Prion Diseases

Prionsjukdomar

Engelsk definition

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)

Svenska synonymer

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Engelska synonymer

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