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Flashcards in Prions Deck (17):
1

Fancy name for Prion disease

Transmissable spongiform encephalopathies

2

What are the 4 human prion disease

CJD, kuru, Grerstmann-Staussler-Scheinker disease and fatal familial insomnia

3

What are the classificaitons of CJD

the sporadic sCJD
variant vCJD
familial fCJD
iatrogenic iCJD

4

what are general characteristics fro all prion

long incubation(mo-yrs)
increase in severity
no host immune response
non-inflammatory process in brain
microscopic spongiform changes, neuronal loss, amyloid plaques
accumulation prion protein PrP

5

what is the prion only hypothesis vs viral

prion is only prion proteins invovlved
viral is that may have small part of nucelic acid

6

Where is PrP encoded in human genome

PRNP on chromosome 20

7

What are the forms of the PrP

PrPc for cellular is normallly expressed
PrPsc for scrapie is assoc with disease

8

What is the therory about PrPsc

presence of it causes other PrPc to switch to PrPsc, spreading the disease
infection caused, inherited, or sporadic

9

What is the SRM material that is now banned in the meet industry

lymphatic tissue, tonsils, neural tissue brain SC dorsal ganglia...

10

What were the iatrogenic CJD caused by

surgical instruments, dural and corneal grafts, admin of cadaveric pituitary hormones

11

All cases of human vCJD have what mutation

homozygous methionine at 129 on PRNP gene

12

What are the signs of v CJD

rapidly progressing dementia
psychiatric symptoms
cerebellar symptoms
involuntary movements
ultimately fatal

13

What is the gold standard Dx for prion disease

histopathologic examination and immunostaining for PrPsc of brain tissue

14

What does CSF look lik ein prion disease

midly elvated protein levels
tau proteins for vCJD
14-3-3 for sCJD

15

What are the MRI findings in prion disease

hockey stick sign
dorsomedial thalamic hyperintensity
pulvinar sign in vCJD

16

What EEG is seen in sCJD

periodic sharp and slow wave complexes

17

What test has high sensitivity and specificity for vCJD in early stages

PrPsc in lymphoreticular tissue
tonsil biopsy