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)
Inga svenska synonymer finns.
Spongiform Encephalopathies, Transmissible — Encephalopathies, Transmissible Spongiform — Encephalopathy, Transmissible Spongiform — Spongiform Encephalopathy, Transmissible — Transmissible Spongiform Encephalopathy — Dementias, Transmissible — Dementia, Transmissible — Transmissible Dementia — Transmissible Dementias — Prion-Induced Disorders — Prion Protein Diseases — Prion Protein Disease — Prion-Induced Disorder — Disorder, Prion-Induced — Disorders, Prion-Induced — Prion Induced Disorder — Prion Disease — Prion-Associated Disorders — Transmissible Spongiform Encephalopathies — Encephalopathies, Spongiform, Transmissible — Human Transmissible Spongiform Encephalopathies, Inherited — Inherited Human Transmissible Spongiform Encephalopathies