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prionózy

Subject h.	prionózy
Subject h.	Prion Diseases
Entry terms	choroby priónové encefalopatie spongiformné prenosné encefalopatie spongioformné prenosné encefalopatie špongioformné prenosné demencie prenosné
English X references	Dementias, Transmissible Spongiform Encephalopathies, Transmissible Transmissible Dementias
Scope note in English	A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)
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subject heading

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