The Cambridge Behavioural Inventory (revised) detects early behavioural and functional impairment in genetic frontotemporal dementia within the GENFI cohort. (31st December 2021)

• Record Type: Journal Article
• Authors: Nelson, Annabel; Russell, Lucy L; Peakman, Georgia; Greaves, Caroline V; Convery, Rhian S; Rohrer, Jonathan D
• Is Part Of: Alzheimer's & dementia. Volume 17(2021)Supplement 6
• Page Start: n/a
• Page End: n/a
• Publication Date: 2021-12-31
• Subjects: Alzheimer's disease -- Periodicals; Alzheimer Disease -- Periodicals; Dementia -- Periodicals; Démence; Maladie d'Alzheimer; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); 616.83
• DOI: 10.1002/alz.051302
• Languages: English
• ISSNs: 1552-5260

Abstract

Abstract: 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<0.001, GRN : rho= 0.8, p<0.001, MAPT : rho= 0.6, p<0.001), and a negative correlation between CBI‐R scores and FTD Rating Scale (FRS) scores ( C9orf72 ; rho= ‐0.9, p<0.001, GRN ; rho= ‐0.9, p<0.001, MAPT ; rho= ‐0.9, p<0.001). Conclusion: The CBI‐R detects early behavioural change in genetic FTD, particularly in those with C9orf72 and GRN mutations.