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)

Annotation

general or unspecified; prefer specifics

všeobecné nebo nevymezené, dej přednost přesnějšímu deskriptoru

DUI

D017096 MeSH Browser

CUI

M0025975

Previous indexing

Slow Virus Diseases (1966-1992)

History note

1993

Public note

1993