Prions

Question 1

Pathology of TSE?

Answer 1

Multifocal spongiform changes*, astrogliosis, neuronal loss, sometimes amyloid, minimal inflammation

Question 2

Prion protein on what chromosome?

Answer 2

20

Question 3

How is PrPc different from PrPSc

Answer 3

Differential folding (alpha helices vs. beta sheets, respectively)

Question 4

T/F – PrPSc is selectively resistant to proteases

Answer 4

T

Question 5

How does PrPSc cause cell death?

Answer 5

Aggregates in intracellular vesicles, causes APOPTOSIS (not necrosis)

Question 6

How does PrPSc amplify misfolding?

Answer 6

Acts as template for folding intermediate to take on its conformation

Question 7

What does the concept of species barrier mean?

Answer 7

Initial infectivity of new species poor, subsequent passage within species is good

Question 8

Is it common for a pathogenic form of PrP to arise spontaneously?

Answer 8

No

Question 9

MC human TSE?

Answer 9

CJD

Question 10

Main clinical features of CJD?

Answer 10

Myoclonus, dementia

Question 11

85% of all CJD cases?

Answer 11

sporadic

Question 12

Inheritance pattern of familial CJD? Difference from sporadic?

Answer 12

AD; younger onset, longer course

Question 13

Specific finding of sporadic CJD?

Answer 13

Periodic complexes on EEG

Question 14

Pathophys of GSS?

Answer 14

AD mutation at codon 102 of PrP

Question 15

Clinical Sx of GSS?

Answer 15

gait/ataxia, atypical but no specific EEG

Question 16

Unique histopath of GSS?

Answer 16

Amyloid

Question 17

Pathophys of FFI?

Answer 17

AD mutations at codons 129 and 178

Question 18

Clinical features of FFI?

Answer 18

Sleep, autonomics

Question 19

Unique histopath?

Answer 19

Neuronal loss w/o spongiform change or plaques

Question 20

T/F – vCJD = BSE?

Answer 20

True

Question 21

Transmission of vCJD?

Answer 21

Contaminated beef AND blood transfusion

Question 22

Pathophys of vCJD?

Answer 22

Homozygous for methionine at codon 129 of PrP

Question 23

Clinical characteristics of vCJD?

Answer 23

Anxiety, depression, visual problems

Question 24

Histopath of vCJD?

Answer 24

numerous FLORID (daisy-like) plaques and changes in basal ganglia