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13 Cards in this Set

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  • Back
type of disease caused by prions?
transmissable spongiform encephalopathies (TSE)
human diseases caused by prions?
kuru, creutzfield jacob, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, note bovine spongiform encecphalopathy has transmitted to ppl
describe the neurocytological lesions.
progressive vacuolation in neurons and, to a lesser extent, in astrocytes and oligodendrocytes. Extensive astroglial hypertrophy and proliferation (gliosis), vacuoles in grey matter, some cases have amyloid plaques but not the same ones seen in alzheimers
describe Kuru.
found in a few valleys in Papua New Guinea. Characterized by cerebellar ataxia, shivering like tremor that progresses to complete motor incapacity with dysarthria and total loss of speech, death in less than year from onset of symptoms. Spread by eating brains of dead relatives. this practice has stopped and thus Kuru is no more.
describe creutzfield Jacob.
begins with memory loss, depression, and behavioral changes. Leads to dimentia, hallucinations, myoclonus, cerebellar ataxia, cortical blindness (from damaged occipital lobes), pyramidal (corticospinal) signs like weakness of certain muscles and exaggerated reflexes, extrapyramidal signs like slowness and rigidity. pts usually die wi 6 months of the onset of illness, incidence is about 0.5 to 1 per million ppl per year, peak age is 60 to 65. most are sporadic but a small number are from a familial form that is AD. can be spread by contaminated instruments in neurosurg, tissue transplant of cornea or dura, and preperation of human growth hormone and gonadotropin from infected human pituitaries. believe that kuru came from a sporadic case of this. unique in that it can be spread rapidly, can be inherited, and can be infectious.
describe Grestmann-straussler-scheinker syndrome.
rare subset of inherited CJD, with younger age of onset and illness evolves more slowly
describe familial fatal insomnia.
inherited, get it at 20 to 70 yrs, duration of 6 to 30 months, inability to sleep, autonmonic dysfunction, myoclonus, ataxia, and late dementia. May not be any spongiform degeneration
describe bovine spongiform enceph.
mad cow disease, probly from food that had bovine remains in it, reminiscant of Kuru
prion resistance to UV radiation, ionizing radiation, and heat cause what problem?
can make some surgical instruments infectious
things we know about the prion protein itself?
forms rods, no nucleic acid, comes from a gene that is normally within our cells, normal cellular protein is PrPc, amyloid plaques of scrapie brains are composed of the proteins, ppl with familial diseases have inherited mutations in their PrP gene
stuff we do not know about prions.
how does it cause disease? How is the prion protein derived from the normal cellular protein PrPc and how does it replicate?
can BSE cause human disease?
they think so, call it variant CJD, only 166 have got it and they all died except 5, this was before safe bovine practices were implimented
differences in sporadic CJD and vCJD?
average age of pts was 27, time bw disease and death varied from 7 to 22 months, EEG findings were atypical but different than sporadic CJD, extensive plaque formation (in sporadic, only 5% had plaques) with much more prion protein, plaques surrounded by spongiform lesions