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The disease trajectory and healthcare requirements of patients with young-onset dementia (YOD) differ from those of older patients. Accurate data about YOD is crucial to improve diagnosis and optimize care. PRECODE-GP aims to set up a prospective national database of patients with YOD to gain insight into the occurrence and characteristics of patients with YOD in memory clinics in the Netherlands. The national database includes data from dementia patients aged <70 years at diagnosis, collected by local memory clinics (MCs). Data included demographic information, clinical variables, and (etiological) diagnoses. Between July 2019 and December 2022, 781 patients with a mean age of 62±6y at diagnosis (range 37 to 69y) were included from 39 MCs. Most (n = 547,70%) were diagnosed with dementia due to Alzheimer's disease (AD). Patients with Frontotemporal lobe dementia (FTD, n = 87, 11%) were youngest (61±6.0y). Over half (55%) of patients were experiencing symptoms for ≥2 years. We initiated a Dutch national YOD database to improve diagnosis and care for this underrepresented and vulnerable patient group. The database provides a basis for future in-depth studies on YOD.

Introduction: Reliable data on the incidence rates for young-onset dementia (YOD) are lacking, but are necessary for research on disease etiology and to raise awareness among health care professionals. Methods: We performed a systematic review and meta-analysis on population-based studies on the incidence of YOD, published between January 1, 1990 and February 1, 2022, according to Meta-analyses of Observational Studies in Epidemiology (MOOSE) guidelines. Data were analyzed using random-effects meta-analyses. Results were age-standardized, and heterogeneity was assessed by subgroup analyses and meta-regression. Results: Sixty-one articles were included. Global age-standardized incidence rates increased from 0.17/100,000 in age 30 to 34 years, to 5.14/100,000 in age 60 to 64 years, giving a global total age-standardized incidence rate of 11 per 100,000 in age 30 to 64. This corresponds to 370,000 new YOD cases annually worldwide. Heterogeneity was high and meta-regression showed geographic location significantly influenced this heterogeneity. Discussion: This meta-analysis shows the current best estimate of YOD incidence. New prospective cohort studies are needed.

Background: Timely diagnosis and adequate care is important for persons with young-onset dementia (YOD) and their caregivers, due to the high impact of the disease. Initiating care can be difficult for the general practitioner (GP) and other healthcare professionals. Objective: Provide insight in the care use of persons with YOD and identify factors influencing care use. Methods: A primary care register was used for this study. Information on the care use of persons with YOD was extracted from the GPs written notes. Information entailed time until start of care use, reasons and factors influencing the GP's decision, and reasons and factors influencing actual care use were included. Analyses included quantitative explorative descriptive analyses, and qualitative manifest content analyses. Results: 75 persons with YOD were included in this study. The main reason for GPs to refer for diagnosis was concerns of caregivers. After diagnosis, 72% of the persons were assigned a case manager, 42.7% received day care, and 44% were admitted to a long-Term care facility. A higher percentage of persons without a case manager was admitted to a long-Term care facility (64%) compared to the persons with a case manager (36%). Reasons for not initiating care were reluctancy of the persons with YOD or their caregivers, the person deceased, or because the GP did not refer for care. Conclusion: Care use differed between persons due to different needs and reasons. Although most persons with YOD receive care in the years after diagnosis, there are still factors that could be improved.

Background: Young-onset dementia (YOD) has many underlying etiologies, leading to a large heterogeneity in first symptoms. This makes it difficult for general practitioners (GPs) to recognize YOD. Objective: Identify early symptoms that are more common in the pre-diagnostic phase of YOD. Methods: We performed a case-control study nested in a primary-care registry on 89 cases and 162 matched controls, where we compared symptoms of people with YOD up to 5 years before diagnosis to their matched control group without YOD. The variables included in this study were International Classification of Primary Care codes and symptoms extracted from written GP notes and categorized in groups. We used Generalized Equation Estimation to analyze symptom's time-trajectories and logistic regression and ROC-curves to analyze differences in number of symptom categories reported. Results: Cognitive symptoms were more common in people with YOD 5 years before diagnosis, affective symptoms 4 years before diagnosis, social symptoms 3 years, behavioral symptoms 2 years, and daily functioning disturbances 1 year before diagnosis. The ROC-curve suggested that reporting two or more symptom categories at the GP gave the best trade-off between sensitivity (85%) and specificity (77%), for the highest percentage of correctly diagnosed persons. Conclusion: This study showed people with YOD present differently than people without YOD. However, it may still be difficult for GPs to use these symptom categories to distinguish people with YOD, since the symptoms also occur in people with other diseases. A combination of reported symptom categories increases the probability of an underlying cause of YOD.

Objectives: Dementia at a young age differs from late onset dementia in pathology and care needs. This requires further research to improve the understanding of this group, support and service provision. Aim of current study is to reach consensus on the terminology and operational definition (i.e., age-related criteria and possible causes) of dementia at a young age, to aid further research. Methods: A classical Delphi technique was used to transform opinions into group consensus by using an online survey. In three rounds statements regarding (1) terminology, (2) age-related criteria, and (3) aetiologies that can be considered as causes of dementia at a young age were sent to international experts in the field to give their opinions and additional comments on the statements. Results: Forty-four experts responded and full consensus was reached on 22 out of 35 statements. Young-onset dementia emerged as the term of preference. Provisional consensus was found for the use of age 65 at symptom onset as preferred cut-off age. Consensus was reached on the inclusion of 15 out of 22 aetiologies and categories of aetiologies as potential cause for dementia at a young age. Conclusions: A clear term and operational definition have been reached. Although beneficial for conducting future research to gain more insight in pathology and care needs of young people living with dementia, still consensus about some details is lacking. To reach consensus about these details and implications for use in research and clinical practice, the organisation of an in person consensus meeting is advised.

Objectives: 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. Design, Setting and Participants: 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). Measures: 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. Results: 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.

Background: Neuropsychiatric symptoms are common in nursing home residents with young-onset dementia and burdensome for nursing staff. It is known that neuropsychiatric symptoms are associated with burn-out complaints and low job satisfaction. An intervention aimed at decreasing neuropsychiatric symptoms in nursing home residents with young-onset dementia might also result in less burnout complaints and job demands and improve job satisfaction in nursing staff.Objectives: The aim was to evaluate the effect of the intervention on nursing staff burnout, job satisfaction and job demands.Design: Cluster randomised controlled trial using a stepped wedge design with a total duration of 18 months, with four assessments.Setting: Thirteen young-onset dementia special care units across the Netherlands were, by means of random allocation software, assigned to three groups crossing over at different time points.Participants: All nursing staff, in total 391, were invited to participate. 305 nursing staff participated during the course of the study of whom 71 participated in all assessments.Intervention: An educational program followed by a structured multidisciplinary care program aimed at the management of neuropsychiatric symptoms. The care program consists of evaluation of psychotropic drug prescription followed by detection, analysis, treatment and evaluation of treatment of neuropsychiatric symptoms.Methods: Emotional exhaustion, depersonalisation and personal accomplishment were assessed with the Utrecht Burnout Scale. Job satisfaction and job demands were assessed with subscales of the Leiden Quality of Work Questionnaire.Results: The baseline burnout risk on emotional exhaustion and personal accomplishment was average, and low on depersonalisation. The mean scores for job satisfaction were above average and for job demands average. Linear mixed models showed that the intervention had no effect on emotional exhaustion (e

Objective: The effect of an intervention on neuropsychiatric symptoms (NPS), particularly agitation and aggression, and psychotropic drug use (PDU) in institutionalized people with young-onset dementia (YOD) was evaluated. Methods: A randomized controlled trial was conducted using a stepped wedge design. Thirteen YOD special care units were randomly assigned to three groups, which received the intervention at different time points. Four assessments took place every 6 months during a period of 18 months. Two hundred seventy-four people with YOD who resided in YOD special care units participated, of whom 131 took part in all assessments. The intervention consisted of an educational program combined with a care program, which structured the multidisciplinary process of managing NPS. The care program included the following five steps: evaluation of psychotropic drug prescription, detection, analysis, treatment, and evaluation of treatment of NPS. The Cohen-Mansfield Agitation Inventory and the Neuropsychiatric Inventory-Nursing Home version were used to assess NPS. Data on PDU were retrieved from residents' medical files. Multilevel models were used to evaluate the effect of the intervention, which accounted for clustering of measurements in clients within units. Results: No significant differences were found in agitation, aggression, other NPS, or PDU after crossing over to the intervention condition. Conclusion: We found no evidence that the intervention for management of NPS in nursing home residents with YOD was more effective in reducing agitation, aggression, other NPS, or PDU compared with care as usual.

Objective: The aims of this study were (1) to investigate the relationship between different neuropsychiatric symptoms (NPS) and the level of distress experienced by nurses caring for residents with young-onset dementia (YOD) and (2) to compare these findings with those for nurses caring for residents with late-onset dementia (LOD). Design/Setting: This is a retrospective study conducted in Dutch long-term care facilities. Data were used from the Behavior and Evolution of Young-ONset Dementia studies (BEYOND) Parts I and II and the WAAL Behavior in Dementia-II (Waalbed-II) study. Participants: A total of 382 nursing home residents with YOD and 261 nursing home residents with LOD were included. Measurements: The Neuropsychiatric Inventory, nursing home version, was used to assess nursing staff distress and the frequency (F) and severity (S) of NPS. Multilevel logistic regression analysis was used to investigate the relationships between nursing staff distress related to NPS and YOD and LOD care units, the F x S score per symptom, gender, dementia subtype, and dementia severity. Results: Nurses working in YOD care units rated sleep and nighttime behavior disorders, delusions, and agitation/aggression most often as highly distressing and euphoria most often as not distressing. Multivariate analyses indicated that the frequency and severity of NPS were significantly associated with staff distress in all symptoms, except for apathy. Comparison of the 2 groups of nurses demonstrated that the odds for distress related to sleep and nighttime behavior disorders were higher for nurses in YOD care units than for nurses in LOD units. For both the YOD and LOD nurses, irritability in male residents had higher impact than similar behavior in female residents. Conclusion: This study provides important insight into distress related to individual NPS and the interaction with residents' characteristics. All NPS result in distress. The frequency and severity of the behavior is an impor

Objectives: A process evaluation was performed for an intervention aimed at improvement of the management of neuropsychiatric symptoms in young-onset dementia. Data about sample quality and intervention quality was evaluated to better understand internal and external validity. In addition, data about the implementation strategy and factors affecting implementation were evaluated to improve further implementation of the intervention. Design: A model proposed by Leontjevas and colleagues consisting of first-order (validity) and second-order (implementation) data was used. Setting and Participants: Care units delivering specialized treatment and support for residents with young-onset dementia. Measures: A description of the recruitment, randomization procedure, and intervention reach was carried out to determine sample quality. To determine intervention quality, data on satisfaction, relevance, feasibility, and fidelity were collected through a questionnaire and reports logged on the server of the web-based intervention. A description of the implementation strategy was provided. Barriers and facilitators for implementation were collected by a questionnaire and analyzed by deductive content analysis. Results: Care units varied in size and were recruited from different geographical regions in the Netherlands. The informed consent rate of the residents was 87.7%. The majority of the nursing home staff were satisfied with the intervention. However, parts of the intervention were perceived as less relevant for their own organization. The feasibility of the intervention was considered low. The fidelity differed between care units. The implementation strategy did not overcome all barriers. Factors affecting implementation covered 3 themes: organizational aspects, culture of the organization, and aspects of the intervention. Conclusions: In general, our results showed sufficient internal and external validity, warranting further effect analyses. Adaptations to specific steps o

Background: The progression of dementia in people with young-onset dementia (YOD) is relatively unknown. Objective: To investigate the progression of dementia and cognitive decline in the three most common subtypes in YOD and to explore which factors are associated with this course. Methods: The course of dementia was examined in 198 people with YOD. The primary outcomes were cognitive function, as assessed by the Mini-Mental State Examination (MMSE) and dementia severity, as assessed by the Global Deterioration Scale (GDS). Mixed-model analyses were used to explore factors associated with the course of dementia of the diagnostic sub-types. Results: The mean overall two-year progression of dementia severity was 0.9 GDS points, this was a statistically significant change (p = 0.012) and was not significant different for the three dementia subtypes. The mean overall two-year decline in cognitive function was 1.6 points on the MMSE. The differences in cognitive decline were statistically significant (p = 0.046) among the three diagnosis groups, AD participants showed the greatest decline, of 2.3 points. In addition to lower education (p = 0.010), higher scores on the Neuropsychiatric Inventory (NPI) sub-syndromes psychosis (p < 0.001) and hyperactivity (p = 0.002) were associated with higher rates of cognitive decline. In contrast, higher scores on the NPI affect cluster were associated with lower levels of cognitive decline (p < 0.001). Conclusion: Different YOD subtypes show different rates of decline in cognitive functioning, and this decline seems less progressive compared to those observed in studies in late-onset AD. Further research is needed to evaluate whether managing neuropsychiatric symptoms can positively influence the decline of cognitive function.

OBJECTIVES: To identify information available in six European countries (England, France, Germany, Netherlands, Portugal, Sweden) that addresses the specific needs of people with young onset dementia (YOD) and their carers, and identify gaps. METHODS: Search of websites of organisations with potential interest in dementia. Narrative synthesis and comparative analysis. RESULTS: 21 sources of information were identified (Netherlands 6, England 6, France 3, Germany 2, Portugal 2, Sweden 2); 11 were from voluntary sector organisations. Sources dedicated to YOD were limited (4 websites, 4 books); all other YOD information was sub-entries in generic dementia sources, difficult to locate and with limited coverage of relevant topics. Gaps related to implications of living with YOD in Germany, Portugal and Sweden. CONCLUSION: Availability of information varies among countries, some having no dedicated source and incomplete coverage of issues of importance to YOD. PRACTICAL IMPLICATIONS: Information is an important means of supporting carers; their needs change as the condition progresses. A comprehensive resource collating key information is needed so that the issues that differentiate the specific needs of people living with YOD from those of people with dementia in older age are available and easily located., RHAPSODY research and strategy for dementia in the young

Background: To explore the aspects of daily life that give people with young-onset dementia (YOD) a sense of usefulness.Methods: Eighteen people with YOD and 21 informal caregivers participated in this qualitative study. Participants were recruited from specialized day-care centers for people with YOD in the Netherlands. Four focus groups were conducted with people with YOD, and four with informal caregivers. Focus groups were audio-recorded, transcribed, and analyzed using inductive content analysis.Results: Four themes emerged from the analysis: (1) staying engaged, (2) loss in daily life, (3) coping and adaptation, and (4) external support. Staying engaged in activities that provide a sense of usefulness or participating in leisure and recreational activities as much as possible in daily life emerged as the key theme. Retaining a sense of usefulness was considered both important and possible by having social roles or participating in functional activities. The importance of activities providing a sense of usefulness seemed to decrease over time, while the need for pleasant activities seemed to increase. Experienced loss, coping, adaptation, and available external support are important parts of the context in which the person with YOD tries to engage in daily life as much as possible. Active coping styles and external support appear to play a facilitating role in staying engaged.Conclusions: t is important for people with YOD to have the opportunity to feel useful; especially in the early stages of the condition. Caregivers should be educated in ways to enhance a sense of usefulness and engagement in daily life for people with YOD.

Background: Young Onset Dementia (YOD), defined by first symptoms of cognitive or behavioral decline occurring before the age of 65 years, is relatively rare compared to dementia of later onset, but it is associated with diagnostic difficulty and heavy burden on affected individuals and their informal carers. Existing health and social care structures rarely meet the needs of YOD patients. Internet-based interventions are a novel format of delivering health-related education, counseling, and support to this vulnerable yet underserved group. Methods: The RHAPSODY (Research to Assess Policies and Strategies for Dementia in the Young) project is a European initiative to improve care for people with YOD by providing an internet-based information and skill-building program for family carers. The e-learning program focuses on managing problem behaviors, dealing with role change, obtaining support, and looking after oneself. It will be evaluated in a pilot study in three countries using a randomized unblinded design with a wait-list control group. Participants will be informal carers of people with dementia in Alzheimer's disease or behavioral-variant Frontotemporal degeneration with an onset before the age of 65 years. The primary outcome will be caregiving self-efficacy after 6 weeks of program use. As secondary outcomes, caregivers' stress and burden, carer health-related quality of life, caring-related knowledge, patient problem behaviors, and user satisfaction will be assessed. Program utilization will be monitored and a health-economic evaluation will also be performed. Conclusions: The RHAPSODY project will add to the evidence on the potential and limitations of a conveniently accessible, user-friendly, and comprehensive internet-based intervention as an alternative for traditional forms of counseling and support in healthcare, aiming to optimize care and support for people with YOD and their informal caregivers.