"It's a Postcode Lottery": How Do People Affected by Dementia in Wales Experience Their Diagnosis and Post-Diagnostic Support, and How May These Be Improved?

National dementia strategies are government policies that guide the provision of appropriate support for people living with dementia. These strategies, developed through extensive stakeholder engagement, should be tailored to the cultural and demographic needs of a country. Using a mixed methods survey design, this study explored the aims of the Dementia Action Plan (2018-2022) for Wales (UK) around assessment, diagnosis, and post-diagnostic support, and assessed whether these are being realized. Further, it sought to gain insight from people living with dementia and their carers around how the experience may be improved for others in the future, as the development of the next iteration of the Action Plan is anticipated. Respondents included 71 people, affected by typical and rarer types of dementia, living in both rural and urban areas. Findings suggest both positive and negative experiences, reflecting a 'postcode lottery' of service provision. Attainable recommendations for improvement were made by respondents, which would ultimately likely be cost-effective and reduce strain on formal services. The findings reported in this paper concur with those reported by people living with dementia in other countries, indicating their relevance for policymakers beyond Wales.

Exploring experiential differences in everyday activities - A focused ethnographic study in the homes of people living with memory-led Alzheimer's disease and posterior cortical atrophy.

BACKGROUND: Supporting ageing in place, quality of life and activity engagement are public health priorities for people living with dementia, but little is known about the needs and experiences of community-dwelling people with rarer forms of dementia with lesser known symptoms. Posterior cortical atrophy (PCA) is a rare form of dementia usually caused by Alzheimer's disease but which is characterised by diminished visual processing (rather than a dominant memory problem), which poses challenges for maintaining independence and accessing appropriate support. METHODS: This study used a comparative qualitative design and focussed ethnographic methods to explore experiential differences in activity engagement for 10 people with the most common, memory-led presentation of Alzheimer's disease and 10 people with posterior cortical atrophy within their everyday home environments. RESULTS: While the data collection revealed much rich variation in individual and contextual factors, some tentative high-level differences in the experiences of everyday activities could be drawn out, seemingly attributable to the different diagnoses' differing dominant symptoms. These included people with posterior cortical atrophy being less likely to use environmental cues to initiate activities, and more likely to withhold from asking for support because of preserved insight into the impact of this on carers. This lack of initiation of activities could be misinterpreted as apathy. People with posterior cortical atrophy also were discouraged from engaging in activities by disorientation within the home, and difficulties localising, identifying and manipulating objects. People with the more common, memory-led presentation of Alzheimer's disease exhibited more memory-based difficulties with engaging with activities such as forgetting planned activities, where to locate the items required for an activity and the steps involved. Despite these distinct symptom-led challenges, all participants and their family members demonstrated resourcefulness and resilience in making creative adaptations to support continued engagement in everyday activities, supporting the widely reported management strategies of people with dementia of the Alzheimer's type more generally. CONCLUSIONS: These findings offer helpful insights into some the differing impacts dementia related visual and memory impairments can have on everyday activity engagement, which will be helpful for others navigating these challenges and the health and social care practitioners working with people affected by these conditions. The findings also highlight the vast individual variation in the multitude of individual and contextual factors involved in everyday activity engagement, and suggest important areas for future work utilising methods which are similarly high in ecological validity and accessibility as the home-based focussed ethnographic methods utilised here.

'Talking lines': the stories of diagnosis and support as told by those with lived experience of rare forms of dementia.

BACKGROUND: People living with, or caring for someone with, rare forms of dementia can encounter issues while obtaining a diagnosis and trying to access appropriate support. This can affect their wellbeing, quality of life, social relationships and employment status. This study makes use of an arts-based narrative approach to explore individual accounts of these experiences whilst also exploring how, in telling their stories, those affected by rare forms of dementia might invoke, and situate their stories in relation to, broader cultural narratives around dementia and illness. METHODS: Semi-structured interviews were conducted via video-conferencing software with participants (N = 27), living with, or caring for someone with, a rare forms of dementia. Participants used line drawings to depict their journey from initial symptoms to the present day, followed by prompts to verbally narrate their experiences. All interview transcripts and line drawings were subjected to narrative analysis. Four sets of transcripts and drawings were then subjected to more in-depth analysis. RESULTS: Analysis shed light on the struggles encountered by both care-partners and people with a diagnosis, while navigating a health and social care system that does not always understand their needs. This often led to individuals feeling isolated and unsupported. Accounts also depicted challenges to identity brought on by the process. The moment of diagnosis was also drawn in a complicated light. Individuals found comfort in gaining understanding, but felt fear at recognising upcoming challenges. Participants situated their own accounts against mainstream cultural narratives around what good support for cognitive impairment and dementia might look like, whilst also demonstrating the influential role they took on in pursuing the right care. CONCLUSIONS: The use of line drawing, alongside narrative interviews, allowed participants to tell complicated, sometimes anachronistic, stories about difficult experiences, whilst also reflecting on, and attaching meaning to, them. These stories highlighted pressing gaps in healthcare services and shone a light on the various pieces of collective action individuals were engaged in in order to improve them. Finally, in modelling some elements of the participants' service provision which were working, the narratives pointed to future directions services might move in.

"I Want to Do Something" - Exploring What Makes Activities Meaningful for Community-Dwelling People Living With Dementia: A Focused Ethnographic Study.

Supporting ageing in place, quality of life, and activity engagement are public health priorities for people with dementia. The importance of maintaining opportunities for meaningful activities has been widely acknowledged for those with dementia in long-term care, but little is known about what makes activities meaningful for, and how they are experienced by, people with different types of dementia in their own homes. This study used focussed ethnographic methods to explore the motivations and meanings of everyday activity engagement within the homes of 10 people with memory-led Alzheimer's disease and 10 people with posterior cortical atrophy. While participants' interactions with their everyday environments were challenged by their diagnoses, they were all finding ways to continue meaning-making via various activities. The main findings are encapsulated in three themes: (1) The fun and the function of activities; (2) Reciprocities of care, and (3) The constitution and continuity of (a changing) self. Ongoing engagement with both fun and functional activities offered participants living with different dementias opportunities to connect with others, to offer care and support (as well as receive it), and to maintain a sense of self and identity. Implications are discussed regarding the development and delivery of tailored interventions and support to enable continued engagement in meaningful activities for people with different types of dementia living in the community.

Symptom-based staging for logopenic variant primary progressive aphasia.

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.

Peripheral hearing loss at age 70 predicts brain atrophy and associated cognitive change.

BACKGROUND: Hearing loss has been proposed as a modifiable risk factor for dementia. However, the relationship between hearing, neurodegeneration, and cognitive change, and the extent to which pathological processes such as Alzheimer's disease and cerebrovascular disease influence these relationships, is unclear. METHODS: Data from 287 adults born in the same week of 1946 who underwent baseline pure tone audiometry (mean age=70.6 years) and two time point cognitive assessment/multimodal brain imaging (mean interval 2.4 years) were analysed. Hearing impairment at baseline was defined as a pure tone average of greater than 25 decibels in the best hearing ear. Rates of change for whole brain, hippocampal and ventricle volume were estimated from structural MRI using the Boundary Shift Integral. Cognition was assessed using the Pre-clinical Alzheimer's Cognitive Composite. Regression models were performed to evaluate how baseline hearing impairment associated with subsequent brain atrophy and cognitive decline after adjustment for a range of confounders including baseline ß-amyloid deposition and white matter hyperintensity volume. RESULTS: 111 out of 287 participants had hearing impairment. Compared with those with preserved hearing, hearing impaired individuals had faster rates of whole brain atrophy, and worse hearing (higher pure tone average) predicted faster rates of hippocampal atrophy. In participants with hearing impairment, faster rates of whole brain atrophy predicted greater cognitive change. All observed relationships were independent of ß-amyloid deposition and white matter hyperintensity volume. CONCLUSIONS: Hearing loss may influence dementia risk via pathways distinct from those typically implicated in Alzheimer's and cerebrovascular disease in cognitively unimpaired older adults.

'Communication is difficult': Speech, language and communication needs of people with young onset or rarer forms of non-language led dementia.

BACKGROUND: People with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy and young onset Alzheimer's disease may experience language and communication difficulties. However, the role of speech and language interventions for people with these non-language led dementias has received little attention. AIMS: This study aimed to explore the experiences and perspectives of people living with these conditions, and their families, regarding their language and communication difficulties and how speech and language therapy could address these needs. METHODS: This study employed a qualitative design to explore the experiences of people living with or caring for somebody with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy or young onset Alzheimer's disease, and to understand their opinions about speech and language therapy. Participants were recruited from a support service connected to a dementia clinic to attend one of five focus group meetings. Videorecorded focus groups and interviews were transcribed, and reflexive thematic analysis was used to analyse data from people affected by each type of dementia. RESULTS: A total of 25 participants were recruited to the study, with representation across the different forms of non-language led dementias. The four main themes identified were: (1) communication difficulties as a key difficulty, (2) loss and loneliness, (3) speech and language therapy, and (4) the role of the caregiver. Sixteen subthemes were also identified which highlighted individual issues across disease types. DISCUSSION: Although all the forms of dementia studied here are not considered to be language-led, people with these conditions and/or their care partners identified speech, language and communication as common challenges. These communication difficulties were reported to have a negative impact on their social participation and mental health and participants felt speech and language interventions could help. There is a need for research exploring speech and language interventions developed for and with people with non-language led dementias and their care partners, to ensure they meet the needs of the people they are designed for. WHAT THIS PAPER ADDS: What is already known on the subject People with primary progressive aphasia present with speech, language and communication difficulties, and several speech and language interventions have been developed to meet the needs of this population. However, people with non-language led dementias may also experience speech, language and communication difficulties, and little is known about interventions that may address these difficulties. What this paper adds to existing knowledge People living with or caring for somebody with behavioural variant frontotemporal dementia, Lewy body dementia, posterior cortical atrophy and young onset Alzheimer's disease report experiencing speech, language and communication difficulties that impact on the person with dementia's social participation and mood. Participants in this study also shared their opinions about how speech and language interventions could help, from the earliest stages of the disease. What are the potential or actual clinical implications of this work? Speech and language therapists need to address the individual speech, language and communication needs of people with dementias, even those that are not thought to be language-led. Current speech and language therapy service provision does not meet the needs of people with non-language led dementias and further research is required to develop interventions and services to meet these needs.

'Misdiagnosed and Misunderstood': Insights into Rarer Forms of Dementia through a Stepwise Approach to Co-Constructed Research Poetry.

This study investigated co-constructed research poetry as a way to understand the lived experiences of people affected by rarer dementia and as a means to use poetry to convey those experiences to healthcare professionals. Using mixed methods, 71 people living with rarer dementia and care-partners (stakeholders) contributed to co-constructing 27 poems with professional poets; stakeholders' verbatim words were analysed with descriptive qualitative analysis. Stakeholders were also surveyed and interviewed about their participation. Healthcare professionals (n = 93) were surveyed to elicit their responses to learning through poetry and its acceptability as a learning tool. Poems conveyed a shared narrative of different aspects of lived experience, often owing to atypical symptoms, misunderstandings by professionals, lack of support pathways, and a continuous struggle to adapt. Stakeholder surveys indicated it was a valuable experience to both co-create and respond to the poems, whilst group interviews revealed people's experiences of the research poetry were characterised by reflection on lived experience, curiosity and exploration. Healthcare professionals' responses reinforced poetry's capacity to stimulate cognitive and affective learning specific to rare dementia support and prompt both empathy and critical thinking in practice. As the largest poetry-based study that we are aware of, this novel accessible approach of creating group poems yielded substantial information about the experiences and needs of those affected by rarer dementia and how poetry can contribute to healthcare education and training.

How Do Care Partners of People with Rare Dementia Use Language in Online Peer Support Groups? A Quantitative Text Analysis Study.

We used quantitative text analysis to examine conversations in a series of online support groups attended by care partners of people living with rare dementias (PLWRD). We used transcripts of 14 sessions (>100,000 words) to explore patterns of communication in trained facilitators' (n = 2) and participants' (n = 11) speech and to investigate the impact of session agenda on language use. We investigated the features of their communication via Poisson regression and a clustering algorithm. We also compared their speech with a natural speech corpus. We found that differences to natural speech emerged, notably in emotional tone (d = -3.2, p < 0.001) and cognitive processes (d = 2.8, p < 0.001). We observed further differences between facilitators and participants and between sessions based on agenda. The clustering algorithm categorised participants' contributions into three groups: sharing experience, self-reflection, and group processes. We discuss the findings in the context of Social Comparison Theory. We argue that dedicated online spaces have a positive impact on care partners in combatting isolation and stress via affiliation with peers. We then discuss the linguistic mechanisms by which social support was experienced in the group. The present paper has implications for any services seeking insight into how peer support is designed, delivered, and experienced by participants.

Demographic, clinical, biomarker, and neuropathological correlates of posterior cortical atrophy: an international cohort study and individual participant data meta-analysis.

BACKGROUND: Posterior cortical atrophy is a rare syndrome characterised by early, prominent, and progressive impairment in visuoperceptual and visuospatial processing. The disorder has been associated with underlying neuropathological features of Alzheimer's disease, but large-scale biomarker and neuropathological studies are scarce. We aimed to describe demographic, clinical, biomarker, and neuropathological correlates of posterior cortical atrophy in a large international cohort. METHODS: We searched PubMed between database inception and Aug 1, 2021, for all published research studies on posterior cortical atrophy and related terms. We identified research centres from these studies and requested deidentified, individual participant data (published and unpublished) that had been obtained at the first diagnostic visit from the corresponding authors of the studies or heads of the research centres. Inclusion criteria were a clinical diagnosis of posterior cortical atrophy as defined by the local centre and availability of Alzheimer's disease biomarkers (PET or CSF), or a diagnosis made at autopsy. Not all individuals with posterior cortical atrophy fulfilled consensus criteria, being diagnosed using centre-specific procedures or before development of consensus criteria. We obtained demographic, clinical, biofluid, neuroimaging, and neuropathological data. Mean values for continuous variables were combined using the inverse variance meta-analysis method; only research centres with more than one participant for a variable were included. Pooled proportions were calculated for binary variables using a restricted maximum likelihood model. Heterogeneity was quantified using I2. FINDINGS: We identified 55 research centres from 1353 papers, with 29 centres responding to our request. An additional seven centres were recruited by advertising via the Alzheimer's Association. We obtained data for 1092 individuals who were evaluated at 36 research centres in 16 countries, the other sites having not responded to our initial invitation to participate to the study. Mean age at symptom onset was 59·4 years (95% CI 58·9-59·8; I2=77%), 60% (56-64; I2=35%) were women, and 80% (72-89; I2=98%) presented with posterior cortical atrophy pure syndrome. Amyloid ß in CSF (536 participants from 28 centres) was positive in 81% (95% CI 75-87; I2=78%), whereas phosphorylated tau in CSF (503 participants from 29 centres) was positive in 65% (56-75; I2=87%). Amyloid-PET (299 participants from 24 centres) was positive in 94% (95% CI 90-97; I2=15%), whereas tau-PET (170 participants from 13 centres) was positive in 97% (93-100; I2=12%). At autopsy (145 participants from 13 centres), the most frequent neuropathological diagnosis was Alzheimer's disease (94%, 95% CI 90-97; I2=0%), with common co-pathologies of cerebral amyloid angiopathy (71%, 54-88; I2=89%), Lewy body disease (44%, 25-62; I2=77%), and cerebrovascular injury (42%, 24-60; I2=88%). INTERPRETATION: These data indicate that posterior cortical atrophy typically presents as a pure, young-onset dementia syndrome that is highly specific for underlying Alzheimer's disease pathology. Further work is needed to understand what drives cognitive vulnerability and progression rates by investigating the contribution of sex, genetics, premorbid cognitive strengths and weaknesses, and brain network integrity. FUNDING: None.

Updating the study protocol: Insight 46 - a longitudinal neuroscience sub-study of the MRC National Survey of Health and Development - phases 2 and 3.

BACKGROUND: Although age is the biggest known risk factor for dementia, there remains uncertainty about other factors over the life course that contribute to a person's risk for cognitive decline later in life. Furthermore, the pathological processes leading to dementia are not fully understood. The main goals of Insight 46-a multi-phase longitudinal observational study-are to collect detailed cognitive, neurological, physical, cardiovascular, and sensory data; to combine those data with genetic and life-course information collected from the MRC National Survey of Health and Development (NSHD; 1946 British birth cohort); and thereby contribute to a better understanding of healthy ageing and dementia. METHODS/DESIGN: Phase 1 of Insight 46 (2015-2018) involved the recruitment of 502 members of the NSHD (median age = 70.7 years; 49% female) and has been described in detail by Lane and Parker et al. 2017. The present paper describes phase 2 (2018-2021) and phase 3 (2021-ongoing). Of the 502 phase 1 study members who were invited to a phase 2 research visit, 413 were willing to return for a clinic visit in London and 29 participated in a remote research assessment due to COVID-19 restrictions. Phase 3 aims to recruit 250 study members who previously participated in both phases 1 and 2 of Insight 46 (providing a third data time point) and 500 additional members of the NSHD who have not previously participated in Insight 46. DISCUSSION: The NSHD is the oldest and longest continuously running British birth cohort. Members of the NSHD are now at a critical point in their lives for us to investigate successful ageing and key age-related brain morbidities. Data collected from Insight 46 have the potential to greatly contribute to and impact the field of healthy ageing and dementia by combining unique life course data with longitudinal multiparametric clinical, imaging, and biomarker measurements. Further protocol enhancements are planned, including in-home sleep measurements and the engagement of participants through remote online cognitive testing. Data collected are and will continue to be made available to the scientific community.

Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia.

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.

Grief and loss in people living with dementia: a review and metasynthesis of qualitative studies.

OBJECTIVES: This review seeks to synthesise qualitative studies that focus on the experience of grief and loss in people living with dementia. METHODS: Included studies were quality appraised, synthesised and analysed using inductive thematic analysis. RESULTS: 19 studies were selected for inclusion in the final review and metasynthesis, including 486 participants (115 participants living with dementia, 152 family carers, 219 professionals). Five key dimensions of grief in people living with dementia were identified during the analysis process: grieving for the person I used to be, grieving for how others see me, grieving for the person I will become, grieving for those who have died and what helps me with my grief. CONCLUSION: It is evident that people living with dementia can experience grief related to a range of previous, current and anticipated losses. Many of the studies included in this review did not directly include people living with dementia in their research and did not ask participants directly about their experience of grief and loss. As grief is a highly personal and individual experience, further research addressing the experience of grief that directly includes participants living with dementia is required, in order to improve awareness of grief-related needs and to develop and deliver support to meet these needs.

Pupil responses to colorfulness are selectively reduced in healthy older adults.

The alignment between visual pathway signaling and pupil dynamics offers a promising non-invasive method to further illuminate the mechanisms of human color perception. However, only limited research has been done in this area and the effects of healthy aging on pupil responses to the different color components have not been studied yet. Here we aim to address this by modelling the effects of color lightness and chroma (colorfulness) on pupil responses in young and older adults, in a closely controlled passive viewing experiment with 26 broad-spectrum digital color fields. We show that pupil responses to color lightness and chroma are independent from each other in both young and older adults. Pupil responses to color lightness levels are unaffected by healthy aging, when correcting for smaller baseline pupil sizes in older adults. Older adults exhibit weaker pupil responses to chroma increases, predominantly along the Green-Magenta axis, while relatively sparing the Blue-Yellow axis. Our findings complement behavioral studies in providing physiological evidence that colors fade with age, with implications for color-based applications and interventions both in healthy aging and later-life neurodegenerative disorders.

Thinking eyes: visual thinking strategies and the social brain.

The foundation of art processes in the social brain can guide the scientific study of how human beings perceive and interact with their environment. Here, we applied the theoretical frameworks of the social and artistic brain connectomes to an eye-tracking paradigm with the aim to elucidate how different viewing conditions and social cues influence gaze patterns and personal resonance with artworks and complex imagery in healthy adults. We compared two viewing conditions that encourage personal or social perspective taking-modeled on the well-known Visual Thinking Strategies (VTS) method-to a viewing condition during which only contextual information about the image was provided. Our findings showed that the viewing conditions that used VTS techniques directed the gaze more toward highly salient social cues (Animate elements) in artworks and complex imagery, compared to when only contextual information was provided. We furthermore found that audio cues also directed visual attention, whereby listening to a personal reflection by another person (VTS) had a stronger effect than contextual information. However, we found no effect of viewing condition on the personal resonance with the artworks and complex images when taking the random effects of the image selection into account. Our study provides a neurobiological grounding of the VTS method in the social brain, revealing that this pedagogical method of engaging viewers with artworks measurably shapes people's visual exploration patterns. This is not only of relevance to (art) education but also has implications for art-based diagnostic and therapeutic applications.

Situating support for people living with rarer forms of dementia.

BACKGROUND: Awareness of a multitude of diseases that can cause neurodegenerative decline and their unique symptom profiles in the dementia care and support sectors remains limited. Obtaining an accurate diagnosis and post-diagnostic care and support is a challenge for many people and their families. As part of a larger study examining multi-component forms of support for people living with rarer dementias, the aim of this present study was to examine how rare dementia was situated within the complex social groupings, their organization and embedded discursive constructions that broadly form dementia care and support delivery. METHODS: Adopting a situational analysis approach, we undertook an examination of public documents and organizational websites within the support sector for people living with dementia in Canada, England, and Wales. We also surveyed professionals to further explore the situation at the point of care and support delivery. Consistent with our approach, data collection and analysis occurred concurrently including the development of a series of analytic maps. RESULTS: Recognizing the complexities within the situation, our findings provided new insights on the situated structures for support action and the discursive representations that illuminate both the limitations of the current support landscape and possibilities for a more flexible and tailored rare dementia support. Alongside, the predominant universal versus tailored support positionings within our data reinforced the complexity from which a promising new social space for people living with rarer dementias is being cultivated. CONCLUSIONS: The social worlds engaged in supportive action with people living with rare dementia are less visible within the shadow of a universally constructed dementia support milieu and appear to be negotiated within this powerful arena. However, their evolving organization and discursive constructions point to an emerging new social space for people living with rarer conditions.

"Better Living with Non-memory-led Dementia": protocol for a feasibility randomised controlled trial of a web-based caregiver educational programme.

BACKGROUND: Non-memory-led dementias such as posterior cortical atrophy (PCA), primary progressive aphasia (PPA) and behavioural variant frontotemporal dementia (bvFTD) are low prevalent and often affect individuals under the age of 65. Tailored educational and support resources for caregivers of people living with these dementia phenotypes are scarce and unevenly distributed geographically. Web-based educational programmes are emerging as promising alternatives to improve caregiver self-efficacy and well-being. Here, we present the protocol of a study aiming to assess the feasibility of a co-produced online educational programme for caregivers of people living PCA, PPA and bvFTD: the Better Living with Non-memory-led Dementia programme. METHODS: A randomised controlled feasibility trial will be conducted on a sample of 30 caregivers of people living with PCA, PPA and bvFTD. Participants will be recruited among members of the support organisation Rare Dementia Support (based at UCL in the UK). The intervention group will be given access to an 8-week co-produced web-based educational programme consisting of 6 modules addressing education about PCA, PPA and bvFTD and support strategies for the person with dementia and for the caregiver. The control group will receive treatment as usual (TAU). Feasibility will be measured through feasibility of recruitment, clinical measurement tools and acceptability. Clinical measures will be used to assess preliminary efficacy and data on completion rates, missing data and variability used to decide on measures to be included in a full-scale trial. Allocation ratio will be 2:1 (intervention:control) stratified by diagnosis. Feasibility of recruitment and acceptability will be assessed. Clinical measures will be administered at baseline and 8-week and 3-month post-randomisation. The control group will be offered access to the intervention at the completion of data collection. Participants will be unblinded, and all measures will be self-reported online. DISCUSSION: Online-delivered educational programmes show potential for improving care competency of caregivers and may contribute to overcoming geographical inequalities in local provision of support services. This pilot study will inform a fully powered international trial to determine the effectiveness of Better Living with Non-memory-led Dementia. TRIAL REGISTRATION: This trial has been registered prospectively on the Clinical Trials Registry on 1st September 2022, registration number NCT05525377.

Neuroimaging, clinical and life course correlates of normal-appearing white matter integrity in 70-year-olds.

We investigate associations between normal-appearing white matter microstructural integrity in cognitively normal ∼70-year-olds and concurrently measured brain health and cognition, demographics, genetics and life course cardiovascular health. Participants born in the same week in March 1946 (British 1946 birth cohort) underwent PET-MRI around age 70. Mean standardized normal-appearing white matter integrity metrics (fractional anisotropy, mean diffusivity, neurite density index and orientation dispersion index) were derived from diffusion MRI. Linear regression was used to test associations between normal-appearing white matter metrics and (i) concurrent measures, including whole brain volume, white matter hyperintensity volume, PET amyloid and cognition; (ii) the influence of demographic and genetic predictors, including sex, childhood cognition, education, socio-economic position and genetic risk for Alzheimer's disease (APOE-ɛ4); (iii) systolic and diastolic blood pressure and cardiovascular health (Framingham Heart Study Cardiovascular Risk Score) across adulthood. Sex interactions were tested. Statistical significance included false discovery rate correction (5%). Three hundred and sixty-two participants met inclusion criteria (mean age 70, 49% female). Higher white matter hyperintensity volume was associated with lower fractional anisotropy [b = -0.09 (95% confidence interval: -0.11, -0.06), P < 0.01], neurite density index [b = -0.17 (-0.22, -0.12), P < 0.01] and higher mean diffusivity [b = 0.14 (-0.10, -0.17), P < 0.01]; amyloid (in men) was associated with lower fractional anisotropy [b = -0.04 (-0.08, -0.01), P = 0.03)] and higher mean diffusivity [b = 0.06 (0.01, 0.11), P = 0.02]. Framingham Heart Study Cardiovascular Risk Score in later-life (age 69) was associated with normal-appearing white matter {lower fractional anisotropy [b = -0.06 (-0.09, -0.02) P < 0.01], neurite density index [b = -0.10 (-0.17, -0.03), P < 0.01] and higher mean diffusivity [b = 0.09 (0.04, 0.14), P < 0.01]}. Significant sex interactions (P < 0.05) emerged for midlife cardiovascular health (age 53) and normal-appearing white matter at 70: marginal effect plots demonstrated, in women only, normal-appearing white matter was associated with higher midlife Framingham Heart Study Cardiovascular Risk Score (lower fractional anisotropy and neurite density index), midlife systolic (lower fractional anisotropy, neurite density index and higher mean diffusivity) and diastolic (lower fractional anisotropy and neurite density index) blood pressure and greater blood pressure change between 43 and 53 years (lower fractional anisotropy and neurite density index), independently of white matter hyperintensity volume. In summary, poorer normal-appearing white matter microstructural integrity in ∼70-year-olds was associated with measures of cerebral small vessel disease, amyloid (in males) and later-life cardiovascular health, demonstrating how normal-appearing white matter can provide additional information to overt white matter disease. Our findings further show that greater 'midlife' cardiovascular risk and higher blood pressure were associated with poorer normal-appearing white matter microstructural integrity in females only, suggesting that women's brains may be more susceptible to the effects of midlife blood pressure and cardiovascular health.

Video Conferencing Peer Support and Rarer Forms of Dementia: An Exploration of Family Carers' Positive Experiences.

Little is known regarding the nuanced experiences of family carers for people living with rare dementias (PLWRD), with no known literature exploring their positive experiences of caring discussed within peer support group settings. This article explores family carers of PLWRD's positive experiences reported in video conferencing peer support groups. Six peer support group sessions involving a total of nine participants were qualitatively analysed using thematic analysis, guided by the conceptual framework of positive aspects of caring (CFPAC) (Carbonneau et al., 2010). Six themes were identified: (1) Protecting, maintaining, enjoying and finding strength in their relationship with the PLWRD; (2) Using tools and resources in response to challenges; (3) Positive impact of interactions and others' responses to the dementia; (4) Overcoming barriers to taking a break while maintaining their wellbeing, (5) Maintaining positive outlooks and showing psychological resilience in adversity; and (6) Attributing meaning to the caring role. This article highlights family carers of PLWRD's positive psychological, physical and social resources, balanced against the challenges of caring and maintaining their wellbeing, and identifies ways of promoting family carers' positive caring experiences and resources within healthcare and supportive settings.

"I felt like I had been put on the shelf and forgotten about" - lasting lessons about the impact of COVID-19 on people affected by rarer dementias.

BACKGROUND: The public health measures imposed in many countries to contain the spread of COVID-19 resulted in significant suspensions in the provision of support and care for people with dementia. The negative effects of these measures have been extensively reported. However, little is known about the specific impact on people with young onset, non-memory-led and inherited dementias. This group may have experienced different challenges compared to those with late onset dementia given their non-memory phenotypes and younger age. We explored the impact of the first COVID-19 lockdown on people living with familial Alzheimer's disease, behavioural variant frontotemporal dementia, familial frontotemporal dementia, dementia with Lewy bodies, posterior cortical atrophy and primary progressive aphasia and their carers in the UK and their self-reported strategies for coping. METHODS: This was a mixed methods study. An online survey was administered to people with dementia and family carers recruited via Rare Dementia Support. Free-text responses were analysed using framework analysis to identify key issues and themes. RESULTS: 184 carers and 24 people with dementia completed the survey. Overall, people with dementia experienced worsening of cognitive symptoms (70%), ability to do things (62%), well-being (57%) and changes to medication (26%) during lockdown. Carers reported a reduction in the support they received (55%) which impacted their own mental health negatively. Qualitative analysis of free-text responses shed light on how the disruption to routines, changes to roles and responsibilities, and widespread disconnection from friends, family and health and social care support varied according to phenotype. These impacts were exacerbated by a more general sense that precious time was being lost, given the progressive nature of dementia. Despite significant challenges, respondents demonstrated resilience and resourcefulness in reporting unexpected positives and strategies for adapting to confinement. CONCLUSIONS: This study has highlighted the specific impacts of the COVID-19 restrictions on people with young onset, non-memory-led and inherited dementias, including behavioural variant frontotemporal dementia, primary progressive aphasia and posterior cortical atrophy, and their carers. The specific challenges faced according to diagnosis and the self-reported strategies speak to the importance of - and may inform the development of - tailored support for these underrepresented groups more generally.