Real World Financial Mismanagement in Alzheimer's Disease, Frontotemporal Dementia, and Primary Progressive Aphasia.

Background: Whereas clinical experience in dementia indicates high risk for financial mismanagement, there has been little formal study of real world financial errors in dementia. Objective: We aimed to compare caregiver-reported financial mistakes among people with Alzheimer's disease, behavioral variant frontotemporal dementia (bvFTD), and primary progressive aphasia (PPA). Methods: Caregivers reported whether participants with dementia had made financial mistakes within the last year; and if so, categorized these as resulting from: (a) being too trusting or gullible, (b) being wasteful or careless with money, or (c) trouble with memory. In a pre-registered analysis https://archive.org/details/osf-registrations-vupj7-v1), we examined the hypotheses that (1) financial mistakes due to impaired socioemotional function and diminished sensitivity to negative outcomes are more prevalent in bvFTD than in Alzheimer's disease, and (2) financial mistakes due to memory are more prevalent in Alzheimer's disease than in bvFTD. Exploratory analyses addressed vulnerability in PPA and brain-behavior relationships using voxel-based morphometry. Results: Concordant with our first hypothesis, bvFTD was more strongly associated than Alzheimer's disease with mistakes due to being too trusting/gullible or wasteful/careless; contrary to our second hypothesis, both groups were similarly likely to make mistakes due to memory. No differences were found between Alzheimer's disease and PPA. Exploratory analyses indicated associations between financial errors and atrophy in right prefrontal and insular cortex. Conclusions: Our findings cohere with documented socioemotional and valuation impairments in bvFTD, and with research indicating comparable memory impairment between bvFTD and Alzheimer's disease.

Gaps in clinical research in frontotemporal dementia: A call for diversity and disparities-focused research.

Frontotemporal dementia (FTD) is one of the leading causes of dementia before age 65 and often manifests as abnormal behavior (in behavioral variant FTD) or language impairment (in primary progressive aphasia). FTD's exact clinical presentation varies by culture, language, education, social norms, and other socioeconomic factors; current research and clinical practice, however, is mainly based on studies conducted in North America and Western Europe. Changes in diagnostic criteria and procedures as well as new or adapted cognitive tests are likely needed to take into consideration global diversity. This perspective paper by two professional interest areas of the Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment examines how increasing global diversity impacts the clinical presentation, screening, assessment, and diagnosis of FTD and its treatment and care. It subsequently provides recommendations to address immediate needs to advance global FTD research and clinical practice.

Neuropsychological Assessment in the Distinction Between Biomarker Defined Frontal-Variant of Alzheimer's Disease and Behavioral-Variant of Frontotemporal Dementia.

BACKGROUND: Frontal-variant of Alzheimer's disease (fvAD) was purposed for patients with AD pathology that, despite the typical amnestic presentation, show early and progressive deterioration of behavior and executive functions, closely resembling the behavioral-variant of frontotemporal dementia (bvFTD). This leads to a challenging differential diagnosis where neuropsychological evaluation and in vivo pathological evidence are essential. OBJECTIVE: To evaluate the contribution of a comprehensive neuropsychological assessment (NP) battery in distinguishing between fvAD-dementia and bvFTD supported by cerebrospinal fluid (CSF) biomarkers. METHODS: We included 40 patients with a baseline NP profile with prominent early executive and/or behavioral dysfunction, who meet both diagnosis of bvFTD and fvAD-dementia, according to international criteria. All patients underwent comprehensive NP assessment and CSF-AD biomarker evaluation. Neuropsychological domains as well as clinical and sociodemographic features, and APOE genotype were compared between groups. RESULTS: 21 patients (52.5%) met the biological criteria for AD (decreased Aß42 together with increased T-tau or P-tau in CSF) and were therefore classified as fvAD (mean age was 64.57, with 47.6% female). There were no differences between groups regarding age/age-at-onset, gender, or educational level. Regarding neuropsychological profile, performances in language and memory functions were equivalent in both groups. Significant differences were found in visuo-constructional abilities (p = 0.004), Trail Making Test A (p < 0.001), and Raven's Colored Progressive Matrices (p = 0.019), with fvAD patients showing worst performances. CONCLUSION: In patients with an early prominent frontal profile, a higher impairment in attention and visuo-spatial functions, signaling additional right hemisphere fronto-parietal dysfunction, point towards a diagnosis of fvAD-dementia and may be useful in clinical practice.

Current Potential for Clinical Optimization of Social Cognition Assessment for Frontotemporal Dementia and Primary Psychiatric Disorders.

Dodich and colleagues recently reviewed the evidence supporting clinical use of social cognition assessment in behavioral variant frontotemporal dementia (Dodich et al., 2021). Here, we comment on their methods and present an initiative to address some of the limitations that emerged from their study. In particular, we established the social cognition workgroup within the Neuropsychiatric International Consortium Frontotemporal dementia (scNIC-FTD), aiming to validate social cognition assessment for diagnostic purposes and tracking of change across clinical situations.

Patient affect and caregiver burden in dementia.

BACKGROUND: Numerous studies focusing on the burden of caregivers of dementia patients have been published. However, there have been few studies focusing on positive affect as an important factor affecting the caregiver burden, and only a few studies comparing the caregiver burden between different dementia diseases have been reported. METHODS: Three hundred and thirty-seven consecutive caregivers of people with dementia participated in this study. The caregiver burden was evaluated by the short version of the Japanese version of the Zarit Burden Interview. RESULTS: Positive affect scores had a significant relationship with the scores of the short version of the Zarit Burden Interview. Caregivers for patients with dementia with Lewy bodies or frontotemporal dementia suffered from a greater burden than those for patients with Alzheimer's disease dementia. CONCLUSIONS: The caregiver burden differed between people caring for patients with different dementia diseases. Positive affect of dementia patients has a significant relationship with caregiver burden, independently from neuropsychiatric symptoms of patients.

Von Economo Neurons in Behavioral Variant Frontotemporal Dementia with Underlying Alzheimer's Disease.

The selective loss of von Economo neurons has been linked to the behavioral deficits in behavioral variant frontotemporal dementia (bvFTD) but whether these neurons are affected in bvFTD patients with underlying Alzheimer's disease (AD) has yet to be established. The present study assesses the von Economo neurons in pathological AD cases clinically diagnosed with either AD or bvFTD. Our results demonstrate no significant loss of von Economo neurons in all pathological AD cases, irrespective of clinical diagnosis or co-existing Lewy body pathology. These results suggest that the behavioral deficits in patients with clinical bvFTD and underlying pathological AD are not driven by the loss of von Economo neurons.

Financial capacity in frontotemporal dementia and related presentations.

BACKGROUND: Changes in financial judgement and skills can herald a neurodegenerative dementia and are a common reason for referral for cognitive neurologic assessment. However, patients with neurodegenerative diseases affecting the frontal or temporal lobes may perform well on standard cognitive tests, complicating clinical determinations about their diagnosis and financial capacity. METHODS: Forty-five patients with possible or probable FTD or Alzheimer's disease and 22 healthy controls completed two financial assessment batteries, the FACT and the FCAI. Patients' performance was compared to study partner estimates of patients' financial abilities. RESULTS: All three patient groups performed worse than controls on both the FACT and the FCAI. Study partners over-estimated the performance of patients with Alzheimer's disease. CONCLUSIONS: These initial findings suggest that accurate clinical assessment of financial skills and judgement in patients with possible neurodegenerative dementias requires performance-based assessment.

Association of Neuropathologically Confirmed Frontotemporal Dementia and Alzheimer Disease With Criminal and Socially Inappropriate Behavior in a Swedish Cohort.

Importance: Criminal and socially inappropriate behavior is encountered among patients with dementia, and it is sometimes the first sign of a dementing disorder. This behavior constitutes a significant burden to society, patients' relatives, and patients themselves. Objectives: To investigate and compare the prevalence and type of criminal and socially inappropriate behavior, as well as recurrence of criminal behavior, associated with Alzheimer disease (AD) and frontotemporal dementia (FTD) neuropathologically verified post mortem, and to assess whether there is a specific type of protein pathology more closely associated with criminal behavior in patients with FTD. Design, Setting, and Participants: Cohort study using medical record review of 220 Swedish patients with a postmortem neuropathologic diagnosis of AD (n = 101) or frontotemporal lobar degeneration (n = 119) (hereinafter referred to as FTD) diagnosed between January 1, 1967, and December 31, 2017. Main Outcomes and Measures: Patient notes containing reports of criminal and socially inappropriate behavior, as well as data on dominant protein pathology for patients with FTD, were duly reviewed and recorded. The Fisher exact test or logistic regression was used to assess possible differences between groups. Results: Of the 220 patients studied, 128 (58.2%) were female, the median (range) age at disease onset was 63 (30-88) years and at death was 72 (34-96) years, and the median (range) disease duration was 9 (1-28) years. Instances of criminal behavior were found in 65 of the 220 patients (29.5%): in 15 of the 101 patients (14.9%) with AD and 50 of the 119 patients (42.0%) with FTD (P < .001). Recurrence of criminal behavior was significantly higher in the FTD group (89.0%) than in the AD group (53.3%) (P = .04). Instances of socially inappropriate behavior were found in 57 patients (56.4%) with AD and 89 (74.8%) with FTD (P = .004). An expression of non-tau pathology increased the odds for criminal behavior by a factor of 9.0 (95% CI, 3.4-24.0) among patients with FTD. Conclusions and Relevance: These results suggest that criminal and socially inappropriate behaviors may be more prevalent and criminal behaviors may be more recurrent in patients with FTD than in those with AD. Non-tau pathology, but not tau pathology, appears to be associated with criminal behavior. These findings may help with the clinical diagnostic process.

Predictors of finance management in dementia: managing bills and taxes matters.

ABSTRACTObjectives:Finance management skills deteriorate early on in dementia, and can influence the ability to maintain control over personal affairs. The aim of this study was to assess the contributions of different types of cognition and motor functioning to finance management. DESIGN: Cross-sectional analysis using secondary data. SETTING: Community living. PARTICIPANTS: Baseline data from the Uniform Data Set from the National Alzheimer's Coordinating Centers were obtained and extracted up until December 2016. MEASUREMENTS: Measures on everyday functioning (Functional Assessment Questionnaire) and cognition (memory, executive functioning, and language), the Clinical Dementia Rating scale, and questions on Parkinsonian motor symptoms (gait disturbance, falls, tremors, and slowness) were included. Data were analyzed using bivariate correlation and linear regression analyses. RESULTS: A total of 9,383 participants were included in the analysis (Alzheimers disease (AD) = 8,201; behavioral variant fronto-temporal dementia (bvFTD) = 796; Dementia with Lewy Bodies (DLB) = 386). Cognition and motor functioning varied significantly across AD, bvFTD, and DLB, with poorer motor functioning and poorer finance management skills in DLB than in AD and bvFTD. In the regression models, slowness, verbal fluency, executive functioning, and language, followed by age, gender, and diagnosis accounted for 13.8% of the variation in managing bills, and for 11.4% of the variation in managing taxes. CONCLUSION: Maintaining finance management abilities for as long as possible is important for people with dementia, to avoid potential financial exploitation. Findings from this study highlight avenues to pursue to delay deterioration in managing bills and taxes, and help maintain financial control.

Quality of Life in People with Young-Onset Dementia: A Nordic Two-Year Observational Multicenter Study.

BACKGROUND: Cross-sectional studies of quality of life (QOL) of people with young-onset dementia show diverging results. OBJECTIVE: To identify factors associated with QOL in people with young-onset Alzheimer's (AD) and frontotemporal dementia (FTD) and explore development in QOL over a two-year period, including differences between the two subtypes. METHODS: A two-year cohort study of 88 community-dwelling people with young-onset AD and FTD recruited from Nordic memory clinics. QOL was assessed using the proxy version of the Quality of Life - Alzheimer's Disease questionnaire, dementia severity was rated with the Clinical Dementia Rating scale, depressive symptoms by the Cornell Scale for Depression in Dementia, awareness with the Reed anosognosia scale, and needs using the Camberwell Assessment of Needs in the Elderly questionnaire. Factors associated with QOL and development in QOL over time were explored with growth mixture model trajectories and mixed model analyses. RESULTS: We identified two groups of people following trajectories with better (n = 35) versus poorer (n = 53) QOL. People with more depressive symptoms at baseline had higher odds of belonging to poorer QOL group, OR 1.2 (CI 1.1; 1.5, p = 0.011). Having Alzheimer's disease was associated with significantly better QOL (p = 0.047 at baseline, p = 0.009 at T1 and p = 0.033 at T2). Increasing number of unmet needs was significantly associated with poorer QOL at baseline (p = 0.007), but not later in follow-up. CONCLUSION: Early assessment and treatment based on dementia subtype, depression, and individual needs may enhance quality of life in young-onset dementia.

Adaptive crossover designs for assessment of symptomatic treatments targeting behaviour in neurodegenerative disease: a phase 2 clinical trial of intranasal oxytocin for frontotemporal dementia (FOXY).

BACKGROUND: There are currently no treatments for empathy deficits in neuropsychiatric disorders. Acute administration of the hormone oxytocin has been associated with symptomatic improvements across animal models and several neuropsychiatric disorders, but results of the majority of oxytocin randomised controlled trials (RCTs) of longer duration have been negative or inconclusive. This lack of efficacy of may be due to rapid habituation to oxytocin with chronic dosing. The objective of the present study is to describe the design of a phase 2 adaptive randomised controlled crossover trial of intranasal oxytocin in frontotemporal dementia (FOXY) as an efficient model for future investigations of symptomatic treatments in neuropsychiatric and neurodegenerative disorders. METHODS: Stage 1 will identify which of three dose schedules is most promising based on change in the primary outcome measure, the Neuropsychiatric Inventory apathy/indifference domain score, over 6 weeks of treatment. In stage 2, additional patients are enrolled at the most promising dose for preliminary efficacy analysis when combined with stage 1 to determine if a phase 3 trial is warranted. Objective measures include facial expression recognition, cerebrospinal fluid (CSF) oxytocin levels, and behavioural ratings of videotaped interactions. RESULTS: A total of 20 patients per arm will be entered into stage 1 for a total of 60 patients. In stage 2, an additional 40 patients will be enrolled in the most promising dose arm. CONCLUSIONS: The use of adaptive, crossover designs and inclusion of objective measures of change in CSF oxytocin levels and social behaviour will improve the efficiency and conclusiveness of RCTs of oxytocin and other symptomatic treatments in neuropsychiatric disorders. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03260920 . Registered on August 24, 2017.

Assessment of Patient Self-awareness and Related Neural Correlates in Frontotemporal Dementia and Corticobasal Syndrome.

OBJECTIVE: We compared two different methods of assessing self-awareness (clinician-rated vs. self- and caregiver report) in participants with neurodegenerative conditions. Additionally, we examined the contribution of memory dysfunction to assessment of self-awareness. METHOD: Sixty-seven participants with various neurodegenerative disorders participated in this study. Data were collected on brain volume, neurocognitive function, demographic characteristics, and two measures of patient self-awareness, defined as (1) the discrepancy between patient and caregiver ratings of dysexecutive syndrome and (2) clinician-observed rating of patient insight. Penalized regression with best subset variable selection and 10-fold cross-validation was used to evaluate three neurocognitive frameworks: self-regulation, language, and perspective-taking, each predicting the results from the two methods of self-awareness measurement. RESULTS: The self-regulation framework was more robustly predictive for both the clinician rating and discrepancy method than language or perspective-taking. Frameworks in which the clinician rating was the criterion were more robust than those with the discrepancy method as criterion. When a measure of memory functioning was added to the framework, there was no appreciable improvement in the prediction of self-awareness. CONCLUSIONS: A self-regulation neurocognitive framework, consisting of regions of interest and neuropsychological test scores, was more effective in understanding patient self-awareness than perspective-taking or language frameworks. Compared to the discrepancy method, a clinician rating of self-awareness was more robustly associated with relevant clinical variables of regional brain volume and neuropsychological performance, suggesting it may be a useful measure to aid clinical diagnosis.

Greater Experience of Negative Non-Target Emotions by Patients with Neurodegenerative Diseases Is Related to Lower Emotional Well-Being in Caregivers.

BACKGROUND: Behavioral symptoms in patients with neurodegenerative diseases can be particularly challenging for caregivers. Previously, we reported that patients with frontotemporal dementia (FTD) and Alzheimer's disease (AD) experienced emotions that were atypical or incongruent with a given situation (i.e., non-target emotions). AIM: We tested the hypothesis that greater experience of non-target emotions by patients is associated with lower caregiver emotional well-being. METHODS: 178 patients with FTD, AD, or other neurodegenerative diseases and 35 healthy individuals watched 3 films designed to induce amusement, sadness, and disgust, and then reported their emotions during the films. Caregivers of the patients reported their own emotional well-being on the Medical Outcomes Study 36-item Short-Form Health Survey. RESULTS: In response to the amusement and sadness (but not disgust) films, greater experience of non-target emotions by patients was related to lower caregiver emotional well-being. These effects were specific to patients' experience of negative non-target emotions (i.e., not found for positive non-target emotions or for negative or positive target emotions). CONCLUSION: The findings reveal a previously unstudied patient behavior that is related to worse caregiver emotional well-being. Future research and clinical assessment may benefit from evaluating non-target emotions in patients.

Can cognitive assessment really discriminate early stages of Alzheimer's and behavioural variant frontotemporal dementia at initial clinical presentation?

BACKGROUND: Neuropsychological testing is considered crucial for differential diagnosis of Alzheimer's disease (AD) and behavioural variant frontotemporal dementia (bvFTD). In-depth neuropsychological assessment revealed specific dysfunctions in the two dementia syndromes. However, a significant overlap of cognitive impairments exists in early disease stages. We questioned whether a standard neuropsychological assessment at initial clinical presentation can delineate patients with AD versus bvFTD. METHODS: In a retrospective approach, we evaluated and compared how cognitive profiles assessed at initial clinical presentation predicted the diagnosis of later verified AD (n = 43) and bvFTD (n = 26). Additionally, the neuropsychological standard domains memory, language, visuospatial skills, executive functions, praxis and social cognition were subjected to stepwise discriminant analysis to compare their differential contribution to diagnosis. RESULTS: Regardless of diagnosis, a percentage of patients presented with major deterioration in a wide range of cognitive domains when compared with age-matched normative data. Only few significant differences were detected on the group level: Patients with AD were relatively more impaired in the verbal recall, verbal recognition, figure copy, and surprisingly in the executive subdomains, set shifting and processing speed whereas bvFTD was characterised by more deficits in imitation of face postures. A combination of tests for verbal recall, imitation of limb and face postures, and figure copy showed the greatest discriminatory power. CONCLUSIONS: Our results imply that the contribution of a standard neuropsychological assessment is limited for differential diagnosis of AD and bvFTD at initial presentation. In contrast to current clinical guidelines, executive functions are neither particularly nor exclusively impaired in patients with bvFTD when assessed within a standard clinical neuropsychological test battery. The significant overlap of bvFTD and AD concerning the profile of cognitive impairments questions current neuropsychological diagnostic criteria and calls for revision, regarding both the degree and the profile of cognitive deficits.

Qualitative Assessment of Verbal Fluency Performance in Frontotemporal Dementia.

BACKGROUND/AIMS: Verbal fluency is impaired in patients with frontotemporal dementia (FTD) and primary progressive aphasia (PPA). This study explored qualitative differences in verbal fluency (clustering of words, switching between strategies) between FTD and PPA variants. METHODS: Twenty-nine patients with behavioral variant FTD (bvFTD) and 50 with PPA (13 nonfluent/agrammatic, 14 semantic, and 23 logopenic) performed a semantic and letter fluency task. Clustering (number of multiword strings) and switching (number of transitions between clustered and nonclustered words) were recorded by two independent raters. Between-group differences, associations with memory, language, and executive functioning, and longitudinal change (subsample) in clustering and switching were examined. RESULTS: Interrater reliability was high (median 0.98). PPA patients generated (a) smaller (number of) clusters on semantic and letter fluency than bvFTD patients (p < 0.05). Semantic variant patients used more switches than nonfluent/agrammatic or logopenic variant patients (p < 0.05). Clustering in semantic fluency was significantly associated with memory and language (range standardized regression coefficients 0.24-0.38). Switching in letter fluency was associated with executive functioning (0.32-0.35). CONCLUSION: Clustering and switching in verbal fluency differed between patients with subtypes of FTD and PPA. Qualitative aspects of verbal fluency provide additional information on verbal ability and executive control which can be used for clinically diagnostic purposes.

Association between Montreal Cognitive Assessment Sub-Item Scores and Corresponding Cognitive Test Performance in Patients with Frontotemporal Dementia and Related Disorders.

The Montreal Cognitive Assessment (MoCA), a brief screening test developed to detect patients with mild cognitive impairment, is used in clinical settings across North America [Nasreddine et al.: J Am Geriatr Soc 2005;53:695-699]. The MoCA has been demonstrated to be sensitive to cognitive deficits in frontotemporal dementias (FTD) and related disorders [Coleman et al.: Alzheimer Dis Assoc Disord 2016;30:258-263]. Given attentional impairments in patients with FTD, whether and to what extent the abbreviated items on the MoCA may predict performance on corresponding assessments is not known. Testing and demographic data were extracted from a clinical database using a sample of 91 patients with FTD and related disorders. The relationship between MoCA items and corresponding neuropsychological tasks was assessed through McNemar tests and Spearman correlations. While some MoCA items such as letter fluency, orientation, and clock drawing were strongly correlated with the corresponding standard cognitive test, the MoCA trails were insensitive to impairment compared to the full Trail Making B Test (p = 0.01). In contrast, MoCA naming and delayed recall sub-items detected cognitive impairment more frequently than available comparison tests. The MoCA is a sensitive screening measure to detect impairment in patients with FTD and related disorders, but cognitive deficits specific to FTD result in differential performance on MoCA items compared to longer standard cognitive tests.

Factors Underpinning Caregiver Burden in Frontotemporal Dementia Differ in Spouses and their Children.

The objectives of this observational study were to (1) compare spousal and child caregiver burden; (2) compare co-resident and live-out child caregiver burden; and (3) investigate factors influencing spousal and child caregiver burden. Data was collected from 90 caregivers of people with frontotemporal degeneration (FTD) recruited from the Frontotemporal Dementia Research Group (Frontier) at Neuroscience Research, Australia. Of this caregiver group, 43 were spousal caregivers and 47 were child caregivers. Caregiver burden and emotional state were evaluated using the short Zarit Burden Interview and the short version of the Depression, Anxiety and Stress Scale-21. The Social Network Index was applied to ascertain the social network of the caregiver, while the Intimate Bond Measure was used to evaluate the current quality of the relationship between the caregiver and the person with dementia. The Frontotemporal Dementia Rating Scale was used to assess severity of dementia. Spousal and child caregivers experienced similar levels of burden, depression, anxiety, and stress, regardless of disease severity. Co-resident child caregivers had smaller social networks and greater burden than live-out caregivers. Dementia severity was key in spousal caregiver burden, whereas caregiver depression was most important in child caregiver burden. Child and spousal caregivers of individuals with FTD share similar levels of burden, influenced by different factors. Future interventions need to account for these differences.

Assessment of free and cued recall in Alzheimer's disease and vascular and frontotemporal dementia with 24-item Grober and Buschke test.

Alzheimer's disease (AD), vascular dementia (VaD) and frontotemporal dementia (FTD) are the most common forms of dementia. It is well known that memory deficits in AD are different from those in VaD and FTD, especially with respect to cued recall. The aim of this clinical study was to compare the memory performance in 15 AD, 10 VaD and 9 FTD patients and 20 normal controls by means of a 24-item Grober-Buschke test [8]. The patients' groups were comparable in terms of severity of dementia. We considered free and total recall (free plus cued) both in immediate and delayed recall and computed an Index of Sensitivity to Cueing (ISC) [8] for immediate and delayed trials. We assessed whether cued recall predicted the subsequent free recall across our patients' groups. We found that AD patients recalled fewer items from the beginning and were less sensitive to cueing supporting the hypothesis that memory disorders in AD depend on encoding and storage deficit. In immediate recall VaD and FTD showed a similar memory performance and a stronger sensitivity to cueing than AD, suggesting that memory disorders in these patients are due to a difficulty in spontaneously implementing efficient retrieval strategies. However, we found a lower ISC in the delayed recall compared to the immediate trials in VaD than FTD due to a higher forgetting in VaD.

Determinants of quality of life in young onset dementia - results from a European multicenter assessment.

BACKGROUND: Promoting adaptation, improving well-being and maintaining an optimal quality of life (QOL) is an important aspect in dementia care. The purpose of this study was to identify determinants of QOL in young onset dementia, and to assess differences in QoL domains between people with Alzheimer's disease (AD) and frontotemporal dementia (FTD). METHODS: In total 135 persons with AD and 58 persons with FTD were included from two prospective cohort studies. QOL was assessed with the proxy reported quality of life in Alzheimer's disease questionnaire (QoL-AD). Possible determinants were explored using multiple linear regression and included sociodemographic variables, diagnosis, dementia severity, disease awareness, neuropsychiatric symptoms, met and unmet needs and hours of personal and instrumental care. Differences between QOL domains in people with AD and FTD were calculated using Mann-Whitney U tests. RESULTS: Lower QOL was associated with more depressive symptoms, lower disease awareness, and a higher amount of needs, both met and unmet. People with AD scored lower on the memory and higher on the friends' subscale. No differences were found for the other items. CONCLUSION: This study demonstrates a unique set of determinants of QOL in AD and FTD. Interventions directed towards these specific factors may improve QOL.

RHAPSODY - Internet-based support for caregivers of people with young onset dementia: program design and methods of a pilot study.

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.