Determinants of survival in behavioral variant frontotemporal dementia.
Neurology
; 73(20): 1656-61, 2009 Nov 17.
Article
em En
| MEDLINE
| ID: mdl-19917988
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Demência Frontotemporal
Tipo de estudo:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Neurology
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Austrália