Dr Roger Cable NHS Dumfries and Galloway

Transcription

1 Dr Roger Cable NHS Dumfries and Galloway

2 Young Onset Dementia Statistics What is dementia Diagnosing dementia History Examination Tests Subtypes of dementia

3 315000

4 Cost of dementia (Alzheimer s society) The overall economic impact of dementia in the UK is 26.3 billion. This works out at an average annual cost of 32,250 per person. This consists of: 4.3 billion of healthcare costs 10.3 billion of social care of which: 4.5 billion spent on publically-funded social care 5.8 billion spent on privately-funded social care 11.6 billion of unpaid care 111 million on other dementia costs

5 Statistics (Alzheimer s society) UK: people with dementia Scotland Two thirds are women Proportion of people with dementia doubles for every 5 year age group One in six people over 80 have dementia 80 percent of people in care homes have dementia Two thirds of people with dementia live in the community Only 44% of people with dementia have a diagnosis Worldwide estimated to affect 35.7 million people

6 Young Onset Dementia Stats with young onset dementia in the UK 3200 with young onset dementia in Scotland Harvey et al prevalence studied in the late 90 s London boroughs, but replicated Age group 30 to 65 (54 per ) Age group 45 to 65 (98 per )

7 What is dementia: according to WHO Dementia is a syndrome usually of a chronic or progressive nature in which there is deterioration in cognitive function (i.e. the ability to process thought) beyond what might be expected from normal ageing. It affects memory, thinking, orientation, comprehension, calculation, learning capacity, language, and judgement. Consciousness is not affected. The impairment in cognitive function is commonly accompanied, and occasionally preceded, by deterioration in emotional control, social behaviour, or motivation.

8 Challenges of current criteria for diagnosis of dementia Need for social and occupational function impairment Problematic for early diagnosis May become more relevant with treatment advances (disease modifying drugs) Need for memory impairment Not all dementias present with memory impairment Examples include semantic dementia, posterior cortical atrophy

9 Young onset dementia (YOD) Dementia presenting before the age of 65 Arbitrary cut-off, more a sociological partition Related to traditional retirement age No clinical reason for this cut-off Age is the biggest risk factor for dementia Rare under the age of 65 Loss of income generating ability Carer loss of employment and negative psychological effects Young carers and children Implications regarding heritability

10 How is YOD different to late onset Wider differential (up to 1/3 unusual conditions) Men over represented in clinics (USA) Less co-morbidity Aggressive course Working age (breadwinner) Higher burden of genetic disease Research Implications for offspring Needs of person and family differ from older people Services often not available for this age group

11 Diagnosing dementia (timely and accurate diagnosis) Structured approach required Multidisciplinary approach includes Nurses Psychologist Neurologist Occupational therapist Psychiatrist Aggressive pursuit of aetiology

12 Structured approach Clinical diagnosis; Definitive diagnosis possible (PM) History and mental state suggestive of dementia: Evaluate for reversible/treatable causes Rapidly progressive course: Investigate for inflamatory, infectious, prion, paraneoplastic (Blood, CSF) Family history: (Genetic testing) Neuropsychological testing CT/MRI

13 Taking a history General medical history Psychiatric history Cognition (including memory) Onset Progression Fluctuation Cognitive profile Family history Functional assessment (ADL and IADL) Collateral history (anosagnosia common)

14 General medical and neurological history Medical and neurological symptoms Medical history to include: Malignancy, autoimmune disease, Diabetes cardiomyopathy, conduction deficit Further Inquiry: Head injury, Seizures, hearing loss, visual impairment, weight loss, change in bowl habit. Substance misuse, alcohol history Travel Heavy metal exposure, solvents or inhalers

15 Psychiatric history Cognitive deficits commonly found in many mental disorders Depressive pseudo-dementia Schizophrenia Bipolar Exclude malingering Personality disorder

16 Cognitive assessment People with dementia do not always have memory loss Cognitive profile is more important Start with simple bedside screening tests MOCA, ACEIII, FAB Interpretation critical Often require neuropsychiatric evaluation from psychologist Functional assessment from occupational therapist

17 Functional assessment Functional impairment required for somebody to meet criteria for diagnosis Deterioration from pre-morbid levels Educational achievement and occupational functioning History: changes in activities and abilities Collateral history Occupational therapy assessment Instrumental activities of daily living affected earlier (more complex tasks such as managing finances transport ect and later ADLs)

18 Physical and neurological examination Helps with differential diagnosis Identifies people who require further neurological investigation Helps identify specific conditions (Upward gaze palsy in PSP) Helps to identify mimics of dementia or other conditions that can cause cognitive impairment Helps to ensure that meds can be prescribed safely

19 Blood tests Basic Screen FBC U&E LFT Lipid profile B12 and Folate TFT More tests according to clinical indications

20 Neuroimmaging CT helpful to exclude space occupying lesions or whole body scan for tumour identification. MRI can be more useful Identify inflammatory changes (MS, Limbic encephalitis, vasculitis) Pulvinar sign in variant CJD Lobar atrophy (different patterns aid diagnosis) Abscess Tumour (Primary vs metastatic) Hydrocephalus FPCIT-DAT Scan Perfusion SPECT

21 Dementia subtypes Dementia of Alzheimer's type Vascular dementia Fronto-temporal dementia Lewy body disease / Parkinson's disease dementia Reversible causes of dementia Rapidly progressive dementia Other examples

22 Dementia of Alzheimer s type Initially described in a 51 year old by Alios Alzheimer Histopathologically characterised by plaques and tangles Typically presents with insidious onset of prominent episodic memory impairment, eventually involving executive function and visuo-spatial function Autosomal dominant familial Alzheimer's typically have mutations in: APP (Amyloid precursor protein) trisomy 21 cause of increase incidence in Downs syndrome PSEN 1 & 2 (Presenilin) Sporadic Alzheimer s is rare before 50

23 Familial Alzheimer's Contrary to sporadic Alzheimer s: Generally have myoclonus Relative preservation of naming Prominent speech production deficits Rarely with PSEN 1 deletions, patients can present with paraparesis (not seen in late onset disease)

24 Young onset sporadic Alzheimer's, non-amnestic phenotypic variants One third of those under 65 compared to 5% of over 65 s Posterior cortical atrophy: Biparietal or posterior biparieto-occipital dysfunction Difficulties in locating and perceiving objects Often many appointments with optician/opthalmologist Logopenic progressive aphasia More aggressive course Prolonged word finding pauses, anomia and impaired sentence processing

25 Vascular Dementia Cerebro-vascular disease needs to be demonstrated and linked to progressive cognitive decline Episodic memory loss less prominent Cognitive symptoms depend on the location of the cerebral pathology Classical description of stepwise decline with focal signs and symptoms rarely seen Sub cortical presentation (cognitive slowing), executive dysfunction commonly seen Stroke related stable cognitive deficits also seen

26 Vascular causes of rapidly progressive cognitive impairment Subdural haematoma CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) Family history of stroke and dementia History of recurrent stroke and migraine Vasculitis (Treatable with cyclophosphamide and steriods) MRI angiography ( beading and narrowing of blood vessels

27 Fronto-temporal dementia Strong association with Motor Neuron Disease Descriptive term that refers to regional atrophy Three classical subtypes Behavioural variant Semantic dementia (Fluent aphasia with loss of word meaning) Progressive non-fluent aphasia (Effortful non-fluent speech) 20% -40 % Heritable Mr G enquiry MWC

28 Lewy body dementia Core symptoms: Spontaneous well formed visual hallucinations Fluctuations in cognition Parkinsonism (in contrast to Parkinson's disease) More symmetrical Absence of rest tremor Poor response to dopaminergic drugs Cognitive profile: Frontal/ parietal Disproportionate visiospatial dysfunction Prone to REM sleep behaviour disorder

29 Parkinson's disease dementia Dementia increasingly recognised as a feature of Parkinson's disease Dementia develops less frequently and with longer latency with younger onset Parkinson's disease (More likely genetic ) PARK2 gene not typically associated with dementia

30 Reversible causes of dementia Limbic encephalitis Infections HIV, Syphilis, Whipple s disease, lymes disease Toxins Alcohol, heroin, arsenic, mercury drugs (Example lithium, bismuth, methotrexate) Medical Thyroid, B12, Folate, sleep apnoea, subdural haematoma, Transient epileptic amnesia

31 Rapidly progressive dementia Requires urgent comprehensive investigation to exclude reversible causes. Definition varies: Death or severe dementia no later than 18 months to 4 years Causes include: Autoimmune Vascular (CADASIL) Infectious (Untreated HIV, Viral- herpes Neoplastic Toxic

32 Autoimmune causes of dementia Limbic Encephalitis Sub-acute memory loss/ executive dysfunction Psychiatric disturbances Irritability Depression Hallucinations Personality change Temporal lobe epilepsy Other possible symptoms Myoclonus, Ataxia, peripheral neuropathy

33 Neural antibodies Two categories Intracellular antigens Often paraneoplastic Markers for T cells (CD8 and cytotoxic T cells) Removal of cancer stops or slows progression but not reversible Neural cell surface proteins or receptors Antibodies cause functional impairment but not damage to the same extent and therefore often reversible Example VGKC (Voltage Gated potassium channels) 20 30% cancer association Hypothalamic involvement can result in SIAHD

34 Diagnosis of limbic encephalitis Autoantibody testing Profile can suggest the most likely cancer Example: VGKC Breast, Prostrate, Small cell lung NMDA Ovarian teratoma Neuroimaging MRI T2 and FLAIR hyperintesities in medial temporal lobe

35 Normal pressure hydrocephalus Classic symptom triad described by Hakim and Adams in 1965 Shuffling Gait (magnetic or glue footed gait) Urinary incontinence (frequency, urgency, nocturia) Dementia (Subcortical/frontal, language preserved) Chronic, communicating, near normal intracranial pressure with elevated CSF pulse pressure Idiopathic or secondary to: Intracranial haemorrhage, meningitis, head trauma Treatment: Shunt

36 Chronic traumatic encephalopathy Dementia pugilistica Repeated trauma to brain or single traumatic injury Important in American football Symptoms of memory loss, aggression, depression May occur many years after the trauma

37 References: The diagnosis of young onset dementia: Lancet neurology 2010;9: Neurology in practice; Dementia Joseph F Quinn Adams and victors principles of Neurology Lishman s: Organic psychiatry Cognitive assessment for clinicians, John Hodges