Prions

Question 1

Kuru: syndromes, etiology, duration til death

Answer 1

Ataxia, myoclonus then dementia
Infectious
Months

Question 2

sCJD, fCJD, iCJD

Answer 2

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

Question 3

vCJD

Answer 3

Psychiatric changes, ataxia, demetia
Infectious
Months to few years

Question 4

FFI

Answer 4

Sleep disturbances and then demetia
Inherited
1 year

Question 5

GSS

Answer 5

ataxia followed by dementia
Inherited
Few years

Question 6

All vCJD cases have what DNA similarities?

Answer 6

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

Question 7

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

Answer 7

Histopathologic examination and staining for PrPSc in brain tissue

Question 8

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

Answer 8

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

Question 9

What are the shared characteristics of prion diseases?

Answer 9

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