Other terms
Prion Disease,Encephalopathies, Spongiform, Transmissible,Transmissible Spongiform Encephalopathies,Prion-Induced Disorder,Disorder, Prion-Induced,Disorders, Prion-Induced,Prion Induced Disorder,Prion-Induced Disorders,Spongiform Encephalopathies, Transmissible,Encephalopathies, Transmissible Spongiform,Encephalopathy, Transmissible Spongiform,Spongiform Encephalopathy, Transmissible,Transmissible Spongiform Encephalopathy,Transmissible Dementias,Dementias, Transmissible,Dementia, Transmissible,Transmissible Dementia,Prion Protein Diseases,Prion Protein Disease,Human Transmissible Spongiform Encephalopathies, Inherited,Inherited Human Transmissible Spongiform Encephalopathies
Description
Prion Diseases: 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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