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Predictors of survival and progression in behavioural variant frontotemporal dementia.

Abstract：

BACKGROUND AND PURPOSE:Predicting the course of behavioural variant frontotemporal dementia (bvFTD) remains a major clinical challenge. This study aimed to identify factors that predict survival and clinical progression in bvFTD. METHODS:Consecutive patients with clinically probable bvFTD were prospectively followed up over an 8-year period. Baseline neuropsychological variables, presence of a known pathogenic frontotemporal dementia gene mutation and a systematic visual magnetic resonance imaging assessment at baseline were examined as candidate predictors using multivariate modelling. RESULTS:After screening 121 cases, the study cohort consisted of 75 patients with probable bvFTD, with a mean age of 60.8 ± 8.5 years, followed up for a mean duration of 7.2 ± 3.5 years from symptom onset. Median survival time from disease onset was 10.8 years and median survival, prior to transition to nursing home, was 8.9 years. A total of 25 of the 75 patients died during the study follow-up period. Survival without dependence was predicted by shorter disease duration at presentation (hazard ratio, 0.49, P = 0.001), greater atrophy in the anterior cingulate cortex (hazard ratio, 1.75, P = 0.047), older age (hazard ratio, 1.07, P = 0.026) and a higher burden of behavioural symptoms (hazard ratio, 1.04, P = 0.015). In terms of disease progression, presence of a known pathogenic frontotemporal dementia mutation (β = 0.46, P < 0.001) was the strongest predictor of progression. Deficits in letter fluency (β = -0.43, P = 0.017) and greater atrophy in the motor cortex (β = 0.51, P = 0.03) were also associated with faster progression. CONCLUSIONS:This study provides novel clinical predictors of survival and progression in bvFTD. Our findings are likely to have an impact on prognostication and care planning in this difficult disease.

journal_name

Eur J Neurol

journal_title

European journal of neurology

authors

Agarwal S,Ahmed RM,D'Mello M,Foxe D,Kaizik C,Kiernan MC,Halliday GM,Piguet O,Hodges JR

doi

10.1111/ene.13887

subject

Has Abstract

pub_date

2019-05-01 00:00:00

pages

774-779

issue

eissn

1351-5101

issn

1468-1331

journal_volume

pub_type

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Predictors of survival and progression in behavioural variant frontotemporal dementia.