another perspective on CJD

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From:"Slap, Steven" <>
To:HistoNet <>
Content-Type:text/plain; charset="iso-8859-1"

Hi HistoNetters,

Some of you might find this article interesting...

> >=======================Electronic Edition========================
> >.                                                               .
> >.           RACHEL'S ENVIRONMENT & HEALTH WEEKLY #683           .
> >.                    ---January 20, 2000---                     .
> >.                          HEADLINES:                           .
> >.                  MAD COW DISEASE AND HUMANS                   .
> >.                          ==========                           .
> >.               Environmental Research Foundation               .
> >.              P.O. Box 5036, Annapolis, MD  21403              .
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> >.                          ==========                           .
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> >=================================================================
> >
> >
> >
> >When a new form of an old human disease appeared in England in
> >1995, some medical specialists immediately suspected that it
> >might be a human version of "mad cow disease," but they had no
> >proof.[1] Mad cow disease had appeared in British dairy cattle
> >for the first time in 1985 and during the subsequent decade
> >175,000 British cows had died from it. British health authorities
> >spent that decade reassuring the public that there was no danger
> >from eating the meat of infected cows. They said a "species
> >barrier" prevented mad cows from infecting humans. A "species
> >barrier" does prevent many diseases from crossing from one
> >species to another -- for example, measles and canine distemper
> >are closely related diseases, but dogs don't get measles and
> >humans don't get distemper.
> >
> >While the British government was placing its faith in the species
> >barrier, British citizens began to die of a new disease, called
> >"new variant Creutzfeld-Jakob disease" or nvCJD. A similar
> >disease, CJD (Creutzfeld-Jakob disease) had been recognized for a
> >long time but it almost never occurs in people younger than 30;
> >nvCJD, on the other hand, strikes people as young as 13. There
> >are several other differences between CJD and nvCJD, so nvCJD
> >represents something new. To date, nvCJD has killed 48 people in
> >England and one or two others elsewhere in Europe. The main
> >feature of both mad cow disease and nvCJD is the progressive
> >destruction of brain cells, inevitably leading to total
> >disability and death.
> >
> >New research published late in 1999 showed that nvCJD is, in
> >fact, a human form of mad cow disease,[2] dashing all hope that a
> >species barrier can protect humans from this deadly bovine
> >affliction.
> >
> >Mad cow disease is formally known as "bovine spongiform
> >encephalopathy" or BSE. BSE is the cow version of a larger class
> >of diseases called "transmissible spongiform encephalopathies,"
> >or TSEs. TSEs can afflict sheep, deer, elk, cows, mink, cats,
> >squirrels, monkeys, humans and other species. In all species the
> >symptoms of TSEs are the same -- progressive destruction of brain
> >cells leading to dementia and death.
> >
> >Traditional Creutzfeld-Jakob disease (CJD) is a rare human
> >affliction. The visible symptoms are similar to Alzheimer's
> >disease; in fact, CJD is sometimes diagnosed as Alzheimer's and
> >therefore may go unrecognized. CJD strikes one in a million
> >people almost all of whom are older than 55. In people younger
> >than age 30, CJD is extremely rare, striking an average of 5
> >people per billion each year, worldwide (not counting the recent
> >outbreak in England).
> >
> >In cows, the latency (or incubation) period for mad cow disease
> >is about 5 years, meaning that cows have the disease for five
> >years before symptoms begin to appear. No one knows the latency
> >period for nvCJD in humans, but it is thought to be around 10
> >years. Because of this uncertainty, no one is sure how many
> >people in England already have the disease but are not yet
> >showing symptoms. The British government's chief medical officer,
> >Professor Liam Donaldson, said December 21, 1999, "We're not
> >going to know for several years whether the size of the epidemic
> >will be a small one, in other words in the hundreds, or a very
> >large one, in the hundreds of thousands."
> >
> >The epidemic of mad cow disease was caused by an agricultural
> >innovation -- feeding dead cows to live cows. Cows are, by
> >nature, vegetarians. But modern agricultural techniques changed
> >that. Cows that died mysteriously were sent to rendering plants
> >where they were boiled down and ground up into the consistency of
> >brown sugar, and eventually added to cattle feed. It was later
> >determined that mad cow disease was being transmitted through
> >such feed, and especially through certain specific tissues --
> >brain, spinal cord, eyes, spleen and perhaps other nerve tissues.
> >
> >Ten new cases of nvCJD were reported in England in 1999, bringing
> >the total to 48. It has been more than 10 years since government
> >authorities banned the use of the particular parts of cows
> >thought to transmit mad cow disease. The appearance of new cases
> >of nvCJD in 1999 implies either that the latency period for the
> >disease is longer than 10 years, or that infected meat was not
> >effectively eliminated from the food chain when government
> >authorities said it was, or both.
> >
> >The SUNDAY TIMES of London reported in late December that some
> >meat banned for human consumption is still being marketed in
> >England. After the mad cow scandal erupted, the British
> >government attempted to eradicate the disease by requiring that
> >all cows older than 30 months be slaughtered. As a result, by
> >last September more than 2.5 million British cows had been
> >killed. But the TIMES reported that British investigators have
> >documented at least 50 cases of farmers and cattle dealers using
> >bogus identity documents to falsify the ages of cows in order to
> >sell them for human consumption. Furthermore, the Agriculture
> >Ministry acknowledged that as many as 90,000 cattle could not be
> >accounted for. About 1600 new cases of mad cow disease are still
> >being reported each year in England.
> >
> >In December, French health authorities announced finding a second
> >case of nvCJD, a 36-year-old woman in Paris. France has continued
> >to refuse to import British beef, even though the European Union
> >on August 1, 1999, formally declared British beef as safe as any
> >in the European Union. The European Union said in December it
> >will take France to the European Court of Justice to force it to
> >import British beef. Germany is also refusing to import British
> >beef.
> >
> >The U.S. government says mad cow disease has never been observed
> >in any U.S. cows. However, a closely-related TSE disease, called
> >chronic wasting disease (CWD), has been increasing for almost 20
> >years among wild deer and elk in northern Colorado and southern
> >Wyoming. Since 1981, CWD has been spreading slowly among wild
> >deer and elk herds in the Rocky Mountains and now afflicts
> >between 4% and 8% of 62,000 deer in the region between Fort
> >Collins, Colorado and Cheyenne, Wyoming.
> >
> >During 1999, CWD erupted among a herd of elk on the David Kesler
> >Game Farm near Philipsburg, Montana, which raised elk
> >commercially. A few of Mr. Kesler's elk had been shipped to
> >Oklahoma and Idaho, and perhaps elsewhere, and CWD was discovered
> >in some of those animals, too. In early December, Montana health
> >authorities slaughtered 81 elk on Mr. Kesler's farm. They
> >initially announced plans to incinerate the carcasses, but later
> >decided that incineration would be too expensive. The animals
> >were finally buried at the High Plains Sanitary Landfill north of
> >Great Falls. Equipment used to feed, water and care for the
> >animals was also buried in the landfill. Montana authorities
> >announced that the fenceline at the elk farm would be
> >decontaminated, but they did not say what procedure they would
> >use. Nor did they announce what would become of Mr. Kesler's
> >contaminated land. The disease agent that causes CWD -- a prion
> >protein -- is very hardy and resists destruction by traditional
> >sterilization techniques like alcohol and heat.
> >
> >The diseased elk carcasses in the High Plains landfill have been
> >buried under a mound of garbage but will still be accessible to
> >rainwater and perhaps to scavenging animals.
> >
> >In northeastern Colorado and southeastern Wyoming, state
> >officials are urging hunters to protect themselves when dressing
> >wild deer and elk they have shot. Hunters should wear rubber
> >gloves, minimize contact with brain and spinal cord tissues,
> >discard the brain, spinal cord, eyes, spleen and lymph nodes and
> >definitely not eat them. There is no evidence that CWD can cross
> >over from deer and elk to humans, but there was no firm evidence
> >that mad cow disease could afflict humans until 1999, so wildlife
> >officials in the Rocky Mountain states say caution is warranted.
> >
> >Writing in the BOSTON GLOBE, Terry J. Allen reported in late 1999
> >that, since 1996, Creutzfeld-Jakob disease has been identified in
> >3 Americans younger than age 30.[3] All three are known to have
> >hunted extensively or eaten venison. There is no evidence that
> >CWD disease has jumped from deer or elk to humans, but the
> >appearance of this extremely-rare disease in young people was the
> >first evidence of a problem in England, so health authorities in
> >the U.S. say they are aggressively investigating all the
> >possibilities.
> >
> >A statistician at the federal Centers for Disease Control (CDC)
> >in Atlanta told Terry Allen that, if one more case of CJD had
> >surfaced in a person younger than 30 in the U.S., it "might tip
> >the balance," meaning it might convince authorities that
> >something truly unusual was occurring. Dr. Michael Hansen of
> >Consumer's Union says, "Given how rare the disease is in young
> >people and how difficult it is to make a diagnosis, the
> >possibility that some cases go undetected cannot be ruled
> >out."[3]
> >
> >Indeed, of the 3 cases detected in the U.S. since 1996, one
> >nearly went undetected. Last year in Utah, Doug McEwan, 28, began
> >to show an array of mysterious symptoms: loss of memory, loss of
> >motor control, mood swings, and disorientation. His wife, Tracey,
> >says his doctors conducted hundreds of tests but could not
> >diagnose his disease. She happened to see a TV program on mad cow
> >disease and she insisted that Doug's doctors must test for CJD. A
> >brain biopsy confirmed the diagnosis.
> >
> >One of the three young CJD victims had eaten deer shot near
> >Rangely, Maine, so last November federal officials took samples
> >of brains from 299 deer shot in western Maine. Authorities said
> >at the time they were quite sure Maine deer are not harboring
> >CWD. So far, test results have not been released.
> >
> >Federal authorities have quarantined two herds of sheep in
> >Vermont because they say the sheep may have been given feed that
> >contained parts of animals afflicted by mad cow disease. The
> >sheep had been imported into Vermont from Belgium and the
> >Netherlands, where they may have been fed improperly. A similar
> >herd of sheep in New York state was recently purchased by the
> >federal government and slaughtered.[4]
> >
> >Meanwhile, a 68-year-old Indiana man with a fondness for
> >beef-brain sandwiches died of CJD last summer. Beef-brain
> >sandwiches are a local delicacy in Indiana, introduced years ago
> >by German immigrants. The EVANSVILLE (INDIANA) COURIER reported
> >that John Hiedingsfelder, a forensic pathologist in Evansville,
> >said he had seen three cases of CJD in the past year. No
> >connection to mad cow disease has been established in the Indiana
> >cases.  Roberta Heiman, a staff writer for the EVANSVILLE
> >(INDIANA) COURIER reportedly received a warning from a
> >cattleman's association not to publish any further articles about
> >this subject.
> >============
> >
> >[1] Unless a specific source is cited, information in this issue
> >of Rachel's was taken from, a web site maintained
> >by Thomas Pringle of Eugene, Oregon. Sources of information are
> >cited at
> >
> >[2] Michael R. Scott and others, "Compelling transgenic evidence
> >for transmission of bovine spongiform encephalopathy prions in
> >No. 26 (December 21, 1999), pgs. 15137-15142.
> >
> >[3] Terry J. Allen, "Rare, Animal-Borne Disease a Medical
> >Mystery; Officials Examine Maine Deer in Hunt for Clues," BOSTON
> >GLOBE December 12, 1999, pg. C26.
> >
> >[4] Matthew Taylor, "Mad Cow Fears, Anger on Farms; Two Imported
> >Sheep Herds Quarantined in Vermont," BOSTON GLOBE October 31,
> >1999, pg. F24.
> >
> >Descriptor terms: mad cow disease; england; france; montana;
> >wyoming; vermont; maine; deer; elk; bse; tse; central nervous
> >system disorders;
> >
> >################################################################
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> >################################################################

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