Dementia Flashcards

1
Q

What is dementia?

A

A condition characterised by evidence of significant cognitive decline in at least 1 cognitive domain (attention, executive, learning and memory, language, perceptuo-motor (ie praxis) or social cognition, interfering with independence and unexplained by any other process

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the main environmental risk factor of dementia?

A

Ageing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are some genetic causes of dementia?

A

ApoE4 in Alzheimer’s increases risk

Huntington’s disease is a monogenetic inherited dementia

Autosomal dominant Alzheimers caused by Presenilin 1 and 2 mutations

Trisomy 21 increases Alzheimers risk between 3rd and 4th decade due to 3 copies of the APP gene on chromosome 21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are some forms of potentially treatable dementia?

A
  • Metabolic e.g. uraemia
  • Toxic e.g. alcohol
  • Vitamin deficiency - B12and thiamine
  • Traumatic - severe or repeated brain injury
  • Intracranial lesions e.g. subdural haematoma, tumours
  • Infections e.g. HIV
  • Endocrine e.g. hypothyroidism
  • Psychiatric - depression causing ‘pseudodementia’
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the main forms of dementia?

A

Alzheimer’s disease
Frontotemporal dementia
Vascular dementia
Lewy-body dementia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the most common form of dementia?

A

Alzheimers disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How does having a 1st degree relative with AD increase risk of AD?

A

It doubles it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is AD occurring in those under 65 known as?

A

Early onset AD (Possibly atypical or genetic)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is AD occurring in those over 65 known as?

A

Sporadic AD (Mostly environmental)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the 2 main atypical sub-types of AD?

A
  • Posterior cortical atrophy (Terry Pratchet)
  • Progressive primary aphasia (Bruce Willis) - A form of frontotemporal dementia, but can cause an atypical form of AD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Describe the pathophysiology of AD

A

Alzheimer’s disease is a neurodegenerative proteinopathy, in which there is a build up of Amyloid-ß proteins

This causes the formation of:

  • Extracellular amyloid plaques
  • Intracellular neurofibrillary tangles

These cause disruption to cholinergic pathways in the brain and synaptic loss, causing cognitive impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What do extracellular amyloid plaques cause in the brain?

A

Disruption of normal cell functional and induced apoptosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What do intracellular neurofibrillary tangles cause in the brain?

A

Disruption of the cell cytoskeleton leading to cell death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe the pathway that AD symptoms usually take?

A

Alzheimer’s disease usually presents initially with general forgetfulness, caused by degeneration of the medial hippocampus and later parietal lobes

This can then progress into apraxia and visuospatial difficulties

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the main symptoms of AD?

A
  • Gradual onset, decline of particularly short-term memory
  • Autobiographical and political memory often well preserved
  • Poor concentration, poor sleep, low mood
  • Personality change - disinhibited, aggression, lack of self-care
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are some end stage symptoms of AD?

A

hallucinations, poor dentition, skin ulcers, loss of verbal communication

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is a symptom of posterior cortical atrophy (Sub-type of AD)?

A

Visuospatial disturbance

18
Q

What investigations are required in AD?

A
  • MRI: Atrophy of temporal / parietal lobes
  • SPECT: temporoparietal ↓metabolism
  • CSF : ↓amyloid : ↑ tau ratio
  • Research: amyloid ligand imaging
19
Q

How is AD managed?

A

Address vascular risk factors

ACh boosting treatments:
- Cholinesterase inhibitors (eg Rivastigmine / Donepezil)
- NMDA receptor blocker (eg Memantine)
- Amyloid monoclonal (Lecanemab – not NICE/SMC approved)

20
Q

Describe the pathophysiology of frontotemporal dementia

A

Frontotemporal dementia is a neurodegenerative proteinopathy, in which there is a build up of Tau or TDP-43 proteins in the brain

Specific degeneration/atrophy occurs in the frontal and temporal lobes of the brain

21
Q

What are the 3 syndromes of frontotemporal dementia?

A

Behavioural variant
Primary progressive aphasia
Semantic dementia

22
Q

What are some symptoms of behavioural variant frontotemporal dementia?

A

Behavioural changes
Executive dysfunction
Disinhibition
Impulsivity
Loss of social skills
Apathy
Obsessions
Change in diet

23
Q

What are some symptoms of primary progressive aphasia?

A
  • Logopenic aphasia (repeating)
  • Non-fluent aphasia (effortful)
    Effortful non-fluent speech, speech sound/articulatory errors, lack of grammar, lack of words
24
Q

What are some symptoms of semantic dementia?

A

Impaired understanding of meaning of words, fluent but empty speech
Difficulty retrieving names

25
Q

What investigations are required in frontotemporal dementia?

A
  • MRI: Atrophy of frontotemporal lobes
  • SPECT: Frontotemporal ↓metabolism
  • CSF: ↑ tau / normal amyloid
26
Q

What are some management options for frontotemporal dementia?

A
  • Trial of trazadone/antipsychotics to help behavioural features
  • Safety management - controlled access to food/money/internet, structured activities
  • Power of attorney
  • Environmental / safety management
  • Controlled access to food / money / internet
  • Structured activities
  • Support: MND nurse specialist if co-existent MND / CPN
27
Q

What causes vascular dementia?

A

Brain damage due to cerebrovascular disease: either major stroke, multiple smaller unrecognised strokes (multi-infarct) or chronic changes in smaller vessels (subcortical dementia)

28
Q

What are some symptoms of vascular dementia?

A
  • Dysphasia
  • Dyscalculia
  • Frontal lobe symptoms
  • Affective symptoms (more common than in Alzheimers)
29
Q

Describe the pattern of decline in vascular dementia

A

Stepwise decline

30
Q

What are some symptoms of subcortical (Small vessel) vascular dementia?

A

↓ attention, executive dysfunction, and slowed processing

31
Q

What are the core criteria for vascular dementia diagnosis?

A
  1. Presence of cerebrovascular disease plus
  2. A clear temporal relationship between the onset of dementia and cerebrovascular disease
32
Q

How is vascular dementia managed?

A

Manage vascular risk factors +/- cholineresterase inhibitor

33
Q

What causes Lewy-body dementia?

A

Lewy-body dementia is a neurodegenerative proteinopathy, in which there is a build up of Alpha-synuclein proteins in the neurons of the brain stem and neocortex

34
Q

How does alpha-synuclein cause dementia in Lewy-body dementia?

A

α-synuclein aggregates are insoluble and so lead to cell dysfunction and therefore cell damage

This leads to disruption of cholinergic and dopaminergic pathways

35
Q

What are some early symptoms of Lewy-body dementia?

A

Common early involvement of reduced attention, executive function and visuospatial skills

36
Q

What are some other symptoms of Lewy-body dementia?

A
  • Visual hallucinations
  • Fluctuating cognition (delirium-like)
  • REM sleep behaviour disorder
  • Extrapyramidal features (Parkinsonism) seen in 75% (Not more than 1 year prior to onset of dementia)
37
Q

What are the core criteria for diagnosis of Lewy-body dementia?

A
  1. Fluctuating cognition plus
  2. Recurrent well-formed visual hallucinations +/-
  3. Presence of extrapyramidal features (seen in 75%)
38
Q

What are some investigations for Lewy-body dementia?

A

DaT (dopamine transporter imaging) if atypical features

New (research) techniques: αsynuclein ligand imaging / αsynuclein in CSF

39
Q

How is Lewy-Body dementia managed?

A

Small dose Levodopa /↓ acetylcholine (amongst other neurotransmitters) → trial cholinesterase inhibitors

Support: PD nurse specialist / CPN

40
Q

What are some generalised investigations for suspected dementia?

A

CT (Standard)
MRI (If young or fast progressing)
Bloods (Dementia screen)
SPECT
DAT

41
Q

What are some blood tests involved in the dementia screen?

A

B12, TFTs, syphilis, HIV, Ca2+

42
Q
A