Dementia Flashcards

1
Q

Define: apraxia

A

Difficulty performing motor tasks

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2
Q

Define: spastic paresis

A

Muscle hypertonia and exaggerated tendon reflexes

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3
Q

Which language impairment is associated with Broca’s area?

A

Expressive aphasia

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4
Q

Which language impairment is associated with Wernicke’s area?

A

Receptive aphasia

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5
Q

Which area of the brain is associated with expressive aphasia?

A

Broca’s area (frontal lobe - near the pre-central gyrus (which controls motor functioning))

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6
Q

Which area of the brain is associated with receptive aphasia?

A

Wernicke’s area (posterior temporal lobe, overlaps with parietal lobe)

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7
Q

What are the functions of the frontal lobe?

A
Judgement
Reasoning
Behaviour
Voluntary movements
Broca's area (expressive language)
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8
Q

What are the functions of the parietal lobe?

A

Spacial orientation
Perception
Initial cortical processing of tactile and proprioception
Wernicke’s area (language comprehension)

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9
Q

What are the functions of the temporal lobe?

A
Emotions
Learning and memory
Auditory
Olfaction
Wernicke's area (language comprehension)
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10
Q

What are the functions of the occipital lobe?

A

Vision

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11
Q

What are the principal clinical features of dementia (in order of progression in AD)?

A
  1. Memory loss
  2. Impaired thinking
  3. Language impairments
  4. Deterioration of personal functioning
  5. Disturbed personality and behaviour
  6. Perceptual abnormalities
  7. Motor impairments
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12
Q

What is syllogomania?

A

Compulsive hoarding of rubbish (an example of deterioration in personal functioning associated with dementia. Associated with Diogenes syndrome).

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13
Q

What is prosopagnosia?

A

Inability to recognise faces (perceptual abnormality associated with dementia)

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14
Q

What is dementia pugilistica?

A

Boxer’s dementia - a form of chronic traumatic encephalopathy caused by repeated concussive/sub-concussive blows to the head. Average onset is ~12-16 years after beginning a career in boxing, affects 15-20% of boxers.

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15
Q

What is the triad of symptoms associated with Normal Pressure Hydrocephalus?

A

Gait disturbance/ataxia
Dementia
Urinary incontinence

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16
Q

What is the prevalence of Alzheimer’s disease?

A

1% at 65 years old.
30-40% at 90 years old
(n.b. prevalence doubles every 5y post 65 - makes up 50% of all dementias, v common)

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17
Q

Describe the main neuropathology seen in Alzheimer’s disease.

A
Neuronal synaptic loss (leads to cholinergic deficit)
Symmetrical cortical atrophy
Senile plaques (core of B-amyloid)
Neurofibrillary tangles (Tau protein - which is also seen in Pick bodies)
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18
Q

What is the role of the cholinergic system in Alzheimer’s disease?

A

Acetylcholine (ACh) is an NT found in the autonomic nervous system (symp/parasymp, all parasympathetic organs), PNS at the NMJ (between motor neurons and skeletal muscle), and in the CNS.

In Alzheimer’s disease, there is a marked decrease in ACh concentration in the cerebral cortex and caudate nucleus. The deterioration of cholinergic innervation of the neocortex is associated with the memory loss in AD (muscarinic receptors are important mediators of behaviour in the CNS).

19
Q

What are the risk factors for Alzheimer’s disease?

A

Age (up to 90), female (2:1), FHx (4x risk), Down’s syndrome, Dialysis, head injury.

20
Q

Why is Down’s syndrome a risk factor for Alzheimer’s disease?

A

Three copies of chromosome 21, which contains the B-amyloid precursor protein (APP) gene.

21
Q

What are the 4 genes associated with Alzheimer’s disease?

A

Inheritance: e4 allele of apolipoprotein E (ch 19) (e2 is protective).
Ass. w/ late onset.

Mutations:
APP gene (B-amyloid precursor protein) and Presenilin 1
Presenilin 2
Ass. w/ rarer early onset.

22
Q

What are the main clinical features of Alzheimer’s disease?

A

MEMORY LOSS and PERSONALITY CHANGES

Memory:
Short term impairment > long term
Leads to disorientation in TTP.

Personality:
Euphoric/labile OR irritable/apathy
Disinhibition, aggression, socially inappropriate
Inattention, distractibility 
Obsessive and stereotyped behaviours
23
Q

What is the neuropathology of dementia with Lewy Bodies?

A

Lewy bodies present. Intracellular eosinophilic inclusions - consisting of abnormally phosphorylated neurofilament proteins, and aggregated with ubiquitin and ALPHA-SYNUCLEIN.
Cortical and subcortical structures affected.

This leads to neuronal loss and cholinergic deficit

24
Q

What are the symptoms of the EARLY STAGES of Alzheimer’s disease?

A
Memory lapses
Forgetting names of people and places
Difficulty finding words for things
Inability to remember recent events
Forgetting appointments
25
Q

What are the symptoms of the LATER STAGES of Alzheimer’s disease?

A

Wandering, disorientation
Apathy
Psychiatric symptoms - depression, hallucinations, delusions
Behavioural problems - disinhibition, aggression, agitation
Altered eating habits
Incontinence

26
Q

What are the symptoms of Alzheimer’s disease AS IT PROGRESSES?

A

Difficulties with language
Apraxia
Problems with planning and decision making
Confusion

27
Q

What is the criteria for diagnosing DLB? (presentation)

A

Presence of dementia.

Plus, two of the three core features:

  1. Fluctuating attention and concentration.
  2. Recurrent well-formed visual hallucinations.
  3. Spontaneous Parkinsonism.
28
Q

What is Diogenes syndrome?

A

Severe senile self-neglect. Often accompanied with syllogomania.

29
Q

What is the neuropathology of Vascular Dementia?

A

Focal disease/Stroke related VaD - from single (big stroke)/more commonly multiple infarcts (lots of little strokes - sometimes unnoticed).
Diffuse disease/Subcortical VaD - small vessel disease leads to diffuse disease (Binswanger’s disease and Lacunar state)

Often coexist 
(Mixed type can also have these changescoexisting with AD)
30
Q

What is a Lacunar state? (VaD)

A

Appearance of small, smooth-walled cavities in the brain

31
Q

What is Binswanger’s disease? (VaD)

A

Small vessel vascular dementia

32
Q

What are the RFs for Vascular Dementia?

A
Hx of stroke/TIA
Older age
M>F
DM
Hypercholesterolaemia
Hypercoaguable states
CVD (including AF)
Obesity / smoking / alcohol
FHx of stroke/CVD
33
Q

What is the presentation of Vascular Dementia (and how is a diagnosis made?)?

A

Presence of dementia (cognitive decline from higher level of functioning)
AND Cardiovascular disease
Relationship between the two, inferred by:
- onset of dementia within 3/12 of recognised stroke
- abrupt deterioration in cognitive function
- fluctuating, stepwise progression of cognitive deficits

34
Q

What is the neuropathology of Frontotemporal Lobe dementia/Pick’s disease?

A

Selective, asymmetrical, ‘knife blade’ atrophy
Get atrophy, neuronal loss and gliosis affecting frontal and temporal lobes.
Characteristic features:
- Pick cells = ballooned neurons
- Pick bodies = Tau-protein positive neuronal inclusions

N.B. no senile plaques/NF tangles as in AD.

35
Q

What is the presentation of FTL/Pick’s disease?

A

Insidious and progressive dementia.
Px at 45-60y

Early and prominent behavioural/personality changes and disinhibition
Executive functioning
Spatial orientation preserved

36
Q

Summarise the aetiology of the main 4 types of dementia.

A

AD - degeneration of cerebral cortex, senile plaques (B-amyloid), NF tangles (Tau), cortical atrophy, decreased ACh production from affected neurons.
DLB - Lewy bodies (alpha-synuclein)
Picks - Pick’s bodies (Tau)
VaD - single/multiple infarcts

37
Q

Summarise the aetiology of Huntington’s disease.

A

Autosomal dominant neurodegenerative disorder - ≥36 CAG repeats encoding glutamine in Huntington’s protein (ch 4)
Anticipation - as number of repeats increases, age of onset decreases (progressively lower w/in generations),

Abn Huntington’s protein leads to degeneration of neurons:

  • caudate nucleus & putamen - movement disorders
  • cerebral cortex - dementia
38
Q

What is the presentation of Huntington’s disease?

A

Choreiform movements (dance-like repeated movements)
Progressive dementia
Psychiatric disturbances (notable early depression)
Behavioural disturbances

39
Q

What is the diagnostic criteria for ALL types of dementia? (NICE)

A

Affect ability to function in normal activities.
Represent a decline from a previous level of function.
Cannot be explained by delirium or other major psychiatric disorder.
Have been established by history-taking from patient and informant, and formal cognitive assessment.
Involve impairment of at least two of the following domains:
- Ability to acquire and remember new information
- Judgement, ability to reason or handle complex tasks
- Visuospatial ability
- Language functions
- Personality and behaviour

40
Q

Which class of drugs can be used to treat AD/DLB?

A
Cholinesterase inhibitors (these dementias have an acetylcholine deficiency)
eg donepezil, rivastigmine
41
Q

How often should a prescription for donepezil/rivastigmine be assessed?

A

Reassess after 2-4/12, and only continue if a demonstratable improvement or lack of decline.
Then reassess 6/12.

42
Q

What is the main difference between delirium and dementia?

A

Delirium has clouding of consciousness, dementia does not.

43
Q

Lesions in the dominant temporal lobe can lead to …

A

Verbal agnosia, visual agnosia, receptive aphasia, hallucinations