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Garcin, B., Lillo, P., Hornberger, M. 
ORCID: https://orcid.org/0000-0002-2214-3788, Piguet, O., Dawson, K., Nestor, P. J. and Hodges, J. R.
(2009)
Determinants of survival in behavioral variant frontotemporal dementia.
Neurology, 73 (20).
pp. 1656-1661.
ISSN 0028-3878
Abstract
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.
| Item Type: | Article |
|---|---|
| Faculty \ School: | Faculty of Medicine and Health Sciences > Norwich Medical School |
| UEA Research Groups: | Faculty of Medicine and Health Sciences > Research Groups > Mental Health Faculty of Medicine and Health Sciences > Research Centres > Lifespan Health |
| Related URLs: | |
| Depositing User: | Pure Connector |
| Date Deposited: | 05 Jan 2016 10:02 |
| Last Modified: | 19 Oct 2023 01:36 |
| URI: | https://ueaeprints.uea.ac.uk/id/eprint/55995 |
| DOI: | 10.1212/WNL.0b013e3181c1dee7 |
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