title: Human prion diseases: surgical lessons learned from iatrogenic prion transmission.
abstract: 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.
authors.affiliation: Department of Neurological Surgery, University Hospitals Case Medical Center, and.
authors.forename: David J
authors.initials: DJ
authors.lastname: Bonda
authors.affiliation: Department of Neurological Surgery, University Hospitals Case Medical Center, and.
authors.forename: Sunil
authors.initials: S
authors.lastname: Manjila
authors.affiliation: Department of Neurology, University of Virginia Health System, Charlottesville, Virginia;
authors.forename: Prachi
authors.initials: P
authors.lastname: Mehndiratta
authors.affiliation: Department of Neurosurgery, Stanford University, Stanford, California;
authors.forename: Fahd
authors.initials: F
authors.lastname: Khan
authors.affiliation: Department of Neurological Surgery, University Hospitals Case Medical Center, and.
authors.forename: Benjamin R
authors.initials: BR
authors.lastname: Miller
authors.affiliation: Department of Neurological Surgery, University Hospitals Case Medical Center, and.
authors.forename: Kaine
authors.initials: K
authors.lastname: Onwuzulike
authors.affiliation: Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy; and.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Puoti
authors.affiliation: National Prion Disease Pathology Surveillance Center.|Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio;
authors.forename: Mark L
authors.initials: ML
authors.lastname: Cohen
authors.affiliation: Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia.
authors.forename: Lawrence B
authors.initials: LB
authors.lastname: Schonberger
authors.affiliation: Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio;|Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy; and.
authors.forename: Ignazio
authors.initials: I
authors.lastname: Cali
mesh_terms: D000820:Animal Diseases / Q000635:transmission; D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology; D003428:Cross Infection; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D007049:Iatrogenic Disease / Q000453:epidemiology; D019635:Neurosurgical Procedures / Q000009:adverse effects*; D017096:Prion Diseases / Q000453:epidemiology / Q000209:etiology* / Q000266:history / Q000635:transmission*
keywords: BSE = bovine spongiform encephalopathy; CDC = Centers for Disease Control and Prevention; CJD = Creutzfeldt-Jakob disease; EEG = electroencephalography; FFI = fatal familial insomnia; GSS = Gerstmann-Schäussler-Scheinker syndrome; NIH = National Institutes of Health; PrP = prion protein; PrPC = cellular PrP; PrPSc = abnormal form of PrP; V = valine; VPSPr = variably protease-sensitive prionopathy; WHO = World Health Organization; dCJD = CJD transmitted by commercially distributed cadaveric dura mater; fCJD = familial CJD; hGH = human growth hormone; iCJD = iatrogenic CJD: M = methionine; iatrogenic; instrument decontamination; neurosurgery; sCJD = sporadic CJD; sFI = sporadic fatal insomnia; scrapie prion protein; transmissible spongiform encephalopathy; vCJD = variant CJD