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Neurology. 2009 Nov 17;73(20):1656-61. doi: 10.1212/WNL.0b013e3181c1dee7.

Determinants of survival in behavioral variant frontotemporal dementia.

Author information

1
Prince of Wales Medical Research Institute, Randwick, Sydney, NSW, Australia.

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.

PMID:
19917988
PMCID:
PMC2881857
DOI:
10.1212/WNL.0b013e3181c1dee7
[Indexed for MEDLINE]
Free PMC Article

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