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All mutation carriers were stratified according to their global CDR plus NACC FTLD score into three groups: asymptomatic (CDR\u2009=\u20090), prodromal (CDR\u2009=\u20090.5) and symptomatic (CDR\u2009=\u20091+). Mixed\u2010effects 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>CBI\u2010R total scores were significantly higher in all CDR 1+ mutation carrier groups compared with controls [<jats:italic>C9orf72<\/jats:italic> mean 70.5 (standard deviation 27.8), <jats:italic>GRN<\/jats:italic> 56.2 (33.5), <jats:italic>MAPT<\/jats:italic> 62.1 (36.9)] as well as their respective CDR 0.5 groups [<jats:italic>C9orf72<\/jats:italic> 13.5 (14.4), <jats:italic>GRN<\/jats:italic> 13.3 (13.5), <jats:italic>MAPT<\/jats:italic> 9.4 (10.4)] and CDR 0 groups [<jats:italic>C9orf72<\/jats:italic> 6.0 (7.9), <jats:italic>GRN<\/jats:italic> 3.6 (6.0), <jats:italic>MAPT<\/jats:italic> 8.5 (13.3)]. The <jats:italic>C9orf72<\/jats:italic> and <jats:italic>GRN<\/jats:italic> 0.5 groups scored significantly higher than the controls. The greatest impairment was seen in the Motivation domain for the <jats:italic>C9orf72<\/jats:italic> and <jats:italic>GRN<\/jats:italic> symptomatic groups, whilst in the symptomatic <jats:italic>MAPT<\/jats:italic>group, the highest\u2010scoring domains were Stereotypic and Motor Behaviours and Memory and Orientation. Neural correlates of each CBI\u2010R domain largely overlapped across the different mutation carrier groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The CBI\u2010R detects early behavioural change in genetic FTD, suggesting that it could be a useful measure within future clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51544","type":"journal-article","created":{"date-parts":[[2022,3,26]],"date-time":"2022-03-26T16:05:07Z","timestamp":1648310707000},"page":"644-658","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["The <scp>CBI\u2010R<\/scp> detects early behavioural impairment in genetic frontotemporal dementia"],"prefix":"10.1002","volume":"9","author":[{"given":"Annabel","family":"Nelson","sequence":"first","affiliation":[{"name":"Dementia Research Centre, Department of Neurodegenerative Disease UCL Queen Square Institute of Neurology  London UK"},{"name":"Department 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