In April he seemed fine. In May his golf game suddenly got worse. In June his arm began rising in the air as if it didn't belong to him, and one night in July he got lost in his own walk-in closet. By early August he had lost his mind. By the end of August he was dead.
This was the swift and terrifying course of Ken Johnston's illness - a disease with an unwieldy name and no cure. According to an autopsy report, he suffered from Creutzfeldt-Jakob Disease, also known as CJD.There is no way to tell what caused Johnson's CJD. But there are some people who believe that cases like his are part of a "hidden epidemic." Maybe as many as 13 percent of Alzheimer diagnoses, they argue, are really CJD, and some of those CJD cases are the human version of mad cow disease, caused by the eating of infected meat.
It's not just British beef that can give you a mind-wasting disease, they argue. U.S. beef, they say, may be infected with a strain of mad cow disease.
Proponents of this theory will gather Saturday at Weber State University to participate in a conference focusing on "Emerging Plagues and Cures."
The conference, sponsored by a Utah-based volunteer organization called LifeSave International, will feature the authors of several books about food dangers, including two about mad cow.
"We don't want to set off a panic. But we do want the government to take preventive measures now," says Ronnie Cummings, director of the national Pure Food Campaign and a researcher for "Mad Cow USA: Could the Nightmare Happen Here?"
What Cummings and other food activists charge is that the U.S. government allows practices to continue that may spread CJD from animals to humans.
Originally it was believed that CJD cases were hereditary or occurred randomly, at a rate of one case per million people. Generally these cases occurred in people over age 50.
But in 1996, when CJD began to crop up in younger Britons following the nation's "mad cow" epidemic in cattle, scientists began to speculate that some strains of the human disease and the cow disease were linked.
Last month, researchers reported in the science journal "Nature" that a "new variant" form of CJD - that has killed 26 people under the age of 42 in Great Britain - is indeed identical to mad cow and was likely caused by eating infected beef.
Mad cow's technical name is bovine spongiform encephalopathy (BSE), which is just one version of a group of diseases called transmittable spongiform encephalopathy (TSE).
Spongy areas in TSE brains cause a progressively degenerative disease with symptoms like Johnston's. Victims eventually lose everything - the ability to see, move, swallow and remember. The losses often occur rapidly, and the result is sort of Alzheimer's on fast-forward.
The two diseases are so similar that some cases of CJD are diagnosed as Alzheimer's. A 1989 University of Pittsburgh autopsy study of 54 patients diagnosed with Alzheimer's or other dementias found that 5.5 percent actually had suffered from CJD. A similar Yale University autopsy study that same year found 13 percent of Alzheimer patients actually had CJD.
"Even if we're conservative and say 1 percent (of Alzheimer patients) have CJD," says Cummings, "that would be 40,000 people in the U.S. with CJD."
And, note John Stauber and Sheldon Rampton, authors of "Mad Cow USA," "40,000 cases could be just the beginning of something much larger" - since CJD often incubates for decades before producing symptoms.
The U.S. Centers for Disease Control in Atlanta is not so quick to buy these figures. But it is beginning a new "active surveillance" of the disease, says CDC spokesperson Tom Skinner. "We'll work with professional associations in neuropathology to come up with better numbers." The new surveillance, he says, has been spurred by the new variant of CJD in Great Britain.
Not all scientists who study TSE diseases believe there is a link between U.S. meat and CJD. Stanley B. Prusiner, who is credited with the discovery of prions - the proteinaceous infectious agent responsible for TSEs - ate a steak after winning the Nobel Prize last month.
"I don't think we in America should be worried" about the disease, Prusiner told reporters after getting the Nobel Prize.
But food activists like Cummings argue that the government is ignoring an epidemic and is glossing over the fact that a strain of mad cow has apparently shown up in U.S. cattle.
The culprit, they argue, is the practice of feeding feedlot animals the rendered bodies of other animals. Last July the USDA banned part of this process - the feeding of the rendered meat and bone from ruminants and mink back to ruminants.
But, notes Cummings, the ban is full of loopholes - allowing, still, the feeding of cow blood to cows, plus pig parts to pigs, as well as pig parts to cows. Cows and pigs are also fed rendered roadkill and euthanized pets.
The problem, says "Mad Cow USA" author Stauber, is that if one cow or pig has a TSE disease, and that pig or cow is fed to other animals, the highly infectious disease can spread. TSEs are notoriously hardy and persistent - they can incubate for decades, and are not killed by either radiation or autoclaves, much less normal cooking.
According to Michael Hansen of Consumer Union's, who will speak at the Weber State conference, two epidemiological studies have linked the consumption of pork to CJD. A 1985 study of 26 CJD patients found that in nine of the patients "food items . . . were statistically linked to increased risk of CJD," including pork, ham, hot dogs and pork chops.
Although no cases of mad cow disease have been found in U.S. cattle, say Hansen and other activists, that may simply be the result of inadequate testing - and the fact that mad cow in America may actually be a different strain, one that causes more subtle symptoms in cattle.
According to Hansen, a TSE outbreak at a Wisconsin mink farm in the mid-1980s has been linked to cows. Ninety-five percent of the diet of the affected mink consisted of "downer" cows - those cattle that die on the range or in feedlots before they are slaughtered, having succumbed to broken legs or disease.
Researchers from the University of Wisconsin injected brains from the dead mink into the brains of two living Holstein calves; within 19 months both calves had developed a fatal TSE. When the researchers then either injected or fed other mink the infected calf brains, the mink developed a TSE.
Unlike the "mad cows" of England, the U.S. cows infected with this U.S. strain of TSE exhibited only a bit of lethargy before keeling over. They looked, in other words, like any ordinary downer cow.
For this reason, say food activists, the government should test more of the 100,000 downer cows each year in the United States. Currently, Hansen says, only a couple hundred downer cows have been examined.
In addition, say food activists, the government should require that all humans diagnosed with Alzheimer's be tested for CJD.
Johnston - who grew up in Utah before moving to Spokane in 1960 - was only discovered to have CJD when his brain was autopsied. For most of his illness, Johnston was believed to be suffering from a psychiatric illness, says his widow Peggy, who will attend the foods conference Saturday.
Peggy Johnston says her husband was told over and over that he simply wasn't trying hard enough to get well.
The conference runs from 9 a.m. to 4 p.m. Pre-registration admission is $10. For more information, call 294-2970. In addition to mad cow, the conference will explore weight loss, Prozac, fish toxicity and vaccinations.