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More deaths from mad cow disease predicted

By Donald G. McNeil Jr. The New York Times

 

Published: June 23, 2006

 

 

NEW YORK The long lives that some former cannibals enjoy before

succumbing to a brain-wasting disease suggest that many more humans

will eventually die of mad cow disease, according to a study released

Friday.

 

But several experts in such illnesses, called prion diseases - which

are blamed for killing New Guinea cannibals and British eaters of

infected beef - disagreed with that frightening implication of the

study, which was published Friday in The Lancet, the British medical

journal.

 

These experts praised the rigorous work the authors of the report did

to confirm that kuru, a disease that once decimated highland tribes in

New Guinea, can incubate for 50 years in a few genetically protected

people.

 

But the experts said they thought the findings did not prove there

would be future waves of deaths among people who ate beef from

prion-infected cows in the 1980s.

 

" That's a provocative conclusion, but I'm not sure it's totally

plausible, " said Dr. David Westaway, a prion expert at the University

of Toronto.

 

Thus far, about 160 people, mostly in Britain, have died of variant

Creutzfeldt-Jakob disease, which humans get from cows that had bovine

spongiform encephalopathy, or mad cow disease.

 

The authors chronicled the deaths and genetic makeup of 11 aging

members of the Fore tribe who died from 1996 to 2004 of kuru, which can

lie dormant for years but then start a quick, irreversible descent into

dementia and death.

 

Kuru killed thousands of Fore beginning in the 1920s and only began to

fade after the Australian authorities in the 1950s outlawed the Fore

practice of honoring their dead by butchering them with bamboo knives

at mortuary feasts, eating them and - by some accounts - smearing

themselves with brain tissue, which would drive infection into cuts and

scratches.

 

Scientists studying kuru eventually realized it was transmitted when

misfolded prion proteins reached another brain and " taught " its

proteins to misfold and congeal into a spongy mass.

 

The new Lancet study relied on teams of local people who visited

mountain villages for years, interviewing and asking for blood samples

from anyone with symptoms. The 11 they found were all born between 1933

and 1949, and cannibalism died out by 1960.

 

The lead author, Dr. John Collinge of University College, London,

concluded that kuru could incubate as long as 56 years before killing.

 

It has been known for years that people with a certain genetic marker

die soonest of prion diseases. Nine of the 11 Fore did not have it.

 

For that reason, Collinge predicted more waves of deaths among people

who ate prion-infected beef decades ago, because most of the world also

lacks that marker.

 

But several experts, including Westaway, said in interviews that they

disagreed there would be a great many future cases. Kuru is a human

disease and is better at infecting people than mad cow disease is, they

argued, and Fore cannibals came into contact with brain matter, while

most people eat meat, which has low concentrations of prions.

 

NEW YORK The long lives that some former cannibals enjoy before

succumbing to a brain-wasting disease suggest that many more humans

will eventually die of mad cow disease, according to a study released

Friday.

 

But several experts in such illnesses, called prion diseases - which

are blamed for killing New Guinea cannibals and British eaters of

infected beef - disagreed with that frightening implication of the

study, which was published Friday in The Lancet, the British medical

journal.

 

These experts praised the rigorous work the authors of the report did

to confirm that kuru, a disease that once decimated highland tribes in

New Guinea, can incubate for 50 years in a few genetically protected

people.

 

But the experts said they thought the findings did not prove there

would be future waves of deaths among people who ate beef from

prion-infected cows in the 1980s.

 

" That's a provocative conclusion, but I'm not sure it's totally

plausible, " said Dr. David Westaway, a prion expert at the University

of Toronto.

 

Thus far, about 160 people, mostly in Britain, have died of variant

Creutzfeldt-Jakob disease, which humans get from cows that had bovine

spongiform encephalopathy, or mad cow disease.

 

The authors chronicled the deaths and genetic makeup of 11 aging

members of the Fore tribe who died from 1996 to 2004 of kuru, which can

lie dormant for years but then start a quick, irreversible descent into

dementia and death.

 

Kuru killed thousands of Fore beginning in the 1920s and only began to

fade after the Australian authorities in the 1950s outlawed the Fore

practice of honoring their dead by butchering them with bamboo knives

at mortuary feasts, eating them and - by some accounts - smearing

themselves with brain tissue, which would drive infection into cuts and

scratches.

 

Scientists studying kuru eventually realized it was transmitted when

misfolded prion proteins reached another brain and " taught " its

proteins to misfold and congeal into a spongy mass.

 

The new Lancet study relied on teams of local people who visited

mountain villages for years, interviewing and asking for blood samples

from anyone with symptoms. The 11 they found were all born between 1933

and 1949, and cannibalism died out by 1960.

 

The lead author, Dr. John Collinge of University College, London,

concluded that kuru could incubate as long as 56 years before killing.

 

It has been known for years that people with a certain genetic marker

die soonest of prion diseases. Nine of the 11 Fore did not have it.

 

For that reason, Collinge predicted more waves of deaths among people

who ate prion-infected beef decades ago, because most of the world also

lacks that marker.

 

But several experts, including Westaway, said in interviews that they

disagreed there would be a great many future cases. Kuru is a human

disease and is better at infecting people than mad cow disease is, they

argued, and Fore cannibals came into contact with brain matter, while

most people eat meat, which has low concentrations of prions.

 

 

 

Defending this corruption on which you are sat

You tell me what to think, you tell me this and that

`Freedom is O.K. you scum` but make sure it`s never used

In your defence of liberty I always stand accused

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