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Constant transmission properties of variant Creutzfeldt-Jakob disease in 5 countries

Abigail B Diack1, Diane Ritchie, Matthew Bishop

  • 1The Roslin Institute, Easter Bush, Scotland.

Emerging Infectious Diseases
|September 29, 2012
PubMed

Insights

A single infectious agent strain likely causes variant Creutzfeldt-Jakob disease (vCJD) globally. Mouse studies showed consistent transmission and characteristics across international vCJD cases, supporting a common origin.

Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Prion Diseases

Background:

  • Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegenerative disorder.
  • vCJD cases have been documented in 12 countries worldwide.
  • The geographic origin of vCJD cases has raised questions about the causative agent's strain.

Purpose of the Study:

  • To test the hypothesis that a common infectious agent strain causes all vCJD cases.
  • To compare vCJD agent properties across different geographic origins.

Main Methods:

  • Inoculation of wild-type mice with brain material from vCJD patients from multiple countries.
  • Assessment of clinical disease, neuropathology, and prion glycoform profiles in mice.
  • Comparison of results with reference vCJD cases from the United Kingdom.

Main Results:

  • Successful transmission of the vCJD agent to mice from all tested international samples.
  • Consistent neuropathologic and glycoform profile data between non-UK and UK vCJD cases.
  • Minor variations observed in the ranking of mean clinical incubation times across mouse lines.

Conclusions:

  • Findings strongly support the hypothesis of a single vCJD agent strain responsible for all human infections.
  • Observed differences in incubation times warrant further investigation through mouse subpassage.
  • Further studies are needed to definitively establish strain property variations between vCJD cases.

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