Prion disease - Basic science (Dr Wille) Flashcards
What is the first prion disease that was described?
Scrapie
Is scrapie transmissible to humans?
No. Only contagious to sheep and goats; forms holes in their brains
What are the 3 manifestations of human prion disease?
- Sporaic: Creutzfeldt-Jakob disease (1920s)
- Inherited: Germlines mutation in PRNP gene (usually dominant)
- Infection: Transmitted via canibalism, medical procedures, consumption of infected fppd
What is most common of human prion disesae?
Sporadic (90%); familial (10%); infectious (1%)
What are the death chances w/ prion disease?
1:5000
What is kuru?
Prion disease trasmitted via ritualistic cannibalism in Fore tribe; discovered by Dr. Carleton Gajdusek;
Slow disease (5-50 years incubation); now extinct
What is bovine spongiform encephalopathy?
- Caused by feeding meat and bone meal to cattle (industrial cannibalism) –> mad cow’s disease
- No contamination to pastures like scrapie
- Three strains: classical; atypical and genetic
- Classical is transmitted to humans
What is the diff between sporadic CJD and variant CJD?
Sporadic affects elderly; variant affects younger
How many people showed signs of abnormal PrP deposits?
Apenndixes showed 1:2000 in Great Britain; high prevalence
Tonsils and appendix are where prions show up first
Where is chronic wasting disease found?
Deer, elk moos
Does CWD transmit via tissue, feces, saliva, urine?
YES. It is the most infectious prion disease we know
Slide 17
TRUE or false: are caribou threatened?
Yes, but it has not been trasnmitted yet
TRUE or False: Cooking reduces infectivity substantially
False, only marginally
Describe the overall human-human transmissions:
- Kuru: very efficient, not a problem anymore
- Iatrogenic CJD: very efficient, may be problem in UK
Describe the overall cattle-human transmissions:
BSE: Primary transmission relatively inefficient, incubation period uncertain; documented secondary transmirrion (eg blood transfusion)