Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients

• Published in: Emerging infectious diseases Vol. 23; no. 6; pp. 946 - 956
• Main Authors: Douet, Jean Y, Lacroux, Caroline, Aron, Naima, Head, Mark W, Lugan, Séverine, Tillier, Cécile, Huor, Alvina, Cassard, Hervé, Arnold, Mark, Beringue, Vincent, Ironside, James W, Andréoletti, Olivier
• Format: Journal Article
• Language: English
• Published: United States U.S. National Center for Infectious Diseases 01-06-2017; Centers for Disease Control and Prevention
• Subjects: Animal biology; Animals; Asymptomatic Diseases; Biological Assay; Bone Marrow - metabolism; Bone Marrow - pathology; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - metabolism; Creutzfeldt-Jakob Syndrome - pathology; Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients; Female; Human health and pathology; Humans; infectivity; Kidney - metabolism; Kidney - pathology; Life Sciences; Liver - metabolism; Liver - pathology; Lung - metabolism; Lung - pathology; Male; Medical research; Mice; Microbiology and Parasitology; prions and related diseases; Protein Folding; PrPC Proteins - chemistry; PrPC Proteins - metabolism; PrPC Proteins - pathogenicity; Salivary Glands - metabolism; Salivary Glands - pathology; tissues; United Kingdom; variant Creutzfeldt–Jakob disease; variant Creutzfeldt–Jakob disease prions; vCJD; asymptomatic patients; tissues; prions and related diseases; Creutzfeldt-Jakob disease; Scotland; United Kingdom; variant Creutzfeldt-Jakob disease; clinical patients; intracerebral inoculation; variant Creutzfeldt-Jakob disease prions; variant infectivity; vCJD; meningitis/encephalitis; protein misfolding cyclic amplification; public health
• ISSN: 1080-6040; 1080-6059
• DOI: 10.3201/eid2306.161734

In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions by protein misfolding cyclic amplification (PMCA) to estimate distribution and level of the vCJD agent in 21 tissues from 4 patients who died of clinical vCJD and from 1 asymptomatic person with vCJD. PMCA identified major levels of vCJD prions in a range of tissues, including liver, salivary gland, kidney, lung, and bone marrow. Bioassays confirmed that the quantitative estimate of levels of vCJD prion accumulation provided by PMCA are indicative of vCJD infectivity levels in tissues. Findings provide critical data for the design of measures to minimize risk for iatrogenic transmission of vCJD.