Cognition and neuropsychiatry in behavioral variant frontotemporal dementia by disease stage
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OBJECTIVE: To characterize the cognitive and neuropsychiatric symptoms of patients with behavioral variant frontotemporal dementia (bvFTD) over the natural course of the disease. METHODS: We examined the initial and subsequent neuropsychological test performance and neuropsychiatric symptoms in a large cohort of patients with bvFTD (n = 204) across progressive stages of disease as measured by the Clinical Dementia Rating (CDR). We also compared cognitive and neuropsychiatric impairments of patients with bvFTD to those of an age-matched cohort with Alzheimer disease (AD) dementia (n = 674). RESULTS: At the earliest stage (CDR = 0.5), patients with bvFTD had profound neuropsychiatric disturbances, insensitivity to errors, slower response times, and poor naming, with intact attention span, memory, and facial affect naming. Tests continuing to show progressive, statistically significant stepwise declines after the CDR = 1 stage included free recall, visuoconstruction, set-shifting, error insensitivity, semantic fluency, design fluency, emotion naming, calculations, confrontation naming, syntax comprehension, and verbal agility. At CDR = 0.5, patients with bvFTD significantly outperformed patients with AD in episodic memory and were faster in set-shifting, while scoring quantitatively worse in lexical fluency, emotion naming, and error sensitivity. The overall rate of disease progression in bvFTD was more rapid than in AD. CONCLUSION: There are distinct patterns of cognitive deficits differentiating the earlier and later disease stages in bvFTD, with the pattern of cognitive decline revealing in greater detail the natural history of the disease. These cognitive symptoms are readily apparent clinical markers of dysfunction in the principal brain networks known to undergo molecular and anatomical changes in bvFTD, thus are important indicators of the evolving pathology in individual patients.
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Growing evidence shows that tests of ventromedial prefrontal cortex function (such as error sensitivity, verbal fluency, inhibition, and decision-making and neuroeconomics derived tasks) are more sensitive than dorsolateral prefrontal tests (such as cognitive control and attention).
“Importantly, growing evidence shows that tests of ventromedial prefrontal cortex function (such as error sensitivity, 33 verbal fluency, 33 inhibition, 34 and decisionmaking and neuroeconomics derived tasks 35 ) are more sensitive than dorsolateral prefrontal tests (such as cognitive control and attention).33”
From Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations (2022) · cites this paper as [33]
The Domain-specific cognitive dysfunction section (page not supplied) reports that error insensitivity and verbal fluency were markedly impaired in early bvFTD, while cognitive control was low-average or normal; the Discussion also describes attention as intact. But the Discussion links error insensitivity to right lateral prefrontal networks, not specifically to ventromedial prefrontal cortex function. This paper therefore supports the early-test-sensitivity comparison, but not the claimed anatomical classification. (LLM, conf=0.88)
Experimental — accuracy not yet measuredAssociations between NPI/CUSPAD and disease severity were inconsistent, and the evidence as a bvFTD-specific progression marker was insufficient.
“While parts of AD-oriented neuropsychiatric scales, such as the NPI and the Columbia University Scale for Psychopathology in Alzheimer’s Disease (CUSPAD), predicted cognitive and functional decline in FTD (2.5 years) (Santacruz Escudero et al., 2019), associations with disease severity were inconsistent (Josephs et al., 2011; Kazui et al., 2016; Ranasinghe et al., 2016) and the evidence as bvFTD-s”
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 Ranasinghe et al., 2016
The earliest stage was characterized by error insensitivity, slower response time and poor naming, while later stages showed deterioration in a range of executive functions, language and memory, visuo-construction and calculations.
“The earliest stage was characterized by error insensitivity, slower response time and poor naming, while later stages showed deterioration in a range of executive functions, language and memory, visuo-construction and calculations (Ranasinghe et al., 2016).”
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 Ranasinghe et al., 2016
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References 46 · Cited by 112
Research works printed but not linked: 1
- [31]31. Rabinovici GD, Miller BL. Frontotemporal lobar degen- eration: epidemiology, pathophysiology, diagnosis and management. CNS Drugs 2010;24:375-398.
- Authors
- 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., Stephens, M. L., Perry, D. C., Lee, S. E., Miller, Z. A., Gorno-Tempini, M. L., Rosen, H. J., Boxer, A., Seeley, W. W., Rabinovici, G. D., Vossel, K. A., & Miller, B. L.
- Affiliations
- Gladstone Institutes (US)
- From the Memory and Aging Center (K.G.R., K.P.R., I.V.L., J.H.K., V.E.S., B.M.B., K.P., S.C.Y., A.K.L., T.S.-U., M.L.S., D.C.P., S.E.L., Z.A.M., M.L.G.-T., H.J.R., A.B., W.W.S., G.D.R., K.A.V., B.L.M.), Department of Neurology, University of California, San Francisco; Departments of Neurosurgery and Neurology (B.M.B.), University of Colorado Anschutz School of Medicine, Aurora; and Gladstone Institute of Neurological Disease (K.A.V.), CA.
- ORCID
- Venue
- Neurology
- Published
- 2016-02-16
- Volume
- 86
- Issue
- 7
- Pages
- 600-610
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC4762418
- OpenAlex
- W2304803376
- Cited by
- 112
- References
- 46
- Citations by year
- 2026: 9 · 2025: 15 · 2024: 11 · 2023: 12 · 2022: 14 · 2021: 12 · 2020: 11 · 2019: 11 · 2018: 4 · 2017: 11 · 2016: 2
- FWCI
- 8.29
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Neurology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- Funders
- Larry L. Hillblom Foundation
- FORUM Pharmaceuticals
- National Institute for Health and Care Research
- National Institutes of Health
- Tau Consortium
- NIHR Cambridge Biomedical Research Centre
- National Institute on Aging (K23AG021606, K23AG038357, R01AG029577, K23AG037566, K23AG042492, K23AG040127, P50AG02350, K23AG048291, P01AG017586, RF1AG029577, K23AG039414, R01AG032306, K23AG045289, K24AG045333, R01AG038791, P01AG019724, P50AG023501)
- National Institute of Neurological Disorders and Stroke (U54NS092089)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Social Sciences › Psychology › Clinical Psychology › Historical and Modern Theater Studies
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- behavioral variant frontotemporal dementia, neuropsychiatric symptoms, cognitive decline, disease progression, Clinical Dementia Rating, set-shifting, semantic fluency, emotion naming, confrontation naming, natural history of disease, cognitive markers
- MeSH terms
- Adult, Aged, Aged, 80 and over, Alzheimer Disease, Cognition, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Severity of Illness Index, Sex Characteristics, Cohort Studies, Disease Progression, Young Adult, Frontotemporal Dementia
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