Clinicopathologic differences among patients with behavioral variant frontotemporal dementia
Open accessAbstract
OBJECTIVE: To characterize the presenting symptoms and signs of patients clinically diagnosed with behavioral variant frontotemporal dementia (bvFTD) and who had different neuropathologic findings on autopsy. METHODS: This study reviewed all patients entered as clinical bvFTD in the National Alzheimer's Coordinating Center's database and who had both clinical and neuropathologic data from 2005 to 2011. Among the 107 patients identified, 95 had unambiguous pathologic findings, including 74 with frontotemporal lobar degeneration (bvFTD-FTLD) and 21 with Alzheimer disease (bvFTD-AD). The patients with bvFTD-FTLD were further subdivided into τ-positive (n = 23) or τ-negative (n = 51) histopathology subgroups. Presenting clinical signs and symptoms were compared between these neuropathologic groups. RESULTS: The patients with bvFTD-FTLD were significantly more likely than patients with bvFTD-AD to have initially predominant personality changes and poor judgment/decision-making. In contrast, patients with bvFTD-AD were more likely than patients with bvFTD-FTLD to have memory difficulty and delusions/hallucinations and agitation. Within the bvFTD-FTLD group, the τ-positive subgroup had more patients with initial behavioral problems and personality change than the τ-negative subgroup, who, in turn, had more patients with initial cognitive impairment and speech problems. CONCLUSION: During life, patients with AD pathology may be misdiagnosed with bvFTD if they have an early age at onset and prominent neuropsychiatric features despite having greater memory difficulties and more intact personality and executive functions than patients with bvFTD-FTLD. Among those with FTLD pathology, patients with τ-positive bvFTD were likely to present with behavior/personality changes. These findings offer clues for antemortem recognition of neuropathologic subtypes of bvFTD.
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In fvAD, memory impairment is the first abnormality in up to 85% of patients, with the remainder developing memory impairment within 3 years from symptom onset.
“Conversely, in fvAD memory impairment is the first abnormality in up to 85% of patients (Fig. 4), with the remainder developing memory impairment within 3 years from symptom onset [1, 22, 23].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [22]
The Results (“First and current symptoms”; page not given) report that memory deficits were the first cognitive symptom in 8 of 21 patients with bvFTD and Alzheimer pathology (38.1%). This supports memory impairment as an initial symptom in some patients, but not the claimed figure of up to 85%; our copy also does not establish that the remainder developed it within three years of symptom onset. (LLM, conf=0.88)
Experimental — accuracy not yet measuredPersonality changes are more common in patients with bvFTD than fvAD, with differences in the nature of the personality change shedding light on the underlying diagnosis.
“Personality changes are more common in patients with bvFTD than fvAD, with differences in the nature of the personality change shedding light on the underlying diagnosis [10, 22].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [22]
The Results passage (indexed under GLOSSARY; page not provided) reports significantly more personality change in patients with bvFTD-FTLD than in those clinically diagnosed with bvFTD who had AD pathology. This supports a related diagnostic distinction, but the study did not directly compare bvFTD with a separately defined fvAD group or characterize differences in the nature of personality change between those groups. (LLM, conf=0.85)
Experimental — accuracy not yet measuredStudies have demonstrated that executive or behavioral dysfunction typically develop after memory deficits in fvAD.
“While not apparent in our cases, studies have demonstrated that executive or behavioral dysfunction typically develop after memory deficits in fvAD [1, 22, 23].”
From Diagnosing the frontal variant of Alzheimer’s disease: a clinician’s yellow brick road (2017) · cites this paper as [22]
The 'First and current symptoms' passage (section labeled GLOSSARY; page unavailable) reports that patients diagnosed with bvFTD who had AD pathology were more likely than those with FTLD pathology to have memory deficits as their first cognitive symptom, while fewer had personality change as a first behavioral symptom. It does not establish that executive or behavioral dysfunction typically develops after memory deficits within individual patients with frontal-variant AD. (LLM, conf=0.82)
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- Authors
- Mendez, M. F., Joshi, A., Tassniyom, K., Teng, E., & Shapira, J. S.
- Affiliations
- University of California, Los Angeles (US)
- Venue
- Neurology
- Published
- 2013-02-05
- Volume
- 80
- Issue
- 6
- Pages
- 561-568
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC3589292
- OpenAlex
- W2130642198
- Cited by
- 96
- References
- 33
- Citations by year
- 2025: 3 · 2024: 5 · 2023: 7 · 2022: 10 · 2021: 6 · 2020: 12 · 2019: 5 · 2018: 5 · 2017: 8 · 2016: 4 · 2015: 7 · 2014: 12 · 2013: 11
- FWCI
- 8.06
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Neurology (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute on Aging (U01AG016976, P50AG016570, P50-AG 16570, R01AG034499)
- 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, frontotemporal lobar degeneration, Alzheimer's disease, tau pathology, clinical features, personality change, misdiagnosis, neuropsychiatric symptoms, delusions and hallucinations, memory impairment
- MeSH terms
- Aged, Alzheimer Disease, Brain, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Frontotemporal Dementia
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