Cannibalism holdswarning of mad cow: By eating their dead, a remote Pacific tribe hoped to become immortal. Instead it cursed the living with a horrifying brain disease.

Cannibalism holdswarning of mad cow:
By eating their dead, a remote Pacific tribe hoped to become immortal.
Instead it cursed the living with a horrifying brain disease.

June 3, 2001 The Ottawa Citizen by Mark Kennedy
For generations, the Fore tribe in the remote mountains of Papua New Guinea held lavish mortuary feasts to commemorate the death of one of their relatives.

But it was a feast with a twist: They ate the deceased.

The ritual would begin as the Fore women scooped the brains from the skull of the cadaver. They then wiped the liquid and tissue all over their own bodies, leaving it in their hair and skin for weeks.

Great fires were built to cook the body. The men of the villages -- who were accorded a higher status than women -- ate the best meat, the muscle.

But there was plenty left over. Nothing was thrown away. The women and young children ate the brain and offal. The organs, even the feces, were all eaten.

The bones were baked for weeks, hammered to a fine dust and sprinkled over vegetables. Everyone was eaten; only those with diseases like leprosy or dysentery were shunned. The dead lived on forever, quite literally consumed by their loving relatives, who themselves would later be eaten by the tribe.

It was a cannibalistic ritual born out of love and respect. But it spawned a killer disease -- called kuru -- that nearly brought the tribe to the brink of extinction. At some point, probably around 1915, someone in the tribe had developed a genetic mutation in their body.

Their brain was rapidly wasted away by tiny, sponge-like holes. They died, and nobody in the Stone-Age tribe knew why. Nor could they know that in eating the remains of their tribe member, they were becoming infected with the same neurological disorder -- scientists now believe it was a random case of Creutzfeldt-Jakob Disease (vCJD). [CJD, not vCJD--BSE coordinator]

But gradually, others began dying from the strange illness they called kuru, the "trembling" or "shivering" disease.

Women were particularly vulnerable. Nobody knew why until scientists later figured it out: The women were eating the most infectious parts of a dead person's contaminated body -- the brain and nervous tissue. Before long, it was feared there wouldn't be enough of them left for the tribe to reproduce (men outnumbered women three-to-one in some villages). In the late 1950s, the disease was killing some 200 people a year out of the 15,000-member tribe.

By 1960, Westerners who had come to populate the island put a stop to the cannibalistic ritual they found so abhorrent. Within a few years, the number of Fore with kuru began diminishing slowly.

Four decades later, the story of kuru is one that could hold the secret to many mysteries surrounding a more pressing public health scare, commonly known as mad-cow disease, which some fear could become a worldwide epidemic.

Kuru foreshadowed the mad-cow disaster that killed at least 180,000 British cattle in the 1980s and '90s. It proved that, putting aside moral or philosophical arguments, cannibalism can be a disease's best friend.

The Fore died of kuru because they ate the remains of other Fore who died of kuru. British cows died of bovine spongiform encephalopathy (BSE) because they were fed the protein-rich remains (known as meat and bonemeal) of other cows infected with BSE.

Dr. Michael Alpers, a recently retired scientist in Perth, Australia, is one of the world's foremost kuru experts. As director of the Papua New Guinea Institute of Medical Research from 1977 to last year, Dr. Alpers spent much of his career studying kuru. He was one of a handful of scientists who discovered the disease in the 1960s.

Along with world-renowned scientist Dr. Carleton Gajdusek (who later won a Nobel Prize for his medical detective work on kuru), Dr. Alpers lived among the Fore in the early '60s.

He watched their symptoms progress: the unsteadiness of gait, sometimes the uncontrolled laughter (early media reports dubbed it the "laughing death"), followed by an inability to walk, talk or eat."It's a terrible, slow death," says Dr. Alpers. "Eventually, the person just lies around the house in a moribund state and dies." Usually, death came within a year. But sometimes it could claim its victim within three months or the agony could last three years.

Dr. Alpers and Dr. Gajdusek collected the brains of some victims and sent them to a lab in the United States.

In 1963, the infected brains were mashed in a blender and injected into the brains of three chimpanzees. Two years later, Dr. Alpers, who by then had moved to the U.S. to work in the lab, was the first scientist to witness a groundbreaking discovery. One of the chimps, named Georgette, began showing similar clinical symptoms to those among the Fore who died of kuru. Georgette shivered, her lip drooped, she constantly lost her balance and fell out of her cage, she grew steadily weaker. Eventually, she was euthanized and her brain was sent to London for analysis. The stunning telex that Dr. Alpers received: "PATHOLOGY OF GEORGETTE INDISTINGUISHABLE FROM HUMAN KURU."

It was a remarkable discovery. The disease had crossed the species barrier, from humans to chimpanzees.

Unfortunately, when BSE emerged among British cattle 20 years later, government scientists insisted there was no reason for alarm. That disease, they said, clearly posed a threat to cows. But humans who ate contaminated beef, they said, were safe. BSE would not cross the species barrier. But it did.

These days, Dr. Alpers doesn't skip a beat when asked to comment on another central issue about the mad-cow scandal: whether governments made a huge mistake in allowing cows to be fed to cows.

"I think that's a valid view," he says. "If we used kuru as a model -- and I wasn't asked for my advice on this matter -- we could have said much earlier there are risks here."

Dr. Alpers says kuru may hold the answer to another critical question now baffling the world's scientists.

No one knows how any people may ultimately die from variant Creutzfeldt-Jakob Disease (vCJD), the strain of the neurological disease that humans got in recent years from eating BSE-contaminated beef.

So far, 104 people have contracted the disease. Scientists disagree on how high the final toll will be.

Part of the answer lies in how long vCJD incubates in humans before it emerges to kill all its victims, usually within 18 months. If the incubation period is 10 to 15 years, that's good news. It means the cases of vCJD seen so far may represent a fair portion of the final tally. But if the disease has a much longer incubation period, it's bad news. It means the cases of vCJD that have emerged represent only the tip of an iceberg.

The truth is, no one knows. No one's ever seen something quite like this before. The closest thing that comes to it is kuru. And with that disease, scientists have found a very long incubation.

For the past 40 years, new cases have continued to emerge of kuru. To this day, scientists see one or two new cases a year of middle-aged Fore tribe members who would have been contaminated as children, before cannibalism was stopped.

Dr. Alpers is not an optimist about the future numbers of vCJD victims. Like kuru, he suspects vCJD has an extended incubation period that could stretch for up to several decades.

"If you use kuru as a model, this is what you can expect," says Dr. Alpers. "The picture is pretty bleak. It's not great news. Everyone around the world is going to have to face this."

GRAPHIC: Black & White Photo: A middle-aged female kuru victim. Women, were particularly vulnerable to kuru, the 'trembling' or 'shivering', disease.; Black & White Photo: Trevor Collens, The Ottawa Citizen ;, Australian medical researcher Michael Alpers was one of a handful of, scientists who discovered the kuru disease in the 1960s.

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