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This report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (-3%, compared with 2022), with five atypical BSE cases reported (four H-type: two in Spain, one in France and one in Ireland; one L-type in the Netherlands); and 46,096 cattle by eight non-EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1).
View Article and Find Full Text PDFMRI changes observed in a case of atypical scrapie in a 7-year-old Herdwick ewe.
J Vet Diagn Invest
November 2024
Department of Small Ruminant Health, Royal GD, Deventer, the Netherlands.
Atypical scrapie is a transmissible spongiform encephalopathy that is rarely diagnosed in living animals. In March 2022, a 7-y-old Herdwick ewe was referred to the Scottish Centre for Production Animal Health and Food Safety because of circling behavior and ill thrift. The ewe had a low body condition score, was obtunded, with a wide-based stance of the pelvic limbs, and was circling to the left.
View Article and Find Full Text PDFCharacterisation of European Field Goat Prion Isolates in Ovine PrP Overexpressing Transgenic Mice (Tgshp IX) Reveals Distinct Prion Strains.
Pathogens
July 2024
Friedrich-Loeffler-Institut, 17493 Greifswald-Isle of Riems, Germany.
After the detection of bovine spongiform encephalopathy (BSE), and a zoonotic transmissible spongiform encephalopathy (TSE) caused by the pathological prion protein (PrP) in two goats, the investigation of goat prions became of greater interest. Therefore, a broad collection of European goat TSE isolates, including atypical scrapie, CH1641 and goat BSE as reference prion strains were biochemically characterised and subsequently inoculated into seven rodent models for further analysis (already published results of this comprehensive study are reviewed here for comparative reasons). We report here the histopathological and immunohistochemical data of this goat TSE panel, obtained after the first passage in Tgshp IX (tg-shARQ) mice, which overexpress the ovine prion protein.
View Article and Find Full Text PDFAssessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions.
J Infect Dis
July 2024
Université Paris-Saclay, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Université Versailles-Saint Quentin, Unité de Virologie Immunologie Moléculaires, Jouy-en-Josas, France.
Background: Atypical/Nor98 scrapie (AS) is an idiopathic infectious prion disease affecting sheep and goats. Recent findings suggest that zoonotic prions from classical bovine spongiform encephalopathy (C-BSE) may copropagate with atypical/Nor98 prions in AS sheep brains. Investigating the risk AS poses to humans is crucial.
View Article and Find Full Text PDFDiagnosis in Scrapie: Conventional Methods and New Biomarkers.
Pathogens
November 2023
Centro de Encefalopatías y Enfermedades Transmisibles Emergentes, Facultad de Veterinaria, IA2, Universidad de Zaragoza, 50013 Zaragoza, Spain.
Scrapie, a naturally occurring prion disease affecting goats and sheep, comprises classical and atypical forms, with classical scrapie being the archetype of transmissible spongiform encephalopathies. This review explores the challenges of scrapie diagnosis and the utility of various biomarkers and their potential implications for human prion diseases. Understanding these biomarkers in the context of scrapie may enable earlier prion disease diagnosis in humans, which is crucial for effective intervention.
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