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Understanding Dementia Dr Asso Fariadoon Ali Amin MRCP(UK) Why is Dementia Important? Dementia is an acquired decline in memory and other cognitive function (s) in an alert non delirious person that is sufficiently severe to affect daily life ( home, work, or social function). There are about 820,000 people in the UK with dementia The number is set to double by 2030 Prevalence:- rare before the age of 65 , increase with age , 65-69 (1.4%), 70-74 ( 2.8%), 75-79 ( 5.6%), 80-84 (11.1%), more than 85 (23.6%) There are about 18,500 people in the UK under 65 who have dementia There is no cure Prevalence of Alzheimer’s Disease Prevalence of Alzheimer’s disease in an aging population. Prevalence increases dramatically with age and approaches 50% of those over 85 years old. (Adapted from Evans et al., 1989.) Dementia - Diagnosis Diagnosis ICD-10 & DSM-IV: Multiple cognitive defects which must include: Amnesia Functional impairment Clear consciousness Clear change from previous level Long duration (>6 months) Forms of Dementia Alzheimer’s disease Vascular Dementia Dementia in Parkinson’s & Dementia with Lewy Bodies Frontotemporal Dementia Reversible (<5%):- Subdural haematoma, normal pressure hydrocephalus, metabolic, drugs Neurological dementias: Cerebral Vasculitis Corticobasal Degeneration Dementia in MS HIV/AIDS Dementia Huntington’s Dementia Lysosomal storage diseases Prion Diseases – CJD Prevalence of the forms of dementia Cause Percentage Alzheimer’s disease 55% Vascular dementia 20% Dementia with Lewy Bodies 15% Frontotemporal dementia 5% Rarer causes (all) 5% Clinical Diagnosis • • History:- Take a careful history from the patient and the relative, concentrate mainly on the onset and progression of symptoms, , take careful drug history, social history. Deterioration of cognitive function is slow in Alzheimer disease within years , faster in vascular dementia, and very rapid in reversible like metabolic causes. Deterioration occurs in :retention of new information like appointments, events, or working a new household appliance) Managing complex tasks e.g. Paying bills , cooking a meal) Language ( word finding difficulty) Behaviour ;- become aggressive, irritability, poor motivation and wandering) orientation getting lost in familiar places. recognition:- failure to recognise people Ability to self care :- bathing , dressing. Reasoning:- poor judgement Alzheimer’s – Diagnosis Fulfil criteria for dementia syndrome Insidious onset Gradual progression No focal neurological signs No evidence for a systemic or brain disease sufficient to cause dementia Alzheimer’s Diagnosis DSM IV The development of multiple cognitive deficits manifested by both: 1. Memory impairment and: 2. One or more of the following cognitive disturbances: a) Aphasia b) Apraxia c) Agnosia d) Disturbed executive function Alzheimer’s Diagnosis DSM IV 1) 2) 3) The cognitive impairments above lead to significant impairment in social or occupational functioning & are a decline from a previous level The course is gradual in onset & shows continuous decline The cognitive impairments are not due to: Other CNS conditions that cause progressive deficits in memory & cognition Systemic conditions that cause dementia Substance induced conditions The deficits do not occur during the course of delirium Alzheimer’s - features Cognitive symptoms Amnesia – early features are impaired new learning & recall, disorientation in time & place, late features include impaired semantic memory & visuospatial memory Aphasia (dysphasia) – deficits in cortical language function – early features are nominal aphasia, verbal perseveration, late features include mutism & echolalia Apraxia (dyspraxia) – common forms are: ideomotor dyspraxia (cannot carry out motor function to command), constructional dyspraxia (manifested by inability to copy intersecting pentagons or draw a clockface) Cognitive Features Agnosia especially visual agnosia (inability to recognise objects) & prosopagnosia (inability to recognise faces) Frontal-executive dysfunction – inflexible (concrete thinking). Difficulties with problem solving or planning, difficulty correctly sequencing behaviour. Dyslexia Dysgraphia Acalculia R/L disorientation Non-cognitive symptoms Psychotic: Delusions often paranoid Hallucinations: commonly visual Mood: Depression Anxiety Euphoria Behavioural: Apathy Over activity Aggression Non-cognitive symptoms Neurovegetative Symptoms: Sleep disturbance, day-night reversal in about 30% patients Eating: poor oral intake or binge eating Sexual disinhibition Personality change Physical Symptoms: Primitive reflexes (grasp & palmomental reflexes) Incontinence (often a late feature in AD) Weight loss Deterioration in gait Falls Vascular Dementia Evidence of dementia and Cerebrovascular disease: focal signs on neurological testing & evidence of cerebrovascular disease on brain imaging (CT or MRI): multiple large infarcts, single infarct in the angular gyrus, thalamus, basal forebrain or PCA or ACA territories, or multiple basal ganglia & white matter lacunar infarcts or extensive periventricular white matter lesions or a combination of the above A relationship between the onset of dementia & the presence of cerebrovascular disease: Onset of dementia within 3 months of a stroke Abrupt deterioration in cognitive function or a fluctuating or stepwise deterioration Vascular Dementia Other features which may be associated: Early gait disturbance: ‘Marche a petit pas’, Parkinsonian (lower limbs), apraxicataxic History of unsteadiness or frequent falls Early urinary symptoms not explained by urological disease Pseudobulbar palsy, depression, psychomotor retardation & abnormal executive function Dementia with Lewy Bodies (DLB) (Consensus Criteria) (1) Evidence of dementia with: (2) Two of the following core features are essential in order to diagnose possible DLB: • • • fluctuations with pronounced variations in alertness & attention recurrent visual hallucinations that are typically well formed & detailed spontaneous features of parkinsonism e.g. rigidity, bradykinesia, tremor (3) Other supportive features: Repeated falls, syncope, systematised delusions, hallucinations in modalities other than vision Parkinson's disease Dementia Elderly with Parkinson's are more likely to develop dementia. Motor symptoms proceed by at least one year. Then followed by cognitive function deterioration No hallucination. Frontal Lobe Dementia Neurodegenerative disease with insidious onset and low progression. Onset is often early ( 35-75), and either behavioural or language symptoms dominate the clinical picture. Forgetfulness is mild, insight is lost early. Difficulties at work may be the first sign. Using MMSE can miss the diagnosis ( require FLT) Behavioural problems include disinhibition, mental rigidity, inflexibility, impairment of executive function, decrease personal care and repetitive behaviours. Language dysfunction:- include word finding difficulties, problem with naming or understanding words. Lack of spontaneous conversation. Neuroimaging usually demonstrate frontal/temporal atrophy 50% positive FH FLD include many spectrum like FL Degenration, Picks disease, MND with dementia Normal Pressure Hydrocephalus Wide gate (gate disturbance) Urinary incontinence Cognitive impairment CT large ventricle disproportional to cerebral atrophy MMSE and gait assessment before LP LP is diagnostic and therapeutic ( normal pressure, remove 20-30ml and re-assess gait and cognitive function) Some improve with ventricular-peritoneal shunt. Gait is more likely to improve. Complication infection and SDH Differential Diagnosis Causes of memory problems / confusion that are not dementia Delirium Depression ‘pseudo-dementia’ Mild cognitive impairment or benign cognitive impairment of aging Learning difficulties Previous brain injury Memory Complaints in Aging, Depression & Dementia Aging Depression Dementia Complaint May report a mild or subtle memory problem More likely to complain about their memory Expresses variable, nonspecific memory problems or may be unaware Functional Interference No interference with daily functioning Minimal interferencefunctional problems more likely due to mood disorder Clearly interferes with daily functioning: missing appointments, unpaid bills, medication compliance Cognitive Status Onset of problem unclear. Cognition is normal on testing Onset may be reported Gradual onset & as sudden, subtle deficits progression on testing only Cognition impaired on testing Mood Not associated with depression or anxiety Associated with a depressed or anxious mood May be associated with fluctuating or blunted affect Assessment Important points in the history: Duration, fluctuation, progression Forgetfulness, repetitiveness Misplacing or losing things Judgement – ability to manage finances Safety concerns Changes in personality or behaviour Loss of hygiene Falls Insight PMH Medications and compliance Assessment II Mental state examination Appearance & behaviour Speech Mood Delusions Hallucinations Personality – past & present Insight Cognition Assessment III Cognitive Assessment MMSE & Frontal Lobe Score MMSE & Clock Drawing Test Addenbrooke’s Cognitive Examination – Revised (ACE-R) Alzheimer’s Disease Assessment Scale for Cognition (ADAS-Cog) Assessment of Mood Geriatric Depression Score Hospital Anxiety & Depression Score Assessment IV Physical Assessment Focal neurological weakness Evidence of Parkinsonism Evidence of intercurrent illness causing a delirium Evidence of significant anaemia or hypothyroidism Evidence of dyspraxia Investigations All patients should have FBC, U&E’s, LFT’s, Ca, glucose to look for systemic causes of confusion B12, Folate, TFT’s VDRL if clinically suspect syphilis Cranial imaging to confirm / exclude : Cerebral tumours, Normal Pressure Hydrocephalus, subdural haematoma & to assess degree of vascular insufficiency DaTSCAN (Ioflupane SPECT) for clinically difficult to diagnose Dementia with Lewy Bodies EEG – not generally indicated but is abnormal in sporadic CJD DaTSCAN in DLB Normal DaTSCAN DaTSCAN in PD & DLB – Decreased dopaminergic neurones in the striatal area Management in Dementia - General Assess for physical illness & depression Establish functional abilities & any risks Capacity assessment Carer assessment Education of carers Assess social care needs & support required Planning for future care: advance directives, power of attorney Cholinesterase inhibitors Management of behavioural problems Terminal care Mild Dementia (Mild symptoms or MMSE 20-24) Appropriate counseling around the diagnosis Advice on how to maintain health & well-being Ensuring the individual has care to meet their needs prior to discharge Written information about dementia – leaflets produced by the Alzheimer’s Society Advice on Power of Attorney & how to plan for the future Details of how to contact the Alzheimer’s Society for ongoing support Convey the diagnosis to the GP so they can arrange follow up & refer to memory clinic if & when appropriate Moderate Dementia (Moderate symptoms or MMSE 10-20) As for mild dementia plus: Assess eligibility for memory clinic & cholinesterase inhibitors Discussions should take place about how someone would wish to be treated in the future: ceilings of treatment, palliative care if appropriate on the ward Severe Dementia If the patient has a clinical picture of dementia with severe symptoms with or without an MMSE of <10: As for mild to moderate dementia Consider stopping cholinesterase inhibitor Discussions should take place about how someone would wish to be treated in the future: ceilings of treatment, palliative care, where the individual would wish to die. Anti-dementia drugs Cholinesterase inhibitors: Donepezil: A reversible inhibitor of acetyl cholinesterase Galantamine: As for Donepezil + nicotinic receptor agonist Rivastigmine: Non-competitive inhibitor of acetyl cholinesterase, Licensed for dementia in PD & DLB N-methyl-D- aspartate (NMDA) receptor antagonist: Memantine: Some evidence it is effective in more advanced dementia, & beneficial in behaviourally disturbed AD in conjunction with a cholinesterase inhibitor PDD,DLB,ALZ have greatest cholinergic deficit What do NICE say? (November 2006) The cholinesterase inhibitors can be prescribed for clinically moderate AD or those with an MMSE 10-20 NMDA receptor antagonists to be prescribed ‘de novo’ only in recognised clinical trials Only specialists in Old Age Psychiatry or those geriatricians with specific expertise may start therapy Patients need to be reviewed at 3/12 & then 6/12 intervals to assess response with an MMSE score, a global functional & behavioural assessment & carer views to be considered If benefit noted they may continue on therapy until the MMSE<10 Management of Behavioural Problems Non-Pharmacological intervention Assess for intercurrent illnesses, pain ,constipation, urinary retention etc Ensure environment is appropriate for their needs: Lighting levels appropriate for the time of day Regular (at least 3xday) cues to orientate Use of clocks & calendars Hearing aids & glasses available & functioning Continuity of care from nursing staff Encouragement of mobility & engagement in activities Approach & handle gently, explain who you are, what you are going to do & why Non-pharmacological measures Elimination of unexpected & irritating noise Good pain control Encourage visits from family & friends especially at meal times Ensure adequate fluid & dietary intake Adequate CNS oxygen delivery Monitor bowels – avoid constipation Encourage a good sleep pattern Avoid inter & intra ward transfers Avoid catheters where possible Pharmacological interventions Indications for sedation: In order to carry out essential investigations or treatment To prevent a patient endangering themselves or others To relieve distress in a highly agitated or hallucinating patient, after assessing whether there is a physical cause for that distress Pharmacological intervention Acutely: Haloperidol, Olanzapine, trazodone 50mg nocte to 300mg max. and Lorazepam are the drugs of choice Do not use Haloperidol in patients with Parkinson’s disease or Dementia with Lewy Bodies Medium term : Haloperidol or atypical antipsychotics: (up to 6 weeks) Amisulpiride, Quetiapine, Olanzapine, Risperidone (caution in cerebrovascular disease) Longer term: Cholinesterase inhibitors, NMDA Receptor antagonists Prevention of Dementia Life style Physical activity Cognitive activity Diet:- fish oil Medication HRT NSAID Antioxidant Vitamin E&C Antihypertensive Statin Dementia Questionnaire Give 3 key features of dementia How long should symptoms have been present for to diagnose dementia? Give 3 different types of dementia. Which blood tests should be done routinely in a possible dementia patient? Why? Give 3 differential diagnoses for cognitive dysfunction. Name 3 assessments of cognitive function. Name a treatment for dementia? What class of drug are these? What are the standard criteria for eligibility for this drug? Give one key clinical feature of Alzheimer’s dementia. Give one key clinical feature of vascular dementia. Dementia Questionnaire An 82 year old lady presents having had recurrent falls, she doesn’t know why she is in hospital, her niece reports that she was fully able to look after herself and was driving 4 weeks ago. She is covered in bruises and her obs/WCC/urine dipstix/chest X-ray are normal. What is the most likely diagnosis? You are asked to review a 79year old surgical patient with “confusion” He has been confused since admission and looks thin and unkempt. He does not know where he is but is GCS 15. His son tells you he has stopped being able to cook meals, and does not recognise his grandchildren anymore. This has been going on for over a year. What is the likely diagnosis? Dementia Questionnaire An 86 year old lady is brought in with dehydration, apart from a raised urea her other investigations are normal. She reports having a memory problem which she is very anxious about, on testing her cognitive function is just below normal. She has trouble concentrating on the test. On the ward she is able to wash and dress herself, but keeps to herself. What is the likely diagnosis? You are called to the ward at night because a patient is threatening the nurses with his Zimmer frame. The nurses report that he is usually a “lovely old man” but today he has been more agitated. He is currently being treated for a UTI. What is the likely diagnosis? www.Alzheimer’s.org.uk Bournemouth Office: Alzheimer’s Society c/o King’s Park Community Hospital Gloucester Road Bournemouth BH7 6JE Telephone: 01202 309084 Thank you for listening