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