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)

Descriptor ID	D017096
MeSH Number(s)	C10.228.228.800 C10.574.843
Concept/Terms	Prion Diseases Prion Diseases 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 Human Transmissible Spongiform Encephalopathies, Inherited Inherited Human Transmissible Spongiform Encephalopathies

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