Early Onset Dementia From the background to the foreground

Transcription

1 Early Onset Dementia From the background to the foreground Dr Jeremy Isaacs Consultant Neurologist St George s Hospital Excellence in specialist and community healthcare

2 Themes of my talk The early onset dementia clinical construct is different from late life dementia The needs of people affected by EOD are different to those with late life dementia So diagnosing and managing it requires some different skills Cognitive neurologists and memory clinics can work collaboratively to provide excellent care

3 The dementias are associated with ageing : : Matthews et al, Lancet 2013

4 There are about 850,000 people with dementia in the UK Of these about 65,000 have earlyonset dementia Are the needs of this 7% different from the older 93%?

5 Issues in young onset dementia Diagnostic complexity Delays in diagnosis Years of life lost to illness Interruption of mid-life plans Early retirement Cessation of childcare responsibilities Financial repercussions Rapid progression Behavioural symptoms in the physically well Lack of age-appropriate facilities Effects on spouses Effects on young adult children Effects on elderly parents Stigma

6 Incidence of early onset dementia Only high quality study performed in Cambridgeshire in mid-2000s Mercy et al, Neurology 2008 Incidence of dementia in age group 11.5 cases per 100,000 person-years Alzheimer s disease 4.2 Frontotemporal dementia 3.5 Huntington s disease 0.8 Extrapolated across England and Wales 460 new cases of FTD and 550 new cases of Alzheimer s each year Each memory clinic will only see a handful of younger people with dementia each year

7 Clinical issues in EOD The casemix in younger people in a memory or cognitive clinic is different Worried well Psychiatric illness presenting with memory symptoms Alcohol and drug misuse A larger range of organic causes of cognitive impairment Overlap with neurological disorders

8 Causes of dementia <65 Causes of dementia > 65 Kester M I, Scheltens P Pract Neurol 2009;9: Dementia UK 2 nd edition 2014

9 Clinical issues in EOD Atypical forms of Alzheimer s common Posterior cortical atrophy Biparietal AD Logopaenic aphasia Frontotemporal dementia common Behavioural variant Semantic dementia Progressive non-fluent aphasia Vascular dementia rare

10 Clinical issues in EOD Involvement of non-mnestic cognitive domains Language Praxis Visuospatial Executive

11 Clinical issues in EOD Investigating younger patients is (rightly) resource intensive MRI preferable to CT in younger people may need repeat imaging subtle changes used to rule in diagnoses, not just rule out mimics Imaging needs to be reported by specialist neuroradiologists Standard imaging (CT/MRI) may be normal Greater reliance on neuropsychology Role for functional and biomarker-based diagnostics e.g. FDG-PET, CSF tau and Abeta

12 Clinical issues in EOD Genetic forms of dementia commoner in young onset cases Alzheimer s: PS1, APP, PS2 FTD: C9ORF72, MAPT, GRN, VCP Vascular: Notch3 (CADASIL) When to test? Which genes in which order? How to counsel patients and worried relatives who accompany them to clinic?

13 Clinical issues in EOD Often well informed and request additional treatment Younger patients can tolerate higher doses of CEIs and dual treatment More likely to enrol in clinical trials

14 Costs of dementia

15 How do people live well with EOD? Spouses do extraordinary things to support the person with the condition Bring the resourcefulness of their generation to the situation Young adult children often heavily involved Emergence of BPSD can precipitate a crisis Some options for personal home care exist Residential care options are extremely limited

16 Do we need EOD hubs? Incidence and prevalence of EOD in individual boroughs/ccgs insufficient to create viable dedicated services Younger people seen alongside older people by memory clinics and social care No systematic evidence that memory clinics don t offer a good service to younger patients But in general you get good at something you do regularly

17 Regional neuroscience centres are expected to provide cognitive services Provide a ready-made option for younger people to get access to additional expertise Travel further from home less of an issue for younger people

18 St George s cognitive neurology service Diagnosis and treatment of cognitive disorders including dementia Weekly consultant-led cognitive neurology clinic at SGH New patient slots one hour Post-diagnosis follow-up is nurse-led Jen Tulloch, dementia clinical nurse specialist Dedicated neuropsychologist Claire O Neill, Senior Clinical Neuropsychologist

19 St George s cognitive neurology service We do not use strict referral criteria, but we tend to see: Younger patients Complex or atypical presentations We run a support group for people with early onset dementia clinical trials in Alzheimer s disease and other dementias

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21 St George s Early Onset Dementia support group Launched 2011 Mailing list reaches > 100 people Evening meetings at St George s Hospital Supported by grants from HEE and St George s Hospital charity Allowed us to commission the meetings from Dementia Pathfinders CIC Shift from didactic to interactive/experiential content Increased frequency of meetings, now every 6 weeks

22 youngdementiasupport.london Website for people affected by EOD Funded by grant from HESL Information of specific relevance to EOD Not designed to replicate excellent web-based resources elsewhere Will promote activities of our support group

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25 Summary Early onset dementia is a small proportion of the total But EOD presents particular challenges to clinicians, patients, carers and health and social care systems

26 Summary We don t know what the best system for diagnosis and support of EOD is At St George s we have been on a journey since 2010 to develop clinical expertise and dedicated support for people with EOD We encourage others in SW London to join us by sharing patients, challenges and ideas

27 Questions?