Creutzfeldt-Jakob Disease (CJD) is Not an Infectious Disease

  • Amos D. Korczyn
Part of the Advances in Behavioral Biology book series (ABBI, volume 44)

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare degenerative disease of the central nervous system with many similarities to Alzheimer’s disease (Table 1). Recent advances in molecular biology have identified the molecular pathology of the disease, consisting of accumulation of “prion protein”. Epidemiologic studies demonstrate that CJD is in most cases a sporadic disease. Cases with a familial background have been shown to result, in many cases, from mutations in the gene responsible for prion synthesis. Such mutations were never demonstrated in sporadic cases. CJD can be transmitted to experimental animals by intracerebral injections, and further passages of brain tissue can lead to subsequent transmission of the disease, probably indefinitely. Human-to-human transmission of the disease has occurred, in single cases with implants of dura mater or cornea from donors who had died with CJD, and as a small epidemic among recipients of human pituitary extracts. These tragic occurrences, however, are a rarity. They prove that CJD is a transmissible disease even in humans. However, this occurs very rarely, and infection is not documented. Most cases of CJD arise spontaneously, possibly due to post-translational modification of the prion protein, leading to progressive transformation of this change throughout the brain, with amyloid deposition and neuronal degeneration.

Keywords

Prion Protein Bovine Spongiform Encephalopathy PRNP Gene Fatal Familial Insomnia Prion Protein Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1995

Authors and Affiliations

  • Amos D. Korczyn
    • 1
  1. 1.Sackler Faculty of MedicineTel-Aviv UniversityRamat AvivIsrael

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