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Flashcards in Prions Deck (9):
1

Kuru: syndromes, etiology, duration til death

Ataxia, myoclonus then dementia
Infectious
Months

2

sCJD, fCJD, iCJD

demetia, myoclonus, then ataxia
Unknown, inherited, and infectious
1 to a few years

3

vCJD

Psychiatric changes, ataxia, demetia
Infectious
Months to few years

4

FFI

Sleep disturbances and then demetia
Inherited
1 year

5

GSS

ataxia followed by dementia
Inherited
Few years

6

All vCJD cases have what DNA similarities?

All of the vCJD cases were homozygous for a methionine at codon 129 for the PRNP gene

7

What is the gold standard to test for a prion disease?

Histopathologic examination and staining for PrPSc in brain tissue

8

What are the two forms of PrP protein and what populations generally express each?

PrPc = cellular = normal populations
PrPSc = scrapie = disease state

9

What are the shared characteristics of prion diseases?

Long incubation time = months to years
Gradual in crease in severity before death
No host immune response
Non-inflammatory process in the brain
Neuro findings = macro is normal but micro = spongiform changes, neuro loss, amyloid plaques and accumulation of PrP