Determinants of survival in behavioral variant frontotemporal dementia
Open accessAbstract
BACKGROUND: Behavioral variant frontotemporal dementia (bvFTD) is a common cause of younger onset dementia. Little is known about its rate of progression but a recently identified subgroup seems to have an excellent prognosis. Other determinants of survival are unclear. METHODS: We analyzed survival in a large group of clinically diagnosed bvFTD patients (n = 91) with particular attention to demographic and clinical features at presentation. Of the 91 cases, 50 have died, with pathologic confirmation in 28. RESULTS: Median survival in the whole group was 9.0 years from symptom onset, and 5.4 years from diagnosis. After the exclusion of 24 "phenocopy" cases, the analysis was repeated in a subgroup of 67 patients. The mean age at symptom onset of the pathologic group was 58.5 years and 16% had a positive family history. Their median survival was 7.6 years (95% confidence interval [CI] 6.6-8.6) from symptom onset and 4.2 years (95% CI 3.4-5.0) from diagnosis. The only factor associated with shorter survival was the presence of language impairment at diagnosis. CONCLUSIONS: Patients with definite frontotemporal dementia have a poor prognosis which is worse if language deficits are also present. This contrasts with the extremely good outcome in those with the phenocopy syndrome: of our 24 patients only 1 has died (of coincident pathology) despite, in some cases, many years of follow-up.
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Language impairment was associated with mortality.
“Also, language impairment was associated with mortality (Garcin et al., 2009).”
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 Garcin et al., 2009
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- Authors
- Garcin, B., Lillo, P., Hornberger, M., Piguet, O., Dawson, K., Nestor, P. J., & Hodges, J. R.
- Affiliations
- University of Cambridge (GB)
- Venue
- Neurology
- Published
- 2009-11-17
- Volume
- 73
- Issue
- 20
- Pages
- 1656-1661
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC2881857
- OpenAlex
- W2083348543
- Cited by
- 94
- References
- 24
- Citations by year
- 2025: 4 · 2024: 2 · 2023: 2 · 2022: 7 · 2021: 9 · 2020: 7 · 2019: 6 · 2018: 2 · 2017: 9 · 2016: 6 · 2015: 12 · 2014: 4 · 2013: 8 · 2012: 6
- FWCI
- 3.29
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Neurology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- UEA Digital Repository (University of East Anglia) (submittedVersion)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- behavioral variant frontotemporal dementia, survival, prognosis, language impairment, family history (medicine), age at symptom onset, pathologic confirmation, disease progression
- MeSH terms
- Family, Female, Follow-Up Studies, Humans, Language Disorders, Male, Middle Aged, Neuropsychological Tests, Prognosis, Retrospective Studies, Time Factors, Kaplan-Meier Estimate, Frontotemporal Dementia
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