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[Transmissible spongiform encephalopathies--illnesses in the human]

H Budka1

  • 1Klinisches Institut für Neurologie, Universität Wien. h.budka@akh-wien.ac.at

Wiener Medizinische Wochenschrift (1946)
|June 5, 1998
PubMed
Summary

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a growing concern due to their link to variant Creutzfeldt-Jakob disease (vCJD). Current diagnostics include CSF 14-3-3 protein tests and MRI, but definitive diagnosis requires neuropathology.

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Clinical neuropathology·2010

Area of Science:

  • Neurology
  • Infectious Diseases
  • Biomedicine

Context:

  • Transmissible spongiform encephalopathies (TSEs), or prion diseases, are of significant current interest.
  • The emergence of variant Creutzfeldt-Jakob disease (vCJD) linked to Bovine Spongiform Encephalopathy (BSE) has heightened concerns.
  • The prion hypothesis, suggesting protein-only infectivity, presents a new paradigm in understanding heredity and transmissibility.

Purpose:

  • To review the current understanding and diagnostic approaches to TSEs.
  • To highlight the diagnostic challenges and the role of emerging tools like CSF 14-3-3 protein determination and MRI.
  • To discuss the implications for laboratory safety and blood product safety, particularly in light of vCJD.

Summary:

  • Clinical examination for TSEs like Creutzfeldt-Jakob disease (CJD) provides a suspect diagnosis, with formal criteria for "probable" or "possible" cases.

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  • Definitive diagnosis of TSEs relies on post-mortem neuropathology or, less commonly, brain biopsy.
  • Promising diagnostic tools include 14-3-3 protein detection in cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI).

    • Impact:

    • Laboratory handling of TSE materials necessitates specific safety and decontamination protocols.
    • While blood and blood products can transmit TSEs, direct evidence for transfusion transmission is limited.
    • New WHO regulations exclude certain blood donors, and leukodepletion is recommended in the UK due to potential differences in vCJD infectivity distribution.