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Bovine Spongiform Encephalopathy

Last updated: March 2, 2017

What Does Bovine Spongiform Encephalopathy Mean?

Bovine Spongiform Encephalopathy (BSE), colloquially called “mad cow disease,” is a disease of the central nervous system that occurs in cattle. BSE is a degenerative disease first described in the United Kingdom in 1986 and is always fatal.

BSE is one of a group of diseases known as transmissible spongiform encephalopathies (TSEs) that can be transmitted to humans. Human consumption of cattle infected with BSE is associated with a human TSE known as "(new) variant Creutzfeldt-Jakob Disease" (nvCJD). Formally identified in 1996, nvCJD is widely suspected to be caused by transmission of the same infectious substance that causes BSE. All human TSEs, including nvCJD, are inevitably fatal.

Safeopedia Explains Bovine Spongiform Encephalopathy

Exposure to BSE is most strongly associated with cattle from the United Kingdom. The first confirmed incidence of the disease was reported in the UK in 1986, and the following years would see the country faced with an epidemic that was far more severe and long-lasting than that experienced by any other country.

BSE and all other forms of TSEs are caused by a group of infectious proteins called prions. Prions are distinct from bacteria and viruses, and it is unknown how a normal protein becomes a prion.

Because BSE poses a direct risk to humans, it is categorized as a biological agent under the UK Health and Safety Executive’s (HSE) Control of Substances Hazardous to Health Regulations (COSHH) 2002, as well as the regulations of equivalent occupational health and safety agencies worldwide.

Although it is considered unlikely that BSE will affect human health within the occupational setting, handling of infected material still requires safety precautions designed to limit exposure. The primary occupational groups at risk of exposure to BSE are agricultural industry workers who may come into contact with infected material through the handling, disposal, or processing of cattle as well as laboratory workers who are conducting work involving infected material.

There is currently no evidence of nvCJD being acquired through occupational exposure to BSE; however, an excess of classical (non-variant) CJD cases has been found among workers on both dairy farms and farms with a confirmed case of BSE. Because the incidence of classical CJD in farm workers in the UK is equal to that of farm workers in countries that have not had BSE epidemics, the significance of this observation in terms of occupational safety is not understood.

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Synonyms

Mad Cow Disease

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