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Neurol Clin Pract. 2018 Apr;8(2):120-128. doi: 10.1212/CPJ.0000000000000429.

Apathy and functional disability in behavioral variant frontotemporal dementia.

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Neurology Department (MSY, TBLdS, VSB, SMDB, RN), University of São Paulo, Brazil; Ageing, Work & Health Research Unit (CMO), Faculty of Health Sciences, University of Sydney, Australia; Nizam's Institute of Medical Sciences (SM, SA), Hyderabad, India; Cognitive and Behavioral Neurology Research Group (VA-C, HCG, PC), Faculdade de Medicina and Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte; Department of Neurology (MLFB, BD), University of Campinas, São Paulo, Brazil; ARC Centre of Excellence in Cognition and its Disorders (JRH, OP, EM), University of New South Wales; Neuroscience Research Australia (JRH, OP), Randwick; and School of Health Sciences (EM), University of East Anglia, Norwich, UK.



Behavioral variant frontotemporal dementia (bvFTD) has profound consequences on patients and their families. In this multicenter study, we investigated the contribution of cognitive and neuropsychiatric factors to everyday function at different levels of overall functional impairment.


In a retrospective cross-sectional study, 109 patients with bvFTD from 4 specialist frontotemporal dementia centers (Australia, England, India, and Brazil) were included. The measures administered evaluated everyday function (Disability Assessment for Dementia [DAD]), dementia staging (Clinical Dementia Rating [CDR]), general cognition (Addenbrooke's Cognitive Examination-revised [ACE-R]), and neuropsychiatric symptoms (Neuropsychiatric Inventory [NPI]). Patients were then subdivided according to functional impairment on the DAD into mild, moderate, severe, and very severe subgroups. Three separate multiple linear regression analyses were run, where (1) total DAD, (2) basic activities of daily living (BADL), and (3) instrumental activities of daily living (IADL) scores were dependent variables; ACE-R total score and selected NPI domains (agitation/aggression, euphoria, apathy, disinhibition, irritability, aberrant motor behavior) were used as independent variables. Age, sex, education, and country of origin were controlled for in the analyses.


Cognitive deficits were similar across the mild, moderate, and severe subgroups but significantly worse in the very severe subgroup. NPI domain scores (agitation/aggression, euphoria, apathy, disinhibition, irritability, aberrant motor behavior) did not differ across the DAD subgroups. In the multiple regression analyses, a model including ACE-R and NPI apathy explained 32.5% of the variance for total DAD scores. For IADL, 35.6% of the variance was explained by the ACE-R only. No model emerged for BADL scores.


Cognitive deficits and apathy are key contributors to functional disability in bvFTD but factors underlying impairment in BADLs remain unclear. Treatments targeting reduction of disability need to address apathy and cognitive impairment to ensure greater efficacy, especially in regards to IADLs.

[Available on 2019-04-01]

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