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Implications of prion diseases for neurosurgery
1Department of Neurology, Universitätsspital Zürich, Frauenklinikstrasse 26, Switzerland. thomas.blaettler@nos.usz.ch
Abstract:
Prion diseases comprise a group of diseases characterised by transmissibility, spongiosis, gliosis, neuronal loss, and accumulation of an abnormally folded membrane protein, PrP(Sc). Infectivity resists almost all chemical and physical processes that inactivate conventional viruses, whereas protein extraction abolishes infectivity. This fact is of great importance to surgery, especially neurosurgery, since conventional cleaning of surgical instruments does not abolish infectivity. As a matter of consequence, few cases of putative neurosurgical transmission of Creutzfeldt-Jakob disease (CJD) have been reported. Putative transmission has also been reported through the use of lyophilised dura mater, corneal transplants, and cortical EEG electrodes. Moreover, many children have been infected with CJD by intramuscular or subcutaneous injection of cadaveric pituitary-derived human growth hormone. In recent years, the occurrence of bovine spongiform encephalopathy and consequently new variant Creutzfeldt-Jakob disease has increased concerns that prions also may contaminate the blood supply. Animal models of prion disease can help the understanding of how prions spread within an infected organism and the identification of which tissues may be contagious. Taking the right precautions against iatrogenic transmission requires knowledge about the nature of prion diseases - not only for the persons working out the directives but also for health care workers potentially involved with CJD patients or contaminated specimens.
Insights
Prion diseases are transmissible neurodegenerative conditions caused by abnormal proteins (PrPSc). Standard sterilization methods fail to inactivate prions, posing risks for surgical instrument and blood supply safety.
Area of Science:
- Neurology
- Infectious Diseases
- Biochemistry
Background:
- Prion diseases are characterized by spongiform changes, gliosis, neuronal loss, and accumulation of abnormal prion protein (PrPSc).
- Prion infectivity is highly resistant to conventional sterilization, posing significant risks for iatrogenic transmission.
- Concerns about prion contamination have increased due to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease.
Purpose of the Study:
- To highlight the challenges in inactivating prions and the implications for surgical safety.
- To discuss the routes of iatrogenic prion transmission, including surgical procedures and contaminated biological materials.
- To emphasize the importance of understanding prion disease for preventing healthcare-associated infections.
Main Methods:
- Review of existing literature on prion disease characteristics and transmission.
- Analysis of reported cases of iatrogenic Creutzfeldt-Jakob disease (CJD).
- Discussion of the resistance of prions to standard inactivation methods.
Main Results:
- Conventional sterilization of surgical instruments does not eliminate prion infectivity.
- Iatrogenic transmission of CJD has been linked to neurosurgery, dura mater grafts, corneal transplants, EEG electrodes, and human growth hormone.
- The potential for prion contamination of the blood supply is a growing concern.
Conclusions:
- Knowledge of prion disease nature is crucial for developing effective decontamination protocols and preventing iatrogenic spread.
- Enhanced precautions are necessary in healthcare settings, particularly in surgery and blood banking.
- Animal models are vital for understanding prion pathogenesis and identifying infectious tissues.