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Transmissible spongiform encephalopathies (TSEs)

Transmissible spongiform encephalopathies (TSEs) are a group of neurodegenerative diseases that affect humans and animals. They are always fatal.

TSEs are caused by transmissible agents called prion which are abnormal forms of proteins.

TSEs include:

  • Classical and atypical bovine spongiform encephalopathy (BSE) in cattle;
  • Classical and atypical scrapie in sheep and goats;
  • Chronic wasting disease (CWD) in cervids;
  • Transmissible mink encephalopathy (TME) in mink;
  • Feline spongiform encephalopathy (FSE) in cats;
  • Creutzfeldt-Jakob disease (CJD); variant Creutzfeldt-Jakob disease (vCJD); Gerstmann-Sträussler-Scheinker syndrome; Fatal Familial Insomnia; Kuru in humans.

With the exception of the BSE agent, which can be transmitted to humans through consumption of contaminated meat causing vCJD, there is no scientific evidence that other animal TSEs can be transmitted to humans.

Latest

In November 2023, EFSA published its latest Summary Report on the surveillance for the presence of TSEs in the EU in 2022.  

The report provides an overview of data collected by 27 EU Member States, the United Kingdom in respect of Northern Ireland, and a further eight non-EU countries (Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland and Türkiye). The data covers cattle, sheep, goats, cervids and other animal species, and genotyping in sheep and goats.

The main findings are:

  • One case of atypical H-BSE in cattle was reported in France. 
  • A total of 781 cases of scrapie were detected in small ruminants in the 27 EU Member States and in the United Kingdom in respect of Northern Ireland: 557 in sheep (551 in 2021) and 224 in goats (the same as in 2021). 
  • Finland and Norway confirmed 5 cases of chronic wasting disease in 2022: one wild European moose in Finland; two European moose, one reindeer and one red deer in Norway.  

EFSA updated its story map, which provides general information on TSEs, including the history of the diseases and related control measures. A dashboard to search and visualise the surveillance data is also available.

Milestones

  1. 2023

    April

    EFSA publishes its latest scientific opinion on the analysis of the monitoring programme of Chronic Wasting Disease (CWD) carried out in Norway, Sweden, Finland, Iceland, Estonia, Latvia, Lithuania, and Poland between September 2017 and February 2022. During this period, 31 cases were detected: reindeer (13), moose (15) and red deer (3), in Finland, Norway and Sweden.

  2. 2022

    November

    EFSA publishes the EU Summary Report on the surveillance for the presence of TSEs. The report includes data collected in 2021 in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2021.

    EFSA also updated its story map. It provides general information on TSEs, including the history of the diseases and related control measures.

  3. 2018

    July

    EFSA publishes a scientific opinion on the risk of BSE posed by processed animal protein (PAP) in feed.

  4. 2017

    August

    EFSA publishes a scientific opinion on genetic resistance to TSEs in goats. The evidence provided in the opinion led to the inclusion in EU legislation of the genetic resistance option to control outbreaks of scrapie in goat herds.

  5. July

    EFSA publishes a scientific opinion on the origin of the 60 cases of classical BSE reported in cattle born after the EU ban on the use of animal proteins in livestock feed was enforced in 2001. M Experts concluded that contaminated feed is the most likely source of infection. A second possibility is that contaminated feed ingredients may have been imported from non-EU countries. They could not rule out other causes due to the difficulty of investigating individual cases.

  6. January

    In a scientific opinion, EFSA experts identify monitoring activities and measures to prevent the introduction and spread of chronic wasting disease into and within the EU. They propose a three-year monitoring system across eight countries to detect if the disease is present, which leads to the detection of the first cases in the EU: in Finland (2018) and Sweden (2019). Chronic wasting disease had been discovered in one wild reindeer and one wild moose in Norway in 2016. It was the first time the disease had been detected in Europe and in reindeer in the wild.

  7. 2016

    December

    EFSA publishes for the first time the annual EU summary report on the monitoring of TSE in cattle, sheep, goats, cervids and other species. Previously, the annual reports on TSEs were produced and published by the European Commission.

  8. 2014

    September

    EFSA assesses the state of scrapie in the EU since the introduction ten years earlier of a series of measures to monitor and control the disease.

EFSA's role

EFSA’s role is to provide independent scientific advice to risk managers on all animal and public health related aspects of TSEs in the EU. Most of EFSA’s work is based on requests from the European Commission.

EFSA’s advice provides the scientific basis for EU measures to reduce the risks from TSEs and to maintain a high level of consumer protection in Europe.

Risk assessments and recommendations

EFSA has carried out comprehensive scientific work on different TSEs, especially on BSE, scrapie and CWD.

EFSA’s scientific work on TSEs is managed by the Panel on Biological Hazards (BIOHAZ), relying on the advice of leading experts in all aspects of TSEs, including epidemiologists, pathologists, molecular biologists.

This work includes reviewing the scientific evidence on the zoonotic potential of animal TSEs, like classical scrapie and CWD.

EFSA has also carried out several country-specific assessments. These include the evaluation of the revision of the BSE monitoring regime in Norway and Croatia, and the evaluation of the applications of Finland, Sweden and Denmark to be recognised as having a “negligible risk” of classical scrapie.

Evaluation of TSE rapid tests

EFSA is also involved in preparing protocols for evaluating new rapid tests used to detect TSEs in animals, including ante- and post-mortem tests, as well as in the different steps of the test evaluation procedure, and the review of the diagnostic methods for CWD.

EU framework

The European Commission and EU Member States have taken a series of measures to manage the risk of TSEs in the EU. There is a large body of legislation in place to protect humans and animals from these diseases.

All EU measures on animal TSEs are consolidated in the Regulation (EC) 999/2001.