What is ‘early onset dementia’?

There are two types of dementia with early onset: (i) presenile dementias; and (ii) senile dementias with early onset. Most patients who develop dementia before 65 years of age have Alzheimer's disease (AD). The remainder are likely to have vascular dementia (VaD), frontotemporal dementia, head injury, alcohol intoxication, or metabolic disorder. Presenile dementias, caused by frontotemporal lobar degeneration, progressive supranuclear palsy, and corticobasal degeneration, usually occur in patients of presenile and are rarely seen in patients of senile age. Although the factors responsible for the accelarted onset of the illness are not fully known, genetic abnormalities appear to be important in some types of presenile dementia, such as frontotemporal dementia with parkinsonism linked to chromosome 17. Conversely, senile dementias such as sporadic AD and VaD commonly occur in patients of senile age. These disorders may also occur in patients of presenile age, although less frequently. Alzheimer's disease was originally classified as a ‘presenile dementia’. Since the 1980s, ‘senile dementia of Alzheimer type’ (SDAT) and ‘Alzheimer's disease’ have been considered to belong to the same pathological entity and both are now known as ‘dementia of Alzheimer's type (DAT)’ or merely ‘Alzheimer's disease’. Rapid progression of cognitive impairment with neuropsychological syndromes and neurological symptoms has been considered a characteristic of early onset AD. However, recently, neurological symptoms such as spastic paraparesis, seizures, and myoclonic convulsions have been reported to occur infrequently in early onset AD, although language problems and visuospatial dysfunctions are common. There are at least three dominant genes that have been identified in cases of familial Alzheimer's disease with early onset, namely the amyloid precursor gene (APP), and the genes encoding presenilin 1 (PSEN1) and presenilin 2 (PSEN2). Therefore, genetic abnormalities are important factors contributing to the earlier onset of the illness. It is also important to investigate the pathophysiological mechanism in relation to genetic abnormalities, environmental factors, physical illnesses, and metabolic disturbances to understand the processes underlying the development of dementia with early onset.     

Supporting family dementia caregivers: testing the efficacy of dementia care management on multifaceted caregivers’ burden

ABSTRACT

Objectives: Current research suggests that dementia care management (DCM) can decrease burden and associated health impairments of caregivers. The objective of this secondary analysis is to investigate the impact of DCM on multifaceted caregivers’ burden dimensions by differentiating between objective and subjective burden.

Methods: A sample of n = 317 dyads of caregivers and community-dwelling people with dementia (PwD) participated in a general practitioner-based, cluster-randomized intervention trial (Identifier:NCT01401582) with two arms and comprehensive data assessment at baseline and 12-month follow-up. Data provided by the caregiver included an inventory with 88 items in 20 different dimensions.

Results: Caregivers in the intervention ‘DCM’ group showed decreased caregiver burden, especially in caregivers’ objective burden due to caring (i.e. emotional support), caregivers’ subjective burden due to behavior change (i.e. cognition, aggression and resistance, depression, late symptoms) and caregivers’ subjective burden due to perceived conflicts between needs and responsibilities to care (i.e. financial losses) compared to caregivers in the control ‘care as usual’ group, which showed significant increased caregiver burden after 12 months.

Conclusion: Our findings support evidence for the effectiveness of DCM to lower family dementia caregivers' burden in multifaceted dimensions.     

Effect of apolipoprotein E status on clinical features of dementia

Objective. The aims of this study were (a) to determine the frequency of APOE genotypes in dementia, (b) to relate e4 allele frequency to clinical symptomatology of dementia and (c) to relate e4 and assess risk factors for different types of dementia. Design. Prospective clinical study setting older patients with dementia known to a community-based old age psychiatry service. Sample. 101 patients fulfilling ICD 10 criteria for dementia. Results. Replication of previous findings of an association between APOE4 and Alzheimer's disease: younger age of onset of dementia; family history of dementia; persecutory ideation; and (retrospectively determined) rate of competent decline. No association was found between APOE4 and vascular dementia. The association between APOE4 and ‘mixed dementia’ was intermediate between that of pure Alzheimer's disease and pure vascular dementia. Conclusion. The results confirm the relationship between clinical features of dementia and APOE4 status. It may be that APOE can be used as an adjunct to clinical diagnosis. © 1998 John Wiley & Sons, Ltd.     

Caring For Me and You: the co-production of a computerised cognitive behavioural therapy (cCBT) package for carers of people with dementia

ABSTRACT

Objectives: Carers of people with dementia face barriers in accessing therapy for mental health difficulties. Computerised cognitive behavioural therapy (cCBT) packages can be effective in treating a range of presentations; however, tailored packages for carers' unique needs are lacking. Our aim was to design a cCBT package for carers to address the limitations of previous online interventions, by including users and experts as consultants and collaborators throughout the project.

Method: We adopted a three-phase approach to the development process. Firstly, a data-gathering phase in which current literature and best practice was reviewed, and semi-structured interviews conducted with service users, academic and clinical experts. Secondly, a co-production and refinement phase with carers testing materials and providing feedback. Thirdly, a pilot field testing phase of service users and the research team testing the package.

Results: The ‘Caring For Me and You’ package adopted a transdiagnostic approach to take account of the range of difficulties that carers face. The package consisted of 20 short sessions with features built in to engage users and personalise content to meet individuals' needs.

Conclusion: User involvement was central to the design of the ‘Caring For Me and You’ package which is currently being evaluated in a three-arm randomised controlled trial.     

Reliability and validity of the Norwegian version of the Brief Agitation Rating Scale (BARS) in dementia

Objective: To examine the test–retest reliability and validity of the Norwegian Brief Agitation Rating Scale (BARS), a short form of the Cohen-Mansfield Agitation Inventory (CMAI) assessing the frequency of agitation in dementia.

Methods: We investigated the internal consistency, test–retest reliability and the validity of BARS. In the validity study, we compared the BARS scores with the Neuropsychiatric Inventory – Nursing Home Version subscale Agitation/Aggression (NPI-NH/AA) and the Cornell Scale for Depression in Dementia subscale Agitation (CSDD/A).

Results: In the reliability study, Cronbach's alpha was 0.76; the test–retest reliability of the BARS showed a Spearman's rho of 0.64, but this increased to 0.86 when we deleted the item ‘complaining’. In the validation study, the BARS score correlated with the NPI-NH/AA and the CSDD/A scores, Spearman's rho 0.55 and 0.52, respectively. These correlations changed when controlling for the Clinical Dementia Rating (CDR) Scale stages. The highest correlations between the BARS and the NPI-NH/AA and the BARS and the CSDD/A were found among patients with CDR score 2.

Conclusions: The study indicates that the Norwegian version of BARS is a reliable and valid instrument to test agitation in dementia, but a version without the item ‘complaining’ would be better.     

Caregiver profiles in dementia related to quality of life,depression and perseverance time in the European Actifcare study: the importance of social health

ABSTRACT

Objectives: To identify caregiver profiles of persons with mild to moderate dementia and to investigate differences between identified caregiver profiles, using baseline data of the international prospective cohort study Actifcare.

Methods: A latent class analysis was used to discover different caregiver profiles based on disease related characteristics of 453 persons with dementia and their 453 informal caregivers. These profiles were compared with regard to quality of life (CarerQoL score), depressive symptoms (HADS-D score) and perseverance time.

Results: A 5-class model was identified, with the best Bayesian Information Criterion value, significant likelihood ratio test (p < 0.001), high entropy score (0.88) and substantive interpretability. The classes could be differentiated on two axes: (i) caregivers' age, relationship with persons with dementia, severity of dementia, and (ii) tendency towards stress and difficulty adapting to stress. Classes showed significant differences with all dependent variables, and were labelled ‘older low strain’, ‘older intermediate strain’, ‘older high strain’, ‘younger low strain’ and ‘younger high strain’.

Conclusion: Differences exist between types of caregivers that explain variability in quality of life, depressive symptoms and perseverance time. Our findings may give direction for tailored interventions for caregivers of persons with dementia, which may improve social health and reduce health care costs.     

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

ABSTRACT

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.     

Is physical activity a potential preventive factor for vascular dementia? A systematic review

Background: Physical exercise has several beneficial effects, including reduced risk for Alzheimer's disease. Although several studies of potential risk factors for vascular dementia (VaD) exist, including physical activity, the studies have usually included few participants and there are no meta-analyses addressing this key topic.

Methods: The MEDLINE database was searched using the key words ‘physical exercise’ ‘activity’ or ‘walking’ in combination with ‘dementia’ and ‘vascular dementia’. Potentially relevant studies were assessed and summarised by two of the authors, and longitudinal studies with operationalised definition of physical activity providing risk for VaD in both groups were included in the meta-analysis using pooled estimates from a random effects model.

Results: A total of 24 longitudinal studies, including 1378 patients with VaD, were included in the review. The majority of individual studies did not report significant associations. Five studies fulfilled criteria for meta-analysis, including 10,108 non-demented control subjects and 374 individuals with VaD. The meta-analysis demonstrated a significant association between physical exercise and a reduced risk of developing VaD: OR 0.62 (95% CI 0.42–0.92).

Conclusions: We conclude that there is evidence supporting the hypothesis that physical activity is likely to prevent the development of VaD, and should be highlighted as part of secondary prevention programmes in people at risk for cerebrovascular disease.     

The development and validation of the Dementia Quality of Life Scale for Older Family Carers (DQoL-OC)

Purpose: Little is known about how caregiving affects the quality of life (QoL) of older family carers and no dementia and age-specific QoL scale is available for use with this population. This study aimed to develop and validate a unique dementia caregiving- and age-specific tool – the ‘Dementia Quality of Life Scale for Older Family Carers’ (DQoL-OC).

Methods: The scale items were identified in focus groups with older family carers in the UK. Content and face validity were evaluated by a panel of six experts. A set of 100 items assessed on a 5-point Likert scale was tested with 182 older family carers. Test–re-test reliability was conducted with 18 individuals. Exploratory factor analysis was used to identify the QoL model and reduce the number of scale items. Convergent construct validity and internal consistency were also established.

Results: A one-factor solution containing 22 items was obtained. Test–re-test reliability (lower bound r = 0.835; p < 0.001), internal consistency (Cronbach's α = 0.936), and convergent construct validity were established. Significantly lower levels of QoL were found in female older carers; those who perceived their relatives with dementia as being at the earlier stages of the disease and with unstable dementia symptoms; those providing care more hours per day and more days per week; and those in younger-old age.

Conclusions: The DQoL-OC is a valid and reliable scale that will be useful for research and in clinical practice with older family carers of people with dementia. These study results will inform future health and social care aiming to improve life quality for this overlooked population of carers.     

Time to reinvent the science of dementia: the need for care and social integration

Objectives: The increasing number of older adults with dementia is a large and growing public health problem. Alzheimer's disease, the prevailing form of dementia, is projected to quadruple worldwide. To date, the care and social integration of individuals with dementia is complicated by limited collaborations between biomedicine and other disciplines. The objective of this paper is therefore to reflect on the orientation of biomedicine with regard to the science of dementia, and to articulate a path for moving forward. Methods: The authors drew upon, and expanded, the insights of an interdisciplinary, international workshop entitled ‘Bioethics and the Science of Aging: The Case of Dementia’ held in October 2012 at the University of California in Berkeley.Results: The care of individuals with dementia compels solid interdisciplinary collaborations. There are several issues affecting the care of individuals with dementia: (1) an evolving definition of dementia; (2) the ambiguous benefits of the diagnosis of dementia; (3) ethical conflicts concerning consent processes and clinical trials; and (4) a limited understanding of the perspective of the person with dementia.Conclusion: We argue that it is time for a renewed dialogue between biomedicine and other disciplines -- particularly public health, the social sciences, the medical humanities and bioethics. This interdisciplinary dialogue would facilitate a process of self-reflection within biomedicine. This dialogue will also provide the foundation for equitable public health interventions and will further prioritize the values and preferences of individuals with dementia, as well as their care and social integration.     

Clinical profiles of late‐onset semantic dementia,compared with early‐onset semantic dementia and late‐onset Alzheimer's disease

Background: Semantic dementia (SD) has been recognized as a representative of dementia with presenile onset; however, recent epidemiological studies have shown that SD also occurs in the elderly. There have been few studies about the differences of clinical profiles between early‐onset SD (EO‐SD) and late‐onset SD (LO‐SD). Age‐associated changes in the brain might cause some additional cognitive and behavioural profiles of LO‐SD in contrast to the typical EO‐SD cases. The aim of the present study was to clarify the characteristics of neuropsychological, and behavioural and psychological symptoms of dementia (BPSD) profiles of LO‐SD patients observed in screening tests in comparison with EO‐SD patients and late‐onset Alzheimer's disease (LO‐AD) patients as controls. Methods: Study participants were LO‐SD (n = 10), EO‐SD (n = 15) and LO‐AD (n = 47). We examined the Mini‐Mental State Examination (MMSE), the Raven's Coloured Progressive Matrices (RCPM), the Short‐Memory Questionnaire (SMQ), the Neuropsychiatric Inventory (NPI) and the Stereotypy Rating Inventory (SRI). Results: Both SD groups scored significantly lower than the LO‐AD patients in ‘naming’ of the MMSE. In the ‘construction’ score of the MMSE and the RCPM score, however, the LO‐SD patients as well as the LO‐AD patients were significantly lower than the EO‐SD patients. In the SMQ score, ‘euphoria’ and ‘disinhibition’ scores of the NPI, the SRI total and subscale scores, both SD groups were significantly higher, whereas in the ‘delusion’ score of the NPI, both SD groups were significantly lower than the LO‐AD patients. Conclusions: Visuospatial and constructive skills of LO‐SD patients might be mildly deteriorated compared with EO‐SD patients, whereas other cognitive and behavioural profiles of LO‐SD are similar to EO‐SD. Age‐associated changes in the brain should be considered when we diagnose SD in elderly patients.     

Development,reliability, and validity of a self-assessment scale for dementia care management

Background

This study aimed to develop a self-assessment scale for care management of people with dementia and examine its reliability and validity.

Methods

Based on Bloom's Taxonomy, previous research, and experts' opinions on dementia and care management, a scale consisting of 18 items was developed to assess care managers' attitudes, knowledge, and skills in their management of people with dementia. To examine the scale's reliability and validity, data were collected from 638 care managers. Construct validity using exploratory factor analysis, known-group validity, and internal consistency reliability of the scale were evaluated.

Results

Exploratory factor analysis supported the construct validity with a four-factor model and explained 59.1% of the total variance. Following were the four factors: Factor I ‘Person centred care’; Factor II ‘Understanding of disease characteristics, treatment and care’; Factor III ‘Understanding of people with dementia and care management according to their characteristics’; and Factor IV ‘Utilization of local resources surrounding people with dementia’. Regarding the known-group validity, results showed that the group with a qualified chief care manager scored significantly higher than the group without one on Factors I (P = 0.013) and III (P = 0.026). Cronbach's alpha coefficient for the 18 items was 0.928.

Conclusions

The findings prove that the scale has acceptable reliability and validity, and can help care managers reflect on their practice. Future research is desirable to measure the validation of change in the scale.     

Exploring the relationship between physical health,depressive symptoms,and depression diagnoses in Hispanic dementia caregivers

Objectives: The present study examined the relationship between self-reported physical health, depressive symptoms, and the occurrence of depression diagnosis in Hispanic female dementia caregivers. Participants: Participants were 89 Hispanic female dementia caregivers. Design: This study used a cross-sectional design. Baseline depression and physical health data were collected from participants enrolled in the ‘Reducing Stress in Hispanic Anglo Dementia Caregivers’ study sponsored by the National Institute on Aging. Measurements: Physical health was assessed using the Medical Outcome Study Short Form-36 (SF-36), a one-item self-report health rating, body mass index, and the presence or history of self-reported physical illness. Depressive symptoms were assessed using the Center for Epidemiologic Studies–Depression Scale (CES-D). The occurrence of depression diagnosis was assessed using the Clinical Interview for DSM-IV Axis I Disorders (SCID). Analysis: Multiple linear and logistic regression analysis was used to examine the extent to which indices of physical health and depressive symptoms accounted for variance in participants’ depressive symptoms and depressive diagnoses. Results. Self-reported indices of health (e.g., SF-36) accounted for a significant portion of variance in both CES-D scores and SCID diagnoses. Caregivers who reported worsened health tended to report increased symptoms of depression on the CES-D and increased likelihood of an SCID diagnosis of a depressive disorder. Conclusion. Self-reported health indices are helpful in identifying Hispanic dementia caregivers at risk for clinical levels of depression.     

ORIGINAL ARTICLE: Investigation of responders and non‐responders to long‐term donepezil treatment

Background: Donepezil is effective in maintaining the cognitive function of patients with mild to moderate Alzheimer's disease (AD). However, not all patients respond to donepezil. In the present study, we examined the clinical features of responders and non‐responders to long‐term donepezil treatment. Methods: The present retrospective study was performed on 95 AD outpatients who had been taking donepezil for ≥2 years. All subjects underwent periodic examinations of cognitive function, namely Mini‐Mental State Examination (MMSE) and Rorschach Cognitive Index (RCI), as well as clinical evaluations using the Clinical Dementia Rating (CDR) scale. Patients were divided into three groups as follows: (i) the ‘maintained’ group (MG), in which the global CDR score was maintained over the ≥2 years of treatment; (ii) the ‘declined’ group (DeG), in which the global CDR score increased one rank over the treatment period; and (iii) the ‘obvious and rapid decline’ group (ORDeG), in which the global CDR score increased two ranks early during the treatment period. Clinical features, treatment outcome, the time lag between a caregiver's recognition of the onset of dementia and the start of treatment, behavioral and psychological symptoms of dementia (BPSD), and cognitive functions were compared between the three groups. Results: Patients in the ORDeG (i.e. non‐responders) were significantly younger and had a longer time lag between the onset of dementia and the start of treatment than patients in the MG (P < 0.05). Of note, patients in the ORDeG had a longer period of executive dysfunction before treatment started than patients in the MG (P < 0.001). Evaluation of cognitive function revealed that mean changes from baseline on the MMSE and RCI were significantly lower for patients in the ORDeG compared with the MG at 8 and 4 months, respectively (P < 0.001 and P < 0.05, respectively). Conclusion: Donezepil non‐responders are likely to be younger and to have a longer time lag between the onset of dementia and the start of treatment, in particular a longer duration of executive dysfunction. Furthermore, the non‐responders do not demonstrate maintenance of cognitive functions in the short term. Thus, the early diagnosis of dementia and prompt initiation of donepezil treatment is indicated for a good outcome. To this end, it is important to educate people to recognize a deterioration of executive function in daily living.     

The effect of stereotype threat on older people’s clinical cognitive outcomes: investigating the moderating role of dementia worry

Objective: Numerous studies have shown that stereotype threat (ST) reduces older people’s cognitive performance, but few have studied its impact on clinical cognitive outcomes. Our study was designed to further examine the impact of ST on the clinical assessment of older subjects’ cognitive functioning, as well as the moderating role of fear of Alzheimer’s Disease (AD) (or ‘dementia worry’). Method: Seventy-two neurologically normal (MMSE > 26) participants aged between 59 and 70 completed a set of neuropsychological tasks in either an ST or a positive condition (condition in which negative stereotypes were invalidated). Results: Regression-based path analyses showed that only participants who expressed moderate or high fear of AD underperformed on executive tasks in the ST condition compared to their counterparts in the positive condition. Moreover, in the ST condition, participants’ performance on executive tasks was more impaired (relative to normative data) than in the positive condition. However, ST had no effect on memory and attention performance. Discussion: Our results showed that ST can cause older people to perform at pathological levels on executive tasks. Results highlight the need for clinicians to be cautious when conducting neuropsychological assessments of older people who express high levels of dementia worry.     

Prevalence of dementia and dementing diseases in the old‐old population in Japan: the Kurihara Project. Implications for Long‐Term Care Insurance data

Background: There have been no reports on the prevalence of dementia among the old‐old people in Japan. Methods: We studied the old‐old population in Kurihara, northern Japan. Analysis 1 of Participants 1 (n = 590) was performed to evaluate the prevalence of dementia and dementing diseases by intensive evaluation including MRI. Analysis 2 aimed to determine a good indicator for detecting ‘suspected dementia condition’ based on the Long‐Term Care Insurance index. Analysis 3 of Participants 2 (n = 3915) aimed to estimate the prevalence of ‘suspected dementia condition’. Results: In Analysis 1, 73 people (12.4%) were diagnosed with dementia. The most common cause was Alzheimer's disease with cerebrovascular disease. In Analysis 2, level I of the Impairment Level of Dementia was found to be a good indicator of ‘suspected dementia condition’. In Analysis 3, the overall estimated prevalence of ‘suspected dementia condition’ was 23.6%. In men, the ratio increased gradually from 75 to 87 years old to about 20%, increased to 40% at the age of 88 and became stable thereafter. In contrast, in women, the ratio increased from 75 to 95+ years old, reaching about 70%. Conclusions: The prevalence was higher than that reported previously. There was a difference between the sexes: an ‘age‐related’ increase occurred in men and an ‘ageing‐related’ increase in women. Alzheimer's disease with cerebrovascular disease was the most common cause, which coincided with the previous findings of individuals aged 65 years and older; however, the ratio of mixed dementia was greater.     

Spouse caregivers and behavioral and psychological symptoms of dementia

Background: Only a few studies have specifically considered the role of caregiver characteristics in the presence of behavioral and psychological symptoms of dementia (BPSD). The aim of this study was to determine whether there were differences in the presence of individual BPSD between community-dwelling dementia care recipients with spouse caregivers and those with non-spouse caregivers.

Methods: Care recipients (n = 109) and their primary caregivers were recruited from memory clinic outpatients at the public psychiatric hospital in Sapporo City, Japan. Data were collected by questionnaire. Relationship with the care recipient was categorized as either ‘spouse’ or ‘non-spouse.’ The frequency of BPSD occurrence observed by the caregiver was assessed using the Troublesome Behavior Scale (TBS). Logistic regression analyses were performed to examine whether there were differences with regard to each of the 14 TBS items between spouse and non-spouse caregivers.

Results: The number of spouse caregivers was 47 (43.1%). TBS items presented by >50% care recipients were ‘repetition and/or clinging’ and ‘ill-natured denial and/or distortion.’ After adjustment for the characteristics of caregivers and care recipients, non-spouse caregivers were found to be significantly associated with the presence of ‘hiding and/or losing things,’ ‘rummaging,’ ‘crying and/or screaming,’ and ‘interfering with a happy home circle,’ compared with spouse caregivers.

Conclusions: Our results suggest that non-spouse caregivers need more support with regard to certain symptoms of individual BPSD compared with spouse caregivers. Identifying caregiver characteristics that are independently associated with each individual BPSD may help customize interventions for caregivers with specific characteristics.     

Depression–dementia medius: Between depression and the manifestation of dementia symptoms

Depression and dementia, among the most common conditions in clinical practice, sometimes coexist, sometimes succeed each other, and often confuse clinicians. In the present paper, the clinical concept of ‘depression–dementia medius’ (which includes pseudodementia and depression in Alzheimer's disease as exemplars) is proposed, in reference to Janet's concept of psychological tension. Because psychosomatically complex human lives are always in a state of dynamic equilibrium, it seems sensible to propose that pseudodementia and depression in Alzheimer's disease are located within a spectrum extending from depression without dementia symptoms to dementia without depression. From the Janetian viewpoint, pseudodementia is regarded as uncovered latent dementia as a result of reduced psychological tension. Dementia is more than a fixed progressive condition under this view, and is a manifestation of dynamic mental activities. Characterizing these entities through perspectives such as psychological tension may yield deep insights in clinical practice.