Prions Flashcards
Fancy name for Prion disease
Transmissable spongiform encephalopathies
What are the 4 human prion disease
CJD, kuru, Grerstmann-Staussler-Scheinker disease and fatal familial insomnia
What are the classificaitons of CJD
the sporadic sCJD
variant vCJD
familial fCJD
iatrogenic iCJD
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
what is the prion only hypothesis vs viral
prion is only prion proteins invovlved
viral is that may have small part of nucelic acid
Where is PrP encoded in human genome
PRNP on chromosome 20
What are the forms of the PrP
PrPc for cellular is normallly expressed
PrPsc for scrapie is assoc with disease
What is the therory about PrPsc
presence of it causes other PrPc to switch to PrPsc, spreading the disease
infection caused, inherited, or sporadic
What is the SRM material that is now banned in the meet industry
lymphatic tissue, tonsils, neural tissue brain SC dorsal ganglia…
What were the iatrogenic CJD caused by
surgical instruments, dural and corneal grafts, admin of cadaveric pituitary hormones
All cases of human vCJD have what mutation
homozygous methionine at 129 on PRNP gene
What are the signs of v CJD
rapidly progressing dementia psychiatric symptoms cerebellar symptoms involuntary movements ultimately fatal
What is the gold standard Dx for prion disease
histopathologic examination and immunostaining for PrPsc of brain tissue
What does CSF look lik ein prion disease
midly elvated protein levels
tau proteins for vCJD
14-3-3 for sCJD
What are the MRI findings in prion disease
hockey stick sign
dorsomedial thalamic hyperintensity
pulvinar sign in vCJD
