Prion Diseases


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)