Mad Cow - CJD Spread
"Far Wider Than Ever Imagined"?

SYDNEY ( - From cannibals in the highlands of Papua New Guinea to European beef eaters, thousands of people have been exposed to an undetectable disease that could slowly eat holes in their brains before killing them.
A new book and a British inquiry have set out to find the true extent of a disease once regarded as a tragic pathological oddity but which has killed thousands around the world.
Creutzfeldt-Jakob Disease (CJD) is the human equivalent of mad cow disease and, like its bovine equivalent, it kills relentlessly and is largely undetectable until after death.
The book "Cannibals, Cows and the CJD Catastrophe" tells a story its author says begins and ends in mortuaries and suggests the disease might be spread far wider than was ever imagined.
"Speculation on how many people will succumb to the disease from a variety of accidental means of transmission is fairly useless at this stage," Jennifer Cooke, author of "Cannibals, Cows and the CJD Catastrophe," told Reuters.
"It will be a long waiting game because CJD can incubate without displaying symptoms for more than four decades."
A British inquiry that started last week was set up after scientists in 1996 linked mad cow disease -- bovine spongiform encephalopathy or BSE -- to a new variant of CJD (nvCJD).
So far 24 nvCJD cases have been confirmed in Britain and France but the long incubation period makes it impossible to predict how many more will be affected.
"The whole population of the United Kingdom and a good part of Europe have been exposed," Professor Colin Masters, head of Australia's CJD Registry, told Reuters. "We know that the material has been widely spread in the human food chain."
From makeshift morgue to British beef eaters
CJD is a debilitating brain-wasting disease which Masters describes as similar to Alzheimer's.
The biggest known outbreak of the disease was among a tribe practising cannibalism in the remote highlands of Papua New Guinea in the 1950s.
Cooke's book opens with crusading U.S. doctor Carleton Gajdusek removing the brain of a CJD victim with a carving knife and handsaw in a storm-tossed makeshift morgue in PNG and goes on to detail all known forms of the disease.
About 3,000 people from the Fore tribe in PNG have died from the disease they call "kuru," the local word for shivering, contracted after eating dead relatives as a mourning rite.
"At the peak of the epidemic in the 1950s it was the major cause of death in (Fore) women and children," Masters said.
CJD and mad cow disease are the two best known types of transmissible spongiform encephalopathies (TSEs).
All forms of TSEs have three common threads -- the agent that activates them is unknown, they all bore microscopic holes in the brain of their victim, and they are always fatal.
CJD was first identified by German neuropsychiatrists Hans Creutzfeldt and Alfons Jakobs in the 1920s. Current theories suggest it is caused by a virus or virus-like agent or a corrupted natural protein known as a prion.
The agent is not only infectious but has also been shown to be inheritable in a small number of cases. It has no cure and no treatment, resists heating to high temperatures, freezing, burial underground, strong chemicals and medical sterilisation.
Diseases linked to cannibalism
Most TSEs also involve either direct cannibalism or the re-use of body parts in agriculture or medicine.
Gajdusek's work with the Fore proved the link to direct cannibalism and won him the 1976 Nobel prize for medicine.
Feeding meal containing TSE-contaminated sheep and cattle offal to cows has been blamed for the development of mad cow disease. Scrapie in sheep is a related disease.
In 1996, the BSE-nvCJD prompted widespread bans on British beef exports, only some of which have been eased.
Links from the medical cannibalisation of body parts have also been established.
More than 100 people in the United States, Britain, France, Brazil, Australia and New Zealand died from CJD after receiving human growth hormone (hGH) injections as children before artificial hGH became available in 1986.
In Australia, four women died after thousands were treated with an injectable fertility drug taken from pituitary glands removed from the brains of bodies in morgues.
British inquiry to examine links
There are concerns the disease might be transmitted through blood transfusions. Britain has ordered that blood plasma must be imported where possible after British donors were discovered to have CJD.
British microbiologist John Pattison, who established the BSE-nvCJD link, told a conference in Atlanta last week that he feared a new outbreak of nvCJD through blood transfusions.
A World Health Organisation (WHO) meeting in Geneva last month was told that the possibility of an epidemic of CJD over the next 10 to 15 years could not dismissed.
The complexity of the diseases is being addressed by the British inquiry, under former senior judge Sir Nicholas Phillips.
So complicated are the issues that the inquiry, originally set to last 12 months at a cost of about $16 million, has already been extended to 18 months.
"This is the first time in the world that a government, as a public health regulator for its population, has had to examine its own past actions," Cooke said.
By PAUL TAIT, Reuters

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