L39_Prions Flashcards

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1
Q

What is the most common prion disease in humans?

A

CJD

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2
Q

What type of CJD is the most common?

A

sporadic or sCJD

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3
Q

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

A

sCJD-NO

vCJD-YES

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4
Q

Is sCJD related to human mad cow disease?

A

no

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5
Q

What main part of the brain does sCJD infect?

A

Cerebrum

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6
Q

What are the two cardinal symptoms of sCJD?

A

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

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7
Q

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.

A

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

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8
Q

How many classes of PrPsc and how are they differentiated?

A

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

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9
Q

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

A

Class 1 is sCJD and class 4 is vCJD

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10
Q

What is the only way to diagnose CJD?

A

Brain biopsy

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11
Q

What are two other diagnostic findings that can support sCJD?

A

abnormal MRI and EEG

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12
Q

What are the biopsy findings of sCJD?

A

Accumulation of PRPsc
Neuronal loss without inflammation
Spongiform Change

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13
Q

Are there treatments for sCJD?

A

NO, you’re fucked (5-6mo - 1 yr)

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14
Q

What demographic tends to suffer the most form sCJD?

A

Elderly, 65 and above

vast majority of deaths in in whites

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15
Q

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

A

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

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16
Q

What are some major differences between sCJD and vCJD

A

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
Q

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

A

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

18
Q

What is the median age of onset for vCJD?

A

29 years old