Mad cow disease is Bovine Spongiform Encephalopathy (BSE)

Bovine Spongiform Encephalopathy, or BSE, is linked to a similar form of the incurable and fatal brain-wasting disease in humans, called variant Creutzfeldt-Jakob Disease, or vCJD. There have been a small number of cases of vCJD reported worldwide, primarily in the United Kingdom, in people who ate BSE-contaminated meat.

Spongy brains, whether in humans, cows, or other animals, are caused by malformed proteins called prions. Researchers have traced recent outbreaks of the bovine version of the disease to farmers’ cost-cutting practice of mixing bits of dead sheep’s neural tissue into the feed of cows, who are naturally herbivorous. If cows eat the brains of other cows who already have BSE or of sheep suffering from a sheep disease called scrapie, the animals can develop mad cow disease. When people eat infected animals, thus far presumed to be cows, they could develop the human version of the disease, new variant Creutzfeldt-Jakob disease (nvCJD).

Millions of cattle suspected of being infected with BSE in England, Scotland, Ireland, France, Belgium, Italy, and other countries have been incinerated, and various safeguards (few of which have been adopted in the U.S.) have been instituted.

No matter what species it strikes, spongiform encephalopathy is always fatal. There is no treatment. The disease eats holes in the brain. In humans, it initially causes memory loss and erratic behavior, and over a period of months, its victims gradually lose all ability to care for themselves or communicate, and eventually, they die. So far, more than 120 people in Europe have died from nvCJD.

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