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Background and purpose: Logopenic variant primary progressive aphasia (lvPPA) is a major variant presentation of Alzheimer's disease (AD) that signals the importance of communication dysfunction across AD phenotypes. A clinical staging system is lacking for the evolution of AD‐associated communication difficulties that could guide diagnosis and care planning. Our aim was to create a symptom‐based staging scheme for lvPPA, identifying functional milestones relevant to the broader AD spectrum. Methods: An international lvPPA caregiver cohort was surveyed on symptom development under an 'exploratory' survey (34 UK caregivers). Feedback from this survey informed the development of a 'consolidation' survey (27 UK, 10 Australian caregivers) in which caregivers were presented with six provisional clinical stages and feedback was analysed using a mixed‐methods approach. Results: Six clinical stages were endorsed. Early symptoms included word‐finding difficulty, with loss of message comprehension and speech intelligibility signalling later‐stage progression. Additionally, problems with hearing in noise, memory and route‐finding were prominent early non‐verbal symptoms. 'Milestone' symptoms were identified that anticipate daily‐life functional transitions and care needs. Conclusions: This work introduces a new symptom‐based staging scheme for lvPPA, and highlights milestone symptoms that could inform future clinical scales for anticipating and managing communication dysfunction across the AD spectrum. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Here we set out to create a symptom‐led staging system for the canonical semantic and non‐fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer's disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed‐methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non‐verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid ("PPA‐Squared"). DISCUSSION: This work introduces a symptom‐led staging scheme and functional scale for semantic and non‐fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA. Highlights: We introduce new symptom‐led perspectives on primary progressive aphasia (PPA).The focus is on non‐fluent/agrammatic (nfvPPA) and semantic (svPPA) variants.Foregrounding of early and non‐verbal features of PPA and clinical trajectories is featured.We introduce a symptom‐led staging scheme for PPA.We propose a prototype for a functional impairment scale, the PPA Progression Planning Aid. [ABSTRACT FROM AUTHOR]

Rare Dementia Support (RDS) is a UK‐based collaborative service led by the UCL Dementia Research Centre, which aims to empower, guide and inform people living with a rare dementia diagnosis and those who care about them. RDS covers familial Alzheimer's disease (FAD), frontotemporal dementia (FTD), familial frontotemporal dementia (fFTD), posterior cortical atrophy (PCA), primary progressive aphasia (PPA), Lewy body dementia (LBD) and young‐onset Alzheimer's disease (YOAD). Following self‐referrals and clinical referrals from memory services and specialist neurology clinics across the UK, we provide one‐to‐one support and facilitate regular opportunities for members to meet each other and the team, through large and small support groups, themed programmes and regional support groups. We will describe the practices of the one‐to‐one Direct Support Team, which provides free information, advice and support from pre‐diagnosis navigation through to post‐bereavement support. This specialist emotional and practical support encompasses compassionate listening; solution focussed outcomes (to complement the diagnosis, treatment and monitoring); helping people to understand and adapt to their condition; empowering people to use their strengths to live fulfilled lives; designing strategies and interventions to manage complex situations (including issues of safeguarding, deprivation of liberty, etc); legal rights and financial support; and supporting transitions into later stages and end of life. We will also relate the practices of direct support to the broader principles of RDS as a whole (see Figure 1), including the implications of never discharging anyone from our service, and balancing person‐centred and disease‐specific approaches (the importance of knowing which disease each person has, and what person the disease has). An evaluation of the service will be presented, including characterisation of the current 4797 members, plus data from individual support consultations in 2022 (N = 1449) relating outcomes to personal (social connectedness, wellbeing, knowledge of condition and services), disease (diagnosis, severity) and service (support usage, intensity of discussion) factors. We will conclude by discussing existing examples of and new opportunities for partnership working – with local, national and international charities, clinical services, academic institutions and funding organisations – to improve access to specialist support for those with atypical, inherited and young onset dementias. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]