Dementia Flashcards
define dementia
syndrome due to a disease of the brain usually chronic and progressive present for more than 6 months
what functions of the brain can be affected in dementia?
multiple higher cortical functions including memory, thinking, orientation comprehension, learning capicty, language and judgment
ESPECIALLY:
o Memory - especially learning and retrieval of new info
o Cognitive abilities - deterioration in judgement, thinking and language
o Emotional control - emotional lability, apathy, irritability, wandering, disinhibition
if you suspect dementia but there is clouding of consciousness, what is it?
delerium
what are the differentials for dementia?
D rugs/delirium E motions/depression (pseudodementia) M etabolic disorder (glucose or thyroid) E eye/ear problems (e.g. deafness) N utritional disorders (e.g. B12/folate deficiency) T umours, toxins, trauma I nfections A lcohol, arteriosclerosis
In what order do you get symptoms in alzheimers?
memory loss impaired thinking language impairment deterioration in personal functioning disturbed personality and behaviour perceptual abnormalities motor impairments
How is memory affected in alzheimers?
short-term > long-term
leads to impaired learning and disorientation (time first, then place and person)
How is thinking impaired in alzheimers?
poor judgement, decreased fluency, concrete thinking and impaired abstraction, lack of ability to plan, delusions
how is language impaired in alzheimers?
expressive and receptive aphasia and dysphasia
how is personal functioning affected in alzheimers?
deterioration in occupational and social functioning and AoDL/ self-care
how is personality and behaviour affected in alzheimers?
euphoria and lability or apathy and irritability. disinhibition which can lead to aggressive and inappropriate behaviour, inattention and distractibility, obsessive and stereotyped behaviours.
How are perceptions altered in alzheimers?
visual and auditory agnosia, visuospatial difficulties, body hemineglect, prosopagnosia (can’t recognise faces), illusions, hallucinations, cortical blindness
how is the motor function altered in alzheimers?
Apraxia, spastic paresis, urinary incontinence
What are primary causes of dementia?
Alzheimer’s disease (most common)
Dementia with Lewy bodies and PD
Pick’s disease and other frontotemporal dementias
Huntington’s disease
what are secondary causes of dementia?
Vascular Infective (AIDs, lyme disease) Inflammatory (SLE) Neoplastic Metabolic (cardiac, hepatic and renal failure) Endocrine (thyroid) Toxic (alcohol) Traumatic
What investigations should you do in dementia?
- A physical examination should be carried out to identify any underlying causes of dementia and any complications of dementia such as malnutrition, burns, or falls.
- Baseline bloods as standard. Other investigations on case by case basis
- Detailed neurocognitive testing by a clinical psychologist can be helpful in identifying cognitive impairments and in confirming a diagnosis.
- Dr Ostler scans anyone under 75 or if something Hx raises a question MRI/CT and can use PET scan
What neurocognitive testing can you do?
o MMSE/AMTS/MOCA for screening and monitoring
MMSE <24 = suggestive of dementia
Only need to know AMTS!
o Addenbrooke’s Cognitive Exam (ACE – III) is what they use in memory clinics (<88/100)
What blood tests should you do in a confusion screen and why?
• FBC
o Infection, anaemia, increased MCV (macrocytic anaemia caused by B12/folate deficiency)
• LFTs
o Liver failure, alcohol abuse
• U&Es
o Kidney failure can cause cognitive impairment?
• INR
o If on warfarin and concerned about bleeds from fall/head injury
• TFTs
o Confusion more common in hypothyroid states
• Calcium
o Hypercalcaemia can cause confusion/delirium (bones, moans, psychotic groans
• B12/folate can cause confusion (alcohol use, leukaemias, dietary deficiency/ malabsorption)
• Glucose
o Hypoglycaemia = common cause of confusion
• Syphilis
o Not often necessary, rare but overlooked
• HIV
o Dementia common in AIDS
what other investigations should you do in a confusion screen?
CT HEAD – stroke, lesion, bleeds from fall/trauma, cerebral atrophy, widening of the sulci or gyri, dilated ventricles (could be hydrocephalus), atrophy of medial temporal lobe in Alzheimer’s
(MRI recommended by NICE but CT usually sufficient)
CXR – as part of sepsis screen – rule out pneumonia, bronchial CA w/cerebral mets
BLOOD CULTURES – if appropriate
URINE DIPSTICK/CULTURE – v common cause in elderly, but positive dipstick w/o clinical signs is not enough to diagnose urosepsis as a cause of delirium (look this up)
URINE DRUG TEST – if illicit drugs considered possible cause of confusion/psychosis
EEG – characteristic 3Hz ‘spike and wave’ in prion disease
LP – normal pressure hydrocephalus, herpes encephalitis
What is the very general management in dementia?
- Secondary dementias may be reversible
- But usually aim of management is to improve/maintain QOL for patient and carer(s).
- This involves treating the SYMPTOMS AND COMPLICATIONS of dementia, addressing FUNCTIONAL problems, addressing SOCIAL problems, and providing EDUCATION and SUPPORT for carers.
What are important considerations in the MDT approach in treating dementia? (general info)
• Personal hygiene & nutrition
• Functional abilities can be maintained and even improved by a regular daily routine, environmental modifications, and graded assistance.
• The patient should be reoriented and reassured, and encouraged to partake in physical and mental activity.
• Memory aids such as clocks, calendars, notebooks, and photographs, and reality orientation and reminiscence therapies may also be helpful, particularly in the early stages of the disease.
• To aid sleep, sleep hygiene, Horlicks or milky drinks, sedation at night, sedative antidepressants e.g. trazadone (may increase risk of falls), change to another AChEi, try NDMA blocker e.g. memantine.
• Telecare to stop wandering.
• If dementia is diagnosed must inform DVLA
• Social problems:
o Isolation, accommodation (?specialist home)
o Financial/legal matters e.g. power of attorney
• Carer education and support
What classes of drug can you use in dementia?
Acetylcholinesterase inhibitors
NMDA receptor antagonist (memantine)
Other possible drugs (use sparingly and infrequently - benzos, SSRIs, Antipsychotics (quetiapine)
Whats the most common cause of dementia?
• AD = most common cause of dementia - over 50% of all cases (850,000 people in UK)
what is the neuropathology of alzheimers?
• Selective neuronal and synaptic loss leads to neurochemical abnormalities (notably decreased ACh) and symmetrical cortical atrophy that is initially more pronounced in the medial temporal and parietal lobes.
• Extracellular senile plaques and intracellular neurofibrillary tangles are seen in normal ageing, but they are more numerous in AD and they are closely related to the degree of cognitive impairment.
• Senile plaques consist of a core of beta-amyloid surrounded by filamentous material.
• Neurofibrillary tangles consist of coiled filaments of abnormally phosphorylated microtubule-associated protein tau (note that tau is also found in Pick bodies)
• Decreased ACh
o Due to degeneration of the cholinergic neurons of the basal forebrain
• Other histopathological findings include glial proliferation, granulovascular degeneration, and Hirano inclusion bodies (intracellular aggregates of actin).
What are risk factors for alzheimers?
Age Female sex (2:1) FH Down’s syndrome Head injury Dialysis (aluminium containing dialysis fluids)
what are protective factors for alzheimers?
Healthy and engaged lifestyle High educational achievement (does this just delay diagnosis? NSAIDs HRT Vit C Vit E Statins
what is the genetic link in alzheimers?
• E4 allele of apolipoprotein E
o Chromosome 19
o Risk factor for common, sporadic, late-onset form
• E2 is protective
• APP (amyloid precursor protein) mutations
o Chromosome 21
o AD inherited, early onset
• PSEN1/PSEN2 also associated with familial presenile dementia
what is the life expectancy after diagnosis of alzheimers?
8 years
what are the 5 As of alzheimers?
- AMNESIA
- APHASIA
- AGNOSIA
- APRAXIA
- ASSOC BEHAVIOUR
What is the medication therapy for alzheimers?
o Mild/moderate = AChEi - donepezil, galantamine, rivastigmine
o Severe AD = memantine monotherapy
o For patients already on AChEi consider adding memantine if progresses
• Pharmacological therapy should only be given by specialist of GPs with special expertise
What is the second most common form of dementia?
vascular dementia
what is mixed dementia?
evidence of both AD and vascular dementia
what is the neuropathology of vascular dementia?
- Focal disease may result from single, or more commonly, from multiple thrombotic or embolic infarcts.
- Note that focal and diffuse disease often co-exist.
What are the risk factors in vascular dementia?
• CV disease and risk factors
o Older age, male, smoking, DM, HTN, hypercholesterolaemia, valvular disease, hypercoagulation disorders, alcohol)
• Cardiovascular disease
• Cerebrovascular disease
what are the signs and symptoms and differences to alzheimers in vascular dementia?
• Vascular dementia classically has an abrupt onset and step wise progression.
• Clinical features are variable and depend on the location of the infarcts, but commonly:
o Personality changes
o Labile mood
o Preserved insight (until late)
• UNEQUAL DISTRIBUTION of defectits (some v affected, others spared)
• Significant comorbidity leads to a shorter mean life expectancy than in Alzheimer’s disease
What is the treatment in vascular dementia?
• Lifestyle changes to reduce CV risk factors (prevent further decline)
• Social care/support, OTs,
• ANTICOAGULATE to prevent further CVAs
• Statins for high cholesterol
• Antiplatelet e.g. aspirin, clopidogrel
• Ensure diabetes is under control
• AChEi or memantine will not affect cognitive decline in vascular dementia!
o But can be used if suspected comorbid AD/PD/DLB
what is the epidemiology of lewy body dementia?
overlaps with Alzheimer’s disease and parkinsonian dementia.
• It is the third commonest cause of dementia, and accounts for about 10-15% of all cases.
• Onset 50-85
• Slightly more M>F
• Lewy bodies = abnormal aggregates of protein that develop inside nerve cells
What is the neuropathophysiology in LBD?
• Both subcortical and cortical structures involved
• Associated neuronal loss leading cholinergic deficit and other chemical abnormalities
o Only minimal cortical atrophy
• Senile plaques may be present but nurofibrilliary tangles are not are marked fearutre
• Aetiology (cause) unclear
What is the difference between LBD and PD?
• Lewy bodies present in both
• PD – lewy bodies are in basal ganglia
• DLB – lewy bodies are in cortical areas
o Esp cortical layers V and VI of temporal lobe, cingulate gyrus and insular cortex
• If parkinsons symptoms present first its parkinson’s dementia, if memory symptoms appear first then parkinsons Sx, it is DLB
What is the classical triad of symptoms in LBD?
o Marked fluctuations in cognitive impairment and alertness.
o Vivid visual hallucinations and other psychotic symptoms (70/80%)
o Parkinson-like movement symptoms e.g. rigidity & lack of spontaneous movement
What are other symptoms in LBD?
• REM sleep disorder vivid dreams w/body movement
• Frequent faints, falls, dizziness
• Neuroleptic sensitivity
o Severe neuroleptic sensitivity affects up to 50% of the LBD patients who are treated with traditional antipsychotic medications
o Characterized by worsening cognition, sedation, increased or possibly irreversible acute onset parkinsonism, or symptoms resembling neuroleptic malignant syndrome
o Can be fatal
• Memory loss may not be a marked feature in early stages
• Life expectancy from diagnosis = 6 years
what is the investigations for parkinsonian dementia?
formally diagnosed by a DaT scan (Ioflupane I 123 injection). Dopamine transporter scan (used in diagnosis of PD)
what is the treatment for PD?
• Not going to cure but may help managing symptoms (shock)
• Before drug treatment
o Evaluate physical health problems that could be provoking behavioural disturbance
o Look at current medications
• Drug treatment
o AChEi
Rivastigmine or donepezil
Only consider galantamine if others not tolerated
o Memantine
If AChEi not tolerated/CI
o Antipsychotics
Avoid unless absolutely necessary (NEUROLEPTIC SENSITIVITY)
Quetiapine (atypical) preferred, or clozapine
Traditional (e.g. haloperidol) should be avoided
What is the epidemiology of frontotemporal dementia?
5% of all cases of dementia.
• F>M
• peak age of onset = 45–60 years
• Aetiology unclear. Familial forms exist and are more common in people of Scandinavian descent.
what is frontotemporal dementia also called?
Pick’s disease
What is the neuropathology of FTD?
selective, often asymmetrical, ‘knife-blade’ atrophy (radiology feature) , neuronal loss, and gliosis affecting the frontal and temporal lobes.
• There are characteristic ‘ballooned’ neurons called Pick cells and tau-positive neuronal inclusions called Pick bodies.
• There are, however, no senile plaques or neurofibrillary tangles as in Alzheimer’s disease.
What are the typical signs and symptoms of FTD?
o early and prominent personality/behaviour changes
early decline in social interpersonal conduct, emotional blunting, regulation of personal conduct – social disinhibition, neglect of responsibilities, loss of interest in personal hygiene
o eating disturbances
o mood changes
o early loss of insight
o expressive dysphasia
o cognitive impairment, language abnormalities, and motor signs.
See everything as “black & white” – can’t see the grey
• All concrete thinking, struggle with abstract
• Can appear bit like autism
Problems with problem solving
• Executive impairment = the range of symptoms of frontal lobe damage
o early preservation of episodic memory and spatial orientation, disinhibiton, hyperorality, stereotyped behaviour and emotional unconcern.
What is the treatment for FTD?
• Stop drugs which may be exacerbating memory problems or confusion (anticholinergics, CNS drugs).
• SSRIs may be helpful
o For loss of inhibitions, compulsions and overeating
• Atypical antipsychotics used occasionally where there are severe behavioural problems such as agitation and psychosis.
• Levodopa/carbodopa may be tried when there are Parkinsonian symptoms.
• AChEi may worsen so do not use
What is the prevalence and onset of huntingtons disease?
- 5-10 per 100 in caucassions
* Onset 30s-40s but in some cases it can be in childhood or old age
What is the neuropathology in huntingtons?
• Abnormal Huntington protein leads to the degeneration of the neurons, notably in the caudate nucleus and putamen in the cerebral cortex
• Degeneration in the caudate nucleus and putamen leads to movement dsorders
o Degeneration in the cerebral cortex leads to dementia
• Due to anticipation each generation age of onset increases
o For mcq, cause = more than 36 repeats of CAG in hungtington gene chromosome 4
What are the classical signs and symptoms of huntingtons?
- Choreiform (dance like movements)
- Schizophrenia like psychosis
- Early depression and behavioural disturbances
- Insight often retained to until late - suicide rate high 10%
Give other dementias and amnesic syndromes.
normal pressure hyrdocephalus HIV related dementia Hypothyroidism chronic subdural haematoma neurosyphillis CJD
How does normal pressure hydrocephalus present?
o Accumulation of CSF = large ventricles, ut no increases in ICP
o Triad = gait disturbance and ataxia, dementia, urinary incontinence (in that order)
How does CJD present?
o Neurodgenerative disease which result from deposition of prion protein in the form of amyloid sheets
o Sx rapidly progressive dementia, myoclonic jerks, constellation of pyriamidal, extrapyrimaldal and cerebellar signs
o EEG changes 3Hz ‘spike and wave’
What is the epidemiology of delerium?
- Delirium (acute confusional state or acute brain syndrome) is common in hospitalised patients and particularly in young children/the elderly.
- It occurs in ~40% of >65s during hospitalisation.
What is the definition of delerium?
an aetiologically non-specific syndrome characterised by concurrent disturbances of consciousness and attention, perception, thinking, memory problems, psychomotor behaviour, emotion, and the sleep wake cycle, that is of transient and fluctuating intensity
what is the onset like in delerium?
quick - hours to days
What do you need for a definite diagnosis of delerium?
- IMPAIRED CONSCIOUSNESS, difficulty focusing and inattention
- GLOBAL DISTURBANCE OF COGNITION:
a) Impairment of abstract thinking and comprehension and in some cases transient delusions
b) Impairment of immediate recall and recent memory, with remote memory being relatively spared
c) Disorientation in time and, if severe, in place and person
d) Perceptual abnormalities including distortions, illusions and hallucinations (usually visual) - PSYCHOMOTOR DISTURBANCES - Hyper/hypo-activity and unpredictable shifts from one to the other
- Disturbance and in severe cases, reversal of SLEEP-WAKE CYCLE
- EMOTIONAL DISTURBANCES e.g. depression, anxiety or fear, irritability, euphoria, apathy
What are possible causes of delerium?
PINCH ME
• Pain
• INfection
o Any, but commonly UTI - Remove unnecessary catheters ( top 3 - UTI, pneumonia and cellulitis)
• Constipation
• Hydration/hypoxia
o Dehydration
o Urinary retention
o Electrolyte imbalance – esp hyponatraemia and hypercalcaemia
o Renal/hepatic failure
• Medication
o Commonest cause
o Alcohol, opiates, sedatives, anticholinergics, diuretics, steroids, digoxin, anticonvulsants, lithium, TCAs, levodopa, polypharmacy
o Also WITHDRAWAL from alcohol, benzos or others
• Environment
o Confusion from unfamiliar/distressing surroundings
Other = hypoglycaemia, DKA, hypo/hyperthyroidism, Cushings, stroke, head injury, stress, sleep deprivation, urinary retention, encephalitis, bleed, tumour
What are the questions in an AMTS?
- Age
- DOB
- 42 West Street
- Year
- Time (nearest hour)
- Place
- Identify two people
- Dates of WW1
- Current monarch
- Count back from 20
- Recall address
What are your differentials in delerium?
- Delirium superimposed on dementia
- Dementia
- Substance misuse
- Affective disorder
- Psychotic disorder
What investigations should you do in delerium?
- Daily assessment of cognitive and mental status
- If delirium suspected full psych hx, MSE, physical exam, delirium screen and COLLATERAL Hx
- Confusion screen as above
How can you prevent delerium?
Routine cognitive assessment
Rationalising the drug chart
ORIENTATE PATIENT - ensure sensory aids e.g. spectacles and hearing aids are in place.
o Also clocks, calendars, familiar items
Encouraging fluids and nutrition and correcting any electrolyte imbalances and nutritional deficiencies.
Encouraging mobilisation.
Encouraging family members to spend time at the bedside.
Nursed in consistent, comfortable and familiar environment
When should tranquilisers be used in delerium and what is the drug of choice?
extreme agitation or psychosis
Haloperidol is the drug of choice (except if the delirium is caused by alcohol or benzodiazepines, in which case it is a benzodiazepine) because of its minimal anticholinergic side
(remember stroke risk in dementia patients)
Doses should be kept to a minimum to avoid precipitating alternating episodes of agitation and sedation and stopped as soon as it is felt no longer indicated
What are possible complications of delerium?
o Prolonged hospital stay and increased risk of nosocomial (hospital) infections. o Accelerated cognitive decline. o Aspiration pneumonia. o Fluid and electrolyte imbalance. o Malnutrition. o Falls. o Injuries. o Decreased mobility o Pressure sores
What is the mortality in delerium?
high, both in hospital and after discharge one year mortality has been estimated to be as high as 50%.
