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From: TSS (216-119-136-143.ipset16.wt.net)
Subject: Mad Cow Disease: Are Americans at Risk?
Date: November 26, 2003 at 11:27 am PST

Mad Cow Disease:
Are Americans at Risk?

Mad Cow Disease belongs to a family of neurological disorders that eat away at the brain, turning it into a sponge-like mass. Known to scientists as bovine spongiform encephalopathy (BSE), the popular name Mad Cow Disease refers to the symptoms of infected cattle: “staggering, drooling, signs of fear, grinding of teeth, aggression toward other animals.” People have contracted Mad Cow Disease through eating the meat of infected animals. In humans, the affliction is known as Creutzfeldt-Jakob Disease (CJD). Symptoms of the Mad Cow-induced form of CJD include hallucinations, loss of memory, dementia, uncontrollable crying or screaming, and inability to speak or walk. These two diseases are always fatal, to humans as to cattle. There is no cure, treatment or vaccine.

Mad Cow on the Rampage: Mad Cow Disease was first detected in the mid-1980s in England, where it has killed over 180,000 cattle, devastated the British cattle industry, and ruined countless farmers. From Britain the epidemic spread to the rest of Europe, infecting over 4,200 cattle in 19 countries by mid-2003. Though apparently under control in Europe, the disease still kills 2-3 cattle each day. Because Mad Cow has jumped the species barrier, killing humans, European authorities have taken a precautionary approach to stop the epidemic, destroying over 5 million potentially infected cattle. Mad Cow is not confined to Europe; infected cattle have also appeared in Canada, Japan, Israel, Oman and the Falkland Islands.

Mad Cow Found in North America: The first North American case not attributable to import of a diseased cow from England was reported in Alberta, Canada in May 2003. Such native-born cases of Mad Cow Disease are alarming because they indicate that feeding practices in the country are to blame, and that other cattle likely have the disease. Given the huge trade in cattle and beef between Canada and the U.S., coupled with inadequate testing and controls in both countries (see below), it is very likely that the U.S. also harbors mad cows that just haven’t been detected.

How is the Disease Spread? Mad Cow might have remained a rare disease were it not for cattle cannibalism. Over the past few decades, it has become a common industrial agricultural practice to process the remains of dead cattle (as well as diseased animals, road kill, dead pets, zoo animals, etc.) into animal feeds that are fed to cattle. Since cattle can become infected by consuming less than 1 gram of diseased tissue , one diseased carcass can contaminate a large batch of animal feed, sickening hundreds of animals. These hundreds, rendered into animal feed in turn, can infect thousands. This is how experts explain the Mad Cow epidemic in Britain.

What is Rendering? Rendering processes the remaining body parts of cattle once all of the edible parts have been removed. Essentially, the brains, spinal cords, and other sections unfit for human consumption are broken down to create two final products: fat and meat-and-bone meal (MBM). The fat is used in a variety of goods such as soap, lipstick and glue, while MBM, with its powdery consistency and high concentration of protein, is often added to animal feed. Both fat and MBM contain varying amounts of the brain and spinal cord tissue that carries the highest risk of transmitting the disease. In the U.S., these products were legally fed to cattle until 1997; they are still fed to horses, pigs and poultry.

Mad Cow Disease in Humans: Over 150 people have contracted variant CJD (vCJD), the human disease most closely associated with Mad Cow, by consuming the meat of infected animals: 143 in the UK , 6 in France, 2 in Canada, and one each in Ireland, Italy and the U.S. vCJD tends to strike young people, is always fatal, and takes about 14 months to kill its victim. Classic or sporadic CJD is of unknown cause and strikes mainly the elderly. Recent evidence that BSE can cause sporadic CJD as well as vCJD may explain the rising numbers of CJD cases in Europe , and the disturbing trend to younger CJD cases in the U.S. Several autopsy studies suggest that 3 to 13 percent of patients diagnosed with Alzheimer's or dementia actually suffered from CJD. These findings imply that at least 120,000 CJD cases may be going undetected and excluded from official statistics. If even a small percentage of these misdiagnosed CJD cases are caused by eating BSE-infected meat, the incidence of human Mad Cow is much worse than anyone has imagined up to now. Yet the U.S. Centers for Disease Control still refuse to make CJD a reportable disease.

What Causes Mad Cow and CJD? Most scientists agree that Mad Cow, CJD and related diseases – including chronic wasting disease, which is spreading among the U.S. deer and elk population – are due to deformed proteins called prions. Prions somehow induce normal brain proteins to become deformed in the same way, causing brain degeneration. Prions are incredibly resistant to heat, chemicals, and even radiation. They cannot be inactivated with disinfection measures used to kill other disease-causing agents like bacteria and viruses.

Is Our Meat Supply Safe? Prions tend to accumulate in the brain, the spinal cord and other nervous system tissues, the eyes and intestine. Hence, a person who consumes meat that contains these tissues, if derived from an infected animal, could contract the human form of Mad Cow. Brain and spinal cord tissue contaminate meat in three major ways. First, powerful stun guns that shoot 4-inch bolts into a cow’s skull prior to slaughter can also drive brain tissue into the animal’s lungs and throughout its body , thus contaminating meat that ends up in the supermarket with potentially infective brain tissue. Secondly, T-bone steaks and other cuts that include vertebral bone may contain spinal cord tissue. Finally, meat salvaged from the carcass and vertebral column after the better cuts have been removed by knife often contains spinal and other nervous system tissue. A recent USDA study shows that a shocking 35% of meat samples obtained with the mechanical “advanced meat recovery” (AMR) system used by many slaughter houses are contaminated with unacceptable nervous tissue. AMR meat is commonly found in lower quality meats such as ground beef, sausages and hot dogs, and is served to children nationwide in the school lunch program. It is also found in meat used by fast food chains. About 45 million pounds of AMR meat are produced every year in the U.S.

Response of USDA & FDA to the Mad Cow Threat: Since 1989, the USDA and FDA have taken a number of measures in three areas to counter the Mad Cow threat:
1) Restrictions on the import of cattle and cattle products from the U.K. and other countries with BSE or at risk of BSE; 2) Testing cattle brains for BSE; and 3) A rule intended to prevent the feeding of cattle parts to cattle.

IMPORT RESTRICTIONS: The USDA and FDA have identified countries with BSE and those at risk of BSE and issued import restrictions on live ruminants, and on products containing ruminant-derived material, from those countries. Despite these efforts, the Government Accounting Office (GAO) concludes that “BSE-risk material may have entered the country before BSE emerged in exporting countries or through gaps in import controls.” For instance, over 50 cattle imported in the 1990s from Japan, which first discovered BSE in a native cow in 2001, have either entered the food/feed chain or remain unaccounted. Even more troubling, nearly 150 million pounds of beef, beef products and by-products (e.g. animal feed) were imported from countries with or at risk of BSE from 1980-2001. Import restrictions cannot be enforced without accurate information, yet U.S. Customs found in 1999 that the information provided for imports of live cattle, fresh and frozen beef and animal feed was inaccurate in 21-24% of cases. In 2000, FDA inspected only 1% of the 4 million food imports under its jurisdiction, and both it and the USDA are seriously understaffed.

ANIMAL TESTING: Government and the cattle industry assure us that U.S. cattle are BSE-free, based mainly on post-mortem tests conducted on cattle brains, the only sure means of detecting BSE. But the USDA has tested relatively few cattle brains since 1990 . Though numbers have increased in the past few years, even the 19,990 cattle tested in 2002 represent just 0.02% of the U.S. cattle population of 96 million (or 1 test for every 5,000 cattle). Still more troubling, USDA has tested less than 2% of high-risk “downer” cattle over the past decade. Downer cattle are animals that collapse, unable to rise, due to neurological disease, broken limbs or undetermined reasons. They number an estimated 195,000 to 1 million in the U.S. each year. The few downers that are tested by the USDA are all cattle brought to slaughter. Hardly any on-farm downers are tested, despite the USDA’s acknowledgement that this is the highest-risk population for BSE infection. Downers with undetected BSE could enter the food supply or be rendered for animal feed, transmitting the disease to humans and cattle. The 2001 discovery of BSE in an Austrian cow soon after more stringent testing was instituted suggests that the U.S. may well remain BSE-free only until it begins testing all high-risk downer cattle, as is done in Europe and Japan.

ANIMAL FEED LABEL AND RESTRICTION: In 1997, the FDA established a policy to prohibit the feeding of most ruminant proteins (from cattle, sheep, deer, etc.) to ruminants. Yet this rule has not stopped the risky practice of cattle cannibalism. For instance, calves are still being weaned on cattle blood as a substitute for milk, despite the finding that blood can transmit prion disease, and the implication of cattle blood in several European cases of Mad Cow . The remains of slaughtered cattle, including high-risk brain and spinal cord tissue, are still legally fed to horses, pigs and poultry in the form of meat and bone meal (MBM); the remains of these cattle-fed animals, which could be silent carriers of BSE, can then be rendered and fed back to cattle, an indirect pathway for infective cattle tissue to be recycled back to cattle. About one million tons of poultry litter – which contains not only excrement but also uneaten poultry feed that may be of ruminant origin – are fed to cattle each year, another indirect route for cattle to continue feeding on cattle. Pet food that contains ruminant MBM may also end up as cattle feed. Finally, the only barrier to illegally feeding ruminant meat-and-bone meal to cattle is a label: “Do not feed to cattle and other ruminants.” British authorities eventually banned the use of ruminant MBM altogether – even as fertilizer – because they found that British farmers continued to illegally feed it to cattle despite a warning label like that employed in the U.S.

FDA enforcement of its “feed ban” has been extremely poor. Significant flaws discovered in a 2001 GAO audit of the agency’s performance include: failure to even identify 1,200 or more feed-related firms that should be subject to the ban; failure to issue warning letters, let alone penalize, hundreds of firms found out of compliance (most commonly for not labeling feed properly as containing ruminant protein); and a seriously deficient database that cast doubt on the veracity of inspection records for those few inspections that have been conducted. Even the FDA’s methods for testing animal feeds are flawed, because they are based on the analysis of genetic material, bone, and protein, all of which are degraded or destroyed in the rendering process.

How does the U.S. compare to the E.U.?

United States European Union
Cattle testing 2% of high-risk downers tested; no required testing of other cattle brought to slaughter 100% of downers tested; 100% of all cattle over 24 months of age that are brought to slaughter
High-risk tissue in meat for human consumption Brain & spinal cord tissue in 35% of low-quality meats; spinal cord may not be removed from better cuts; stunning methods drive brain tissue into lungs and body Brain, spinal cord, eyes and tonsils from all cattle > 12 months of age prohibited from human food supply ; high-risk stunning methods banned
Animal feed restrictions Partial prohibition allows cattle tissue to be fed to chickens, pigs, horses and vice versa; and cattle blood to be fed to calves More restrictive feed bans generally prohibit the feeding of animal tissues, whether ruminant or not, to all animals raised for food
Use of cattle blood as feed for calves No prohibition; accepted industry practice Authorities strongly discourage the practice


How can we reduce the risk posed to our health and to farmers?
We propose that the FDA not only strengthen enforcement of the animal feed restrictions, but raise the safety standards to the levels adopted by the E.U. We propose that the USDA test all cattle that are slaughtered for consumption or that die on a farm. Until the safety regulations are strengthened and better enforced, we suggest that meat-eating consumers avoid high-risk beef products and only consume beef from organic, grass-fed cattle or beef alternatives.

Endnotes

Rampton and Stauber (1997). Mad Cow U.S.A. Could the Nightmare Happen Here?, Maine: Common Courage Press, p. 10.
Bren, L. “Trying to Keep ‘Mad Cow Disease’ Out of U.S. Herds,” FDA Consumer Magazine, March-April 2001.
Office International des Epizooties. For UK, see: http://www.oie.int/eng/info/en_esbru.htm; for rest of world, see: http://www.oie.int/eng/info/en_esbmonde.htm.
“Mad Cow Disease: Improvements in the Animal Feed Ban and Other Regulatory Areas Would Strengthen U.S. Prevention Efforts,” U.S. General Accounting Office Report GAO-02-183, January 25, 2002, p. 1 (henceforth, “GAO Report”).
MacKenzie, D., “BSE crosses the Atlantic,” New Scientist, May 31, 2003.
BSE Inquiry Report to the U.K. House of Commons, Oct. 2000, Volume 1: Findings and Conclusions, pp. xxii, 18.
Bren, 2001.
UK Department of Health. Monthly Creutzfeldt Jakob Disease Statistics, 10/6/2003. See http://www.doh.gov.uk/cjd/index.htm for latest monthly update.
UK Spongiform Encephalopathy Advisory Committee. Draft minutes of the 78th meeting held on June 24, 2003, No. 52. http://www.seac.gov.uk/minutes/draft78.pdf
Asante et al (2002). “BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein,” European Molecular Biology Organization Journal 21(23), pp. 6358-66.
Butler, D. (2002). “Prion data suggest BSE link to sporadic CJD,” Nature 420(6915), p. 450.
Mitchell, Steve, “CJD screening may miss thousands of cases,” United Press International, July 21, 2003.
Ibid
Garland et al (1996). “Brain emboli in the lungs of cattle after stunning,” Lancet 348(9027), p. 610.
Analysis of 2002 FSIS Bovine AMR Products Survey Results, Food Safety and Inspection Service,
United States Department of Agriculture, February 2003. http://www.fsis.usda.gov/OA/topics/AMRAnalysis.pdf
“Meat Derived from Advanced Meat Recovery, “American Meat Institute Fact Sheet, May 2003. See: http://www.meatami.com/content/presscenter/factsheets_Infokits/FactSheetAdvancedMeatRecovery.pdf
GAO Report (2001), pp. 11-20.
GAO Report (2001), p. 21.
“USDA Increases Testing for Mad Cow,” Associated Press, January 23, 2003.
Greger, M. (2003). “U.S. Violates Global Standards on Preventing Mad Cow Disease,” for the Organic Consumers Association, June 4, 2003. http://www.organicconsumers.org/madcow/greger060603.cfm.
“Current Thinking On Measures That Could Be Implemented To Minimize Human Exposure To Materials That Could Potentially Contain the Bovine Spongiform Encephalopathy Agent,” Food Safety and Inspection Service (FSIS), January 15, 2002, p. 9. http://www.fsis.usda.gov/oa/topics/bse_thinking.htm
Greger, M. (2003), op. cit.
“Substances prohibited from use in animal food or feed,” Federal Register, Vol. 62, No. 108, pp. 30935-30978.
Hunter et al (2002). “Transmission of prion diseases by blood transfusion,” Journal of General Virology, Vol. 83.
UK Spongiform Encephalopathy Advisory Committee. Minutes of the 74th meeting held on June 13, 2002. http://www.seac.gov.uk/minutes/final74.pdf.
GAO Report (2001), pp. 22-27.
GAO Report (2001), p. 7.
European Commission press release, “BSE: Commission toughens measures against Transmissible Spongiform Encepalopathies,” IP/01/827, Brussels, Belgium, June 12, 2001, http://europa.eu.int/rapid/start/cgi/guesten.ksh?p_action.gettxt=gt&doc=IP/01/827|0|AGED&lg=EN&display=
European Commission press release, IP/00/636, “Byrne states Commission intention to adopt key health protection measure to reduce BSE risk,” Brussels, Belgium, June 19, 2000, http://europa.eu.int/rapid/start/cgi/guesten.ksh?p_action.gettxt=gt&doc=IP/00/636|0|AGED&lg=EN&display=
“Current Thinking…” FSIS. Pg. 3.
http://europa.eu.int/comm/food/fs/bse/legislation_en.html#feed%20ban
Quigley, Dr. Jim, “Calf Note #49 – Red blood cell protein in calf milk replacers,” December 10, 1998, updated January 2, 2001, http://www.calfnotes.com/pdffiles/CN049.pdf
European Commission’s Health & Consumer Protection Scientific Steering Committee
“Opinion on: The implications of the recent papers on transmission of BSE by blood and transfusion in sheep,” September 12, 2002, http://europa.eu.int/comm/food/fs/sc/ssc/out280_en.pdf
=====================================

For Immediate Release
Oct.10, 2003

Contact:
Larry Bohlen, 202-783-7400, ext. 251 or 202-270-1547 cell
John Kinsman, 608-986-3815 or 608-260-0900 alt.

Mad Cow Prevention Rules Violated by 300 U.S. Companies
FDA Asked to Take Action to Protect Public Health and Farmers

Washington, D.C. – A total of 300 U.S. companies are in violation of federal regulations meant to prevent Mad Cow disease, also known as Bovine Spongiform Encephalopathy (BSE). The data comes from the Food and Drug Administration’s (FDA) own database of animal feed company inspection records updated this week for the first time in 17 months The number of companies violating the law is more than double the number listed by the FDA in April 2002.

The regulations are meant to prevent cattle and other ruminant parts from being fed to cattle and other ruminants, a form of animal cannibalization once commonly practiced and now considered most likely to spread Mad Cow disease. The disease is suspected of causing a deadly brain disorder in humans called Creutzfeldt-Jakob disease. Human symptoms, which may not appear for 20 years, include hallucinations, loss of memory, dementia, uncontrollable crying or screaming, and inability to speak or walk. There is no known cure for the disease which is always fatal.

“Just one bad apple in the barrel could put Americans at risk of getting Mad Cow disease,” said Brent Blackwelder, president of Friends of the Earth, the group that disclosed the increase in violators as well as numerous lapses in FDA data. “In addition to the risks posed to consumers, it would cost the U.S. cattle industry billions to recover, just like it cost the Canadian cattle industry earlier this year.”

As part of its prevention program, the FDA inspects feed companies in the United States to determine whether they are in compliance with federal Mad Cow prevention rules, which include keeping feed made with cattle parts separate from feed for cattle and labeling feed with the banned material. The company database is maintained by the FDA’s Center for Veterinary Medicine. A new version was posted at http://www.fda.gov/cvm/index/bse/RuminantFeedInspections.htm Oct. 7.

Of 300 firms in violation of FDA regulations, 173 handle or distribute prohibited materials. And 32 of these handle both prohibited materials and ruminant feed, making them the most likely firms to spread Mad Cow disease. Additionally, 1779 records out of 11,172 have no listing of any action taken by the FDA after it completed its own inspection.

“Given the terrible costs to Canadian farmers of just one mad cow, you would think that the FDA would have perfected their system by now. If cattle ranchers and dairy operators want to know where to buy safe feed, the information available from FDA is insufficient,” said John Kinsman, who owns dairy cattle and is president of the Wisconsin-based group Family Farm Defenders.

A bull in Canada was infected with Mad Cow in May 2003 and an eighth incidence of Mad Cow was announced in Japan this week. No reports have occurred in the United States, but only 9 percent of downer cattle (animals that cannot walk, exhibit symptoms of neurological disease, and/or that die or are killed for reasons other than routine slaughter) are tested in the United States compared to 100 percent in the European Union and Japan.

“As long as U.S. federal oversight is weaker than that in other nations and loopholes in Mad Cow prevention regulations exist, the safest way for individuals to avoid the disease is to eat organic, grass fed beef or know the farmer who produced it,” continued Kinsman.

-end-

For a listing of the 300 firms out of compliance and an assessment of the FDA database of mad cow prevention inspection records, see www.foe.org/factoryfarms.

The mission of Family Farm Defenders (FFD) is to create a farmer-controlled and consumer-oriented food and fiber system, based upon democratically controlled institutions that empower farmers to speak for and respect themselves in their quest for social and economic justice. FFD has worked to create opportunities for farmers to join together in new cooperative endeavors, form a mutual marketing agency, and forge alliances with consumers through providing high quality food products while returning a fair price to farmers.

Friends of the Earth is the world's largest environmental network with member groups in 70 countries. The mission of Friends of the Earth is to defend the environment and champion a healthy and just world...

http://www.foe.org/new/releases/1003madcow.html

http://www.foe.org/
====================

some additional comments...TSS

Gerald Wells: Report of the Visit to USA, April-May 1989

snip...

The general opinion of those present was that BSE, as an
overt disease phenomenon, _could exist in the USA, but if it did,
it was very rare. The need for improved and specific surveillance
methods to detect it as recognised...

snip...

It is clear that USDA have little information and _no_ regulatory
responsibility for rendering plants in the US...

snip...

3. Prof. A. Robertson gave a brief account of BSE. The US approach
was to accord it a _very low profile indeed_. Dr. A Thiermann showed
the picture in the ''Independent'' with cattle being incinerated and thought
this was a fanatical incident to be _avoided_ in the US _at all costs_...

snip...

http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

To be published in the Proceedings of the
Fourth International Scientific Congress in
Fur Animal Production. Toronto, Canada,
August 21-28, 1988

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

R.F. Marsh* and G.R. Hartsough

•Department of Veterinary Science, University of Wisconsin-Madison, Madison,
Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092

ABSTRACT
Epidemiologic investigation of a new incidence of
transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
suggests that the disease may have resulted from feeding infected
cattle to mink. This observation is supported by the transmission of
a TME-like disease to experimentally inoculated cattle, and by the
recent report of a new bovine spongiform encephalopathy in
England.

INTRODUCTION

Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough
and Burger who demonstrated that the disease was transmissible with a long incubation
period, and that affected mink had a spongiform encephalopathy similar to that found in
scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965).
Because of the similarity between TME and scrapie, and the subsequent finding that the
two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was
concluded that TME most likely resulted from feeding mink scrapie-infecied sheep.
The experimental transmission of sheep scrapie to mink (Hanson et al., 1971)
confirmed the close association of TME and scrapie, but at the same time provided
evidence that they may be different. Epidemiologic studies on previous incidences of
TME indicated that the incubation periods in field cases were between six months and
one year in length (Harxsough and Burger, 1965). Experimentally, scrapie could not be
transmitted to mink in less than one year.
To investigate the possibility that TME may be caused by a (particular strain of
scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie
agent, including their sheep or goat sources, were inoculated into a total of 61 mink.
Only one mink developed a progressive neurologic disease after an incubation period of
22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused
by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent
from an unidentified source.

OBSERVATIONS AND RESULTS

A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin
reported that many of his mink were "acting funny", and some had died. At this time, we
visited the farm and found that approximately 10% of all adult mink were showing
typical signs of TME: insidious onset characterized by subtle behavioral changes, loss of
normal habits of cleanliness, deposition of droppings throughout the pen rather than in a
single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over
their _backs like squirrels. These signs were followed by progressive deterioration of
neurologic function beginning with locomoior incoordination, long periods of somnolence
in which the affected mink would stand motionless with its head in the corner of the
cage, complete debilitation, and death. Over the next 8-10 weeks, approximately 40% of
all the adult mink on the farm died from TME.
Since previous incidences of TME were associated with common or shared feeding
practices, we obtained a careful history of feed ingredients used over the past 12-18
months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy
cattle and a few horses. Sheep had never been fed.

Experimental Transmission. The clinical diagnosis of TME was confirmed by
histopaihologic examination and by experimental transmission to mink after incubation
periods of four months. To investigate the possible involvement of cattle in this disease
cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally
with a brain suspension from affected mink. Each developed a fatal spongiform
encephalopathy after incubation periods of 18 and 19 months.

DISCUSSION
These findings suggest that TME may result from feeding mink infected cattle and
we have alerted bovine practitioners that there may exist an as yet unrecognized
scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). A new
bovine spongiform encephalopathy has recently been reported in England (Wells et al.,
1987), and investigators are presently studying its transmissibility and possible
relationship to scrapie. Because this new bovine disease in England is characterized by
behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be
confused with rabies in the United Stales and not be diagnosed. Presently, brains from
cattle in the United States which are suspected of rabies infection are only tested with
anti-rabies virus antibody and are not examined histopathologically for lesions of
spongiform encephalopathy.
We are presently pursuing additional studies to further examine the possible
involvement of cattle in the epidemiology of TME. One of these is the backpassage of
our experimental bovine encephalopathy to mink. Because (here are as yet no agent-
specific proteins or nucleic acids identified for these transmissible neuropathogens, one
means of distinguishing them is by animal passage and selection of the biotype which
grows best in a particular host. This procedure has been used to separate hamster-
adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The intracerebral
backpassage of the experimental bovine agent resulted in incubations of only four months
indicating no de-adaptation of the Stetsonville agent for mink after bovine passage.
Mink fed infected bovine brain remain normal after six months. It will be essential to
demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic
association is to be confirmed.

ACKNOWLEDGEMENTS
These studies were supported by the College of Agricultural and Life Sciences,
University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United
States Department of Agriculture. The authors also wish to acknowledge the help and
encouragement of Robert Hanson who died during the course of these investigations.

REFERENCES
Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and
natural transmission. J. Infec. Dis. 115:393-399.
Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson,
D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861.
Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and
clinical observations. 3. Infec. Dis. 115:387-392.
Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the
transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible
diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460.
Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle?
Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary
Medicine. University of Arizona, pp 20.
Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M.,
Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy
in cattle. Vet. Rec. 121:419-420.

MARSH

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

just what is sporadic CJD ???

It apparently arises from a one-in-a-million spontaneous conversion of a normal cellular protein (encoded by a gene on chromosome 20) into an abnormal 3-dimensional shape, or configuration.

WRONG, THIS HAS NEVER BEEN PROVEN !!!

85% OF ALL CJDs, ''SPORADIC'' DO NOT JUST HAPPEN WITHOUT
ROUTE AND SOURCE, THIS IS ONLY A MYTH. please read the data
below, if you are seeking the truth.

there are new findings that dispute this. why does the USA ignore these findings?

Asante/Collinge et al, that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;


http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm


Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States

Terry S. Singeltary Neurology Online, 27 Jan 2003 [Full text]

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States 26 March 2003

Next Post-Publication Peer Review Top Terry S. Singeltary,


Email Terry S. Singeltary:

flounder@wt.net


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


http://www.neurology.org/cgi/eletters/60/2/176#535

RE-re- Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

IN the reply from Dr. Maddox to Terry S. Singeltary Sr., he states;

snip...

If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication)...

snip...

http://www.neurology.org/cgi/eletters/60/2/176#535

THERE seems to be some difference of opinion;

Mouse model sheds new light on human prion disease

snip...

Professor John Collinge said “We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be – the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.

snip...

http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm

ALSO, Dr. Maddox states;

make routine mortality surveillance a useful surrogate for ongoing CJD surveillance...

THIS has proven not very useful in the U.K.;

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

DR. Maddox states here;

In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

HOWEVER in a recent article in the UPI out of Washington;

CJD screening may miss thousands of cases

By Steve Mitchell UPI Medical Correspondent Published 7/21/2003 3:00 PM

snip...

In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC's investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases.

snip...

http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r

ALSO in Philip Yams book 'The Pathological Protein';

''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''.... (CHAPTER 14)

http://www.thepathologicalprotein.com/

THERE has been a _documented_ case of nv/v CJD in a 74 year old, so the errors Schonberger speaks of (above) would be of significant importance, if one believes in the nv/v CJD 'only' theory.

NOW we have _documented_ cases of very young CJD victims in the USA continuing to appear in several different states.

HOW does Dr. Maddox explain this, and does he still believe that a National CJD surveillance program with a CJD questionnaire to every victims family is still not warranted?

WITH CWD and Scrapie running rampant in the USA, with BSE now _documented_ in North America, with the feeding of ruminant-to-ruminant animal protein still happening in the USA in 2003 even though there has been a partial voluntary ban on ruminant feeding since 8/4/97, with only 48,000 BSE/TSE tests done on USA cattle in some 14 years of surveillance, when in any given year there are 100 million cattle in the USA, with all this, i think refusing to make CJD/TSEs reportable Nationally in the USA is not ONLY a grave mistake, but in my opinion, should be looked at with great suspicion...

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, TEXAS USA 77518

http://www.vegsource.com/talk/madcow/messages/1072.html

NEW 'atypical' BSE/TSE in cattle in Japan/France/Italy and the NEW Nor98 Scrapie strain in sheep ? - TSS 11/17/03 (1)

http://www.vegsource.com/talk/madcow/messages/91394.html

Re: NEW 'atypical' BSE/TSE in cattle in Japan/France/Italy and the NEW Nor98 Scrapie strain in sheep ? - TSS 11/18/03 (0)

http://www.vegsource.com/talk/madcow/messages/91395.html

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt–Jakob Disease [FULL TEXT] - Terry S. Singeltary Sr. 11/06/03 (1)

http://www.vegsource.com/talk/madcow/messages/91334.html

Re: Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt–Jakob Disease [FULL TEXT] - Terry S. Singeltary Sr. 11/07/03 (0)

http://www.vegsource.com/talk/madcow/messages/91342.html

Tracking Spongiform Encephalopathies in North America (Lancet Infectious Disease Volume 3, Number 8 01 August 2003) - TSS 8/14/03 (0)

http://www.vegsource.com/talk/madcow/messages/951.html

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in
Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed
Comment Number: EC -10
Accepted - Volume 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html

PART 2

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html

REPORT OF THE MEETING OF THE BUREAU OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION -- (NSCS) non species coding system - TSS 11/22/03 (0)

http://www.vegsource.com/talk/madcow/messages/91429.html

O.I.E. WILL NOT CHANGE MAD COW GUIDELINES UNTIL MAY 2004 (at the earliest)

http://www.vegsource.com/talk/madcow/messages/91426.html


TSS




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