Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
Open accessAbstract
Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, 'possible' behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). 'Probable' behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia 'with definite frontotemporal lobar degeneration' requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer's disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86%) met 'possible' criteria, and 104 (76%) met criteria for 'probable' behavioural variant frontotemporal dementia. In contrast, 72 cases (53%) met previously established criteria for the syndrome (P < 0.001 for comparison with 'possible' and 'probable' criteria). Patients who failed to meet revised criteria were significantly older and most had atypical presentations with marked memory impairment. In conclusion, the revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotemporal lobar degeneration. Greater sensitivity of the proposed criteria may reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations. Future studies will be needed to establish the reliability and specificity of these revised diagnostic guidelines.
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Frontotemporal dementia is caused by frontotemporal lobar degeneration (FTLD), which features abnormal aggregation of tau (FTLD-tau) or transactivating DNA-binding protein 43.
“Frontotemporal dementia is caused by frontotemporal lobar degeneration (FTLD), which features abnormal aggregation of the microtubule associated protein consisting of tau (FTLD-tau) or the transactivating DNA-binding protein 43.16,18–20”
From Association of Cognitive and Behavioral Features Between Adults With Tuberous Sclerosis and Frontotemporal Dementia (2020) · cites this paper as [19]
The section headed “Development of the Proposed Classification of PPA and Its Variants” (page unavailable) identifies tau-positive and TDP-43-positive FTLD pathology, supporting the protein-pathology portion of the claim. It discusses primary progressive aphasia, however, and also identifies Alzheimer pathology as a possible cause; it does not establish that frontotemporal dementia as a whole is caused by FTLD or that these are its only pathological forms. (LLM, conf=0.79)
Experimental — accuracy not yet measuredIn bvFTD, language dysfunction is initially absent, unlike other frontotemporal dementia phenotypes (progressive nonfluent aphasia and semantic dementia).
“In bvFTD, language dysfunction is initially absent, unlike other frontotemporal dementia phenotypes, namely progressive nonfluent aphasia and semantic dementia [17].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [17]
The indexed section headed “Classification of primary progressive aphasia and its variants” describes language impairments in the nonfluent and semantic variants (page not provided). It does not establish that language dysfunction is initially absent in bvFTD, which is the central contrast in the citing sentence. (LLM, conf=0.78)
Experimental — accuracy not yet measuredThe machine learning model was trained on a mainly sporadic cohort and tested in a held-out population of genetic bvFTD to ensure generalizability.
“To ensure the generalizability of the results, the machine learning model was trained on a mainly sporadic cohort and tested in a held-out population of genetic bvFTD, therefore relying on one of the gold standards for diagnosis (i.e., definite bvFTD). 7”
From MRI data-driven algorithm for the diagnosis of behavioural variant frontotemporal dementia (2021) · cites this paper as [7]
The supplied passages are labeled as a paper on primary progressive aphasia, despite the cited DOI being identified as a bvFTD paper. Searches for machine-learning training, a mainly sporadic cohort, held-out genetic bvFTD cases, and definite bvFTD returned no passage addressing the claim; the available text discusses definite pathology for PPA instead. (LLM, conf=0.75)
Experimental — accuracy not yet measuredThe performance of the classifier is superior to the performance reported for standard diagnostic methods currently used in clinical practice.
“The performance of our classifier is superior than the performance reported in several articles that have analyzed the standard diagnostic methods currently used in the clinical practice.”
From MRI data-driven algorithm for the diagnosis of behavioural variant frontotemporal dementia (2021) · cites this paper as [7]
The indexed passages are labelled as a paper on primary progressive aphasia, despite the cited DOI being for Rascovsky et al. The Discussion passage addresses consistency of diagnostic criteria across centres, but none of the available passages reports the performance of standard clinical diagnostic methods for comparison with the citing authors’ classifier. Searches for revised behavioural-variant frontotemporal dementia criteria and diagnostic sensitivity returned only the aphasia passages; no page numbers were supplied. (LLM, conf=0.76)
Experimental — accuracy not yet measuredWithin a pathology-confirmed cohort, the sensitivity of the revised diagnostic criteria for bvFTD was 86% for possible diagnosis and 75% for probable bvFTD with neuroimaging support.
“Within a pathology-confirmed cohort, the sensitivity reported for the revised diagnostic criteria for bvFTD was 86% for possible diagnosis and 75% for probable bvFTD (with neuroimaging support). 7”
From MRI data-driven algorithm for the diagnosis of behavioural variant frontotemporal dementia (2021) · cites this paper as [7]
The indexed passages are labelled as sections of “Classification of primary progressive aphasia and its variants” and discuss PPA criteria, not sensitivity of bvFTD criteria. Searches for 86%, 75%, and possible and probable bvFTD found no passage reporting the claimed results. The available copy therefore cannot verify the figures or the pathology-confirmed cohort. (LLM, conf=0.74)
Experimental — accuracy not yet measured
Patients were clinically evaluated by expert neurologists at the time of recording and classified at a weekly consensus meeting using established clinical criteria.
“All patients (n = 96) were clinically evaluated by expert neurologists (M.G., D.J.I.) at the time of recording at the frontotemporal degeneration center (FTDC) in the neurology department of the Hospital of the University of Pennsylvania, and they were classified at a weekly consensus meeting at the FTDC using established clinical criteria.”
From Automatic classification of AD pathology in FTD phenotypes using natural speech (2024) · cites this paper as [27]
The indexed passages are labeled as a paper on primary progressive aphasia, despite the supplied source metadata. Its classification-development section describes clinicians meeting three times to develop criteria, but no passage addresses the citing study’s 96 patients, their evaluation at recording, or a weekly FTDC consensus meeting. Searches for those details found no supporting passage; the copy cannot verify the attributed procedure. (LLM, conf=0.76)
Experimental — accuracy not yet measuredAtrophy of specific brain regions shown by structural MRI is an important part of the diagnostic criteria for FTD and AD.
“the atrophy of specific brain regions shown by structural MRI is an important part of the diagnostic criteria for FTD and AD (McKhann et al., 2011; Rascovsky et al., 2011)”
From Deep Learning-Based Classification and Voxel-Based Visualization of Frontotemporal Dementia and Alzheimer's Disease (2021) · cites this paper as Rascovsky et al., 2011
The indexed copy returned for this DOI contains sections headed “Classification of primary progressive aphasia and its variants,” rather than the cited Rascovsky paper. Its diagnostic-features table includes MRI atrophy for an imaging-supported PPA variant, but the available passages do not establish what the cited paper says about FTD or AD diagnostic criteria. Searches for the revised behavioural-variant FTD criteria returned only those PPA passages. (LLM, conf=0.72)
Experimental — accuracy not yet measuredBased on these criteria, a degree of probability of underlying frontotemporal lobar degeneration can be attributed to cases presenting with at least three of five behavioural criteria and one cognitive criterion.
“Based on these criteria, a degree of probability of underlying frontotemporal lobar degeneration can be attributed to cases presenting with at least three of five behavioural criteria and one cognitive criterion.”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [3]
The passages supplied and returned for this DOI are labelled as sections of a paper on primary progressive aphasia, not the cited paper’s behavioural-variant frontotemporal dementia criteria. Searches for the three-of-five behavioural and one cognitive criterion, including possible and probable bvFTD criteria, found no passage in our copy that addresses the claimed threshold. (LLM, conf=0.76)
Experimental — accuracy not yet measuredThe clinical diagnosis of behavioural variant frontotemporal dementia is based on consensus diagnostic criteria derived from an international cohort of 406 pathologically verified cases of frontotemporal dementia.
“The clinical diagnosis of behavioural variant frontot emporal dementia is based on consensus diagnostic criteria that have been derived from an international cohort of 406 pathologically verified cases of frontotemporal dementia.3”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [3]
The retrieved copy is labelled as a paper on primary progressive aphasia, despite the index identifying it as the cited Rascovsky paper. Its Discussion and diagnostic-criteria passages do not address criteria for behavioural variant frontotemporal dementia derived from 406 pathologically verified cases. Searches for “406” and the claimed cohort found no relevant passage, so the claim cannot be judged from this copy. (LLM, conf=0.72)
Experimental — accuracy not yet measuredAtrophy in the frontal or temporal lobes supports the clinical suspicion of frontotemporal dementia.
“Atrophy in the frontal or temporal lobes, which can be either symmetrical or very focal or asymmetrical, supports the clinical suspicion of frontotemporal dementia.3,4”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [3]
The retrieved copy’s diagnostic-criteria passages concern primary progressive aphasia: they describe frontal or temporal atrophy as supporting diagnoses of particular aphasia variants, not clinical suspicion of frontotemporal dementia generally. Searches for the behavioural-variant criteria and for the stated atrophy patterns did not retrieve a passage addressing the attributed claim; the indexed text appears to belong to a different paper. (LLM, conf=0.72)
Experimental — accuracy not yet measuredLack of support is more concerning for carers of people with frontotemporal dementia especially when behavioural symptoms are severe.
“lack of support is more concerning for these carers especially when behavioural symptoms are severe (Gorno-Tempini et al. , 2011; Rascovsky et al. , 2011)”
From Which interventions work for dementia family carers?: an updated systematic review of randomized controlled trials of carer interventions. (2018) · cites this paper as Rascovsky et al., 2011
The retrieved passages are labelled as sections of “Classification of primary progressive aphasia and its variants,” rather than the named Rascovsky paper. Searches for carers, caregiver support, and severe behavioural symptoms found no passage addressing whether lack of support is especially concerning when symptoms are severe. The available copy therefore cannot verify the claim. (LLM, conf=0.72)
Experimental — accuracy not yet measuredExecutive dysfunction is not always the most prominent deficit in bvFTD and may not even be present on formal neuropsychological test results in the early stages.
“Contrary to popular belief, executive dysfunction is not always the most prominent deficit in bvFTD and may not even be present on formal neuropsychological test results in the early stages (Pachana et al. , 1996; Kipps et al. , 2008; Rascovsky et al. , 2011).”
From Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders (2020) · cites this paper as Rascovsky et al. , 2011
The supplied passages and additional indexed results are labeled as sections of “Classification of primary progressive aphasia and its variants,” rather than the cited bvFTD paper. Searches for executive dysfunction and formal neuropsychological testing did not retrieve a passage addressing whether executive deficits can be absent or less prominent in early bvFTD, so the claim cannot be verified from the available copy. (LLM, conf=0.70)
Experimental — accuracy not yet measuredThe presence of pathological atrophy in frontal or anterior temporal areas increases the bvFTD diagnostic certainty from 'possible' to 'probable' in current diagnostic criteria.
“The presence of pathological atrophy in frontal or anterior temporal areas increases the bvFTD diagnostic certainty from 'possible' to 'probable' in current diagnostic criteria (Rascovsky et al. , 2011), which increases the specificity from 82% to 95% (Harris et al. , 2013).”
From Recommendations to distinguish behavioural variant frontotemporal dementia from psychiatric disorders (2020) · cites this paper as Rascovsky et al. , 2011
The supplied passages and DOI-indexed results are labelled as a paper on primary progressive aphasia, not the cited bvFTD paper. Searches for possible versus probable bvFTD and frontal or anterior temporal atrophy returned no passage addressing that diagnostic transition, so the claim cannot be verified from our copy. No page numbers are available for these passages. (LLM, conf=0.76)
Experimental — accuracy not yet measuredMany of the DSM-5 specific dementia categories have separate criteria sets for 'Probable' and 'Possible', which in most cases have been adapted from the neurological literature.
“In addition, many of the DSM-5 specific dementia categories have separate criteria sets for 'Probable' and 'Possible' , which in most cases have been adapted from the neurological literature 88-91 .”
From An organization‐ and category‐level comparison of diagnostic requirements for mental disorders in <scp>ICD</scp> ‐11 and <scp>DSM</scp> ‐5 (2021) · cites this paper as [91]
The supplied passages and passages retrieved under this DOI are labelled as a paper on primary progressive aphasia, not the cited Rascovsky paper. The retrieved diagnostic-criteria table concerns clinical, imaging-supported and pathology-based PPA diagnoses; it does not address DSM-5 ‘Probable’ and ‘Possible’ dementia categories or whether their criteria were adapted from neurological literature. The available copy therefore cannot verify the claim about the cited paper. (LLM, conf=0.76)
Experimental — accuracy not yet measuredWhile semantic (category) fluency is usually impaired to a greater degree in fvAD, phonemic (letter) fluency is more affected in bvFTD.
“While semantic (category) fluency is usually impaired to a greater degree in fvAD, phonemic (letter) fluency is more affected in bvFTD [17].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [17]
The passages returned from our indexed copy are labeled as a section on primary progressive aphasia, despite the supplied source title. Its discussion of semantic and phonological language deficits does not compare category and letter fluency in fvAD and bvFTD. Searches for both fluency types and the fvAD–bvFTD comparison found no passage addressing the claim. (LLM, conf=0.71)
Experimental — accuracy not yet measuredClinical diagnosis of bvFTD is based on behavioral changes antedating cognitive deficits, with sensitivity 76-95% and specificity 82-95%.
“The clinical diagnosis of bvFTD is currently based on the presence of behavioral changes antedating cognitive deficits with a sensitivity of 76–95% and specificity of 82–95% [16, 17].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [17]
The supplied passages and further indexed results are labeled as a classification of primary progressive aphasia, not as the cited bvFTD study. Searches for the claimed sequence of behavioural and cognitive changes and for the 76–95% sensitivity and 82–95% specificity ranges found no passage addressing them. The apparent mismatch in the indexed copy prevents verification of this citation. (LLM, conf=0.74)
Experimental — accuracy not yet measuredFTD is a neurodegenerative disorder that predominantly affects the frontal and/or temporal lobes and presents with a spectrum of social, behavioral, language, psychiatric, and motor problems.
“Frontotemporal dementia (FTD) is a neurodegenerative disorder that predominantly affects the frontal and/or temporal lobes and presents with a spectrum of social, behavioral, language, psychiatric, and motor problems. 1 , 2 , 3”
From Clinical recognition of frontotemporal dementia with right anterior temporal predominance: A multicenter retrospective cohort study (2024) · cites this paper as [2]
The development of DSM-5 criteria for various subtypes involved extensive discussions with experts from diverse fields to harmonize with other expert groups.
“The development of the DSM-5 criteria for the various subtypes involved extensive discussions with experts from diverse fields to harmonize the DSM criteria with criteria developed by other expert groups, such as the NIA-AA, 20,27 the frontotemporal dementia expert group, 28,29 the consortium on DLB, 30 the VASCOG working group, 31 the Movement Disorder Study Task Force on Parkinson Disease 32 and”
From Classifying neurocognitive disorders: the DSM-5 approach (2014) · cites this paper as [29]
These frontotemporal criteria are consistent with external expert groups, but identification of frontotemporal degeneration as the aetiology for mild neurocognitive disorder without genetic or neuroimaging evidence is not yet established.
“These criteria are consistent with those published by external expert groups, 28,29 but it must be pointed out that the identification of frontotemporal degeneration as the aetiology for mild neurocognitive disorder in the absence of genetic and/or neuroimaging evidence is not yet established.”
From Classifying neurocognitive disorders: the DSM-5 approach (2014) · cites this paper as [29]
Patients received diagnoses based on established clinical criteria only, without testing for ATN biomarkers in AD.
“Second, patients received diagnoses based on established clinical criteria only,37,38 without testing for ATN biomarkers in AD such as the deposition of amyloid-β and tau proteins established via Positron Emission Tomography, CSF, or plasma in AD.94, 95, 96, 97”
From Altered spatiotemporal brain dynamics of interoception in behavioural-variant frontotemporal dementia (2025) · cites this paper as [37]
The diagnosis was concluded in a multidisciplinary meeting using consensus criteria for probable FTD, PPD (DSM-V), and AD.
“The diagnosis was concluded in a multidisciplinary meeting using consensus criteria for probable FTD, PPD (DSM-V), and AD [25,31,32].”
Final diagnoses were based on internationally established criteria.
“Final diagnoses were based on internationally established criteria [12, 46, 49–52].”
Two reviewers evaluated the overall methodological quality of full text papers taking into account high diagnostic accuracy, sample size, follow-up time, and use of appropriate outcome measures.
“Two reviewers (JF and DP) evaluated the overall methodological quality of the full text papers taking into account eligibility criteria of (I) high diagnostic accuracy [i.e., probable or definite bvFTD by international diagnostic criteria (Rascovsky et al., 2011)]; (II) sample size; (III) follow-up time; (IIII) use of appropriate outcome measures, when weighing research evidence.”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Rascovsky et al., 2011
In current international diagnostic criteria, the cognitive profile of bvFTD is characterized by executive deficits, with relative sparing of memory and visuospatial functioning.
“In current international diagnostic criteria, the cognitive profile of bvFTD is characterized by executive deficits, with relative sparing of memory and visuospatial functioning (Rascovsky et al., 2011).”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Rascovsky et al., 2011
bvFTD is marked by typical frontal and anterior temporal atrophy, hypometabolism or hypoperfusion.
“Since bvFTD is marked by typical frontal and (anterior) temporal atrophy, hypometabolism or hypoperfusion (Rascovsky et al., 2011), the use of neuroimaging offers an essential measure of disease progression.”
From The pursuit for markers of disease progression in behavioral variant frontotemporal dementia: a scoping review to optimize outcome measures for clinical trials (2024) · cites this paper as Rascovsky et al., 2011
The logopenic variant of PPA is usually associated with AD pathology.
“A logopenic variant of PPA (lvPPA) has also been described, but is usually associated with AD pathology.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [5]
In current clinical practice, the place of CSF analysis is to exclude AD pathology and verify neurodegeneration-it does not have utility for affirmative diagnosis of FTD.
“In current clinical practice, the place of CSF analysis is to exclude AD pathology and verify neurodegeneration—it does not have utility for affirmative diagnosis of FTD. 5”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [5]
bvFTD is characterised by profound changes to behaviour and personality, with early brain atrophy in the ventromedial prefrontal cortex and insula, extending to temporal lobes and subcortical regions with disease progression.
“Clinically, bvFTD is characterised by profound changes to behaviour and personality, with early brain atrophy observed in the ventromedial prefrontal cortex and insula (Rascovsky et al., 2011), extending to the temporal lobes and subcortical regions with disease progression (Landin-Romero et al., 2017).”
From Thinking versus feeling: How interoception and cognition influence emotion recognition in behavioural-variant frontotemporal dementia, Alzheimer's disease, and Parkinson's disease (2023) · cites this paper as Rascovsky et al., 2011
Diagnosis was established by multidisciplinary teams at each research institute based on current consensus diagnostic criteria.
“Diagnosis was established by the multidisciplinary teams at each research institute based on current consensus diagnostic criteria (McKhann et al., 2011; Postuma et al., 2015; Rascovsky et al., 2011).”
From Thinking versus feeling: How interoception and cognition influence emotion recognition in behavioural-variant frontotemporal dementia, Alzheimer's disease, and Parkinson's disease (2023) · cites this paper as Rascovsky et al., 2011
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Self-citationPatterns of Frontal Lobe Atrophy in Frontotemporal Dementia: A Volumetric MRI Study
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Pathological correlates of frontotemporal lobar degeneration in the elderly
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Anosognosia Is a Significant Predictor of Apathy in Alzheimer's Disease
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Initial Symptoms in Frontotemporal Dementia and Semantic Dementia Compared with Alzheimer’s Disease
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Behavioral disturbances in the course of frontotemporal dementia
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Self-citationMMSE Scores Decline at a Greater Rate in Frontotemporal Degeneration than in AD
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Self-citationA Model for Management of Behavioral Symptoms in Frontotemporal Lobar Degeneration
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Decomposition of metabolic brain clusters in the frontal variant of frontotemporal dementia
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Topographical Patterns of Lobar Atrophy in Frontotemporal Dementia and Alzheimer’s Disease
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Self-citationClinical features of pathologic subtypes of behavioral--variant frontotemporal dementia.
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Self-citationRehabilitation applications in caring for patients with Pick’s disease and frontotemporal dementias
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Laboratory investigations and treatment in frontotemporal dementia
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Self-citationLong-Term Care of Patients with Frontotemporal Dementia
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Self-citationFrontal-executive Versus Posterior-perceptual Mental Status Deficits in Early-onset Dementias
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Self-citationLong-Term Care of Patients With Frontotemporal Dementia
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Self-citationCSF sAPPβ, YKL-40, and neurofilament light in frontotemporal lobar degeneration
Alcolea, D., Vilaplana, E., Suárez-Calvet, M., Illán-Gala, I., Blesa, R., Clarimón, J., Lladó, A., Sánchez-Valle, R., Molinuevo, J. L., García-Ribas, G., et al. (2017) · Neurology · doi:10.1212/wnl.0000000000004088 · Cited by 123 · see citing works
Fluid biomarkers in frontotemporal dementia: past, present and future
Swift, I. J., Sogorb-Esteve, A., Heller, C., Synofzik, M., Otto, M., Graff, C., Galimberti, D., Todd, E., Heslegrave, A. J., van der Ende, E. L., et al. (2021) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2020-323520 · Cited by 122 · see citing works
Self-citationCerebrospinal fluid neurofilament light chain protein levels in subtypes of frontotemporal dementia
Landqvist Waldö, M., Frizell Santillo, A., Passant, U., Zetterberg, H., Rosengren, L., Nilsson, C., & Englund, E. (2013) · BMC Neurology · doi:10.1186/1471-2377-13-54 · Cited by 128 · see citing works
Scialò, C., Tran, T. H., Salzano, G., Novi, G., Caponnetto, C., Chiò, A., Calvo, A., Canosa, A., Moda, F., Caroppo, P., et al. (2020) · Brain Communications · doi:10.1093/braincomms/fcaa142 · Cited by 123 · see citing works
Bernstein, A., Rogers, K. M., Possin, K. L., Steele, N. Z. R., Ritchie, C. S., Kramer, J. H., Geschwind, M., Higgins, J. J., Wohlgemuth, J., Pesano, R., et al. (2019) · BMC Health Services Research · doi:10.1186/s12913-019-4603-2 · Cited by 120 · see citing works
Self-citationNeuroinflammation and protein aggregation co-localize across the frontotemporal dementia spectrum
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Profiles of white matter tract pathology in frontotemporal dementia
Mahoney, C. J., Ridgway, G. R., Malone, I. B., Downey, L. E., Beck, J., Kinnunen, K. M., Schmitz, N., Golden, H. L., Rohrer, J. D., Schott, J. M., et al. (2014) · Human Brain Mapping · doi:10.1002/hbm.22468 · Cited by 124 · see citing works
Self-citationClinical value of neurofilament and phospho-tau/tau ratio in the frontotemporal dementia spectrum
Meeter, L. H. H., Vijverberg, E. G., Del Campo, M., Rozemuller, A. J. M., Donker Kaat, L., de Jong, F. J., van der Flier, W. M., Teunissen, C. E., van Swieten, J. C., & Pijnenburg, Y. A. L. (2018) · Neurology · doi:10.1212/wnl.0000000000005261 · Cited by 122 · see citing works
Self-citationParkinsonism, movement disorders and genetics in frontotemporal dementia
Baizabal-Carvallo, J. F., & Jankovic, J. (2016) · Nature Reviews Neurology · doi:10.1038/nrneurol.2016.14 · Cited by 121 · see citing works
Katisko, K., Cajanus, A., Jääskeläinen, O., Kontkanen, A., Hartikainen, P., Korhonen, V. E., Helisalmi, S., Haapasalo, A., Koivumaa-Honkanen, H., Herukka, S.-K., et al. (2020) · Journal of Neurology · doi:10.1007/s00415-019-09567-8 · Cited by 112 · see citing works
Chronic traumatic encephalopathy: Where are we and where are we going?
Mez, J., Stern, R. A., & McKee, A. C. (2013) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-013-0407-7 · Cited by 120 · see citing works
Kishita, N., Hammond, L., Dietrich, C. M., Mioshi, E., Haapala, I., Biggs, S., & Kurrle, S. (2018) · International Psychogeriatrics · doi:10.1017/s1041610218000947 · Cited by 119 · see citing works
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Quantifying the Eating Abnormalities in Frontotemporal Dementia
Ahmed, R. M., Irish, M., Kam, J., van Keizerswaard, J., Bartley, L., Samaras, K., Hodges, J. R., & Piguet, O. (2014) · JAMA Neurology · doi:10.1001/jamaneurol.2014.1931 · Cited by 120 · see citing works
Self-citationCognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage
Ranasinghe, K. G., Rankin, K. P., Lobach, I. V., Kramer, J. H., Sturm, V. E., Bettcher, B. M., Possin, K., Christine You, S., Lamarre, A. K., Shany-Ur, T., et al. (2016) · Neurology · doi:10.1212/wnl.0000000000002373 · Cited by 112 · see citing works
Self-citationSerum neurofilament light chain in behavioral variant frontotemporal dementia
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Self-citationReduced CSF p-Tau <sub>181</sub> to Tau ratio is a biomarker for FTLD-TDP
Hu, W. T., Watts, K., Grossman, M., Glass, J., Lah, J. J., Hales, C., Shelnutt, M., Van Deerlin, V., Trojanowski, J. Q., & Levey, A. I. (2013) · Neurology · doi:10.1212/01.wnl.0000436625.63650.27 · Cited by 113 · see citing works
Self-citation
- Authors
- Rascovsky, K., Hodges, J. R., Knopman, D., Mendez, M. F., Kramer, J. H., Neuhaus, J., van Swieten, J. C., Seelaar, H., Dopper, E. G. P., Onyike, C. U., Hillis, A. E., Josephs, K. A., Boeve, B. F., Kertesz, A., Seeley, W. W., Rankin, K. P., Johnson, J. K., Gorno-Tempini, M.-L., Rosen, H., Prioleau-Latham, C. E., Lee, A., Kipps, C. M., Lillo, P., Piguet, O., Rohrer, J. D., Rossor, M. N., Warren, J. D., Fox, N. C., Galasko, D., Salmon, D. P., Black, S. E., Mesulam, M., Weintraub, S., Dickerson, B. C., Diehl-Schmid, J., Pasquier, F., Deramecourt, V., Lebert, F., Pijnenburg, Y., Chow, T. W., Manes, F., Grafman, J., Cappa, S. F., Freedman, M., Grossman, M., & Miller, B. L.
- Affiliations
- University of Pennsylvania (US)
- UNSW Sydney (AU)
- Mayo Clinic (US)
- University of California, Los Angeles (US)
- University of California, San Francisco (US)
- Erasmus MC (NL)
- Johns Hopkins University (US)
- Western University (CA)
- University Hospital Southampton NHS Foundation Trust (GB)
- UCL Queen Square Institute of Neurology (GB)
- University of California San Diego (US)
- Sunnybrook Health Science Centre (CA)
- Northwestern University (US)
- Université de Lille (FR)
- Amsterdam UMC Location Vrije Universiteit Amsterdam (NL)
- University of Toronto (CA)
- Favaloro University (AR)
- Kessler Foundation (US)
- Vita-Salute San Raffaele University (IT)
- ORCID
- Hodges, John R.
- Knopman, David
- Mendez, Mario F.
- Kramer, Joel H.
- Neuhaus, John
- Seelaar, Harro
- Dopper, Elise G. P.
- Onyike, Chiadi U.
- Hillis, Argye E.
- Josephs, Keith A.
- Boeve, Bradley F.
- Kertesz, Andrew
- Seeley, William W.
- Rankin, Katherine P.
- Johnson, Julene K.
- Kipps, Christopher M.
- Lillo, Patricia
- Piguet, Olivier
- Rohrer, Jonathan D.
- Rossor, Martin N.
- Warren, Jason D.
- Fox, Nick C.
- Galasko, Douglas
- Salmon, David P.
- Black, Sandra E.
- Mesulam, Marsel
- Pasquier, Florence
- Deramecourt, Vincent
- Lebert, Florence
- Pijnenburg, Yolande
- Chow, Tiffany W.
- Manes, Facundo
- Grafman, Jordan
- Cappa, Stefano F.
- Freedman, Morris
- Grossman, Murray
- Miller, Bruce L.
- Venue
- Brain
- Publisher
- Oxford University Press (OUP)
- Published
- 2011-08-02
- Volume
- 134
- Issue
- 9
- Pages
- 2456-2477
- ISSN
- 0006-8950 (ISSN-L)
- 1460-2156
- PMC
- PMC3170532
- OpenAlex
- W2128030851
- Cited by
- 5,221
- References
- 179
- Citations by year
- 2026: 333 · 2025: 445 · 2024: 486 · 2023: 427 · 2022: 456 · 2021: 460 · 2020: 459 · 2019: 355 · 2018: 338 · 2017: 325 · 2016: 293 · 2015: 276 · 2014: 225 · 2013: 208 · 2012: 115
- FWCI
- 122.40
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Brain (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Amsterdam UMC (VU Amsterdam) - Institutional Repository (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
- Iris (University of Trento) (submittedVersion, other-oa)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Digital Academic REpository of VU University Amsterdam (Vrije Universiteit Amsterdam) (submittedVersion)
- ePrints Soton (University of Southampton) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- mediaTUM (Technical University of Munich) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Funders
- U.S. Department of Veterans Affairs
- Alzheimer's Association
- Bristol-Myers Squibb (AG17586)
- Eli Lilly and Company (AG17586)
- Pfizer (AG17586)
- GlaxoSmithKline
- Sanofi
- Heart and Stroke Foundation of Canada
- Baxter International
- Wellcome Trust (091673)
- Canadian Stroke Network
- Myriad Genetics
- Alzheimer Society
- National Institute for Health and Care Research (NF-SI-0508-10123)
- University of Cambridge
- University of Oxford
- Elan
- Fonds Wetenschappelijk Onderzoek (P6/29)
- Eisai
- KU Leuven
- Vlaamse regering
- H. Lundbeck A/S
- National Institutes of Health (DC008552, AG016574, AG023501, AG019724, NS53488, AG06786, AG 17586, AG13854, AG15116, AG16574, AG32953)
- Canadian Institutes of Health Research (13129)
- Natural Sciences and Engineering Research Council of Canada
- National Institute on Aging (AG13854, P50‐AG16574, R01-AG15116, U01AG06786, RO1 AG15866, AG 16574, P01 AG-17586, U01AG006786, R01AG015116, P01AG017586, AG17586, R01AG034499, R01AG032306, AG019724, P01-AG32953, P01AG032953, P01AG019724, P50AG023501, P50AG016574)
- National Institute on Deafness and Other Communication Disorders (AG13854, DC008552, R01DC005375)
- National Institute of Neurological Disorders and Stroke (R01-NS44266, R01NS047691, R01NS044266)
- National Center for Research Resources (UL1RR025741)
- 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, diagnostic criteria, sensitivity, frontotemporal lobar degeneration, disinhibition, apathy, hyperorality, dysexecutive syndrome, functional disability, neuroimaging, diagnostic accuracy
- MeSH terms
- Aged, Behavior, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Sensitivity and Specificity, Reproducibility of Results, Guidelines as Topic, Frontotemporal Dementia
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