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      Chronic wasting disease (CWD) in cervids

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          Abstract

          In April and May of 2016, Norway confirmed two cases of chronic wasting disease (CWD) in a wild reindeer and a wild moose, respectively. In the light of this emerging issue, the European Commission requested EFSA to recommend surveillance activities and, if necessary, additional animal health risk‐based measures to prevent the introduction of the disease and the spread into/within the EU, specifically Estonia, Finland, Iceland, Latvia, Lithuania, Norway, Poland and Sweden, and considering seven wild, semidomesticated and farmed cervid species (Eurasian tundra reindeer, Finnish (Eurasian) forest reindeer, moose, roe deer, white‐tailed deer, red deer and fallow deer). It was also asked to assess any new evidence on possible public health risks related to CWD. A 3‐year surveillance system is proposed, differing for farmed and wild or semidomesticated cervids, with a two‐stage sampling programme at the farm/geographically based population unit level (random sampling) and individual level (convenience sampling targeting high‐risk animals). The current derogations of Commission Implementing Decision (EU) 2016/1918 present a risk of introduction of CWD into the EU. Measures to prevent the spread of CWD within the EU are dependent upon the assumption that the disease is already present; this is currently unknown. The measures listed are intended to contain (limit the geographic extent of a focus) and/or to control (actively stabilise/reduce infection rates in an affected herd or population) the disease where it occurs. With regard to the zoonotic potential, the human species barrier for CWD prions does not appear to be absolute. These prions are present in the skeletal muscle and other edible tissues, so humans may consume infected material in enzootic areas. Epidemiological investigations carried out to date make no association between the occurrence of sporadic Creutzfeldt–Jakob disease in humans and exposure to CWD prions.

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          Most cited references181

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          Environmental Sources of Prion Transmission in Mule Deer

          Whether transmission of the chronic wasting disease (CWD) prion among cervids requires direct interaction with infected animals has been unclear. We report that CWD can be transmitted to susceptible animals indirectly, from environments contaminated by excreta or decomposed carcasses. Under experimental conditions, mule deer (Odocoileus hemionus) became infected in two of three paddocks containing naturally infected deer, in two of three paddocks where infected deer carcasses had decomposed in situ ≈1.8 years earlier, and in one of three paddocks where infected deer had last resided 2.2 years earlier. Indirect transmission and environmental persistence of infectious prions will complicate efforts to control CWD and perhaps other animal prion diseases.
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            Chronic wasting disease of captive mule deer: a spongiform encephalopathy.

            In the past 12 years (1967-79) a syndrome we identify as chronic wasting disease has been observed in 53 mule deer (Odocoileus hemionus hemionus) and one black-tailed deer (Odocoileus hemionus columbianus) held in captivity in several wildlife facilities in Colorado and more recently in Wyoming. Clinical signs were seen in adult deer and included behavioral alterations, progressive weight loss and death in 2 weeks to 8 months. Gross necropsy findings included emaciation and excess rumen fluid admixed with sand and gravel. Consistent histopathologic change was limited to the central nervous system and characterized by widespread spongiform transformation of the neuropil, single of multiple intracytoplasmic vacuoles in neuronal perikaryons and intense astrocytic hypertrophy and hyperplasia. Presented is a clinical characterization of chronic wasting disease and pathologic evidence supporting the conclusion that the disease is a specific spontaneously occurring form of spongiform encephalopathy.
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              Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.

              Transgenic (Tg) mice expressing both Syrian hamster (Ha) and mouse (Mo) prion protein (PrP) genes were used to probe the mechanism of scrapie prion replication. Four Tg lines expressing HaPrP exhibited distinct incubation times ranging from 48 to 277 days, which correlated inversely with HaPrP mRNA and HaPrPC. Bioassays of Tg brain extracts showed that the prion inoculum dictates which prions are synthesized de novo. Tg mice inoculated with Ha prions had approximately 10(9) ID50 units of Ha prions per gram of brain and less than 10 units of Mo prions. Conversely, Tg mice inoculated with Mo prions synthesized Mo prions but not Ha prions. Similarly, Tg mice inoculated with Ha prions exhibited neuropathologic changes characteristic of hamsters with scrapie, while Mo prions produced changes similar to those in non-Tg mice. Our results argue that species specificity of scrapie prions resides in the PrP sequence and prion synthesis is initiated by a species-specific interaction between PrPSc in the inoculum and homologous PrPC.
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                Author and article information

                Journal
                EFSA J
                EFSA J
                10.1002/(ISSN)1831-4732
                EFS2
                EFSA Journal
                John Wiley and Sons Inc. (Hoboken )
                1831-4732
                18 January 2017
                January 2017
                : 15
                : 1 ( doiID: 10.1002/efs2.2017.15.issue-1 )
                : e04667
                Author notes
                [*] Correspondence: biohaz@ 123456efsa.europa.eu
                Article
                EFS24667
                10.2903/j.efsa.2017.4667
                7010154
                32625260
                88434884-2ed3-4df4-9471-917aea563a24
                © 2017 European Food Safety Authority. EFSA Journal published by John Wiley and Sons Ltd on behalf of European Food Safety Authority.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited and no modifications or adaptations are made.

                History
                Page count
                Figures: 7, Tables: 16, Pages: 62, Words: 42714
                Categories
                Scientific Opinion
                Scientific Opinion
                Custom metadata
                2.0
                January 2017
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.7.5 mode:remove_FC converted:21.01.2020

                chronic,wasting,cervids,surveillance,risk,introduction,spread
                chronic, wasting, cervids, surveillance, risk, introduction, spread

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