Am J Alzheimers Dis Other Demen. 2013 Feb;28(1):42-6.

The needs of patients with early onset dementia.

Armari E, Jarmolowicz A, Panegyres PK.

Neurodegenerative Disorders Research Pty Ltd, Subiaco, Perth, Australia.



The burden of early onset dementia (EOD) is often overshadowed by an ageing population.

A questionnaire comprising of 12 items was completed by 18 EOD patients (AD= 15, FTD=3) and 39 caregivers (spouses=20, children=8, siblings=7, carers=2, health professionals= 2) prior to the commencement of a symposium on EOD care and research. The onset of patients’ symptoms was prior to the age of 65 years. Caregivers had to be supporting someone who matched these criteria.  Early recognition and referral was perceived as the principle area of improvement by both patients (94.4%) and carers (69.2%) (p <0.0002). Patients evaluated ‘diagnosis’ as the area of most need (88.9%) compared with caregivers who rated ‘Treatment’ (69.2%) as their principle concern.  The perceived concerns of EOD patients differ from caregivers. Continued consumer involvement is essential in ensuring a tailored approach to young people with dementia.

PMID: 23220922



Dementia in young adults is typically misdiagnosed, under-recognised and inadequately managed with limited services; however, this is rapidly changing. Disease awareness has gained increasing momentum following campaigning efforts of patients, their caregivers and health service providers within the last decade (1).

Obtaining an accurate diagnosis of EOD is challenging and requires the consideration of an extensive list of differential diagnoses (2). As a consequence, it is likely that the real prevalence of EOD  has been underestimated (1).  Alzheimer’s disease (AD) remains the most common dementing illness in EOD patients (3), although it is only representative of a third of cases (4). Dementia evolving as a terminal consequence of other neurodegenerative processes, metabolic disorders or inborn errors of metabolism are emerging as important aetiologies in younger populations (1). Early identification of these predisposing disorders may provide an opportunity for earlier intervention and secondary prevention.

Misdiagnosis is common and has been reported in 30-50% of patients with EOD (5). These figures have often been attributed to poor clinician understanding of the disease in younger populations (6) and the consequent symptomatic progression which follows (4). In fact, clinicians often express limited confidence in their diagnosis of dementia even amongst elderly populations (7).

On average, the duration between first presentation and diagnosis of EOD exceeded that of late onset dementia (LOD) by 1.6 years (8). However, several studies have demonstrated that the time to diagnosis may exceed 5 years (6),(8).

Insidious cognitive and behavioural changes in absence of a timely medical diagnosis adversely affect relationships between patient and their family networks (5),(8),(9). Once the diagnosis of EOD has been established, maintaining relationships appears to become increasingly challenging (10). As a consequence the grief accompanying diagnosis, the loss of roles and responsibilities and appropriate healthcare support are markedly different for younger patients and their families and therefore, demand a distinct approach (6),(9).

Establishing the diagnosis early in the disease course provides the opportunity to engage with appropriate health services, understand the consequences of disease progression and plan for the future (6),(7),(11). Providing patients and their families with knowledge is a fundamental prerequisite to assist with their grief process, adjust to the change in circumstances and cope with the situation in the long term (9). Appropriate changes to the family routine, income providers and care of dependents must be assessed and planned for at each stage of disease progression.

For caregivers, providing daily assistance for their spouse highlights their changing role within the family hierarchy and realisation that their own future plans have changed significantly (9),(12). Carers may be forced to resume employment, have a greater role in child rearing and gain more responsibilities in matters of household finance and insurance, while their partner became increasingly unable to fulfill their previous roles (9). Not surprisingly, carers of EOD patients suffer significantly higher levels of stress and depression than experienced in LOD (13). Those most vulnerable to psychological distress appear to be younger females caring for their spouse as well as dependent children (6).

The severity of stress and depression endured by carers increases with the severity of behavioural change and lack of insight most commonly accompanying frontotemporal dementia (FTD) (10). In addition, social isolation and feelings of exclusion are heightened in younger caregivers as a consequence of limited public awareness and acceptance of EOD as well as inadequate formal support services (5),(12).

Existing health services are predominantly geared towards elderly patients (4) and are resultantly ill-equipped to appropriately support patients with EOD and their families. Initial entry into these existing health services however, remains complicated and problematic for EOD patients (11). Services are often uncoordinated; meaning that several referrals are usually made and little multidisciplinary organization is maintained throughout the course of diagnosis and subsequent management (11).

Eliciting the experiences and perspectives of young patients and their caregivers remains paramount in augmenting health services to adequately support individuals and their unique needs (4),(11). Currently, this consumer participation is largely absent (4). In absence of a timely diagnosis, appropriately focused health services and informal support networks for younger caregivers those with EOD are falling by the wayside at a huge financial and moral cost to the broader community. Consequently, this paper seeks to gain further insight from consumers so to assess the appropriateness of existing health services and management of EOD within an Australian context.

While misdiagnosis and consequent mismanagement remains common, the efforts of patients, their carers and clinicians within the last decade have provided the momentum needed to highlight EOD as an emerging and significant public health issue. It is evident that the needs of patients with EOD and their carers are unique, providing distinct challenges for existing health services. Subsequently, adequate disease management can only be achieved when the perspectives of patients and caregivers are considered.

A timely diagnosis is essential for the grief process and long term coping as well as for foreword planning of finances, insurance and care of any dependent children (9). The importance of an accurate diagnosis is further highlighted in this study, with both patients and caregivers revealing ‘Early recognition and referral’ as the principle area of EOD care requiring significant improvement. This finding further supports the apparent complications of entry to health services even when a diagnosis is made (11).

Despite the literature highlighting the importance of early diagnosis, our caregiver sample interestingly highlighted ‘Treatment’ as the principle area of need in EOD. While there are indeed stark differences in the presentation and consequences of EOD compared to LOD, evidence suggests that the median survival time of 6.08 years from first presentation is similar for both (2),(13). Coupled with lengthy diagnostic time, the survival of EOD patients is tragically short. Requests for prioritizing treatment may be a reflection of a focus in prolonging life and also prolonging functionality.

While reassuring that EOD is increasingly acknowledged as an important public health challenge, its relatively low prevalence inherently hampers efforts to generate solid research findings and large scaled public interest (1). In addition, the lack of consistency and implementation of appropriate, EOD-specific diagnostic criteria for research participants is problematic.



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