Dementias Flashcards

1
Q

What is dementia?

A

Dementia is a syndrome due to a disease of the brain, usually chronic and progressive in nature, in which there is a disturbance of multiple higher cortical functions

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2
Q

Which functions are affected by dementia? (7)

A
  1. Memory
  2. Cognition
  3. Orientation
  4. Comprehension
  5. Language
  6. Judgement
  7. Learning capacity
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3
Q

What is important to remember with regards to consciousness and dementia?

A

Consciousness is not clouded

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4
Q

In terms of memory loss, how does this often present in dementia?

A

Short-term memory loss, leading to impaired learning and disorientation (first in time and then in place and person)

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5
Q

In terms of impaired thinking, what chances in someone with dementia?

A

They have poor judgement, decreased fluency, lack of ability to plan or sequence behaviour, and sometimes delusions

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6
Q

What is the name of the syndrome which refers to severe senile self-neglect, meaning a deterioration in activities of daily living and social functioning?

A

Diogenes syndrome

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7
Q

What kind of disturbed behaviour and personality changes can occur in someone with dementia? (2)

A
  1. Emotional lability, apathy or irritability.

2. Disinhibition leading to aggressive and socially inappropriate behaviour.

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8
Q

What is the term given to the inability to recognise faces?

A

Prosopagnosia

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9
Q

What type of hallucinations are common in someone with a dementia?

A

Visual hallucinations

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10
Q

If the frontal lobe is affected by dementia, what clinical features/problems will someone display? (5)

A

Problems with:

  1. Judgement
  2. Reasoning
  3. Behaviour
  4. Voluntary movements
  5. Expressive language (Broca’s)
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11
Q

If the temporal lobe is affected by dementia, what clinical features/problems will someone display? (5)

A

Problems with:

  1. Emotions
  2. Learning and memory
  3. Audition
  4. Olfaction
  5. Language comprehension (Wernicke’s)
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12
Q

If the parietal lobe is affected by dementia, what clinical features/problems will someone display? (4)

A
  1. Spatial orientation
  2. Perception
  3. Initial cortical processing of tactile and proprioceptive information
  4. Language comprehension (Wernicke’s)
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13
Q

In orbito-frontal syndrome, what behaviours are most likely to be seen?

A
  1. Disinhibition
  2. Aggressive and socially inappropriate behaviour
  3. Emotional liability
  4. Poor insight
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14
Q

In dorsolateral prefrontal syndrome, what behaviours are most likely to be seen? (4)

A
  1. Apathy
  2. Irritability
  3. Lack of ability to plan
  4. Decreased fluency
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15
Q

What are the types of dementias? (6)

A
  1. Alzheimers
  2. Vascular
  3. Lewybodies
  4. Parkinsons
  5. Pick’s disease and other frontotemporal dementias
  6. Huntington’s disease
    (lots of secondary dementias e.g. infective. inflammatory neoplastic, metabolic, endocrine, toxic, traumatic)
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16
Q

What % of all dementias does Alzheimer’s account for?

A

Over 50%

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17
Q

Which neurochemical/neurotransmitter is there a deficit of in Alzheimer’s due to affected neurones not producing it?

A

Acetylcholine

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18
Q

Where is cortical atrophy in Alzheimer’s initially most pronounced (in which lobes)?

A

In the temporal and parietal lobes

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19
Q

What type of plaques build up in Alzheimer’s?

A

Beta-amyloid plaques

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20
Q

Which protein is associated with the neurofibrillary tangles that are coiled abnormally?

A

Tau

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21
Q

What are the risk factors for Alzheimer’s disease (AD)?

A
  1. Increasing age
  2. Female sex
  3. Family history/Genetics (young onset dementia often have a mutation in the amyloid precursor protein gene APP)
  4. Mild cognitive impairment
  5. Cardiovascular disease (diabetes, smoking, hypercholesterolaemia, hypertension)
  6. Parkinsons disease
  7. Depression
  8. Stroke
  9. Heavy alcohol consumption
  10. Low educational attainment
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22
Q

Inheritance of which type of allele of apolipoprotein E on chromosome 19 is a risk factor late-onset forms of Alzheimers?

A

E4

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23
Q

Which allele of apolipoprotein E is protective for Alzheimer’s?

A

E2

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24
Q

What is the life expectancy from the time of diagnosis of AD?

A

8 years

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25
Q

What % of dementias does lewybody (DLB) detention account for?

A

Approximately 20%

26
Q

What is the difference in pathology between AD and DLB?

A

In AD there is cortical atrophy and neurofibrillary tangles, but this isn’t seen in DLB.

27
Q

What is seen in DLB?

A

Lewy bodies are intracellular eosinophilic inclusions consisting of abnormally phosphorylated neurofilament proteins which aggregate. There is also associated neuronal loss leading to cholinergic deficit.

28
Q

What are the clinical features of DLB?

A
  1. Visual hallucinations (and other psychotic symptoms)
  2. Fluctuations in cognitive impairment and alertness
  3. Early parkinsonism
  4. Frequent faints and falls
29
Q

What is the life expectancy from the point o diagnosis of DLB?

A

6 years

30
Q

What is the peak age of onset for Pick’s disease, and in which gender is it most common?

A

45-60 years, more common in females

31
Q

What are the behavioural and psychological symptoms of dementia AKA BPSD? (8)

A
  1. Agitation
  2. Depression
  3. Apathy
  4. Repetitive questioning
  5. Psychosis
  6. Aggression
  7. Sleep problems
  8. Wandering
32
Q

What are all the symptoms that as a combination, result in people with dementia often being institutionalised? (3)

A
  1. Loss of ability to perform ADLs
  2. BPSD
  3. Increasing dependence and lack of appropriate care at home
33
Q

What aspects of a carers life is affected by caring for someone with dementia?

A

Physical, mental, employment, education prospects, finances, and social and community life can all be affected negatively by caring for someone with dementia

34
Q

Summarise the different symptoms specific to each subtype of dementia:

  1. Alzheimers
  2. Vascular
  3. Lewy bodies
  4. FTD (Pick’s)
A
  1. Alzheimer’s - early impairment of episodic memory - memory loss of recent events, repeated questioning and difficulty learning new information
  2. Vascular - stepwise increase in severity of symptoms, may present insidiously with gait and attention problems and changes in personality (hemiparesis/visual field defects may be present)
  3. Lewy bodies - repeated falls, syncope, transient LOC, severe sensitivity to antipsychotics, delusions, hallucinations. (Memory impairment may not be apparent in early stages). Parkinsonian motor features may be present.
  4. FTD (Pick’s) - personality change and behavioural disturbance (such as apathy or social/sexual disinhibition) may develop insidiously. (Memory and perception may be relatively preserved).
35
Q

What is the new form of dementia identified?

A

Late - limbic-predominant age-related TDP-43 encephalopathy

36
Q

What are the biological treatments for the cognitive symptoms of dementia and BPSD?

A

Cognitive:
1. Acetylcholinesterase (AChE) inhibitors - donepezil, galantamine and rivastigmine
2. Memantine (a N-methly-D-aspartic acid reporter antagonist)
(these can be prescribed together)
BPSD:
1. Antipsychotic - risperidone

37
Q

What non-pharmacological treatments can be used to treat BPSD? (6)

A
  1. Aromatherapy
  2. Multisensory stimulation
  3. Exercise
  4. Therapeutic use of music and/or dancing
  5. Animal-assisted therapy
  6. Massage
38
Q

What is the difference in neuropathology between AD and Pick’s disease?

A

Unlike in AD, there are no senile plaques or neurofibrillary tangles, however there are ‘ballooned’ neurones called Pick cells and tau-positive neuronal inclusions called Pick bodies.

39
Q

In huntington’s disease (HD), degeneration of neurons in which area of the brain leads to dementia?

A

Cerebral cortex

40
Q

What are the risk factors for vascular dementia? (9)

A
  1. Male sex
  2. Older age
  3. Cardiovascular disease
  4. Cerebrovascular disease
  5. Valvular disease
  6. Hypertension
  7. Diabetes
  8. Smoking
  9. Alcohol
41
Q

What is the typical onset of vascular dementia like?

A

Rapid/abrupt onset - as often due to TIA or stroke

42
Q

Why are the clinical features of vascular dementia variable?

A

Because it depends on where the infarcts occur in the brain - but often the clinical features include mood and behavioural changes

43
Q

Side effects of acetylcholinesterase inhibitors? (6)

A
  1. Vasodilation
  2. Constriction of pupils
  3. Increased sweating, saliva and tears
  4. Bradycardia
  5. Mucus secretion
  6. Constriction of the respiratory tract
44
Q

Mechanism of memantine?

A

Acts on glutamate system by blocking NMDA receptors, believed to help prevent excess level of glutamate from damaging the brain. Glutamate carries nerve signals, but AD patients have too much glutamate in the brain and this can kill nerve cells

45
Q

What are the three acetylcholinesterase inhibitors used to treat mild to moderate Alzheimer’s?

A
  1. Donepezil
  2. Galantamine
  3. Rivastigmine
46
Q

What is an adjuvant treatment for AD, or for the treatment of moderate-severe AD?

A

Memantine

47
Q

When should donepezil be prescribed with caution or possible contraindicated? (3)

A

People with:

  1. Asthma
  2. COPD
  3. Supraventricular conduction abnormalities
48
Q

What are the common side effects associated with AChE inhibitors? (4)

A
  1. Nausea, vomiting and diarrhoea
  2. Loss of appetite
  3. Muscle pain
  4. Feeling tired/sleeping problems
49
Q

What are the characteristic features of Lewy body dementia?

A
  1. Day-to-day fluctuating levels of cognitive functioning
  2. Visual hallucinations
  3. Sleep disturbance
  4. Transient LOC
  5. Recurrent falls
  6. Parkinsonian features - tremor, hypokinesia, rigidity, postural instability
50
Q

Although people with lewy body dementia are prone to hallucinations, why must antipsychotics be avoided?

A

As they precipitate severe Parkinsonism in 60% of cases

51
Q

What are lewy-bodies and where are they found in someone with Lewy body dementia?

A

Lewy bodies are abnormalities of the cytoplasm found within a neurone, containing various proteins and granular material. They are found in the cerebral cortex in patients with Lewy body dementia, and also found in patients with Parkinson’s disease.

52
Q

What is Creutzfeldt-Jacob disease, and what are the features of the disease?

A

CJD is a rapidly progressive dementia caused by prions. Features include rapid cognitive impairment, which may be preceded by anxiety and depression. Physical features will become prominent including;

  1. Muscle disturbance (rigidity, tremor, wasting, spasticity, fasciculations)
  2. Convulsions
53
Q

What is the prognosis for CJD?

A

Death occurs within 6-8 months

54
Q

What is new variant CJD?

A

It occurs secondary to ingestion of bovine spongiform encephalopathy-infected beef. Decline is slower than for CJD.

55
Q

What are the features of frontotemporal dementia? (5)

A
  1. Disinhibition
  2. Inattention
  3. Antisocial behaviour
  4. Personality changes
  5. Apathy
56
Q

What are Pick’s bodies, and where are they found?

A

They are cytoplasmic inclusion bodies of tau protein, found in the substantia nigra

57
Q

What is the triad associated with normal-pressure hydrocephalus?

A
  1. Dementia (mainly memory problems)
  2. Gait disturbance
  3. Urinary incontinence
58
Q

What causes normal-pressure hydrocephalus?

A

It is due to an increased volume of CSF, but with only a slightly raised pressure (as the ventricles dilate to compensate). There is an underlying obstruction in the SA space that prevents CSF from being reabsorbed but allows it to flow from the ventricular system into the SA space. Diagnosis is by lumbar puncture followed by MRI.

59
Q

How is normal-pressure hydrocephalus diagnosed?

A
  1. Lumbar puncture

2. MRI (showing enlarged ventricles)

60
Q

How is normal-pressure hydrocephalus treated?

A

Ventriculoperitoneal shunting

61
Q

In Huntington’s disease, what are the trinucleotide repeats and which chromosome are they found on?

A

They are CAG repeats found on chromosome 4, and inherited in an autosomal dominant fashion

62
Q

What is the triad of symptoms in Wernicke’s encephalopathy?

A
  1. Confusion
  2. Ataxia
  3. Nystagmus