Rare Form Of Dementia Research Articles

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

BackgroundPeople 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.MethodsSemi-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.ResultsAnalysis 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.ConclusionsThe 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.

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

BackgroundSupporting 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. MethodsThis 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. ResultsWhile 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. ConclusionsThese 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.

Patient and public involvement and engagement with underserved communities in dementia research: Reporting on a partnership to co-design a website for postdiagnostic dementia support.

Despite the advancements in Patient and Public Involvement and Engagement (PPIE), the voices of traditionally underserved groups are still poorly reflected in dementia research. This study aimed to report on a PPIE partnership between academics and members of the public from underserved communities to co-design Forward with Dementia-Social Care, a resource and information website supporting people receiving a dementia diagnosis. The PPIE partnership was set up in four stages: 1-identifying communities that have been under-represented from PPIE in dementia research; 2-recruiting PPIE partners from these communities; 3-supporting PPIE partners to become confident to undertake their research roles and 4-undertaking research co-design activities in an equitable fashion. To address under-representation from PPIE in dementia research we recruited seven PPIE partners from Black, Asian and other minority ethnic groups; lesbian, gay, bisexual, transgender, queer+ communities; remote/rural area; religious minoritiesand partners living with rare forms of dementia. The partners met regularly throughout the project to oversee new sections for the study website, refine existing contentand promote the website within their communities. Strategies can be used to successfully recruit and involve PPIE partners from underserved communities in co-design activities. These include networking with community leaders, developing terms of reference, setting out 'rules of engagement', and investing adequate resources and time for accessible and equitable involvement. These efforts facilitate the co-design of research outputs that reflect the diversity and complexity of UK contemporary society. This study received support from seven members of the public with lived experience of dementia from communities that have been traditionally underserved in dementia research. These seven members of the public undertook the role of partners in the study. They all equally contributed to the study design, recruitment of participants, development and revision of topic guides for the interviewsand development of the website. Three of these partners were also co-authors of this paper. On top of the activities shared with the other partners, they contributed to write independently of the academic team the section in this paper titled 'Partners' experiences, benefits and challenges of the partnership'. Further, they provided input in other sections of the paper on a par with the other (academic) co-authors.

Objects of hope: using object elicitation to open up conversations about hope for people affected by rarer forms of dementia

AbstractBackgroundHope is an essential component of the human experience and can play a critical role in sustaining wellbeing and a good quality of life. It can seem somewhat at odds however with the dominant societal discourses around dementia, which, stemming from a biomedical perspective, are often (naturally) focused on deficits and decline. With recent promising developments in the field of therapeutic interventions, it is of timely importance to explore the concept of hope for those affected by rarer forms of dementia, charactersised by atypical symptoms and associated with unique challenges, for whom emerging treatments may be too late. Here, we explored the use of an innovative methodology – object elicitation – to facilitate conversations on this complex and sensitive topic with these underrepresented groups.Method17 people affected by dementia and primary caregivers took part in 3 facilitated discussions using object elicitation to explore the concept of hope within the context of rarer forms of dementia, including: familial Alzheimer’s disease, behavioural variant frontotemporal dementia, and posterior cortical atrophy. Transcripts of the discussions were analysed thematically.ResultFindings were organised into 4 themes: (i) Reframing and reconceptualising hope in the context of dementia; (ii) Temporality: from hopes for the future to finding hope in the moment; (iii) Hope for the individual and hope for the collective; and (iv) The other side of the coin: lost hope, false hope and barriers to hope.ConclusionThe findings of this study shed light on some of the challenges to sustaining hope for people affected by lesser known forms of dementia and importantly, their resilience and resourcefulness in finding ways to continue to cultivate a sense of hope during difficult times within their everyday lives. These findings have implications for others living with these conditions and the unpaid carers and health and social care professionals supporting them, and are of particular relevance in the context of current treatment developments. This study has also demonstrated the utility and accessibility of the object elicitation method for opening up and facilitating conversations about emotionally charged topics for people living with and supporting people with different diagnoses of dementia.

More than words: Photovoice as an innovative participatory approach to explore perceptions of rare dementia, a pilot study

AbstractBackgroundThere is limited evidence internationally exploring the everyday experiences of people living with rare dementia (PLWRD)– by which we mean atypical, inherited, and young onset forms of dementia– from their own subjective perspectives. Photovoice (Wang and Burris, 1997) is an inclusive, empowering visual research methodology that facilitates collaboration and supports shared understandings. This pilot study presents findings from the first known study where an inclusive research team, including individuals with different rare dementia diagnoses (e.g. Primary Progressive Aphasia, Posterior Cortical Atrophy, Dementia with Lewy bodies) used Photovoice methodology to visually represent experiences and perspectives of living with these conditions.MethodDrawing on Freire’s empowerment pedagogy, the study aims were consistent with global Photovoice aims: enabling people to visually record critical dialogue about their experiences of dementia through photography. We investigated the benefits and challenges of Photovoice methodology with this population and sought to identify perspectives of rare dementia from eight PLWRD. Data analysis utilised an iterative, participatory approach in which researchers and co‐researchers selected, contextualised, and codified the data. Thematic analyses involved both inductive and realist approaches.ResultThis paper discusses some of the practical challenges associated with using this methodology with PLWRD, as well as issues concerning co‐researcher’s perspectives on the acceptability and utility of Photovoice. We examine the benefits and challenges of using Photovoice and discuss lessons learned relating to the co‐production of topics and outputs, including barriers and facilitators to co‐researcher engagement with the study processes (e.g. use of cameras, photo upload).ConclusionThis pilot study will help inform a larger scale project where Photovoice and other creative participatory approaches (e.g. graphic elicitation) will be applied within a participatory action research framework that will promote group and individual reflection on dementia perspectives. In turn we hope this will lead to social action on a local and national level by co‐researchers with rare forms of dementia. This study contributes to the existing, if limited, international literature on creative participatory approaches with PLWRD.

P72: Diagnostic pathways for people living with rare types of dementia

Background:The pathways to diagnosis for people living with rare types of dementia are poorly understood. In Wales, a predominantly rural country, only 53% of people living with dementia have received a formal diagnosis of any form of dementia compared to 71% in England (Welsh Government, 2018). Some practitioners believe that a number of Welsh communities remain fearful of statutory services, particularly the large bilingual population who tend to access dementia services between 4.3 and 7.3 years later than monolinguists (Hedd Jones, 2018). This inevitably impacts on the numbers of people diagnosed with rarer forms of dementia and their ability to locate and negotiate appropriate pathways to access an accurate diagnosis. This study investigated the experience of people using these pathways to understand how a rare dementia diagnosis is delivered in Wales.Method:Ten semi-structured interviews were conducted with people living with a rare dementia or caring for a person with a rare dementia. These interviews considered the onset of symptoms, the experience of using healthcare services to receive a diagnosis and the support provided following diagnosis. The interview data was mapped across individual pathways and a thematic analysis was undertaken to explore shared perspectives.Result:A diagnosis of rare dementia was illustrated as a journey with five potentially recurring landmark points (initial contact, initial referrals, provider referrals, private referrals and diagnosis). Despite some identical diagnoses, no participants followed the same pathways with private healthcare often used to gain a degree of control over a pathway, if the person had the financial means to access this. Two themes were identified throughout the landmark points (involvement and dispute). Involvement illustrated the need to have a degree of control over the pathway whilst dispute acknowledged the overwhelming feeling that accessing a diagnosis often felt like a personal battle between those affected and health services, or between different aspects of health services themselves.Conclusion:Whilst the experiences illustrated the unstructured pathways to a rare dementia diagnosis, these experiences will support services in Wales and countries with similar populations to consider how structured pathways can result in a timelier diagnosis and more supportive experiences.

Situating support for people living with rarer forms of dementia

BackgroundAwareness 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.MethodsAdopting 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.ResultsRecognizing 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.ConclusionsThe 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.

Talking Lines: A Research Protocol Integrating Verbal and Visual Narratives to Understand the Experiences of People Affected by Rarer Forms of Dementia

People affected by rarer forms of dementia often have a long and difficult experience obtaining a diagnosis and appropriate support, impacting family, employment and social relationships, quality of life and wellbeing. For this population progressive cognitive symptoms affect skills other than memory and disproportionately occur under the age of 65 years, often resulting in misdiagnosis and lack of appropriate care pathways. The objective of this study will be to better understand the subjective experience of the time period from first noticing symptoms to obtaining a formal diagnosis, through to accessing support, and onward to the present time. Through the concurrent use of line drawings and video-recorded interviews we will collect the stories of people living with different rarer dementias and/or family members who are care partners in Canada and the United Kingdom. Narrative and visual analysis will be used in parallel to methodologically explore how line drawing and verbal discourse interact and inform each other to construct knowledge, and how the use of drawing lines might enrich research interviews and increase accessibility of research participation. This novel research approach may also have implications for clinical interviewing, support services, and public engagement. To the best of our knowledge, this is the first study to retrospectively explore over time the experiences of people affected by rarer forms of dementia from initial symptoms—to diagnosis—to accessing support—to the present, using visual and verbal methodologies.

A mixed methods evaluation of a program exploring predeath grief and loss for carers of people with rarer dementias.

Predeath grief conceptualizes complex feelings of loss experienced for someone who is still living and is linked to poor emotional well-being. The Road Less Travelled program aimed to help carers of people with rarer dementias identify and process predeath grief. This study evaluated the feasibility, acceptability, and preliminary effectiveness of this program. Pre-post interventional mixed methods study. Online videoconference group program for carers across the UK held in 2021. Nine family carers of someone living with a rare form of dementia. Eight were female and one male (mean age 58) with two facilitators. The Road Less Travelled is an online, facilitated, group-based program that aims to help carers of people with rarer dementias to explore and accept feelings of grief and loss. It involved six fortnightly 2-hour sessions. We collected measures for a range of well-being outcomes at baseline (T1), post-intervention (T2), and 3 months post-intervention (T3). We conducted interviews with participants and facilitators at T2. Participant attendance was 98% across all sessions. Findings from the semistructured interviews supported the acceptability of the program and identified improvements in carer well-being. Trends in the outcome measures suggested an improvement in quality of life and a reduction in depression. The program was feasible to conduct and acceptable to participants. Qualitative reports and high attendance suggest perceived benefits to carers, including increased acceptance of grief, and support the need for a larger-scale pilot study to determine effectiveness.

Frontotemporal dementia: Addressing the scattered harbingers of genetics and its relationship with glucose metabolism, bipolar disorder, and amyotrophic lateral sclerosis

Frontotemporal Dementia, also known by the name Pick's disease, is a rare form of dementia that can run for several generations. The two key characteristics are argyrophilic, spherical intraneuronal inclusions, which most frequently impact the frontal and temporal poles, and localized cortical atrophy (Pick bodies). Although personality decline and memory loss are frequently more severe than the visuospatial and apraxia disorders that are common in Alzheimer's disease, clinical overlap with other non-Alzheimer degenerative disorders is being increasingly recognized. The limbic system, which includes the hippocampus, entorhinal cortex, and amygdala, typically experiences the greatest levels of neuronal loss and degeneration. In the hippocampus's dentate fascia, several Pick bodies are frequently seen. Leukoencephalopathy and inflated cortical neurons are less specific symptoms (Pick cells). In this paper, we review the factors leading to Picks disease along with its pathophysiology, clinical manifestations, diagnosis, imaging, treatment, prognosis, and a comprehensive discussion on the same. We have also discussed the relationship of frontotemporal dementia with glucose metabolism, bipolar disorder, and amyotrophic lateral sclerosis, all of which are emerging fields of interest and need more studies.

‘I found it the only place that spoke the same language’: a thematic analysis of messages posted to an online peer support discussion forum for people living with dementia

Despite the proliferation of online support communities, little is known about how people with dementia engage with them. The aim of this study was to explore the content of messages exchanged between members of the asynchronous Talking Point online discussion forum. Using the sub-forum 'I have dementia', 100 conversation threads were randomly selected yielding 863 individual messages for analysis. Our data set was subjected to reflexive thematic analysis. Three themes were generated: journeying through dementia, reciprocal peer support and therapeutic creativity. The findings suggest that individuals used the forum to share their experiences of obtaining and adjusting to a dementia diagnosis, their current symptoms and how they addressed the challenges of daily living as well as the impacts on mood, confidence and connectedness. The forum was viewed as a safe space to reflect on the future and what it might hold for them and loved ones. Throughout these discussions, users supported each other, particularly in relation to the emotional impact of diagnosis and shared experiential information and advice, with referrals to external sources of support commonplace. Discussion around the role of therapeutic creativity was evident and its role as a coping mechanism described. Through engagement, a sense of community and companionship was evident and new members welcomed, supported and encouraged to join in. The forum appears to be filling an important gap in post-diagnostic support provision, particularly for those with rare forms of dementia. Clinicians, support workers and organisations could consider recommending online support forums to people with dementia.

Rare health conditions 61: Binswanger's disease, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, Huntington's disease and progressive supranuclear palsy

The purpose of this series is to briefly highlight a range of rare health conditions. Rare health conditions are those that affect no more than 1 person in every 2000. Many healthcare assistants and nurses will encounter patients with these conditions given their wide range. This 61st article will explore four rare forms of dementia.

Impact of COVID-19 Related Work Model Changes on Service Pathways for New Patient Assessments at East Kent Neuropsychiatry Service: Service Evaluation Comparing One Year Pre-COVID-19 Lockdown With One Year Post-Lockdown

AimsNeuropsychiatry Service in East Kent typically receives referrals for patients with brain injury, progressive neurological conditions, epilepsy specific neuropsychiatric conditions, rare forms of dementia, and functional neurological conditions. COVID-19 pandemic disrupted routine functioning of the service requiring multiple service innovations including introduction of remote access assessments, skills development clinics, and video-conferencing based psychoeducation groups. We conducted a service evaluation with governance approval to understand the impact of COVID-19 work model changes on referral sources, patient attendance, discharge destinations and the mental health professionals’ involvement in the management of the patients referred to the service.MethodsWe applied to Service Evaluation and Audit Group of Kent and Medway NHS Partnership Trust for permission to collect service data using routinely collected clinical and business administration information. We used an approved data collection form for anonymized data collection. We analysed data for new patient assessments conducted over one-year prior to COVID-19 lockdown announced on 23rd March 2020 and compared it with one-year post-COVID lockdown period ending on 22 March 2021. We used Statistical Package for Social Sciences (SPSS) to carry out descriptive and statistical analysis of the data from two service evaluation period.ResultsThe total number of new patient assessments conducted during the two designated service evaluation periods was 365. 233 new patient assessments (64%) were conducted during the one-year pre-COVID-19 lockdown and 132 (36%) new patient assessments were conducted during the one-year post-COVID-19 lockdown.Neurology teams in the local area were the main source of referrals during the two study periods, referring 59% and 51% of total referrals during the two evaluation periods respectively. Other referral sources included local memory service, inpatient psychiatric units, community mental health teams, neuropsychology, neurorehabilitation, palliative care and acute medicine. The primary management model was multidisciplinary. 49% of assessment contacts were made by specialist nursing during the first evaluation period. 48% of assessment contacts were made by the medical staff during the post-lockdown period. 13.3% of patients did not attend their appointments during the first period, dropping to 9.8% in the Post-Lockdown period.Most patients who completed treatment were discharged to GP care (89% pre-COVID-19 and 94% post-lockdown). 12% patients from Pre-Lockdown period were still receiving care at the end of one year and 35% were still receiving care in at the end of post-lockdown period.ConclusionThe service evaluation identifies systemic differences in service use characteristics during Pre-lockdown and Post-lockdown periods.

A Matter of Timing: Rudy Tanzi on Alzheimer's Disease

A Matter of Timing: Rudy Tanzi on Alzheimer's Disease

Developing Poetry as a Research Methodology with Rarer Forms of Dementia: Four Research Protocols

People living with rarer forms of dementia tend to have progressive cognitive symptoms affecting skills other than memory and/or onset before the age of 65 years. They are often misdiagnosed and due to symptom profile or age of onset, do not usually fit well with care pathways designed for older people with typical Alzheimer’s disease or vascular dementia. Although the arts have been increasingly used as interventions to support people with dementia, there is very little attention given to rarer dementia forms in arts and health research or practice. The objective of the present international study seeks to systematically explore four diverse forms of poetry writing within this population through virtual and in-person small and large group formats. Our approach includes investigating poetic processes as methodology through the lens of an arts-based methodological approach in order to explore how poems construct knowledge and a felt experience. We will also use more traditional qualitative approaches to understand the experience of writing, reading and listening to poetry as an intervention that can be used with different rarer forms of dementia. To the best of our knowledge, this will be the first study to explore poetry using multiple research protocols. The results will have implications for methodology development, co-constructed poetic inquiry and multiple opportunities for involving poetry in supporting people with dementia and family members.

A Pilot Longitudinal Evaluation of MicroRNAs for Monitoring the Cognitive Impairment in Pediatric Multiple Sclerosis

MicroRNAs (miRNAs), a class of non-coding RNAs, seem to play a key role in complex diseases like multiple sclerosis (MS), as well as in many cognitive functions associated with the disease. In a previous cross-sectional evaluation on pediatric MS (PedMS) patients, the expression of some miRNAs and their target genes were found to be associated with the scores of some neuropsychiatric tests, thus suggesting that they may be involved in early processes of cognitive impairment. To verify these data, we asked the same patients to be re-evaluated after a 1-year interval; unfortunately, only nine of them agreed to this further clinical and molecular analysis. The main results showed that 13 differentially expressed miRNAs discriminated the two time-points. Among them, the expression of miR-182-5p, miR-320a-3p, miR-744-5p and miR-192-5p significantly correlated with the attention and information processing speed performances, whereas the expression of miR-182-5p, miR-451a, miR-4742-3p and miR-320a-3p correlated with the expressive language performances. The analysis of mRNA expression uncovered 58 predicted and/or validated miRNA-target pairs, including 23 target genes, some of them already associated with cognitive impairment, such as the transducing beta like 1 X-linked receptor-1 gene (TBL1XR1), correlated to disorders of neurodevelopment; the Snf2 related CREBBP activator protein gene (SRCAP) that was found implicated in a rare form of dementia; and the glia maturation factor beta gene (GMFB), which has been reported to be implicated in neurodegeneration and neuroinflammation. No molecular pathways involving the most targeted genes survived the adjustment for multiple data. Although preliminary, these findings showed the feasibility of the methods also applied to longitudinal investigations, as well as the reliability of the obtained results. These findings should be confirmed in larger PedMS cohorts in order to identify early markers of cognitive impairment, towards which more efficient therapeutic efforts can be addressed.

Frontotemporal dementia: diagnosis, treatment and medical management

Frontotemporal dementia is a rare form of dementia and around 2% of people with dementia have frontotemporal dementia ( Warren et al, 2013 ). This article explains the pathophysiology and clinical features of frontotemporal dementia and how it is diagnosed, treated and managed.

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

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. 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. The UCL Research Ethics Committee have approved this study. Data collection commenced in January 2020. 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.

Dementia 6. Frontotemporal dementia: diagnosis, treatment and medical management

Frontotemporal dementia is a rare form of dementia and around 2% of people with dementia have frontotemporal dementia ( Warren et al, 2013 ). This article, the sixth in a series, explains about the pathophysiology and clinical features of frontotemporal dementia and how it is diagnosed, treated and managed.

MR fingerprinting as a diagnostic tool in patients with frontotemporal lobe degeneration: A pilot study.

Several very rare forms of dementia are associated with characteristic focal atrophy predominantly of the frontal and/or temporal lobes and currently lack imaging solutions to monitor disease. Magnetic resonance fingerprinting (MRF) is a recently developed technique providing quantitative relaxivity maps and images with various tissue contrasts out of a single sequence acquisition. This pilot study explores the utility of MRF-based T1 and T2 mapping to discover focal differences in relaxation times between patients with frontotemporal lobe degenerative dementia and healthy controls. 8 patients and 30 healthy controls underwent a 3T MRI including an axial 2D spoiled gradient echo MRF sequence. T1 and T2 relaxation maps were generated based on an extended phase graphs algorithm-founded dictionary involving inner product pattern matching. A region of interest (ROI)-based analysis of T1 and T2 relaxation times was performed with FSL and ITK-SNAP. Depending on the brain region analyzed, T1 relaxation times were up to 10.28% longer in patients than in controls reaching significant differences in cortical gray matter (P=.047) and global white matter (P=.023) as well as in both hippocampi (P=.001 left; P=.027 right). T2 relaxation times were similarly longer in the hippocampus by up to 19.18% in patients compared with controls. The clinically most affected patient had the most control-deviant relaxation times. There was a strong correlation of T1 relaxation time in the amygdala with duration of the clinically manifest disease (Spearman Rho=.94; P=.001) and of T1 relaxation times in the left hippocampus with disease severity (Rho=.90, P=.002). In conclusion, MRF-based relaxometry is a promising and time-saving new MRI tool to study focal cerebral alterations and identify patients with frontotemporal lobe degeneration. To validate the results of this pilot study, MRF is worth further exploration as a diagnostic tool in neurodegenerative diseases.