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[Transmissible spongiform encephalopathies in humans].

C Rogivue1, J Gottwein, A Aguzzi

  • 1Bundesamt für Gesundheit, Bern. colette.rogivue@bluewin.ch

Schweizer Archiv Fur Tierheilkunde
|February 15, 2003
PubMed
Summary

Transmissible spongiform encephalopathies (TSEs) are untreatable human brain diseases. Switzerland has seen no variant CJD cases despite BSE exposure, but an unexplained rise in sporadic CJD since 2001.

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Area of Science:

  • Neurology
  • Prion Diseases
  • Epidemiology

Context:

  • Human transmissible spongiform encephalopathies (TSEs) are progressive, fatal neurodegenerative disorders.
  • Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form, with familial (fCJD) and iatrogenic (iCJD) subtypes.
  • A variant form (vCJD) emerged in 1996, linked to Bovine Spongiform Encephalopathy (BSE) prions.

Purpose:

  • To review the landscape of human TSEs, including Creutzfeldt-Jakob disease (CJD) subtypes.
  • To discuss the epidemiological situation in Switzerland, particularly regarding BSE exposure and sCJD trends.
  • To highlight the importance of preventive measures given the current untreatable nature of TSEs.

Summary:

  • Human TSEs, including various forms of CJD, are untreatable dementing diseases.

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  • Biochemical and neuropathological differences distinguish vCJD from sCJD, suggesting vCJD arises from BSE prion transmission.
  • Switzerland has not reported vCJD cases despite BSE exposure, but has observed an unexplained increase in sCJD since 2001.
  • Impact:

    • Understanding the distinct characteristics of CJD subtypes is crucial for diagnosis and surveillance.
    • The findings underscore the need for continued monitoring and stringent preventive strategies against prion diseases.
    • The unexplained rise in sCJD warrants further investigation into potential environmental or epidemiological factors in Switzerland.