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Journal Articles Annals of Clinical and Translational Neurology Year : 2022

The CBI‐R detects early behavioural impairment in genetic frontotemporal dementia

1 UCL Queen Square Institute of Neurology
2 LSHTM - London School of Hygiene and Tropical Medicine
3 Erasmus MC - Erasmus University Medical Center [Rotterdam]
4 Donostia Universitary Hospital [Spain]
5 Biodonostia Health Research Institute
6 IDIBAPS - Institut d'Investigacions Biomèdiques August Pi i Sunyer
7 CHU de Québec–Université Laval
8 Karolinska Institutet [Stockholm]
9 KUH - Karolinska University Hospital [Solna, Sweden]
10 SRI - Sunnybrook Research Institute [Toronto]
11 University of Toronto
12 CAM - University of Cambridge [UK]
13 UniBs - Università degli Studi di Brescia = University of Brescia
14 UWO - University of Western Ontario
15 Hertie Institute for Clinical Brain Research [Tubingen]
16 University of Tübingen
17 DZNE - Deutsches Zentrum für Neurodegenerative Erkrankungen [Ulm]
18 Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico
19 Centro Dino Ferrari [Milano]
20 KU Leuven - Catholic University of Leuven = Katholieke Universiteit Leuven
21 University Hospitals Leuven [Leuven]
22 LBI - Leuven Brain Institute [Leuven, Belgium]
23 ULISBOA - Universidade de Lisboa = University of Lisbon
24 University of Oxford
25 Imperial College London
26 WMIC - Wolfson Molecular Imaging Centre
27 Universität Duisburg-Essen = University of Duisburg-Essen [Essen]
28 MUHC - McGill University Health Center [Montreal]
29 Montreal Neurological Institute and Hospital
30 ICM - Institut du Cerveau = Paris Brain Institute
31 CHU Pitié-Salpêtrière [AP-HP]
32 Hospital of the University of Coimbra
33 UC - University of Coimbra [Portugal]
34 LilNCog - Lille Neurosciences & Cognition - U 1172
35 CNRMAJ - Centre national de référence pour les malades Alzheimer jeunes
36 LMU - Ludwig Maximilian University [Munich] = Ludwig Maximilians Universität München
37 DZNE - German Research Center for Neurodegenerative Diseases - Deutsches Zentrum für Neurodegenerative Erkrankungen
38 SyNery - Munich Cluster of Systems Neurology
39 UniFI - Università degli Studi di Firenze = University of Florence = Université de Florence
Lucy L Russell
Robert Laforce
Elizabeth Finger
Markus Otto
  • Function : Author


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.
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Dates and versions

hal-04537252 , version 1 (08-04-2024)



Annabel Nelson, Lucy L Russell, Georgia Peakman, Rhian S Convery, Arabella Bouzigues, et al.. The CBI‐R detects early behavioural impairment in genetic frontotemporal dementia. Annals of Clinical and Translational Neurology, 2022, 9 (5), pp.644-658. ⟨10.1002/acn3.51544⟩. ⟨hal-04537252⟩
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