A patient who traveled extensively in Europe and the Middle East died recently in Texas of the rare fatal brain disorder associated with the human version of “Mad Cow Disease.” The death, due to variant Creutzfeldt-Jakob Disease (vCJD), was confirmed by laboratory tests conducted following an autopsy of the patient’s brain. Variant CJD is acquired when humans eat products from cows with bovine spongiform encephalopathy (BSE), known in non-scientific circles simply as “Mad Cow Disease.” The Texas vCJD case is the four fatality of its kind identified by U.S. health authorities since 1996, when the fatal human brain disorder was first identified in the United Kingdom. The patients in the three other cases also died in the U.S., but likely acquired vCJD overseas. The first vCJD death in the U.S. occurred 10 years ago. That patient was from the U.K., where exposure to the prion disease occurred. (A prion is an infectious agent which causes tissue damage and cell death in mammals. Prion diseases are currently untreatable and always fatal.) The other two vCJD deaths in the U.S. occurred in 2006, with one patient being from the U.K. and the other from Saudi Arabia. Worldwide, vCJD is also rare. Most of the 220 known cases date back to the late 1990s, when both England and France fought back to bring Mad Cow under control in their herds, and many people were exposed before the danger was completely understood. The U.K. has had 177 cases of vCJD, and France has had 27. Prion disease is less rare. According to the National Prion Disease Pathology Surveillance Center in Cleveland, OH, 3,061 deaths have been blamed on prion diseases. It is not usually identified by biopsy, but by the collection of brain tissue through autopsy after death. Sporadic, inherited and acquired prion diseases are not associated with BSE. Since 1996 and earlier, Cleveland’s prion center has identified 2,567 sporadic cases, 457 inherited (familial) cases, and seven acquired (or iatrogenic) cases in the U.S. Texas and the U.S. Centers for Disease Control and Prevention (CDC) are investigating the new vCJD case. They have not yet released any real details about the victim. Typically vCJD strikes people younger than 55 (average age is 28). Experiencing psychiatric symptoms and dysaesthesia/paresthsias progressing to chorea/dystonia or myoclonus, dementia, ataxia and/or kinetic mutism is common, with death occurring within about 13 months. Sporadic or classic CJD, which is not caused by BSE, is found in only one or two out of every 1 million people in the U.S. Photo Courtesy of National Prion Disease Pathology Surveillance Center: Slide of brain tissue with CJD.