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Concept information

Preferred term

Prion Diseases  

Type

  • mesh:Descriptor

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)

Entry terms

  • Dementias, Transmissible
  • Encephalopathies, Spongiform, Transmissible
  • Prion-Associated Disorders
  • Prion Disease
  • Prion-Induced Disorder
  • Prion-Induced Disorders
  • Prion Protein Diseases
  • Spongiform Encephalopathies, Transmissible
  • Transmissible Dementias
  • Transmissible Spongiform Encephalopathies

In other languages

  • French

  • Démences transmissibles
  • Encéphalopathies spongiformes transmissibles
  • EST (Encéphalopathie Spongiforme Transmissible)
  • Maladies à prion

URI

http://data.loterre.fr/ark:/67375/JVR-JN2F94PZ-Q

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