Prion Diseases

Question 1

What is the other name for prion diseases, the pathological finding at autopsy?

Answer 1

spongiform encephalopathy

Question 2

Which protein is defective in prion diseases?

Answer 2

PrP

Question 3

What molecular change occurs in prion diseased proteins?

Answer 3

alpha-helices stack and change into beta sheets

Question 4

What are amyloids?

Answer 4

fibrous protein deposits often associated w/ disease but not necessarily containing beta-amyloid

Question 5

What type of amyloid builds up in kuru?

Answer 5

PrP

Question 6

Which stain binds PrP?

Answer 6

congo red

Question 7

Fatal familial insomnia (inherited prion disease) genetic defect

Answer 7

variant in codon 129

Question 8

EEG findings of prion diseases

Answer 8

periodic, sharp wave complexes

Question 9

CSF findings of prion diseases

Answer 9

14-3-3 marker for degeneration

Question 10

MRI findings of CJD

Answer 10

hockey stick caudate

Question 11

What is classic CJD?

Answer 11

familial or sporatic trait that causes CJD in only humans

Question 12

What is variant CJD?

Answer 12

CJD caused by consuming food contaminated with bovine spongiform encephalopathy