Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes
Accepted version
Peer-reviewed
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Abstract
Objective
To test the hypothesis that in syndromes associated with frontotemporal lobar degeneration, behavioural impairment predicts loss of functional independence and motor clinical features predict mortality, irrespective of syndrome subtype.Method
We used a transdiagnostic approach to survival in an epidemiological cohort, testing the association between clinical features, independence and survival in patients with clinical diagnoses of behavioural variant frontotemporal dementia (n=64), non-fluent variant primary progressive aphasia (n=36), semantic variant primary progressive aphasia (n=25), progressive supranuclear palsy (n=101) and corticobasal syndrome (n=68). A principal components analysis identified six dimensions of clinical features. Using Cox proportional hazards and logistic regression we identified the association between each of these dimensions and functionally independent survival (time from clinical assessment to care home admission), and absolute survival (time to death). Analyses adjusted for the covariates of age, gender and diagnostic group. Secondary analysis excluded specific diagnostic groups.Results
Behavioural disturbance, including impulsivity and apathy, was associated with reduced functionally independent survival (OR 2.46, p<0.001), even if patients with bvFTD were removed from the analysis. Motor impairments were associated with reduced absolute survival, even if patients with PSP and CBS were removed from the analysis.Conclusion
Our results may help individualised prognostication and planning of disease-modifying trials and support a transdiagnostic approach to symptomatic treatment trials in patients with clinical syndromes associated with frontotemporal lobar degeneration.Description
Keywords
5202 Biological Psychology, 42 Health Sciences, 52 Psychology, Dementia, Aphasia, Neurodegenerative, Clinical Trials and Supportive Activities, Acquired Cognitive Impairment, Rare Diseases, Aging, Brain Disorders, Frontotemporal Dementia (FTD), Clinical Research, Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD), 2.1 Biological and endogenous factors, 2 Aetiology, Neurological, 3 Good Health and Well Being
Journal Title
Journal of Neurology, Neurosurgery and Psychiatry
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0022-3050
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BMJ Publishing Group
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All rights reserved
Sponsorship
Wellcome Trust (103838/Z/14/Z)
Cambridge University Hospitals NHS Foundation Trust (CUH) (146281)
MRC (unknown)
PSP Association (unknown)
(unknown)
Medical Research Council (MC_U105597119)
Medical Research Council (MC_UU_00005/12)
Cambridge University Hospitals NHS Foundation Trust (CUH) (146281)
MRC (unknown)
PSP Association (unknown)
(unknown)
Medical Research Council (MC_U105597119)
Medical Research Council (MC_UU_00005/12)
This study was funded by the Holt Fellowship (AGM), Wellcome Trust (JBR, 103838), the PSP Association, the Medical Research Council, the National Institute for Health Research Cambridge Biomedical Research Centre including the Cambridge Brain Bank; and the Cambridge Centre for Parkinson Plus.