Young-onset Dementia Research Articles

Frontotemporal Dementia: A Clinical Review

Frontotemporal dementia is a disease in which atrophic changes occur in the frontal lobes and frontal temporal lobes of the brain. Frontotemporal dementias are a clinically, neuroanatomically and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. The most common form of frontotemporal dementia is the so-called behavioral variant of frontotemporal dementia. Underlying these pathological changes is the degeneration of nerve cells (i.e. neurons), which occurs through the accumulation of abnormal proteins inside them. Therefore, the review of current studies in the subject of Frontotemporal dementia was conducted in order to access possible risk factors and new ways of management and treatment of this complex disease.

Interventions for Persons with Young-Onset Dementia and Their Families: A Scoping Review.

Dementia occurring before age 65 is known as young-onset dementia (YOD), with Alzheimer's disease being the most common type. YOD poses unique challenges for persons and families, impacting their working-age years and family responsibilities. Person-centered interventions and services are essential to improve their quality of life and social engagement. This study aims to synthesize non-pharmacological interventions for persons with YOD and their families to inform future targeted interventions. We conducted a systematic literature search across four databases: PubMed, PsycINFO, Scopus, and CINAHL. The included articles were carefully screened, categorized, and synthesized by following Arksey and O'Malley's five stages framework. We included 20 studies reported in 24 papers, with 11 studies (14 papers) on persons with YOD and nine studies (10 papers) on families. Quantitative intervention results vary, but qualitative interviews show positive feedback. Stakeholders provided positive evaluations, stating these interventions provided a sense of normalcy, facilitated communication among families, enhanced the independence of persons with YOD, and improved the families' caregiving self-efficacy, thereby reducing care burden and psychological distress. The heterogeneity among the studies posed integration challenges. Interventions for YOD can improve the quality of life for both persons with YOD and their families. More extensive intervention studies are urgently needed, especially in developing countries, with a focus on family-centered and life course perspectives. In future intervention research design, a more extensive incorporation of stakeholder involvement is essential for successful implementation. Moreover, the integration of new technologies shows promise as a potential avenue for intervention advancement.

Can psychotic symptom identification help to improve Young-onset dementia care?

Can psychotic symptom identification help to improve Young-onset dementia care?

Social care planning and provision for people with young onset dementia and their families: Protocol for the DYNAMIC study.

Social care is vital to quality of life for people with young onset dementia and their families. Yet care is hugely variable, frequently lacking and poorly coordinated. We aim to establish current practice in English social care for people with young onset dementia and co-produce evidence-based recommendations and resources for improvement. In Work-Package 1, we will gather qualitative data from 25 people with young onset dementia and/or main supporters residing in England. We will ask them about their experiences of social care (broadly defined, including independent and voluntary sector provision) and suggestions for improvement. In Work-Package 2, we will conduct a short on-line survey with a wide range of staff with a role in adult social care in England. We will find out about current awareness, knowledge and practice and suggestions for improvements. Quantitative and qualitative analysis will provide a picture of current practice. In Work-Package 3, we will use convergence analysis to synthesise the findings from Work-Packages 1 and 2 and present the findings to a stakeholder workshop, to identify feasible priorities for improvement. We will establish what is already known about good practice relating to these key priorities using a scoping review and interviews with professionals. This knowledge will then feed into the co-production of resources and recommendations with key stakeholders to improve social care for people with young onset dementia and their families. This study seeks to address a gap in our understanding of social care provision for people with young onset dementia and develop recommendations and practical resources for improvements. The findings will help people with young onset dementia and supporters to receive higher quality social care. Study registration number: ISRCTN10653250.

Supporting a family living with young-onset dementia: a case study

In the UK, it is estimated that people living with young-onset dementia (YOD) accounts for a small, but significant, number of the total population living with dementia. The diagnosis happens at an unexpected time in one's life, and as a life-limiting condition, there are implications for the whole family, including managing employment, finances and debt, supporting dependent children and accessing age-appropriate support services. YOD causes a disproportionate burden on family members and appropriate care services can be challenging to find. The case study discussed follows a family's journey from pre-diagnosis, to subsequently engaging with the Admiral Nurse Helpline and then an Admiral Nurse in the family's locality, and considers the issues and challenges faced by them.

Diagnostic challenges and disparities in young-onset dementia: insights from a Southeast London memory clinic study.

Diagnostic challenges and disparities in young-onset dementia: insights from a Southeast London memory clinic study.

Role of behavioural problems in carer burden in young onset dementia.

Role of behavioural problems in carer burden in young onset dementia.

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

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. 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. 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 β 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%). 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. None.

Investigating Equivalence of In-Person and Telehealth-Based Neuropsychological Assessment Performance for Individuals Being Investigated for Younger Onset Dementia.

Given the rapid shift to in-home teleneuropsychology models, more research is needed to investigate the equivalence of non-facilitator models of teleneuropsychology delivery for people with younger onset dementia (YOD). This study aimed to determine whether equivalent performances were observed on neuropsychological measures administered in-person and via teleneuropsychology in a sample of people being investigated for YOD. Using a randomized counterbalanced cross-over design, 43 participants (Mage = 60.26, SDage = 7.19) with a possible or probable YOD diagnosis completed 14 neuropsychological tests in-person and via teleneuropsychology, with a 2-week interval. Repeated measures t-tests, intraclass correlation coefficients (ICC), and Bland Altman analyses were used to investigate equivalence across the administration conditions. No statistical differences were found between in-person and teleneuropsychology conditions, except for the Hospital Anxiety and Depression Scale Anxiety subtest. Small to negligible effect sizes were observed (ranging from .01 to .20). ICC estimates ranged from .71 to .97 across the neuropsychological measures. Bland Altman analyses revealed that the Wechsler Adult Intelligence Scale-Fourth Edition Block Design subtest had slightly better overall performance in the in-person condition and participants reported higher levels of anxiety symptoms during the teleneuropsychology condition; however, average anxiety symptoms remained within the clinically normal range. Participants reported a high level of acceptability for teleneuropsychology assessments. These results suggest that performances are comparable between in-person and teleneuropsychology assessment modalities. Our findings support teleneuropsychology as a feasible alternative to in-person neuropsychological services for people under investigation of YOD, who face significant barriers in accessing timely diagnoses and treatment options.

A Rare Cause of Dementia in a Young Adult – A Case Report

Frontotemporal dementia is a clinically and pathologically heterogeneous group of disorders with two main phenotypes; the commoner being a disorder of behavior and function. This dementia is most common before age 60 with more than half being seen in the age range 45-65 years. We report the case of a 27-year-old gentleman who presented with behavioral & personality changes 2 years before his presentation in our clinic. A neurological examination revealed a restless patient with gibberish speech and a positive snout reflex. The rest of the examination was non-focal. MRI brain showed significant atrophy of frontal and temporal lobes with intact parietal and occipital cortex. Based on clinical and radiological findings, we made the diagnosis of Frontotemporal dementia. Our case emphasizes the need to differentiate young-onset dementia from primary psychiatric disorders which are relatively more common at a young age.

Optimising Online Peer Support for People with Young Onset Dementia.

People with Young Onset Dementia (YOD) can be hesitant to engage with online peer support. This work aims to explore (1) why people are hesitant to engage in online peer support, (2) how to get more people involved in online peer support, and (3) what makes online peer support work well. Nine interviews with people with YOD were conducted on MS Teams. Participants were recruited through purposive sampling. Data were analysed thematically. Reasons for being hesitant to engage with online peer support include being unsure what to expect and concerns about seeing others in more advanced stages of dementia. Additionally, it can be difficult to identify groups that suit one's needs and interests. Group facilitators of online peer support groups should provide a detailed description of their group so that people can better assess whether the group would suit them. The insights obtained from this study will be used to develop a Best Practice Guidance on online peer support for people with YOD. Moreover, the findings can be useful for further research exploring how to support people with dementia in general in accessing online health and social care services.

Younger people with dementia registered to public mental health services in Victoria, Australia.

Objectives Individuals living with young-onset dementia fall through the gaps of adult and geriatric services. Given that non-cognitive symptoms of dementia in younger people are often psychiatric and behavioural in nature, these people may contact mental health services. There is sparse information investigating the frequency of people with young-onset dementia who contact mental health triage in Victoria. Methods Triage data were obtained from the Victorian Agency for Health Information. The data contained demographics for people registered with Victorian mental health services. Consumers who had an International Classification for Diseases code for dementia and were aged less than 65 years were identified as people with young-onset dementia. Using information of the frequency of people with young-onset dementia who were registered with each area-based mental health service, population census statistics were used to make estimates of the frequency of young-onset dementia. Results Of the 6172 people who had a dementia diagnosis, 1020 of them were aged less than 65 years and had young-onset dementia. There were proportionally more men who had young-onset dementia compared to women. There were more people with young-onset dementia registered to rural mental health services compared to metropolitan services. Conclusion Findings provide important information for service planning in Victoria. Recommendations include upskilling and education for the assessment of dementia for those clinicians who work in mental health services, particularly in rural regions.

Experience of Younger-Onset Dementia Stigma: Protocol for a Constructivist Grounded Theory Study Based on Qualitative Episodic Interviews With People Living With Dementia and Their Significant Others

Stigma based on younger-onset dementia (YOD) is a significant challenge for individuals living with the condition and their significant others (i.e., family and/or friends) as well as for a society that aspires to be inclusive, ensure social participation, and enable all people to exercise their civil and human rights. The STELDA study aims to develop a theoretical framework of YOD stigma and stigmatization that is empirically grounded in the lived experience of people living with younger-onset dementia (PlwYOD) and their significant others and to identify ways of counteracting YOD stigmatization processes in the sociocultural context of Germany on a lifeworld level. The research question is as follows: How do PlwYOD and their adult significant others experience stigma and stigmatization based on YOD in their lifeworlds, and what implications do these experiences have for their actions in their lifeworlds? The study is designed as a constructivist grounded theory (CGT) study. Qualitative episodic interviews with community-dwelling PlwYOD and their adult significant others will be conducted to give participants the opportunity to share their individual narratives and express their subjective views. As an outcome, recommendations for action will be developed for the public and for professionals in diverse settings. On a methodological level, novel insights will be gained regarding specific issues related to qualitative research with PlwYOD and their significant others.

Referral reasons to a Victorian mental health triage service for individuals living with young-onset dementia.

Objectives Mental health triage services are the first contact for people wanting support for their mental health and provide access to public mental health services in Victoria, Australia. People living with young-onset dementia and their families may contact triage services seeking assistance for behaviour changes and pathways to care as there are few alternative services available. Methods The authors reviewed the minimum triage dataset from one of the largest mental health services in Victoria, Australia from 2018 to 2021 investigating reasons for referral for people with young-onset dementia. Results Of the 1766 referrals for 'dementia', 145 were for young-onset dementia. Of these, 69% were referred for behaviour changes. About half of the referrals for behaviour changes were triaged as 'advice only'. One-third of referrals were from general practitioners. Conclusions The availability of dementia-specific support services that could provide advice on dementia as well as advice for behaviour changes related to the disease could potentially ease the burden of phone calls received by mental health triage services.

Characteristics of domestic violence perpetrators with dementia from police records using text mining.

Few studies have examined the characteristics of domestic violence (DV) committed by people with dementia. We provide an overview of DV perpetrated by people with dementia in the community based on police reports of attendances at DV events. A text mining method was used on 416,441 New South Wales (NSW) police narratives of DV events from January 2005 to December 2016 to extract information for Persons of Interest (POIs) with mentions of dementia. Events involving those with dementia accounted for a relatively low proportion of total DV events (<1%). Of the 260 DV events with a dementia mention for the POI, the most common abuse types were assault (49.7%) and verbal abuse (31.6%). Spouses were the largest group of victims (50.8%) followed by children (8.8%). Physical abuse was common, occurring in 82.4% of events, but injuries were relatively mild. Although weapons were infrequently used, they were involved in 5% of events, mostly by POIs aged 75 years and older. Similarly, the POIs were mainly aged 75+ years (60%), however the proportion of those aged <65 was relatively high (20.8%) compared to the reported prevalence of dementia in that age group. This study demonstrates that some cases of DV perpetrated by people with reported dementia are significant enough to warrant police involvement. This highlights the need to proactively discuss the potential for violence as part of the holistic management and support family members, particularly those caring for people with young-onset dementias.

Incidence of young-onset dementia in Italy: The Brescia register study.

The goal of the present work was to assess the incidence of dementia with onset before the age of 65 years (i.e., young-onset dementia [YOD]) and define the frequencies of young-onset Alzheimer's disease (AD), frontotemporal lobar degeneration (FTLD), and dementia with Lewy bodies (DLB) in the general population. The study was conducted from January 1, 2019 to December 31, 2019 in Brescia province (population: 1,268,455). During the study period, all new YOD cases (incident YOD) were counted, and all patients' records reviewed. The incidence was standardized to the Italian general population in 2019. A total of 29 YOD patients were diagnosed. The age-sex standardized incidence rate was 4.58 (95% confidence interval, 3.07-6.58) per 100,000 person-years. No difference in incidence rate between YOD due to AD or FTLD (P=0.83) and between sexes (P=0.81) was observed. YOD incidence increased with age, reaching its peak after 60 years. Presenting neurodegenerative YOD phenotypes encompasses both AD and FTLD. Improved knowledge on YOD epidemiology is essential to adequately plan and organize health services.

Cognitive-Linguistic Profiling in Young Onset Dementia: A case Study

The onset of dementia is after 65 years. Though there are evidence reported in the past regarding the onset of dementia before this age, it is very rare. Studies carried out in the past have concentrated on the site of lesion, genetic base for the young onset dementia and the studies focusing on cognitive linguistic profiling are extremely rare. The current study focussed on the cognitive linguistic profiling and profiling the effect of dementia on the activities of daily living. The findings suggested that the cognitive linguistic deficit was very severe and had a note-worthy impact on the activities of daily living. This is pivotal in designing the plan of intervention and deciding the course of treatment.

Risk Factors for Young-Onset Dementia in the UK Biobank

There is limited information on modifiable risk factors for young-onset dementia (YOD). To examine factors that are associated with the incidence of YOD. This prospective cohort study used data from the UK Biobank, with baseline assessment between 2006 and 2010 and follow-up until March 31, 2021, for England and Scotland, and February 28, 2018, for Wales. Participants younger than 65 years and without a dementia diagnosis at baseline assessment were included in this study. Participants who were 65 years and older and those with dementia at baseline were excluded. Data were analyzed from May 2022 to April 2023. A total of 39 potential risk factors were identified from systematic reviews of late-onset dementia and YOD risk factors and grouped into domains of sociodemographic factors (education, socioeconomic status, and sex), genetic factors (apolipoprotein E), lifestyle factors (physical activity, alcohol use, alcohol use disorder, smoking, diet, cognitive activity, social isolation, and marriage), environmental factors (nitrogen oxide, particulate matter, pesticide, and diesel), blood marker factors (vitamin D, C-reactive protein, estimated glomerular filtration rate function, and albumin), cardiometabolic factors (stroke, hypertension, diabetes, hypoglycemia, heart disease, atrial fibrillation, and aspirin use), psychiatric factors (depression, anxiety, benzodiazepine use, delirium, and sleep problems), and other factors (traumatic brain injury, rheumatoid arthritis, thyroid dysfunction, hearing impairment, and handgrip strength). Multivariable Cox proportional hazards regression was used to study the association between the risk factors and incidence of YOD. Factors were tested stepwise first within domains and then across domains. Of 356 052 included participants, 197 036 (55.3%) were women, and the mean (SD) age at baseline was 54.6 (7.0) years. During 2 891 409 person-years of follow-up, 485 incident YOD cases (251 of 485 men [51.8%]) were observed, yielding an incidence rate of 16.8 per 100 000 person-years (95% CI, 15.4-18.3). In the final model, 15 factors were significantly associated with a higher YOD risk, namely lower formal education, lower socioeconomic status, carrying 2 apolipoprotein ε4 allele, no alcohol use, alcohol use disorder, social isolation, vitamin D deficiency, high C-reactive protein levels, lower handgrip strength, hearing impairment, orthostatic hypotension, stroke, diabetes, heart disease, and depression. In this study, several factors, mostly modifiable, were associated with a higher risk of YOD. These modifiable risk factors should be incorporated in future dementia prevention initiatives and raise new therapeutic possibilities for YOD.

THE MANY FACES OF DEMENTIA IN CANADA: EXAMINING SEX, ETHNICITY, AND AGE OF ONSET IN THE LANDMARK STUDY

Abstract With the rapidly increasing size of the dementia population, it is important to enhance our understanding of the similarities and differences that are found across the people living with condition. This work takes a closer look at the many faces of dementia in Canada by highlighting the diversity that is found within the national population projections for dementia. To conduct these forecasts, a simulation model was developed using demographic characteristics and risk factors for dementia to estimate the numbers of people living with dementia in Canada from 2020-2050. From the results of the Landmark Study, we will highlight findings related to sex, Indigenous Peoples, ethnic origins, and young-onset dementia. In 2020, 61.3% of dementia diagnoses were in females and this sex ratio is projected to stay constant over the three decades. For Indigenous Peoples of Canada, dementia numbers are estimated to increase by 273% (2020: 10,800; 2050: 40,300). People of Asian origin will increase from 8% of people living with dementia in 2020 to 24% by 2050. The changes are driven both by future immigration, and by people who have already immigrated to Canada in the past few decades. The Landmark Study also projects that there could be over 40,000 people under the age of 65 living with dementia in Canada by 2050.These findings illustrate the changing landscape of people living with dementia in Canada. These demographic characteristics, as well as other distinctions across population groups, profoundly affect the way in which dementia is experienced and their care needs.

FC19: Remaining engaged through work in young onset dementia: first results of the WorkDEM study

Background:Focus on the capacity and potential of persons with dementia is needed to enable people and their families to adapt to the changes dementia brings in their lives. For those with young onset dementia (YOD), support to remain in work for as long as possible can preserve one’s self-esteem and sense of purpose in life. However, guidance on how to support people with YOD at their workplace is lacking. This study therefore aims to explore experiences, work values, and support needs of people with YOD in the workplace and other stakeholders involved.Methods:In this qualitative study, semi-structured interviews were held with several target groups namely employees with an established dementia diagnosis, relatives of employees with YOD, employers and co-workers, occupational physicians, human resource professionals, and healthcare professionals involved in dementia care. A topic guide was developed, based on recent literature and consultation of experts, and addressed the following themes: experiences regarding the influence of dementia in the workplace, values such as the importance of work, and support needs in the workplace. Themes were explored in the period before and after diagnosis. The interview data were transcribed and analyzed by means of an inductive content analysis.Results:In total 33 semi-structured interviews were conducted. Eight themes were derived from the interviews in people with YOD, namely 1) difficulties experienced at work, 2) long diagnostic trajectory and involvement of work-and care professionals, 3) Impact of YOD and coping with YOD, 4) Wish to work, 5) Diagnostic disclosure, 6) The role of the work environment, 7) Phasing out work and future perspectives, and 8) Perception and awareness of YOD. Data of the other target groups is currently being analysed. These results are expected in spring 2023.Conclusion:This study will result into a better understanding of the possibilities of working with YOD. These insights can be used as a starting point to develop practical tools to support and provide guidance to people with YOD and their (work)environment to prevent loss of work or find meaningful alternatives.