Disease Diagnosed in Texas: Variant Creutzfeldt-Jakob


Disease from beef products infected with “mad cow” has caused the death of a Texas man from Creutzfeldt-Jakob Disease (vCJD). The diagnosis was confirmed after an autopsy of victim’s brain was completed.The CDC and the state of Texas have said the death is related to beef consumed while the victim was traveling outside the United States.

Twelve countries have reported a total of 220 human fatalities from vCJD since 1996. One hundred seventeen cases have been reported in the United Kingdom and 27 in France. The latest fatality in Texas, reported on June 2, is the fourth case in U. S since the first case was confirmed in 2003.

The Center for Disease Control (CDC) suggests that of the three previous cases in the U. S., two of the deaths came from beef from the United Kingdom and one death came from beef consumed in Saudi Arabia. The most recent victim had a history of traveling extensively throughout Europe and the Middle East. Because infection is likely to have originated outside the United States, the CDC and the state of Texas have determined that there is no imminent health threat in the United States.

VCJD was first described in 1996 in the United Kingdom. It is a rare, degenerative and fatal brain disease in humans that results from people eating bovine products that have been infected with “mad cow” disease or bovine spongiform encephalopathy (BSE). To put it another way, cows get BSE or “mad cow,” and people consume products from those infected cows and develop vCJD. When translated to humans, the infectious agents known as prions are most concentrated in nerve tissue.

Cows pick up “mad cow” disease after they are fed various cannibalistic mixes of the bone meal and carcasses from their deceased herd-mates. The parts of cows that are most likely to transmit the infection when included in cattle feed are bovine brain, spinal cord, and digestive tracts. The practice of including brain and spinal cord tissue in cattle feed was banned in Canada and the United States in August of 1997. The first confirmed case of “mad cow” in America was reported in December 2003. The cow was born in Canada in April of 1997, just four months before the North American cousins established the feed ban.

The disease is nothing but misery for both cattle and humans. Symptoms of vCJD in humans include psychiatric symptoms and behavioral changes, movement deficits, memory disturbances, and cognitive impairments. Suspect symptoms of BSE in cattle include decreased milk production, anorexia, loss of muscular control, and lethargy. Between 1986 and 2001, 180,000 head of British cattle were affected. Because traditional diagnosis could only be performed post mortem, 4.4 million cattle were slaughtered prophylactically. The outbreak devastated the island farming communities.

BSE is known as a prion disease. It occurs in cattle when a normal “brain prion protein” turns into a toxic form of the prion. The process for this toxic transformation, or the incubation period for the disease, lasts from 30 months to eight years, making timely diagnosis a challenge. A new technology called SOFIA (Surround Optical Fiber Immunoassay) shows promise of being able to detect the toxic prion much earlier. Scientific literature suggests that SOFIA may eventually be able to test for other diseases and may be able to be used to test in vitro for diseases like Alzheimer’s and Parkinson’s.

By Kaley Perkins

Department of Defense
The Lancet
Texas Department of State Health Services