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32 Cards in this Set
- Front
- Back
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What is scrapie?
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this is the first identified prion disease.
scrapie caused a sheep to scrap all its wool off (by going bonkers) |
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was scrapie ever transmitted to people?
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no it was not
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what is the prion disease in humans called?
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creutzfeldt-jakob disease
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What do prions do to the brain structure?
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these cause spongiform alterations in the brain (makes spongy holes)
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Who are the only people who can get Kuru?
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Fore tribe of papua new guinea (they get it via diet, as they are cannibals)
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What are the clinical signs of Kuru?
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severe ataxia, dementia, Choreiform movements*
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What does the brain look like in Kuru?
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widespread neuronal loss with amyloid plaques in brain
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How common is CJD?
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1 person per million, per year
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What is the common pt for CJD?
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60's and 70's with sudden dementia
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How can you get infected with CJD?
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conreal transplants, neurosurgery equipment, and pituitary derived growth hormone**
pituitary transplants= very very high prion transfer rate |
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What usually is associated with new variant CJD?
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young pts, associated with bovine spongiform encephalopathy
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What are prions?
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infectious proteins! without any sort of genome
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What do prions actually do to cause disease?
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prions induce a conformational change in pre-existing normal host cells protein of unknown function
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what is PrP?
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this is the prior protein
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What is PrP(c)
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this is the normal form of proteins
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How do prions propagate?
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this propagates by a post translational process, in which PrP(sc)- pathologic....then forces normal PrP(c) into the pathologic PrP(sc) form.
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is there usually a genetic cause for prion disease?
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no there is not, almost always is sporadic form
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What structure shift occurs in prion disease?
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it shifts from alpha helix to beta sheets
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What is elevated in the CSF with prion disease?
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14-3-3 protein will be elevated
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What is the most common form of prion disease?
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the sporadic form. has nothing to do with genetics, more common with old age
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what is the infectious dose of prion disease?
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8 particles...now thats scary
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How did vCDJ originate?
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this was caused by feeding cow parts (meat bone meal) to cows. this produced mad cow disease
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what kinds of cows got mad cow disease?
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dairy cows
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What is PML?
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progressive multifocal leukoencephalopathy
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what virus causes PML?
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the latent JC virus infection
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How many people carry JC virus with them? what is it doing?
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about 70% of people carry JC virus, but it sits latent
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What groups of people commonly have PML?
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AIDS pts
and the elderly |
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Is cognitive dysfunction common with AIDS pts?
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yes it is, caused directly by the HIV virus-
known as AIDS dementia- occurs typically in the end stage |
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What is PML?
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progressive multifocal leukoencephalopathy
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what virus causes PML?
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the latent JC virus infection
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How did vCDJ originate?
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this was caused by feeding cow parts (meat bone meal) to cows. this produced mad cow disease
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what kinds of cows got mad cow disease?
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dairy cows
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