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AttributesValues
type
sameAs
notation
  • C10.228.228.800
  • C10.574.843
  • C01.207.800
alternative label
  • Encéphalopathies spongiformes transmissibles humaines familiales
  • Dementia, Transmissible
  • EST (Encéphalopathie Spongiforme Transmissible)
  • Prion Disease
  • Prion-Induced Disorder
  • Prion-Induced Disorders
  • Spongiform Encephalopathies, Transmissible
  • Transmissible Dementias
  • Transmissible Spongiform Encephalopathies
  • Inherited Human Transmissible Spongiform Encephalopathies
  • Encephalopathies, Transmissible Spongiform
  • Maladies humaines familiales à prions
  • Maladies à prion
  • Prion Diseases
  • Prion Induced Disorder
  • Prion Protein Diseases
  • Spongiform Encephalopathy, Transmissible
  • Transmissible Dementia
  • Transmissible Spongiform Encephalopathy
  • Dementias, Transmissible
  • Disorder, Prion-Induced
  • Disorders, Prion-Induced
  • Démences transmissibles
  • Encephalopathies, Spongiform, Transmissible
  • Encephalopathy, Transmissible Spongiform
  • Encéphalopathies spongiformes transmissibles
  • Prion Protein Disease
  • Prion-Associated Disorders
  • Human Transmissible Spongiform Encephalopathies, Inherited
preferred label
  • Maladies à prions
is in scheme
note
  • 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)
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