TSE (BSE/Scrapie/CWD)

Transmissible Spongiform Enzephalopathie

Profile

TSEs are so-called "transmissible spongiform encephalopathies" in animals. These diseases include BSE, scrapie and CWD. The causative agent is a pathogenic prion protein that has heat resistance. It is also resistant to UV and ionised radiation and to disinfectants.

Occurrence

BSE was first described in cattle in the UK in 1986. There are also atypical forms of BSE based on spontaneous mutations of the prion protein. A new variant of Creutzfeldt-Jakob disease in humans was first diagnosed in 1996. Today, transmission of BSE to humans is thought to be caused by food.

Scrapie is a prion disease in sheep and goats that has been known for centuries in Europe and is not transmissible to humans. Atypical scrapie is a single-animal disease whose more precise nature is still being researched, but which also occurs in countries free of classical scrapie.

CWD (chronic wasting disease) is a brain disease occurring in North America in various deer and elk species, the significance of which to humans is not yet clear. In 2016, 5 CWD cases were detected in Norway and thus for the first time in Europe, since then CWD cases have also occurred in Finland and Sweden.

Host animals

The actual origin of the disease is unknown.

Infection route

The disease was spread by feeding cattle with meat and bone meal produced from contaminated animal carcasses and insufficiently treated.

Symptoms

Behavioural changes (anxious/aggressive reactions), uncoordinated gait, falling down, abnormal reactions to touch and sound, fear of crossing the ditch, fear of passageways, fear of smallest obstacles, hypersensitivity to light, muscle tremors.

Situation in Austria

In 2023, no case of BSE was diagnosed in Austria, but one case of "atypical scrapie" was diagnosed in a 10-year-old sheep that had died/been killed. The diagnosis was made at the NRL Mödling using a Western blot and confirmed by immunohistochemical analysis. Since May 2012, Austria has been classified by the World Organisation for Animal Health (WOAH) as a country with a "negligible BSE risk". Since 18 November 2014, Austria has had the status "negligible risk for classical scrapie".

BSE-tests in Austria

The two positive detections in 2010 were atypical BSE cases

Scrapie-tests in Austria

Specialist information

In 2023, cattle aged 48 months and over that died/killed in Austria had to be tested for BSE if they were born in Austria, the EU or the United Kingdom (incl. Northern Ireland), provided that the cattle were moved from the United Kingdom (excl. Northern Ireland) to the EU by 31 December 2020. Cattle that were slaughtered in an emergency/special slaughter or killed due to a slaughter ban because of illness had to be tested for BSE from the age of 24 months. Cattle from Bulgaria and Romania (no revised monitoring programme) as well as Switzerland and third countries such as the United Kingdom (excl. Northern Ireland) had to be tested from the age of 30 months for normal slaughter or 24 months for all other categories, provided that the cattle were moved from the United Kingdom (excl. Northern Ireland) to the EU from 01.01.2021. Testing of healthy slaughtered cattle from 20 months of age was possible at the expense of the person authorised to dispose of the animals. No animals were sent in for testing in 2023.

As part of a risk-based sampling programme, both dead/killed and slaughtered sheep and goats aged 18 months and over were tested for scrapie.

On 18 January 2017, the European Food Safety Authority (EFSA) published a comprehensive scientific opinion - Chronic wasting disease (CWD) in cervids.

In accordance with this opinion, a three-year surveillance programme for CWD was set up in eight European countries (Estonia, Finland, Iceland, Latvia, Lithuania, Norway, Poland and Sweden) from 1 September 2017, with Sweden requesting an extension of the programme until 28 February 2022. Based on the data from this surveillance programme, EFSA prepared a revised and updated scientific opinion, which was published on 17 April 2023.

Contact

Institut für veterinärmedizinische Untersuchungen Mödling

Last updated: 06.05.2024

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