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)

Synonyms: Disorder, Prion-Induced, Prion-Induced Disorders, Human Transmissible Spongiform Encephalopathies, Inherited, Transmissible Spongiform Encephalopathies, Encephalopathies, Spongiform, Transmissible, Prion-Induced Disorder, Encephalopathy, Transmissible Spongiform, Transmissible Spongiform Encephalopathy, Dementias, Transmissible, Disorders, Prion-Induced, Spongiform Encephalopathy, Transmissible, Prion Protein Diseases, Prion Diseases, Transmissible Dementia, Dementia, Transmissible, Transmissible Dementias, Spongiform Encephalopathies, Transmissible, Inherited Human Transmissible Spongiform Encephalopathies, Prion Protein Disease, Prion-Associated Disorders, Prion Disease, Encephalopathies, Transmissible Spongiform, Prion Induced Disorder