JAMDA 14 (2013) 230e232

JAMDA

journal homepage: www.jamda.com

Editorial

The Challenges of Service Provision in Younger-Onset Dementia

Adrienne Withall PhD *School of Public Health and Community Medicine, University of New South Wales, Sydney, Australia

Younger-onset dementia refers to an onset of memory or othercognitive signs and/or behavioral symptoms before the age of 65years.1 Clinically, younger-onset dementia is much more diverse thanlate-onset dementia, with a much higher proportion of preventablecauses. Regarding presentation, there is great heterogeneity withinthis group, although prominent behavioral signs are characteristic.Dementia at a relatively young age is unexpected, distressing, and canhave devastating consequences for the person, and his or her partnerand children. It is associated with significant social and financialimpacts and high levels of burden.

People with younger-onset dementia face issues very differentfrom older persons with the condition and do not fit in well withmainstream dementia and/or aged care services, which are aimed atthe elderly. To enable adequate service provision for younger peoplewith dementia, whether in a community or residential setting, it isnecessary to understand the clinical features, prevalence, burden, andservice needs of this group.

Causes and Clinical Features of Younger-Onset Dementia

Etiologically, younger-onset dementia is diverse and includesa range of dementias not typically seen in later life. Alzheimerdisease, although still relatively common, accounts for only 30% to50% of all cases.2,3 Instead, there is an increase in the rate of fronto-temporal dementias (including behavioral and language variants), aswell as dementia occurring secondary to another medical illness(such as Huntington disease, Down syndrome, and multiple scle-rosis).2,3 Importantly, there are also more preventable causes inwhich there are opportunities for intervention, such as dementia dueto HIV/AIDS, chronic traumatic encephalopathy, and dementiasecondary to alcohol or other substance abuse.4,5 This latter groupwith dementia attributable to alcohol or other substance abuse isoften particularly isolated clients, who are usually estranged fromfamily members and present to acute care services because ofa medical incident, such as a fall.4 Indeed, not all clients have carers orsupporters, and this causes difficulties in locating and presenting toappropriate services.

Genetically inherited dementias, such as certain cases of Alzhei-mer disease and frontotemporal dementia, are more common in

DOI of original article: 10.1016/j.jamda.2012.09.011.* Address correspondence to Adrienne Withall, PhD, School of Public Health and

Community Medicine, Samuels Building, University of New South Wales, Sydney,Australia, 2052.

E-mail address: a.withall@unsw.edu.au (A. Withall).

1525-8610/$ - see front matter Copyright � 2013 - American Medical Directors Associahttp://dx.doi.org/10.1016/j.jamda.2013.01.012

younger patients, although the proportion of familial, autosomaldominant cases is relatively low.6 There are strong associationsbetween motor or Parkinsonian disorders and familial dementias, andaccordingly, it is necessary when taking a family history in suspectedyounger-onset dementia cases to query across all domains of motor/neurologic, psychiatric, and cognitive functions. These associationsinclude relationships between motor neuron disease and fronto-temporal dementia and between spastic paraparesis and Alzheimerdisease.7,8 Heritability is a major concern of patients with younger-onset dementia and their family members. If genetic testing isdesired, as is often the case when the person is of childbearing age,then counseling should be provided first to mitigate any psycholog-ical effects.9

Younger-onset dementia is associated with high rates of behav-ioral and psychological symptoms, largely due to the types of prev-alent dementias. These behavioral disturbances are a leadingchallenge in providing long term care in younger-onset dementia.These symptoms also present a considerable burden to carers,supporters, and family members of people with younger-onsetdementia,10 and, as shown by Bakker et al11 in this issue, they area strong contributor toward earlier time to institutionalization.

Prevalence of Younger-Onset Dementia

Although there are few prevalence studies, recent work indicatesan increasing number of cases, with approximately 1 in 750 peopleaged 45 to 64 years affected.3 There also appears to be considerablegeographical diversity. The often-cited study by Harvey et al2 in theUnited Kingdom was the first broad prevalence study of younger-onset dementia. This work indicated a prevalence rate of 54.0/100,000 (95% confidence interval [CI] 45.1e64.1) in people aged 30 to64. The main subtypes were Alzheimer disease (34%), vasculardementia (18%), frontotemporal dementia (12%), and alcohol-relateddementia (10%). A further study from Japan12 revealed a lowerprevalence rate of 42.3/100,000 for the 20- to 64-year age band andvery different clinical subtypes: vascular dementia (42%), Alzheimerdisease (26%), dementia following head injury (7%), and Lewy bodydementia/Parkinson disease dementia (6%). A subsequent Australianstudy by our group3 yielded a much higher prevalence rate of 67.4/100,000 (95% CI 55.5e73.9) in people aged 30 to 64 years. Theprimary subtypes again differed: alcohol-related dementia (22%),Alzheimer disease (16%), frontotemporal dementia (13%), andvascular dementia (10%).

These prevalence studies, with their very different clinicalprofiles, emphasize the heterogeneity evident in younger-onset

tion, Inc.

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dementia. They also indicate that younger-onset dementia isperhaps more common than previously thought and that there area significant number of clients requiring long term care. Work iscurrently under way to provide further clarification on this issue.The Australian INSPIRED Study (Improving Service Provision forEarly Onset Dementia; www.inspiredstudy.org) will establish theprevalence and incidence of younger-onset dementia, as well asdescribe the clinical profiles, the experiences, needs, and burden ofthe patients, their supporters, and family members, and willdetermine models of optimal service provision for the differentdiagnostic groups.

Psychosocial Effects of Younger-Onset Dementia

Dementia can occur at any age. Younger-onset dementia typicallyaffects people in their prime of life, and, consequently, they haveyoung spouses and partners, may have children living at home, areoften still working and driving, and may have significant financialcommitments. The social effects of younger-onset dementia aregreat, and the psychosocial and financial burden outweighs thatseen in late-onset dementia.13 The stress of this unexpected diag-nosis has considerable effects on the person’s carers. Carers forpeople with younger-onset dementia comprise partners and chil-dren, as well as older parents, and they report higher levels ofburden than those experienced by carers of older patients.14,15 Thisis partly because of delays in obtaining a diagnosis of dementia,which is often because of the prominent behavioral presentation.The behavioral symptoms also cause increased carer stress10,15 andlead to disruption of family life.

There has been little research in younger-onset dementiaregarding effects on the family, particularly children, although anec-dotally the effects are great. Svanberg et al16 indicated a range ofeffects caused by the symptoms of the dementia, as well as lifestyleupheaval. These include adjusting to the person’s clinical features, tohis or her altered role in the family unit, and the changing powerdifferential as regards the need for assistance. Apathy is a particularlydifficult behavioral symptom for children to understand, particularlythe loss of empathy and emotional blunting. In Bakker et al’s article inthis issue,11 using results derived from the NEEDYD study (Needs inYoung onset Dementia), apathy predicted time to institutionalizationin younger-onset dementia. Once a person is admitted to long termcare, children can also find this environment particularly confrontingand need additional support to adjust to these changes.

Issues concerning intimacy and sexuality are also key in this po-pulation,17 and partners may need counseling to understand theirown emotions as their relationship evolves. These issues also presentdilemmas for staff in a care environment. Specialized trainingregarding the unique issues faced by their group is recommended forall staff working with people with younger-onset dementia.

Service Provision in Younger-Onset Dementia

People with younger-onset dementia struggle to find appropriatecommunity and residential services that are able to meet their uniqueneeds. Many services are couched in terms that do not seem relevantto younger people with dementia, such as “aged,” “older persons,”“frail,” and “disability,” and this presents a significant barrier toservice delivery. Even the prominence of the term “Alzheimer’s” canpresent a deterrent for people with other types of dementia.

One of the primary challenges in service delivery for younger-onset dementia is long term care, and younger people withdementia and their carers and supporters express significant diffi-culties as regards a lack of suitable care environments and issueswith accessibility because of being below the age threshold to

receive services.17 On one side, there is a desire to keep the patientat home as long as possible to maintain a “usual” family environ-ment. This is particularly an issue when there are younger childrenstill living at home with the patient. Family carers and supportersare also usually negative about placing the person in a care envi-ronment where they are surrounded by residents who are consid-erably older and much more frail. On the other side, however, arethe patient’s increasing behavioral symptoms, which increase bothcarer and family burden and often drive placement into residentialcare.11 Once the decision to access long term care is made, however,many carers/supporters of people with younger-onset dementia andtheir family members report significant difficulties in finding suit-able facilities.17

The combination of better physical function and mobility inconjunction with behavioral symptoms often seen in younger-onsetdementia makes for a challenging client. This is particularly evidentwhen surrounding residents are slower and more physically frail. Asa result, day and long term care facilities struggle to care for theseclients and, hence, are often reluctant to accept them into theirservice.18 Additionally, there are considerable cohort effects betweenclients in their 40s and 50s versus those older than 75. For example,women in these younger age groups are more likely to have receivedlonger periods of education and to have held lifelong careers. Youngerclients often request much more active activities, and differences arealso evident with respect to their preferences for music, movies, andother lifestyle choices. However, it must also be said that significantcohort effects also exist within the younger-onset dementia group,further complicating service delivery and indicating the importanceof person-centered care.

Unlike older people, where entering residential care is often seenas more of a medical necessity, for younger people this change ofresidence is bound with social issues that pertain to their muchyounger stage of life. The factors that seem crucial for good serviceprovision for this group include age-appropriateness, flexibility,having consumer input, and being individualized.11,19 Life historybooklets, such as the All About Me resource from the Alzheimer’sSociety, are useful for all clients with dementia regardless of theirage and care setting but have particular utility in younger-onsetdementia. These are invaluable clinical resources with respect todetermining client preferences, assisting with the planning ofactivities, and with smoothing the transition to respite and/or longterm residential care.

A useful model in guiding clinical services for younger-onsetdementia is considering the needs of the patient from 3 perspec-tives: cognitive and functional abilities, motor function, and behav-ioral and psychological disturbances. For example, patients with thebehavioral variant of frontotemporal dementia will have their needsweighted toward the behavioral signs domain, whereas a patientwith multiple sclerosis and dementia will tend to have more pro-minent motor signs. Understanding the clinical features of theperson’s dementia and any associated illnesses, asking the clientabout personal needs and preferences, as well as determining familysupports (if any) are crucial toward providing optimal services for thisgroup.

The Age Issue

Although this editorial has indicated some key differencesbetween older and younger clients with dementia, we mustremember that all clients, regardless of age, should be treated usingan individualized, person-centered approach. The threshold of 65years that is used to denote younger-onset dementia is servicesdriven rather than a response to any key clinical differences betweenthese age groups.1 As such, we must remember that people of all ages

Editorial / JAMDA 14 (2013) 230e232232

can be more or less physically active and can share similar interestsand the same sense of humor. Certainly, the modest prevalence ofyounger-onset dementia can make it difficult to establish age-appropriate services.

Whereas there are very good examples of age-specific units incountries such as the Netherlands and the United Kingdom, theseservices may not be feasible in countries such as Australia, wherethere is a relatively small population spread over a large geographicalregion. This means that services not only need to be tailored to theclient but also to the resources available. Carers, supporters, and otherfamily members also need to be consulted about their preferences, forexample with support groups, as they might prefer to be aroundother younger people with similar needs. Options such as onlinesupport groups or teleconferencing facilities might have utility inyounger-onset dementia. CANDID (Counselling And Diagnosis InDementia) is a national teleconference service for younger peoplewith dementia and their family members in the United Kingdom.20 Itis a nurse-led information and advice service that provides assistanceon individual needs and was rapidly accepted by carers for peoplewith younger-onset dementia. Further research is needed to deter-mine whether people’s preferences are for age-appropriate servicesor for a more person-centered approach, with people grouped byinterests and personality styles. Noneface-to-face services also needto be explored.

Future Directions

The diversity of case presentations, burden, needs, and familysupport systems in younger-onset dementia presents challenges forservice delivery and long term care. Often there has been a history ofdistress as the person with dementia has made a slow navigationthrough the health care system. This necessitates a case-by-caseconsideration of clinical features (including cognitive, motor, andbehavioral symptoms), needs (including personal preferences), andfamily situation (whether support is available, levels of burden anddistress, and whether there are young children involved). Large,ongoing studies, such as the Dutch NEEDYD study and the AustralianINSPIRED study, are vital in shedding light on the needs of this groupand their family members, as well as defining the challenges of careand establishing models for optimal service provision in younger-onset dementia.

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