Bovine spongiform encephalopathy (BSE), commonly known as “mad cow disease,” is a fatal disease that strikes the nervous system of cattle.
BSE is part of a group of diseases called prion diseases that occur in both animals and humans. The main animal forms are chronic wasting disease in deer, scrapie in sheep, and mad cow disease. In humans, there are three different prion diseases: Creutzfeldt-Jacob disease (CJD), also named sporadic CJD (sCJD), Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia. In 1996, another form of CJD was identified; it has since been named “variant CJD” (vCJD). This variant form of CJD has been linked to the consumption of meat products infected with BSE.
Currently, no vaccine or treatment exists to treat BSE, and affected animals display a variety of neurological symptoms before they die (think of television reports showing cows having trouble standing up).
An animal with outward symptoms of BSE may survive for 2 weeks to 6 months, though it may have carried the disease for up to 8 years. BSE has an incubation period (the time between infection and development of symptoms) ranging from 30 months to 8 years, which is a long time for a disease to remain undetected.
Scientists believe that BSE is most often spread through the practice of feeding cattle various meats (rendered material) from slaughtered animals such as sheep, goats, and other cattle.
During this process, an abnormal protein that is linked to BSE can spread from a slaughtered diseased animal to a healthy one. This abnormal protein, called a prion, can withstand high temperatures and does not get destroyed during the rendering procedure. Since the incubation period for BSE is so long, it is possible for an infected animal to enter the food chain before the symptoms appear.
Proteins are long molecules that are folded up into particular shapes. A prion is folded differently from a normal protein, and it can cause normal proteins to change and fold abnormally. When this happens, the proteins (normally found in liquid form in cells) begin to solidify.
The cells most often infected are the brain cells. The resulting solidification of the proteins causes the infected brain tissues to look like a sponge with several tiny holes, hence the name “spongiform encephalopathy.”
Because BSE damages the brain tissue, it has a variety of symptoms ranging from behavioural changes to coordination problems. Cows with BSE may show nervousness or aggressive behaviour, difficulty with coordination, trouble standing up, decreased milk production, and weight loss. The disease is fatal, with death usually occurring 2 weeks to 6 months after symptoms start.
sCJD usually occrs in older patients with an average age of 67, but it has been reported in teenagers and people in their 90s. It is fatal after only a few months. vCJD usually occurs in younger patients and lasts about one and a half years.
Live animals cannot be tested for the disease. The only way to confirm the presence of BSE is by checking the brain tissue of an animal after it dies. Upon examination, the brain is found to be full of small holes, like a sponge.
In humans, a genetic test exists to determine if a person might be susceptible to vCJD, but again, the only way to confirm the diagnosis is through a sample of brain tissue obtained through a biopsy or autopsy.
There is no cure, treatment, or vaccine for BSE, CJD, or vCJD.
The best way to prevent the disease is to avoid feeding cattle rendered material from slaughtered animals, and to isolate and destroy all infected animals. Most countries have developed policies for monitoring BSE in their cattle herds and procedures for dealing promptly and thoroughly with BSE cases when they do arise.
Canada is continuing to work to prevent and control BSE:
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