The Cambridge Behavioural Inventory (revised) detects early behavioural and functional impairment in genetic frontotemporal dementia within the GENFI cohort

Background Behavioural dysfunction is a key feature of genetic frontotemporal dementia (FTD) but validated clinical scales measuring behaviour are lacking at present. Method We assessed behaviour using the revised version of the Cambridge Behavioural Inventory (CBI‐R) in 733 participants from the Ge...

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Published in:Alzheimer's & dementia 2021-12, Vol.17 (S6), p.n/a
Main Authors: Nelson, Annabel, Russell, Lucy L, Peakman, Georgia, Greaves, Caroline V, Convery, Rhian S, Rohrer, Jonathan D
Format: Article
Language:English
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Summary:Background Behavioural dysfunction is a key feature of genetic frontotemporal dementia (FTD) but validated clinical scales measuring behaviour are lacking at present. Method We assessed behaviour using the revised version of the Cambridge Behavioural Inventory (CBI‐R) in 733 participants from the Genetic FTD Initiative study: 466 mutation carriers (195 C9orf72, 76 MAPT, 195 GRN) and 267 non‐mutation carriers (healthy controls). All mutation carriers were stratified according to their CDR® plus NACC FTLD into three groups: asymptomatic (CDR=0), mildly symptomatic (CDR=0.5) and fully symptomatic (CDR=1+). CBI‐R total scores were compared between mutation carrier groups using a mixed effects model that adjusted for age, education, sex and family clustering, with 95% bootstrapped confidence intervals with 2000 repetitions to adjust for non‐normally distributed data. We used the same mixed effects model to run a within‐group analysis between the 10 CBI‐R domains in CDR 1+ groups. Spearman rank correlations were run to assess the relationship of the CDR® plus NACC FTLD SOB and FRS scale with the CBI‐R total in CDR 1+ mutation carrier groups. Result CBI‐R total scores were significantly higher in all CDR 1+ mutation carrier groups compared to controls (C9orf72 mean 70.5 (standard deviation 27.8), GRN 56.2 (33.5), MAPT 62.1 (36.9)), as well as their respective CDR 0.5 groups (C9orf72 13.5 (14.4), GRN 13.3 (13.5), MAPT 9.4 (10.4)) and CDR 0 groups (C9orf72 6.0 (7.9), GRN 3.6 (6.0), MAPT 8.5 (13.3)). Both C9orf72 and GRN 0.5 groups scored significantly higher than controls and their respective CDR 0 groups. Motivation and Memory were the highest scoring domains in both C9orf72 and GRN CDR 1+ groups, and in the MAPT group it was Stereotypic Behaviour and Memory. There was a positive correlation between the CBI‐R scores and the CDR® plus NACC FTLD SOB scores in all mutation carrier groups (C9orf72: rho= 0.8, p
ISSN:1552-5260
1552-5279
DOI:10.1002/alz.051302