Brevard doctor: U.S. should prepare for 'Mad Cow' arrival

April 19, 2001 Florida Today by Susan Jenks

Federal agencies have stepped up inspections of cows and the feed industry nationwide, amid growing fears that the Mad Cow disease found in Europe will spread to the United States. Humans eating meat from infected cows can contract a disease that is always fatal.

So far, the problem has been confined mostly to England. There have been no confirmed cases in the United States of either Mad Cow disease in cattle or of its human counterpart, a new form of an old illness called Creutzfeldt-Jakob disease.

Even so, Rockledge neurologist Dr. Richard Newman and others think, it may be only a matter of time before the disease makes its way here from Europe, where it first erupted in 1996.

Indeed, Newman might have seen one local patient with the new form of Creutzfeldt-Jakob disease about a year ago. However, "her case was not clearly positive," he said. It was one of dozens of suspicious cases in the United States.

The otherwise-healthy 52-year-old woman suddenly developed suspicious neurological symptoms: rapidly progressive dementia, blindness and an early loss of motor control. She died in less than a year. She had no known exposure to contaminated meat from abroad, Newman said.

Humans get the disease by eating meat from infected cattle that were either slaughtered using processes that mix brain or spinal-cord tissue with meat, or fed rendered animal byproducts that included diseased brain or spinal-cord tissues.

To get a certain diagnosis of Creutzfeldt-Jakob disease requires a brain biopsy or an examination of the brain at autopsy. The patient had a brain biopsy, Newman said, but the results were inconclusive. No autopsy was performed because doctors were unable to get consent from next of kin, so no firm diagnosis was established.

The grim expectation that the disease may eventually show up in the United States is based on two realities:

The uncertain incubation period of Creutzfeldt-Jakob, which scientists say may be as long as 20 years. With that many years between exposure to Mad Cow and the incidence of illness, it is extremely hard to trace the source of exposure.

The large number of farmers, ranchers, feed producers, meat packers and others who are involved in the meat industry.

"One way or the other, a case may break through," said Dr. Richard Johnson, chairman of the neurology department and a Mad Cow disease expert at Johns Hopkins University School of Medicine in Baltimore.

Diagnosing and tracking the disease is made more difficult by the fact that there are two forms of Creutzfeldt-Jakob disease. The classic form, described in the 1920s, and not associated with Mad Cow, is extremely rare, striking only one in 1 million people.

The new form of the disease is the one associated with Mad Cow disease. That version is even more rare than the classic form, with 99 cases since 1996.

To put it in perspective, Johnson said, consider there are at least a half-million deaths in the United States from food-borne illnesses. For greatest safety on a statistical basis, "you would have to give up all your other foods, like chicken and shellfish first, before beef," he said.

A cattle state

In Florida, which ranks No. 12 among the nation's beef-producing states, Mad Cow disease is viewed as a distant threat to the state's estimated 1.8 million cattle.

At Duda & Sons in Oviedo, for example, David Willis, vice president of the cattle division, said only beef cows are raised, not dairy cows - an important distinction in terms of Mad Cow disease. In general, because dairy cows fatten more slowly, they are allowed to live longer than beef cattle, giving the disease more time to incubate.

Also, Duda, the largest cattle breeder in this part of the state, does not import cattle from Europe, Willis said. The risk of Mad Cow disease can be reduced by not importing cattle from Europe - a government policy in effect since 1989 - and by banning the importation of potentially contaminated feed, a restriction in effect for several years.

In addition to the U.S. Department of Agriculture and the Food and Drug Administration focusing on inspecting of cows and the feed industry, the Agriculture Department seized and destroyed several flocks of sheep in Vermont last month, fearing they might harbor an illness related to Mad Cow.

Mad Cow disease and scrapie, the name for a like disease in sheep, belong to a group of disorders called transmissible spongiform encephalopathy. While Mad Cow disease can spread to humans, the disease in sheep has never been linked to human disease.

Transmissible spongiform disorders are so named because they literally turn the brain to sponge. On autopsy, Johnson said, "you can actually see tiny holes in the neurons or nerve cells in the brain."

It is thought that the disease first spread from sheep to cows in the early 1980s, when British cattle were fed infected sheep parts.

Damage to the brain results when the disease causes a special protein called a prion to form plaques in the brain and spinal cord.

"All the other organs are normal," Johnson said.

99 European cases

The first evidence that the disease affecting cows - Bovine Spongiform Encephalopathy - in the United Kingdom had spilled over into humans came in March 1996, when British doctors reported the first 10 human cases.

Since then, there have been 99 confirmed or suspected cases of the related human disease or new variant Creutzfeldt-Jakob - all in Europe, and all but four cases in the United Kingdom, according to the European Commission on Food Safety.

In the United States, there have been "dozens of suspected cases" similar to that seen by Newman, but none has been confirmed, said Tom Skinner, a spokesman for the Centers for Disease Control and Prevention in Atlanta.

"Any time there's a case in a person under age 55, we take a look," he said. Autopsy samples of brain tissue are taken from the infected individual and sent to a neuropathologist who is under contract with the federal agency.

All of them have turned out to be the classic form of the disease, in which heredity plays a role in about 10 percent of patients and the rest have no known infectious source and no evidence of a genetic link.

Dr. Thomas Hoffman, a neurologist at Osler Medical in Melbourne, said he has seen several patients in his practice with the classic, or original form of Creutzfeldt-Jakob, but no clear cause was determined for any of the cases.

The form of Creutzfeldt-Jakob associated with Mad Cow disease differs from its older counterpart in several ways.

While patients with the older version are typically in their 60s or older, the median age of patients who contract the newer illness is 28, and they develop not only dementia, but suffer delusions as well.

In addition, there may be a burning sensation in the extremities, pain and an early loss of motor control, Johnson said.

The course of disease is different as well. There is a different pattern of abnormal protein deposits in the brain, which is clear-cut on autopsy, the only definitive way to distinguish the new from the old form of the disease, Johnson said.

No matter which type of disease develops, though, the onset of dementia is rapid and so is death.

For now, at least, experts agree that in the United States, the Mad Cow risk remains relatively small.

"The way we handle meat products is much different than in Europe," Hoffman said. Investigators say the old-fashioned technique used in some areas of Europe allowed cow brains or spinal cord to mix in with the rest of the cow, contributing to the outbreak of disease there.

Still, concerns remain.

Newman, whose daughter is going to Europe this summer, said he has forbidden her to eat any beef or beef products.

"It's OK for her to eat chicken and fish," he said. "No one gets the disease" from them.

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