Officials also stressed that consumers should not yet be anxious by the case because the disease did not enter the human food chain.
BSE attacks a cow's central nervous system and is usually fatal.
A case of mad cow disease has been reported at a farm in Scotland.
The last breakout of BSE in the United Kingdom that involved human deaths happened in 1986, in which over 100 people died of the human form of mad cow disease.
Officials have stressed the case poses no risk to human health and that its discovery proves the surveillance system in place is working effectively.
Ms Voas told BBC Radio's Good Morning Scotland programme: "The animal itself is dead, she died before she was tested, and there are three other animals, possibly four, on the farm that will need to be slaughtered purely as a precautionary basis".
She said the disease could occur "spontaneously" and that it was believed this was probably the case in this latest situation.
Britain grappled with a massive outbreak throughout the 1990s when hundreds of thousands of cattle were diagnosed with BSE, resulting n a worldwide ban on beef exports from the United Kingdom.
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Ian McWatt, director of operations at Food Standards Scotland, said: "There are strict controls in place to protect consumers from the risk of BSE, including controls on animal feed, and removal of the parts of cattle most likely to carry BSE infectivity".
The finding of the disease is a "disappointment", said Scotland's Chief Veterinary Officer, Sheila Voas. But there were two more recent cases in 2015. And one positive test has overturned it. In recent months, the industry has been courting China as a new market.
Does it mean the meat is any less safe?
More than 180,000 cattle were thought to have been struck down by the disease and the European Union put a ban on importing British beef between 1996 and 2006.
"It's not the start of an outbreak, it's a single isolated case that won't affect the food chain".
'We are working closely with the Animal and Plant Health Agency to answer this question, and in the meantime, I would urge any farmer who has concerns to immediately seek veterinary advice'.
Mad cow disease - as it is more commonly known, because of the animals' erratic behaviour and movements - destroys their brains by eating away the nerve tissue.