The CBI-R detects early behavioural impairment in genetic frontotemporal dementia.
Authors
Nelson, Annabel
Peakman, Georgia
Convery, Rhian S
Bouzigues, Arabella
Greaves, Caroline V
Cash, David M
van Swieten, John C
Jiskoot, Lize
Moreno, Fermin
Graff, Caroline
Masellis, Mario
Tartaglia, Maria Carmela
Rowe, James B
Vandenberghe, Rik
de Mendonça, Alexandre
Butler, Chris R
Gerhard, Alexander
Santana, Isabel
Pasquier, Florence
Levin, Johannes
Otto, Markus
Sorbi, Sandro
Genetic FTD Initiative (GENFI)
Publication Date
2022-05Journal Title
Ann Clin Transl Neurol
ISSN
2328-9503
Publisher
Wiley
Language
en
Type
Article
This Version
AO
VoR
Metadata
Show full item recordCitation
Nelson, A., Russell, L. L., Peakman, G., Convery, R. S., Bouzigues, A., Greaves, C. V., Bocchetta, M., et al. (2022). The CBI-R detects early behavioural impairment in genetic frontotemporal dementia.. Ann Clin Transl Neurol https://doi.org/10.1002/acn3.51544
Description
Funder: UK Dementia Research Institute
Funder: NIHR UCL/H Biomedical Research Centre
Funder: The Wolfson Foundation
Funder: Brain Research UK; Id: http://dx.doi.org/10.13039/100013790
Funder: Alzheimer’s Research UK; Id: http://dx.doi.org/10.13039/501100002283
Abstract
INTRODUCTION: Behavioural dysfunction is a key feature of genetic frontotemporal dementia (FTD) but validated clinical scales measuring behaviour are lacking at present. METHODS: 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 (controls). All mutation carriers were stratified according to their global CDR plus NACC FTLD score into three groups: asymptomatic (CDR = 0), prodromal (CDR = 0.5) and symptomatic (CDR = 1+). Mixed-effects models adjusted for age, education, sex and family clustering were used to compare between the groups. Neuroanatomical correlates of the individual domains were assessed within each genetic group. RESULTS: CBI-R total scores were significantly higher in all CDR 1+ mutation carrier groups compared with 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)]. The C9orf72 and GRN 0.5 groups scored significantly higher than the controls. The greatest impairment was seen in the Motivation domain for the C9orf72 and GRN symptomatic groups, whilst in the symptomatic MAPTgroup, the highest-scoring domains were Stereotypic and Motor Behaviours and Memory and Orientation. Neural correlates of each CBI-R domain largely overlapped across the different mutation carrier groups. CONCLUSIONS: The CBI-R detects early behavioural change in genetic FTD, suggesting that it could be a useful measure within future clinical trials.
Keywords
Research Article, Research Articles
Sponsorship
Wellcome Trust (220258/Z/20/Z)
Identifiers
acn351544, acn3-2022-01-0023.r1
External DOI: https://doi.org/10.1002/acn3.51544
This record's URL: https://www.repository.cam.ac.uk/handle/1810/335605
Rights
Licence:
http://creativecommons.org/licenses/by/4.0/
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