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 2022, no case of BSE was diagnosed in Austria, but one case of "atypical scrapie" was diagnosed in a 14-year-old dead/killed sheep. The diagnosis was made at the NRL Mödling by Western blot and confirmed by immunohistochemical examination. Since May 2012, Austria has been classified by the World Organization for Animal Health (WOAH) as a country with "negligible BSE risk". Since November 18, 2014, Austria has the status of "negligible risk for classical scrapie".

BSE-tests in Austria

The two positive detections in 2010 were atypical BSE cases

Scrapie-tests in Austria

Specialized information

In 2022, cattle that died/killed in Austria had to be tested for BSE from 48 months of age if they were born in Austria, the EU, or the United Kingdom (incl. Northern Ireland), provided the movement of cattle from the United Kingdom (excl. Northern Ireland) to the EU occurred by Dec. 31, 2020. Cattle that were emergency/special slaughtered or killed when slaughter was banned due to disease had to be tested for BSE from 24 months of age. Cattle from Bulgaria and Romania (no revised surveillance program) as well as Switzerland and third countries such as the United Kingdom (excl. Northern Ireland) had to be tested from 30 months of age for normal slaughter or 24 months of age for all other categories, provided the movement of cattle from the United Kingdom (excl. Northern Ireland) to the EU occurred on or after Jan. 1, 2021. Testing of healthy slaughtered cattle 20 months and older was available at the expense of the disposer. Eleven animals were sent for testing in 2022. Under a risk-based sampling program, both dead/killed and slaughtered sheep and goats 18 months of age and older were tested for scrapie. The European Food Safety Authority (EFSA) published a comprehensive scientific opinion - Chronic wasting disease (CWD) in cervids on January 18, 2017. According to this opinion, a three-year surveillance program for CWD was established in 2018 in eight European countries (Estonia, Finland, Iceland, Latvia, Lithuania, Norway, Poland and Sweden), with Sweden applying for a program extension for 2021/2022. Based on the data from this surveillance program, a revised and updated scientific opinion will be prepared by EFSA.

Contact

Institut für veterinärmedizinische Untersuchungen Mödling

Last updated: 10.10.2023

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