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)

Also Known As:

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

Infections: 526564
- Central Nervous System Infections: 1390
  - Prion Diseases: 5345
Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - Prion Diseases: 5345
- Central Nervous System Diseases: 4213
  - Central Nervous System Infections: 1390
    - Prion Diseases: 5345

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