T1 L15: Dementia - Neurobiology and clinical aspects Flashcards Preview

2.1 T1: Fundamental Neuroscience and the Brain > T1 L15: Dementia - Neurobiology and clinical aspects > Flashcards

Flashcards in T1 L15: Dementia - Neurobiology and clinical aspects Deck (34)
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1
Q

What is the prevalence of different types of dementias?

A
50-60% Alzheimer's disease
15% Vascular dementia 
10-15% Lewy body dementias 
1-2% Frontotemporal lobar degeneration
Mixed cases
alcohol related (ARBI)
2
Q

What are the presenting symptoms of dementia?

A
Forgetfulness
Difficulty learning new things 
Giving up hobbies and interests
Getting muddled in new situations
Difficulty finding words 
Difficulty handling bills
Social withdrawal
Personality change 
Circadian rhythm disturbance
Weight loss
Poorly controlled medical conditions
3
Q

What are some Microscopic changes in the brain due to dementia?

A
  • Intraneuronal inclusion bodies (Lewy bodies)

- Extra neuronal changes (Amyloid-beta plaques)

4
Q

What are some macroscopic changes in the brain due to dementia?

A
  • Region specific atrophy
  • Generalised brain atrophy
  • Enlarged ventricles
5
Q

Which structure is one of the first to shrink with Alzheimer’s?

A

The hippocampus

6
Q

Which proteins create the neuropathological mechanism of dementia?

A

Beta-amyloid, Tau, TDP43, FUS, alpha-synuclein, and prion protein

7
Q

What is the pathophysiology behind Alzheimer’s disease?

A

Beta-amyloid and Phosphorylated Tau cause cell loss, plaques, and tangles that impact neurotransmitters and result in cognitive deficits

8
Q

What are the 2 types of products from the amyloid precursor protein and what is their clinical significance?

A

Alpha-secretase and beta-secretase create alpha and beta amyloids.

Only the beta-amyloids go on to from amyloid plaques

9
Q

What is the Tau pathology behind Alzheimer’s disease?

A

The formation of it is normal but during phosphorylation, Tau will detach from the microtubules. Hyperphosphorylation of Tau will therefore cause accumulation of Tau-P that destabilises microtubules

10
Q

What is Braak staging used for?

A

To classify the degree of pathology in Parkinson’s disease and Alzheimer’s disease

11
Q

What is the nucleus Basalis?

A

A somewhat diffuse collection of large cholinergic neurons in the basal forebrain

12
Q

What are some risk factors for Alzheimer’s?

A

Age, Vascular risk factors, lack of exercise, low level of education, diet, family history, genetics

13
Q

What is familial Alzheimer’s disease?

A

Mutation in 3 genes that causes Alzheimer’s disease

14
Q

What are some atypical presentations of Alzheimer’s disease?

A

Posterior cortical atrophy (PCA), Logopenic aphasia, behavioural (frontal) Alzheimer’s disease

15
Q

What are some typical presentations of Alzheimer’s disease?

A
Impaired episodic memory
Visuospatial dysfunction
Word-finding difficulties
Visual perceptual/spatial difficulties
Apraxia
Difficulties in arrhythmic
Difficulties reading/spelling 
Apathy 
Behavioural problems
16
Q

What are alpha-synucleinopathies?

A

Lewy bodies

17
Q

What is Lewy body dementia?

A

Dementia and Parkinson’s disease together

18
Q

Where are Lewy bodies found in patients?

A

Found in dopaminergic neurons of the substantia nigra

19
Q

What is the gene behind familial Parkinson’s disease?

A

alpha-synuclein gene

20
Q

Where is alpha-synuclein found?

A

Localised in the cytosol of mitochondria

21
Q

What are some clinical features of Lewy body dementia?

A
  • Progressive cognitive decline like in ordinary dementia
  • Disproportionate attentional, executive, and visual processing deficits related to memory and naming
  • REM sleep behaviour disorder
  • Spontaneous features of Parkinsonism
  • Recurrent visual hallucinations, typically well formed and detailed
22
Q

What are some clinical presentations of Frontotemporal Lobar Degeneration (FTLD)?

A

Classical syndromes:
Behavioural variant
Progressive non-fluent aphasia
Sematic dementia

Motor disorders:
Parkinsonian syndromes
Motor neurones disease

These are all diseases affecting the frontal temporal lobe

23
Q

What are the 3 main pathological proteins behind Frontotemporal Lobar Degeneration (LTLD)?

A

Tau, TDP43, and FUS

24
Q

What is the Fazekes scale?

A

Used to quantify the amount of white matter T2 hyperintense lesions usually attributed to chronic small vessel ischaemia

It can be used to measure vascular dementia progression

25
Q

What is Vascular dementia?

A

It’s not a neurodegenerative dementia. It’s caused by vascular pathologies that interact with neurodegenerative changes

It can be caused as a result of ischaemic or haemorrhagic brain damage

26
Q

What are the 3 most common mechanisms behind vascular dementia?

A
  • Single, strategically placed infarcts
  • Multiple cortical infarcts
  • Subcortical small-vessel disease
27
Q

What causes Huntington’s disease?

A

It’s an autosomal dominant mutations in the Huntingtin gene on chromosome 4

28
Q

What are some early symptoms of Huntington’s disease?

A
Chorea
Anxiety
Depression
Apathy
Irritability
Disinhibition
Psychosis
Compulsive behaviour
29
Q

What are the 2 types of prion?

A

Sporadic creutzfeldt-jakob disease that develops within the body

And variant creutzfeldt-jakob disease that is ingested

30
Q

When is the peak age of onset for sporadic CJD?

A

55-75 years

31
Q

What is the pathology behind CJ disease?

A

The transformation of the normal cellular prion protein PrP into an abnormal, structurally changed, disease-causing form

32
Q

How is CJ disease diagnosed?

A
History
Examination
Blood screen
cognitive screen
neurological examination
neuroimaging
fluid biomarkers
33
Q

What is the treatment for creutzfeldt-jakob disease?

A

There is no cure.

Only some symptoms can be treated Eg. psychiatric drugs

34
Q

What are some ways of preventing dementia?

A

Minimise diabetes, treat hypertension, prevent head injury, stop smoking, reduce air pollution, reduce midlife obesity - to reduce neuropathological damage

Exercise, reduce occurrence of depression, avoid excessive alcohol, treat hearing impairments, maintain social contact, and attain high level of education - to maintain and increase the cognitive reserve