Neuropathology - Degeneration Flashcards

1
Q

What is demyelination?

A

preferential destruction of myelin sheath around an axon

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2
Q

What cells are damaged in demyelination?

A

oligodendrocytes

schwann cells

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3
Q

What cells produce myelin in the CNS?

A

oligodendrocytes

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4
Q

What cells produce myelin in the PNS?

A

Schwann cells

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5
Q

What is the importance of myelin?

A

insulates nerves and allows rapid conduction of electrical impulses along cell membranes

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6
Q

What condition is an example of primary demyelination?

A

Multiple Sclerosis

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7
Q

Is MS more common in females or males?

A

2:1 females

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8
Q

How does MS commonly present?

A

episodes of neurological deficit separated by time and body site.

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9
Q

What are some common manifestations of MS?

A

visual disturbance
spasticity
speech disturbance
gait abnormalities

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10
Q

MS affects grey matter in the brain - T/F?

A

False - white matter!

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11
Q

What type of scan is used to diagnose MS?

A

MRI Scan

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12
Q

What are the main features seen on histology in a patient with MS?

A

demyelination
inflammation
gliosis

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13
Q

What is dementia?

A

impairment of previously acquired occupational or social functioning due to acquired cognitive impairment with normal consciousness

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14
Q

Dementia is not always a pathological process - T/F?

A

False - always pathological

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15
Q

What are the two groups of dementias?

A

primary

secondary

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16
Q

Name some primary dementias?

A

AD
LBD
HD
Picks disease

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17
Q

Name some secondary dementias?

A

vascular
metabolic
infection/trauma

18
Q

Which is the most common dementia?

A

Alzheimer’s disease

19
Q

Which common genetic syndrome carries an increased risk of AD?

A

Down’s Syndrome - trisomy 21

20
Q

What macroscopic pathology is seen on the brain?

A
cortical atrophy 
widened sulci 
narrowed gyri 
dilated ventricles 
normal brainstem and cerebellum
21
Q

What microscopic features of dementia are there?

A

neurofibrillary tangles
B-amyloid plaques
amyloid angiopathy
neuronal loss

22
Q

What stain is used to show up amyloid protein?

A

congo red stain

23
Q

What two types of amyloid protein are there?

24
Q

What are the hallmarks of Lewy Body dementia?

A

progressive dementia
hallucinations
fluctuating attention levels

25
What protein is stained for in the identification of lewy bodies?
ubiquitin
26
What is the common triad of Huntington's disease symptoms?
emotional cognitive motor disturbance
27
What is the movement disorder in HD known as?
chorea
28
When does dementia occur in HD?
later in disease
29
What is the pattern of inheritance in HD?
autosomal dominant
30
Which chromosome is the huntingtin gene on?
Chromosome 4p
31
What is seen on HD histology?
loss of caudate nucleus neurones | reactive gliosis
32
What is Pick's disease?
a progressive dementia commencing in middle life characterised by changes in character and social deterioration leading to cognitive impairment
33
Where is extreme atrophy seen in a Pick's Disease brain?
frontal and temporal lobe cortices
34
What are Pick's cells?
swollen neurons
35
What are Pick's bodies?
intracytoplasmic filamentous inclusion
36
How long will a person usually live after a diagnosis of Pick's disease?
7 years average (2-10)
37
Which is the most common secondary dementia?
vascular dementia
38
What is multi-infarct dementia?
deterioration in cognitive function due to hypoxia induced brain damage as a result of multiple blood clots.
39
Which cardiovascular conditions are particularly important in multi-infarct dementia?
hypertension hyperlipidaemia diabetes previous stroke
40
What type of progression is seen in multi-infarct dementia?
stepwise progression