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Chapter 8 - Frontotemporal Dementia

from Section 2 - Diagnosing Different Types of Dementia

Published online by Cambridge University Press:  25 October 2024

Simon Gerhand
Affiliation:
Hywel Dda Health Board, NHS Wales
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Summary

This chapter reviews the different forms of frontotemporal dementia. These make up the most common forms of young-onset dementia alongside Alzheimer’s disease. The most frequent form is behavioural variant, which is initially characterised by behavioural changes before cognitive change becomes apparent. Incidence and prevalence rates are reviewed, alongside underlying pathology and diagnostic criteria. The chapter moves onto primary progressive aphasia, which is characterised by an initial presentation affecting language function. The three subtypes – aggramatic, logopenic, and semantic dementia – are reviewed, alongside how to distinguish between them.

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Chapter
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The Neuropsychology of Dementia
A Clinician's Manual
, pp. 105 - 120
Publisher: Cambridge University Press
Print publication year: 2024

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References

Alexander, G. E. (1994). Basal ganglia-thalamocortical circuits: Their role in control of movements. Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society, 11 (4), 420–31.CrossRefGoogle ScholarPubMed
Alexander, G. E., & Crutcher, M. D. (1990). Functional architecture of basal ganglia circuits: Neural substrates of parallel processing. Trends in Neurosciences, 13 (7), 266–71.CrossRefGoogle ScholarPubMed
Astington, J. W., & Jenkins, J. M. (1999). A longitudinal study of the relation between language and theory-of-mind development. Developmental Psychology, 35 (5), 1311.CrossRefGoogle ScholarPubMed
Baron-Cohen, S. (1997). Mindblindness: An Essay on Autism and Theory of Mind. Cambridge, MA: MIT Press.Google Scholar
Bird, T., Knopman, D., VanSwieten, J., et al. (2003). Epidemiology and genetics of frontotemporal dementia/Pick’s disease. Annals of Neurology, 54, S29S31.CrossRefGoogle ScholarPubMed
Bonelli, R. M., & Cummings, J. L. (2007). Frontal-striatal circuitry and behaviour. Dialogues in Clinical Neuroscience, 9 (2), 141–51.CrossRefGoogle Scholar
Bora, E., Velakoulis, D., & Walterfang, M. (2016). Meta-analysis of facial emotion recognition in behavioral variant frontotemporal dementia: Comparison with Alzheimer disease and healthy controls. Journal of Geriatric Psychiatry and Neurology, 29 (4), 205–11.CrossRefGoogle ScholarPubMed
Broe, M., Hodges, J. R., Schofield, E., et al. (2003). Staging disease severity in pathologically confirmed cases of frontotemporal dementia. Neurology, 60 (6), 1005–11.CrossRefGoogle ScholarPubMed
Brun, A. (1993). Frontal lobe degeneration of non-Alzheimer type revisited. Dementia, 4, 126–31.Google Scholar
Cicerone, K. D., & Tanenbaum, L. N. (1997). Disturbance of social cognition after traumatic orbitofrontal brain injury. Archives of Clinical Neuropsychology, 12 (2), 173–88.CrossRefGoogle ScholarPubMed
Coyle-Gilchrist, I. T., Dick, K. M., Patterson, K., et al. (2016). Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes. Neurology, 86 (18), 1736–43.CrossRefGoogle ScholarPubMed
Duff, K., Paulsen, J. S., Beglinger, L. J., et al. (2010). ‘Frontal’ behaviors before the diagnosis of Huntington’s disease and their relationship to markers of disease progression: Evidence of early lack of awareness. The Journal of Neuropsychiatry and Clinical Neurosciences, 22 (2), 196207.CrossRefGoogle ScholarPubMed
Eid, H. R., Rosness, T. A., Bosnes, O., et al. (2019). Smoking and obesity as risk factors in frontotemporal dementia and Alzheimer’s disease: The HUNT Study. Dementia and Geriatric Cognitive Disorders Extra, 9 (1), 110.Google Scholar
Ekman, P., & Friesen, W. V. (1976). Pictures of Facial Affect. Palo Alto: Consulting Psychologists Press.Google Scholar
Folstein, M. F., Folstein, S. E., & McHugh, P. R. (1975). ‘Mini-mental state’: A practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, 12 (3), 189–98.Google Scholar
Goldman, J. S., Farmer, J. M., Wood, E. M., et al. (2005). Comparison of family histories in FTLD subtypes and related tauopathies. Neurology, 65 (11), 1817–19.CrossRefGoogle ScholarPubMed
Goodglass, H., Kaplan, E., & Barresi, B. (2001). Boston Diagnostic Aphasia Examination. 3rd ed. Baltimore: Lippincott, Williams & Wilkins.Google Scholar
Gorno‐Tempini, M. L., Dronkers, N. F., Rankin, K. P., et al. (2004). Cognition and anatomy in three variants of primary progressive aphasia. Annals of Neurology: Official Journal of the American Neurological Association and the Child Neurology Society, 55(3), 335–46.CrossRefGoogle ScholarPubMed
Gorno-Tempini, M. L., Hillis, A. E., Weintraub, S., et al. (2011). Classification of primary progressive aphasia and its variants. Neurology, 76 (11), 1006–14.CrossRefGoogle ScholarPubMed
Hodges, J. R., Davies, R., Xuereb, J., Kril, J., & Halliday, G. (2003). Survival in frontotemporal dementia. Neurology, 61 (3), 349–54.CrossRefGoogle ScholarPubMed
Hodges, J. R., Patterson, K., Oxbury, S., & Funnell, E. (1992). Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain, 115, 1783–806.CrossRefGoogle ScholarPubMed
Howard, D., & Patterson, K. (1992). The Pyramids and Palm Trees: A test of Semantic Access from Pictures and Words. Bury St Edmunds: Thames Valley Test Company.Google Scholar
Josephs, K. A., Petersen, R. C., Knopman, D. S., et al. (2006). Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology, 66 (1), 41–8.CrossRefGoogle ScholarPubMed
Josephs, K. A., Whitwell, J. L., Knopman, D. S., et al. (2009). Two distinct subtypes of right temporal variant frontotemporal dementia. Neurology, 73 (18), 1443–50.CrossRefGoogle ScholarPubMed
Kalkonde, Y. V., Jawaid, A., Qureshi, S. U., et al. (2012). Medical and environmental risk factors associated with frontotemporal dementia: A case-control study in a veteran population. Alzheimer’s & Dementia, 8 (3), 204–10.CrossRefGoogle Scholar
Kaplan, E., Goodglass, H., & Weintraub, S. (1983). Boston Naming Test. Philadelphia: Lea & Febiger.Google Scholar
Kay, J., Coltheart, M., & Lesser, R. (1992). PALPA: Psycholinguistic Assessments of Language Processing in Aphasia. New York: Psychology Press.Google Scholar
Keane, J., Calder, A. J., Hodges, J. R., & Young, A. W. (2002). Face and emotion processing in frontal variant frontotemporal dementia. Neuropsychologia, 40 (6), 655–65.CrossRefGoogle ScholarPubMed
Kertesz, A., Davidson, W, & Fox, H. (1997). Frontal behavioral inventory: Diagnostic criteria for frontal lobe dementia. Canadian Journal of Neurological Sciences, 24, 2936.CrossRefGoogle ScholarPubMed
Kipps, C. M., Hodges, J. R., & Hornberger, M. (2010). Nonprogressive behavioural frontotemporal dementia: Recent developments and clinical implications of the ‘bvFTD phenocopy syndrome’. Current Opinion in Neurology, 23 (6), 628–32.CrossRefGoogle ScholarPubMed
Kipps, C. M., Nestor, P. J., Acosta-Cabronero, J., Arnold, R., & Hodges, J. R. (2009). Understanding social dysfunction in the behavioural variant of frontotemporal dementia: The role of emotion and sarcasm processing. Brain, 132(3), 592603.CrossRefGoogle ScholarPubMed
Knopman, D. S., & Roberts, R. O. (2011). Estimating the number of persons with frontotemporal lobar degeneration in the US population. Journal of Molecular Neuroscience, 45, 330–5.CrossRefGoogle ScholarPubMed
Krueger, C. E., Dean, D. L., Rosen, H. J., et al. (2010). Longitudinal rates of lobar atrophy in frontotemporal dementia, semantic dementia, and Alzheimer’s disease. Alzheimer Disease & Associated Disorders, 24 (1), 43–8.CrossRefGoogle ScholarPubMed
Lanata, S. C., & Miller, B. L. (2016). The behavioural variant frontotemporal dementia (bvFTD) syndrome in psychiatry. Journal of Neurology, Neurosurgery & Psychiatry, 87(5), 501–11.CrossRefGoogle ScholarPubMed
Leyton, C. E., Ballard, K. J., Piguet, O., & Hodges, J. R. (2014a). Phonologic errors as a clinical marker of the logopenic variant of PPA. Neurology, 82 (18), 1620–7.CrossRefGoogle ScholarPubMed
Leyton, C. E., Savage, S., Irish, M., et al. (2014b). Verbal repetition in primary progressive aphasia and Alzheimer’s disease. Journal of Alzheimer’s Disease, 41 (2), 575–85.Google ScholarPubMed
Lichter, D. G., & Cummings, J. L. (2001). Introduction and overview. In Lichter, D. G, & Cummings, J. L. (Eds.). Frontal-Subcortical Circuits in Psychiatric and Neurological Disorders (pp. 143). Guilford Press.Google Scholar
Lomen-Hoerth, C. (2011). Clinical phenomenology and neuroimaging correlates in ALS-FTD. Journal of Molecular Neuroscience, 45 (3), 656–62.CrossRefGoogle ScholarPubMed
Mackenzie, I. R., & Neumann, M. (2016). Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies. Journal of Neurochemistry, 138, 5470.CrossRefGoogle ScholarPubMed
Mackenzie, I. R., Neumann, M., Baborie, A., et al. (2011). A harmonized classification system for FTLD-TDP pathology. Acta Neuropathologica, 122 (1), 111–13.CrossRefGoogle ScholarPubMed
Marshall, C. R., Hardy, C. J., Volkmer, A., et al. (2018). Primary progressive aphasia: A clinical approach. Journal of Neurology, 265 (6), 1474–90.CrossRefGoogle ScholarPubMed
Mathuranath, P. S., Nestor, P. J., Berrios, G. E., Rakowicz, W., & Hodges, J. R. (2000). A brief cognitive test battery to differentiate Alzheimer’s disease and frontotemporal dementia. Neurology, 55(11), 1613–20.CrossRefGoogle ScholarPubMed
McDonald, S., Flanagan, S., Rollins, J., & Kinch, J. (2003). TASIT: A new clinical tool for assessing social perception after traumatic brain injury. Journal of Head Injury Trauma Rehabilitation, 18, 219–38.Google ScholarPubMed
Mesulam, M. (1982). Slowly progressive aphasia without generalised dementia. Annals of Neurology, 11, 592–98.CrossRefGoogle ScholarPubMed
Mesulam, M. M. (2001). Primary progressive aphasia. Annals of Neurology, 49 (4), 425–32.CrossRefGoogle ScholarPubMed
Neary, D., Snowden, J. S., Gustafson, L., et al. (1998). Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology, 51 (6), 1546–54.CrossRefGoogle ScholarPubMed
Neumann, M., Rademakers, R., Roeber, S., et al. (2009). A new subtype of frontotemporal lobar degeneration with FUS pathology. Brain, 132(11), 2922–31.CrossRefGoogle ScholarPubMed
Nunnemann, S., Last, D., Schuster, T., Förstl, H., Kurz, A., & Diehl-Schmid, J. (2011). Survival in a German population with frontotemporal lobar degeneration. Neuroepidemiology, 37 (3–4), 160–5.CrossRefGoogle Scholar
Onyike, C. U., & Diehl-Schmid, J. (2013). The epidemiology of frontotemporal dementia. International Review of Psychiatry, 25 (2), 130–7.Google ScholarPubMed
Perry, R. J., Graham, A., Williams, G., et al. (2006). Patterns of frontal lobe atrophy in frontotemporal dementia: A volumetric MRI study. Dementia and Geriatric Cognitive Disorders, 22 (4), 278–87.CrossRefGoogle ScholarPubMed
Pick, A. (1892). Ueber die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager Med Wochenschr, 17, 165–7.Google Scholar
Poos, J. M., Jiskoot, L. C., Leijdesdorff, S. M. J., et al. (2020). Cognitive profiles discriminate between genetic variants of behavioral frontotemporal dementia. Journal of Neurology, 267 (6), 1603–12.Google ScholarPubMed
Premack, D., & Woodruff, G. (1978). Does the chimpanzee have a theory of mind? Behavioral and Brain Sciences, 1(4), 515–26.CrossRefGoogle Scholar
Rabin, L. A., Barr, W. B., & Burton, L. A. (2005). Assessment practices of clinical neuropsychologists in the United States and Canada: A survey of INS, NAN, and APA Division 40 members. Archives of Clinical Neuropsychology, 20 (1), 3365.CrossRefGoogle Scholar
Ramos, E. M., Dokuru, D. R., Van Berlo, V., et al. (2019). Genetic screen in a large series of patients with primary progressive aphasia. Alzheimer’s & Dementia, 15 (4), 553–60.CrossRefGoogle Scholar
Rascovsky, K., & Grossman, M. (2013). Clinical diagnostic criteria and classification controversies in frontotemporal lobar degeneration. International Review of Psychiatry, 25 (2), 145–58.CrossRefGoogle ScholarPubMed
Rascovsky, K., Hodges, J. R., Knopman, D., et al. (2011). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain, 134 (9), 2456–77.CrossRefGoogle ScholarPubMed
Rasmussen, H., Rosness, T. A., Bosnes, O., et al. (2018). Anxiety and depression as risk factors in frontotemporal dementia and Alzheimer’s disease: The HUNT study. Dementia and Geriatric Cognitive Disorders Extra, 8 (3), 414–25.CrossRefGoogle ScholarPubMed
Ratnavalli, E., Brayne, C., Dawson, K., & Hodges, J. R. (2002). The prevalence of frontotemporal dementia. Neurology, 58 (11), 1615–21.CrossRefGoogle ScholarPubMed
Roberson, E. D., Hesse, J. H., Rose, K. D., et al. (2005). Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology, 65 (5), 719–25.CrossRefGoogle ScholarPubMed
Rogalski, E. J., Rademaker, A., Wieneke, C., et al. (2014). Association between the prevalence of learning disabilities and primary progressive aphasia. JAMA Neurology, 71 (12), 1576–7.CrossRefGoogle ScholarPubMed
Rogalski, E., Cobia, D., Harrison, T. M., et al. (2011). Progression of language decline and cortical atrophy in subtypes of primary progressive aphasia. Neurology, 76 (21), 1804–10.CrossRefGoogle ScholarPubMed
Roman Meller, M., Patel, S., Duarte, D., Kapczinski, F., & de Azevedo Cardoso, T. (2021). Bipolar disorder and frontotemporal dementia: A systematic review. Acta Psychiatrica Scandinavica, 144 (5), 433–47.CrossRefGoogle ScholarPubMed
Rosso, S. M., Van Swieten, J. C., Roks, G., et al. (2002). Apolipoprotein E4 in the temporal variant of frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry, 72 (6), 820.CrossRefGoogle ScholarPubMed
Savage, S., Hsieh, S., Leslie, F., et al. (2013). Distinguishing subtypes in primary progressive aphasia: application of the Sydney language battery. Dementia and Geriatric Cognitive Disorders, 35 (3–4), 208–18.CrossRefGoogle ScholarPubMed
Schoder, D., Hannequin, D., Martinaud, O., et al. (2010). Morbid risk for schizophrenia in first-degree relatives of people with frontotemporal dementia. The British Journal of Psychiatry, 197(1), 2835.CrossRefGoogle ScholarPubMed
Snowden, J. S., Goulding, P. J., & Neary, D. (1989). Semantic dementia: A form of circumscribed cerebral atrophy. Behavioural Neurology, 2, 167–82.CrossRefGoogle Scholar
Spinelli, E. G., Mandelli, M. L., Miller, Z. A., et al. (2017). Typical and atypical pathology in primary progressive aphasia variants. Annals of Neurology, 81 (3), 430–43.CrossRefGoogle ScholarPubMed
Swartz, J. R., Miller, B. L., Lesser, I. M., & Darby, A. L. (1997). Frontotemporal dementia: Treatment response to serotonin selective reuptake inhibitors. Journal of Clinical Psychiatry, 58 (5), 212–17.Google ScholarPubMed
Ulugut, H., Stek, S., Wagemans, L. E., et al. (2022). The natural history of primary progressive aphasia: Beyond aphasia. Journal of Nneurology, 269 (3), 1375–85.Google ScholarPubMed
Utianski, R. L., Botha, H., Martin, P. R., et al. (2019). Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia. Brain and Language, 197, 104676.CrossRefGoogle ScholarPubMed
Walker, A. J., Meares, S., Sachdev, P. S., & Brodaty, H. (2005). The differentiation of mild frontotemporal dementia from Alzheimer’s disease and healthy aging by neuropsychological tests. International Psychogeriatrics, 17 (1), 5768.CrossRefGoogle ScholarPubMed
Warrington, E. K. (1975). Selective impairment of semantic memory. (1975) Quarterly Journal of Experimental Psychology, 27, 635–57.CrossRefGoogle ScholarPubMed
Woolley, J. D., Khan, B. K., Murthy, , et al. (2011). The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with early neurodegenerative disease. The Journal of Clinical Psychiatry, 72 (2), 126–33.CrossRefGoogle ScholarPubMed

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  • Frontotemporal Dementia
  • Simon Gerhand, Hywel Dda Health Board, NHS Wales
  • Book: The Neuropsychology of Dementia
  • Online publication: 25 October 2024
  • Chapter DOI: https://doi.org/10.1017/9781009025911.009
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  • Frontotemporal Dementia
  • Simon Gerhand, Hywel Dda Health Board, NHS Wales
  • Book: The Neuropsychology of Dementia
  • Online publication: 25 October 2024
  • Chapter DOI: https://doi.org/10.1017/9781009025911.009
Available formats
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  • Frontotemporal Dementia
  • Simon Gerhand, Hywel Dda Health Board, NHS Wales
  • Book: The Neuropsychology of Dementia
  • Online publication: 25 October 2024
  • Chapter DOI: https://doi.org/10.1017/9781009025911.009
Available formats
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