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.

Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes

“…With disease progression many patients with nfvPPA develop the phenotype of PSP or CBS, which is an adverse prognostic sign [44][45][46][47]. In keeping with these observations, previous survival analyses of frontotemporal dementia (bvFTD and PPA) have shown that reduced letter fluency, motor cortex atrophy and brainstem hypoperfusion were associated with reduced survival [5,48,49]. Our results go beyond these findings, suggesting that development of motor impairments, irrespective of diagnostic group, is an adverse prognostic sign.…”

“…Prognosis in syndromes associated with frontotemporal lobar degeneration (FTLD) is highly variable and difficult to predict. Disease duration is not fully explained by the diagnostic categorisation to behavioural variant frontotemporal dementia (bvFTD), non-fluent (nfvPPA) or semantic (svPPA) variants of primary progressive aphasia, progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) [1][2][3][4][5]. Better prognostic models would aid both trial design and clinical management.…”

“…Mortality is a definite endpoint in FTD, PSP and CBS. However, these disorders also engender dependency and caregiver burden [5,[12][13][14][15]. Community-based studies suggest increased dependency, due to cognitive or physical impairment, predicts care home admission [16,17].…”

Old Age Dementia: Clinical Patterns of Progression. Part 1

“…Второе место после БКЯ в группе деменций с быстрым прогрессированием почти единодушно отводится лобно-височной деменции (ЛВД) и, прежде всего, ее поведенческому варианту [58][59][60][61][62]. Однако исследователи признают, что верификация диагноза ЛВД нередко следует за диагнозом психического заболевания, что искажает представление о скорости прогрессировании болезни [58,63].…”

“…По мнению авторов, они отражают бóльшую скорость во многом естественного прогрессирования ЛВД в сопоставлении с БА. Дефицит беглости письменной речи и более выраженная атрофия в двигательных отделах коры также рассматриваются в качестве предикторов быстрого прогрессирования ЛВД [62].…”