In Texas, Man Died From Variant CJD

According to the CDC, lab tests have confirmed a diagnosis of variant CJD in a patient who recently died in Texas. Variant CJD is a rare, degenerative, fatal brain disorder caused by consumption of products from cattle with mad cow disease.

Bovine spongiform encephalopathy (BSE), or mad cow disease, has been a problem in many countries for decades. The disease was first reported in 1996, with 229 patients from 12 countries. According to the CDC, from 1996 to June 2, 2014, variant CJD cases have occurred in these countries: 77 from the United Kingdom, 27 from France, 5 from Spain, 4 from Ireland, 4 from the United States, 3 from the Netherlands, 2 from Portugal, 2 from Italy, 2 from Canada and one each from Japan, Saudi Arabia, and Taiwan.

Scientists believe mad cow disease is caused when cattle are fed a diet that contains spinal cord or brain tissue of infected cows. vCJD has a long incubation period. When a person contracts the disease, it means they ate contaminated beef years or even a decade earlier.

The four cases of vCJD that have been reported in the U.S. all lived in other countries. The government says there is “strong evidence” that two of the four cases were exposed to the BSE agent in the UK, and the third was exposed while living in Saudi Arabia. The fourth patient traveled extensively to Europe and the Middle East.

There was an epidemic of BSE in the United Kingdom in the early 1990s, when about 1,000 new cases per week were reported. The outbreak most probably occurred because scrapie-containing sheep meat and bone meal was fed to cattle.

The illness can only be diagnosed after death through a brain autopsy, although “probable cases” can be diagnosed on clinical criteria. CDC monitors the trends and incidences of CJD and variant CJD in this country. Variant CJD is not the same as CJD, which is endemic in this country and around the world. There are several forms of CJD; the most common is sporadic, which means it is random and may develop as a consequence of aging. The average age of death of those with classic CJD is around 68, and has remained stable over the years. The median age of death in patients with vCJD in the UK is 28 years.

The government does have prevention measures against BSE in place, including restrictions against the import of live ruminants from countries where BSE exists, and the prohibition of feed containing animal parts. A study by Harvard Center for Risk Assessment in 2001 concluded that the FDA ruminant feed rule “provides a major defense against this disease.”