Human prion diseases: surgical lessons learned from iatrogenic prion transmission

Abstract

<jats:p>The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.</jats:p>

Large retrospective study of artificial dura substitute in patients with traumatic brain injury undergo decompressive craniectomy

“…These diseases are sometimes infectious and hence referred to as transmissible spongiform encephalopathies (Norrby, ). In the past over, 60% reported prion diseases caused by cadaveric dura mater transplantation were from Japan, and it is related to frequent use of Lyodura (Bonda et al., ). After improvement of manufacture, the incidence is significantly reduced (Bonda et al., ).…”

“…In the past over, 60% reported prion diseases caused by cadaveric dura mater transplantation were from Japan, and it is related to frequent use of Lyodura (Bonda et al., ). After improvement of manufacture, the incidence is significantly reduced (Bonda et al., ). In this study, we used decellularized bovine pericardium membranes which meet criteria of Chinese FDA, and de‐contamination of prion procedure has been performed.…”

Implications of peptide assemblies in amyloid diseases

“…424, 425 Less common ways of transmission in humans are surgical instruments and human derived growth hormones. 426 …”

Optimization of the photodynamic inactivation of prions by a phthalocyanine photosensitizer: The crucial involvement of singlet oxygen

“…The widespread presence of PrP TSE in CNS and other organs and proven infectivity of these tissues , even in asymptomatic carriers and asymptomatic patients, poses risks for nosocomial transmission . Iatrogenic infections via reusable brain electrode probes, neurosurgical instruments, corneal transplants, dura matter grafts, hormones isolated from pituitary glands and recently via blood transfusion have been already documented .…”