Skip to main content
SpringerLink
Log in

Major or Mild Frontotemporal Neurocognitive Disorder

  • Chapter
  • First Online:
Geriatric Psychiatry

  • 2484 Accesses

Abstract

Frontotemporal neurocognitive disorder (NCD) constitutes the leading form of NCD in patients younger than age 65. It can lead to the behavioral-variant frontotemporal NCD or pure cognitive changes, as in primary progressive aphasia. Although patients with behavioral variant have relatively preserved memory and language function, they display major deficits in insight, judgment, working memory, and executive functions. Patients with primary progressive aphasia most commonly have difficulty with naming and/or word finding. Other common presenting symptoms include hesitancy or nonfluent speech production, motor speech abnormalities (such as dysarthria), impaired language comprehension, and repetition. Primary progressive aphasia has been divided into three separate subsyndromes: nonfluent/agrammatic, semantic, and logopenic variants. The clinical presentation is determined by the anatomical areas involved and the pathognomonic atrophy. An accurate clinical diagnosis helps to predict probable neuropathology, which is essential for targeted treatment planning. In this chapter, we review the primary symptomatology of these frontotemporal NCD variants, diagnostic and treatment challenges, and comorbidity complexity that needs to be considered in differential diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  1. American Psychiatric Association. Diagnostic and statistical manual of mental disorders. 5th ed. Arlington: American Psychiatric Publishing; 2013. p. 614–5.

    Book  Google Scholar 

  2. World Health Organisation. ICD-10 Classifications of Mental and Behavioural Disorder. Clinical descriptions and diagnostic guidelines. Geneva: World Health Organization; 1992.

    Google Scholar 

  3. Kirshner HS. Frontotemporal dementia and primary progressive aphasia, a review. Neuropsychiatr Dis Treat. 2014;10:1045–55.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Seltman RE, Matthews BR. Frontotemporal lobar degeneration: epidemiology, pathology, diagnosis and management. CNS Drugs. 2012;26(10):841–70.

    Article  CAS  PubMed  Google Scholar 

  5. Knopman DS, Roberts RO. Estimating the number of persons with frontotemporal lobar degeneration in the US population. J Mol Neurosci. 2011;45:330–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Baborie A, Griffiths TD, Jaros E, et al. Frontotemporal dementia in elderly individuals. Arch Neurol. 2012;69(8):1052–60.

    Article  PubMed  Google Scholar 

  7. Onyike CU, Diehl-Schmid J. The epidemiology of frontotemporal dementia. Int Rev Psychiatry. 2013;25(2):130–7.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Bird TD, Knopman D, van Swieten J, et al. Epidemiology and genetics of frontotemporal dementia/Pick’s disease. Ann Neurol. 2003;54(5, Suppl):S29–31.

    Article  PubMed  Google Scholar 

  9. Goldman JS, Farmer J, Wood EM, et al. Comparison of family histories in FTLD subtypes and related taupathies. Neurology. 2005;65:1817–9.

    Article  CAS  PubMed  Google Scholar 

  10. Rademakers R, Eriksen JL, Baker M, et al. Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia. Hum Mol Genet. 2008;17:3631–42.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. Brain. 2011;134:2456–77.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Gorno-Tempini ML, Hillis AE, Weintraub S, et al. Classification of primary progressive aphasia and its variants. Neurology. 2011;76:1006–14.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Mesulam MM, Wieneke C, Thompson C, Rogalski E, Weintraub S. Quantitative classification of primary progressive aphasia at early and mild impairment stages. Brain. 2012;135(Pt 5):1537–53.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Freitas S, Simões MR, Alves L, et al. Montreal Cognitive Assessment (MoCA): validation study for frontotemporal dementia. J Geriatr Psychiatry Neurol. 2012;25(3):146–54.

    Google Scholar 

  15. Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: a frontal assessment battery at bedside. Neurology. 2000;55(11):1621–6.

    Article  CAS  PubMed  Google Scholar 

  16. Manoochehri M, Huey ED. Diagnosis and management of behavioral issues in frontotemporal dementia. Curr Neurol Neurosci Rep. 2012;12(5):528–36.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. De Deyn PP, Engelborghs S, Saerens J, et al. The Middelheim Frontality score: a behavioural assessment scale that discriminates frontotemporal dementia from Alzheimer’s disease. Int J Geriatr Psychiatry. 2005;20:70–9.

    Article  PubMed  Google Scholar 

  18. Fastenau PS, Denburg NL, Mauer BA. Parallel short forms for the Boston Naming Test: psychometric properties and norms for older adults. J Clin Exp Neuropsychol. 1998;20(6):828–34.

    Article  CAS  PubMed  Google Scholar 

  19. Bora E, Velakoulis D, Walterfang M. Meta-analysis of facial emotion recognition in behavioral variant frontotemporal dementia: comparison with Alzheimer disease and healthy controls. J Geriatr Psychiatry Neurol. 2016;29(4):205–11.

    Article  PubMed  Google Scholar 

  20. Trzepacz PT, Hochstetler H, Wang S, Walker B, Saykin AJ, Alzheimer’s Disease Neuroimaging Initiative. Relationship between the Montreal Cognitive Assessment and Mini-mental State Examination for assessment of mild cognitive impairment in older adults. BMC Geriatr. 2015;15:107. https://doi.org/10.1186/s12877–015–0103–3.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Burnett J, Dyer CB, Naik AD. Convergent validation of the Kohlman Evaluation of Living Skills as a screening tool of older adults’ ability to live safely and independently in the community. Arch Phys Med Rehabil. 2009;90(11):1948–52.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Hornberger M, Piguet O, Graham AJ, Nestor PJ, Hodges JR. How preserved is episodic memory in behavioral variant frontotemporal dementia? Neurology. 2010;74(6):472–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Hutchinson AD, Mathias JL. Neuropsychological deficits in frontotemporal dementia and Alzheimer’s disease: a meta-analytic review. J Neurol Neurosurg Psychiatry. 2007;78(9):917–28.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Hogan DB, Bailey P, Carswell A, Clarke B, Cohen C, Forbes D, et al. Management of mild to moderate Alzheimer’s disease and dementia. Alzheimers Dement. 2007;3(4):355–84.

    Article  CAS  PubMed  Google Scholar 

  25. Whitwell JL, Weigand SD, Boeve BF, et al. Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics. Brain. 2012;135(3):794–806.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Warren JD, Rohrer JD, Rossor MN. Frontotemporal dementia. BMJ. 2013;347:f4827. https://doi.org/10.1136/bmj.f4827.

  27. Jicha GA, Nelson PT. Management of frontotemporal dementia: targeting symptom management in such a heterogeneous disease requires a wide range of therapeutic options. Neurodegener Dis Manag. 2011;1(2):141–56.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Lopez OL, Gonzalez MP, Becker JT, Reynolds CF, Sudilovsky A, DeKosky ST. Symptoms of depression and psychosis in Alzheimer’s disease and frontotemporal dementia. Neuropsychiatry Neuropsychol Behav Neurol. 1996;9:154–61.

    Google Scholar 

  29. Grossman M. The non-fluent/agrammatic variant of primary progressive aphasia. Lancet Neurol. 2012;11(6):545–55.

    Article  PubMed  PubMed Central  Google Scholar 

  30. Nardell M, Tampi RR. Pharmacological treatments for frontotemporal dementias: a systematic review of randomized controlled trials. Am J Alzheimers Dis Other Demen. 2014;29(2):123–32.

    Article  PubMed  Google Scholar 

  31. Ikeda M, Tanabe H, Horino T, et al. Care for patients with Pick’s disease—by using their preserved procedural memory. Seishin Shinkeigaku Zasshi. 1995;97:179–92.

    CAS  PubMed  Google Scholar 

  32. Merrilees J. A model for management of behavioral symptoms in frontotemporal lobar degeneration. Alzheimer Dis Assoc Disord. 2007;21(4):S64–9.

    Article  PubMed  Google Scholar 

  33. Brett L, Traynor V, Stapley P. Effects of physical exercise on health and well-being of individuals living with a dementia in nursing homes: a systematic review. J Am Med Dir Assoc. 2016;17(2):104–16.

    Article  PubMed  Google Scholar 

  34. Portugal Mda G, Marinho V, Laks J. Pharmacological treatment of frontotemporal lobar degeneration: systematic review. Rev Bras Psiquiatr. 2011;33(1):81–90.

    Article  PubMed  Google Scholar 

  35. 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(1):84–7.

    Google Scholar 

  36. Inouye SK, van Dyck CH, Alessi CA, et al. Clarifying confusion: the confusion assessment method. A new method for detection of delirium. Ann Intern Med. 1990;11:941–8.

    Article  Google Scholar 

  37. Hategan A, Bourgeois JA, Saperson K, Chiu S. The chief psychiatric complaints. In: Hategan A, Bourgeois JA, Hirsch CH, editors. On-call geriatric psychiatry: handbook of principles and practice. Cham: Springer; 2016.

    Chapter  Google Scholar 

  38. Wylie MA, Shnall A, Onyike CU, Huey ED. Management of frontotemporal dementia in mental health and multidisciplinary settings. Int Rev Psychiatry. 2013;25(2):230–6.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Chow TW, Binns MA, Cummings JL, et al. Apathy symptom profile and behavioral associations in frontotemporal dementia vs. Alzheimer’s disease. Arch Neurol. 2009;66(7):888–93.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Padala PR, Burke WJ, Bhatia SC, Petty F. Treatment of apathy with methylphenidate. J Neuropsychiatry Clin Neurosci. 2007;19(1):81–3.

    Article  CAS  PubMed  Google Scholar 

  41. Landes AM, Sperry SD, Strauss ME, Geldmacher DS. Apathy in Alzheimer’s disease. J Am Geriatr Soc. 2001;49(12):1700–7.

    Article  CAS  PubMed  Google Scholar 

  42. Mendez MF, Fras IA, Kremen SA, Tsai PH. False reports from patients with frontotemporal dementia: delusions or confabulations? Behav Neurol. 2011;24(3):237–44.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Alladi S, Xuereb J, Bak T, et al. Focal cortical presentations of Alzheimer’s disease. Brain. 2007;130:2636–45.

    Article  CAS  PubMed  Google Scholar 

  44. Budson AE, Solomon PR. New diagnostic criteria for Alzheimer’s disease and mild cognitive impairment for the practical neurologist. Pract Neurol. 2012;12(2):88–96.

    Article  PubMed  Google Scholar 

  45. Keable A, Fenna K, Yuen HM, et al. Deposition of amyloid β in the walls of human leptomeningeal arteries in relation to perivascular drainage pathways in cerebral amyloid angiopathy. Biochim Biophys Acta. 2016;1862(5):1037–46.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Schott JM, Warren JD. Alzheimer’s disease: mimics and chameleons. Pract Neurol. 2012;12(6):358–66.

    Article  PubMed  Google Scholar 

  47. Gauthier S, Patterson C, Chertkow H, et al. Recommendations of the 4th Canadian consensus conference on the diagnosis and treatment of dementia (CCCDTD4). Can Geriatr J. 2012;15(4):120–6.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Reus VI, Fochtmann LJ, Eyler AE, et al. APA’s practice guideline on the use of antipsychotics to treat agitation or psychosis in patients with dementia. Am J Psychiatry. 2016;173(5):543–6.

    Article  PubMed  Google Scholar 

  49. Bak TH. Motor neuron disease and frontotemporal dementia: one, two, or three diseases? Ann Indian Acad Neurol. 2010;13(Suppl2):S81–8.

    Article  PubMed  PubMed Central  Google Scholar 

  50. Hodges J. Familial frontotemporal dementia and amyotrophic lateral sclerosis associated with the C9ORF72 hexanucleotide repeat. Brain. 2012;135(3):652–5. https://doi.org/10.1093/brain/aws033

    Article  PubMed  Google Scholar 

  51. Mesulam M, Wieneke C, Rogalski E, Cobia D, Thompson C, Weintraub S. Quantitative template for subtyping primary progressive aphasia. Arch Neurol. 2009;66(12):1545–51.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Reed DA, Johnson NA, Thompson C, Weintraub S, Mesulam MM. A clinical trial of bromocriptine for treatment of primary progressive aphasia. Ann Neurol. 2004;56(5):750.

    Article  PubMed  Google Scholar 

  53. Effects of Tolcapone on Frontotemporal Dementia. ClinicalTrials.gov. Available at www.clinicaltrials.gov/ct2/show/NCT00604591. Accessed 19 Feb 2017.

  54. Dolder CR, Davis LN, McKinsey J. Use of psychostimulants in patients with dementia. Ann Pharmacother. 2010;44(10):1624–32.

    Article  CAS  PubMed  Google Scholar 

  55. Jesso S, Morlog D, Ross S, et al. The effects of oxytocin on social cognition and behaviour in frontotemporal dementia. Brain. 2011;134:2493–501.

    Article  PubMed  Google Scholar 

  56. Finger EC, MacKinley J, Blair M, et al. Oxytocin for frontotemporal dementia: a randomized dose-finding study of safety and tolerability. Neurology. 2015;84(2):174–81.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  57. Liljegren M, Naasan G, Temlett J, et al. Criminal behavior in frontotemporal dementia and Alzheimer disease. JAMA Neurol. 2015;72(3):295–300.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Psychiatry and Behavioural Neurosciences, McMaster University, Hamilton, ON, Canada

    Ana Hategan MD

  2. Baylor Scott and White Department of Psychiatry, Texas A&M University College of Medicine, Temple, TX, USA

    James A. Bourgeois

  3. Division of General Medicine, University of California Davis Medical Center, Sacramento, CA, USA

    Calvin H. Hirsch

Authors
  1. Ana Hategan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James A. Bourgeois
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Calvin H. Hirsch
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ana Hategan MD .

Editor information

Editors and Affiliations

  1. Department of Psychiatry and Behavioural Neurosciences, McMaster University, Hamilton, Ontario, Canada

    Ana Hategan

  2. Baylor Scott and White Department of Psychiatry, Texas A&M University College of Medicine, Temple, Texas, USA

    James A. Bourgeois

  3. Division of General Medicine, University of California Davis Medical Center, Sacramento, California, USA

    Calvin H. Hirsch

  4. Department of Psychiatry and Behavioral Sciences, University of California Davis Medical Center, Sacramento, California, USA

    Caroline Giroux

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Hategan, A., Bourgeois, J.A., Hirsch, C.H. (2018). Major or Mild Frontotemporal Neurocognitive Disorder. In: Hategan, A., Bourgeois, J., Hirsch, C., Giroux, C. (eds) Geriatric Psychiatry. Springer, Cham. https://doi.org/10.1007/978-3-319-67555-8_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-67555-8_19

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-67554-1

  • Online ISBN: 978-3-319-67555-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation