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Concept information

Nervous System Diseases > Neurodegenerative Diseases > Prion Diseases
Nervous System Diseases > Central Nervous System Diseases > Central Nervous System Infections > Prion Diseases
Infections > Central Nervous System Infections > Prion Diseases

Terme préférentiel

Prion Diseases  

Type

  • mesh:Descriptor

Définition

  • 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)

Concept générique

Concepts spécifiques

Concepts associés

Synonyme(s)

  • 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

Qualificatif(s) autorisé(s)

Traductions

  • Maladies à prions

    français

  • 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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RDF/XML TURTLE JSON-LD Date de création 22/05/1992, dernière modification le 17/06/2019