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Prion Diseases

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)

Year introduced: 1993

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Tree Number(s): C01.207.800, C10.228.228.800, C10.574.843

MeSH Unique ID: D017096

Entry Terms:

  • Dementias, Transmissible
  • Dementia, Transmissible
  • Transmissible Dementia
  • Transmissible Dementias
  • Encephalopathies, Spongiform, Transmissible
  • Prion-Induced Disorders
  • Prion-Induced Disorder
  • Disorder, Prion-Induced
  • Disorders, Prion-Induced
  • Prion Induced Disorder
  • Transmissible Spongiform Encephalopathies
  • Prion Disease
  • Prion-Associated Disorders
  • Spongiform Encephalopathies, Transmissible
  • Encephalopathies, Transmissible Spongiform
  • Encephalopathy, Transmissible Spongiform
  • Spongiform Encephalopathy, Transmissible
  • Transmissible Spongiform Encephalopathy
  • Prion Protein Diseases
  • Prion Protein Disease
  • Human Transmissible Spongiform Encephalopathies, Inherited
  • Inherited Human Transmissible Spongiform Encephalopathies

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