Mad Cows and Englishmen

In 1913, in the town Breslau, Germany, a young physician named Hans Creutzfeldt was confronted by a most unusual medical case. His patient was a twenty-three-year-old maid in a convent who had suffered a bizarre and abrupt personality change. She had stopped bathing, stopped eating, and had become dirty and emaciated. She suffered from sudden fits of giggling and laughter, alternating with shrieks of horror and claims that she was possessed by the devil. Her eyes twitched, her arms jerked, and she was unable to walk without falling. Over the next two months, she suffered repeated seizures, eventually lapsing into a coma. She died only two months after the onset of her first symptoms.

At autopsy, a stunning abnormality was found on microscopic examination. Her brain tissue was riddled with so many holes it resembled a sponge. The term “spongiform encephalopathy” was born. This was the first recorded case of what later came to be known as Creutzfeldt-Jakob Disease, or CJD. Occurring sporadically, CJD strikes one person in a million, both men and women, usually around age 60. The first signs are of rapidly progressive memory loss and impaired judgment, often accompanied by wild mood changes and frightening hallucinations. The patient twitches, stumbles around, and often falls. Within weeks to months, he is dead. There is no cure.

For years, no one knew its cause.

DON’T READ THIS WHILE YOU’RE EATING

In 1957, physicians on the island of New Guinea struggled to make sense of a baffling epidemic among women and children of the Fore tribe. Hundreds were dying of a disease that caused its victims to shiver, to laugh wildly and fall twitching to the ground. All the afflicted died. Biopsies of their brains revealed tissue that had so many holes, it looked like a sponge. You guessed it — the victims were dying of Creutzfeldt-Jakob Disease. But why were there so many cases? And why were the men unaffected? What were the women and children doing that the men weren’t?

The truly creepy answer: They were eating their dead relatives. That’s right. Feasting on grandma and grandpa, on dear old mom and dad. If your husband died, you, as the surviving spouse, got first dibs on your favorite body part.

Late at night, after a death, the women would take the corpse into the fields. There they would expertly butcher their dear departed loved one, disjointing the flesh to be steamed over cooking fires. They dined on every part of the body, distributing the meat only among the women and children. The men, poor things, were not invited to these feasts — unless they were the main course.

Alas, the eating of human flesh has its drawbacks. What the women had ingested, along with human brains, was the infectious agent for CJD. As more and more women died, and were in turn consumed, the disease spread until there were twice as many men alive as women in this tribe.

It was the controversial Dr. Stanley Prusiner who finally identified the infectious agent. it is neither a virus nor a bacteria. In fact it is not, strictly speaking, even a living organism. It is a protein. Prusiner dubbed them “Prions.”

THE DISEASE JUMPS SPECIES

In 1990, a pet cat in England began to twitch and stumble. When he was finally euthanized, and his brain examined, veterinarians declared him the first case of feline CJD ever reported. Over the next four years, sixty two more cats died of the same disease. All had been fed pet food derived from English beef.

“Mad Cow Disease,” the bovine form of CJD, had in fact been identified years earlier in English cattle. Its spread through the herds in England may have been caused by the routine use of “downer cow” remains in cattle feed. (“Downer cows” are those found dead of unknown causes.) In order to speed up the growth of veal calves, farmers would feed them extra sources of protein. In this case, the protein source was dead cows. Bovine cannibalism had proven just as unhealthy as human cannibalism.

In 1993, the epidemic passed over into humans when two English farmers died of CJD. By 1996, a dozen Englishmen and women were dead of the disease. The source of their infection? English beef. For the first time we had incontrovertible proof that prions could be passed from one species to another. And if humans could catch it from eating beef, why couldn’t we also catch it from milk or butter or even that old childhood favorite, Jell-O, which is derived from beef products? If cows, cats, and humans can catch CJD, what about other mammals? The list, in fact, keeps growing, with reports of “mad mink disease” and “mad squirrel disease.” Every mammalian species may, in fact, be susceptible.

Yet another cause of worry is the fact that scientists do not yet know how many people have been infected by eating beef. Since the latency period for CJD may be as long as ten years, England may be incubating a silent epidemic that is yet to make itself apparent. The British are now launching a study to find out just how many undiagnosed cases of CJD they may have brewing. They will search for prions in hundreds of surgically removed tonsils and appendixes which have been kept preserved and stored over the years. (And you thought you collected weird things!)

A SNACK YOU MIGHT WANT TO AVOID

Finally, one last bizarre tidbit of information. In 1997, a dozen people in Western Kentucky died of CJD. In the valley where these victims lived, a favorite local snack was … squirrel brains. A traditional gift to present your hostess when you went visiting was a sack of squirrel heads. The hostess would prepare the delicacy by first shaving off the fur, then frying up the heads in a cast iron skillet. The diners would then crack open the skulls and suck out the brains. It’s rumored to be quite a tasty snack. Yum.

In Tess’s medical thriller LIFE SUPPORT, an E.R. doctor tracks down the source of a mysterious outbreak of Mad Cow Disease among rich men in Boston. The solution to the mystery lies with a pregnant teenage prostitute who carries, in her womb, a secret horror…