We aimed to understand the facilitators to developing and sustaining high-quality services for people with young onset dementia (YOD) and their families/supporters.

This qualitative study used semi-structured interviews with commissioners and service managers, analyzed using inductive thematic analysis.

A purposive sample of providers was selected from diverse areas and contrasting YOD services.

Eighteen senior staff from YOD services and two dementia service commissioners took part.

For commissioners, key interview topics were experiences of commissioning YOD services, perceived facilitators or barriers, and how future guidance should be structured for ease of use. For service providers, key topics explored experiences of delivering YOD services; what was achievable or challenging; how the service was funded; how it linked with broader provision for YOD in the area; and how guidance should be structured.

Recorded interviews lasted 30–40 minutes. Seven key facilitators to the development and sustaining of YOD services were identified: having knowledgeable, committed local champions; involvement of people living with YOD and family supporters; initial delivery within existing resources; partnership working within and between sectors; having a reflective, supportive organizational culture; gathering evidence of impact; and having wider support and guidance.

Improvements in provision for those with YOD and their families need to be built on understanding of service-level and interpersonal influences as well as on understanding of YOD itself. Our findings highlight a set of facilitators which need to be in place to establish and sustain high-quality YOD services that fit the local context.

A number of studies have compared Alzheimer’s disease (AD), the commonest form of dementia, based on their age of onset, i.e. before the age of 65 years (early-onset AD, EO-AD) to those developing after 65 years of age (late-onset AD, LO-AD), but the differences are not clear. We performed a systematic review and meta-analysis to compare clinical characteristics between EO-AD and LO-AD.

Medline, Embase, PsycINFO, and CINAHL databases were systematically searched for studies comparing time to diagnosis, cognitive scores, annual cognitive decline, activities of daily living (ADLs), neuropsychiatric symptoms (NPS), quality of life (QoL), and survival time for EO-AD and LO-AD patients.

Forty-two studies were included (EO-AD participants n = 5,544; LO-AD participants n = 16,042). An inverse variance method with random effects models was used to calculate overall effect estimates for each outcome. People with EO-AD had significantly poorer baseline cognitive performance and faster cognitive decline but longer survival times than people with LO-AD. There was no evidence that EO-AD patients differ from people with LO-AD in terms of symptom onset to diagnosis time, ADLs, and NPS. There were insufficient data to estimate overall effects of differences in QoL in EO-AD compared to LO-AD.

Our findings suggest that EO-AD differs from LO-AD in baseline cognition, cognitive decline, and survival time but otherwise has similar clinical characteristics to LO-AD. Larger studies using standardized questionnaires focusing on the clinical presentations are needed to better understand the impact of age of onset in AD.

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.

Carer burden is common in younger-onset dementia (YOD), often due to the difficulty of navigating services often designed for older people with dementia. Compared to Alzheimer’s disease (AD), the burden is reported to be higher in behavioral variant frontotemporal dementia (bvFTD). However, there is little literature comparing carer burden specifically in YOD. This study hypothesized that carer burden in bvFTD would be higher than in AD.

Retrospective cross-sectional study.

Tertiary neuropsychiatry service in Victoria, Australia.

Patient-carer dyads with YOD.

We collected patient data, including behaviors using the Cambridge Behavioral Inventory-Revised (CBI-R). Carer burden was rated using the Zarit Burden Inventory-short version (ZBI-12). Descriptive statistics and Mann-Whitney U tests were used to analyze the data.

Carers reported high burden (ZBI-12 mean score = 17.2, SD = 10.5), with no significant difference in burden between younger-onset AD and bvFTD. CBI-R stereotypic and motor behaviors, CBI-R everyday skills, and total NUCOG scores differed between the two groups. There was no significant difference in the rest of the CBI-R subcategories, including the behavior-related domains.

Carers of YOD face high burden and are managing significant challenging behaviors. We found no difference in carer burden between younger-onset AD and bvFTD. This could be due to similarities in the two subtypes in terms of abnormal behavior, motivation, and self-care as measured on CBI-R, contrary to previous literature. Clinicians should screen for carer burden and associated factors including behavioral symptoms in YOD syndromes, as they may contribute to carer burden regardless of the type.

Awareness of dementia is examined in different scientific fields as significant for assessment of diagnosis, and for treatment and adaptation to the disease. There are very few longitudinal studies of individual experiences of awareness among people with dementia, related to quality of life.

To examine how younger people (< 65 years) with dementia (YOD) express awareness of the dementia and how, over time, they seem to handle awareness as a strategy to preserve quality of life.

A longitudinal qualitative study with individuals with YOD was performed with interviews every six months over five years for a maximum of ten interviews. The interviews were analysed by modified grounded theory.

Awareness is a complex, multidimensional concept. Awareness of dementia is predisposed by personality, life history and established coping styles. The main coping styles – live in the moment, ignore the dementia, and make the best of it – seem to be rather consistent throughout the progression of the disease. Transitions in life situation, such as moving to a nursing home, may change the individual’s awareness of dementia.

Unawareness of dementia may have an important adaptive function to preserve quality of life. To increase awareness must be approached with reflexivity and the utmost sensitivity.

Routine psychiatric assessments tailored to older patients are often insufficient to identify the complexity of presentation in younger patients with dementia. Significant overlap between psychiatric disorders and neurodegenerative disease means that high rates of prior incorrect psychiatric diagnosis are common. Long delays to diagnosis, misdiagnosis and lack of knowledge from professionals are key concerns. No specific practice guidelines exist for diagnosis of young-onset dementia (YOD).

The review evaluates the current evidence about best practice in diagnosis to guide thorough assessment of the complex presentations of YOD with a view to upskilling professionals in the field.

A comprehensive search of the literature adopting a scoping review methodology was conducted regarding essential elements of diagnosis in YOD, over and above those in current diagnostic criteria for disease subtypes. This methodology was chosen because research in this area is sparse and not amenable to a traditional systematic review.

The quality of evidence identified is variable with the majority provided from expert opinion and evidence is lacking on some topics. Evidence appears weighted towards diagnosis in frontotemporal dementia and its subtypes and young-onset Alzheimer's disease.

The literature demonstrates that a clinically rigorous and systematic approach is necessary in order to avoid mis- or underdiagnosis for younger people. The advent of new disease-modifying treatments necessitates clinicians in the field to improve knowledge of new imaging techniques and genetics, with the goal of improving training and practice, and highlights the need for quality indicators and alignment of diagnostic procedures across clinical settings.

None.

Frontotemporal lobar degeneration (FTLD) is the second-most common cause of young-onset dementia. Personality and behavior changes lead to high caregiver stress and burden, but little support is available. Our aim is to present the evidence on the characteristics, challenges and unmet needs of caregivers as well as on possible interventions.

We conducted a scoping review on caregiver burden using PubMed, Web of Science and ScienceDirect. A total of 69 articles were considered eligible and were analyzed in the present study.

Through the analysis of 69 empirical articles, our results show that caregivers of patients with FTLD are often younger in age, have children and find behavioral disturbances to be the most burdensome. Nine studies assessed the needs of and support for caregivers. Ten studies compared the burden in different forms of FTLD, 19 compared FTLD to other types of dementia, and one compared the caregiver burden between two countries. Eight studies reported on interventions for caregivers or interventions taking burden into account. One study assessed the support structure for caregivers of FTLD patients. Five case reports, eight research overviews and three reviews addressed specific needs and challenges.

Further research should reproduce and validate efficacious interventions and focus on underage children of FTLD patients and findings from non-Western countries. Additionally, support structures for FTLD caregivers should be assessed and extended. Awareness both in the wider population and among healthcare professionals is an urgent need for the future.

The aim of this study was to investigate survival time and life-expectancy in people with young-onset dementia (YOD) and to examine the relationship with age, sex, dementia subtype and comorbidity.

Survival was examined in 198 participants in the Needs in Young-onset Dementia study, including participants with Alzheimer’s dementia (AD), vascular dementia (VaD) and frontotemporal dementia (FTD).

The primary outcomes were survival time after symptom onset and after date of diagnosis. Cox proportional hazards models were used to explore the relationship between survival and age, sex, dementia subtype and comorbidity. Additionally, the impact on remaining life expectancy was explored.

During the six-year follow-up, 77 of the participants died (38.9%), 78 participants survived (39.4%) and 43 were lost to follow-up (21.7%). The mean survival time after symptom onset and diagnosis was 209 months (95% CI 185-233) and 120 months (95% CI 110-130) respectively. Participants with AD had a statistically significant shorter survival compared with VaD participants, both regarding survival after symptom onset (p = 0.047) as well as regarding survival after diagnosis (p = 0.049). Younger age at symptom onset or at diagnosis was associated with longer survival times. The remaining life expectancy, after diagnosis, was reduced with 51% for males and 59% for females compared to the life expectancy of the general population in the same age groups.

It is important to consider the dementia subtype when persons with YOD and their families are informed about the prognosis of survival. Our study suggests longer survival times compared to other studies on YOD, and survival is prolonged compared to studies on LOD. Younger age at symptom onset or at diagnosis was positively related to survival but diagnosis at younger ages, nevertheless, still diminishes life expectancy dramatically.

Both neuropsychiatric symptoms (NPS) and psychotropic drug use (PDU) are common in institutionalized People with Young Onset Dementia (PwYOD) and can produce negative outcomes such as reduced quality of life and high workload. In community-dwelling PwYOD, NPS are found to be associated with unmet care needs. This emphasizes the importance of a care program for the management of NPS in institutionalized PwYOD that also addresses unmet care needs and PDU. The objectives of the Behavior and Evolution of Young ONset Dementia part 2 (BEYOND-II) study are to develop a care program for the management of NPS in institutionalized PwYOD and to evaluate its effectiveness.

The care program consists of an educational program combined with an intervention to manage NPS with the following five steps: the evaluation of psychotropic drug prescription, detection, analysis (including the detection of unmet needs), treatment and the evaluation of NPS. A stepped wedge design will be used to evaluate its effectiveness. The primary outcomes are agitation and aggression and other NPS. The secondary outcomes are PDU, quality of life, the workload of nursing staff and job satisfaction. Additionally, a process analysis and a cost-consequence analysis will be conducted.

The study protocol of the Beyond-II study describes the development, implementation and evaluation of a care program for the management of NPS in institutionalized PwYOD. This care program provides a structured method for the management of NPS, in which unmet needs and PDU are also addressed.

Caregiver burden is a significant issue in the treatment of dementia and a known contributor to institutionalization of patients with dementia. Published data have documented increased caregiver burden in behavioral variant frontotemporal dementia (bvFTD) compared to Alzheimer's disease (AD). Another atypical dementia with high-perceived caregiver burden is sporadic Creutzfeldt–Jakob disease (sCJD), but no formal studies have assessed this perception. The aim of this study was to compare caregiver burden across atypical dementia etiologies.

76 adults with atypical dementia (young-onset AD [YOAD], bvFTD, language variant FTD [lvFTD], and sCJD) were administered an abbreviated version of the Zarit Burden Interview (ZBI), Neuropsychiatric Inventory (NPI-Q), and other assessment instruments during a five-year time period at Johns Hopkins Hospital (JHH). A Cox regression model examined differences between disease categories that impact mean ZBI scores.

Mean ZBI scores were significantly different between dementia etiologies, with bvFTD and sCJD having the highest caregiver burden (p = 0.026). Mean NPI-Q caregiver distress scores were highest in bvFTD and sCJD (p = 0.002), with sCJD and bvFTD also having the highest number of endorsed symptom domains (p = 0.012). On regression analyses, an interactive variable combining final diagnosis category and NPI-Q total severity score demonstrated statistically significant differences in mean ZBI scores for sCJD and bvFTD.

This study demonstrates that bvFTD and sCJD have increased levels of caregiver burden, NPI-Q caregiver distress, total severity scores, and number of endorsed symptom domains. These results suggest that higher caregiver burden in bvFTD and sCJD are disease specific and possibly related to neuropsychiatric symptoms.

Service planning for people with younger onset dementia (YOD; an onset of symptoms before the age of 65 years) relies on prevalence estimates, with existing models based upon older people. This pilot study investigated the prevalence and causes of YOD in a defined catchment area of Eastern Sydney, Australia.

The study was conducted in three stages: publicity building, case finding, and case validation. A brief structured questionnaire was sent to health professionals in the catchment area asking how many patients with YOD they had seen over the previous 12 months. Memory clinics and hospital records were also searched for YOD patients. Clinicians assigned a Statistical Linkage Key to each patient to prevent double counting, and indicated the cause of dementia. The majority of patients were validated by a review of medical case notes. Prevalence data were calculated for the following age groups: 30–64, 30–44, and 45–64 years.

Two hundred and four potential patients were identified, of which 141 met inclusion criteria. The primary clinical subtypes were alcohol-related dementia (18.4%), Alzheimer's disease (17.7%), vascular dementia (12.8%), and frontotemporal dementia (11.3%). Eighty-eight patients were aged 30 to 64 years on census date and were therefore included in the prevalence calculations. The overall prevalence was 68.2 per 100,000 population at risk for the 30–64-year age group (95% Confidence Interval (CI): 54.9–83.4); 11.6 per 100,000 for the 30–44-year age group (95% CI: 5.3–21.7); and 132.9 per 100,000 for the 45–64 age group (95% CI: 105.8–164.2).

Younger onset dementia affects a significant number of people in Eastern Sydney with a diverse range of clinical types. This prevalence rate is higher than previous reports from the United Kingdom and Japan, with a different distribution of etiologies, which have important implications for service planning for this group.

People with Young Onset Dementia (YOD) have specific needs for care. These people eventually require institutional care, usually delivered by institutions designed for the elderly. The Dutch network of care organizations delivering specialized YOD care offers a unique opportunity to obtain more knowledge of this special population.

Our cross-sectional study collected data from 230 people with YOD in eight care homes providing YOD specialized care. Data collected: demographic data, disease duration, dementia subtype, comorbidity, dementia severity (Global Deterioration Scale – GDS), neuropsychiatric symptoms (NPS; Neuropsychiatric Inventory – NPI, Cohen Mansfield Agitation Inventory – CMAI), disease awareness (Guidelines for the Rating of Awareness Deficits – GRAD), need for assistance (hierarchic Activities of Daily Living (ADL) scale – Resident Assessment Instrument – Minimum Data Set (RAI-MDS)).

The mean age of the residents with YOD in care homes was 60 years and 53% of them were men. There is a large variety of etiologic diagnoses underlying the dementia. Dementia severity was very mild to mild in 18%, moderate in 25%, and severe or very severe in 58% of the participants. The prevalence of NPS was high with 90% exhibiting one or more clinically relevant NPS. Comorbidity was present in more than three quarters of the participants, most frequently psychiatric disorders.

The institutionalized YOD population is heterogeneous. NPS occur in almost all institutionalized people with YOD, and frequency and severity of NPS are higher than in late onset dementia (LOD) and community-dwelling YOD patients. Care should be delivered in settings accommodating a mixed male and female population, with appropriate, meaningful activities for all individuals. Further research is needed on NPS in YOD, to enhance quality of life and work in specialized YOD-care.

Children of patients with young onset dementia (YOD) who are confronted with a parent who has a progressive disease, often assist in caregiving tasks, which may have a great impact on their lives. The objective of the present study is to explore the experiences of children living with a young parent with dementia with a specific focus on the children's needs.

Semi-structured interviews with 14 adolescent children between the ages of 15 and 27 years of patients with YOD were analyzed using inductive content analysis. Themes were identified based on the established codes.

The emerging categories were divided into three themes that demonstrated the impact of dementia on daily life, different ways of coping with the disease, and children's need for care and support. The children had difficulties managing all of the responsibilities and showed concerns about their future. To deal with these problems, they demonstrated various coping styles, such as avoidant or adaptive coping. Although most children were initially reluctant to seek professional care, several of them expressed the need for practical guidance to address the changing behavior of their parent. The children felt more comfortable talking to someone who was familiar with their situation and who had specific knowledge of YOD and the available services.

In addition to practical information, more accessible and specific information about the diagnosis and the course of YOD is needed to provide a better understanding of the disease for the children. These findings underline the need for a personal, family-centered approach.

The extent to which specific factors influence diagnostic delays in dementia is unclear. Therefore, the aim of the present study was to compare duration from symptom onset to diagnosis for young-onset dementia (YOD) and late-onset dementia (LOD) and to assess the effect of age at onset, type of dementia, gender, living situation, education and family history of dementia on this duration.

Data on 235 YOD and 167 LOD patients collected from caregivers from two prospective cohort studies were used. Multiple linear regression analysis was performed.

The duration between symptom onset and the diagnosis of YOD exceeded that of LOD by an average of 1.6 years (2.8 v. 4.4 years). Young age and being diagnosed with frontotemporal dementia were related to increases in the time to diagnosis. Subjects with vascular dementia experienced shorter time to diagnosis.

There is a need to raise special awareness of YOD to facilitate a timely diagnosis.