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Flashcards in L39_Prions Deck (18):
1

What is the most common prion disease in humans?

CJD

2

What type of CJD is the most common?

sporadic or sCJD

3

Can sCJD be transmitted person to person by blood transfusion, what about vCJD?

sCJD-NO
vCJD-YES

4

Is sCJD related to human mad cow disease?

no

5

What main part of the brain does sCJD infect?

Cerebrum

6

What are the two cardinal symptoms of sCJD?

RAPID progressive mental deterioration and myoclonus (muscle twitching), myoclonus may have later onset

7

What is the normal form of the prion protein and what is the pathogenic form, what is the difference in their structure? What gene encodes the normal one.

The normal protein PRPc is encoded by the Prnp gene and is alpha helical. The pathogenic protein PRPsc is Beta sheet and can arise from either a misfolded PrPc or an exogenous source

8

How many classes of PrPsc and how are they differentiated?

4, differentiated by western blot (ratio of 3 bands)

9

What are the two classes of CJD that are most focussed on, which one is sCJD and which is vCJD?

Class 1 is sCJD and class 4 is vCJD

10

What is the only way to diagnose CJD?

Brain biopsy

11

What are two other diagnostic findings that can support sCJD?

abnormal MRI and EEG

12

What are the biopsy findings of sCJD?

Accumulation of PRPsc
Neuronal loss without inflammation
Spongiform Change

13

Are there treatments for sCJD?

NO, you're fucked (5-6mo - 1 yr)

14

What demographic tends to suffer the most form sCJD?

Elderly, 65 and above
vast majority of deaths in in whites

15

With regard to vCJD, do muscle and milk of a cow contain BSE?

No, but if they are contaminated with it, you can get it

16

What are some major differences between sCJD and vCJD

Loss of brain function is slower in vCJD, Duration of vCJD is longer, vCJD has peripheral pathogenesis distinct from classical sCJD with prominent involvement of lymphoreticular tissues

17

What is the easiest way to differentiate between sCJD and vCJD?

Brain biopsy, sCJD has a more diffuse staining, vCJD consist of large round amyloid core

18

What is the median age of onset for vCJD?

29 years old