Id http://uri.neuinfo.org/nif/nifstd/birnlex_12686
http://uri.neuinfo.org/nif/nifstd/birnlex_12686
Preferred Name

Prion Disease

Definitions
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 (MeSH).
Synonyms
Transmissible Dementia
Transmissible Spongiform Encephalopathy
Prion-Induced Disorder
Prion Protein Disease
Type http://www.w3.org/2002/07/owl#Class
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