Summary
Young‐onset dementia comprises a heterogeneous range of dementias, with onset at less than 65 years of age. These include primary dementias such as Alzheimer disease, frontotemporal and ...vascular dementias; genetic/familial dementias; metabolic disorders; and secondary dementias such as those that result from alcohol use disorder, traumatic brain injury, and infections.
The presentation of young‐onset dementia is varied and may include cognitive, psychiatric and neurological symptoms. Diagnostic delay is common, with a frequent diagnostic conundrum being, “Is this young‐onset dementia or is this psychiatric?”.
For assessment and accurate diagnosis, a thorough screen is recommended, such as collateral history and investigations such as neuroimaging, lumbar puncture, neuropsychology, and genetic testing.
The management of young‐onset dementia needs to be age‐appropriate and multidisciplinary, with timely access to services and consideration of the family (including children).
Iron has been increasingly implicated in the pathology of neurodegenerative diseases. In the past decade, development of the new magnetic resonance imaging technique, quantitative susceptibility ...mapping (QSM), has enabled for the more comprehensive investigation of iron distribution in the brain. The aim of this systematic review was to provide a synthesis of the findings from existing QSM studies in neurodegenerative diseases. We identified 80 records by searching MEDLINE, Embase, Scopus, and PsycInfo databases. The disorders investigated in these studies included Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, Wilson's disease, Huntington's disease, Friedreich's ataxia, spinocerebellar ataxia, Fabry disease, myotonic dystrophy, pantothenate-kinase-associated neurodegeneration, and mitochondrial membrane protein-associated neurodegeneration. As a general pattern, QSM revealed increased magnetic susceptibility (suggestive of increased iron content) in the brain regions associated with the pathology of each disorder, such as the amygdala and caudate nucleus in Alzheimer's disease, the substantia nigra in Parkinson's disease, motor cortex in amyotrophic lateral sclerosis, basal ganglia in Huntington's disease, and cerebellar dentate nucleus in Friedreich's ataxia. Furthermore, the increased magnetic susceptibility correlated with disease duration and severity of clinical features in some disorders. Although the number of studies is still limited in most of the neurodegenerative diseases, the existing evidence suggests that QSM can be a promising tool in the investigation of neurodegeneration.
Objectives
Carer burden in dementia is associated with poor outcomes, including early nursing home placement for people with dementia and psychological distress for their carers. Carers of people ...with young‐onset dementia (YOD) are particularly vulnerable to carer burden. Yet they are often overlooked by clinicians as dementia services are generally designed for older people. We sought to estimate the rate of burden and psychological distress in carers of YOD at a state‐wide tertiary service based in Australia.
Methods
We conducted a cross‐sectional study examining 71 dyads from a Neuropsychiatry service. We collected patient demographic and clinical data including the Neuropsychiatry Unit Cognitive Assessment tool (NUCOG) and Mini‐Mental State Examination (MMSE). Carer data, such as demographics and psychological distress, were obtained using Depression Anxiety Stress Scale 21 (DASS‐21). Carer burden was rated using the Zarit Burden Inventory‐short version (ZBI).
Results
Higher carer burden, measured using ZBI, was associated with longer duration of dementia and greater severity of overall cognitive impairment. Carers who felt burdened reported higher levels of stress, depression, and anxiety measured using DASS‐21. Multiple linear regression analysis found carer burden was independently predicted by duration of dementia, total cognition score and carers experiencing psychological stress.
Discussion
We found that patient variables of dementia duration and cognitive impairment and carer variable of carer stress to be associated with carer burden. Poor executive function was associated with carer stress. Early identification and management of carer burden and psychological distress is important for outcomes. Ideally, this should be provided by a specialist YOD service.
Key points
In an Australian tertiary neuropsychiatric service, almost a half of informal carers of young‐onset dementia reported feeling significantly burdened.
Furthermore, over a third of the carers felt depressed, anxious, or stressed.
Higher carer burden was associated with longer duration of dementia and greater severity of overall cognitive impairment.
Early identification and management of carer burden and psychological distress is important to achieve better outcomes for both the patient and carer.
Young-onset dementia (YOD) refers to a dementia for which symptom onset occurs below the age of 65. This review summarizes the recent literature in this area, focusing on updates in epidemiology, ...diagnosis and service provision.
In the last year, internationally, the prevalence of YOD was reported as 119 per 100 000, but this may vary according to population types. Although the commonest causes of YOD are Alzheimer's disease (AD) and frontotemporal dementia (FTD), there is increasing recognition that YOD is diagnostically and phenotypically broader than AD and FTD. YOD may be due to many other diseases (e.g. Huntington's disease, vascular dementia) whereas accumulation of the same protein (e.g. amyloid protein) may lead to different phenotypes of Alzheimer's disease (such as posterior cortical atrophy and behavioural-variant/frontal-variant AD). This heterogeneity of phenotypic presentation is also seen in YOD due to known genetic mutations. Biomarkers such as plasma and cerebrospinal fluid proteins, neuroimaging and genetics have shown promise in the early identification of YOD as well as providing further understanding behind the overlap between psychiatric and neurodegenerative conditions occurring in younger people. The management of YOD needs to consider age-specific issues for younger people with dementia and their family networks together with better integration with other health services such as aged, disability and improved access to services and financial assistance.
These findings emphasize the need for early identification and appropriate age-specific and person-centred management for people with young-onset dementia.
Objectives:
To provide a clinical update for general psychiatrists on the assessment and diagnosis of Alzheimer’s disease (AD), highlighting current issues regarding epidemiology, risk factors and ...pathophysiology from recent relevant research findings.
Conclusions:
Psychiatrists can apply their skills and training in the diagnosis of AD, which is based upon a comprehensive assessment comprising history, investigations, and cognitive and functional assessment, guided by accepted diagnostic criteria.
While early diagnosis of younger-onset dementia (YOD) is crucial in terms of accessing appropriate services and future planning, diagnostic delays are common. This study aims to identify predictors ...of delay to diagnosis in a large sample of people with YOD and to investigate the impact of a specialist YOD service on this time to diagnosis.
A retrospective cross-sectional study.
The inpatient unit of a tertiary neuropsychiatry service in metropolitan Victoria, Australia.
People diagnosed with a YOD.
We investigated the following predictors using general linear modeling: demographics including sex and location, age at onset, dementia type, cognition, psychiatric diagnosis, and number of services consulted with prior to diagnosis.
A total of 242 inpatients were included. The mean time to diagnosis was 3.4 years. Significant predictors of delay included younger age at onset, dementia type other than Alzheimer's disease (AD) and behavioral-variant frontotemporal dementia (bvFTD), and increased number of services consulted. These predictors individually led to an increased diagnostic delay of approximately 19 days, 5 months, and 6 months, respectively. A specialized YOD service reduced time to diagnosis by 12 months.
We found that younger age at onset, having a dementia which was not the most commonly occurring AD or bvFTD, and increasing number of services were significant predictors of diagnostic delay. A novel result was that a specialist YOD service may decrease diagnostic delay, highlighting the importance of such as service in reducing time to diagnosis as well as providing post-diagnostic support.
Posterior Cortical Atrophy (PCA) is a rare form of young-onset dementia that causes early visuospatial and visuoperceptual deficits. The symptom profile of Posterior Cortical Atrophy leads to very ...specific care needs for those affected, who often rely on informal caregivers (including friends and family). Rare dementia support groups can be useful for both patients and their caregivers to assist with knowledge sharing, psychoeducation, and the provision of psychosocial support. Despite this, few such support groups exist. The purpose of this study was to examine a PCA support group for caregivers of individuals living with PCA. We held a structured psychoeducation support group comprised of four sessions with the aim being to provide education, strategies for the management of the disease, and peer support. Caregivers' mental health and quality of life were assessed. The results of our study showed that support group participation was a positive experience and assisted with increasing the knowledge of caregivers and fostering social connections. We suggest that peer support groups may be beneficial for both people living with PCA and their caregivers. We recommend that future quantitative and qualitative research is conducted to further assess health-promotion benefits to people living with PCA and their caregivers, and to assess their development and implementation in different contexts.
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