mate and that the national prevalence may be over. 40 000.2 Comparatively, the estimated national preva- lence for all-age dementia is around 850 000 in 2015.
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Service provision for young-onset dementia in the UK Dane Rayment MRCPsych, Tarun Kuruvilla MRCPsych
In this review article, Drs Rayment and Kuruvilla look at the problems faced by younger people with dementia, the current state of care delivery in the country for young-onset dementia (YOD), guidelines for service provision and they suggest what could improve the availability of specialised YOD services in the UK.
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n 2003 it was estimated that there were over 18 000 people in the UK with YOD, or 67 cases per 100 000 people aged 30-64 years.1 A more recent study by the Alzheimer’s Society suggests this was an underestimate and that the national prevalence may be over 40 000.2 Comparatively, the estimated national prevalence for all-age dementia is around 850 000 in 2015 increasing to one million by 2025.3 The projected public health burden of dementia has led to increased awareness and governmentbacked drivers to increase diagnostic rates. Currently only around 44% of people in the UK with dementia get a diagnosis while they are alive.3 This unmet need is often called the ‘treatment gap’. This gap may be significantly wider for the 40 000 younger people with dementia. Younger people with dementia have complex health and social care needs. They are more likely to be working, physically robust and active, looking after children or elderly relatives, and paying off mortgages or other loans. Their partners often become their carers, further depleting household income. There are significant implications when someone is diagnosed with dementia as a younger person, which can often be very far reaching when compared with diagnosis in later life. The impact on patients and their families, and the needs of patients, can be very different from late-life dementia. Dementia can present differently in younger people. There is often a higher level of mood and behavioural problems. It can be difficult to distinguish dementia from depression in the early stages of YOD, particularly in frontotemporal dementia (FTD), which may present with apathy, irritability, dietary changes and deteriorating self-care.4 The prevalence rates of the dementia subtypes differ in younger people. While Alzheimer’s disease (AD) is still the most common subtype it accounts for only around a third of YOD, compared with around two-thirds in all-age dementia. 28
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Conversely, FTD, which accounts for only around 2% of all-age dementia, makes up around 12% of YOD.1 Other YOD subtypes include vascular dementia, alcohol-related cognitive impairment and various neurological and systemic diseases more frequently encountered as causes of dementia in the under 65 year olds. These include Parkinson’s disease, Huntington’s disease, multiple sclerosis, corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), and more rarely human immunodeficiency virus (HIV), and prion diseases like Creutzfeldt-Jakob disease (CJD). YOD has a higher genetic burden compared with late-life dementia, examples being the dominantlyinherited variants of AD, cerebral autosomal dominant arteriopathy with sub-cortical infarcts and leucoencephalopathy (CADASIL),5 which is a genetic form of vascular dementia, and FTD. Hence genetic counselling of relatives of people with YOD arises more frequently clinically than in older people. People with learning disabilities (LD) are more likely to develop YOD. Down’s syndrome is associated with young-onset AD due to over-expression of the amyloid precursor protein gene in trisomy of chromosome 21 leading to excess amyloid deposition.6 Again, people with LD who develop YOD have significantly different needs. This wide range of diseases causing the YOD syndrome can make differential diagnosis particularly difficult, so it should involve close collaboration between neurologists, psychiatrists, radiologists, other physicians, psychologists, occupational therapists (OT), speech and language therapists (SALT) and other members of a multidisciplinary team. Sampson et al.7 and Rosser et al.8 detail the assessment and diagnosis of YOD. Getting a diagnosis is often the key required to access support services. Once diagnosed, a person with YOD and their carers should receive a comprehensive care plan involving close collaboration between health ser vices, social ser vices, housing, www.progressnp.com
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work and pensions and the voluntary sector. Medical treatments may involve disease-specific licensed medication such as acetylcholinesterase inhibitors and memantine for AD9, thiamine supplementation for Wernicke-Korsakoff syndrome10 and antiretroviral combination therapy for HIV related cognitive impairment.11 It may also include off-licence but evidence-based medication like serotonergic antidepressants for the apathy commonly seen in FTD.12 Psychosocial interventions may include psychological management of behavioural symptoms, meaningful occupational activity, appropriate financial assistance, carer respite, counselling for young children and safeguarding vulnerable adults. Current state of care delivery Current service provision for people with YOD in the UK is highly variable. In a few regions there are dedicated multidisciplinar y specialist ser vices. However, in the majority of regions there is no dedicated service with a single point of access and no clear referral pathways. Consequently, people with suspected YOD are often unsure of how to access help and general practitioners are unsure who to refer such patients to, contributing to the wide treatment gap. Some people with YOD are seen in memory services, the majority of which are old age psychiatrybased in the UK. These ser vices may have practitioners skilled in the management of dementia in the elderly and who are competent at ruling out psychiatric disorders and there may be access to neuropsychology, OT and SALT thereby enabling good coordination of long-term care. However, the team may lack the expertise and resources to investigate for the broad range of neurological and systemic diseases that can cause the YOD syndrome. These investigations may include: structural neuroimaging with CT and MRI; functional neuroimaging with 99mTechnetium-hexamethylpropanolamine oxime single photon emission computed tomography (HMPAO-SPECT), fluorodeoxyglucose positron emission tomography (FDG-PET) and amyloid PET; cerebrospinal fluid (CSF) assay; electroencephalography (EEG); antibody titres; genetic assay, and other expensive tests. An audit of an old age psychiatry-based memory service by O’Kelly et al. showed that the yield rate of a diagnosis of dementia in younger referrals is very low and they argued that it may not be cost effective to investigate these increasing referrals.13 Some people with YOD are seen by neurologists who are proficient in neurological examinations and www.progressnp.com
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physical investigations but may lack expertise and resources for assessing mood and behaviour and in providing long term follow up after diagnosis. Other services where YOD patients are seen include general adult psychiatr y, liaison psychiatr y, neuropsychiatry, substance misuse, HIV, and LD services. The latter may be best placed to diagnose and manage YOD in people with LD as they are usually well known to the service. Existing ser vices based on informal arrangements without dedicated commissioning are not always able to build on sustained clinical experience and may not develop a good knowledge of support within the wider community. YOD patients and their carers report poor coordination between the different service providers, and highlight the problems of day care, respite ser vices and residential care being designed around the needs of older people. Some specialised YOD services may be at risk of being absorbed into old age psychiatry or even ‘ageless’ services in line with the current service reorganisation in the UK. Where specialised YOD ser vices are available, they are highly valued by patients and carers.14 Guidelines for service provision and sources of support The Alzheimer’s Society began campaigning to raise awareness of YOD in the 1990s. It published its ‘Charter for younger people with dementia and their carers’ which stated that all people with YOD, their families and carers should have access to specialist services from diagnosis to long term care.15 The Alzheimer’s Society and the Royal College of Psychiatrists published a joint policy document on YOD in 1999, recommending that local commissioning bodies appoint one named individual responsible for planning YOD services and one consultant acting as a focus for referrals. These two individuals would form the hub of the service, liaising with other people already involved in the provision of care for YOD, and developing ser vices appropriate to the region.16 The National Service Framework for Older People (2001)17 directed the National Health Service and councils to ‘review current arrangements, in primary care and elsewhere, for the management of dementia in younger people, and agree and implement a local protocol across primary care and specialist services, including social care’, but it did not make any specific recommendations. In 2006 NICE published its dementia clinical guideline, 18 which was more specific in its Progress in Neurology and Psychiatry July/August 2015
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recommendations that: ‘younger people with dementia have special requirements, and specialist multidisciplinar y ser vices should be developed, allied to existing dementia services, to meet their needs for assessment, diagnosis and care’. In 2013 NICE published guidance on commissioning dementia care,19 which reiterated the above, also advising a single point of access and a single care coordination system. The West Midlands regional forum for YOD has produced two strategic documents: commissioning guidelines for YOD dementia services 20 and an integrated care pathway for YOD.21 Alzheimer’s Society supports people with YOD at a regional level, offering practical information on dementia and services, and connecting patients and carers with others for mutual support. It has an online discussion forum – ‘Talking Point’. It also maintains a national database of services which, in 2004, showed a significant increase in service provision, particularly community support, day centres, and carer groups. However, much more is needed, with respite and residential care remaining in short supply.22 YoungDementia UK, formed in 1998, also keeps a record of ser vices in different regions, runs forums and networks, and provides community services. The organisation commissions groups and research, and its sister charity, YoungDementia UK Homes, helps establish suitable specialist residential support.23 National support groups for rarer causes of YOD have emerged, including the Pick’s disease support group, the CJD support network and the PSP association. National conferences such as the recent ‘A life worth living: YOD services and support’ are useful for clinicians and commissioners involved in planning services.24 Despite national guidelines, there is wide regional variation in ser vice provision for YOD. Involving patients and carers at all stages of service development, along with persistent, committed pressure from patients and carers and other organisations, will help in the on-going development of dedicated YOD services and prioritisation of funding for YOD ser vices, especially in the current economic climate. Dr Rayment is a Specialist Registrar in General and Older Adult Psychiatry in the Department of Liaison Psychiatry at Bristol Royal Infirmary and Dr Kuruvilla is a Consultant Psychiatrist in Old Age Psychiatry, at the 2gether NHS Foundation Trust, Cheltenham. 30
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Declaration of interests No conflicts of interest were declared. References 1. Harvey RJ, Skelton-Robinson M, Rossor MN. The prevalence and causes of dementia in people under the age of 65 years. J Neurol Neurosurgery Psychiatry 2003;74:1206–09. 2. Prince M, Knapp M, Guerchet M, et al. Dementia UK: overview 2014. 3. Alzheimer’s Society. (2015) Dementia Diagnosis Rates . alzheimers.org.uk/site/scripts/documents_info.php?documentID=2165 (accessed January 2015). 4. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134(9):2456–77. 5. Chabriat H, Joutel A, Dichgans M, et al. Cadasil. Lancet Neurol 2009;8(7):643–53. 6. Holland AJ, Hon J, Huppert FA, et al. Incidence and course of dementia in people with Down's syndrome: findings from a population‐based study. J Intellect Disabil Res 2000;44(2):138–46. 7. Sampson EL, Warren JD, Rossor MN. Young onset dementia. Postgrad Med J 2004;80:125–39. 8. Rossor MN, Fox NC, Mummery CJ, et al. The diagnosis of young-onset dementia. Lancet Neurol 2010;9(8):973-806. 9. National Institute for Health and Care Excellence. Donepezil, galantamine, rivastigmine and memantine for the treatment of Alzheimer’s disease NICE technology appraisal guidance [TA217]. London: National Institute for Health and Care Excellence 2011. 10. Day E, Bentham PW, Callaghan R, et al. Thiamine for prevention and treatment of Wernicke‐Korsakoff Syndrome in people who abuse alcohol. Cochrane Database Syst Rev. 2013 Jul 1;7:CD004033. doi: 10.1002/14651858.CD004033.pub3. 11. Cysique LA, Vaida F, Letendre S, et al. Dynamics of cognitive change in impaired HIV-positive patients initiating antiretroviral therapy. Neurology 2009;73(5):342-8. 12. Nardell M, Tampi RR. Pharmacological treatments for frontotemporal dementias: a systematic review of randomized controlled trials. Am J Alzheimer's Dis Other Demen 2014;29(2):123–32. 13. O’Kelly A, Richards G, Gorospe MF. An audit of younger patient referrals to a memory clinic. Progress in Neurology and Psychiatry 2015;19(2):21–5. 14. Alzheimer’s Society. Younger people with dementia: A guide to service development and provision. 2005. 15. Ferran J, Wilson K, Doran M, et al. The early onset dementias: a study of clinical characteristics and service use. Int J Geriatr Psychiatry 1996;11:863–9. 16. Royal College of Psychiatrists and Alzheimer’s Society London. Services for younger people with Alzheimer’s disease and other dementias. Council Report CR135; 2006. 17. Department of Health. National Service Framework for Older People 2001. 18. National Institute for Health and Care Excellence. Dementia: Supporting people with dementia and their carers in health and social care NICE Clinical Guideline 42 [CG42]. London: National Institute for Health and Care Excellence 2006. 19. National Institute for Health and Care Excellence. Support for commissioning dementia care NICE Commissioning Guide 48 [CMG48]. London: National Institute for Health and Care Excellence 2013. 20. Elliott T, Read K. Bridging the gap. J Dementia Care 2002;10:15. 21 Saad K. Integrated care pathways for young onset dementia. J Dementia Care 2004;12:29–31. 22. Alzheimer’s Society. Younger people with dementia. alzheimers.org.uk/site/scripts/documents_info.php?documentID=164 (accessed January 2015). 23. YoungDementia UK Homes. youngdementiaukhomes.org/ (accessed January 2015). 24. YoungDementia UK. YoungDementia 2014 presentations. youngdementiauk.org/young-dementia-2014-presentations (accessed January 2015).
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