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Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a neurodegenerative disease of cattle.
Symptoms include abnormal behavior, trouble walking, and weight loss.Later in the course the cow becomes unable to move. The time between infection and onset of symptoms is generally four to five years.Time from onset of symptoms to death is generally weeks to months. Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). As of 2018, a total of 231 cases of vCJD have been reported globally.
BSE is thought to be due to an infection by a misfolded protein, known as a prion. Cattle are believed to have been infected by being fed meat-and-bone meal (MBM) that contained the remains of cattle who spontaneously developed the disease or scrapie-infected sheep products. The outbreak increased throughout the United Kingdom due to the practice of feeding meat-and-bone meal to young calves of dairy cows. Cases are suspected based on symptoms and confirmed by examination of the brain.
Efforts to prevent the disease in the UK include not allowing any animal older than 30 months to enter either the human food or animal feed supply. In Europe all cattle over 30 months must be tested if they will become human food. In North America tissue of concern, known as specified risk material, may not be added to animal feed or pet food. About 4.4 million cows were killed during the eradication program in the UK.
Variant Creutzfeldt–Jakob disease (vCJD) is a type of brain disease within the transmissible spongiform encephalopathy family.
Symptoms include psychiatric problems, behavioral changes, and painful sensations. The length of time between exposure and the development of symptoms is unclear, but is believed to be years. Average life expectancy following the onset of symptoms is 13 months.
It is caused by prions, which are mis-folded proteins.[6] Spread is believed to be primarily due to eating bovine spongiform encephalopathy (BSE)-infected beef. Infection is also believed to require a specific genetic susceptibility. Spread may potentially also occur via blood products or contaminated surgical equipment. Diagnosis is by brain biopsy but can be suspected based on certain other criteria. It is different from classic Creutzfeldt–Jakob disease, though both are due to prions.
Treatment for vCJD involves supportive care. As of 2012 about 170 cases of vCJD have been recorded in the United Kingdom, and 50 cases in the rest of the world. The disease has become less common since 2000.
vCJD is a separate condition from classic Creutzfeldt–Jakob disease (though both are caused by PrP prions). Both classic and variant CJD are subtypes of Creutzfeld–Jakob disease. There are three main categories of CJD disease: sporadic CJD, hereditary CJD, and acquired CJD, with variant CJD being in the acquired group along with iatrogenic CJD. The classic form includes sporadic and hereditary forms. Sporadic CJD is the most common type.
ICD-10 has no separate code for vCJD and such cases are reported under the Creutzfeldt–Jakob disease code.
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