Though the exact cause of BSE is not known but it is generally accepted by health experts that the likely cause of BSE in animals is the formation of unnatural protein called prion. This abnormal protein Prion is generally found in central nervous tissues such as brain and spinal cord and other tissues of infected cattle. Prions eat away the brain and create tiny sponge like holes in parts of the brain. These so-called spongy holes cause slow degeneration of the brain of the cattle. The symptoms eventually spread to the whole body. BSE has a long incubation period, about 3 to 4 years, usually effecting adult cattle at a productive age onset of 4 to 5 years.

Most health experts believe that the disease may be transmitted to human beings who eat meat of the cattle infected with BSE. The tainted protein prion is spread from infected cattle to humans when the persons eat meats or beef products. In humans it is known as new variant Creutzfeldt-Jakob disease. This disease was first described by German neurologist Hans Gerhard Creutzfeldt in 1920 and shortly afterwards by Alfons Maria Jakob in 1922. Hence, it is known as Creutzfeldt-Jakob disease. People can also inherit Creutzfeldt-Jakob disease genetically through mutation of gene that codes for the Prion protein this only occurs in 5 to 10 percent of all CJD disease.

At present there is no treatment for Creutzfeldt-Jakob disease. The course of the disease is disease varies from 4 weeks to 24 months, usually resulting in death of a patient. People can reduce their risk of getting Creutzfeldt-Jakob disease by abstaining from eating beef completely or contaminated beef products.

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