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Clinicopathological correlates in frontotemporal dementia

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Hodges, John R., Davies, R. Rhys, Xuereb, John H., Casey, Barney, Broe, Melissa, Bak, Thomas H., Kril, Jillian J., Halliday, Glenda M.·2004·Annals of Neurology
DOI 10.1002/ana.20203
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Abstract

The term frontotemporal dementia (FTD) encompasses a range of clinical syndromes that are believed not to map reliably onto the spectrum of recognized pathologies. This study reexamines the relationships between clinical and pathological subtypes of FTD in a large series from two centers (n = 61). Clinical subtypes defined were behavioral variant FTD (n = 26), language variants (semantic dementia, n = 9; and progressive nonfluent aphasia, n = 8), and motor variants (corticobasal degeneration, n = 9; and motor neuron disease, n = 9), although most cases presented with a combination of behavioral and language problems. Unexpectedly, some behavioral cases (n = 5) had marked amnesia at presentation. The pathological subtypes were those with tau‐immunopositive inclusions (with Pick bodies, n = 20; or without, n = 11), those with ubiquitin immunopositive inclusions (n = 16), and those lacking distinctive histology (n = 14). Behavioral symptoms and semantic dementia were associated with a range of pathologies. In contrast, other clinical phenotypes had relatively uniform underlying pathologies: motor neuron disease predicted ubiquitinated inclusions, parkinsonism and apraxia predicted corticobasal pathology, and nonfluent aphasia predicted Pick bodies. Therefore, the pathological substrate can be predicted in a significant proportion of FTD patients, which has important implications for studies targeting mechanistic treatments. Ann Neurol 2004

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  • 10% of pathologically-confirmed bvFTD subjects show marked episodic memory deficits at initial presentation.

    “Furthermore, 10% of pathologically-confirmed bvFTD subjects show marked episodic memory deficits at initial presentation, contrary to current diagnostic criteria (Hodges et al. , 2004; Hornberger and Piguet, 2012; Bertoux et al. , 2018).”

    From Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders (2020) · cites this paper as Hodges et al. , 2004

    The Diagnoses section (pp. 5–6) reports marked amnesia at presentation in five pathologically confirmed cases later grouped with behavioral-variant FTD. That is 8% of the full 61-case FTD cohort—approximately 10%—but five of the 26 behavioral-variant cases (19%), so the claimed percentage uses the wrong denominator. The Discussion (p. 8) also notes that early severe amnesia was an exclusion criterion in the consensus diagnostic criteria. (LLM, conf=0.90)

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Research works printed but not linked: 42

  1. [1]1. Ratnavalli E, Brayne C, Dawson K, Hodges JR. The prevalence of frontotemporal dementia. Neurology 2002;58:1615-1621.
  2. [2]2. Goedert M, Crowther RA, Spillantini MG. Tau mutations cause frontotemporal dementias. Neuron 1998;21:955-958.
  3. [3]3. Rosen HJ, Hartikainen KM, Jagust W, et al. Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD. Neurology 2002;58:1608-1614.
  4. [4]4. Raskovsky K, Salmon DP, Ho GJ, et al. Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and AD. Neurology 2002;58:1801-1808.
  5. [5]5. Dickson DW. Pick's disease: a modern approach. Brain Pathol 1998;8:339-354.
  6. [6]6. Jackson M, Lowe J. The new neuropathology of degenerative frontotemporal dementias. Acta Neuropathol 1998;91:127-134.
  7. [7]7. McKhann GM, Albert MS, Grossman M, et al. 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.
  8. [8]8. Hodges JR, Davies R, Xuereb J, et al. Survival in frontotemporal dementia. Neurology 2003;61:349-354.
  9. [9]9. Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998;51:1546-1554.
  10. [10]10. Hodges JR, Miller BL. The classification, genetics and neuropathology of frontotemporal dementia (FTD). Introduction to the special topic papers: part I. Neurocase 2001;7:31-35.
  11. [11]11. Bozeat S, Gregory CA, Lambon Ralph MA, Hodges JR. Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatry 2000;69:178-186.
  12. [12]12. Hodges JR, Patterson K, Oxbury S, Funnell E. Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain 1992;115:1783-1806.
  13. [13]13. Snowden JS, Goulding PJ, Neary D. Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol 1989;2: 167-182.
  14. [14]14. Nestor PJ, Graham NL, Fryer TD, et al. Progressive non-fluent aphasia is associated with hypometabolism centered on the left anterior insula. Brain 2003;126:2406-2418.
  15. [15]15. Grossman M. Frontotemporal dementia: a review. J Int Neuropsychol Soc 2002;8:566-583.
  16. [16]16. Bak T, Hodges JR. Motor neurone disease, dementia and aphasia coincidence, co-occurrence or continuum? J Neurol 2001; 248:260-270.
  17. [17]17. Kertesz A, Martinez-Lage P, Davidson W, Munoz DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 2000;55:1368-1375.
  18. [18]18. Broe M, Hodges JR, Schofield E, et al. Staging disease severity in pathologically confirmed cases of frontotemporal dementia. Neurology 2003;60:1005-1011.
  19. [19]19. The National Institute on Aging and Reagan Institute Working Group on Diagnostic Criteria for the Neuropathological Assessment of Alzheimer's Disease. Consensus recommendations for the postmortem diagnosis of Alzheimer's disease. Neurobiol Aging 1997;18:S1-S2.
  20. [20]20. McKeith IG, Galasko D, Kosaka K, et al. Clinical and pathological diagnosis of dementia with Lewy bodies (DLB): report of the Consortium on Dementia with Lewy Bodies (CDLB) international Workgroup. Neurology 1996;47:1113-1124.
  21. [22]22. Lantos PL, Papp MI. Cellular pathology of multiple system atrophy: a review. J Neurol Neurosurg Psychiatry 1994;57: 129-133.
  22. [23]23. Landis JR, Koch GG. An application of hierarchical kappa-type statistics in the assessment of majority agreement among multiple observers. Biometrics 1977;33:363-374.
  23. [24]24. Litvan I, Agid Y, Goetz C, et al. Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. Neurology 1997;48:119-125.
  24. [25]25. Bathgate D, Snowden JS, Varma A, et al. Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia. Acta Neurol Scand 2001;103:367-378.
  25. [26]26. Okamoto K, Hirai S, Yamazaki T, et al. New ubiquitin-positive intraneuronal inclusions in the extra-motor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett 1991;129: 233-236.
  26. [27]27. Yaguchi M, Okamoto K, Nakazato Y. Frontotemporal dementia with cerebral intraneuronal ubiquitin-positive inclusions but lacking lower motor neuron involvement. Acta Neuropathol 2003;105:81-85.
  27. [28]28. Mackenzie IRA, Feldman H. The relationship between extramotor ubiquitin-immunoreactive neuronal inclusions and dementia in motor neuron disease. Acta Neuropathol 2003;105:98-102.
  28. [29]29. Rossor MN, Revesz T, Lantos PL, Warrington EK. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain 2000;123:267-276.
  29. [30]30. Gibb WRG, Luthert PJ, Marsden CD. Corticobasal degeneration. Brain 1989;112:1171-1192.
  30. [31]31. Rinne JO, Lee MS, Thompson PD, Marsden CD. Corticobasal degeneration: a clinical study of 36 cases. Brain 1994;117: 1183-1196.
  31. [33]33. Tsuchiya K, Mitani K, Arai T, et al. Argyrophilic grain disease mimicking temporal Pick's disease: a clinical, radiological, and pathological study of an autopsy case with a clinical course of 15 years. Acta Neuropathol 2001;102:195-199.
  32. [34]34. Togo T, Cookson N, Dickson DW. Argyrophilic grain disease: neuropathology, frequency in a dementia brain bank and lack of relationship with apolipoprotein E. Brain Pathol 2002;12:45-52.
  33. [35]35. Jellinger KA. Dementia with grains (argyrophilic grain disease). Brain Pathol 1998;8:377-386.
  34. [36]36. Togo T, Sahara N, Yen S-H, et al. Agyrophilic grain disease is a sporadic 4-repeat tauopathy. J Neuropathol Exp Neurol 2002; 61:547-556.
  35. [37]37. Maurage C-A, Sergeant N, Schraen-Maschke S, et al. A diffuse form of agyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease. Acta Neuropathol 2003;106:575-583.
  36. [38]38. Braak H, Braak E. Argyrophilic grain disease: frequency of occurrence in different age categories and neuropathological diagnostic criteria. J Neural Transmission 1998;105:801-819.
  37. [39]39. Graham NL, Patterson K, Bak T, Hodges JR. Language function and dysfunction in corticobasal degeneration. Neurology 2003;61:493-499.
  38. [40]40. Kertesz A, Munoz DG. Pick's disease and Pick complex. New York: Wiley-Liss, 1998.
  39. [42]42. Mesulam MM. Primary progressive aphasia. Ann Neurol 2001; 49:425-432.
  40. [43]43. Hayashi S, Toyoshima Y, Hasegawa M, et al. Late-onset frontotemporal dementia with a novel exon 1 (Arg5His) tau gene mutation. Ann Neurol 2002;51:525-530.
  41. [44]44. Tsuchiya K, Ikeda M, Hasegawa K, et al. Distribution of cerebral cortical lesions in Pick's disease with Pick bodies: a clinicopathological study of six autopsy cases showing unusual clinical presentations. Acta Neuropathol 2001;102:553-571.
  42. [45]45. Binetti G, Locascio JJ, Corkin S, et al. Differences between Pick disease and Alzheimer disease in clinical appearance and rate of cognitive decline. Arch Neurol 2000;57:225-232.

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Authors
Hodges, J. R., Davies, R. R., Xuereb, J. H., Casey, B., Broe, M., Bak, T. H., Kril, J. J., & Halliday, G. M.
Affiliations
  • MRC Cognition and Brain Sciences Unit (GB)
  • Addenbrooke's Hospital (GB)
  • The University of Sydney (AU)
  • UNSW Sydney (AU)
Corresponding author
Hodges, John R.
ORCID
Venue
Annals of Neurology
Publisher
Wiley
Published
2004-08-31
Volume
56
Issue
3
Pages
399-406
ISSN
  • 0364-5134 (ISSN-L)
  • 1531-8249
DOI
10.1002/ana.20203
OpenAlex
W2068424345
Cited by
580
References
1
Citations by year
2026: 4 · 2025: 7 · 2024: 8 · 2023: 8 · 2022: 9 · 2021: 12 · 2020: 18 · 2019: 15 · 2018: 15 · 2017: 15 · 2016: 27 · 2015: 23 · 2014: 19 · 2013: 54 · 2012: 33
FWCI
25.94
Type
journal article
Language
en
Open access
Licence
Locations
  • Annals of Neurology (publishedVersion)
  • PubMed (publishedVersion)
  • Edinburgh Research Explorer (University of Edinburgh) (publishedVersion, other-oa)
Funders
  • Wellcome Trust (066511)
  • Medical Research Council
  • National Health and Medical Research Council
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
Keywords
frontotemporal dementia, clinicopathological correlation, behavioral variant FTD, semantic dementia, progressive nonfluent aphasia, corticobasal degeneration, motor neuron disease, Pick bodies, parkinsonism, apraxia, amnesia, clinical subtypes, pathological subtypes
MeSH terms
Aged, Aged, 80 and over, Brain, Dementia, Diagnosis, Differential, Female, Humans, Male, Middle Aged, Retrospective Studies, Reproducibility of Results, tau Proteins

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