Dementia |X Flashcards

Question

What are the genetics linked to late onset AD?

Answer 1

Linked to the apolipoprotein E gene (APO-E). There are 3 types: APO-E2, APO-E3 and APO-E4. Everyone has two copies of the gene and these may be of the same type or different. 1. APO-E4 and E3 associated with higher risk of Alzheimer's 2. APO-E2 is mildly protective against Alzheimer's

Answer 2

Single-gene defects are responsible for rare variants of the disease. 1. The Notch3 gene is linked with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) 2. A variation in the APP gene causes heritable cerebral haemorrhage with amyloidosis (HCHWA).

Answer 3

Patients with Down’s have three copies of chromosome 21 Approximately 50% will develop Alzheimer’s by their sixth decade.

Answer 4

Autosomal dominant Defect in Huntingtin gene Dementia can occur at any stage but often presents quite young.

Answer 5

These are strongly heritable. A number of faults on the tau gene have been implicated

Answer 6

1. Mild cognitive impairment 2. Genetics 3. Age

Answer 7

1. Smoking 2. Alcohol 3. Atherosclerosis 4. Hypercholesterolaemia

Answer 8

1. Early onset (rare) – fewer than 10% of Alzheimer’s patients. Associated with the development of myoclonus 2. Late onset (most common) - symptoms begin after 65 3. Familial (rare) –entirely inherited. Fewer than 5% cases and very early onset usually in 40’s

Answer 9

1. Early 2. Middle 3. Late

Answer 10

1. Minor changes in abilities or behaviour; often only realised in hindsight 2. Loss of recent memory, repetition of questions, slow at grasping ideas, occasional confusion, mislaying items and blaming others 3. Unwilling to embrace change and difficulty dealing with money

Answer 11

1. Changes seen in phase one become more marked and more support is needed with day-to-day tasks. 2. Prompting required; increasingly forgetful. 3. Increasingly repetitive due to the decline in short term memory. 4. Failure to recognise friends/family. 5. Frustration may lead to aggression or loss of self confidence. 6. Disorientation to time, place and person. 7. Development of hallucinations 8. Memory for the distant past often remains good

Answer 12

1. Increasing dependence on others for care 2. Inability to recognise familiar objects or even relatives. 3. Increasing frailty; eventually confined to bed or wheelchair. 4. Poor appetite, dysphagia and weight loss. 5. Deteriorating speech and understanding. 6. Incontinence 7. Restlessness, agitation, distress or aggressive behaviour

Answer 13

Last 1-2 years of life

Answer 14

From infection such as pneumonia. Malnutrition is common and contributes to risk of developing infection

Answer 15

1. Characterised by loss of neurons and synapses from the cerebral cortex and certain subcortical regions 2. This loss results in gross atrophy of the affected regions, including degeneration in the temporal lobe (hippocampus and amygdala), parietal lobe, parts of the frontal cortex and cingulate gyrus 3. There is an excess of plaques and tangles

Answer 16

1. The cortex is atrophic; damaged areas are involved in thinking, planning and remembering. 2. The hippocampus is severely atrophic; this area plays a key role in memories and is affected early 3. The ventricles of the brain are enlarged 4. Amygdala later damaged - controls emotions

Answer 17

1. Made up of deposits of a protein fragment called beta-amyloid 2. These proteins are sticky and clump together and form plaques that build up within the spaces between cells. 3. Plaques are thought to block cell-to-cell signaling or activate immune system responses that trigger inflammation and cell death within the brain.

Answer 18

1. Neurofibrillary Tangles are thought to destroy the cell transport system which is made from a protein called tau. 2. Tau usually helps the strands of the transport system link together. 3. Tangles are twisted fibres of tau that are no longer able to link the transport system of the cell and thus this falls apart and the cell dies.

Answer 19

1. Memory deficit that can be demonstrated objectively by cognitive tests 2. Plus at least one other cognitive deficit (aphasia, apraxia, agnosia or impairment with executive functioning) 3. Reduced ability to perform activities of daily living (ADLs) 4. A gradual onset and progressive course 5. A decline from a previous level of functioning 6. No evidence of other medical or neurological condition

Answer 20

Vascular dementia

Answer 21

A problem with the blood supply to the brain | -like most vascular problems it is potentially preventable

Answer 22

1. Post-stroke dementia 2. Multi-infarct dementia - a series of small strokes in the cerebral cortex (cortical vascular dementia) 3. Subcortical vascular dementia - affects inner parts of brain 4. Mixed cortical and subcortical dementia

Answer 23

People with HTN

Answer 24

Same as for vascular disease 1. Hypertension 2. Hypercholesterolaemia 3. Diet high in saturated fats 4. Lack of physical activity 5. Smoking 6. Family history of stroke or vascular dementia 7. Cardiovascular Disease 8. Diabetes 9. Excess alcohol

Answer 25

1. Memory problems (may be less apparent earlier on) - Problems planning, organising, making decisions etc 2. ‘Step wise’ progression with stable disease and then sudden deteriorations 3. Difficulty with concentration and communication, and following steps 4. Seizures 5. Depression 6. Symptoms and signs of stroke disease 7. Incontinence 8. Emotional lability 9. Changes in behaviour 10. Visual problems and perceptual difficulties 11. Early gait disturbance, unsteadiness and falls 12. Slower speed of thought

Answer 26

1. Dementia defined by cognitive decline manifesting as memory impairment and impairment in a further cognitive domain (as for AD). 2. Deficits should be causing limitation with ADLs not due to the physical effects of stroke alone. 3. Evidence of cerebrovascular disease on clinical examination and imaging.

Answer 27

Slightly more common in men

Answer 28

1. Presence of Lewy bodies, made up of protein alpha synuclein 2. Their presence disrupts the brain’s functioning by interrupting the action of acetylcholine and dopamine. 3. Areas affected in Lewy Body dementia include: - The substantia nigra. Lewy bodies seen here are called classical Lewy bodies - as seen in Parkinson’s disease - Degeneration in the cortex. Lewy bodies here are called cortical Lewy bodies 4. Shrinkage of the brain affects mainly the parietal and temporal lobes and the cingulate gyrus.

Answer 29

Core: 1. Progressive memory impairment = main feature 2. Fluctuating cognition 3. Visual hallucinations 4. Features of parkinsonism Supporting 1. Falls 2. Syncope 3. REM sleep behaviour - During periods of REM sleep, the person will move, gesture and/or speak 4. Sensitivity to neuroleptics - treatment with this should be avoided

Answer 30

If significant cogntiive decline begins within a year or the Parkinsonian features diagnosis is likely to be LBD

Answer 31

- Progressive cognitive decline. Prominent memory impairment may not occur in the early stages. - Deficits on tests of attention and frontal-sub-cortical skills and visuospatial ability may be especially prominent. - Reduced ability to perform ADLs. 2 of the following are required for a probable, and one for a possible diagnosis of LBD: 1. Fluctuating cognition with pronounced variations in attention and alertness 2. Recurrent visual hallucinations which are typically well-formed and detailed 3. Spontaneous motor features of parkinsonism

Answer 32

1. Abnormal protein aggregation in brain cells. The implicated protein is tau. 2. Affects the frontal and temporal lobes, responsible for behaviour, emotional changes and language skills

Answer 33

1. In early stages, cognition often intact 2. It typically causes changes to behaviour and personality - person may be increasingly more extrovert and disinhibited. 3. Some have a change in eating habit, overindulging or developing a sweet tooth and later may develop hyperphagia 4. Language problems are also prominent and this may manifest initially as word finding difficulties and resultant reduction in amount of speech

Answer 34

As the disease progresses a number of primitive reflexes may develop (frontal release signs) 1. Palmar grasp reflex 2. Rooting reflex

Answer 35

1. Insidious onset and gradual progression 2. Early decline in social interpersonal conduct 3. Early impairment in regulation of personal conduct 4. Early emotional blunting 5. Early loss of insight

Answer 36

1. Behavioural - Decline in personal hygiene; grooming - Mental rigidity & inflexibility - Distractibility - Hyperorality and dietary change - Utilisation behaviour 2. Speech and language - Altered speech output including reduced spontaneity and economy of speech - Echolalia - Perseveration - Mutism 3. Physical signs - Primitive reflexes - Incontinence - Akinesia - Tremor - Low BP

Answer 37

1. Neuropsychological tests – impaired frontal lobe tests but no amnesia or perceptual deficits 2. EEG – normal 3. Imaging predominant frontal lobe +/- anterior temporal abnormalities

Answer 38

1. Marked forgetfulness 2. Lethargy 3. Loss of executive functioning 4. Loss of emotional control 5. Any Psychotic symptoms may be exacerbated by the anti-Parkinson’s medication and therefore the drugs may need to be reduced or withdrawn. 6. Often neuroleptics prescribed to help with the disruptive behaviour, eg neuroleptics, make the Parkinson’s symptoms worse. Haloperidol, chlorpromazine and sulpiride should be avoided.

Answer 39

1. Therapy aimed at cognitive enhancement | 2. Treatment of behavioural and psychological symptoms of dementia (BPSD)

Answer 40

1. Acetylcholinesterase inhibitor 2. NMDA receptor antagonist (drugs are not curative but may help control symptoms or slow progression of the condition)

Answer 41

1. Donepezil (Aricept) 2. Galantamine (Reminyl) 3. Rivastigmine (Exelon)

Answer 42

Inhibiting the breakdown of acetylcholine, a neurotransmitter which is important in cognitive processes.

Answer 43

Blocks NMDA glutamate receptors. At normal levels, glutamate aids in memory and learning, but if levels are too high, it leads to overstimulation of nerve cells resulting in degeneration.

Answer 44

``` 21-26 = mild dementia 15-20 = moderate 10-14 = moderate to severe >10 = severe ```

Answer 45

Mild-moderate

Answer 46

1. Moderate AD who are unable to take AChE inhibitors or 2. Severe AD

Answer 47

1. Only specialists in the care of patients with dementia should initiate treatment. 2. Treatment should be continued only when it is considered to be having a worthwhile effect on cognitive, global, functional or behavioural symptoms. 3. Patients who continue on treatment should be reviewed regularly for any changes in behaviour and cognition as well as any medication side effects.

Answer 48

1. NICE recently suggested trial of ACHEI 2. Management mainly aimed at managing neuropsychiatric disturbances and movement disorders 3. No cure or definitive treatment for LBD

Answer 49

Reducing vascular risk (bp, lipid profile, glycaemic control etc to slow progression of VD

Answer 50

Symptoms of disturbed perception, thought content, mood and behaviour that frequently occur in patients with dementia

Answer 51

Psychological 1. Anxiety 2. Delusions 3. Depression 4. Hallucinations Behavioural 1. Aggression 2. Wandering 3. Agitation 4. Screaming 5. Shadowing 6. Cursing 7. Sexual disinhibition

Answer 52

1. place extra burden on the carer 2. increase the cost of care 3. reduce quality of life for the patient and their carers 4. increase need for hospitalisation 5. increase need for early placement in care facilities

Answer 53

2/3 of patients with dementia exhibit BPSD at some point Can occur at any stage

Answer 54

1. Identification of behaviour - Observe/obtain history from patient and carers to: a. identify circumstances under which it occurs b. when it started c. whether onset was gradual or sudden 2. Perceptions -If the behaviour appears inappropriate, consider whether there is an underlying goal (e.g. exit-seeking) or misperception (e.g. misperceiving the corner of a room as a urinal) 3. Psychiatric history - Review the patient's psychiatric history, social history and premorbid personality 4. Life events - Inquire about life events and the quality of the relationship between carer and patient. 5. Mental status - Examine for changes in mental status. - Look for signs of discomfort or distress that may be a cause of agitation or aggressiveness 6. Diagnosis - Always consider an alternative diagnosis e. g. delirium, side effects of medication or concurrent mental illness

Answer 55

1. Family involvement 2. Communication - Speak slowly, use shorter sentences and less complex instructions. - Give positive non-verbal cues and use gentle touch (if patients allow) 3. Encourage conversation 4. Encourage and help with orientation 5. Hallucinations - When the patient is hallucinating, do not rebuke or flatly contradict 6. Senses - Ensure that there are no barriers to their vision or hearing 7. Familiarity - Stimulation on the ward, e.g. family pictures or music that patients can relate to. - Help the person feel at home 8. Mobility - Encourage mobility with an emphasis on safety as well

Answer 56

As BPSD affects each individual differently, a person-centred approach is needed. Remember that behaviour is usually an expression of an underlying need

Answer 57

1. Citalopram 2. Zopiclone 3. Risperidone 4. Senna 5. Paracetamol

Answer 58

Help with physical aggression, agitation and psychosis

Answer 59

1. 3-fold increase in the risk of stroke with antipsychotics - Risk of stroke may be even higher above the age of 80 2. Side effects include parkinsonism, falls and lethargy - Typical antipsychotics have more side effects than atypical ones - Should only be prescribed by specialists

Answer 60

LBD and PDD

Answer 61

1. the person’s present living arrangements, and arrangements for care 2. the person’s health and disabilities, and what they can and cannot do 3. the person’s worries, and how they want to be supported 4. the types of service sought and how they want these to be arranged 5. the concerns of the carers

Answer 62

1. home help to assist with activities of daily living 2. specific equipment and adaptations 3. meals on wheels 4. respite service 5. day care facilities 6. care home placement

Answer 63

Reviewed after 3 months, then annually or as the need changes

Answer 64

1. Memory loss - forget names, people and places - repetitive - misplacing items in old places - confusion about time of day - getting lost - inability to remember new information 2. Difficulties with thinking 3. Problem-solving 4. Language All types of dementia are progressive. This means that the structure and chemistry of the brain become increasingly damaged over time. The person's ability to remember, understand, communicate and reason gradually declines