Variant CJD is a rare degenerative brain disease that is caused by consumption of beef products from cows infected with bovine spongiform encephalopathy (BSE), which is commonly referred to as mad cow disease.
Worldwide, more than 220 variant CJD patients have been reported, with a majority of them in the United Kingdom and France. CDC recently confirmed the fourth case in the United States. The patient was admitted to a Houston hospital two weeks prior to death. In each of these cases, infection is believed to have occurred during extensive travel in Europe and the Middle East.
Variant CJD belongs to a group of human and animal diseases called transmissible spongiform encephalopathies (TSEs). Accumulation of prions, which are insoluble misfolded proteins, is thought to be responsible for neuronal death. Patients typically present with behavioral and psychiatric changes.
More information can be found at the Centers for Disease Control and Prevention web site.
http://www.cdc.gov/ncidod/dvrd/vcjd/other/confirmed-case-in-texas.htm
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