A Texas man who recently died from variant Creutzfeldt-Jakob disease (vCJD) probably contracted the disease while travelling overseas, according to the U.S. Centers for Disease Control and Prevention (CDC). Variant CJD is the fatal brain disorder linked to consumption of products from cattle with Bovine Spongiform Encephalopathy (BSE).
According to the CDC, the patient had travelled extensively in Europe and the Middle East. Likewise, the three previous cases of vCJD in the United States involved two people who had lived in the United Kingdom and one who had lived in Saudi Arabia. According to a CDC fact sheet on the disease, there has never been a case of vCJD that did not have a history of exposure within a country where the cattle disease, BSE, was occurring.
Variant CJD is distinct from Classic CJD, which occurs around the world and is not known to be linked to BSE or other animal diseases. In the United States, Classic CJD occurs in one to two people per million, and it tends to affect older people, in contrast with vCJD where cases have a lower median age.
Variant CJD remains extremely rare worldwide. Since the disease was first identified in 1996, 229 cases have been reported. The largest concentration of cases occurred in the UK, with 177, and in France with 27. In the early 1990s, BSE became an epidemic in the UK, and reported cases reached a peak of nearly 1,000 per week in 1993. Scientists believe the emergence of BSE resulted from feeding cattle processed byproducts such as bone meal from sheep infected with scrapie , which is a related disease. Once that practice stopped, the incidence of BSE and vCJD dropped off significantly.
In the United States, the FDA instituted a ruminant feed ban in June 1997 that became fully effective as of October 1997. In October 26, 2009, FDA implemented enhanced BSE-related feed ban with tighter controls. In late 2001, the Harvard Center for Risk Assessment study of various scenarios involving BSE in the U.S. concluded that the FDA ruminant feed rule provides a major defense against this disease.
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