#UK, #Scotland: A case of classical Bovine Spongiform Encephalopathy (#BSE) has been confirmed in a #cow on a farm in #Dumfries and Galloway

 

A case of classical Bovine Spongiform Encephalopathy (BSE) has been confirmed in a cow on a farm in Dumfries and Galloway.

    Precautionary movement restrictions have been put in place on the offspring of the animal and its ‘cohort’ at the impacted premises.  

    Further investigations to identify the origin of the disease are ongoing. This is standard procedure for a confirmed case of classical BSE.

    The case was identified as a result of our routine though intensive BSE surveillance and stringent control measures that are in place. 

    The animal did not enter the human food chain and Food Standards Scotland have confirmed there is no risk to human health as a result of this isolated case.

    The owners of the affected animals are working with authorities on next steps.

    Agriculture Minister Jim Fairlie said:

        "Following confirmation of a case of classical BSE in Dumfries and Galloway, the Scottish Government and other agencies acted swiftly and decisively to protect the agriculture sector.

        "The fact we identified this isolated case so quickly is clear proof that our surveillance system for detecting this type of disease is working effectively.

        "I want to thank the animal's owner for their diligence. Their swift action allowed us to identify and isolate the case immediately, minimising its impact on the wider industry."

Background

Bovine Spongiform Encephalopathy 

    The Animal Plant and Health Agency (APHA) is investigating the source of the disease.

    All animals over four years of age that die on farm are routinely tested for BSE under Scotland’s comprehensive surveillance system.  

    While the disease is not directly transmitted from animal to animal, its cohorts including offspring, have been traced and isolated, and will be destroyed in line with legal requirements.

    In addition to the measures in place for fallen stock and animal feed, there is a strict control regime to protect consumers. This includes the removal of specified risk material such as the spinal column, brain and skull from carcasses destined for human consumption.

Source: 

Link: https://www.gov.scot/news/bse-3/

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#UK - Bovine spongiform encephalopathy #BSE - Immediate notification

    ° Isolated, single case of classical bovine spongiform encephalopathy (BSE) has been confirmed in a 7½- year- old indigenous cow on a beef suckler farm. 

    ° The cow was pregnant, with no clinical signs presented before being found dead. 

    ° The case was disclosed during routine national statutory surveillance and testing of fallen stock cattle aged over 48 months. 

    ° The animal was not presented for slaughter and did not enter the food chain. 

    ° There is no threat to food safety or human health. 

    ° A full epidemiological investigation is being carried out to identify relevant risk pathways for infection and any evidence to support these. 

    ° All of the cohorts and offspring of this single BSE case have been identified and placed under movement restrictions. 

    ° They will be culled and screened for BSE. 

    ° The detection of this single case does not change the ‘Negligible risk’ status for the zone of Scotland or UK. 

    ° The precise location of the farm has not been entered on the report to protect the farmer's identity and well-being. 

    Note from WOAH 20/08/2026: 

        The statement above is not related to the official disease status delivered by WOAH. 

        Official disease status are published on the WOAH website at https://www.woah.org/en/what-we-do/animal-health-and-welfare/official-disease-status/

    ° Isolated case of classical Bovine Spongiform Encephalopathy (BSE) has been confirmed in a 7 and ½ -year-old female beef cattle, in a beef suckler herd with approx. 740 animals.

Source: WOAH, https://wahis.woah.org/#/home

Link: https://wahis.woah.org/#/in-review/7773

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Isolation of a novel #human #prion #strain from a PRNP codon 129 heterozygous #vCJD #patient

Abstract

The epizootic prion disease of cattle, bovine spongiform encephalopathy (BSE), caused variant Creutzfeldt-Jakob disease (vCJD) in humans following dietary exposure. Codon 129 polymorphism of the human prion protein gene (PRNP), encoding either methionine (M) or valine (V), dictates the propagation of distinct human prion strains and up to now all but one neuropathologically confirmed vCJD patients have had a 129MM genotype. Concordant with this genetic association, transgenic modelling has established that human PrP 129V is incompatible with the vCJD prion strain and that depending on codon 129 genotype, primary human infection with BSE prions may, in addition to vCJD, result in sporadic CJD-like or novel phenotypes. In 2016 we saw the first neuropathologically confirmed case of vCJD in a patient with a codon 129MV genotype. This patient’s neuropathology and molecular strain type were pathognomonic of vCJD but their clinical presentation and neuroradiological features were more typical of sporadic CJD, suggestive of possible co-propagation of another prion strain. Here we report the transmission properties of prions from the brain and lymphoreticular tissues of the 129MV vCJD patient. Primary transmissions into transgenic mice expressing human PrP with different codon 129 genotypes mainly produced neuropathological and molecular phenotypes congruent to those observed in the same lines of mice challenged with prions from 129MM vCJD patient brain, indicative that the vCJD prion strain was the dominant propagating prion strain in the patient’s brain. Remarkably however, some transgenic mice challenged with 129MV vCJD patient brain propagated a novel prion strain type which at secondary passage was uniformly lethal in mice of all three PRNP codon 129 genotypes after similar short mean incubation periods. These findings establish that cattle BSE prions can trigger the co-propagation of distinct prion strains in humans.

Source: PLoS Pathogens, https://journals.plos.org/plospathogens/article?id=10.1371/journal.ppat.1012904

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