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[The prion diseases]

K Kondo1

  • 1Department of Public Health, Hokkaido University School of Medicine, Sapporo, Japan.

[Hokkaido Igaku Zasshi] the Hokkaido Journal of Medical Science
|January 1, 1997
PubMed
Summary
This summary is machine-generated.

Prion diseases, like bovine spongiform encephalopathy (BSE), can cause human subacute spongiform encephalopathies (SSE). Japan implemented surveillance and controls to prevent BSE-related Creutzfeldt-Jakob disease (CJD) cases, with no new variant CJD (vCJD) observed.

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Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Public Health

Background:

  • Prions cause subacute spongiform encephalopathies (SSE) in humans and animals.
  • The 1996 UK outbreak of bovine spongiform encephalopathy (BSE) raised concerns about human health risks.
  • New variant Creutzfeldt-Jakob disease (vCJD) emerged, distinct from classical CJD.

Observation:

  • Human SSE includes classical CJD, Gerstmann-Sträussler-Scheinker disease, transmitted CJD, and Kuru.
  • vCJD cases, all in the UK, differ in age of onset and pathology from classical CJD.
  • Prions from vCJD share properties with BSE prions, suggesting a potential dietary link.

Findings:

  • Japan implemented agent and host controls to mitigate BSE risks.
  • A national CJD surveillance system was established in July 1996.

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  • Surveillance identified 766 CJD cases, including 51 familial, but no vCJD cases.

    • Implications:

    • BSE poses a potential human health risk, necessitating stringent control measures.
    • Effective surveillance and control strategies are crucial for preventing prion disease transmission.
    • Continued monitoring is essential to detect and manage emerging prion diseases like vCJD.