Pronounced Impairment of Activities of Daily Living in Posterior Cortical Atrophy.

INTRODUCTION: The impact of several dementia syndromes on activities of daily living (ADLs) has been well documented, but no study has yet investigated functional ability in posterior cortical atrophy (PCA). The primarily visual nature of deficits in this condition is likely to have a pronounced impact on ADLs. OBJECTIVE: The aim of this study was to profile functional change in PCA and identify predictors of change. METHOD: Twenty-nine PCA patients and 25 patients with typical Alzheimer's disease (AD) and their caregivers were included in this cross-sectional study. ADLs were assessed using the Disability Assessment for Dementia (DAD), administered to caregivers, assessing basic ADLs (e.g., eating, dressing) and instrumental ADLs (e.g., managing finances, meal preparation). The predictive utility of cognitive domains (Addenbrooke's Cognitive Examination), behavioural impairment (Cambridge Behavioural Inventory-Revised) and demographic variables on ADL ability was also examined. RESULTS: PCA patients showed significantly reduced total ADL scores compared to AD patients (medium effect size, d = -0.7; p < 0.05), with significantly more impairment on basic ADLs (large effect size, d = -0.8; p < 0.05) but similar impairment on instrumental ADLs (medium effect size, d = -0.5; p > 0.05). A model combining patient mood, disinhibition, apathy, symptom duration, and memory and attention/orientation scores explained the variance of scores in functional decline (61.2%), but the key factor predicting ADL scores was attention/orientation (p = 0.048). CONCLUSION: This study shows the profound impact of PCA on ADLs and factors underpinning patients' disability. Attention/orientation deficits were found to correlate and contribute to variance in ADL scores. Future work to develop tailored interventions to manage ADL impairment in PCA should take these findings into account.

Protocol for the Rare Dementia Support Impact study: RDS Impact.

OBJECTIVES: The Rare Dementia Support (RDS) Impact study will be the first major study of the value of multicomponent support groups for people living with or supporting someone with a rare form of dementia. The multicentre study aims to evaluate the impact of multicomponent support offered and delivered to people living with a rare form of dementia, comprising the following five work packages (WPs): (a) longitudinal cohort interviews, (b) theoretical development, (c) developing measures, (d) novel interventions, and (e) economic analysis. METHODS: This is a mixed-methods design, including a longitudinal cohort study (quantitative and qualitative) and a feasibility randomised control trial (RCT). A cohort of more than 1000 individuals will be invited to participate. The primary and secondary outcomes will be in part determined through a co-design nominal groups technique prestudy involving caregivers to people living with a diagnosis of a rare dementia. Quantitative analyses of differences and predictors will be based on prespecified hypotheses. A variety of quantitative (eg, analysis of variance [ANOVA] and multiple linear regression techniques), qualitative (eg, thematic analysis [TA]), and innovative analytical methods will also be developed and applied by involving the arts as a research method. RESULTS: The UCL Research Ethics Committee have approved this study. Data collection commenced in January 2020. CONCLUSIONS: The study will capture information through a combination of longitudinal interviews, questionnaires and scales, and novel creative data collection methods. The notion of "impact" in the context of support for rare dementias will involve theoretical development, novel measures and methods of support interventions, and health economic analyses.