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MAD COW CONFIRMED ALABAMA

thank you mrj on correction of my spelling. never use the checker much and always not enough time..............


http://www.fsis.usda.gov/OFO/TSC/bse_information.htm


i too am very curious about any test, blood, bone, teeth, whatever to correctly determine the age of cattle. this BSe the USDA et al are saying is just not good enough ;

> But U.S. Department of Agriculture officials believe the cow was at least
> 10 years old


these are the same folks that said that Texas cow was negative for 7+ months too, until the Honorable Phyllis Fong of the OIG did the end around Johanns/Dehaven et al. Maybe she could get the age of this cow determined as well via Weybridge.................TSS
 
http://www.fsis.usda.gov/OPPDE/rdad/FSISNotices/5-04.htm


http://www.ianrpubs.unl.edu/epublic/live/nf593/build/nf593.pdf


TSS
 
I JUST Love IT,It keeps the bureaucrat's busy looking at records when the www.SCORINGAG.COM database would have ID ed everything within 3 seconds. My best sales point that it costs $0.55 cents. Link to the best working multi country traceback database in the world;
http://www.scoringag.com/Public/docs/disease_tracking_demonstration.pps
 
What good is a database with nothing in it? Scorning System?
I think we may be getting the cart before the horse. In order to track cattle we must first find a way to ID the cattle and also find a way to have the movements of ID'ed cattle to be tracked. Who is going to inter the data into a database? We would like to say the stockyard of sale barn but many cattle change hands by privet treaty. And what about all the farmers that say they ain't going to tag no cows.
 
flounder said:
thank you mrj on correction of my spelling. never use the checker much and always not enough time..............


http://www.fsis.usda.gov/OFO/TSC/bse_information.htm


i too am very curious about any test, blood, bone, teeth, whatever to correctly determine the age of cattle. this BSe the USDA et al are saying is just not good enough ;

> But U.S. Department of Agriculture officials believe the cow was at least
> 10 years old


these are the same folks that said that Texas cow was negative for 7+ months too, until the Honorable Phyllis Fong of the OIG did the end around Johanns/Dehaven et al. Maybe she could get the age of this cow determined as well via Weybridge.................TSS

TSS, you are welcome on the spelling, however I was definitely not certain that was not a word describing another method of age detection, thus my question to you.

Why does it seem strange or sinister to you that a cow texting positive, then inconclusive or negative would go into a research testing project (and I believe it is correct that results of such research are legitimately not considered useful for anything other than that particular project)?

It seems like political posturing by OIG to me, that declared it must be further tested. I believe it is accurate that the person doing the tests in England said it was a very difficult case to get a positive from that animal, and that it was NOT a bad reflection on the US methods that were used, considering that difficulty in getting a positive result.

MRJ
 
MRJ said:
flounder said:
thank you mrj on correction of my spelling. never use the checker much and always not enough time..............


http://www.fsis.usda.gov/OFO/TSC/bse_information.htm


i too am very curious about any test, blood, bone, teeth, whatever to correctly determine the age of cattle. this BSe the USDA et al are saying is just not good enough ;

> But U.S. Department of Agriculture officials believe the cow was at least
> 10 years old


these are the same folks that said that Texas cow was negative for 7+ months too, until the Honorable Phyllis Fong of the OIG did the end around Johanns/Dehaven et al. Maybe she could get the age of this cow determined as well via Weybridge.................TSS

TSS, you are welcome on the spelling, however I was definitely not certain that was not a word describing another method of age detection, thus my question to you.

Why does it seem strange or sinister to you that a cow texting positive, then inconclusive or negative would go into a research testing project (and I believe it is correct that results of such research are legitimately not considered useful for anything other than that particular project)?

It seems like political posturing by OIG to me, that declared it must be further tested. I believe it is accurate that the person doing the tests in England said it was a very difficult case to get a positive from that animal, and that it was NOT a bad reflection on the US methods that were used, considering that difficulty in getting a positive result.

MRJ

MRJ, enough with the conspiracy theories!
 
MRJ WROTE;


>>>Why does it seem strange or sinister to you that a cow texting positive, then inconclusive or negative would go into a research testing project (and I believe it is correct that results of such research are legitimately not considered useful for anything other than that particular project)? <<<



because it would have never ever gone into any research project if not for the honorable Phyllis Fong. in the original bse emergency response plan, there was suppose to be a 48 to 96 hour turn around from time of finding to confirmation, and hand carried to Weybridge, not 7+ months and a congressional 'end around' LATER.


2 things happened, and there is no in-between. usda et al have spouting off for years, well since 8/4/97, triple firewalls in place, feed ban working, no mad cows. ALL WRONG! then you had the token purina Gonzales give away, where fda said it was o.k. to feed 5.5 grams of tainted TSE product to cattle, and they will be O.K. WRONG AGAIN! and then you had the infamous stumbling and staggering Texas cow that went to render without any test what so ever. THEN you had the other Texas mad cow that sat on the shelf for 7+ months. that cow would have never been positive if not for the Honorable Phyllis Fong of the OIG. Dehaven and Johanns would have kept that mad cow on ice till hell froze over. and you think that protocol is o.k. ??? not to mention the 9,200 other cows in that fluke of a enhanced 2004 usda june cover-up. one of the top prion gods in the world, says ;


These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general. ........


CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ...
Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...
www.cdc.gov/ncidod/eid/vol7no1/brown.htm


CDC - Afterthoughts about Bovine Spongiform Encephalopathy and ...
Afterthoughts about Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease. Paul Brown Senior Investigator, National Institutes of Health, ...
www.cdc.gov/ncidod/eid/vol7no3_supp/brown.htm



http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf



like i said, no in-between, either usda et al triple fire walls, feed ban of 8/4/97, no mad cow rhetoric was just that i.e. cover-up, or they all fell out of the Stupid tree and hit every branch on the way down a long time ago. ...TSS
 
TSS: " like i said, no in-between, either usda et al triple fire walls, feed ban of 8/4/97, no mad cow rhetoric was just that i.e. cover-up, or they all fell out of the Stupid tree and hit every branch on the way down a long time ago. ...TSS":lol: :lol: :lol:

MRJ, if you don't start thinking about what is going on instead of doing your little cheerleading act all the time, you might get accused of climbing the stupid tree also.

flounder, Delay is out. Seems like the Indians did him in. I wonder what will happen when the cowboys have their turn.
 
http://www.usda.gov/documents/vs_bse_ihctestvar.pdf


MRJ: It seems like political posturing by OIG to me, that declared it must be further tested. I believe it is accurate that the person doing the tests in England said it was a very difficult case to get a positive from that animal, and that it was NOT a bad reflection on the US methods that were used, considering that difficulty in getting a positive result.

MRJ, The US knew the protocol and the unreliable results possible for IHC testing but insisted on using it even though better tests were available.

Your beloved Gary Weber even fought the use of Western Blot (Prionics) tests. Looks like a USDA-NCBA cover-up of positive animals in the US.

It was not hard to get a positive on the animal using the correct test. It's just that the USDA was insistant on using one that they could "Tweak" at will to get the results they wanted?

Just think a year or so ago back when some were touting the "GOLD STANDARD" BSE test as the ultimate. :roll: :roll:

Read the above link and tell us why they insisted on using the IHC when there are as many variables as there are involved in it.
 
MIKE WROTE;

==========================

MRJ, The US knew the protocol and the unreliable results possible for IHC testing but insisted on using it even though better tests were available.

Your beloved Gary Weber even fought the use of Western Blot (Prionics) tests. Looks like a USDA-NCBA cover-up of positive animals in the US.

It was not hard to get a positive on the animal using the correct test. It's just that the USDA was insistant on using one that they could "Tweak" at will to get the results they wanted?

Just think a year or so ago back when some were touting the "GOLD STANDARD" BSE test as the ultimate.

Read the above link and tell us why they insisted on using the IHC when there are as many variables as there are involved in it.

==================================


:shock: :shock: :shock:

now were getting somewhere, thanks mike.

now, let me take this about 60 + pages of gigabits further please, and don't get mad, i have been only posting short snips of late as to not upset some here with long postings, but this should be posted here in full. Dr. Detwiler, former top vet at the USDA and TSE aka mad cow expert tried to tell the bush administration this in more ways than one at a BSE ROUNTABLE event on Wednesday, December 11, 2003, in Denver, Colorado. shortly after this Dr. Detwiler was put out to pasture along with Dr. Miller and a few others that knew what was going on and spoke out about it. There is no url that i am aware of, this is pdf file i have. i think you all need to read this. if the moderator deletes it due to length of it i will understand. but maybe it will stay long enough for some interested to download. here goes ;




Completely Edited Version
PRION ROUNDTABLE

Dr. Max Thornsberry: The question was asked: What brought this roundtable about? I was fortunate enough to listen to Dr. Prusiner's lecture by satellite at Washington State University on prion-related diseases. I was fascinated by the information he presented and the research he had administered. A number of livestock producers in the West this fall have found deer and elk on their property that have tested positive for chronic wasting disease. During a joint telephone conversation representing South Dakota Stockgrower's Association, Montana Stockgrower's Association, Missouri Stockgrower's Association and others, the question was asked: Is chronic wasting disease infectious to cattle? Is it possible for cattle to get BSE (Mad Cow Disease) from deer and elk? From that series of questions came this meeting.
I'm going to go on the program first and go over Dr. Prusiner's lecture—the information I took down and thought was most pertinent. We want to have a basic understanding of what the human medical field thinks in terms of prion-relation diseases. Dr. Prusiner has won the Nobel Prize in Medicine for making the discovery that BSE is caused by an abnormal protein, which he calls prion Scrapie (PrPSc). For many, that's kind of a generic term for an aberrant prion. It may not mean it's the literal Scrapie prion, so he refers to all aberrant prions as Scrapie prions.
There are normal cellular prions, and Dr. Prusiner believes that sometimes, in some particular animals, the normal prion is converted into a prion Scrapie. That Scrapie prion is acquired at a certain level, and somehow stimulates the cell to start producing more of this abnormal prion. A conversion process takes place within the cell that converts normal cellular prions into abnormal Scrapie prions. The cell cannot clear these abnormal prions fast enough, resulting in the eventual death of the cell.
BSE is also called new variant Creutzfeldt-Jakob Disease (CJD) in human patients. Aberrant metabolism and the resultant accumulation of this BSE prion protein is what causes BSE or CJD in people.
The normal prion is soluble in a non-denaturing detergent, but prion pre-Scrapie is not soluble. This is a very tough piece of protein. Where the normal prion is readily digested by protease enzymes, the Scrapie prion is radically resistant to protease digestion.
Dr. Prusiner said BSE in humans is caused by consuming tainted beef. Apparently he is totally convinced of that. He also made a very poignant comment about a young woman, about nineteen, who has Creutzfeldt-Jakob Disease. They can find nothing in her history that would indicate she ever consumed beef. She was raised as a vegetarian. However, she was a consumer of gelatin in the form of Jello salads. He believes she picked that prion up through gelatin, and he's convinced that the normal processes that are used to denature protein and produce gelatin have no negative effect on the Scrapie prion and don't destroy the infective nature of this Scrapie prion.
He also says it's possible the normal prion is converted to the Scrapie prion. Something triggers the conversion process within the cell structure itself. It becomes, then, what he called, an inherited prion disease. There are some people who have, within their metabolism, the ability for this to occur. When it does occur, it somehow gets the cell oriented to producing more of the abnormal protein, and then it literally kills itself.
I'm sure you're familiar with a lot of these prion diseases, such as Kuru from cannibalism. Dr. Prusiner lists six ways for prion diseases to be transmitted from person to person:
1. Kuru—Ritualistic cannibalism of brain and nervous tissues.
2. Improperly sterilized brain electrodes (transfers the disease from person to person). He knows of no effective way to sterilize a brain probe.
3. Cornea transplants.
4. Use of human pituitary-gland proteins (growth hormone, HCG)
5. Dura mater graphs (surgery for spinal bifida)
6. Protein X—a factor defined by molecular genetic studies that binds the normal protein and facilitates prion pre-Scrapie formation
Great Britain has had one million cattle die of BSE and the average incubation time was five years. Dr. Prusiner has documented that brain extracts from cows with BSE will cause BSE-like disease if injected in the brain of cattle, sheep, mice, pigs and mink. It can occur in a little time as 200 to 300 days. The theory that this is a long-term incubation disease depends on the exposure. If the exposure is into the nervous system, then the incubation period appears to be much shorter.
There is no reliable specific test for prion diseases in a live animal that is known or available to the scientific community at this time.
Dr. Prusiner's theory is that BSE accumulated in Great Britain because of a major change in the manufacture of meat and bone meal. They went from a heat-type extraction to a chemical solvent extraction. When this change was made, they were able to solubalize and accumulate much more fat, and with that fat goes neural tissue. As they fed cattle to cattle and more cattle were slaughtered, it became an epidemic.
They are still seeing cases of BSE in those countries, but nothing like they were. Several new cases this past year have occurred in much younger animals that were not exposed to meat and bone meal and were born from animals not fed meat and bone meal. Where are these animals getting exposed to BSE prions? That is an unanswered question at this point.
Twenty-five percent of mice were able to develop CJD in 60 days when brain tissue from young people with new variant CJD was injected into the brains of susceptible mice. These prions are considered to be extremely infective. Dr. Prusiner believes new variant CJD is an infectious disease. If we get these prions into an environment where they can be consumed or come in contact with people, they are extremely infectious. His theory is that humans get new variant CJD from consuming BSE prions from cows.
He also made some comments about sheep and Scrapie. He said sheep with Scrapie may have a few BSE prions, but when the rendering process takes place, there is a transformation from the Scrapie prion or those prions die off or are inactivated. The only ones that survive are the BSE prions. Dr. Prusiner's theory is that when they were using sheep for meat and bone meal, the Scrapie prion was inactivated, but the BSE prion was much more resistant to inactivation, so in the processing of sheep for meat and bone meal, they actually concentrated the prion that he believes causes BSE.
He also developed a few therapeutic approaches to treating people with BSE. They have discovered these therapies through cell cultures. They've found that thorazine, chlorpromazine, and acepromazine have some effect in slowing down the progression of the disease. But the most interesting discovery was with a synthetic quinine product, Quinacrine. In cell culture, they have been able, literally, to slow the progression of prion diseases. They've had some clinical improvement in CJD patients. They have to use it at such high levels, a toxicity problem occurs.
In a minority of patients where the disease progression is slowed, some patients actually have a measured decrease in PRP Scrapie levels.
He classified prion diseases into three classes: Infectious (BSE, chronic wasting disease), sporadic, and genetic. He believes there are prion-related diseases such as ALS and Parkinson's disease.
Dr. Prusiner commented a number of times that prions are infectious. This is not an end-stage disease. He talked about the potential for spreading it through after-birth and the licking of newborn calves and possibly decomposing carcasses.
Prions contain protein. They are miss-formed proteins and they have no RNA or DNA. Somehow they are able to get a cell to reproduce themselves without the presence of genetic material. Prions also do not trigger the immune system, so there is no way to vaccinate against this disease, in his opinion. PRP Scrapie prions are natural at low levels in human patients, but the cells are able to clear that abnormal protein in a normal situation.
Dr. Prusiner said we don't know the function of a normal PRP Scrapie prion in cells, but he believes there is a normal level of abnormal prions in cellular tissue of human patients. We don't know what that level is or when it reaches the point where it kills the cell. He believes there is some PRP Scrapie being formed all the time from normal prions, but the cell has a mechanism to clear the abnormal prion before it ever accumulates to a dangerous level.
The question was asked following the lecture: "How did BSE start in cows?" Dr. Prusiner believes in two theories. One is the sheep Scrapie theory mentioned previously. He does not believe the BSE prion is the sheep Scrapie prion. He believes they are two distinct prions, and the sheep Scrapie prion is much easier to inactivate than the BSE prion.
He also believes in a theory of spontaneous BSE or sporadic BSE. I thought Pasteur disproved spontaneous generation a long time ago, but evidently it's come back in vogue because Dr. Prusiner believes, and stands by the hypothesis that, for some reason, a cow just starts producing these abnormal BSE prions. He believes that cow died and ended up in the meat and bone meal and then the process started. That's the theory he outlines as his most significant hypothesis.

Dr. Spraker: If all people and animals always have a small amount of this abnormal prion, then why would you need a theory of spontaneous disease? Why not say something allows the abnormal to accumulate?

Dr. Thornsberry: Dr. Prusiner made that comment. He listed several human prion-related diseases and what the percentage was in the population. He feels that some of the prion-related diseases are genetic and some are spontaneous in a certain percentage of human patients. That was his hypothesis. I was surprised at the idea of spontaneous generation being brought up. That was the big theory before Pasteur came along—that maggots spontaneously came out of a piece of rotting meat. But that was exactly what he said: Spontaneous or sporadic BSE caused this disease to start to England

Dr. Bartz: I think of it as a stochastic process in humans. Sporadic CJD, to me, is real. You get sporadic production of an infectious agent. The way I look at it, the reason you don't see it in cattle or in sheep is a time factor. People come down with the clinical forms of sporadic CJD in their fifties and sixties. Cattle and sheep don't live that long. The theory is that as PrPC is being produced in the cell, it mis-folds into the Sc conformation randomly. If you're unlucky enough that this happens to you at an early stage of your life, it can progress and cause clinical signs.

Dr. Thornsberry: He believes that that occurs in everybody a little bit and then for some reason some individuals are not able to clear that abnormal prion?

Dr. Bartz: That I'm not sure about. I think sporadic CJD and familial CJD—something with mutations in the prion protein of the host—you increase the probability of this miss-folding.

Dr. Thornsberry: Here's another question—How does a prion get from the intestinal tract to the brain? Dr. Prusiner says it takes one billion or more prions orally to cause BSE to multiply in the lymphoid cells. From there, it goes to the brain. It may travel up the nerves, the spinal cord, or by the blood, the theory being that its primary means of getting to the brain is by traveling through spinal tissue.
The question was asked "How do you disinfect medical instruments?" His answer to that question was "Throw them away." He has found no effective way to disinfect them from prions.
There is no reliable blood test for prion-related diseases at this time. Conversion of PRP normal to PRP Scrapie occurs on the cell membrane with the relationship to cholesterol-rich domains on the cell membrane. This was the comment he made about natural conversion taking place.
"How do they multiply?" New prion pre-normal proteins are being formed and degraded into prion pre-Scrapie. As the Scrapie increases, more of it is somehow stimulated to be degraded into the Scrapie prion. In a situation where the Scrapie prion is there, as normal prion is being converted into abnormal prion, then the abnormal prion somehow triggers more abnormal prion to be converted until it builds up enough that it kills the cell.
Dr. Prusiner made some comments about Parkinson's disease. He said a protein being improperly handled in the substantia nigra cells is what causes Parkinson's disease because it causes a decrease in dopamine production. His theory is that Parkinson's disease is a prion-related problem, that Parkinson's disease is the direct result of those cells being killed because of prion accumulation that he believes is a familial transfer.
"Can it be transmitted by blood transfusions?" His answer is that We don't know. We believe that ALS, or Lou Gehrig's disease, is a prion-related disease.
The last question that was asked of Dr. Prusiner was a simple question. The person said, "Dr. Prusiner, do you eat meat?" He answered immediately "Not in Europe." He was asked to elaborate, and he said these prion-related diseases are all over Europe. He said wherever you go, if you eat meat, you have the potential of picking them up. He believes the prions are in the muscle tissue. He does not believe they're just in the spinal column.
My son is an M.D. and they are getting a lot of information on these prion-related diseases and most of it is coming from Dr. Prusiner. The medical community has an understanding about prion-related diseases that's much different from that of the veterinary community, from what I can determine

At this point, I'd like each of you to introduce yourself and give a short summary of what you do.


Dr. Linda Detwiler: I formally worked for the USDA in the area of Transmissible Spongioform Encepthalopathy diseases. I work currently as an independent consultant to the food service industry, particularly, McDonalds Corporation, ….rest of comments inaudible…….

Dr. Susan Keller: After graduating from veterinary school, I had practiced for ~10 years as a predominately large animal veterinarian in North Dakota before becoming the Deputy State Veterinarian with the North Dakota Board of Animal Health and the North Dakota Department of Agriculture in 1997. Current responsibilities include serving as the Designated Scrapie Epidemiologist for ND and have been certified at Plumb Island as a Foreign Animal Disease Diagnostician.

Dr. Jason Bartz: I received a PhD from the University of Wisconsin in 1998 and I worked with Drs. Judd Akin and Dick Marsh. I studied mechanisms of inter-species transmission of prion diseases. I was a post-doctoral fellow with Richard Besson. I studied pathogenesis, asking questions about how the agent gets from the periphery to the central nervous system. In September, I started a faculty position at Creighton University and I'm setting up a lab to continue study of prion pathogenesis.

Dr. Terry Spraker: I graduated from vet school in 1972. I have a PhD in pathology and I'm board-certified in veterinary pathology. I saw CWD as a graduate student and didn't know what it was at that time. I've been working with it since then, looking at pathogenesis of the disease.

Dr. Thornsberry: I feel honored to have you all here and I hope we'll have an interesting dialogue. We want to make this information available to the cattle industry and to veterinarians, so anything you think is pertinent, please tell us.


TRANSMISSION OF BSE

Dr. Jason Bartz: Dr. Thornsberry asked me to talk about what is the risk of transmission of chronic wasting disease in cattle? Unfortunately, I cannot directly answer that. What I'm going to talk about is some of the basis science behind it and what we know about interspecies transmission. You can have five factors affecting interspecies transmission in the natural setting. The first is the question "is infectivity shed into the environment?" Can PrPSc survive in the environment? Can PrPSc reach a new host species? Is the agent transferred between deer and cattle? I'm going to spend most of the time on these last two points: "how does the agent get from the periphery to the central nervous system where it causes disease?" Finally, I'll talk about the species barrier effect. "Can PRPSC from one species convert to the PRPSC from the host into a new PRPSC molecule?"
What are prion diseases? All prion diseases are transmissible. They're neurodegenerative diseases and they have long incubation periods of months to decades. They're characterized by the spongiform degeneration. Clinically, they have motor and/or behavioral/cognitive deficits. There's no effective therapeutic treatment. They're all inevitably fatal. There's no adaptive immune response and there are no antibodies produced.
The animal prion diseases include Scrapie, found in sheep and goats. It's been known in the United Kingdom for over 200 years. It was first identified in the US in 1947. It has a worldwide occurrence with the exception of New Zealand and Australia
Transmissible mink encephalopathy is a prion disease of ranch-raised mink. It was first identified in the 1940s. There have been three major occurrences in the United States. The latest outbreak was in Wisconsin in 1985. It's a rare disease.
Bovine spongiform encephalopathy was first identified in the UK in 1986. Clinically it's characterized by an aggressive behavior and it's named mad cow disease. It was appreciated that it was a food-borne disease, so in 1988, a bovine feed ban was instituted. Amidst concerns about the transmission of BSE to humans, a specified risk materials ban was put in place in 1990. This banned tissues such as central nervous system tissues and spleen, which harbor high level of infectivity in cattle, from going into the human food chain. In 1996, a variant Creutzfeldt-Jakob disease was identified. It's distinct from the classical forms of CJD. First of all, the age of onset of variant CJD is much younger. The first few cases of variant CJD were people in their teens and twenties. Normally this disease is in people in their fifties and sixties.
The neuropathology is very distinct in variant CJD compared to other forms of CJD. Stains reveal distinctive amyloid plaques that are not observed in other forms of CJD. In the original study that identified variant CJD, an epidemiological study was performed and the only common risk factor that was identified was the emergence of BSE in the UK at that time. This suggested that BSE was able to infect people, resulting in variant CJD.
Two studies support this hypothesis. The first is experimental transmission of BSE to macaques. The neuropathology of BSE in infected macaques is indistinguishable from that of variant CJD. The second series of experiments uses a technique called lesion profile. Lesion profiling is a technique where you can distinguish distinct prion strains by passage in mice, by quantifying their neuropathological features. In this series of experiments, mice were infected with either BSE, variant CJD, or sporadic CJD. The mice became clinically ill. Their neuropathological features were quantified and represented as lesion profiles. Importantly, the lesion profile of mice infected with BSE is the same as that of mice infected with variant CJD and it's distinct from that of mice infected with sporadic CJD. This indicates that the same prion strain that causes BSE also causes variant CJD and is consistent with the hypothesis that BSE was able to infect humans, resulting in variant CJD.
I'd like to speak a little bit about chronic wasting disease. It was first identified in 1967 in Ft. Collins, Colorado. It has an expanding geographical range. It's been found in several states in captive herds and has also been found in wild deer and elk. The clinical signs include excessive salivation, reduced fear of humans, and distribution of infectivity in the host. It seems to differ in deer infected with chronic wasting disease, PRPSC seems more predominant in the secondary lymphoreticular tissues compared to elk.
There have been quite a few experimental transmission studies of CWD. It's been successfully transmitted by the IC route in goats, mink, ferrets, cattle, and squirrel monkeys.

Physical Properties and Etiological Agents
This agent is highly resistant to UV radiation. UV radiation historically is used to inactivate agents, and a mechanism it uses is to destroy the nucleic acid of the infectious agent. However, prion diseases are very resistant to UV inactivation. One of the practical aspects of this is that for persistence of an agent in an environment, sunlight is a very good means of handling it by destroying nucleic acid genomes.
The prion agent is very stable in acid and alkali. Little infectivity is lost between ranges of pH 2.5 and 10. However, you can lose infectivity in protein denaturance or chemotropic agents such a phenol or high concentrations of urea. RNAase and DNAase do not have any elicitable effect on infectivity.
Treatments that destroy protein and nucleic acids do not destroy infectivity. This has ramifications in what the etiological agent really is.
Another physical property is that it can survive in the environment. This is a paper by Brown in 1991 where he buried the prion in flower pots in his garden for three years. When he dug up the flower pots, there was still infectivity detected in the soil but not in the sub-soil. The two points we want from this paper are that (1) the agent can survive in the soil for three years and (2) based on this study, it does not seem to be leaching out.
In another paper from Brown in 2000, he took either fresh brain or formalin fixed brain and treated it at different temperatures ranging from 150 degrees Celsius to 1000 degrees Celsius. When he treated brain material at 600 degrees Celsius, the brain material actually caught on fire and all that was left was black ash. When he took this material and inoculated it back into recipient animals by the IC route, these animals came down with disease, indicating how resistant this agent is to different environmental and physical treatments.


What Do We Know About the Agent?

When you enrich the amyloid deposits or amyloid plaques that occur in the CNS of prion-infected animals and examine them by electronmicroscopy, you see these plaques are comprised of linear non-branching amyloid fibrils. When you do biochemical analysis of this amyloid fibrils, the main component of the amyloid fibrils is the prion protein. When you enrich the plaques from the brain and the fibrils from the plaques and the protein from the fibrils, you enrich for infectivity. Subsequent studies have shown that the prion protein is encoded by the host, and this is important because it eliminates the possibility that the prion protein is a viral by-product.
This suggests the prion agent is comprised solely of protein. The idea for a self-replicating protein is not new. It was first proposed in 1967 by a mathematician name Griffith. In 1982, following the identification of the prion protein, Stan Prusiner developed the prion hypothesis that states that the etiological agent for these diseases is comprised solely of the prion protein.
The prion protein exists in two forms. It's encoded by the host. The PRPC is for cellular and the PRPSC is for Scrapie. PRPC has a high ________ content. It's sensitive to proteinase digestion. It's soluble in detergents. It's not associated with infectivity. The function of PRPC right now is not known.
PRPSC is post-translationally-derived from PRPC. It has a high beta sheet content and this is consistent with PRPSC being an amyloid protein. It has a core that is highly resistant to protease digestion. PRPSC is a soluble in detergent and it is thought to be the sole component of the infectious agent, although I should point out this has not been formally proven.
How does a protein replicate? I think replication is a poor word for what prions do. "Conversion" is a much better descriptive word. One mechanism that's been proposed is nucleation dependent polymerization where in the infectious form of the disease, where the PRPSC of aggregate is being inoculated into the host, it encounters a PRPC molecule from the host, and it is incorporated into the growing amyloid fibril. Based on this model, PRPC should be very limiting in this conversion step. This has been borne out by transmission studies wherein normal mice, if you IC inoculate, that incubation period is about 130 days. If you take out PRPC, this is in transgenic mice that do not express PRPC, these are resistant to infection. Then if you make transgenic animals that over-express PRPC, these animals have a much shorter incubation period.
This is a paper showing that RNA is enhancing PRPC to PRPSC. What we know where the conversion occurs in the cell, there's not a good likelihood of RNA being located there. I don't know if RNA has specific properties that aid in conversion that another molecule in the cell may have.

Strain Diversity in Prions
Historically, prion strains have been characterized by differences in incubation period, and most importantly, differences in neuropathological features in the central nervous system. Sometimes they even have different clinical signs. One of the main arguments against the prion hypothesis has been "how do you have strain diversity in the absence of the nucleic acid genome, since, according to biological dogma, the differences in the genome of an infectious agent result in strain differences? It's recently becoming increasingly clear, that the conformation of PRPSC can encode for strain diversity. Different strains have different properties such as susceptibility to protease digestion, migration, polypeptide migration on western blot analysis. FTIR analysis has been used to demonstrate that the hyper and drowsy strains of TME have different ratios of alpha and beta sheet structure.
Investigators have also used a technique called glycoform ratio where they quantify the three different glycoforms of PRPSC and come up with a distinct ratio that correlates with strain properties as well. Based on these and many other studies, it seems that the conformation of PRPSC is actually the molecule that encodes for strain diversity in prion diseases. So here's a mechanism to try to explain how an agent in the absence of nucleic acid genome can have strain diversity.
To summarize the agent, there's no nucleic acid genome. It seems to be comprised solely of PRPSC. It's very resistant to physical degradation. The strains of prions are encoded by the conformation of PRPSC.

Pathogenesis
How does the agent get from the periphery to the central nervous system where is causes disease? I'll talk about oral exposure, since there's experimental and epidemiological evidence that this likely occurs in kuru through the practice of ritualistic cannibalism, and also in TME and BSE through contaminated foodstuffs. People have speculated it happens in variant CJD through the consumption of BSE-contaminated food, and also in chronic wasting disease and Scrapie in sheep and goats.
Infectivity, or PRPSC, is first detected in secondary lymphoreticular tissues, such as spleen and lymph nodes. Within spleen and lymph nodes, PRPSC accumulates in follicular dendritic cells. One the agent replicates in secondary LRS tissues, it's next detected in the central nervous system. There is rapidly replicates in the CNS. It's thought this replication kills neurons, leading to the onset of clinical signs and eventual death of the host.
In lymphoreticular system tissues, PRPSC is mainly detected in follicular dendritic cells, but also to a limited extent in tingible body macrophages. This and other studies have fairly clearly demonstrated that FDCs can replicate prions, and right now it's unclear if the tingible body macrophages are replicating agents or simply just phagocytosing PrPSc
Two pathways of neuro-invasion have been identified. One utilizes sympathetic internervation. This was an experiment performed by Kimberlin in 1989. This was following intra-gastric infection of rodents with prions. At selected time points, tissues were collected and inoculated back into recipient mice to assay for infectivity. The first place that infectivity was detected was in secondary LRS, tissues, such as Peyer's patches and lymph nodes. The first place in the central nervous system that infectivity was detected was in the thoracic spinal cord, and once it reached the thoracic spinal cord, it could travel caudally and cranially, eventually reaching the brain and killing the host. From this and numerous other studies, it seems that infectivity can spread via the enteric and sympathetic nervous system to the thoracic spinal cord and then eventually reach the brain.
A second route of neuro-invasion has been identified that utilizes para-sympathetic internervation. From experiments in hamsters, it seems that following oral infection, the agent can be transported directly to the brain stem via the vagal nerve to the DMNV.
To summarize these two routes of infection, once the agent gets of the GI tract, it can go up sympathetic internervation to the thoracic spinal cord and travel to the brain, or it can get into parasympathetic internervation and be directly transmitted to the brain stem.
This part of the pathway has been fairly well worked out. So what's happening in the gut to get it into the nerve? This is not so well worked out. One study looks at transporting it across the gut lumen into the host. It seems that M cells are able to transport PRPSC across the lumen. Once it gets across the lumen, it's thought it associated with follicular dendritic cells, replicates, and then can neuro-invade.
So how does the agent get from the follicular dendritic cell into the nerve? That's another black box. Recently there's been a paper where they use transgenic animals, and in these transgenic animals, the distance between the follicular dendritic cells and the sympathetic internervation has been reduced. When you reduce the distance between the nerves and the FDCs, you reduce the incubation period. This suggests there's a direct transfer between infectivity from FDCs and to the nerves.
Some of the work I've been involved in is figuring out alternative routes following oral exposure. Once the agent gets into the gut, it neuro-invades via sympathetic or parasympathetic internervation. I was interested in alternative routes along the alimentary tract. A tissue that is highly internervated and also has associated LRS tissue is the tongue. But as you know, the tongue has an epithelial layer that prevents infectious agents from crossing it. The epithelial lay can become damaged. Infections of the oral cavity can damage the epithelial layer. Microbial agents such as thrush, which are common in infants but also can occur in many compromised adults can degrade the epithelial layer of the oral cavity.
More common than tongue infections is probably lesions in the oral cavity. In animals, especially ruminant species, cuts in the oral cavity are quite common. We wanted to test the hypothesis that if the agent can get in the tongue, it can cause disease. To do that, we inoculated hamsters in the lingual muscles of the tongue with a hamster-adaptive strain of TME called hyper-TME. We found that it can cause disease and the incubation period is 79 days. To put this into context, if you inoculate animals directly into the brain, the incubation period is 59 days. If you inoculate in the peripheral nervous system, in the sciatic nerve, it's slightly longer—68 days. When you go extra-neurally, the incubation period is much longer. Intraperitoneal is 101 days; IV is 118; IM is 142. When you take the same dose of agent and dry it on a food pellet and feed it to an animal, this is a long incubation period and very inefficient. Only one out of six animals comes down with clinical signs.
A short incubation period does not necessarily mean an efficient establishment of infection. To test for efficiency, we did the dose-response curve. In this experiment, we took 10-fold serial-dilutions of agent and inoculated them into the lingual muscles of the tongue. What we found was that, as you dilute out the agent, you extend the incubation period. You get about 107 dilution and only one of the five animals comes down with this very long incubation period. From this we can calculate the titer. The titer by this route is 108.4 LD-50s per gram of inoculum. When we do the same sort of dose-response experiment, this time directly inoculating into the brain, the relative titer is 109.8 LD-50s per gram of inoculum and you can see that inter-tongue infection is only 10100-fold less efficient than a direct inoculation to the brain.
When we do this same experiment drying the inoculum on a food pellet and feeding it, it's a very inefficient route of infection, 103.4 LD-50s per gram, and this indicates that inter-tongue infection is 100,000-fold more efficient at causing disease than ingestion of the agent.
Next we wanted to ask the question, "Can lesions on the surface of the tongue increase the susceptibility of the host to prion infection?" Here we had four experimental groups. Our positive control was direct inoculation of the tongue, and as expected they came down with a short incubation period, 182 days. All the animals got the disease.
Our second control group was per os. Here, three of the 15 animals came down with the clinical signs at 185 days. For our third group, we placed inoculum on the surface of an unlesioned tongue. In these animals, five of the 15 developed clinical signs with an incubation period of 184 days, similar to the per os. In our experimental group, a small lesion was placed on the surface of the tongue. The same dose of inoculum was placed on that lesion, and in this group, all fifteen animals developed clinical signs with an incubation period of 161 days. Interestingly, these first five animals had an incubation period similar to direct inoculation of the tongue. This experiment demonstrates that a lesion on the surface of the tongue can increase the susceptibility of a host to prion infection.
To summarize the transmission data, inoculation into the tongue is 100,000-fold more effective than ingestion, at least in hamsters. Lesions on the surface of the tongue increase the susceptibility of the host to TME infection.

Routes of Efficiency
Why is this route so efficient? If we go back to our general schematic of prion replication, in peripheral routes, prions replicate in secondary LRS tissues prior to neuro-invasion. We set up a temporal study. Following tongue infection, we collected spleen and the sub-mandibular lymph node (the lymph node that drains the oral cavity, including the tongue). In the spleen, we were unable to detect PRPSC by western blot out to 10 weeks post-infection. This was just prior to the onset of clinical signs. As a positive control, we took a spleen from an animal that was intra-cerebrally inoculated and we could detect PRPSC in the spleen.
In the sub-mandibular lymph node, we were able to detect PRPSC at one week post-infection. It increased in abundance to three weeks post-infection and then plateaued out at five weeks post-infection. Based on this data, we would think the sympathetic route was involved in transport of the agent to the central nervous system. Based on this model, following inter-tongue infection, the agent drains to the sub-mandibular lymph node, replicates in this lymph node, travels via sympathetic internervation through the superior cervical ganglia, down the sympathetic chain. It should first be detected in the central nervous system in the thoracic spinal cord.
To test this, we set up a temporal study following inter-tongue infection and we collected the spinal cord and did western blot analysis. At seven weeks post-infection, we could not detect PRPSC in the cord. At eight weeks post-infection, we could detect PRPSC in the spinal cord. We detected it not only on the thoracic segments but also in the cervical segments. At nine and 10 weeks post-infection, PRPSC levels accumulate. This data was inconclusive in that not only was it in the thoracic, but in the cervical.
Next we asked the question, "Where in the brain or brain stem do you first see PRPSC following IT infection?" This time we used immunohisto-chemistry. We found that the first place PRPSC is identified is in the hypoglossal nucleus. By six weeks post-infection, PRPSC staining was wide-spread throughout the hypoglossal nucleus. The significance of this staining is that the hypoglossal nucleus contains only motor neurons, and the only function of the motor neurons in the hypoglossal nucleus is to internervate the tongue.

Dr. Detwiler: Do you say that the prion goes from the tongue to the sub-mandibular lymph node through nerves or through the lymphatic flow?

Dr. Bartz: We think it gets to the lymph node just via the lymphatic flow. There's no neural connection between the lymph node and the tongue.

Dr. Thornsberry: So when the lymphatic flow gets to the lymph node, then it dumps into the blood.

Dr. Bartz: Right. But why do we see PRPSC in the sub-mandibular lymph node at one week post-infection, but by the time clinical disease occurs, we can't detect it in the spleen? If it's in the blood, it should seed all the lymphatics in the host, but it doesn't

Dr. Spraker: Deer lymph nodes are 10 times better than spleen.

Dr. Detwiler: In sheep with sheep Scrapie, on this widespread distribution from multiple tissues at multiple sites, within the incubation period, how would you explain that from the spread of either the sympathetic or the para-sympathetic without some kind of circulatory process?

Dr. Bartz: It could get in the blood and it could be neuro-invading by 10 different routes. We don't know. But this is the main route, so the first place we see it is in the hypoglossal nucleus. It's the only place we see it in the brain until four weeks post-infection.
This mapping study is consistent with previous reports. When people use trans-neural viral tracers such as rabies and they inject the hypoglossal nerve, they find that it goes to the hypoglossal nucleus, and then the second order neurons that project afferents onto the hypoglossal nucleus in this reticular formation and all the different structures we identify PRPSC, so it seems to be transynaptically transported to second-order neurons, the same as other trans-neural viral tracers.
To summarize inter-tongue, as we think of inter-tongue infection, the agent is getting into the hypoglossal nerve and is being retrogradily transported at the hypoglossal nerve to the hypoglossal nucleus. It can replicate in the hypoglossal nucleus and then be transynaptically transported to second-order neurons, and eventually we think that PRPSC gets to the clinical target areas, resulting in clinical signs and death of the host.

Replication and Neuro-Invasion
The hypoglossal nucleus was not the first place we identified PRPSC in the host. It was in the sub-mandibular lymph node. We wanted to ask the question, is replication in the LRS system required for neuro-invasion following inter-tongue infections? To investigate this, we used mMT mice, which are transgenic mice that has a disruption in the immunoglobulin chain and they lack B lymphocytes. Since they lack B lymphocytes, which secrete factors that support FDC maintenance, they do not have follicular dendritic cells.
Previous studies using these in prion diseases have shown that they're susceptible to IC infection, and this means that once the agent gets to the brain, it can replicate and cause disease. But following a IP infection, they don't come down with disease. If you look in secondary LRS tissues, there's no detectable infectivity or PRPSC.
Using these mice following inter-tongue infection, if the animals come down, that would mean that replication in the secondary LRS tissues is not required for neuro-invasion and to set up this experiment, we had two different sets of mice. We had the mMT mice, which cannot replicate agent in the secondary LRS tissues, and we used C-57 black six mice, which can replicate prions in secondary LRS tissues. We inoculated them directly into the brain and had an incubation period of 160 days. We did an IP inoculation. Wild types come down at 216 days. The mMT's are resistant. All of this is consistent with previously-published studies. We did an IT inoculation into wild type mice with 213 days incubation. All the mice came down. We did an IT inoculation with the mMT mice and got a similar incubation period—217 days with five of the six mice developing clinical signs. We did western blot analysis on secondary LRS tissue in all these mice, and consistent with what other people found, we were unable to detect PRPSC in the spleen or sub-mandibular lymph node. This data is consistent with the hypothesis that replication in secondary LRS tissues is not required for neuro-invasion following inter-tongue infection.
To summarize the pathogenesis, in general, following a peripheral infection, you get transport and replication in secondary LRS tissues. Once it replicates in these tissues, neuro-invasion occurs and you get a rapid accumulation of PRPSC in the brain. So far there have been three routes of neuro-invasion identified following oral infection, two of which originate in the gut using either sympathetic or parasympathetic internervation. The third are the studies with motor neurons—the oral cavity directly transporting agent to the brain.

Question: You said that's the normal progression of infection—that it normally goes into those tissues and then makes its way to the nervous system. But you've proven that's not required?

Dr. Bartz: I think the LRS is an amplifier of the agent in the periphery. You probably need a certain dose. You either need the agent to be in proximity to the nerve or replicate to a high enough dose it can get there. You can probably overcome this with a high enough dose, but if you give a low dose, you probably need replication in the host. So I think the LRS is a spot in the host where the agent can replicate in the periphery to facilitate neuro-invasion. The LRS tissues that prions replicate in—as far as I know, there's no deficit in these tissues.

Comment: With deer, the hypoglossal nucleus is one of the latter nuclei to be infected. In deer, 99% of the lymphoid tissue will be infected in early cases, even before the brain is infected. On the opposite side, there's a percentage of elk infected that do not have PRP in lymph nodes. So an alternate route of infection has been postulated.

Dr. Bartz: I'm not making any sort of assertion that this happens in natural cases. The only reason I think it's interesting to discuss is because it's so efficient.

Question: Why couldn't you have multiple routes?

Dr. Bartz: You could. If it occurs in one out of every thousand cases, would we find it? Maybe, maybe not.

Question: If you look at lymphoid tissue only for diagnosing CWD, you're going to miss a few. If there are several different route of infection, one of them could be straight to the nerve in heavy doses.

Dr. Bartz: The last think I want to touch upon is interspecies transmission. What are the outcomes of inter-species transmission? First, it cannot convert the host PRPC. There's no infection, no clinical disease. End of story. The second thing that can happen is the agent can convert the host PRPC and you get clinical disease. Or the agent can convert host PRPC and there's no clinical disease, and this is persistence.
I'm going to start with the occurrence of clinical disease and break this up into two parts. What is the species barrier effect? An example is if you take mink that have transmissible mink encephalopathy and do an IC inoculation into the same host species, the incubation period is four months. But if you go into a new host species, this time the ferret, and do an IC inoculation, you get a huge extension of the incubation period of 28 to 30 months. The mink and ferret are both mustalids and differ by only two amino acids.
When you keep passaging the TME in the same host species—the ferret, eventually you see a shortening of the incubation period and eventual stabilization of the agent for the new host species. I break down the species barrier effect into two components. The first is the inter-species transmission and the second is adaptation of the agent for the new host species.
One way to look at the species barrier effect is a technique called cell-free conversion. This technique was developed in Byron Caughey's lab at NIH. It takes PRPC that's radio-labeled and growing tissue culture. You purify it and incubate it with brain-derived PRPSC, and when you co-incubate these two molecules, if the PRPSC can convert the radio-labeled PRPC to a PK resistant form, when you put this onto a piece of film, the only thing that should be radioactive is the converted radioactive PRPSC, which is proteinase-K sensitive to a PK-resistant form.
We know that PRPC is resistant to proteinase-K digestion. When you treat PRPSC with PK, it all goes away. If you incubate the radio-labeled mouse PRPC with mouse PRPSC, you'll get mouse PRPSC converting the mouse PRPC to a PK-resistant form. What's nice about this system is that you can use it to start to ask questions. What is important between the host PRPC and the agent PRPSC as far as inter-species transmission? This example uses hamster PRPSC. We know that if you inoculate hamster PRPSC into mice, there are no clinical signs. If you take hamster PRPSC, incubate it with the radio-labeled mouse PRPC, and treat it with proteinase-K, consistent with the biological properties, there's no conversion.
Now you can use this methodology and map out areas of PRPC that are important for inter-species transmission. Hamster PRPSC cannot convert. When you start to use these different chimeras, you can use it to start to map out regions of the molecule that are important for interspecies transmission.
You can also use it to try to predict species barriers where you don't know where transmission data is. This is a paper from Raymond in 2000. Here you can take PRPSC from sheep Scrapie with a certain genotype and incubate it with radio-labeled PRPC of sheep with the same genotype, and that's said to be 100% conversion because we know it's the same PRPSC going by intra-species transmission. You can start doing modifications where you use different genotypes of sheep PRPC, and you get a reduction in conversion, and then you can go to different species. Here they looked at mule deer, white tail deer or elk. You get a reduction in conversion.
You can also look at human PRPC, and there's a very low conversion. This low conversion is thought to be consistent, at least in sheep and humans. Epidemiologically, there's no evidence of sheep Scrapie transmitting to humans. You can also use this to assess the transmission of the white-tail deer PRPSC again using PRPC from a mule deer or a white tail deer of the same genotype. You can start modifying the genotypes of either deer or elk PRPC, and you get either an increase or decrease in conversion. You can ask questions about converting bovine PRPC and human PRPC. You get these much-reduced abilities to convert in this assay.
This reduction in conversion, then, one would predict would correlate with the transmission studies if they were done.

Inter-Species Transmission
The host genotype is important in whether the host is susceptible or not. I want to talk about the subsequent transmissions in the same host species. Why are you getting a reduction in the incubation period and adaptation or the intra-species transmission? When you have PRPSC from one host species interacting with PRPC from another, you get conversion to PRPSC. But I don't think you always get just one prion strain. On this inter-species transmission, you can get multiple prion strains. Data to support this hypothesis comes from some work I did using transmissible mink encephalopathy where we took biologically-cloned transmissible mink encephalopathy agent and transmitted it into hamsters. The bottom line is when we took the single strain and put it into hamsters, we were able to get the hyper and drowsy strains out. On first interspecies transmission, incubation periods are very long and there's quite a range—219 days to 501 days. They ranged anywhere from 130 days out to over 600 days.
In passage line B, 219-day incubation period, PRPSC was similar to that of hyper TME. The second passage has a reduction to 67 _____________

[tape change]

On western blots, we can tell hyper and drowsy apart. Otherwise, you have to do lesion profiling. So this is a nice system for trying to work this out.
In this passage line A, this first inter-species transmission, PRPSC is like drowsy TME. Drowsy is clinically characterized by lethargy. Hyper is clinically characterized by hyper-excitability ataxia. When you passage this, if you passage it at a high titer, now you have mixture clinical signs but you also have PRPSC that looks like a mixture of both hyper and drowsy. By fourth passage, you get the emergence of hyper. If you passage this animal at a low titer, you can get the emergence of drowsy. What we think is going on here is that inter-species transmission of the single TME strain in hamsters produces at least the hyper and drowsy strains, and we think, depending on the ratio of the strains produced in this first inter-species transmission, will result in either the rapid emergence or a longer emergence of the prion disease.

Dr. Detwiler: How would you explain that biochemically?

Dr. Bartz: When PRPC is converted to PRPSC, it's misfolded. There have to be many different stable energy states for the misfolded protein. I would hypothesize that mink PRPSC, when it interacts with hamster PRPC, it can fold into several different stable PRPSC molecules. So initially you get the mink interacting with hamster, and then you get a strain produced. I think early on in those first few rounds of replication, whatever strain is produced is probably going to be the predominant one because it has a jump start on the rest of them. On this really complicated western blot, we are mixing hyper and drowsy at known ratios, and basically we can mimic these effects. So it really is the ratio of hyper/drowsy produced by interspecies transmission that's causing this sort of effect.
To summarize inter-species transmission, we have PRPSC interacting with the new host PRPC molecule to change it into PRPSC. We think that, in certain instances, multiple strains can be produced. Intra-species transmission results in competition between these strains and eventual emergence of a predominant strain. We think the initial ratio of strains is important and affects this whole passage history. Probably the replication properties of strains is important. We think that drowsy is the predominant strain produced, but hyper replicates so much faster, it has an advantage.
One really important thing I want to point out here is that strain properties can change upon inter-species transmission. Chronic wasting disease doesn't cause disease when you passage it in a hamster, but if you passage CWD into ferrets, and then take that ferret passage tissue, it can cause disease in hamsters. So inter-species transmission can expand the host range. Also, with the hyper and drowsy, the more hamster passages you do, if you back-passage the inoculum into mink, hyper loses pathogenicity for mink quite quickly, where drowsy retains pathogenicity for mink.
The important point I want to make is that, when you're assessing inter-species transmission and you do a transmission study and it's negative, you have to be careful in saying it's negative for the strains you looked at. With this example, it's clear you could take hyper TME, inoculate mink, and they don't come down with the disease, so you might assume hamster prions don't cause disease in mink. That strain doesn't. You have to be careful assessing negative transmission results based on what's known about the strain properties.
The last thing I want to talk about is persistence. This would be the case where PRPSC interacts with the host PRPC and you get really slow replication. The replication agent is so slow that the animal dies of old age before clinical signs can occur.
This study is from Rick Race at NIH, transmitting hamster PRPSC into mice. He collected animals post-infection out to 782 days. None of these animals had clinical signs of prion disease, which is consistent with everything we knew about this species barrier. But when he went back and looked for PRP residue in these animals, he couldn't detect hamster PRP residue, but in a few of these animals with very long times post-infection, he could detect mouse PRP residue.
When he did the second passage, into either hamsters or mice, clinical signs appeared in the second passage. The point is that first inter-species transmission may not cause clinical signs, but you still can get replication to agent that subsequently, when you passage it into the same host species, results in clinical signs of the disease.
In the cell-free conversion studies, hamster PRPSC could not convert mouse PRPSC. Every sort of assay has limitations. The cell-free conversion said it couldn't replicate. It could, but it was so slow and so long that the assay could not detect them.
I think persistence is very important. If you have inter-species transmission occurring and it doesn't cause clinical disease, and if you take the tissue and keep feeding it to that same host species, you're going to get amplification and potentially emergence of the disease.
Is PRPSC shed in the environment? I have no idea. Terry can talk about that. Does PRPSC survive in the environment? The studies on deer PRPSC have not been done, but if deer PRPSC behaves like any other PRPSC, yes it can survive in the environment. Can PRPSC reach a new host species? I don't know. If they share common pastures, it's a possibility. Can PRPSC get to the central nervous system? Clearly, cattle are susceptible to oral infection, so that's yes. Can deer PRPSC convert cattle PRPC to the host PRPSC? Self-reconversion experiments would say yes, but very inefficiently. But really, the gold standard is the transmission studies, and there are two of these ongoing right now. One is at the USDA at Ames, and this is intra-cerebral inoculation. They are susceptible to IC infections. This means that once the agent reaches the brain, it can cause disease, but obviously in the field, that's not the natural route. Beth Williams is doing some oral infection studies, but I'm not sure of the status of those.

Dr. Thornsberry: So what you're saying is that, inter-cerebrally, we can get CWD/PRPSC conversion, but that has not occurred, to anyone's knowledge, in the natural route.

Dr. Bartz: Right. IC inoculation is used because it has a short incubation period. It only tells us that replication can occur once the agent reaches the brain.

Dr. Thornsberry: Let's hypothesize that I had some cattle on the eastern slope and they were in the same pasture with elk with CWD. If a cow had been exposed to the PRP Scrapie and it did develop disease four years later, would that look like BSE? Would there be a way to determine if it came from CWD?

Dr. Bartz: The IC studies in cattle indicate it does not look like BSE. The clinical signs of the IC/CWD cattle are more like downer cattle, and not aggressive. As far as finding the source of a bovine TSE, the gold standard is the lesion profile study where you take cattle tissue and inoculate it into mice with appropriate controls, wait until the mice come down, and do the lesion profiling.

Dr. Thornsberry: There were two cases in Japan, but they indicated that tissue was not classical BSE as seen in Europe. Have you heard anything about that?

Dr. Bartz: This is based on differences on migration and the glycoform ratio of PrPSc.

Dr. Detwiler: Canada based that question because the herd that that animal came from was in Saskatchewan, in an area with CWD. That was one of the questions they faced right off the bat: is this BSE or is this some kind of transmission from CWD-infected elk in the area? Not only the histological lesions were classic BSE lesions, but clinically it's very difficult because if you miss the other behavioral changes, which this owner did. It was someone who had been a catfish farmer. He missed the early signs. The animal presented to slaughter as a down animal, non-responsive. Clinically it looked like just a down cow, but they did send that on to the United Kingdom and they did do some comparison glycoform patterns. Those haven't been validated, but at least on preliminary work, it looked like classical BSE.
The Japanese case was a 23-month-old which was born in October. Their scientists say the western blot pattern looked different. The most recent case, which was a 21-month-old, looked more like classic BSE. The Italian cases were older animals, 15 and 16 years of age. But is it methodology? Is it really standard? That has to be sorted out before too much can be said.

Dr. Bartz: Glycoform ratio is dependent on very technical matters, what antibodies you use, what detection system you use. Those have to be standardized before you can start comparing from one lab to another.

Dr. Detwiler: The Japanese used a western blot they'd developed in their lab. It can't be compared across laboratories.

Dr. Bartz: That's problematic.





CHRONIC WASTING DISEASE

Dr. Terry Spraker: Max asked me to give an overview of CWD and then talk about the research being done at CSU. CWD was first noticed in the Colorado Division of Wildlife pens in Ft. Collins. CWD in the literature was first said to be seen in captive deer pens in 1967. I remember being there and those deer pens weren't even built until 1968, so the early history of CWD is confusing, but CWD was at least seen in the very early 1970's as far as I can remember.
Beth Williams verified that is was truly a spongiform encephalopathy in the deer. She and Stuart Young described CWD in deer and elk in Colorado and Wyoming. George Bear A big Game Biologist for the Colorado Division of Wildlife found a sick elk in Rocky Mountain National Park in 1981 and I posted it for him and it turned out to be a case of CWD. At that time, there was lots of interchange from the Colorado Division of Wildlife with the animals from Rocky Mountain National Park. They took animals from the deer facilities and released them into the park to study food habits, and brought the deer and elk back.
We found the first case in mule deer in 1984 about half a mile west of the Colorado Division of Wildlife deer pens. In 1985, we found the first case in white-tail deer in Loveland, about 30 miles south of Ft. Collins. There has in the past been a question whether CWD started in the CDOW deer pens or came from the wild. The man who built the deer pens in 1968 did put deer and sheep together. These animals were placed on starvation trials. He obtained the sheep from CSU. This is where this theory has come about that one biologist started CWD in the Colorado Division of Wildlife deer pens. He still has sheep today from the original group, and he's never had a case of scrapie. There is no real evidence that this was the start of CWD.
The other part of the history of CWD was when it was first found in captive elk in 1996. The man who had CWD in Canada has gotten elk from a place in South Dakota that had CWD for probably 10 years, but it was not diagnosed. The year after it was diagnosed in Canada, it was diagnosed on his farm in South Dakota. He claims he received deer from Colorado. So there is a fairly good link from deer and elk originally from Colorado and spreading northward.
The natural host for CWD is only three cervids: mule deer, elk and white-tail deer. Recently, CWD has been found everywhere north of Interstate 70 in Colorado. Now a case has been found south of I-70 and one in central Utah. We just looked at several deer from Mexico and those were negative. CWD has been found in wild populations in Wyoming, Nebraska, Wisconsin, Utah, South Dakota, New Mexico, and Illinois.

Dr. Thornsberry: I have a classmate who deals with the Wisconsin Department of Natural Resources, and he's done a lot of work with them on CWD. How did we get this hot pocket of CWD in Wisconsin? Did they trace that to somebody introducing it, or did it just show up?

Dr. Spraker: No one knows. People bring carcasses back to their homes. I've had calls from so many states and people want to know what to do with the carcass. One guy in Maine called me after he'd already thrown the carcass away. One person has taken six cases of CWD to British Columbia. The last couple he said he fed to the crabs—threw them in the ocean. There is a lot of movement of animals and lots of movement of animal parts. There was a big trade of Wisconsin deer that somehow went to Missouri and then were released in Texas.

Dr. Spraker: The captive herds are primarily in the same areas where there's lots of CWD in the wild. I'm sure there's some evidence that it goes both ways. What are some of the epidemiological observations? There is evidence of transmission of the disease in captive animals to wild and vice versa. In the wild, there's natural expansion.
Clinical signs include emaciation. These clinical signs are not manifested until the terminal aspects of the disease, so an animal might have the disease for two or three years without showing clinical signs. You'll see emaciation and occasionally you'll see hair loss. There are similarities to Scrapie, although you don't get the weight loss with Scrapie.
The clinical signs are obvious only in the terminal stages. They are depressed and you'll find them lying down. If you approach them, they do not get up.

Dr. Thornsberry: Is the chronic weight loss due to lack of neural functions of the muscles, or is it due to being mentally affected and they just don't eat?

Dr. Spraker: I don't know. The rumens are always full, so they are eating right up to the end. Because of the olfactory system that's heavily hit in these animals, I think that, at least in the wild, they lose their sense of smell. Deer are very dependent on smell for which forages to eat. Prion is present in the retina, so thei
 
well cr@p, wings have been clipped :wink: totally understand, i can be long winded sometimes. but sometimes that is what it takes, and this is why the site i post long winded stuff too is still there. i have to have a place to document this, and they have been there all along. but as with others, they make money off me with advertisements, and those things really get under my skin. one way i have found to get around all that advertisements that move that slow the site where i post to is to use Mozilla Firefox web browser, and you can unplug just about everyone of those damn things to where the page will not freeze up. money talks everywhere sorry to say. i have no control there and make no money, but thank God it's there. nuff said. bottom line it's the dreeded vegsource site, use that mozilla firefox and you can fix that though, cause i have tried everything i can to get them to fix it. many many folks complain about it, i just cant do nothing about it, and do not want to deal with my own website........nuff said, ...............here is short of it, but you need to read the complete roundtable event. THAT URL IS AT THE BOTTOM and will probably freeze up. IF anyone would like the pdf file, or text, i can email them privately. .......



Subject: BSE Roundtable Discussions 06/14/05
Date: June 15, 2005 at 7:06 am PST

06/14/05 BSE Roundtable Discussions

http://www.aphis.usda.gov/lpa/issues/bse/bse.html


Videos

Recorded Webcast: Opening Remarks as Prepared - Agriculture Secretary Mike Johanns (Part 1, Time 26:37)

Agriculture Secretary Mike Johanns led a roundtable panel discussion entitled, "The Safety of North American Beef and the Economic Effect of BSE on the U.S. Beef Industry," at the University of Minnesota St. Paul Campus - June 9, 2005.


The Safety of North American Beef and the Economic Effect of BSE on the U.S. Beef Industry, (Part 2, Time 1:06:40)


The Safety of North American Beef and the Economic Effect of BSE on the U.S. Beef Industry, (Part 3, Time 53:11)


The Safety of North American Beef and the Economic Effect of BSE on the U.S. Beef Industry, (Part 4, Time 40:14)



Remarks and Transcripts

REMARKS AS PREPARED FOR DELIVERY by Secretary Mike Johanns - U.S. Department of Agriculture BSE Roundtable at The University of Minnesota in St. Paul, Minnesota (06/09/05)


REMARKS AS PREPARED FOR DELIVERY By USDA Chief Economist Keith Collins - The Economic Consequences of BSE For the North American Cattle and Beef Industries The USDA BSE Roundtable The University of Minnesota St. Paul, Minnesota (06/09/05)


REMARKS AS PREPARED FOR DELIVERY By Ron DeHaven, Administrator Animal and Plant Health Inspection Service (APHIS) U.S. Department of Agriculture BSE Roundtable The University of Minnesota St. Paul, Minnesota (06/09/05)


REMARKS AS PREPARED FOR DELIVERY By Acting Administrator, Dr. Barbara Masters The Food Safety and Inspection Service U.S. Department of Agriculture BSE Roundtable The University of Minnesota St. Paul, Minnesota (06/09/05)



Fact Sheets

USDA Secretary's BSE Roundtable Panelists (pdf)


Safety of North American Cattle and Beef (pdf)


Economic Consequences of BSE for the North American Cattle and Beef Industries (pdf)



Last Modified: 06/10/2005

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=webcast_bse.xml


USDA 2003

We have to be careful that we don't get so set in the way we do things that
we
forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.
Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS
.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.


snip.............


Dr. Detwiler: It seems a good idea, but I'm not aware of it.
Another important thing to get across to the public is that the negatives
do not guarantee absence of infectivity. The animal could be early in the
disease and the incubation period. Even sample collection is so important.
If you're not collecting the right area of the brain in sheep, or if
collecting lymphoreticular tissue, and you don't get a good biopsy, you
could miss the area with the PRP in it and come up with a negative test.
There's a new, unusual form of Scrapie that's been detected in Norway. We
have to be careful that we don't get so set in the way we do things that we
forget to look for different emerging variations of disease. We've gotten
away from collecting the whole brain in our systems. We're using the brain
stem and we're looking in only one area. In Norway, they were doing a
project and looking at cases of Scrapie, and they found this where they did
not find lesions or PRP in the area of the obex. They found it in the
cerebellum and the cerebrum. It's a good lesson for us. Ames had to go
back and change the procedure for looking at Scrapie samples. In the USDA,
we had routinely looked at all the sections of the brain, and then we got
away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an
'official' test result as recognized by APHIS
.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't
they still asking for the brain? But even on the slaughter, they're looking
only at the brainstem. We may be missing certain things if we confine
ourselves to one area.


snip...


FULL TEXT;


Completely Edited Version
PRION ROUNDTABLE


Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado


FULL TEXT, A MUST READ.......


http://www.vegsource.com/talk/madcow/messages/94543.html


TSS
 
Guys, when people promote ideas that are contrary to mainstream, conventional, generally accepted practices and ideas, and such unconventional ideas tie in quite well with various "conspiracy group" thought (John Birch type for one), what can you expect them to be called if not "conspiracy theories"?

And Mike, you are just plain silly! I have respect for Dr. Webers' professional work.......and you can only think of a silly comment rather than saying anything constructive! it's sad that you are that defeated by your own mind.

flounder, where is OIE in all this? Isn't the USA using their recommendations and standards in the BSE situation?

MRJ
 
MRJ, "Isn't the USA using their recommendations and standards in the BSE situation?"

No, we're not. One of the reasons R-CALF was against the Final Rule with Canada et al.
 
hello mrj,


mrj wrote;

> flounder, where is OIE in all this? Isn't the USA using their

> recommendations and standards in the BSE situation?


when the oie jumped in bed with gw et al at usda on this mrr policy, and threw 30 years of attempted BSE eradication down the tube for trade, and it was working in the countries that had a real 'enhanced' surveillance system and one that adhered to the feed ban and other stringent regulations, i said then the oie should 'hang up there jock strap', that they were nothing more than a International Brokerage firm for the legal trading of all strains of TSE globally ;




THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE.
AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. With Science like this, Japan would be fully justified in declining to be a member. ...


snip...



SO, the already terribly flawwed OIE BSE surveillance system is too burdensome for trade.
Aint that just too bad. SO, they decide to make it even weaker. The damn thing never worked
anyway. ALL one has to do is look at the documented BSE Countries that went by it. Did them
a lot of good.

TO think that a sample survey of 400 or so cattle in a population of 100 million, to think this will find anything, especially after seeing how many TSE tests it took Italy and other Countries to find 1 case of BSE (1 million rapid TSE test in less than 2 years, to find 102 BSE cases), should be proof enough to make drastic changes of this system. the OIE criteria for BSE Country classification and it's interpretation is very problematic. a text that is suppose to give guidelines, but is not understandable, cannot be considered satisfactory...


http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt




http://72.14.203.104/search?q=cache:rnEhHXKbnEsJ:prwatch.org/forum/showthread.php%3Ft%3D5560+oie+bse+jock+strap+tss&hl=en&gl=us&ct=clnk&cd=1



http://p079.ezboard.com/fwolftracksproductionsfrm2.showMessage?topicID=470.topic



kind regards,
tss
 
MRJ said:
Guys, when people promote ideas that are contrary to mainstream, conventional, generally accepted practices and ideas, and such unconventional ideas tie in quite well with various "conspiracy group" though (John Birch type for one), what can you expect them to be called if not "conspiracy theories"?



MRJ

MRJ- Are you saying that bank owning Republican South Dakota NCBA members ideas are the mainstream and that all others are unconventional and conspiracist :???:
 
Subject: APRIL 1-5 2006 BSE ALABAMA UPDATE
Date: April 5, 2006 at 2:18 pm PST

APRIL 1-5 2006 BSE ALABAMA UPDATE


APRIL FOOLS !!!


April 3 4,and 5, 2006
There is nothing new to report and the investigation continues.

http://www.aphis.usda.gov/newsroom/hot_issues/bse/bse_al_epi-update.shtml



out of sight, out of mind, maybe there hoping we'll forget about it. ...TSS




Greetings again Ranchers et al,

i wish to document here some old history again from about 1999 for other to have access to. i think some might find interest in this and see what was, was, and what is, is now, and you might see why we are still even discussing this issue. i must admit, it is another long winded post, but read the data, you will see what i speak of re-Detwiler, Miller et al, the bush administration, and BSE. Dr. Detwiler was begging for help back then, but instead of help, they just put them all out to pasture and thought BSE could be sweeped under the rug. NOW the cattle rancher and the consumer must pay for there blunder, and they will be paying for years to come ;


Subject: U.S. Emergency Bovine Spongiform Encephalopathy Response Plan
Summary
Date: Tue, 4 May 1999 18:25:12 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: [email protected]
From: Terry S. Singeltary Sr., Bacliff, Texas......

I thought it might be interesting for those of you who have not seen
this plan, to do so. So here it is...........

The mission of the U.S. Department of Agriculture (USDA) is to enhance
the quality of life for the American people by supporting production
agriculture; ensuring a safe, affordable, nutritious, and accessible
food supply; caring for agricultural, forest, and range lands;
supporting sound development of rural communities; providing economic
opportunities for farm and rural residents; expanding global markets for
agricultural and forest products and services; and working to reduce
hunger in America and throughout the world.

USDA's Animal and Plant Health Inspection Service (APHIS) is responsible
for ensuring the health and care of animals and plants. APHIS improves
agricultural productivity and competitiveness and contributes to the
national economy and the public health. USDA's Food Safety and
Inspection Service (FSIS) is responsible for protecting the Nation's
meat and poultry supply--making sure it is safe, wholesome,
unadulterated, and properly labeled and packaged. These two agencies
have come together to lead USDA's actions in the prevention, monitoring,
and control of bovine spongiform encephalopathy (BSE) in the U.S.
livestock and food supply.
The public knows BSE as "MAD COW DISEASE", a disease linked to human
cases of new-variant Creutzfeldt-Jakob disease (nvCJD). USDA knows BSE
as the disease that devastated the livestock industry in the United
Kingdom and shattered consumer confidence in Europe. BSE has affected
international trade and all aspects of the animal and public health
communities. It has called even greater attention to the U.S.
Government's accountability for a safe food supply.
No case of BSE has ever been found in the United States. Since 1989,
USDA has had a number of stringent safeguards in place to prevent BSE
from entering the country. USDA conducts an ongoing, comprehensive
interagency surveillance program for BSE. This surveillance program
allows USDA to monitor actively for BSE to ensure immediate detection in
the event that BSE were to be introduced into the United States.
Immediate detection allows for swift response. As an emergency
preparedness measure, USDA has developed this BSE Response Plan to be
initiated in the event that a case of BSE is diagnosed in the United
States. The Plan details comprehensive instructions for USDA staff as to
who is to do what, when, where, and how in the event that BSE were to be
diagnosed in the United States.

BACKGROUND

APHIS is responsible for being prepared for potential FOREIGN animal
disease outbreaks. The purpose of such preparation is to provide a
step-by-step plan of action in the event that a FOREIGN animal disease,
such as BSE, is detected in the United States. These plans, often
referred to as "RED BOOKS", provide guidance by outlining certain
actions that should take place, such as identification of a suspect
animal, laboratory confirmation, epidemiologic investigation, and animal
and herd disposition activities. Copies of Red Books for specific
FOREIGN animal diseases are distributed to agency headquarters and each
regional and field office to have in preparation for a disease outbreak.

In 1990, APHIS developed a plan to respond to a confirmation of BSE in
the United States. In August 1996, a joint APHIS-FSIS working group
updated the BSE Red Book in accordance with current science and research
surrounding BSE and the related family of disease called transmissible
spongiform encephalopathies (TSE's). The BSE Red Book is officially
entitled BSE EMERGENCY DISEASE GUIDELINES.
The APHIS-FSIS working group determined that the BSE Red Book, which
detailed laboratory and field activities to be carried out in an
emergency, needed another component. After the March 1996 announcement
by the United Kingdom that BSE was linked to nvCJD, it became apparent
to the working group that the Plan needed to address communication
issues, both internally within USDA and the Federal Government and
externally to the public at large. A confirmed case of BSE would affect
such a vast array of stakeholders-consumers, cattle producers, the food
animal industry, international trading partners, animal and public
health communities, media, and others. Having clear, accurate
information readily available would build trust and credibility and
facilitate any response measures needed. There needed to be a
notification plan. Who was responsible for notifying who, what, when,
and how? The plan needed to identify clear channels of communication as
to ensure immediate collection and dissemination of accurate
information.
The joint APHIS--FSIS working group became formally known as the BSE
Response Team and is responsible for the development of this BSE
Response Response Plan. BSE Response Team members represent a mix of
backgrounds and expertise, including veterinary medicine, food safety,
public health, epidemiology, pathology, international trade, and public
affairs. The Team is coordinatied by two Team Leaders, one each from
APHIS and FSIS, who serve as liaisons and technical advisors to their
respective agencies on regulations and policies regarding BSE.
Over the past 2 years, the BSE Response Plan has been reviewed, edited,
revised, and approved by officials at all levels of APHIS, FSIS, and
USDA. The Plan has also been shared with other Government agencies, such
as the Food and Drug Administration (FDA), the Centers for Disease
Control and Prevention (CDC), and the National Institutes of Health
(NIH), and other stakeholders, such as the Animal Ag Coalition.
The BSE Response Team monitors and assesses all ongoing events and
research findings regarding TSE's. The Team leaders are responsible for
ensuring that prevention and diagnostic measures are continually revised
and adjusted as new information and knowledge become available.

NOTIFICATION: Roles and Responsibilities

Surveillance

As part of USDA's surveillance program for BSE in the United States,
veterinary pathologists and field investigators from APHIS and FSIS have
received training from British counterparts in diagnosing BSE. FSIS
inspects cattle before they go to slaughter; these inspection procedures
include identifying animals with central nervous system conditions.
Animals with such conditions are considered suspect for BSE, prohibited
from slaughter, and referred to APHIS for examination as explained
below.
Pathologists at APHIS National Veterinary Services Laboratories (NVSL)
histopathologically examine the brains from these condemned animals. In
addition, samples are tested using a technique called
immunohistochemistry, which tests for the presence of the
protease-resistant prion protein (a marker for BSE). NVSL also examines
samples from neurologically ill cattle and nonambulatory ("DOWNER")
cattle identified on the farm or at slaughter and from rabies-negative
cattle submitted to veterinary diagnostic laboratories and teaching
hospitals.

NOTIFICATION

Because of their responsibility for examining condemned or BSE-suspect
animals, NVSL is the organization responsible for activating the
notification and BSE response process. It is NVSL that will begin the
activation of the BSE Response Plan. From the time a sample is
submitted, it takes 14 to 18 days to confirm a diagnosis of BSE In the
first 10 to 13 days, pathologists at NVSL have enough information to
either rule out BSE or determine the need for additional tests. If it is
determined that there is no evidence of BSE, the results are added to
the more than 7,500 others that have also been negative. NVSL maintains
these data.
If additional tests do suggest a presumptive diagnosis of BSE, an NVSL
pathologist will hand carry the sample to the United Kingdom for
confirmation. It is at this critical point, when NVSL suggests a
diagnosis of BSE and is preparing to send the sample to the United
Kingdom, that this BSE Response Plan is initiated. The Plan begins the
preliminary notification from NVSL to APHIS.

Prelimanary Notification

The director of NVSL is responsible for immediately notifying the APHIS,
Veterinary Services (VS) deputy administrator when tests suggest a
presumptive diagnosis of BSE.
Once NVSL has made a presumptive diagnosis of BSE, APHIS and FSIS field
activities will also be initiated. APHIS will receive notification
(either confirming or not confirming NVSL's diagnosis) from the United
Kingdom anywhere between 24 and 96 hours. (The international animal
health community has recognized the United Kingdom's Central Veterinary
Laboratory {CVL} as the world's reference laboratory for diagnosing BSE.
Other countries, including Belgium, France, Ireland, Luxembourg, the
Netherlands, Portugal, and Switzerland, have all sent samples to this
lab to confirm their first case of BSE).

NVSL

NVSL will provide all laboratory support in carrying out this BSE
Response Plan and serve as the liaison with CVL. NVSL will prepare its
facility to receive and process additional samples from the suspect
animal's progeny or herdmates or other suspects. NVSL will also
coordinate any other assistance from State or university diagnostic
laboratories if necessary.

APHIS, VS DEPUTY ADMINISTRATOR

Veterinary Services is the animal health arm of APHIS and the program
responsible for carrying out field actions in response to BSE. Upon
notifiction of a presumptive diagnosis from NVSL, the APHIS, VS deputy
administrator immediately notifies the FSIS, Office of Public Health and
Science (OPHS) deputy administrator. APHIS and FSIS deputy
administrators will alert the BSE Response Team and activate the
Response Plan. The VS deputy administrator serves as the liaison between
the BSE Response Team and the APHIS administrator.
The APHIS, VS deputy administrator notifies the APHIS administrator and
the VS regional director of the State from which the suspect animal
originated.

APHIS Administrator

The APHIS Administrator immediately notifies the USDA Assistant
Secretary for Marketing and Regulatory Programs. This immediate
notification will be followed by an official informational memorandum
from the APHIS Administrator, through the Assistant Secretary for
Marketing and Regulatory Programs, to the Secretary of Agriculture. This
memorandum will be prepared by the BSE Response Team; a draft is
maintained by the Team leaders in the reserved section of their plans.
The APHIS Administrator is responsible for securing indemnity funds for
depopulation of the herd if CVL confirms NVSL's diagnosis.

Assistant Secretary for Marketing and Regulatory Programs

The Assistant Secretary for Marketing and Regulatory Programs, in
conjuction with the Undersecretary for Food Safety, is responsible for
notifying the Secretary. The Assistant Secretary serves as the liaison
between APHIS and Department-level officials.

Secretary of Agriculture

The Secretary has the authority to declare a Federal EMERGENCY if
appropriate and approve funding as necessary. Information will be
provided to the Secretary up the chain of command from the BSE Response
Team.

FSIS, OPHS Deputy Administrator

The OPHS Deputy Administrator, together with the APHIS, VS Deputy
Administrator, alert the BSE Response Team leaders and instruct them to
assemble the BSE Response Team and activate the Plan. The OPHS Deputy
Administrator serves as the liaison between the BSE Response Team and
the FSIS Administrator.
The OPHS Deputy Administrator is responsible for notifying the FSIS
regional director in charge of the State from which the suspect animal
originated.

FSIS Deputy Administrator

The FSIS Deputy Administrator is responsible for notifying the
Undersecretary for Food Safety.

Undersecretary for Food Safety

The Undersecretary for Food Safety, in conjuction with the Assistant
Secretary for Marketing and Regulatory Programs, notifies the Secretary
of Agriculture.

APHIS, VS, Regional Director

The APHIS, VS regional director in charge of the State from which the
suspect animal originated notifies the VS Area Veterinarian-in-Charge
(AVIC) for that State. The regional director is the liaison between VS
field staff and the VS Deputy Administrator at headquarters. In
addition, the regional director shares all information with the BSE
Response Team.

APHIS, VS, AVIC

The VS AVIC, in cooperation with State animal health authorities, is
responsible for coordination the field activities surrounding the
emergency response to BSE. The AVIC assembles the local VS staff to
initiate activities outlined in the BSE Red Book including tracing the
progeny and herdmates of the suspect animal and beginning an
epidemiologic investigation. The VS AVIC coordinates with the State
Veterinarian to quarantine the suspect animal's herd of origin. The
State has the authority to order a routine quarantine for a neurological
disease. The BSE Response Team surveyed every State to determine if they
would utilize this authority in the event that NVSL identifies a
presumptive diagnosis of BSE. All States responded that they would issue
a quarantine.

BSE Response Team

The BSE Response Team leaders will notify each team member and instruct
them to assemble in the Situation Room at APHIS headquarters in
Riverdale, MD. The Team leaders are responsible for ensuring that all of
the Team's duties are fulfilled. It is their responsibility to ensure
that the technical information and expert recommendations reach the
decisionmakers in a timely fashion. Together with VS Emergency Programs
staff, the Team leaders will obtain APHIS, VS administrative support
staff in Riverdale, MD, to ready the room for use as BSE headquarters.
The Team will begin gathering and assembling information from APHIS and
FSIS region and field staff. The Team will pull the draft documents from
the third section in the Team leaders manuals and begin filling in
current information as it becomes available.

Public Notification

Should NVSL receive notice from CVL confirming a case of BSE, the next
level of notification is activated. Each player will follow the same
notification protocol as described above for preliminary notification to
confirm the diagnosis of a case of BSE.

BSE Response Team

The BSE Response Team will complete the informational memorandum for the
Secretary. The Team will prepare the letter to the Office of
International Epizootics (OIE), the international animal health
organization, for signature by the APHIS, VS Deputy Administrator. OIE
requires that all countries submit official notification within 24 hours
of confirming a diagnosis of BSE.
The BSE Response Team and the office of the APHIS, VS Deputy
Administrator would coordinate a teleconference to inform all APHIS
regional directors and AVIC'S.
The BSE Response Team and the office of the FSIS, OPHS Deputy
Administrator would coordinate a teleconference to inform all regional
and field FSIS offices.
The BSE Response Team would coordinate a teleconference to notify other
Federal agencies.
The BSE Response Team would coordinate a teleconference to notify key
industry/consumer representatives.
The BSE Response Team and APHIS International Services would notify
foreign embassies.
The BSE Response Team would establish a toll-free 800 telephone line for
industry representatives, reporters, and the public.
The BSE Response Team would coordinate with APHIS Legislative and Public
Affairs and USDA office of Communications to issue a press release the
day the diagnosis is confirmed. The press release would announce a press
conference to be held the morning after the diagnosis is confirmed......

THE END



BSE Red Book 2.1-35

7.0 Emergency Operations

The section below would be implemented only after a first case of BSE is
confirmed in the United States.

7.1 READEO Activation

READEO activation will rarely be necessary for BSE outbreaks. Different
from most other foreign animal diseases and infectious diseases, BSE is
not a rapidly spreading, acute epizootic; is not thought to be
transmitted horizontally between animals within a herd, has an extremely
long incubation period, and usually affects only isolated single animals
or, at most, a few animals within herds. Because BSE does not spread
rapidly, the workload to investigate and manage most outbreaks should
not normally exceed the capability of existing local field personnel.
READEO activation should be considered only if the particular
circumstances of a BSE outbreak warrant. If field personnel feel they
are unable to manage a BSE outbreak, they should communicate this to
their Regional Director and VS, Emergency Program staff, who will
evaluate the need for READEO activation.

7.2 READEO Organization

If READEO is activated, a reference should be made to the revised READEO
Manual for further guidance on READEO organization and operations.

7.2.1 Office of the Director
When an animal disease emergency exists, the Task Force Directors are
responsible for the READEO activities. The directors immediately move to
the location of the outbreak and setup the READEO headquarters. Work is
coordinated with State officials of the States involved in the outbreak.
7.2.1.1 State Director--(Note: This is the new designation for the
Assistant Director.) Each READEO may have one or more State Directors
since each State where the disease outbreak is found will be represented
in the READEO by State officials designated by the State Veterinarian.
7.2.1.2 Emergency Program Officer--This individual, designated by the
Chief Staff Veterinarian of VS, Emergency Programs, provides liaison
between the READEO and the Emergency Programs at APHIS headquarters.
7.2.1.3 Public Affairs Officer--The Public Affairs Officer plans,
develops, supervises, and maintains information activities for the
READEO.
7.2.1.4 Legal--The Legal Advisor provides counsel and assistance to the
READEO.
7.2.1.5 Military--The U.S. Armed Forces Command will designate a senior
line officer to be the Military Support Officer on the staff of the
READEO Task Force Directon The individual is assigned to be the liaison
between the Depart-

October 1998

BSE Red Book 2.1-36

ment of Defense and VS, Emergency Programs,and to coordinate needed
military assistance during eradication of an FAD outbreak.
7.2.1.6 Meat and Poultry Inspection Operations--The Meat and Poultry
Inspection Operations, Food Safety and Inspection Service, will
designate personnel to report to the READEO Task Director and to provide
liaison between the Task Force and the Meat and Poultry Inspection
Operations.
7.2.1.7 Laboratory Coordination--The Laboratory Coordination Officer
will advise the READE(3 Director concerning laboratory capabilities and
appropriate laboratory examinations to be conducted to provide needed
results as rapidly as possible. This individual will assist with
interpretation of results.

7.2.2 Administration
The Administrative Officer assigned to the READEO will direct and
coordinate all facets of general administrative functions. Refer to the
revised READEO Manual for a detailed description of the organization and
responsibilities.

7.2.3 Field Operations
The Field Operations Officer will direct line operations and supervise
field personnel in a READEO. Disease investigation, field epidemiology,
disease security and personnel security, animal movement control and
quarantine enforcement, appraisals of animals and materials,
depopulation and disposal, and cleaning and disinfection are among this
person's responsibilities.

7.2.4 Technical Support
Staff support consists of a technically competent staff designed to act
as a resource for the READEO Task Force. Personnel may include but are
not limited to individuals who have expertise in the following areas:
animal welfare, data systems, disease reporting, economics,
environmental impact, epidemiology, evaluation, orientation and
training, risk analysis, and wildlife. The staff communicates the needs
of the Field Epidemiology Delivery System (FEDS) to the READEO Director
as required to maintain an efficient, accurate, up-to-date FEDS.
7.2.4.1 Animal Welfare---Animal Welfare Officers must be knowledgeable
about current Federal and State animal welfare regulations, humane
methods of animal depopulation, and socioeconomic concerns related to
animal welfare issues. They advise the technical support staff and field
operations concerning current procedures and accepted methods for use in
the humane depopulation of livestock and poultry.
7.2.4.2 Wildlife-- Wildlife Officers participate with the Director and
other officials of the READEO to establish and carry out wildlife
policies and objectives for the emergency animal disease operation.
Through familiarity with the topography, wildlife density, susceptible
wildlife species, and movements of susceptible wildlife, the Wildlife
Officers can review maps and make recommendations concerning areas to be
included in the quarantined high-risk and buffer zones. These officers
maintain contact with local, State, and Federal wildlife enforcement
officers and wildlife biologists. They develop strategies for conducting
surveys of susceptible wi!dlife in the outbreak

October 1998

BSE Red Book 2.1-37

area to determine the incidence of the disease. They direct and
coordinate the vaccination and depopulation of wild animals as necessary
to eliminate the disease.

7.3 Supplies and Equipment

During an outbreak of BSE, supplies and equipment should be obtained
through normal procurement procedures. If a READEO is activated,
supplies and equipment should be ordered through the READEO Procurement
and Supply Officer.

7.3.1 General Field Supplies Guidelines
Refer to APHIS Directive 326.1, 10/10/77 and 221.1, 1/29/74.

7.4 Personnel Responsibilities

During a BSE outbreak, field personnel should follow instructions issued
through the normal chain of command. If a READEO is activated, personnel
should refer to the revised READEO Manual for detailed descriptions of
individual responsibilities.

7.4.1 Personnel
Personnel assigned to the READEO Task Force are individually accountable
for equipment and supplies checked out to them. They should order
replacement equipment and supplies or return equipment for repairs
through the READEO Procurement and Supply Officer. All damages or losses
to equipment or vehicles should be reported immediately to the READEO
Administrative Officer, and the required forms should be completed and
submitted promptly.

7.4.2 Travel
Employees of the READEO Task Force are responsible for recording and
preparing all travel-related documents. Claims for travel, lodging, per
diem, and incidental expenses should be submitted to the READEO
Administrative Officer for processing.

7.4.3 Vehicles
Employees of the READEO Task Force are responsible for operating,
cleaning, and performing routine maintenance of assigned vehicles. They
also are responsible for recording mileage, expenses, and services.
Required reports are to be submitted to the READEO Vehicle Officer.

7.4. 4 Clothing
Employees of the READEO Task Force are issued protective clothing to
wear when entering a premises where BSE has been diagnosed or is
suspected. Clean clothing should be worn on each premises. Employees are
responsible for laundering the clothing before reusing it.

October 1998

BSE Red Book 2.1-38

In a large task-foree operation, arrangements may be made for a
commercial laundry service to handle the clothing. If it is possible and
practical, all clothing should be labeled to identify the employees to
whom it is assigned.

7.4.5 Miscellaneous Responsibilities
Employees are responsible for conducting their assigned tasks in a
professional manner. Complaints concerning task force employees should
be directed to the READEO Director for resolution or appropriate action.

All animals, products, and materials to be destroyed because of BSE
should be appraised according to 9 CFR 53.3 and appropriate State
regulations.

7.5.1 Appraisal Teams
Appraisals must represent the interests of the owner, the State, and the
Federal Government and be consistent with fair market values. If State
authorities approve, State and Federal interests may be represented by a
VS employee alone. Owners may, at their discretion and expense, employ a
professional appraiser to advise them or to act as their agent. Either
the owner or the owner's agent must be present at appraisals.
No animals may be destroyed until after the appraisal forms are signed
by the owner or the owner's agent. Appraisers should be certain that the
owner or the owner's agent is aware of the indemnity form's clause
concerning liens and mortgages.
When the number of animals to be destroyed is small, and the total value
of animals, products, and materiais is low, APHIS field personnel may
negotiate the appraised value with the animal's owner without assistance
from a professional appraiser. The appraised value of a BSE suspect
should be the slaughter value of the animal, taking into account any
existing defects or diseases that would affect the slaughter value but
ignoring those signs that caused the animal to be classified as a BSE
suspect. If field personnel are in doubt concerning the need to use a
professional appraiser, they should consult their supervisor or VS,
Emergency Programs staff.
If a determination is made that healthy progeny, ova, semen, or embryos
must be destroyed, they should be appraised at 100 percent of
replacement value.
Feeds or feed ingredients located on suspect farms will rarely need to
be destroyed. If a determination is made that feeds or feed ingredients
must be destroyed (for example, to comply with a policy decision to
remove all rendered products from animal feeds), then these materials
should be appraised and indemn'ff~ed according to 9 CFR 53.3.

October 1998

BSE Red Book 2.1-39

7.6 Depopulation Procedures

7.6.1 Factors and Considerations
If the owner is agreeable, a humane method of euthanasia of BSE suspects
will be necessary to facilitate the accurate diagnosis of the disease
problem, to ensure that the suspect animal is not slaughtered or
rendered, and to terminate the animal's suffering. Under no
circumstances may BSE suspects be sent fo slaughhter or rendering.
Notify FDA, CVM if you suspect that the carcass of a BSE-confirmed
animal has moved to rendering or animal feed manufacturing. The VS,
Emergency Programs staff, Riverdale, MD, must authorize the use of
euthanasia, depopulation, and indemnity payments for READEO operations.

7.6.2 Humane Euthanasia Methods
Only experienced veterinarians should perform euthanasia because there
are inherent dangers. Precautions should be taken to prevent accidents.
Owners should be given a complete explanation of what to expect, and
only humane euthanasia methods should be used. Euthanasia should be
performed away from public view, and, if possible, the owner should not
be present. Euthanized animals must be checked to confirm death. (See VS
Memo 583.1, 1992.)
7.6.2.1 Mechanical (Firearms)--Because the only acceptable method for
euthanizing an animal by using firearms is to shoot it in the head, and
because the animal's brain must be preserved to diagnose BSE, firearms
are not an acceptable euthanasia method.
7.6.2.2 Chemicals(Toxic Gas or Lethal Injection)--Follow guidelines
established by the American Veterinary Medical Association. When using a
regulated controlled substance (e.g., barbiturates), control and
administration of the euthanasia agent must be given by a veterinarian
having a Drug Enforcement Administration (DEA) number issued by the U.S.
Treasury. Control and administration of chemical substances for
euthanasia must be authorized by the AVIC unless directed by the VS
Deputy Administrator. (See VS Memorandum 583.1, 1992.)

7.6.3 Supervision of Depopulation
Field personnel should never perform depopulation or euthanasia without
explicit permission from their supervisor or, if appropriate, the READEO
Humane and Disposal Officer. (Refer to the revised READEC) Manual.)

7. 6.4 Permits for Movement
All BSE suspects may be moved under permit to facilitate medical
treatment, euthanasia, necropsy examination, or carcass disposal.
Permitted movement will be according to the quarantine restrictions and
will be administered by the State or Federal officials.

7. 6. 5 Security
Because BSE is neither contagious nor vector borne, strict disease
security measures are not necessary. Personnel should observe normal
disease security measures that are standard procedure for all farm
visits. The READEO's Security and Disease Prevention Officer has the
responsibility for establishing biosecurity measures.

October 1998

BSE Red Book 2.1-40

7.7 Disposal
Under no circumstances may BSE suspects be sent to slaughter or
rendering. Notify FDA, CVM if you suspect that the carcass of a
BSE-confirmed animal has moved to rendering or animal feed
manufacturing. Field personel should arrange for the carcass to be
transported to and examined by a qualified veterinary pathologist or
field veterinary medical officer. After the pathologic examination has
been completed and the necessary diagnostic specimens have been
obtained, field personnel should arrange for disposal of the carcass.
Before a method of disposal is selected, there are many factors that
must be considered, and often other State and Federal agencies must be
consulted. The environmental and legal impacts of the operation must be
considered. Upon recommendation of the State or Federal agencies, VS may
consider other disposal methods.

7.7.1 Incineration
Incineration, although more expensive than burial, is the preferred
disposal method for BSE-suspect carcasses. Federal, State, and local
environmental regulations may restrict the use of this method and
permits may be necessary. As soon as BSE suspects are reported to APHIS,
field personnel should investigate the location and availability of
incinerators of sufficient size to process a bovine carcass.
Institutions likely to have incinerators include State and university
diagnostic laboratories, waste contractors, large municipalities, and
private industries. Ideally, the diagnostic laboratory where the
pathologic examination was done will have incineration facilities.
The BSE-suspect carcass disposal is APHIS' responsibility (not the
diagnostic laboratory's). Field personnel should arrange for
transportation and final disposal of the suspect carcass and should
inform their supervisors and/or the READEO Humane and Disposal Officer
of these arrangements.
Personnel should be aware that some laboratories dispose of carcasses by
rendering and should specifically inquire if this is the case. CNS
suspects should be incinerated or held from rendering until a diagnosis
of BSE can be ruled out. Under no circumstances may BSE susuects be sent
to slaughter or rendering. Notify FDA, CVM if you suspect that the
carcass of a BSE-confirmed animal has moved to rendering or animal feed
manufacturing.
Field personnel should be prepared to accompany the carcass from the
farm of origin to the diagnostic laboratory and then to the disposal
site if any doubt exists concerning the final disposal method.

7.7.2 Burial
If there are no other avenues for carcass disposal, burial of
BSE-suspect carcasses may be an acceptable disposal method. APHIS field
personnel should inquire with environmental authorities concerning
Federal, State, and local regulations that may impose restrictions on
this method.
The burial site may be on the affected farm, at the diagnostic
laboratory where the carcass is examined, or in a local landfill. The
site should be inaccessible to animals, removed from populated areas,
not used for agricultural purposes, clearly marked, and properly
protected.

October 1998


BSE Red Book 2.1-41

Burial sites should also be located a sufficient distance from
underground utility lines, septic systems, water wells, and surface
water. Local environmental or public works officers may be helpful in
locating a satisfactory site.
Field personnel should consult with their supervisors and/or the READEO
Environmental Impact Officer before digging. Burial trenches are
normally at least 9 feet deep with floor dimensions of 7 by 2 feet per
adult bovine carcass. Carcasses should be covered with at least 6 feet
of soil. This soil should not be tightly packed because gas formation
may cause a tightly packed trench to crack and leak.

7.7.3 Rendering
Because BSE is spread by rendered animal protein, BSE-suspect and
confirmed carcasses must not be rendered, unless the rendered material
is incinerated. Notify FDA, CVM if you suspect that dead BSE animals or
carcasses have moved to rendering or animal feed manufacturing.

7.7.4 Other Disposal Methods
The AVIC, the State animal health officials, and the READEO Director may
recommend other methods of disposal to the Deputy Administer, VS, for
approval (9 CFR 53.4). Options for disposal must be discussed and
approved by VS, Emergency Programs staff and must comply with all State
and local Environmental Protection Agency regulations.

7.8 Cleaning and Disinfecting (C&D)

Although BSE is neither contagious nor vector borne, appropriate C&D is
required to prevent farm-to-farm transmission of most other infectious
diseases. Field personnel must remember, however, that at the time they
are requested to euthanize a BSE-suspect animal, a confirmed diagnosis
of BSE will not be available. Signs compatible with BSE may be caused by
numerous infectious diseases and many BSE-suspect animals will, in fact,
have some other disease. Although the C&D of items such as manure,
bedding, feed, stalls, halters, milking machines, and other supplies and
equipment that have been in contact with BSE suspects is not
specifically necessary to control BSE, C&D is still advisable to control
other diseases that may be present.

7.8.1 Procedures for Cleaning and Disinfecting
7.8.1.1 Premises and Items--Field personnel are not responsible for C&D
of premises such as barns, stalls, and animal pens unless invasive
diagnostic procedures (such as a necropsy examination or the removal of
the suspect animal's brain) were performed on the premises. If possible,
field personnel should avoid doing such procedures on the farm. If
circumstances require that such procedures must be done on the farm,
personnel should clean and disinfect the immediate area after completing
the work.
7.8.1.2 Vehicles--Vehicles used to transport personnel to affected
premises should be kept clean, and normal precautions against the
farm-to-farm spread of any disease should be observed.

October 1998

BSE Red Book 2.1-42

7,8.1.3 Carriers--Thoroughly clean trucks and trailers transporting BSE
suspects. Manure and bedding may be disposed of by any environmentally
accepted method such as spreading on fields or composting. After
conveyances have been thoroughly cleaned, disinfectant should be sprayed
on the sides and floor of the truck bed.
7.8.1.4 Livestock Markets--The risk of BSE transmission at livestock
markets is negligible. If a BSE suspect is found at a livestock market,
it should be managed the same as if it were found at a farm. Because of
the high risk of transmission of diseases other than BSE, invasive
diagnostic procedures, such as a necropsy examination or removal of the
suspect animal's head, should not be performed at livestock markets. Due
to the recent research findings concerning maternal transmission, any
pens or areas in which calving occurs should be thoroughly cleaned and
disinfected.
Cleaning and disinfection is not necessary to prevent the spread of BSE.
However, the C&D procedures are recommended to prevent the spread of
other diseases from pens or buildings where BSE suspects were held.
7.8.1.5 Slaughter Plants--Becanse BSE is spread by rendered animal
protein in cattle feeds, BSE suspects must not be slaughtered nor
rendered. If a BSE suspect is found at a slaughter plant, it should be
managed similarly to finding a suspect at a farm.

7.8.2 Approved Disinfectants
Field personnel should use professional judgment in the choice of a
disinfectant. Preferred disinfectants to inactivate the BSE agent
include 1N sodium hydroxide solution or sodium hypochlorite solution
containing 2 percent chlorine (1 hour exposure at 20 %C [68 %F]). This
should be used whenever there is reason to strongly suspect that BSE is
in fact the cause of the suspect animal's disease. Such reasons include
previously confirmed BSE in the geographic area or signs more compatible
with BSE than with any other neurologic disease.
If the suspect animal's signs are more compatible with diseases such as
rabies or listeriosis, then a phenolic disinfectant such as "One Stroke"
may be preferable. (Refer to appendix A Survival of BSE Agent and sec.
1.4.4.)

7. 8. 3 Precautions
All disinfectants are hazardous to human beings, animals, and the
environment. Label directions should be carefully read and followed.
Many disinfectants, including sodium hypochlorite solution, are also
corrosive and should be used with caution on metal and other corrodible
materials. Thorough rinsing is necessary if corrosive disinfectants are
used on metallic items.
Disinfectants, especially in concentrated form, may irritate skin, eyes,
and respiratory systems. Protective equipment such as appropriate
clothing, rubber boots, rubber gloves, mask and goggles should be worn
during mixing and application of disinfectants. If areas of the body are
exposed to a disinfectant, they should be washed thoroughly with water.
Employees should notify their supervisor and their Health and Safety
Officer if excessive human or animal exposure to disinfectants occurs or
if there is accidental release into the environment.

October l998

BSE Red Book 2.1-43

Field personnel should use normal hygienic procedures (such as washing
and disinfecting boots and removing the outer layer of clothing) when
leaving the farm. Unless the disease problem is noncontagious, personnel
should not travel to other livestock premises for the duration of that
day.

7.9 Vector Control

Current scientific data indicate that BSE is not spread by vectors.

7.10 Disease Prevention and Philosophy

The goal of disease prevention and control is to confine the occurrence
of BSE to as few herds as possible and to prevent recycling of the BSE
agent in the ruminant food supply. If undiagnosed cases are rendered and
included in ruminant rations, the long incubation period may allow many
animals to be exposed.
Action should be taken immediately after the detection and confirmation
of BSE to initiate an extensive epidemiologic investigation to determine
the source and extent of the disease, to stop the spread, and to
eradicate the disease.
7.10.1 Philosophy--Immediate action should be taken to prevent
contamination of the animal food supply by prohibiting rendering of any
infected or suspect bovine carcasses. In addition, care should be taken
to monitor those animals born and raised in affected herds and to
prevent their becoming a source of infection to other herds.
7.10.2 Agent Spread--Epidemiologic evidence indicates that the primary
route of BSE transmission is through the feeding of contaminated meat
and bone meal that has been manufactured using scrapie infected sheep
carcasses or BSE infected bovine carcasses. Recent research findings
suggest that maternal transmission may occur at a rate of approximately
1 percent in some species. It is believed that this route of
transmission is not significant enough to maintain an epidemic. Cases of
apparent maternal transmission have also been identified in captive
exotic ruminants.
7.10.3 Control of Products and Conveyances--Carcasses of BSE suspects
should be incinerated. Carcasses must not be rendered and incorporated
in animal feed. If carcasses are transported for disposal, conveyances
should be cleaned and disinfected after use with either a sodium
hypochlorite solution (2 percent available chlorine) or 1 N lye (sodium
hydroxide solution).
7.10.4 Control of Biologics and Drugs--Although no documented cases of
BSE have resulted from the use of biologics derived from bovines,
tissues from suspect or exposed animals must not be used for the
production of biologics and drugs.
The agents responsible for causing the transmissible spongiform
encephalopathies are highly resistant to normal inactivation processes.
Careful selection of source materials is the best way to secure maximum
safety of ingredients or reagents of bovine origin used in the
manufacture of biologics or other medicinals. Factors that should be
considered are the age of the animals, exposure to the agent, and the
tissue or organ from which the product is derived.

October 1998

BSE Red Book 2.1-44

7.10.5 Wild Birds, Wind and Insects---Wild birds, wind and insects are
not known factors in the spread of BSE.
7.10.6 Rodents--Rodents are not known factors in the spread of BSE.
7.10.7 Hunting--Restrictions on the hunting of wild animals are not
necessary to prevent BSE.
7.10.8 Exhibitions--Cancelling scheduled exhibitions is not necessary.
7.10.9 Rendering Trucks and Drivers--The carcasses from BSE suspects
must not be rendered. If any rendering truck is used to transport a
suspect, it should be cleaned, washed, and disinfected as above. (Refer
to appendix A--Agent Survival and sec. 7.8.2--Disinfectants.)
7.10.10 Treatment--Currently there is no known treatment for BSE.
7.10.11 Prevention--Suspects and animals confirmed to have BSE must not
be rendered. Producers, feed mills, and rendering establishments should
adhere to U.S. State and local rendering policies and FDA regulations
concerning the feeding of rendered animal protein to ruminants. Because
of the possibility that some transmissible spongiform encephalopathies
may be transmitted at the time of parturition, precautions should be
taken to prevent exposure of healthy animal to placenta and reproductive
fluids. Importation of live animals and animal products from countries
with BSE or having high risk factors for BSE should be restricted based
upon scientific risk assessment.
7.10.11.1 Immunization--The agent that causes BSE elicits no detectable
immune response in the host. Therefore, vaccination is not a viable
option. There is no vaccine currently developed for BSE or other TSE's.
7.10.11.2 Sanitation--Although it is unknown whether a contaminated
environment plays any role in the spread of BSE, it is suggested that
pens having contained BSE-infected animals be cleaned and disinfected.
The disinfectants o choice are sodium hydroxide (lye) and sodium
hypochlorite, in infected herds it is also advisable that all placentas
be removed promptly and buried or incinerated. The calving pens also
should be cleaned and disinfected.
7.10.113 Producer Defense---The most effective way to prevent an
intruduction of BSE into a herd is not to feed ruminant byproducts to
ruminants. As of August 4, 1997, the FDA has a ban in place which
prohibits the feeding of most mammlian proteins to ruminants.

7.11 Records Maintenance in a Foreign Animal Disease Outbreak

The APHIS FEDS will be used by the READEO to record information. FEDS a
computerized network designed to transmit accurate information rapidly
during any emergency disease outbreak. The use of FEDS will allow the
READEO to direct its attention to the minute-to-minute business of
containing and eradicating the disease.
For an accurate record of the activities, all field supervisors in a
READEO task force should maintain a diary. Activities and observations
should be recorded in the diary when they occur. Date all documents and
enter events by time and date to show a correct chronology.
Enter events as they occur in the diary as well. An accurate history is
of considerable value in developing policies and plans for future
disease-eradication

October 1998

BSE Red book 2.1-45

programs, and it may be important if there is litigation. A diary will
be helpful for day-to-day administration of funds, personnel, and
equipment. It is also useful as a later reference in preparing reports
and summaries of activities.

7.11.1 Daily Reporls
Submit daily reports of significant activities to the READEO Director
and the VS, Emergency Programs staff Riverdale, MD. (Refer to appendix F
for current telephone listings.)
Include the following as part of the historical file of an outbreak:

*Maps showing premises where BSE-infected animals were found;
*Inventory of feeds and feed sources;
*Origin of BSE-suspeet and confirmed animals;
*Public information material distributed, newspaper clippings; and,
Administrative reports to support the expenditure of funds, utilization
of personnel and equipment, and disposition of excess materials and
equip­ment at the end of the program.

7.11.2 Distribution
The VS, Emergency Programs staff will distribute reports of significant
activities to all AVIC's, State cooperators, and industry cooperators at
least weekly. As soon as significant events occur, Emergency Programs
will inform all APHIS


will inform all APHIS headquarters units through normal reporting
channels. Emergency Programs also will immediately report any
significant events to the Deputy Administrator, VS, who will immediately
advise the APHIS Administrator, especially of legal or politically
important events. A weekly summary report of control and eradication
activities will be provided to the APHIS Administrator and the Deputy
Administrator, VS. See BSE Response Plan, communications section.

7.11.3 Disposition
Records should be maintained until a historical account of the program
has been prepared and all pertinent information has been gleaned from
the records.
Furthermore, all records should be maintained if there may be legal
action pending as a result of the program activities. Usually,
administrative records are maintained a minimum of 3 years for audit
purposes.


THE END. ...TSS


Approved-By: [email protected].
References:
Content-Type: text/plain; charset=us-ascii
Message-ID: <[email protected]>
Date: Thu, 6 May 1999 08:07:19 -0500
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: Re: hunkering down in the APHIS BSE Situation Room



Dr. Pringle or Anyone, why is it, in the U.S.'s B.S.E. Response Plan, the U.S.D.A. refers to it as a FOREIGN ANIMAL DISEASE. With the feeding and rendering practices of the U.S. over the years, scrapie in the U.S. for years, (it was proven in the defense of Oprah Winfrey trial that neurologically ill cattle went to the renderers showed pictures of sheep heads in 55 gallon drums, along with all kind of road-kill at the rendering plants.)
Why do they call this a Foreign Disease, or a U.K. disease???
Under the circumstances, could it not happen here, because of OUR OWN STUPIDITY???
I also find it odd, that the letters, of the announcement of the first case of BSE, are already drafted and ready to go, for a disease, they say, can't happen here, because of the EXTENSIVE B.S.E. program that has been in place for years.
Under the present circumstances, out of the 900 MILLION cattle raised since 1990, and the examination of ONLY 7,535 brains since 1990, It would have to be a FREAK ACCIDENT, for the U.S. to ever find a case of B.S.E.
I believe, this could be a WORLD PROBLEM, AND COULD HAPPEN ANYWHERE, where the feeding and rendering practices were that of the U.K. or U.S.//////

Terry S. Singeltary Sr.
Bacliff, Texas, U.S.A.

tom wrote:

> i am looking now a bizarre Oct 98 internal USDA publication describing a james bond-type US effort to control media


=======================================================


Subject: hunkering down in the APHIS BSE Situation Room
Date: Wed, 12 May 1999 01:55:54 -0800
From: tom
Reply-To: Bovine Spongiform Encephalopathy
To: [email protected]

i am looking now a bizarre Oct 98 internal USDA publication describing
a james bond-type US effort to control media should the long-anticipated
first case of BSE in the US be admitted.

'Players' on the 27 member BSE Response Team are to be flown in from all
over the country to a BSE Headquarters 'situation room' apparently an
underground bunker in Riverdale, Maryland under the command of the
Assistant Secretary of Marketing.

Authentic press releases are already prepared and ready to go out after a
few specifics have been filled in. They are spelled out in a separate
document, the BSE Red Book, aka BSE Emergency Disease Guidelines.

Aphis' National Veterinary Services Laboratories (NVSL) activates team
assembly. From the time a bovine brain sample is submitted, it takes
14-18 days to confirm a diagnosis of BSE. In the first 10-13 days, NVSL
have enough information to determine the need for additional tests. If a
provisional BSE diagnosis is made, the sample is 'hand-carried' (are
they going to tell the airline and customs?) to the Central Veterinary
Laboratory in England for confirmation, where they are expecting a 24 to
96 hour turn-around.

I guess that means we can get the white tiger brain analyzed by Friday
despite the 22 year delay to date. Maybe we could throw in a few cougar
brains from NE Colorado too.

A Team Member is designated to silently monitor this listserve and
www.mad-cow.org (among others) -- for what, it doesn't say. The
Freedom of Information Act request from the East Coast consumer group
turned up numerous top-secret USDA downloads from that site and
Dealler's.

After 24 hours of secret briefings for 'select industry and trading
partners' (to allow them to take positions on the commodities markets
opposite the 'non-select' industry and trading partners?), a press
conference will be held the next day.

There are plans to trace the cow, its lineage, its herdmates, the
renderer, traceout of product, buyout of herd, farm of origin, to get the
state involved to quarantine the herd (pre-arranged for all 50 states),
expectations for trade bans, notification of OIE within 24 hours, media
800 numbers, spokespersons and backups, notify CDC, FDA, NIH, and many
other commendable activities. The Flow Chart is a sight to behold, I
will try to scan it in tomorrow.

In short, that cow is going to be toast by the time the public first
hears about it.

The Plan does not speak to the scenario in which the CVL says, yes, this
is bovine spongiform encephalopathy all right but it is one of your
strains, not ours. Invoking their Absence of Evidence is Evidence of
Absence principle, there may be no perceived need for public disclosure
in this case.

USDA is caught completely unprepared if BSE first turns up in a US zoo
animal. These animals could easily be diagnosed outside the "system"
and be the subject of a publicity-seeking lab press release. I think
this is a more likely scenario because the US has likely imported many
thousands of zoo animals with advanced infections from Britain and
France and there has been zero monitoring. Unlike with downer cows,
anyone with the right colleagues can get ahold of a fallen zoo animal.
Zoo animals enter the food chain in some cases after being rendered.

Another scenario would be some stock market speculator obtaining the Red
Book and issuing a flurry of bogus but authentic-looking press releases
that included bogus 800 and hacked USDA web links. The press here is so
lazy and so accustomed to putting out public relation handouts as news
that the objectives would be accomplished for a few hour (or days,
depending on the Response Team's paralysis vis-a-vis off-flow chart
events). Some people think a practise run for this happened in the
Indiana case a year or two back.

The first case of nvCJD in an American will also be a public relations
fiasco. In the dim bulb of the public mind, any American with mad cow
disease would have gotten it from eating meat here. USDA has no way to
prove that the victim acquired it on a three week trip to England in
1987. This will sound lame even to the press. All CJD is synonymous with
mad cow disease in the public perception; the more often the different
kinds are explained, the more their suspicions are aroused. The first
case of nvCJD in an American will simply validate what they already know
and just be viewed as an overdue admission from the government.

tom


=============================================



Content-Type: text/plain; charset=us-ascii
Approved-By: Nora E Wineland
Message-ID: <"990528151900Z.WT26810.
14*/PN=Nora.E.Wineland/OU=APHISNOTES/O=APHIS/PRMD=GOV+USDA/ADMD=ATTMAIL/C=US/"@MHS>
Date: Fri, 28 May 1999 09:05:10 -0600
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: Nora E Wineland
Subject: More on the USDA:APHIS BSE Response plan


I would like to add some more information from APHIS in response to some recent postings to the list:

Tom Pringle suggests that the 27 member team he labels as the "BSE swat team" be directed at TSE science and education. I would like to clarify that of the APHIS employees designated to travel to our headquarters and assist with the communication aspect of a BSE outbreak only two of us are involved with the TSEs on a nearly full time basis. The others are those in APHIS which have experience working with scrapie, CWD or BSE prevention and surveillance policy. They work either in the field, at headquarters, at the National Veterinary Services Laboratory (NVSL) or the Centers for Epidemiology and Animal Health (CEAH). Their duties include all of our other programs such as preventing the entry of other foreign animal diseases such as Foot and Mouth, Classical Swine Fever, etc., tuberculosis, brucellosis, pseudorabies (etc) eradication, regulating the importation of animals and animal products, facilitating the exportation of healthy animals and safe animal products, as well as the oversight of the veterinary accreditation program. Our webpage at www.aphis.usda.gov can provide you with more detailed information on the multitude of tasks and complexities involved with the APHIS mission.


Linda A. Detwiler
Senior Staff Veterinarian


======================================================================




Content-Type: text/plain; charset="us-ascii"
Approved-By: tom
Message-ID:
Date: Sun, 16 May 1999 21:21:34 -0800
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: tom
Subject: Re: hunkering down in the APHIS BSE Situation Room
In-Reply-To: <"990514144119Z.WT11141.



>"The updated BSE Response Plan has been posted on the APHIS website for months. It was also distributed to the contact list which includes other government agencies, industry and consumer groups. I would hardly call it an internal document. I will only address one of the many misinterpretations in Tom Pringle's posting: In the event of a BSE case, the person assigned to monitoring the APHIS BSE website will keep this site up to date on an almost hourly basis, there is no intention of monitoring the sites of others. If anyone has questions about the document I can be reached as follows:
>
>Linda A. Detwiler
>Senior Staff Veterinarian
>USDA, APHIS, Veterinary Services
>609-259-5825"
>

Yes, I had four questions:

1. Could you please post on this listserve or the APHIS site the contact list of industry and consumer groups? My concern is that these are not bona fide consumer groups but simply industry-funded shells. It is important as a tax-supported public agency for USDA to promote a level informational playing field.

2. How do I go about getting my name added to this contact list to receive future messages?

3. Could you please post here a list of the "select industry and trading partners" that get the one-day advance warning that mad cow disease has been confirmed in the US? There are many stakeholders in this issue including public health and consumer interests -- I am hoping this list will demonstrate balance. Please add my stockbroker to this list.

4. Could you please post here copies of the press releases that have been made up in advance of this hypothetical event? The facts are not in, how it is possible to issue reassurances to the consumer already? Maybe the actual event won't be all that reassuring.

Thanks,
Tom


===================================================


END TSS/2006
 
> In the event of a BSE case, the person assigned to monitoring the

> APHIS BSE website will keep this site up to date on an almost hourly

> basis...



:lol: :lol: :lol: what a hoot :lol: :lol: :lol:


Dr. Detwiler would have kept it up, and or kept us informed somehow, that's why she is gone. these bozo's they have in there now though, are too busy still trying to cover BSE up, and they cannot seem to even do that right. ...TSS
 
Subject: Mad cow madness
Date: April 5, 2006 at 6:36 pm PST

Mad cow madness

When the first American case of mad cow disease was detected in December 2003, the Bush administration swung into action. It quickly announced plans for an "enhanced" testing program, talked about creating a national tracking system for beef cattle and acknowledged the need to revise rules governing animal feed. A second mad cow case was reported last year. Federal officials have confirmed a third case of bovine spongiform encephalopathy.
So where do those plans stand now?

The tracking system isn't yet in place; disease investigators don't know much about the infected animal's background, except that it lived in Alabama for the last year or so. The new food regulations aren't yet final, but what's been proposed contains dangerous loopholes. An inspector general's report released in February found that animals at high risk of infection, including some "downer cows," weren't being screened. And the enhanced testing? That's already begun winding down.

We're not trying to create panic. The odds that someone will contract the human variant of mad cow disease from eating American beef today remain remote. But the U.S. Department of Agriculture is one of several federal agencies charged with both policing and promoting an industry, and it seems as if the promoting part is trumping the policing part. That is a formula for disaster. Sadly, however, it would fit a pattern. Since the very first U.S. case of mad cow, federal officials have worked hard to persuade consumers there was no food safety problem. That became a hard sell after reports that federal regulators couldn't find many of the animals raised with the infected cow.

The second case was even more troubling. Two preliminary tests found that the animal was infected. But when a confirmatory test was inconclusive, USDA inspectors dropped the matter without trying to resolve it. Only later, when pressed to do a second confirmation test, did they recognize their mistake.

In February, the USDA's own inspector general found "a lack of quality assurance controls over (the) testing program," meaning there's no way to know if tests were conducted properly and high-risk animals were appropriately screened. Now, we discover that the enhanced testing program will be phased out, just when it should be expanded.

At its height, enhanced testing was screening less than one percent of American cattle. That might be enough if it were a representative sample. But without a tracking system, we can't know that. In any case, the testing was voluntary.

Mad cow disease is a storm that experts have seen coming for a decade. It swept through Europe, nearly destroying the British beef industry, and spread into Asia. The time to prepare an American response was before it hit.

Instead, the Bush administration has shown few signs of urgency and much wishful thinking.

Raising cattle is big business, and we'd hate to see that industry harmed. But that's exactly what will happen unless the USDA gets on the ball.

It should expand testing, get that tracking system up and running and close loopholes that allow cow blood to be fed to cattle as a "protein substitute." Only then can we be confident that our food supply will be safe until the storm blows over.

Reprinted from the St. Louis Post-Dispatch


http://www.fortmorgantimes.com/Stories/0,1413,164~8309~3283756,00.html



TSS
 
April 5, 2006 - Sparks Asks for Executive Amendment to HB 254
MONTGOMERY – Agriculture & Industries Commissioner Ron Sparks has asked Governor Bob Riley to attach an Executive Amendment to House Bill 254 that would add clarifying language regarding participation in the Confidential Animal Identification program. The Executive Amendment requested by Sparks would make it clear that participation in the program is currently "voluntary" in Alabama and would remain voluntary until federal law or regulation requires mandatory participation.


"I became concerned about clarifying this part of the bill after my office received many calls from concerned farmers who had been confused by the misinformation put out prior to the bill being passed," said Sparks. "I hope that adding this language will ensure that everyone can easily tell that this bill does not implement a mandatory animal identification program in Alabama and that the program would not be mandatory until it is required by federal regulations. It only means that the information collected in the program will remain confidential to protect the privacy of those who participate. I want to thank Representative Blaine Galliher and Senator Zeb Little for introducing this bill and Governor Riley for working with us to clarify the language in it with an Executive Amendment."


http://www.agi.state.al.us/press_releases/april-05-2006---sparks-asks-for-executive-amendment-to-hb-254?pn=1

http://www.agi.state.al.us/press_releases/april-05-2006---sparks-asks-for-executive-amendment-to-hb-254?pn=2


TSS
 
Subject: BSE ALABAMA UPDATE Apr 6, 2006
Date: April 7, 2006 at 6:57 am PST
Apr 6, 2006 — As of today, 14 locations and 45 movements of cattle have been examined with 40 of those being substantially completed. Additional investigations of locations and herds will continue. A location includes stockyards or farms where the index cow lived previously or where her immediate family members may have lived. The movements include any arrivals or departures from those locations.


http://www.aphis.usda.gov/newsroom/hot_issues/bse/bse_al_epi-update.shtml


HOW OLD IS THIS COW?


TSS
 

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