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Supporting people with young onset dementia in primary care

Supporting people with young onset dementia in primary care

Key learning points:

– Young onset dementia is rare, and in a GP practice with a list of 7,000 there would only be one-two people with the condition

– It differs substantially from late onset dementia in its presentation and impact on the person and their family

– Primary care staff are well positioned to suspect early dementia, and signpost to appropriate financial, emotional support for the patient and their family

Young onset dementia (YOD) starts under the age of 65 years and is also referred to as early onset or working age dementia. Just 2% of cases of dementia occur under the age of 65 years, and it is estimated that over 42,000 people in the UK live with YOD. (1) This means the condition is both rare and atypical, giving rise to a number of issues. Its rarity impacts on recognition, diagnosis and availability of services; and its onset during middle age means there are life stage differences, as well as differences in the impact of YOD on family life compared with late onset dementia. YOD includes a different distribution of types of dementia compared with late onset, including higher proportions of several rarer types that have distinctive symptom profiles and give rise to particular issues that require support and services different from those suitable for people with Alzheimer’s disease. An overview of some of the issues related to YOD that may be of particular relevance to staff in primary care will be explained. Nurses working in primary care settings may be well positioned to respond to people with YOD and their families. Therefore, it is important to be informed of the nature of the condition, its potential impact and sources of support.

The prevalence and nature of young onset dementia

Dementia is an umbrella term that covers a range of conditions all of which involve impairment in cognition and behaviour. The diagnostic criteria have been revised in recent years in the light of advances in understanding. The US Institute on Ageing and Alzheimer’s Association define the symptoms as changes in cognition or behaviour that affect at least two domains out of five possible areas: memory, reasoning, language, visuospatial functioning, and personality. The changes need to be severe enough to interfere with ability to function at work or in everyday activities, represent a decline from previous levels, which not be explained by delirium or a major psychiatric illness. (2) These criteria are the same whether dementia is of young or late onset.

Although the age that defines YOD is a cut off of convenience, the profile of types of dementia differs in the younger age group. In late onset dementia, Alzheimer’s disease (AD) is by far the most common type accounting for 80% of all cases. It is also the most common of young onset dementias, however, it only accounts for 34%. Eighteen percent of people with YOD have a diagnosis of vascular dementia, 12% have dementias related to frontal-temporal lobe degeneration (FTLD), 10% have alcohol related dementias, 7% have dementia with Lewy bodies, and 19% have other rarer types. (3)

The prevalence of YOD is debatable. The best UK estimate from research suggests that among people aged 45-65 years, 98 in every 100,000 are affected. (3) However, a more recent study that based estimates on consensus opinion from experts in the field produced higher estimates. (1) Using the survey based figures, in a GP practice with a list size of 7,000 people just one or two people would be expected to have YOD, and even if the higher rates are accepted this rises to only two-five people.

Delay in diagnosis

Due to its rarity, YOD is unexpected. A person who starts to find it hard to manage at work in his or her 40s or 50s may blame problems on stress, burnout or changes in the work place but seldom would they imagine their difficulties are due to the cognitive changes of dementia. Similarly, if a spouse or partner notices that the person is less empathic or less organised than usual, he or she will be more likely to blame it on mood or relationship problems than on a health condition. There may therefore be a considerable delay before the person approaches their doctor. (4) When the person does attend the primary care practice, the GP is likely to refer them to exclude possibilities that are more common at this age, including focal brain problems such as tumours, psychiatric problems such as anxiety or depression, or sensory problems such as hearing, balance or sight related conditions. Thus it may take years for someone eventually to receive a diagnosis of YOD.

This lengthy process has a number of consequences. Having a diagnosis often brings relief after a lengthy period of uncertainty. It allows re-interpretation, with hindsight, of the changes in behaviour of the person with dementia. However, this may also lead to the partner feeling guilt for his or her lack of understanding earlier on, and for some the marital relationship, which would normally have been relied upon for support, has already become strained or even broken down completely. The person and their relative may be resentful and angry at the delay. As a primary care practitioner, it is important to be able to respond sympathetically to this, giving accurate information about how dementia is a diagnosis of exclusion and offering post-diagnostic support and understanding.

Atypical presentation

A further issue around diagnosis arises from the way dementia presents itself in YOD. The typical presentation in late onset dementia, where Alzheimer’s disease is by far the most common type, is of memory problems. Indeed, dementia assessment clinics are often named memory assessment clinics. However, in YOD, it is far less common for the presenting issue to be one of memory difficulties. In the third of cases where a person with YOD has Alzheimer’s disease, one-in-three will have visual difficulties associated with posterior cortical atrophy (PCA) (5) rather than early memory problems. PCA, as its name suggests, affects the back of the cortex of the brain, particularly the occipital lobe, and hence its impact on visual functioning. People with PCA therefore experience problems related to sight, such as finding it hard to locate or recognise objects. They may have made repeated visits to their opticians but not found new glasses to make any difference.

People with dementia related to fronto-temporal lobar degeneration (FTLD) also have different early difficulties, with the clinical presentation dependent on the areas of the brain that are initially affected. Behavioural variant fronto-temporal dementia (bvFTD) presents with changes in the person’s behaviour and ability to carry out complex tasks. (6) The behavioural changes include social dis-inhibition, whereby the individual may not assert a usual degree of control over what is socially acceptable, and lack of empathy for others. So, for example, the person may act on aggressive, sexual or appetitive instincts by swearing, touching others, or eating lots of sweet foods; and they may appear selfish or unconcerned about others around them. Changes in managing complex tasks may be shown up in them becoming apathetic (as they cannot get started on an activity), getting stuck in a groove of the activity (repeating one aspect in a ritualised way) or not planning ahead sufficiently. The latter can lead people into difficult situations such as running out of petrol in the car, getting locked out of their home, or even picking up a hot dish without thinking to use an oven glove. Other variants of FTLD show up first in changes in language. These are collectively known as primary progressive aphasias. (7) In the semantic variant, the person has an intact memory and fluent speech but has trouble with finding the meaning of words; in the non-fluent agrammatic variant, the person has very effortful production of speech; and in the logopenic version the individual has problems in finding single words.

As these unusual early features do not coincide with descriptions of dementia as memory loss, they can act as a further smokescreen to delay detection of dementia. It is worth primary care practitioners being aware of the different types of YOD, and the early symptoms of each, as practice nurses may be alerted to such information during appointments held for other reasons, including menopause clinics, or health screenings.


A third issue, that is pertinent for primary care teams to be aware of, is around inheritance of risk for dementia. As YOD affects people in mid-life rather than old age, most adults will wish to know if their children are going to be at risk when they grow up. Also because of the age of onset, many people who develop YOD have children or young people in the family home, or around student age. These young people witness the changes brought about by dementia and are often involved in care, so they also may have concerns about whether they will develop the condition. The genetic risk varies with the type of YOD, with FTLD carrying the greatest risk. (8) About a third of people with dementia due to FTLD have had relatives with a similar illness. For those with AD, about one-in-1,000 people with AD have a known genetic cause but YOD is more likely to run in the family than late onset. Primary care staff are in a good position to ascertain whether family members have worries about inheritance. The Alzheimer’s Society has a good leaflet on this aspect, which families can be directed to.

Practical and emotional consequences

Dementia at any age is a feared condition, but for those who are younger it has far-reaching effects. (9) This is partly because of the stage of family life at which it occurs. Families often have a generation above the person with YOD, as well as a dependent generation below. Both older family members and young people may step into providing some support or care for the person with dementia, but also they may need support in their own right. This can mean that the well parent, in a conventional family, is over-stretched to provide care.

On a practical level, a family in which there is YOD may have on-going financial commitments to support children and to make mortgage or loan payments. Yet dementia will inevitably lead to loss of employment, for the person themselves, and also at some point, the spouse or partner may need to reduce their working hours to provide sufficient support. Having accurate information about financial support for dementia and for carers, and having information about powers of attorney therefore is very important for people with YOD.

On an emotional and cognitive level, having dementia presents challenges to identity, as we all like to think we are competent to manage our own lives, so to be advised that the brain is not working and that cognitive functions will get progressively worse is hard to adjust to. It may also be a difficult condition to own up to with others as it carries stigma and shame. In recent years, a number of people with YOD have spoken out about how they live with the condition and this is giving a powerful message that it is possible to live well. People with newly diagnosed YOD may find it helpful to see the accounts of others and to meet up with other people who live with YOD, either face-to-face or on-line. A good source of information and mutual support is YoungDementia UK.

Age appropriate services

As YOD is rare, and most dementia is related to old age, it can be very hard to locate age-appropriate services. Many people with YOD and their relatives talk about this as a key issue both while living at home and needing support, and if the occasion arises that the person needs to move into care. Most people in middle age have grown up and lived with different experiences from those who are in their old age, and so they may have differing generational attitudes, likes and dislikes. In addition, many people with late onset dementia who are living in care home settings are quite physically frail, whereas people with YOD may be fit and wish to be active.

It is worth primary care centres finding out about local and regional community and residential service that provide for YOD so they can direct families to appropriate sources of support.


YOD is rare, and in most GP practices there would only be a very small number of people with the condition. When YOD occurs it is often initially unrecognised, so it is worth primary care nursing staff being aware of the distinctive ways in which it presents in case they come across it in the course of other consultations. YOD has far-reaching consequences for the person with the condition and is also a family issue with the well partner and children being affected. Primary care staff may be well positioned to listen out for their needs, and to signpost on to appropriate financial, emotional and peer support.


Alzheimer’s Society



1. Prince M, Knapp M, Guerchet M, McCrone P, Prina M, Comas-Herrera A, et al. Dementia UK Update. London: Alzheimer’s Society; 2014.

2. McKhann G, Knopman D, Chertkow H, Hyman B, Jack C, Kawas C, et al. The diagnosis of dementia due to Alzheimer’s disease: Recommendations from the National Institure on Aging – Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease.’ Alzheimer’s & Dementia: The Journal of the Alzheimer’s Association 2011; 7:263–269.

3. Harvey RJ, Skelton-Robinson M, Rossor MN. The prevalence and causes of dementia in people under the age of 65 years, Journal of Neurology, Neurosurgery & Psychiatry. 2003; 74: 1206–1209.

4. Oyebode J, Bradley P, & Allen J. (2012) Relatives’ Experiences of Frontal-variant Frontotemporal Dementia. Qualitative Health Research E-publication ahead of print, doi: 10.1177/1049732312466294

5. Crutch S, Lehmann M, Schott J, Rabinovici G, Rossor M, Fox N. Posterior cortical atrophy. Lancet Neurology. 2012; 11(2):170-8.

6. Graham A, Hodges J. Frontotemporal dementia. Psychiatry, 2005; 4: 55-58.

7. Gorno-Tempini M, Hillis A, Weintraub S, Kertesz A, et al. Classification of primary progressive aphasia and its variants, Neurology 2011; 76:1006-14

8. Sampson E, Warren J, Rossor M. Young onset dementia. Postgraduate Medical Journal, 2004, 80: 125-139

9. Van Vliet D, De Vugt M, Bakker C, Koopmans R, Verhey, F. Impact of early onset dementia on caregivers: A review. International Journal Geriatric Psychiatry 2010; 25:1091-1100.

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