Testing for TSEs in the EU, 2019

The European Union summary report on surveillance for the presence of transmissible spongiform encephalopathies (TSE) in 2019, 17 November 2020, EFSA:

This report presents the results of surveillance on transmissible spongiform encephalopathies (TSE) in cattle, sheep, goats, cervids and other species, and genotyping in sheep, carried out in 2019 by 28 Member States (MS), and by Iceland, Montenegro, North Macedonia, Norway, Serbia and Switzerland (non‐MS).

In total, 1,150,388 cattle were tested by MS, a 2.7% decrease from 2018 and 44,557 by the six non‐MS. Six cases of H‐BSE were reported by France (4) and Spain (2), and 1 L‐BSE by Poland. The number of H‐ BSE cases was the largest reported per year including the youngest ever case (5.5 years of age).

In total, 338,098 sheep and 143,529 goats were tested in the EU, an increase of 3.9% in both species compared with 2018. In sheep, 17 inconclusive cases by two MS and 997 cases of scrapie were reported: 911 classical (97 index cases (IC), one of ARR/ARR genotype and 98.7% with genotypes of susceptible groups) by seven MS, 86 atypical (AS) (80 IC) by 11 MS. Thirty‐one ovine scrapie cases were reported by Iceland and Norway. Random genotyping was only reported by eight MS: Cyprus excluded, 15.7% of genotyped sheep carried genotypes of susceptible groups. In goats, three inconclusive cases by two MS and 390 cases of scrapie were reported: 379 classical (24 IC) by six MS, 11 atypical (10 IC) by six MS.

The heterogeneous enforcement of a 3‐year surveillance programme for chronic wasting disease (CWD) in six MS (Estonia, Finland, Latvia, Lithuania, Poland and Sweden) resulted in the testing of 7,980 cervids and confirmation of three CWD cases in wild moose in Sweden. Other seven MS tested 2,732 cervids with no positive results. Norway tested 30,147 cervids in 2019, with two new moose cases. In total, 122 animals from four other species reported by three MS TSE tested negative.


Low incidence of TSEs in the EU, says EFSA

EFSA has published its first EU summary report on the monitoring of Transmissible Spongiform Encephalopathies (TSEs) in cattle, sheep and goats. Previously, the annual reports on TSEs were compiled by the European Commission.

TSEs are a group of diseases that affect the brain and nervous system of humans and animals.  With the exception of Classical BSE, there is no scientific evidence that other TSEs can be transmitted to humans.

mad-cows-mothers-milkA low number of BSE cases in cattle were detected in EU Member States, none of which entered the food chain.

Some of the main findings of the report are:

Five cases of BSE in cattle have been reported in the EU, out of about 1.4 million animals tested.

641 cases of scrapie in sheep (out of 319,638 tested) and 1,052 in goats have been reported (out of 135,857 tested) in the EU.

This report provides results on data collected by all EU Member States, Iceland, Norway and Switzerland for 2015 on the occurrence of bovine spongiform encephalopathy

Can scrapie in sheep cause disease in humans?

On March 20, 1996, British Health Secretary Stephen Dorrell rose in the House to inform colleagues that scientists had discovered a new variant of Creutzfeldt-Jacob disease (CJD) in 10 victims, and that they could not rule out a link with consumption of beef from cattle with bovine spongiform encephalapthy (BSE), also known as mad cow disease.

timmy.timeThe announcement of March 20, 1996 was the culmination of 15 years of mismanagement, political bravado and a gross underestimation of the public’s capacity to deal with risk.  More important than any of the several lessons to be drawn from the BSE fiasco was this: the risk of no-risk messages.  For 10 years the British government and leading scientific advisors insisted there was no risk — or that the risk was so infintesimly small that it could be said there was no risk — of BSE leading to a similar malady in humans, CJD, even in the face of contradictory evidence.  The no-risk message contributed to the devastating economic and social effects on Britons, a nation of beefeaters, the slaughter of over 1 million British cattle, and a decrease in global consumption of beef, especially in Japan, at a cost of billions of dollars.

Part of that logic stemmed from the apparent absence of zoonotic or human effects from the sheep transmissible spongiform Encephalopathy, scrapie, which had been know in the UK for hundreds of years.

mad.cows.mothers.milkEuropean researchers have now reviewed the available evidence.

The factors that modulate the transmissibility of Transmissible Spongiform Encephalopathies (TSE) and the approaches for the study of their zoonotic potential are reviewed. The paper ‘Evidence for zoonotic potential of ovine scrapie prions’ by Cassard et al. (2014) is scientifically appraised, focussing on the experimental design, the results and the conclusions.

The paper provides evidence in a laboratory experiment that some Classical scrapie isolates can propagate in humanised transgenic mice and produce prions that on second passage are similar to those causing one form of sporadic Creutzfeldt-Jakob disease (sCJD). It is concluded that the results from the study raise the possibility that scrapie prions have the potential to be zoonotic, but do not provide evidence that transmission can or does take place under field conditions.

The conclusions of the 2011 ECDC-EFSA ‘Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans’ are reviewed in the light of the new scientific evidence available since its publication. This supports and strengthens the conclusions of that opinion with regard to the potential for some animal TSE to be zoonotic, but does not provide evidence of a causal link between Classical or Atypical scrapie and human TSE. Current evidence does not establish this link, and no consistent risk factors have been identified for sCJD.

The possibility of scrapie-related public health risks from the consumption of ovine products cannot be assessed. Recommendations are formulated on further studies and data that are needed to investigate the zoonotic potential of animal TSE and to estimate the amount of infectivity from TSE-infected products sourced from small ruminants and entering the food chain in the European Union.

Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions

EFSA Journal 2015;13(8):4197 [58 pp.]. doi:10.2903/j.efsa.2015.4197

EFSA Panel on Biological Hazards (BIOHAZ)