Cited by 31Dementia and Cognitive Impairment ResearchAmyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatments

Predictors of survival and progression in behavioural variant frontotemporal dementia

Agarwal, S., Ahmed, R. M., D'Mello, M., Foxe, D., Kaizik, C., Kiernan, M. C., Halliday, G. M., Piguet, O. +1 more·2019·European Journal of Neurology

Abstract

Background and purpose: Predicting the course of behavioural variant frontotemporal dementia (bv FTD ) remains a major clinical challenge. This study aimed to identify factors that predict survival and clinical progression in bv FTD . Methods: Consecutive patients with clinically probable bv FTD 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 bv FTD , 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 bv FTD . Our findings are likely to have an impact on prognostication and care planning in this difficult disease.

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Crossref · 10.1111/ene.13887

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Authors
Agarwal, S., Ahmed, R. M., D'Mello, M., Foxe, D., Kaizik, C., Kiernan, M. C., Halliday, G. M., Piguet, O., & Hodges, J. R.
Affiliations
  • The University of Sydney (AU)
  • School of Psychology and Brain and Mind Centre The University of Sydney Sydney New South Wales
Corresponding author
Agarwal, S.
Venue
European Journal of Neurology
Publisher
Wiley
Published
2019-01-29
Volume
26
Issue
5
Pages
774-779
ISSN
  • 1351-5101 (ISSN-L)
  • 1468-1331
OpenAlex
W2904582713
Cited by
31
References
36
Citations by year
2026: 3 · 2025: 5 · 2024: 2 · 2022: 5 · 2021: 9 · 2020: 4 · 2019: 3
FWCI
3.14
Type
journal article
Language
en
Open access
closed
Locations
  • European Journal of Neurology (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Health and Medical Research Council (NHMRC‐APP1037746)
  • Australian Research Council
  • Royal Society of Medicine
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Keywords
behavioral variant frontotemporal dementia, survival prediction, disease progression, prognostic factors, phonemic fluency, behavioural symptoms, disease duration, nursing home admission
MeSH terms
Age Factors, Aged, Atrophy, Female, Follow-Up Studies, Gyrus Cinguli, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Motor Cortex, Mutation, Neuropsychological Tests, Nursing Homes, Predictive Value of Tests, Prospective Studies, Cohort Studies, Survival Analysis, Disease Progression, Kaplan-Meier Estimate, Frontotemporal Dementia

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