Prion Diseases


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

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