Definition: prion diseases

A group of genetic, infectious, or sporadic
degenerative human and animal nervous system
disorders associated with abnormal prions. In
humans, these conditions generally feature
dementia, ataxia, and a fatal outcome. Diseases in
this category include Creutzfeldt-Jakob Syndrome;
scrapie; bovine spongiform encephalopathy; chronic
wasting disease of mule deer and elk; and
transmissible mink encephalopathy. Pathologic
features include a spongiform encephalopathy
without evidence of inflammation.

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