The Relationship between Behavioural Changes, Cognitive Symptoms, and Functional Disability in Primary Progressive Aphasia: A Longitudinal Study

Abstract

Background: The contribution of behavioural changes to functional decline is yet to be explored in primary progressive aphasia (PPA). Objectives: (1) investigate functional changes in two PPA variants [semantic (svPPA) and non-fluent (nfvPPA)], at baseline and after 12 months; (2) investigate baseline differences in behavioural changes between groups, and (3) explore predictors of functional decline after a 12-month period. Methods: A longitudinal study involving 29 people with PPA (18 svPPA; 11 nfvPPA) seen annually in Sydney/Australia was conducted. A total of 114 functional and behavioural assessments were included for within-group (repeated-measures ANOVA; annual rate of change; multiple regression analyses) and between-group analyses (pairwise comparisons). Results: Functional profiles in svPPA and nfvPPA were similar in people with up to 5 years of disease duration. Behavioural changes were marked in svPPA patients (stereotypical behaviour and apathy) but did not predict annual rate of change of functional abilities; global cognitive scores at baseline did. Despite mild behavioural changes in nfvPPA (disinhibition, apathy), these were significant predictors of annual rate of functional change. Conclusions: The presentation and interplay of behavioural changes and functional disability differ in svPPA and nfvPPA. These varying factors should be taken into account when considering prognosis, disease management, and selection of outcome measures for interventions.

Distinguishing Frontotemporal Dementia From Alzheimer Disease Through Everyday Function Profiles: Trajectories of Change

“…The bvFTD group had on average higher severity levels (mean = 6.5) based on the CDR ratings than people with AD-dementia (mean =4.2, p<0.001). Chi-square tests showed significant differences across ethnicity (c 2 (1)=30.5, p<0.001), gender (c 2 (1) =40.5, p<.001), and marital status (c 2 (1)=48.39, p<0.001), with a larger proportion of people with bvFTD being male (64.7%; AD-dementia=45.6%), white (bvFTD=93.7%; AD-dementia=80.8%), and married (bvFTD=86.3%; AD-dementia=68.7%). Table 2 shows the number of people with assessment data at different visits for each patient group.…”

“…In particular, only PNFA patients were reported to have a significant deterioration in total ADL and total IADL functioning over 12 months. In two recent studies similarly focusing on FTD syndromes, O'Connor et al 2 reported similar levels of everyday functioning decline between semantic and non-fluent variant of primary progressive aphasia patients over five years, whilst in a separate analysis bvFTD patients were found to show faster levels of IADL decline 19 . These studies provide some important first insights, however, to existing knowledge, no study to date has compared individual IADL functioning across bvFTD and AD over a prolonged period of time.…”

“…Increased dependency in everyday life is a hallmark of dementia 1 . Whilst deteriorations in basic and instrumental activities of daily living (bADLs/ IADLs) are common in all dementia syndromes, some research indicates that everyday activities deteriorate differently across syndromes 2,3 .…”

The tailored activity program (TAP) to address behavioral disturbances in frontotemporal dementia: a feasibility and pilot study

“…Three clinical subtypes are generally recognised: behavioural variant frontotemoral dementia and two primary progressive aphasias, semantic variant and non-fluent variant. These FTD subtypes present differently in the early stages, and while all experience progressive declines, the patterns of cognitive, behavioural and functional decline differ [1][2][3][4]. The impact of FTD is not limited to the person diagnosed with dementia but also affects the informal carers.…”

Executive Dysfunction and Behavioral Symptoms Are Associated with Deficits in Instrumental Activities of Daily Living in Frontotemporal Dementia

“…Most of the prior work has focused on bvFTD, which in some [4,5] but not all [6–9] studies produces more profound functional deficits than Alzheimer’s disease (AD). A subset of reports have also included svPPA and/or nfvPPA [4,5,7,10–12] and generally concluded that ADL performance, particularly for basic ADLs, is also poorer in bvFTD than in these other clinical FTD subtypes. However, due to the relatively smaller sizes of FTD cohorts at individual research centers, it remains uncertain whether these clinical FTD subtypes differ in their patterns of functional impairment as well as in their overall severity [4,7].…”