BSE, also known as mad cow disease, stands for bovine spongiform encephalopathy. This infectious disease affects the central nervous system of cattle. Wageningen Bioveterinary Research (WBVR) conducts research into this disease.
BSE belongs to the group of transmissible spongiform encephalopathies (TSEs). It is caused by prions; proteins that can deform the normal proteins in the brain. Mad cow disease spreads primarily through reuse of animal proteins in feed.
In 1986, BSE was first found in the United Kingdom where it resulted in disaster for cattle farmers the following years with over 180,000 infected cows. From the UK, mad cow disease spread to other countries. In the Netherlands the last case of BSE was reported in January 2011.
This infectious cattle disease also causes a variant of Creutzfeldt-Jakob disease; a deadly human brain disease. This could be caused by eating contaminated organs from infected cattle (particularly brains).
BSE and other TSEs are caused by prions; proteins that can deform the normal proteins in the brain. Prion diseases can be caused by infection, but also by small changes in the genes that produce these proteins. Prions are very resistant to heating and disinfection, and are also resistant to protein-splitting enzymes.
The disease is seen in adult cattle, mainly at the age of three years and older. There are concerns that it may also occur in other small ruminants. Until now, however, only one case of the disease has been detected under natural circumstances in a French goat And one case in a British goat. No BSE cases have been reported in sheep so far.
The central nervous system of the animals is affected by BSE. Microscopically small cavities develop in the brains of the animals, which cause behavioural changes (startling, hypersensitivity to light, sound, touch) and movement disorders. The period between the infection and the first symptoms is usually several years. Due to progressive paralysis, the disease eventually leads to death.
The disease begins gradually and is therefore difficult to diagnose in the early stages. Especially in the early stages, the symptoms of BSE can be easily confused with symptoms of metabolic diseases and other diseases affecting the central nervous system.
Usually, the mad cow disease starts with behavioural changes, such as seeking isolation, hypersensitivity to noise and other stimuli, after which severity gradually increases. The animal can become unmanageable, can panic and sometimes become aggressive. Teeth grinding and muscle twitching have also been observed. Movement disorders also occur: a swinging gait, hindlimb ataxia, and at a later stage difficulty in turning and frequent falling.
Epidemiological data have clearly shown that BSE spreads primarily through the reuse of animal proteins (cattle proteins) in animal feed. This results in a circular flow of animal material in which pathogens can multiply. This problem was recognised long ago. As a result, sterilisation has become an important part of processing animal cadavers.
Other transmission routes may also be possible, including transmission from mother cow to calf at birth and transmission via the environment, but none of these transmission routes have been proven conclusively. If the infection can be transmitted via these other routes, the role of these routes is very limited.
It is assumed that the outbreak of BSE was caused by feed concentrates that included insufficiently sterilised meat and bone meal originating from carcasses of sheep or cows that were infected with prion disease. In Great Britain, the processing method for carcasses was changed in the early 1980s, and a second heat treatment with steam was discontinued. Because BSE prions are resistant to high temperatures, they were no longer eliminated during processing, and it became possible for them to contaminate meat and bone meal. This possibly contaminated meat and bone meal was included in feed concentrate for cattle.
Animals contracting BSE became infected especially during their first year of life by eating contaminated concentrate.
BSE is getemd, maar onzekerheid blijftBSE is getemd, maar onzekerheid blijft, Food & Agri business, 2022-03-25, Rene Stevens
Stability of BSE infectivity towards heat treatment even after proteolytic removal of prion proteinVeterinary Research 52 (2021)1. - ISSN 0928-4249
Inactivation of H-type and L-type bovine spongiform encephalopathy following recommended autoclave decontamination proceduresTransboundary and Emerging Diseases 67 (2020)5. - ISSN 1865-1674 - p. 1872 - 1878.
Characterization of goat prions demonstrates geographical variation of scrapie strains in Europe and reveals the composite nature of prion strainsScientific Reports 10 (2020)1. - ISSN 2045-2322
Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methodsVeterinary Research 50 (2019). - ISSN 0928-4249
Protecting effect of PrP codons M142 and K222 in goats orally challenged with bovine spongiform encephalopathy prionsVeterinary Research 48 (2017)1. - ISSN 0928-4249
EU-approved rapid tests might underestimate bovine spongiform encephalopathy infection in goatsJournal of Veterinary Diagnostic Investigation 29 (2017)2. - ISSN 1040-6387 - p. 232 - 236.
Genetic, histochemical and biochemical studies on goat TSE cases from CyprusVeterinary Research 47 (2016). - ISSN 0928-4249 - 14 p.
Prion type-dependent deposition of PRNP allelic products in heterozygous sheepJournal of Virology 90 (2016)2. - ISSN 0022-538X - p. 805 - 812.
Behoud TSE-ongevoelige geiten (TSE=overdraagbare sponsvormige hersenaandoening zoals scrapie, atypische scrapie, of BSE)Zeldzaam huisdier 2015 (2015). - ISSN 0929-905X