prions Flashcards
NOT from food
sCJD
linked to contaminated surgical instruments, dural and cornel grafts, and admin of cadaveric pituitary hormones.
Iatrogenic cases of CJD (iCJD)
symp
- Rapidly progressive dementia.
- Psychiatric symptoms.
- Cerebellar symptoms (ataxia).
- Involuntary movements (myoclonic jerks and choreoathetosis).
- Fatal
caused by ritualistic cannibalism.
kuru epidemic
how long vCJD take to kill?
mo- few yrs
psychiatric changes, ataxia, and dementia.
vCJD
how long sCJD, fCJD, iCJD take to kill?
1- a few yrs
Ataxia followed by dementia.
GSS
atxia, myoclonus followed by dementia
kuru
from eating bad beef
vCJD
dementia, myoclonus, and followed by ataxia.
sCJD, fCJD, iCJD
dx
Best option is histopathologic examination and immunostaining for PrP-sc of brain tissue
CSF is normal except with mildly elevated protein levels.
o Tau protein is useful marker for vCJD.
o 14-3-3 protein is a useful marker for sCJD (sporadic CJD).
MRI can show the hockey stick sign (dorsomedial thalamic hypersensitivity) and pulvinar sign in vCJD.
Serial EEG: esp useful for sCJD.
o There is an abnormal wave pattern (periodic sharp and slow wave complexes, PSWC).
o Found in 2/3 of sCJD, but none of vCJD.
Tonsil biopsy looking for PrP-sc is useful as an early diagnostic tool for vCJD.
sleep disturbances followed by dementia
FFI
how long FFI take to kill?
yr
homozygous mutation in met129
vCJD
