Determinants of survival in behavioral variant frontotemporal dementia

B. Garcin, P. Lillo, M. Hornberger, O. Piguet, K. Dawson, P. J. Nestor, J. R. Hodges

Research output: Contribution to journalArticlepeer-review

69 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1656-1661
Number of pages6
JournalNeurology
Volume73
Issue number20
DOIs
Publication statusPublished - Nov 2009
Externally publishedYes

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