Boeve B, et al. Off-label medication use in frontotemporal dementia. Am J Alzheimers Dis Other Demen 2010;25:128-133. https://doi.org/10.1177/1533317509356692
  53. Swartz JR, Miller BL, Lesser IM, Darby AL. Frontotemporal dementia: treatment response to serotonin selective reuptake inhibitors. J Clin Psychiatry 1997;58:212-216. https://doi.org/10.4088/JCP.v58n0506
  54. Lebert F, Stekke W, Hasenbroekx C, Pasquier F. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004;17:355-359. https://doi.org/10.1159/000077171
  55. Moretti R, Torre P, Antonello RM, Cazzato G, Griggio S, Bava A. Olanzapine as a treatment of neuropsychiatric disorders of Alzheimer's disease and other dementias: a 24-month follow-up of 68 patients. Am J Alzheimers Dis Other Demen 2003;18:205-214. https://doi.org/10.1177/153331750301800410
  56. U.S. Food and Drug Administration. Information for healthcare professionals: conventional antipsychotics. June 16, 2008 [cited 2014 Nov 10]. Available from: http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformationforPatientsandProviders/ucm124830.htm.
  57. Huey ED, Putnam KT, Grafman J. A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia. Neurology 2006;66:17-22. https://doi.org/10.1212/01.wnl.0000191304.55196.4d
  58. Moretti R, Torre P, Antonello RM, Cattaruzza T, Cazzato G, Bava A. Rivastigmine in frontotemporal dementia: an open-label study. Drugs Aging 2004;21:931-937. https://doi.org/10.2165/00002512-200421140-00003
  59. Mendez MF, Shapira JS, McMurtray A, Licht E. Preliminary findings: behavioral worsening on donepezil in patients with frontotemporal dementia. Am J Geriatr Psychiatry 2007;15:84-87. https://doi.org/10.1097/01.JGP.0000231744.69631.33
  60. Boxer AL, Knopman DS, Kaufer DI, Grossman M, Onyike C, Graf-Radford N, et al. Memantine in patients with frontotemporal lobar degeneration: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2013;12:149-156. https://doi.org/10.1016/S1474-4422(12)70320-4
  61. Sanders DW, Kaufman SK, DeVos SL, Sharma AM, Mirbaha H, Li A, et al. Distinct tau prion strains propagate in cells and mice and define different tauopathies. Neuron 2014;82:1271-1288. https://doi.org/10.1016/j.neuron.2014.04.047
  62. Cenik B, Sephton CF, Dewey CM, Xian X, Wei S, Yu K, et al. Suberoylanilide hydroxamic acid (vorinostat) up-regulates progranulin transcription: rational therapeutic approach to frontotemporal dementia. J Biol Chem 2011;286:16101-16108. https://doi.org/10.1074/jbc.M110.193433
  63. Merrilees J. A model for management of behavioral symptoms in frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord 2007;21:S64-S69. https://doi.org/10.1097/WAD.0b013e31815bf774