Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

Dementia & Wernicke-Korsakoff Syndrome S Z E Q I N G N G ( Y E A R 4 M E D S T U D E N T ) Contents 01. Alzheimer’s disease 02. Vascular dementia 03. Lewy body dementia 04. Frontotemporal lobar degeneration 05. Wernicke’s Encephalopathy 06. Korsakoff Syndrome 07. Alcohol Withdrawal C O N T E N T S P A G E 0 2 Alzheimer’s Disease most common form of dementia Clinical Features memory loss (affects recent events more, difficulty learning new information, vague with dates) problems with reasoning and communication difficulty in making decisions/ executive function nominal dysphasia depression agitation psychosis apathy disinhibition difficulties with activities of daily living (initially more difficult tasks, then simpler task) P A G E 0 3 Alzheimer’s Disease widespread cerebral atrophy (particularly involving the cortex and hippocampus) cortical plaques due to deposition of type A-Beta amyloid protein and intraneuronal neurofibrillary tangles caused by abnormal aggregration of tau protein Management offering range of activities to promote wellbeing that are tailored to the person’s preference offering group cognitive stimulation therapy for patients with mild and moderate dementia include group reminiscence therapy and cognitive rehabilitation Mild-to-moderate acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine) memantine (2nd line, if patient is intolerant or contraindicated for acetylcholinesterase inhibitors) (as an add-on drug for mod-to-severe) P A G E 0 4 Alzheimer’s Disease Severe memantine At risk of harming themselves or others, agitation, hallucinations or delusions antipsychotics Donepezil relatively contraindicated in patients with bradycardia adverse effects: insomnia P A G E 0 5 Vascular Dementia second most common dementia Risk Factors hx of stroke or TIA atrial fibrillation hypertension diabetes mellitus hyperlipidaemia smoking obesity coronary heart disease family history of stroke or cardiovascular P A G E 0 6 Vascular Dementia Classifications stroke-related VD – multi-infarct or single-infarct dementia subcortical VD – caused by small vessel disease mixed dementia – the presence of both VD and Alzheimer’s disease Clinical Features several months or several years of a history of a sudden or stepwise deterioration of cognitive function focal neurological abnormalities e.g. visual disturbance, sensory or motor symptoms difficulty with attention and concentration seizures memory disturbance gait disturbance speech disturbance emotional disturbance P A G E 0 7 Vascular Dementia Diagnosis a comprehensive history and physical examination formal screen for cognitive impairment medical review to exclude medication cause of cognitive decline MRI scan – may show infarcts and extensive white matter changes NINDS-AIREN criteria 1. presence of cognitive decline that interferes with activities of daily living, not due to secondary effects of the cerebrovascular event 2. cerebrovascular disease (neurological signs/brain imaging) 3. a relationship between the above 2 disorders inferred by: the onset of dementia within three months following a recognised stroke an abrupt deterioration in cognitive functions fluctuating, stepwise progression of cognitive deficits P A G E 0 8 Vascular Dementia Management symptomatic treatment detect and address cardiovascular risk factors cognitive stimulation programmes multisensory stimulation music and art therapy animal-assisted therapy managing challenging behaviours (avoid overcrowding, clear communication) AChE inhibitors or memantine if suspected comorbid Alzheimer’s disease, Parkinson’s disease dementia or Lewy body dementia P A G E 0 9 Lewy Body Dementia Lewy body (alpha-synuclein cystoplasmic inclusions) in the substantia nigra, paralimbic and neocortical areas Clinical Features progressive cognitive impairment (impairments in attention and executive function) cognition may be fluctuating parkinsonism (develops after cognitive impairment) visual hallucinations delusions P A G E 1 0 Lewy Body Dementia Diagnosis usually clinical single-photon emission computed tomography (SPECT) - show reduced dopamine transporter uptake in brain P A G E 1 1 Lewy Body Dementia Management AChE inhibitors (donepezil, rivastigmine) memantine avoid antipsychotics (may develop irreversible parkinsonism) P A G E 1 2 Frontotemporal Lobar Degeneration Progressive illness leading to behavioural changes and language problems Causes positive family history of dementia autosomal dominant inheritance (10%) genetic mutations (MAPT resulting in tau inclusion bodies/pick bodies, progranulin gene resulting in TDP-43 inclusion bodies, C9orf72) P A G E 1 2 Frontotemporal Lobar Degeneration Classification Behaviour variant frontotemporal dementia (bvFTD) the most common subtype (50% of cases) progressive decline and deterioration with early behavioural changes such as coarsening personality and impulsiveness Primary progressive aphasia (PPA) - language difficulty nonfluent/agrammatic variant PPA (apraxia and agrammatism) semantic variant PPA (difficulty naming single items whilst other language domains are spared) logopenic variant PPA (difficulty with repetition and single word retrieval) P A G E 1 2 Frontotemporal Lobar Degeneration Clinical Features insidious onset behaviour or speech changes are noticed before memory problems behavioural changes - predominant feature in behavioral variant FTLD 1.early apathy 2.decline in social interpersonal conduct 3.early disinhibition 4.early loss of empathy 5.early perseverative 6.hyperorality 7.decline in hygiene 8.primitive reflexes P A G E 1 2 Frontotemporal Lobar Degeneration Clinical Features difficulties with speech - predominant feature in PPA 1.language difficulty as the predominant feature 2.aphasia 3.decrease in speed of speech 4.difficulties with comprehension 5.syntax 6.object naming decline in executive functioning and cognition 7.poor concentration 8.difficulty planning memory problem 9.short term memory problems 10.difficulty with recall P A G E 1 6 Frontotemporal Lobar Degeneration Clinical Features neurological examination: 1.parkinsonism (tremor, rigidity, bradykinesia) 2.progressive muscle weakness including bulbar muscles (10-15%) P A G E 1 7 Frontotemporal Lobar Degeneration Investigations clinical diagnosis dementia screening bloods to exclude organic causes: 1.FBC - to rule out anaemia 2.ESR & CRP - signs of infection or vasculitis 3.T4 and TSH - to rule out hypothyroidism 4.U&Es - evidence of renal failure or electrolyte disturbances (e.g. hypercalcaemia, hypocalcemia etc) 5.B12 and folate - vitamin deficiencies may cause dementia 6.coagulation screen and albumin - assess liver function 7.glucose midstream urine (if concerned about delirium syphilis serology and HIV testing P A G E 1 8 Frontotemporal Lobar Degeneration Investigations Mini-mental state examination (MMSE) Montreal clinical assessment (MoCA) P A G E 1 9 Frontotemporal Lobar Degeneration Investigations Mini-mental state examination (MMSE) Montreal clinical assessment (MoCA) P A G E 2 0 Frontotemporal Lobar Degeneration Investigations MRI or CT scan (may show atrophy in the frontal and temporal regions) **FDG-PET (injection **FDG-PET or SPECT scan (only performed if the diagnosis is still unclear) of FDG into your vein, genetic testing (only performed if positive family history) a substance similar to glucose, PET scan detects high glucose conc. within the body hypometabolism = darker P A G E 2 1 Frontotemporal Lobar Degeneration Management referral to specialist psychiatry or neurology services patient and carer education (+ driving advice) PT/OT assessment follow up 6-12 monthly end of life care planning P A G E 2 2 Frontotemporal Lobar Degeneration Management Symptomatic control 1st line: group therapy, support groups pharmacological therapy (if non-drug therapies have failed) 1.benzodiazepines (for acute agitation and distress) 2.SSRI 3.antipsychotics (very low dose and as a last resort) P A G E 2 3 Wernicke’s Encephalopathy acute thiamine (vit B1) deficiency Causes alcohol abuse reduced absorption of thiamine (due to fasting, severe diarrhoea, bariatric surgery, hyperemesis gravidarum) increased metabolic requirements (febrile illness) P A G E 2 4 Wernicke’s Encephalopathy Clinical Features triad of confusion, ophthalmoplegia and ataxia ophthalmoplegia (uni or bilateral dysfunction of eye movement, often lateral rectus and medial rectus muscles) nystagmus (horizontal, vertical or rotary nystagmus, exacerbated by lateral gaze) ataxia (truncal ataxia, limb ataxia less prominent) confusion (disorientated unable to concentrate, difficulty following conversations) memory deficits (anterograde and retrograde amnesia) apathy or agitation hypothermia altered consciousness (can lead to coma) GI symptoms ((nausea, vomiting, abdominal pain tachycardia P A G E 2 5 Wernicke’s Encephalopathy Investigations diagnosed clinically serum thiamine (if clinical findings alone are insufficient for diagnosis) P A G E 2 6 Wernicke’s Encephalopathy Management urgent administration of parenteral thiamine for minimum of 5 days, then followed by oral if suspected of exhibiting WE as glucose metabolism, thiamine must be administered before or concurrently with any glucose administration concurrent treatment for alcohol withdrawal P A G E 2 7 Korsakoff Syndrome seen in alcoholics thiamine deficiency causes damage and haemorrhage to mammillary bodies of the hypothalamus and medial thalamus follows on from untreated Wernicke’s encephalopathy Clinical Features anterograde amnesia retrograde amnesia confabulation can improve with prolonged abstinence P A G E 2 8 Alcohol Withdrawal Clinical Features symptoms start at 6-12 hours (tremor, sweating, tachycardia, anxiety) peak incidence of seizures at 36 hours peak of delirium tremens is at 48-72 hours (coarse tremor, confusion, delusions, auditory and visual hallucinations, fever, tachycardia) Management patients with a history of complex withdrawals --> admission to hospital 1st line: benzodiazepines (chlordiazepoxide, lorazepam in patients with hepatic failure) carbamazepine P A G E 2 9 Thanks! T H A N K S ! P A G E 3 0