Neuropsychiatric Symptom Profile Differs Based on Pathology in Patients with Clinically Diagnosed Behavioral Variant Frontotemporal Dementia
Open accessAbstract
Background: Behavioral variant frontotemporal dementia (bvFTD) is pathologically heterogeneous. With emerging therapeutics, determining underlying pathology during life is increasingly important. Neuropsychiatric symptoms are prevalent and diagnostic in bvFTD. Methods: We assessed the neuropsychiatric profile of patients with clinically diagnosed bvFTD as a function of pathology at autopsy. Patients with a clinical diagnosis of bvFTD at the initial visit were selected from the National Alzheimer's Coordinating Center (NACC) database. Neuropsychiatric symptoms endorsed on the Neuropsychiatric Inventory Questionnaire (NPI-Q) were analyzed. Results: Of 149 patients with clinically diagnosed bvFTD, pathology was primarily Alzheimer's disease (AD) in 20.5%. These patients differed from those with underlying frontotemporal lobar degeneration: patients with AD pathology (plaques and tangles) were more likely to have hallucinations, delusions, or agitation. Patients were further differentiated into tau-positive (30% of cases, including Pick's disease, FTD and parkinsonism with tau-positive or argyrophilic inclusions, and other tauopathies) or tau-negative cases (70% of cases, including bvFTD tau-negative ubiquitin-positive inclusions). These patients also differed in some of the neuropsychiatric symptoms seen. Tau-negative cases were more likely to demonstrate depression, delusions, and changes in appetite and eating. Conclusions: These preliminary findings contribute to our increasing ability to predict, using simple clinical tools, the neuropathological underpinnings of bvFTD during life.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
bvFTD patients may exhibit compulsive perseverative thoughts or behaviors, including collecting, hoarding, and hyperphagia, while these compulsive changes are typically absent in fvAD.
“Relatedly, bvFTD patients may exhibit compulsive perseverative thoughts or behaviors, including collecting, hoarding, and hyperphagia, while these compulsive changes are typically absent in fvAD [27].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [27]
The Introduction (page not available) reports that stereotypic behaviors and changes in eating are more characteristic of clinically diagnosed bvFTD than AD. The Discussion (page not available) likewise reports more appetite and eating changes in bvFTD, but only suggests that some patients with AD pathology might have frontal-variant AD. The paper does not establish that compulsive changes are typically absent in fvAD or specifically document collecting and hoarding. (LLM, conf=0.85)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Clinicopathologic differences among patients with behavioral variant frontotemporal dementia
Mendez, M. F., Joshi, A., Tassniyom, K., Teng, E., & Shapira, J. S. (2013) · Neurology · doi:10.1212/wnl.0b013e3182815547 · Cited by 96 · see citing works
The Cambridge behavioral inventory: validation and application in a memory clinic
Nagahama, Y., Okina, T., Suzuki, N., & Matsuda, M. (2006) · Journal of Geriatric Psychiatry and Neurology · doi:10.1177/0891988706286545 · Cited by 45 · see citing works
Flagged first, then by citations
Research works printed but not linked: 38
- [1]Neary D Snowden J Gustafson L Passant U Stuss D Black S Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria Neurology 1998 12 1 51 1546 1554 9855500
- [2]Rademakers R Neumann M Mackenzie IR Advances in understanding the molecular basis of frontotemporal dementia Nat Rev Neurol 2012 6 26 8 423 434 22732773 PMC3629543
- [3]Halliday G Bigio EH Cairns NJ Neumann M Mackenzie IRA Mann DMA Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effects Acta Neuropathol 2012 8 10 10.1007/s00401-012-1030-4 PMC3445027 22878865 · DOI
- [4]Gorno-Tempini ML Hillis AE Weintraub S Kertesz A Mendez M Cappa SF Classification of primary progressive aphasia and its variants Neurology 2011 3 15 76 1006 1014 21325651 PMC3059138
- [5]Chow TW Alobaidy AA Incorporating new diagnostic schemas, genetics, and proteinopathy into the evaluation of frontotemporal degeneration Continuum (Minneap Minn) 2013 4 19 438 456 23558488 PMC10563897
- [6]Bigni B Premi E Pilotto A Padovani A Borroni B Disease-modifying therapies in frontotemporal lobar degeneration Curr Med Chem 2012 19 1008 1020 22257060
- [7]Rascovsky K Hodges JR Knopman D Mendez MF Kramer JH Neuhaus J Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Brain 2011 9 10 134 2456 2477 21810890 PMC3170532
- [8]Cummings J Mega M Gray K Carusi D Gornbein J null The Neuropsychiatric Inventory: comprehensive assessment of psychopathology in dementia Neurology 1994 12 1 44 2308 2314 7991117
- [9]Barber R Snowden J Craufurd D Frontotemporal dementia and Alzheimer's disease: retrospective differentiation using information from informants J Neurol Neurosurg Psychiatry 1995 7 1 59 61 70 7608712 PMC1073603
- [10]Kertesz A Davidson W Fox H Frontal behavioral inventory: diagnostic criteria for frontal lobe dementia Can J Neurol Sci 1997 2 1 24 29 36 9043744
- [11]Mendez MF Perryman KM Miller BL Cummings JL Behavioral Differences Between Frontotemporal Dementia and Alzheimers Disease: A Comparison on the BEHAVE-AD Rating Scale Int Psychogeriatr 1999 10 155 162 9677502
- [12]Lebert F Pasquier F Petit H null Frontotemporal behavioral scale Alzheimer Dis Assoc Disord 1998 12 1 12 335 339 9876962
- [13]Bozeat S Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease? J Neurol Neurosurg Psychiatr 2000 8 1 69 178 186 10896690 PMC1737062
- [14]Bathgate D Snowden JS Varma A Blackshaw A Neary D Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia Acta Neurol Scand 2001 6 103 367 378 11421849
- [15]De Deyn PP Engelborghs S Saerens J Goeman J Marin P Maertens K The Middelheim Frontality Score: a behavioural assessment scale that discriminates frontotemporal dementia from Alzheimer's disease Int J Geriatr Psychiatr 2004 20 70 79 15578673
- [16]Levy ML Miller BL Cummings JL Fairbanks LA Craig A Alzheimer Disease and Frontotemporal Dementias: Behavioral Distinctions Arch Neurol 1996 7 1 53 687 690 8929178
- [17]Rozzini L Lussignoli G Padovani A Trabucchi M Alzheimer's Disease and Frontotemporal Dementia Archives of Neurology 1997 12 30 54 350 9109732
- [18]Hirono N Mori E Tanimukai S Kazui H Hashimoto M Hanihara T Distinctive Neurobehavioral Features Among Neurodegenerative Dementias J Neuropsychiatry Clin Neurosci 1999 11 1 11 498 503 10570764
- [19]Kaufer DI Cummings JL Ketchel P Smith V MacMillan A Shelley T Validation of the NPI-Q, a brief clinical form of the Neuropsychiatric Inventory J Neuropsychiatry Clin Neurosci 2000 12 233 239 11001602
- [20]Kertesz A Davidson W null The Frontal Behavioral Inventory in the differential diagnosis of frontotemporal dementia J Int Neuropsychol Soc 2000 5 1 6 460 468 10902415
- [21]Marczinski C Davidson W Kertesz A A longitudinal study of behavior in frontotemporal dementia and primary progressive aphasia Cogn Behav Neurol 2004 12 1 17 185 190 15622012
- [22]Blair M Kertesz A Davis-Faroque N Hsiung G Black S Bouchard R Behavioural measures in frontotemporal lobar dementia and other dementias: the utility of the frontal behavioural inventory and the neuropsychiatric inventory in a national cohort study Dement Geriatr Cogn Disord 2007 23 406 415 17446701
- [23]Clinical and neuropathological criteria for frontotemporal dementia. The Lund and Manchester Groups J Neurol Neurosurg Psychiatr 1994 4 57 416 418 8163988 PMC1072868
- [24]Rankin KP Kramer JH Mychack P Miller BL Double dissociation of social functioning in frontotemporal dementia Neurology 2003 1 28 60 266 271 12552042 PMC2701364
- [26]Liscic RM Storandt M Cairns NJ Morris JC Clinical and psychometric distinction of frontotemporal and Alzheimer dementias Arch Neurol 2007 4 64 535 540 17420315
- [27]Forman MS Farmer J Johnson JK Clark CM Arnold SE Coslett HB Frontotemporal dementia: Clinicopathological correlations Ann Neurol 2006 6 59 952 962 16718704 PMC2629792
- [28]Kulisevsky J Roldan E Hallucinations and sleep disturbances in Parkinson's disease [Internet] Neurology 2004 1 26 63 S28 S30 15505140
- [29]Compta Y Martí MJ Rey MJ Ezquerra M Parkinsonism, dysautonomia, REM behaviour disorder and visual hallucinations mimicking synucleinopathy in a patient with progressive supranuclear palsy J Neurol Neurosurg Psychiatr 2009 5 80 578 579 19372296
- [30]Snowden JS Rollinson S Thompson JC Harris JM Stopford CL Richardson AMT Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations Brain 2012 3 135 693 708 22300873 PMC3286329
- [31]Josephs KA Hodges JR Snowden JS Mackenzie IR Neumann M Mann DM Neuropathological background of phenotypical variability in frontotemporal dementia Acta Neuropathol 2011 5 26 122 137 153 21614463 PMC3232515
- [32]Whitwell JL Josephs KA Neuroimaging in frontotemporal lobar degeneration|[mdash]|predicting molecular pathology Nat Rev Neurol 2011 8 131 142 22290573
- [33]Urwin H Josephs KA Rohrer JD Mackenzie IR Neumann M Authier A FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration Acta Neuropathol 2010 5 20 120 33 41 20490813 PMC2887939
- [34]Woodward M Jacova C Black SE Kertesz A Mackenzie IR Feldman H Differentiating the frontal variant of Alzheimer's disease Int J Geriatr Psychiatry 2010 7 25 732 738 19823987
- [35]Tekin S Mega MS Masterman DM Chow T Garakian J Vinters HV Orbitofrontal and anterior cingulate cortex neurofibrillary tangle burden is associated with agitation in Alzheimer disease Ann Neurol 2001 3 49 355 361 11261510
- [36]Kim EJ Sidhu M Gaus SE Huang EJ Hof PR Miller BL Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia Cerebral Cortex 2012 1 1 22 251 259 21653702 PMC3256403
- [37]Whitwell JL Jack CR Parisi JE Knopman DS Boeve BF Petersen RC Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia J Mol Neurosci 2011 11 45 372 378 21556732 PMC3401589
- [38]Hodges JR Davies RR Xuereb JH Casey B Broe M Bak TH Clinicopathological correlates in frontotemporal dementia Ann Neurol 2004 56 399 406 15349867
- [39]Banks SJ Weintraub S Neuropsychiatric symptoms in behavioral variant frontotemporal dementia and primary progressive aphasia J Geriatr Psychiatry Neurol 2008 6 21 133 141 18474722 PMC2892801
Cited by
Sorted by citations
Tauopathies: new perspectives and challenges
Zhang, Y., Wu, K.-M., Yang, L., Dong, Q., & Yu, J.-T. (2022) · Molecular Neurodegeneration · doi:10.1186/s13024-022-00533-z · Cited by 323 · see citing works
Clinicopathological correlations in behavioural variant frontotemporal dementia
Perry, D. C., Brown, J. A., Possin, K. L., Datta, S., Trujillo, A., Radke, A., Karydas, A., Kornak, J., Sias, A. C., Rabinovici, G. D., et al. (2017) · Brain · doi:10.1093/brain/awx254 · Cited by 309 · see citing works
Determinants of behavioral and psychological symptoms of dementia: A scoping review of the evidence
Kolanowski, A., Boltz, M., Galik, E., Gitlin, L. N., Kales, H. C., Resnick, B., Van Haitsma, K. S., Knehans, A., Sutterlin, J. E., Sefcik, J. S., et al. (2017) · Nursing Outlook · doi:10.1016/j.outlook.2017.06.006 · Cited by 165 · see citing works
Alzheimer's disease including focal presentations
Villain, N., & Dubois, B. (2019) · Seminars in Neurology · doi:10.1055/s-0039-1681041 · Cited by 113 · see citing works
Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road
Sawyer, R. P., Rodriguez-Porcel, F., Hagen, M., Shatz, R., & Espay, A. J. (2017) · Journal of Clinical Movement Disorders · doi:10.1186/s40734-017-0052-4 · Cited by 40 · see citing works
“Each profile creates a distinct caregiver burden in these disorders [22, 27].”
BackgroundApathy and disinhibition related to neuropathology in amnestic versus behavioral dementias
Borges, L. G., Rademaker, A. W., Bigio, E. H., Mesulam, M.-M., & Weintraub, S. (2019) · American Journal of Alzheimer's Disease & Other Dementias® · doi:10.1177/1533317519853466 · Cited by 16 · see citing works
- Authors
- Léger, G. C., & Banks, S. J.
- Affiliations
- Cleveland Clinic (US)
- Venue
- Dementia and Geriatric Cognitive Disorders
- Publisher
- S. Karger AG
- Published
- 2013-10-10
- Volume
- 37
- Issue
- 1-2
- Pages
- 104-112
- ISSN
- 1420-8008 (ISSN-L)
- 1421-9824
- PMC
- PMC4041327
- OpenAlex
- W2090936875
- Cited by
- 50
- References
- 2
- Citations by year
- 2025: 3 · 2024: 2 · 2023: 3 · 2022: 6 · 2021: 1 · 2020: 7 · 2019: 4 · 2018: 4 · 2017: 6 · 2016: 6 · 2015: 3 · 2014: 4
- FWCI
- 3.08
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Dementia and Geriatric Cognitive Disorders (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institute on Aging (U01AG016976)
- 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
- behavioral variant frontotemporal dementia, neuropsychiatric symptoms, neuropathology, tauopathy, frontotemporal lobar degeneration, Alzheimer's disease pathology, hallucinations, delusions, agitation, depression, ubiquitin-positive inclusions, Pick's disease, Neuropsychiatric Inventory Questionnaire
- MeSH terms
- Aged, Aged, 80 and over, Alzheimer Disease, Appetite, Mental Disorders, Feeding Behavior, Female, Humans, Longitudinal Studies, Male, Middle Aged, Neuropsychological Tests, Surveys and Questionnaires, Socioeconomic Factors, Biomarkers, Databases, Factual, Neurofibrillary Tangles, tau Proteins, Frontotemporal Dementia, Plaque, Amyloid
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review