Aani Micro: PRION & Zoonosis Flashcards
Which chromosome is the prion protein on?
Chr20
Describe the normal and abnormal prion protein?
Alpha helix normal –> Beta sheet abnormal. These Beta sheets aggregate irreversibly
Why is prion hard to treat?
Resistant to radiation and proteases
Which codon puts you at risk of Prion?
Codon 129
What are the types of prion disease?
Sporadic CJD (most common) Acquired CJD (includes: variant CJD, Iatrogenic, Kuru) Inherited (includes familial CJD, GSS, FFI,)
What are the differentiating features of Sporadic CJD?
EEG will have periodic triphasic changes (these are bad)
Protein 14-3-3 will be HIGH
On post-mortem you’ll see Spongiform Vacuolation and PrP amyloid plaques
Tonsilar biopsy is useless
What are the differentiating features of Variant CJD?
PULVINAR SIGN (post thalamus highlighted on MR)
Caused by Mad Cow (bovine spongiforme encephalitis).
Age of onset 30 y/o
Can have normal 14-3-3 protein
TONSILAR BIOPSY is V specific and will show Type 4t
What are the differentiating features of Inherited CJD?
MUTATIONS PRESENT (Diagnostic) Young age of onset 20-60
Which prion disease detection can be done by tonsilar biopsy?
Variant CJD
Which prion disease has a 45 year incubation period?
Kuru (acquired from eating brains)
What is the abnormal prion protein called?
PRP SC
What is the treatment of CJD?
Can’t cure it.
Give clonazepam for myoclonus (symptomatic)
How is brucellosis transmitted?
Cattle
What is brucellosis gram stain?
Negative Aerobic bacilli
Ix for Brucellosis?
Serology: anti-O-polysaccharide Abs
