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Prion Diseases (Transmissible Spongiform Encephalopathies), Pediatric

John Bower, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of progressive neurodegenerative diseases of humans and animals that cause irreversible cumulative bra...

DIAGNOSIS

HISTORY

  • Evidence of a familial form of prion disease

  • Potential iatrogenic exposures such as administration of human-derived pituitary growth hormones, implantation of dura mater or corneal gra...

TREATMENT

GENERAL MEASURES

  • No treatment is effective in slowing or stopping the progression of disease. Appropriate supportive care should be provided. Prognosis for patients with human prion diseases ...

ADDITIONAL READING

  • Aguzzi A, Heikenwalder M. Pathogenesis of prion diseases: current status and future outlook. Nat Rev Microbiol.  2006;4(10):765–775. [View Abstract on OvidInsights]

  • Diack AB, He...

CODES

ICD9

  • 046.79 Other and unspecified prion disease of central nervous system

  • 046.19 Other and unspecified Creutzfeldt-Jakob disease

  • 046.0 Kuru

  • 046.71 Gerstmann-Sträussler-Scheinker syndrome

  • 046.72 Fatal...

FAQ

  • Q: Is transmission of prion disease from human blood possible?

  • A: There have been three verified cases of vCJD attributed to transfusion of blood products in the United Kingdom. There have been no ...

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