US "Atypical" Mad Cow Threat Was Predicted

Submitted by John Stauber on

The small scientific world of prion researchers -- the scientists who investigate "transmissible spongiform encephalopathies" (TSE) such as mad cow disease in cattle and Creutzfeldt-Jakob Disease (CJD) in humans -- is abuzz. That's because the two confirmed cases of US mad cow disease in Texas and Alabama are an "atypical" strain different from the British strain but identical to an atypical strain found so far in a small number of cattle in France, Germany, Poland and Sweden. The discovery of "atypical" mad cow disease in the US should not be surprising. Sheldon Rampton and I reported way back in 1997 that very strong evidence of an "atypical" TSE disease infecting US cattle was established by the work of Dr. Richard Marsh, the researcher to whom we dedicated our book Mad Cow USA.

Even before Britain confirmed its first case of mad cow, Dr. Marsh of the University of Wisconsin was investigating a similar disease in Stetsonville, Wisconsin, a 1985 outbreak in mink that he traced to Wisconsin dairy cattle. Marsh's published research confirmed suspicions among US scientists since the mid 1960s that the rare but deadly TSE disease in US mink -- transmissible mink encephalopathy or TME -- resulted from their having eaten TSE-infected US cattle.

Did the U.S. Infect Europe with Atypical Mad Cow Disease?

The discovery that the Texas and Alabama BSE cases are a variant strain identical to EU cases begs the question of whether the atypical EU cases resulted from European cattle being fed infected US feed made from rendered by-products and sold in Europe. After all, the US has been the biggest creator, user and exporter of by-product feed made from slaughterhouse waste. Also, scientists need to examine the TSE isolated by Richard Marsh in mink and traced to Wisconsin cattle, and compare it to the atypical BSE strain found in Texas, Alabama, France, Poland, Germany and Sweden. Is the Stetsonville TSE strain discovered by Richard Marsh the same strain as the US and EU atypical BSE cases, or is it another atypical strain?

Here below is our report on Marsh's discovery in 1985 of an atypical strain of BSE in US cattle from our 1997 book Mad Cow USA. (You can order the book for free from your favorite library and it is for sale in the usual places.)

Mad Cow USA

(The following excerpt is from Mad Cow USA, by Sheldon Rampton and John Stauber, pages 154-156:)

The common denominator in all of these [transmissible mink encephalopathy - TME] outbreaks was either "cattle" or "unknown." It was possible, of course, to imagine other scenarios, but Marsh believed he had at least strong circumstantial evidence that a TSE similar to mad cow disease already existed in U.S. cattle. "You can trace it back to feed real easy in mink," Marsh said. "And then you're left with the question, what was it in the feed that affected them? And what we find is it's these downer cows that are the common link. You don't have to be a genius to figure it out."

Within the field of veterinary medicine, "downer cow syndrome" was a "garbage can" category, used indiscriminately as the official diagnosis for any animal that died or had to be put down after failing to stand on its own legs for 24 hours or more. These included cows suffering from paralysis, arthritis, grass tetany, ketosis, bone fractures, and a form of calcium deficiency known as "milk fever." Most downer cows died from causes unrelated to the spongiform encephalopathies, but it was possible that the generic nature of the classification enabled some TSE-infected cows to slip into the mix.

It was impossible in practice to absolutely prove the link between downer cows and transmissible mink encephalopathy. By the time the disease appeared in mink, any cow that might have been the source would be long gone, its tissues unavailable for testing. To test his theory, therefore, Marsh did the next best thing—a series of experiments using brain matter from one of the mink that had died in the Stetsonville outbreak. He puréed the brain in a blender and used hypodermic syringes to inject the homogenized liquid into test animals: fourteen healthy mink, eight ferrets, two squirrel monkeys, twelve hamsters, forty-five mice and two Holstein bull calves.

The mice, remarkably, all stayed healthy, but every other species proved susceptible. The mink went down first, four months after inoculation. The two monkeys were the next to show neurological signs, at months nine and thirteen respectively. Two of the twelve hamsters survived, but the other ten succumbed in the fifteenth and sixteenth months. The two calves went down in months eighteen and nineteen. The ferrets lasted longest, but eventually the disease emerged in all but one of them, with incubation periods ranging between twenty-eight and thirty-eight months. These species barrier effects corresponded closely to the results from experiments with previous mink outbreaks.

Cattle are expensive test animals, and Marsh's experiments marked the first time that cattle had been tested for susceptibility to transmissible mink encephalopathy. His results proved that cattle could get the mink disease, and in turn led to unexpected new questions. "The real surprise of this experiment is that the clinical signs were quite different from what we've seen in Great Britain," he said. "This is what's changed our perspective on a surveillance of BSE in the United States. We thought BSE in the U.S. would look like BSE in Great Britain—a mad cow type of disease where the animal would have behavioral changes, become aggressive and look very much like a rabies infection does in cows."

Marsh's bull calves showed none of the unusual "mad" behavior that emerged as early warning signs in British cattle. "Eighteen months after inoculation, one animal simply collapsed in its holding room and could not be returned to a standing position," he reported. "This animal had shown no previous signs of behavioral change or loss of body condition. . . . The second animal was normal until nineteen months after inoculation when it too suddenly collapsed."

Indeed, the test bulls behaved exactly like downer cows—the type of animals which the Stetsonville rancher had been feeding to his mink. "The most disturbing finding of all is that they have very minimal spongiform lesions in their brains," Marsh said. In previous experiments with mink, he had shown that the spongy holes in brains were a secondary effect of the disease which did not always appear in noticeable quantities. Some mink breeds infected with TME would develop all of the usual clinical symptoms, but upon autopsy their brains showed a marked lack of spongiform degeneration. Now it appeared that cattle could also develop a form of TSE without the telltale lesions to aid in diagnosis. Their symptoms would look like downer cow syndrome, and even a brain autopsy might find nothing out of the ordinary.

"Without the brain lesions, the best way to diagnose the infection is a protein in the brain," Marsh said. "But there are only a few labs in the country that can look for this protein. This is not something that can be done by the local veterinarian or even most state diagnostic laboratories. You need to have pretty sophisticated means of testing. This is going to complicate our efforts at surveillance and testing for BSE in this country."

Histopathology and immunohistochemistry tests confirmed that Marsh's bulls had died of a spongiform encephalopathy, but it was a different strain of spongiform encephalopathy than the one that was killing cows in England. Its behavior in test animals showed significant differences also. In England, mice succumbed when exposed to brain tissue from mad cows, but hamsters seemed immune. In Marsh's experiments with the Stetsonville isolate of TME, the pattern was exactly the opposite: mice lived, but hamsters died.

From Mad Cows to Mad Mink and Back

To test whether passage through cattle altered the characteristics of the Stetsonville isolate, Marsh injected another 45 mice with brain tissue from his two test bulls. They also stayed healthy, just like the mice he had previously injected with mink brains. By itself, the fact that mink encephalopathy could infect cows was not terribly significant or surprising. After all, scientists had previously shown that TME could be transmitted to a wide variety of other test animals. What was significant was the result when Marsh took the brains of the dead bulls and used them on further tests with healthy mink. When backpassaged into mink, the bull brains behaved exactly the way mink brains behaved, causing symptoms of TME to emerge within four months after exposure by inoculation, or within seven months after oral exposure.

"There was no evidence for any deadaptation of the bovine agent for mink compared to . . . non-bovine-passaged mink brain," Marsh observed. "This suggests that there are no species barrier effects between mink and cattle in relation to the Stetsonville source of TME" — more evidence pointing to cattle as the source of the infection. "If mink on the Stetsonville ranch were exposed to TME by feeding them infected cattle, there must be an unrecognized scrapie-like disease of cattle in the United States," Marsh concluded. "If this is true, the disease is rare. The low incidence rate of TME and the fact that the Stetsonville mink rancher had fed products from fallen or sick cattle to his animals for the past 35 years suggests a very low prevalence of this disease."

The rarity of the disease, however, did not mean that it posed no danger. In fact, it could mean the very opposite. Mad cow disease had also been rare once in England. The very fact that it was rare, combined with its slow incubation period, were the factors that prevented the British from recognizing its dangers until it had already infected tens of thousands of animals. Moreover, the British had an advantage that U.S. farmers might not enjoy. Their strain of bovine spongiform encephalopathy was picked up fairly soon once cattle started behaving strangely. If a different strain of BSE existed in U.S. cattle— a strain where the animals didn't act deranged but simply fell over, like the cows in Marsh's tests—the disease could conceivably go unrecognized for a long time, invisible within the larger population of U.S. downer cows.

Every year, some 100,000 U.S. cows get classified as downers. Marsh was not suggesting that all 100,000 were carriers of a spongiform encephalopathy. What concerned him was the possibility that downer cow syndrome could mask the emergence of a TSE in the cattle population, allowing the disease to invisibly spread until it reached dangerous levels. It could multiply the same way it had multiplied in England, as rendering plants recycled the infection by converting sick animals into meat and bone meal which was then fed back to other cattle. The only certain way to prevent a cattle epidemic, therefore, would be to adopt the same policy that the British had already been forced to adopt: ban the practice of feeding rendered cows and other ruminant animals back to members of their own species.

### (End of excerpt)

How to Hide a Mad Cow

Today the ability to test cattle for mad cow disease has greatly advanced, and so-called rapid tests are used on all cattle before they are allowed into the human food chain in Japan, for example. I describe the situation in the United States as a cover-up of the extent of mad cow disease because the US needs to test millions of cattle a year, and in a transparent and verifiable way, before we can know with accuracy how much disease is present in the US herd. Currently the US is testing less than 1% of its cattle a year, and the procedures are shrouded in secrecy. The US forbids anyone but the government to conduct tests in the United States making it impossible for Americans to purchase meat that has been tested and found free of the disease.

In addition, despite the false PR assurances from government and the livestock industry, there is no "firewall feed ban" in the United States to completely stop the spread of mad cow disease. Today it is legal and widespread to feed US cattle on cattle fat contaminated with cattle protein, on cattle blood, and on poultry shit and litter contaminated with cattle protein. In addition, slaughterhouse waste from cattle is fed to pigs, and in turn the slaughterhouse waste from pigs is fed back to cattle.

We now know we have "atypical" mad cow disease in the US and even the USDA admits that it has probably been spreading for at least a decade through feeding cattle to cattle. Yet, the cannibal feeding practices continue and the US's mad cow testing program is a farce.

Dick Marsh died in 1997 before our book Mad Cow USA was published. He was a careful scientist who understood the precautionary principle and who worked tirelessly and was terribly and personally attacked for his prescient warnings that a unique strain of mad cow disease already existed in the US, and that unless the dangerous feeding practices of cow cannibalism were stopped, it would spread through cattle and threaten human health.

Perhaps if cancer had not silenced Dick Marsh a decade ago, his strong voice would have helped change the current dangerous policies of the United States Department of Agriculture (USDA) and the Food and Drug Administration (FDA). Currently these federal agencies are threatening animal and human safety in the US simply so the US government can protect and preserve the livestock industry's deadly but lucrative practice of animal cannibalism, turning slaughterhouse waste into cheap feed for cattle and other livestock.

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flounder replied on

hi john, indeed, usda et al should have listened to you, sheldon, and everybody else that have tried to tell them for eons about TSE in the USA. but instead, USDA et al goes into cover-up mode, which is why this agent has now mutated and spread to hell and back. in essence, the USA was worse than the UK about spreading the agent via exports. now, well, i think it is too late. lets compare ; IN CONFIDENCE Perceptions of unconventional slow virus disease of animals in the USA G A H Wells REPORT OF A VISIT TO THE USA APRIL-MAY 1989 john, check out pages 13 to 17 http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf Gerald Wells: Report of the Visit to USA, April-May 1989 snip... The general opinion of those present was that BSE, as an overt disease phenomenon, _could exist in the USA, but if it did, it was very rare. The need for improved and specific surveillance methods to detect it as recognised... snip... It is clear that USDA have little information and _no_ regulatory responsibility for rendering plants in the US... snip... 3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a _very low profile indeed_. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be _avoided_ in the US _at all costs_... snip... http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf To be published in the Proceedings of the Fourth International Scientific Congress in Fur Animal Production. Toronto, Canada, August 21-28, 1988 Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle R.F. Marsh* and G.R. Hartsough .Department of Veterinary Science, University of Wisconsin-Madison, Madison, Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092 ABSTRACT Epidemiologic investigation of a new incidence of transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin suggests that the disease may have resulted from feeding infected cattle to mink. This observation is supported by the transmission of a TME-like disease to experimentally inoculated cattle, and by the recent report of a new bovine spongiform encephalopathy in England. INTRODUCTION Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough and Burger who demonstrated that the disease was transmissible with a long incubation period, and that affected mink had a spongiform encephalopathy similar to that found in scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965). Because of the similarity between TME and scrapie, and the subsequent finding that the two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was concluded that TME most likely resulted from feeding mink scrapie-infecied sheep. The experimental transmission of sheep scrapie to mink (Hanson et al., 1971) confirmed the close association of TME and scrapie, but at the same time provided evidence that they may be different. Epidemiologic studies on previous incidences of TME indicated that the incubation periods in field cases were between six months and one year in length (Harxsough and Burger, 1965). Experimentally, scrapie could not be transmitted to mink in less than one year. To investigate the possibility that TME may be caused by a (particular strain of scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie agent, including their sheep or goat sources, were inoculated into a total of 61 mink. Only one mink developed a progressive neurologic disease after an incubation period of 22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent from an unidentified source. OBSERVATIONS AND RESULTS A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin reported that many of his mink were "acting funny", and some had died. At this time, we visited the farm and found that approximately 10% of all adult mink were showing typical signs of TME: insidious onset characterized by subtle behavioral changes, loss of normal habits of cleanliness, deposition of droppings throughout the pen rather than in a single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over their _backs like squirrels. These signs were followed by progressive deterioration of neurologic function beginning with locomoior incoordination, long periods of somnolence in which the affected mink would stand motionless with its head in the corner of the cage, complete debilitation, and death. Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed. Experimental Transmission. The clinical diagnosis of TME was confirmed by histopaihologic examination and by experimental transmission to mink after incubation periods of four months. To investigate the possible involvement of cattle in this disease cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally with a brain suspension from affected mink. Each developed a fatal spongiform encephalopathy after incubation periods of 18 and 19 months. DISCUSSION These findings suggest that TME may result from feeding mink infected cattle and we have alerted bovine practitioners that there may exist an as yet unrecognized scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). A new bovine spongiform encephalopathy has recently been reported in England (Wells et al., 1987), and investigators are presently studying its transmissibility and possible relationship to scrapie. Because this new bovine disease in England is characterized by behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be confused with rabies in the United Stales and not be diagnosed. Presently, brains from cattle in the United States which are suspected of rabies infection are only tested with anti-rabies virus antibody and are not examined histopathologically for lesions of spongiform encephalopathy. We are presently pursuing additional studies to further examine the possible involvement of cattle in the epidemiology of TME. One of these is the backpassage of our experimental bovine encephalopathy to mink. Because (here are as yet no agent- specific proteins or nucleic acids identified for these transmissible neuropathogens, one means of distinguishing them is by animal passage and selection of the biotype which grows best in a particular host. This procedure has been used to separate hamster- adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The intracerebral backpassage of the experimental bovine agent resulted in incubations of only four months indicating no de-adaptation of the Stetsonville agent for mink after bovine passage. Mink fed infected bovine brain remain normal after six months. It will be essential to demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic association is to be confirmed. ACKNOWLEDGEMENTS These studies were supported by the College of Agricultural and Life Sciences, University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United States Department of Agriculture. The authors also wish to acknowledge the help and encouragement of Robert Hanson who died during the course of these investigations. REFERENCES Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and natural transmission. J. Infec. Dis. 115:393-399. Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson, D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861. Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and clinical observations. 3. Infec. Dis. 115:387-392. Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the transmissible mink encephalopathy agent. 3. ViroL 3:176-180. Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460. Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle? Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary Medicine. University of Arizona, pp 20. Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M., Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 121:419-420. MARSH http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf WORLD ASSOCIATION FOR BUIATRICS Edinburgh 8 -12 July 1996 http://www.bseinquiry.gov.uk/files/mb/m09/tab04.pdf Transmission Studies of BSE in Sheep http://www.bseinquiry.gov.uk/files/mb/m09/tab01.pdf J. Comp. Path. 2006, Vol. 134, 63-69 Experimental Second Passage of Chronic Wasting Disease (CWDmule deer) Agent to Cattle A. N. Hamir, R. A. Kunkle, J. M. Miller, J. J. Greenlee and J. A. Richt Agricultural Research Service, United States Department of Agriculture, National Animal Disease Center, 2300 Dayton Avenue, P.O. Box 70, Ames, IA 50010, USA Summary To compare clinicopathological findings in first and second passage chronic wasting disease (CWDmule deer) in cattle, six calves were inoculated intracerebrally with brain tissue derived froma first-passageCWD-affected calf in an earlier experiment. Two uninoculated calves served as controls. The inoculated animals began to lose both appetite and weight 10-12 months later, and five subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months, all cattle had been subjected to euthanasia because of poor prognosis. None of the animals showed microscopical lesions of spongiform encephalopathy (SE) but PrPres was detected in their CNS tissues by immunohistochemistry (IHC) and rapid Western blot (WB) techniques. Thus, intracerebrally inoculated cattle not only amplified CWD PrPres from mule deer but also developed clinicalCNSsigns in the absence of SElesions.This situation has also been shown to occur in cattle inoculated with the scrapie agent. The study confirmed that the diagnostic techniques currently used for diagnosis of bovine spongiformencephalopathy (BSE) in theUS would detect CWDin cattle, should it occur naturally. Furthermore, it raised the possibility of distinguishing CWDfromBSE in cattle, due to the absence of neuropathological lesions and to a distinctive multifocal distribution of PrPres, as demonstrated by IHC which, in this study, appeared to be more sensitive than the WB technique. snip... Discussion CWD, like all other TSEs, is characterized by a long incubation period, which in deer is seldom less than 18 months (Williams and Young, 1992). In an experimental study of cattle inoculated intracerebrally with CWD from mule deer (first passage), amplification of PrPres was demonstrated in only five of 13 (38%) cattle, after incubation periods that ranged from 23 to 63 months (Hamir et al., 2001a, 2005a). In contrast, all inoculated cattle in the present study were positive for PrPres within 16.5 months. This increased attack rate with shorter incubation periods probably indicates adaptation of the CWDmule deer agent to a new host. It could also be argued that the inoculum used for the primary passage (Hamir et al., 2001a, 2005a) had a lower infectivity titre than that used for the second passage. However, the former successfully transmitted CWD to each of five white tailed deer within two years of intracerebral inoculation (Kunkle et al., Unpublished). In cervids, clinical CWD is characterized by emaciation, changes in behaviour, and excessive salivation (Williams and Young, 1992). Although the latter was not observed in the CWD inoculated cattle, all animals showed anorexia and considerable weight loss. Five cattle also showed intermittent neurological signs. Although none of these animals showed histopathological changes in the brain, all were shown to be positive for PrPres by the IHC and WB methods. The presence of isolated vacuoles in the red nucleus is regarded as an incidental finding in cattle (McGill and Wells, 1993). The uniform susceptibility, relatively short incubation, and absence of microscopical lesions in cattle given CWD brain material passaged once through cattle resembled findings in cattle inoculated intracerebrally with the scrapie agent (Cutlip et al., 1997). In that experiment, 100% of cattle died 14-18 months after inoculation with material from the first cattle-passage of a US strain of the scrapie agent; none showed microscopical lesions and all were positive for PrPres. In the present experiment, the possibility that the PrPres seen in tissue sections represented residual CWD material from the inoculum was ruled out because of the multifocal distribution of PrPres throughout the brain (excluding cerebellar folia) and cervical spinal cord of most of the affected animals. Had the PrPres represented residual inoculum, it would probably have been confined to the sites of deposition in the midbrain or cerebrum. Moreover, in studies on sheep scrapie, Hamir et al. (2002) showed that intracerebrally inoculated brain material containing PrPres was present for only a few days in sufficient quantity to be detectable immunohistochemically. The present work confirms previous observations that PrPres IHC labelling in cattle inoculated with the mule deer CWD agent is multifocal and glial cell-associated. This unusual pattern was first reported in descriptions of the primary CWD transmission to cattle (Hamir et al., 2001a, 2005a), and the study described here showed that it was maintained through the second passage in cattle. Further studies now in progress will determine whether this feature also characterizes CWD transmission to cattle fromother cervid species other than mule deer, namely, white tailed deer and elk. In this and an earlier study of CWD in cattle (Hamir et al., 2001a), IHC labelling differed from that seen in cattle with BSE or experimental transmissible mink encephalopathy (TME), both of which are associated with widespread diffuse labelling of grey matter neuropil, with labelled particles that are not obviously cell-associated except occasionally at neuronal cell membranes (Wells and Willsmith, 1995; Hamir et al., 2005a). The IHC pattern in bovine CWD also contrasts markedly with that seen in scrapie-inoculated cattle, in which intracytoplasmic labelling of neurons is a prominent feature (Cutlip et al., 1994, 1997). When brainstems of CWD-infected cattle were analysed by WB for the presence of PrPres, only three of six samples were found to be positive (Table 1). In contrast, all samples from the midbrain area were positive by this technique (Table 1; Fig. 5). It was noteworthy, however, that both brainstem and midbrain sections of all animals infected with CWD gave positive IHC results (Table 1) and a positive WB was associated with strong IHC labelling. This may indicate that the IHC procedure is more sensitive than the WB method for cattle-passaged CWD. However, given the multifocal nature of PrPres distribution in the CNS of CWD-infected cattle, this result is not surprising. WB analysis requires a small sample of brain tissue (e.g. 0.2 g, as in the present study) to produce a 10% homogenate; approximately 10 ml (1 mg brain tissue equivalent) of this homogenate are loaded on to an SDS-PAGE gel for further analysis. Bearing in mind the multifocal pattern of PrPres distribution, the brain tissue used for the preparation of WB homogenate, unlike the large amount examined in the IHC procedure, might well contain few if any foci of PrPres deposition, whereas the larger piece of tissue section used for IHC may contain detectable PrPres. In this respect, therefore, the IHC method would seem preferable to the WB procedure and to other procedures (e.g. ELISA-based tests) in which only small amounts of tissue are used for analysis. In comparison with experimental TME in cattle (Hamir et al., 2005b), the experimental bovine CWD in this study was associated with less extensive IHC labelling in non-CNS (i.e. other than brain and spinal cord) neural tissues. Whereas the retina was positive in all cattle inoculated with TME, none of the CWD-infected cattle in this experiment had any retinal labelling. Similarly, in the present study there was no labelling in the pituitary gland, a tissue sometimes positive in TME-infected cattle. Because the incubation time for second passage CWD transmission (mean of 468 days) was only slightly longer than for TME (mean of 430 days), it seems likely that these different tissue affinities reflect a biological difference between these two TSE agents. PrPres IHC labelling was not observed in striated muscles (heart, tongue, masseter, diaphragm) of the experimental animals. This observation accorded with our previous findings (Hamir et al., 2004a) in which striated muscle tissues from 20 animals (cattle, sheep, elk and raccoons) were examined for PrPres. In these animals, all of which had developed a TSE after experimental inoculation, PrPres was found by IHC examination in the brains, but not in muscle tissues. However, recent investigations with an enriched WB technique (Mulcahy et al., 2004) have enabled us to detect PrPres in the tongues of some sheep and elk experimentally inoculated with scrapie and CWD, respectively. This technique failed, however, to detect PrPres in cattle inoculated with CWD or TME (Bessen et al., unpublished). This study is still in progress, and the tongues of TSE-infected animals are currently being tested after careful removal from the carcasses to ensure non-contamination with infected brain material. The present study and a previous experiment (Hamir et al., 2005a) have established the biological characteristics of the CWDmule deer agent in cattle. However, isolates of CWD from other cervids (e.g. CWDwhite-tailed and CWDelk) may differ. Transmission experiments with different CWD isolates are therefore needed to examine the possibility of variation in the CWD agent in wild cervids. Such experiments have recently been initiated at the National Animal Disease Center (NADC).............snip...END...TSS ALSO, I THINK THE DOWNER COW FIGURE IS UNDERESTIMATED; Released May 5, 2005, by the National Agricultural Statistics Service (NASS), Agricultural Statistics Board, U.S. Department of Agriculture. For information on Non-ambulatory Cattle and Calves call Mike Miller at 720-3040, office hours 7:30 a.m. to 4:30 p.m. ET. Non-Ambulatory Cattle and Calves Non-ambulatory cattle and calves in the United States totaled 465,000 head during 2003 and 450,000 head during 2004. The number of non-ambulatory cattle 500 pounds or greater totaled 280,000 head in 2003 and 270,000 head in 2004. The number of calves under 500 pounds reported as non-ambulatory totaled 185,000 head in 2003 and 180,000 head in 2004. The number of operations that reported non-ambulatory cattle and calves was 103,000 in 2003 and 81,000 in 2004. In 2003, there were 66,800 beef cow operations reporting non-ambulatory cattle and calves compared to 49,700 in 2004. There were 22,800 dairy operations reporting nonambulatory cattle and calves in 2003 compared to 23,000 in 2004. This report is released as a cooperative effort between the National Agricultural Statistics Service and Animal and Plant Health Inspection Service - Veterinary Services. Data for this report were collected on the January 1, 2004 and 2005 Cattle Surveys. .......END....TSS From: TSS () Subject: Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) Date: October 19, 2005 at 8:33 am PST 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved. Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) Richard F. Marsh,1, Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4* Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska 68178,4 Department of Veterinary Molecular Biology, Montana State University, Bozeman, Montana 597183 Received 3 May 2005/ Accepted 10 August 2005 Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates. ---------------------------------------------------------------------------- ---- * Corresponding author. Mailing address: Department of Medical Microbiology and Immunology, Creighton University, 2500 California Plaza, Omaha, NE 68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail: jbartz@creighton.edu . Deceased. ---------------------------------------------------------------------------- ---- Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved. http://jvi.asm.org/cgi/content/abstract/79/21/13794?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=cwd&searchid=1129736446553_4280&stored_search=&FIRSTINDEX=0&volume=79&issue=21&journalcode=jvi =================================== Intra- & Inter-species Transmission of Chronic Wasting Disease (Show All REXs) Description: This research is intended to investigate the intra- and inter-species transmissibility of the causative agent of chronic wasting disease (CWD), believed to be a structurally modified form of the prion protein (PrPCWD), of white-tailed deer (Odocoileus virginianus). Our lab has identified five alleles in the PrP-coding region of white-tailed deer from the CWD-affected region of southern Wisconsin. Combinations of the alleles represent variability within the population and may result in differences in incubation period, levels of susceptibility, variable clinical symptoms and/or pathology within deer. We will test these ideas by inoculating white-tailed deer of known genotype with known-genotype PrPCWD and by conducting cell-free conversion experiments with the possible combinations of PrPCWD. We believe environment may be a reservoir of CWD, which opens possibilities of transmission to wildlife that share habitat with white-tailed deer. We will identify the species that consume deer carrion, as they are the most likely to encounter PrPCWD, collect 100 of each species from the CWD-affected region of southern Wisconsin and evaluate them for lesion profiles indicative of prion disease. We believe the primary carrion consumers will include coyote (Canis latrans), red fox (Vulpes vulpes), gray fox (Urocyon cinereoargenteus), raccoon (Procyon lotor), striped skunk (Mephitis mephitis), Virginia opossum (Didelphis virginiana), and mink (Mustela vison). Since species barriers are difficult to cross we dont expect to find a large prevalence of prion disease in this population of wildlife. To address the possibility of transmission to these species, we will inoculate raccoons, striped skunks, Virginia opossums and Eastern cottontails (Sylvilagus floridanus) with PrPCWD. We will test transmission to other species by cell-free conversion with as many species as possible, starting with those whose life history are most likely to expose them to PrPCWD. The species that we are collecting from the CWD-affected region of southern Wisconsin for prion disease assessment are a significant collection that we will use to survey a range of other wildlife diseases. This is a five-year project with publications anticipated in the third through fifth year. Students that would join me for work could experience lab or field work. We will be placing dead deer on the landscape, setting remote-triggered cameras on the deer and checking the cameras every other day. We will collect raccoons, skunks, opossums, coyotes and foxes from trappers and conduct necropsies on them at the WVDL. We will be running Western blots for TSE testing on the brains of the animals we necropsy. We will do PCR/Sequencing on the prion-coding region of each species, clone it into an expression vector and conduct cell-free conversions on the resulting protein. Date: Feb 20 Week: Week 1 (Week of Feb 20) Location (where students meet host): Room 237 Animal Health & Biomedical Sciences Building, 1656 Linden Drive Meets From: 3:30 pm until 5:30 pm Pre-REX Reading: None Laboratory: Lab Address: 237 Animal Health & Biomedical Sciences Bldg, 1656 Linden Drive Lab Phone: 262-7362 Lab Website: http://www.ahabs.wisc.edu/Faculty/Aiken-j/index.html Department or Institute: Animal Health & Biomedical Sciences College or School: School of Veterinary Medicine Host: Dr. Judd Aiken Host Email: jma@ahabs.wisc.edu Host Phone: 262-7362 Co-Host: Chad Johnson Co-Host Email: cjjohns3@students.wisc.edu Co-Host Phone: Total Number of Students Allowed: 5 Number of Openings: 0 ================================ USA EXPORTS 2006 http://www.ers.usda.gov/Briefing/Cattle/Data/AnnualLivestockTable.xls TOP FIVE COUNTRIES IMPORTING USDA MAD COW PRODUCTS The Economic Impact of BSE on the U.S. Beef Industry: Product Value Losses, Regulatory Costs, and Consumer Reactions 3.4 U.S. Beef Export Customers Table 3.4 provides a dollar value ranking, by country, of beef export shipments during 2003. Five countries, Japan, Mexico, South Korea, Canada, and Hong Kong, were the recipients of 90 percent of U.S .beef exports during 2003, based on value. Japan, historically the largest U.S. beef export customer, represented 35 percent of U.S. beef exports during 2003. http://www.ksda.gov/Default.aspx?tabid=349&mid=2252&ctl=Download&method=attachment&EntryId=479 WHOS EATING THOSE USDA MAD COW BRAINS OF AN ATYPICAL STRAIN ? 0206.29.0010: HEARTS OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 1,180,635 1,038 17,267,397 12,630 Angola 0 0 47,849 31 China 0 0 97,868 41 Colombia 0 0 355,787 379 Costa Rica 0 0 4,816 4 El Salvador 4,545 4 4,545 4 Greece 0 0 15,000 6 Guatemala 0 0 19,051 18 Honduras 0 0 9,780 8 Hong Kong 45,347 110 454,574 862 Indonesia 597,243 459 8,098,035 4,681 Ivory Coast 0 0 27,216 8 Japan 0 0 19,835 20 Korea, South 49,890 50 213,036 213 Lithuania 0 0 55,194 31 Mexico 280,421 234 2,664,118 2,384 Netherlands 0 0 108,698 61 Peru 0 0 452,116 458 Russia 203,189 181 4,528,474 3,280 Saudi Arabia 0 0 3,293 6 Singapore 0 0 44,906 21 Switzerland 0 0 8,010 8 United Arab Emirates 0 0 135 3 United Kingdom 0 0 35,061 105 0206.29.0020: KIDNEYS OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 330,004 231 3,566,918 1,818 China 49,424 26 141,576 64 Gabon 0 0 49,437 28 Greece 0 0 966 6 Indonesia 0 0 23,610 15 Ivory Coast 49,891 25 1,699,427 704 Jamaica 115,626 67 875,874 436 Mexico 115,063 113 521,638 465 Russia 0 0 115,377 70 Saudi Arabia 0 0 1,660 3 South Africa 0 0 111,960 18 Thailand 0 0 25,393 10 0206.29.0030: BRAINS OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 57,279 56 192,198 225 Ivory Coast 0 0 24,971 8 Mexico 57,279 56 161,158 211 Sweden 0 0 6,069 6 0206.29.0040: SWEATBREADS OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 48,735 69 581,306 689 Bahamas 0 0 4,551 5 Hong Kong 0 0 48,988 15 Japan 0 0 18,629 51 Mexico 48,735 69 507,453 611 Switzerland 0 0 1,685 6 0206.29.0050: LIPS OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 620,626 1,393 16,539,594 23,262 Hong Kong 0 0 23,587 8 Mexico 620,626 1,393 16,513,038 23,245 Taiwan 0 0 2,969 10 0206.29.0090: OFFAL OF BOVINE ANIMALS, EDIBLE, NESOI, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 8,464,956 16,117 118,262,413 197,532 Argentina 1,497 9 1,497 9 Australia 6,103 6 72,627 71 Bahamas 0 0 25,367 55 Barbados 0 0 1,282 6 Belgium 0 0 718,837 142 Bulgaria 0 0 328,698 261 Burkina 0 0 23,496 21 Canada 304,064 276 8,137,388 6,048 China 734,212 1,750 7,554,286 16,429 Colombia 0 0 109,398 141 Costa Rica 0 0 53,911 37 Denmark 0 0 8,327 33 Dominican Republic 19,578 168 112,192 767 Egypt 0 0 167,000 96 Federal Rep. of Germany 104,016 21 2,266,317 583 Gabon 24,494 16 339,168 177 Greece 23,610 26 47,220 51 Guatemala 0 0 200,509 233 Guyana 0 0 11,555 12 Hong Kong 339,453 704 4,490,896 7,651 Indonesia 104,013 108 1,231,976 666 Israel 0 0 119,230 121 Ivory Coast 0 0 1,429,316 876 Jamaica 79,203 73 780,910 696 Japan 2,614,703 7,006 29,370,030 78,245 Jordan 0 0 72,709 390 Korea, South 1,084,495 2,217 19,825,887 37,280 Macedonia (Skopje) 0 0 143,699 51 Malaysia 0 0 24,776 10 Mexico 2,463,516 2,922 30,710,290 37,936 Netherlands 0 0 38,512 65 Nicaragua 0 0 9,411 11 Panama 0 0 480,391 472 Peru 0 0 47,135 29 Philippines 37,875 15 216,218 116 Poland 47,175 36 954,552 532 Romania 0 0 991,737 765 Russia 368,385 325 3,490,349 2,441 Singapore 0 0 5,307 15 St Lucia 2,442 3 10,896 14 Sweden 0 0 46,200 45 Taiwan 106,122 436 1,601,333 3,327 Turks and Caicos Islands 0 0 8,536 14 United Arab Emirates 0 0 27,439 130 United Kingdom 0 0 1,842,710 369 Uruguay 0 0 112,893 95 Top of page http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-December/Exports/02/020629.html 0206.21.0000: TONGUES OF BOVINE ANIMALS, EDIBLE, FROZEN Skip this table U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted (FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 1,377,073 7,372 27,349,941 105,661 Canada 0 0 5,159 7 China 66,968 208 675,449 1,382 Costa Rica 0 0 6,567 18 Hong Kong 121,237 431 2,176,415 3,917 Indonesia 24,957 13 39,957 17 Japan 920,049 5,943 17,255,240 83,562 Korea, South 89,412 404 2,435,561 8,129 Malaysia 0 0 23,596 10 Mexico 45,264 126 1,258,740 3,282 Poland 0 0 23,596 14 Russia 51,472 49 3,083,619 3,942 Taiwan 57,714 198 354,691 1,260 Vietnam 0 0 11,351 121 Top of page Source: Foreign Trade Division , U.S. Census Bureau. Presented by: Office of Trade and Economic Analysis (OTEA), International Trade Administration, U.S. Department of Commerce. http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-December/Exports/02/020621.html ##################### Bovine Spongiform Encephalopathy ##################### Subject: USDA, SPONTANEOUS MAD COW DISEASE, THE TOOTH FAIRY AND SANTA CLAUS Date: June 12, 2006 at 5:18 am PST IF we all believe the BSe that the USDA is trying to put out now about atypical BSE in USA cattle just arising spontaneously, then we all should believe in the tooth fairy and santa claus as well. IF USA scrapie transmitted to USA cattle long ago in experiments in a lab in Mission Texas did not produce UK BSE, but something very different, then why would USA TSE cattle produce the UK human version of mad cow i.e. nvCJD? IT wouldn't. USA sporadic cjd is increasing, the USA also has atypical human cases of unknown origin as well? THERE are over 20 strains of scrapie, plus the atypical in sheep, and these strains are increasing in numbers. SCRAPIE, CWD, AND TSE IN CATTLE i.e. ANIMAL TSE RAMPANT IN USA FOR DECADES, and amplified via rendering and feeding practices, where USDA triple firewalls against BSE were nothing more than a mere smoke screen. NO test tube TSE by either Prusiner or Soto, to date, have ever produced a TSE identical to the sporadic CJD. IN fact, no test tube TSE has ever been produced that resembles _any_ natural field TSE. IF you feed BSE tainted materials to cattle and primate, you have BSE and nvCJD. IF you feed USA sheep strain to USA cattle, you get USA TSE. IF you feed USA tainted cattle to humans, you get USA mad cow disease. IF you feed sporadic CJD to primate you get a CJD infected primate. NOTHING spontaneous about it at all. USA is in a very unique situation. there are more documented TSE in different species than any other country, all of which have been rendered and fed back to animals for human and animal consumption, for decades. Millions exposed, and of these Millions, how many surgical and dental procedures have been done on these exposed, to pass on to others, via the 'friendly fire' mode of transmission? IF, the spontaneous TSE was true, then this would be Prusiner and everyone else that is trying to cash in on this agent with there TSE rapid test, this would be there dream come true. IT would require mandatory BSE/TSE testing of all species, due to the fact you could not ever eradicate it through any intervention. BUT, then again, the spontaneous TSE is like believing in the tooth fairy or santa clause will be arriving at your house this year. How long can this sharade continue $ How many more will become exposed and have to die $ Medical Sciences Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease Cristina Casalone *, Gianluigi Zanusso , Pierluigi Acutis *, Sergio Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco ||, and Maria Caramelli * *Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico "Carlo Besta," Via Celoria 11, 20133 Milan, Italy Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003) Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called "species barrier" between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease-resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt-Jakob disease. -------------------------------------------------------------------------------- C.C. and G.Z. contributed equally to this work. ||To whom correspondence should be addressed. E-mail: salvatore.monaco@mail.univr.it. www.pnas.org/cgi/doi/10.1073/pnas.0305777101 http://www.pnas.org/cgi/content/abstract/0305777101v1 : J Infect Dis 1980 Aug;142(2):205-8 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. PMID: 6997404 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract Atypical cases of TSE in cases of TSE in cattle and sheep cattle and sheep H. De H. De Bosschere Bosschere CODA/CERVA CODA/CERVA Nat. Ref. Lab. Vet. Nat. Ref. Lab. Vet. TSEs TSEs Belgium http://www.var.fgov.be/pdf/1100_TSEDAY.pdf USDA 2004 ENHANCED BSE SURVEILLANCE PROGRAM AND HOW NOT TO FIND BSE CASES (OFFICIAL DRAFT OIG REPORT) snip... CATTLE With CNS Symptoms Were NOT Always Tested snip... Between FYs 2002 and 2004, FSIS condemned 680 cattle of all ages due to CNS symptoms. About 357 of these could be classified as adult. We could validate that ONLY 162 were tested for BSE (per APHIS records. ... snip... WE interviewed officials at five laboratories that test for rabies. Those officials CONFIRMED THEY ARE NOT REQUIRED TO SUBMIT RABIES-NEGATIVE SAMPLES TO APHIS FOR BSE TESTING. A South Dakota laboratory official said they were not aware they could submit rabies-negative samples to APHIS for BSE testing. A laboratory official in another State said all rabies-negative cases were not submitted to APHIS because BSE was ''NOT ON THEIR RADAR SCREEN." Officials from New York, Wisconsin, TEXAS, and Iowa advised they would NOT submit samples from animals they consider too young. Four of the five States contacted defined this age as 24 months; Wisconsin defined it as 30 months. TEXAS officials also advised that they do not always have sufficient tissue remaining to submit a BSE sample. ... snip... FULL TEXT 54 PAGES OF HOW NOT TO FIND BSE IN USA ; http://www.house.gov/reform/min/pdfs_108_2/pdfs_inves/pdf_food_usda_mad_cow_july_13_ig_rep.pdf HUMAN TSE USA 2005 Animal Prion Diseases Relevant to Humans (unknown types?) Thu Oct 27, 2005 12:05 71.248.128.109 About Human Prion Diseases / Animal Prion Diseases Relevant to Humans Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later. Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.) Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.) http://www.cjdsurveillance.com/abouthpd-animal.html SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here; p.s. please note the 47 PENDING CASES to Sept. 2005 p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ??? p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN??? p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN??? http://www.cjdsurveillance.com/resources-casereport.html CWD TO HUMANS = sCJD ??? AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease. snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf snip...end full text ; http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf VERY VERY IMPORTANT THING TO REMEMBER >> Differences in tissue distribution could require new regulations >> regarding specific risk material (SRM) removal. Research Project: Study of Atypical Bse Location: Virus and Prion Diseases of Livestock Project Number: 3625-32000-073-07 Project Type: Specific C/A Start Date: Sep 15, 2004 End Date: Sep 14, 2007 Objective: The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. Approach: This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal. http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490 3.57 The experiment which might have determined whether BSE and scrapie were caused by the same agent (ie, the feeding of natural scrapie to cattle) was never undertaken in the UK. It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE. http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820543 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820546 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE. 3.58 There are several possible reasons why the experiment was not performed in the UK. It had been recommended by Sir Richard Southwood (Chairman of the Working Party on Bovine Spongiform Encephalopathy) in his letter to the Permanent Secretary of MAFF, Mr (now Sir) Derek Andrews, on 21 June 1988, 35 though it was not specifically recommended in the Working Party Report or indeed in the Tyrrell Committee Report (details of the Southwood Working Party and the Tyrell Committee can be found in vol. 4: The Southwood Working Party, 1988-89 and vol. 11: Scientists after Southwood respectively). The direct inoculation of scrapie into calves was given low priority, because of its high cost and because it was known that it had already taken place in the USA. 36 It was also felt that the results of such an experiment would be hard to interpret. While a negative result would be informative, a positive result would need to demonstrate that when scrapie was transmitted to cattle, the disease which developed in cattle was the same as BSE. 37 Given the large number of strains of scrapie and the possibility that BSE was one of them, it would be necessary to transmit every scrapie strain to cattle separately, to test the hypothesis properly. Such an experiment would be expensive. Secondly, as measures to control the epidemic took hold, the need for the experiment from the policy viewpoint was not considered so urgent. It was felt that the results would be mainly of academic interest. 38 http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm#820550 http://www.bseinquiry.gov.uk/report/volume2/chaptea3.htm REPORT OF THE COMMITTEE ON SCRAPIE Chair: Dr. Jim Logan, Cheyenne, WY Vice Chair: Dr. Joe D. Ross, Sonora, TX Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN. The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance. The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings. Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories. For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory’s certification was revoked by APHIS-VS. snip... Infected and Source Flocks As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats. Regulatory Scrapie Slaughter Surveillance (RSSS) RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1, 2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below. Scrapie Testing In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9). Animal ID As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises. *This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans. http://www.usaha.org/committees/reports/2005/report-scr-2005.pdf Date: April 30, 2006 at 4:49 pm PST SCRAPIE USA UPDATE AS of March 31, 2006 2 NEW CASES IN GOAT, 82 INFECTED SOURCE FLOCKS, WITH 4 NEW INFECTED SOURCE FLOCKS IN MARCH, WITH 19 SCRAPIE INFECTED RSSS REPORTED BY NVSL http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html Published online before print October 20, 2005 Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102 Medical Sciences A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes ( sheep prion | transgenic mice ) Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude * *Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005) Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health. -------------------------------------------------------------------------------- Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper. A.L.D. and V.B. contributed equally to this work. To whom correspondence should be addressed. Hubert Laude, E-mail: laude@jouy.inra.fr www.pnas.org/cgi/doi/10.1073/pnas.0502296102 http://www.pnas.org/cgi/content/abstract/0502296102v1 12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY snip... A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf Like lambs to the slaughter 31 March 2001 Debora MacKenzie Magazine issue 2284 What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie? FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease. Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America. Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in ... The complete article is 889 words long. full text; http://www.newscientist.com/article.ns?id=mg16922840.300 Neurobiology Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys* * Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and Institute for Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom Edited by D. Carleton Gajdusek, Centre National de la Recherche Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000 (received for review October 16, 2000) Abstract There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment. http://www.pnas.org/cgi/content/full/041490898v1 USDA CWD PROGRAM http://www.aphis.usda.gov/vs/nahps/cwd/ USDA CWD MAP (slow to update) http://www.aphis.usda.gov/vs/nahps/cwd/cwd-distribution.html DRAFT WYOMING GAME AND FISH DEPARTMENT CHRONIC WASTING DISEASE MANAGEMENT PLAN February 17, 2006 snip... 5. Predicted population effects on free-ranging elk based on captive elk chronically exposed to the CWD prion. Forty-three female elk calves were trapped at the National Elk Refuge and transported to Sybille in February 2002. Elk were housed in pens, assumed to be environmentally contaminated with the CWD prion. Elk will be held throughout their lifetimes. Elk dying will be examined and cause of death determined. From these data, it will should be possible to model free-ranging elk mortality and population dynamics under extreme circumstances of CWD prion exposure and transmission. As of December 2005 (46 months post capture), 11 of 43 elk have died due to CWD. This compares to 100% mortality in less than 25 months in elk orally inoculated with different dosages of the CWD prion. REVISED DRAFT http://gf.state.wy.us/downloads/pdf/CWD2005reviseddraft.pdf Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§ 1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA. *These authors contributed equally to this work. †Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA. ‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland. §To whom correspondence should be addressed: E-mail: gtell2@uky.edu Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD. To test whether skeletal muscle of diseased cervids contained prion infectivity, Tg(CerPrP)1536 mice (2) expressing cervid prion protein (CerPrP), were inoculated intracerebrally with extracts prepared from the semitendinosus/semimembranosus muscle group of CWD-affected mule deer or from CWD-negative deer. The availability of CNS materials also afforded direct comparisons of prion infectivity in skeletal muscle and brain. All skeletal muscle extracts from CWD-affected deer induced progressive neurological dysfunction in Tg(CerPrP)1536 mice with mean incubation times ranging between 360 and ~490 d, whereas the incubation times of prions from the CNS ranged from ~230 to 280 d (Table 1). For each inoculation group, the diagnosis of prion disease was confirmed by the presence of PrPSc in the brains of multiple infected Tg(CerPrP)1536 mice (see supporting online material for examples). In contrast, skeletal muscle and brain material from CWD-negative deer failed to induce disease in Tg(CerPrP)1536 mice (Table 1) and PrPSc was not detected in the brains of sacrificed asymptomatic mice as late as 523 d after inoculation (supporting online material). Our results show that skeletal muscle as well as CNS tissue of deer with CWD contains infectious prions. Similar analyses of skeletal muscle BSE-affected cattle did not reveal high levels of prion infectivity (3). It will be important to assess the cellular location of PrPSc in muscle. Notably, while PrPSc has been detected in muscles of scrapie-affected sheep (4), previous studies failed to detect PrPSc by immunohistochemical analysis of skeletal muscle from deer with natural or experimental CWD (5, 6). Since the time of disease onset is inversely proportional to prion dose (7), the longer incubation times of prions from skeletal muscle extracts compared to matched brain samples indicated that prion titers were lower in muscle than in CNS where infectivity titers are known to reach high levels. Although possible effects of CWD strains or strain mixtures on these incubation times cannot be excluded, the variable 360 to ~490 d incubation times suggested a range of prion titers in skeletal muscles of CWD-affected deer. Muscle prion titers at the high end of the range produced the fastest incubation times that were ~30% longer than the incubation times of prions from the CNS of the same animal. Since all mice in each inoculation group developed disease, prion titers in muscle samples producing the longest incubation times were higher than the end point of the bioassay, defined as the infectious dose at which half the inoculated mice develop disease. Studies are in progress to accurately assess prion titers. While the risk of exposure to CWD infectivity following consumption of prions in muscle is mitigated by relatively inefficient prion transmission via the oral route (8), these results show that semitendinosus/semimembranosus muscle, which is likely to be consumed by humans, is a significant source of prion infectivity. Humans consuming or handling meat from CWD-infected deer are therefore at risk to prion exposure. References and Notes 1. P. J. Bosque et al., Proc. Natl. Acad. Sci. U.S.A. 99, 3812 (2002). 2. S. R. Browning et al., J. Virol. 78, 13345 (2004). 3. A. Buschmann, M. H. Groschup, J. Infect. Dis. 192, 934 (2005). 4. O. Andreoletti et al., Nat. Med. 10, 591 (2004). 5. T. R. Spraker et al., Vet. Pathol. 39, 110 (2002). 6. A. N. Hamir, J. M. Miller, R. C. Cutlip, Vet. Pathol. 41, 78 (2004). 7. S. B. Prusiner et al., Biochemistry 21, 4883 (1980). 8. M. Prinz et al., Am. J. Pathol. 162, 1103 (2003). 9. This work was supported by grants from the U.S. Public Health Service 2RO1 NS040334-04 from the National Institute of Neurological Disorders and Stroke and N01-AI-25491 from the National Institute of Allergy and Infectious Diseases. Supporting Online Material www.sciencemag.org/ Materials and Methods Fig. S1 21 November 2005; accepted 13 January 2006 Published online 26 January 2006; 10.1126/science.1122864 Include this information when citing this paper. Table 1. Incubation times following inoculation of Tg(CerPrP)1536 mice with prions from skeletal muscle and brain samples of CWD-affected deer. Inocula Incubation time, mean d ± SEM (n/n0)* Skeletal muscle Brain CWD-affected deer H92 360 ± 2 d (6/6) 283 ± 7 d (6/6) 33968 367 ± 9 d (8/8) 278 ± 11 d (6/6) 5941 427 ± 18 d (7/7) D10 483 ± 8 d (8/8) 231 ± 17 d (7/7) D08 492 ± 4 d (7/7) Averages 426 d 264 d Non-diseased deer FPS 6.98 >523 d (0/6) FPS 9.98 >454 d (0/7) >454 d (0/6) None >490 d (0/6) PBS >589 d (0/5) *The number of mice developing prion disease divided by the original number of inoculated mice is shown in parentheses. Mice dying of intercurrent illnesses were excluded. http://www.sciencemag.org/ www.sciencemag.org/ Supporting Online Material for Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers, Shawn R. Browning, Tanya S. Seward, Christina J. Sigurdson, Michael W. Miller, Edward A. Hoover, Glenn C. Telling§ §To whom correspondence should be addressed: E-mail: gtell2@uky.edu Published 26 January 2006 on Science Express DOI: 10.1126/science.1122864 This PDF file includes: Materials and Methods Fig. S1 Supporting Online Materials Materials and Methods Homogenates of semitendinosus/semimembranosus muscle (10% w/v in phosphate buffered saline) were prepared from five emaciated and somnolent mule deer, naturally infected with CWD at the Colorado Division of Wildlife, Wildlife Research Center. These deer were identified as D10, D08, 33968, H92, and 5941. CWD infection was confirmed in all cases by the presence of histologic lesions in the brain including spongiform degeneration of the perikaryon, the immunohistochemical detection of disease-associated PrP in brain and tonsil, or by immunoblotting of protease-resistant, disease associated PrP (CerPrPSc). Semitendinosus/semimembranosus muscle was also obtained from two asymptomatic, mock inoculated deer, referred to as FPS 6.68 and 9.98, that originated from a CWD non-endemic area and which were held indoors at Colorado State University from ten days of age. These control deer were confirmed negative for CWD by histopathological and immunohistochemical analysis of brain tissue at autopsy. The utmost care was taken to avoid inclusion of obvious nervous tissue when muscle biopsies were prepared and to ensure that contamination of skeletal muscle samples with CNS tissue did not occur. Fresh, single-use instruments were used to collect each sample biopsy and a central piece from each sample was prepared with fresh, disposable instruments to further isolate muscle tissue for inoculum preparation. Brain samples for transmission were prepared separately from muscle as additional insurance against cross contamination. 1 Groups of anesthetized Tg(CerPrP)1536 mice were inoculated intracerebrally with 30 µl of 1 % skeletal muscle or brain extracts prepared in phosphate buffered saline (PBS). Inoculated Tg(CerPrP) mice were diagnosed with prion disease following the progressive development of at least three neurologic symptoms including truncal ataxia, ‘plastic’ tail, loss of extensor reflex, difficultly righting, and slowed movement. The time from inoculation to the onset of clinical signs is referred to as the incubation time. For PrP analysis in brain extracts of Tg(CerPrP)1536 mice, 10 % homogenates prepared in PBS were either untreated (-) or treated (+) with 40 µg/ml proteinase K (PK) for one hour at 37oC in the presence of 2% sarkosyl. Proteins were separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis, analyzed by immunoblotting using anti PrP monoclonal antibody 6H4 (Prionics AG, Switzerland), incubated with appropriate secondary antibody, developed using ECL-plus detection (Amersham), and analyzed using a FLA-5000 scanner (Fuji). 2 Fig. S1 PrP in brain extracts from representative Tg(CerPrP)1536 mice receiving muscle or CNS tissue inocula from CWD-affected or CWD-negative deer. Extracts were either treated (+) or untreated (-) with proteinase K (PK) as indicated. The positions of protein molecular weight markers at 21.3, 28.7, 33.5 kDa (from bottom to top) are shown to the left of the immunoblot. 3 http://www.sciencemag.org/ Chronic Wasting Disease and Potential Transmission to Humans Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger* *Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm Research Environmental Sources of Prion Transmission in Mule Deer Michael W. Miller,* Elizabeth S. Williams,† N. Thompson Hobbs,‡ and Lisa L. Wolfe* *Colorado Division of Wildlife, Fort Collins, Colorado, USA; †University of Wyoming, Laramie, Wyoming, USA; and ‡Colorado State University, Fort Collins, Colorado, USA Suggested citation for this article: Miller MW, Williams ES, Hobbs NT, Wolfe LL. Environmental sources of prion transmission in mule deer. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm ATYPICAL TSEs in USA CATTLE AND SHEEP ? http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf UKBSEnvCJD only theory Singeltary et al 2006 (please note, et al in this term means all victims and familes of the sporadic CJD that are still looking for answers. ...TSS) http://www.microbes.info/forums/index.php?act=Attach&type=post&id=13 http://www.microbes.info/forums/index.php?showtopic=306 NEW STRAIN OF TSE USA CATTLE OR JUST INCOMPETENCE IN TESTING??? http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf CJD WATCH http://www.fortunecity.com/healthclub/cpr/349/part1cjd.htm CJD WATCH MESSAGE BOARD http://disc.server.com/Indices/167318.html Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 #################### https://lists.aegee.org/bse-l.html ####################

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Subject: SEAC Position statement vCJD and Endodontic dentistry and atypical TSE ??? Date: June 14, 2006 at 12:56 pm PST SEAC Position Statement -------------------------------------------------------------------------------- Position statement vCJD and Endodontic dentistry Issue 1. The Department of Health (DH) asked SEAC to advise on the findings and implications of a preliminary risk assessment of potential vCJD transmission via endodontic procedures (dental procedures involved in the maintenance of dental pulp and the treatment of the pulp cavity) 1. This is particularly pertinent because of the large number of endodontic procedures undertaken in the UK. Background 2. There are no reported definite or suspected cases of vCJD transmission arising from dental procedures. However, prions are more resistant than other types of infectious agent to the conventional cleaning and sterilisation practices used to decontaminate dental instruments 2. Therefore, should dental instruments become contaminated from tissues in the oral cavity of infected individuals, there is a risk of transmission to subsequent patients. 3. A quantitative DH risk assessment 3, accepted by SEAC in 2003, considered two possible mechanisms for the transfer of vCJD infectivity via dental instruments: (i) accidental abrasion of the lingual tonsil, known to carry infectivity in vCJD cases; and (ii) contact with dental pulp that evidence from animal studies suggested may be infective. On the basis of the information available, the DH analysis suggested that the risk of transmission to individual patients via accidental abrasion of the lingual tonsil is very low. Furthermore, should dental pulp be infective, the risk of transmission via endodontic procedures, although higher, is also low. Although a very large number of dental procedures are conducted, the relative risk to public health from potential transmission via dental, compared with hospital, surgery was considered to be relatively low. 4. In 2006, SEAC considered a new preliminary risk assessment by DH of the risks of vCJD transmission via endodontic procedures, taking into account new information on decontamination of dental instruments, the potential infectivity of dental pulp, and the possible existence of subclinical vCJD carrier cases. Endodontic instruments 5. Evidence suggests that the files and reamers used in endodontic procedures are reused and are difficult to reliably decontaminate 4. Appreciable quantities of residual material remain adherent to the surface after normal cleaning and sterilisation 5. Thus, there is potential for transfer of dental pulp between patients undergoing endodontic procedures. vCJD infectivity in dental tissues 6. There are no data on vCJD infectivity in dental pulp. Although no abnormal prions were found in a study of dental tissues, including dental pulp, from vCJD cases 6, dental pulp includes blood and peripheral nerve tissue known to carry vCJD infectivity 7,8. In addition, appreciable infectivity has been found in the dental pulp of hamsters with hamster scrapie 9. Although it is possible that the peripheral nerve may only become infective close to, or after, the onset of clinical vCJD, inflammation may promote the propagation of prions 10. Thus, although the data are limited and indirect, it is reasonable to assume that the dental pulp of individuals subclinically-infected with vCJD may be infectious although the level of infectivity is unknown. Studies underway will provide direct data on the infectivity in dental tissues from vCJD cases. Subclinical carrier state 7. A study of humanised mice showed that vCJD infections may not always progress to clinical disease within the normal lifespan of the animals 11. Another study suggested that prion infections in mice that remain at a subclinical level can be transmitted to other mice, resulting in clinical disease 12. Thus, there is evidence to suggest that individuals infected with the BSE / vCJD agent may remain in a subclinical infection carrier state instead of developing vCJD. A discrepancy between prevalence estimates based on a survey of abnormal prion protein in appendix and tonsil tissue and data on vCJD cases supports this hypothesis 13. As no diagnostic test exists to identify such individuals, they could over the course of their lives be potential sources of numerous secondary infections arising from invasive medical or dental procedures. 8. The prevalence of subclinical infection in the UK population is uncertain. A recent estimate suggests the number of subclinical carriers may be of the order of several thousand 14. SEAC has strongly recommended that further studies to ascertain better the prevalence of vCJD infection be urgently considered 15. Transmission risks 9. The new DH analysis suggests that, on the basis that residual dental pulp on endodontic files and reamers is transferred relatively efficiently to patients on reuse, dental pulp is as infective as peripheral nerve tissue and a subclinical carrier population for vCJD exists, a self-sustaining vCJD epidemic arising from endodontic surgery is plausible. There are uncertainties about the efficiency of vCJD transmission via endodontic procedures, the vCJD infectivity of dental pulp and the existence of a subclinical infection carrier state. However, even if a self-sustaining epidemic were not possible, clusters of vCJD infections could arise from the use of instruments contaminated with the vCJD agent from endodontic procedures on infected patients. Interactions between this and other routes of secondary transmission, such as blood transfusion and hospital surgery, would make a self-sustaining epidemic more likely. Potential risk reduction measures 10. Endodontic files and reamers have a limited lifespan, restricting the number of possible secondary transmissions. Improving the effectiveness of procedures used to decontaminate dental instruments would reduce the risk of transmission. Restricting endodontic files and reamers to single use would prevent potential secondary transmission via these instruments. Conclusions 11. A preliminary risk assessment produced by DH suggests that vCJD transmission via endodontic dentistry may, under certain hypothetical but plausible scenarios, be sufficient to sustain a secondary vCJD epidemic. However, there are uncertainties around the data and assumptions underpinning the assessment. Research underway will address some of these uncertainties and allow the risk assessment to be refined. Once the research is complete and / or other data become available, the risks should be reassessed. A watching brief should be maintained. 12. It is unclear whether or not vCJD infectivity can be transmitted via endodontic files and reamers. However, given the plausibility of such a scenario and the large number of procedures undertaken annually, it would be prudent to consider restricting these instruments to single use as a precautionary measure. Since sufficiently rigorous decontamination of these instruments is difficult, single use of these instruments would eliminate this risk, should it exist. SEAC May 2006 -------------------------------------------------------------------------------- 1. Department of Health. Dentistry and vCJD: the implications of a “carrier state” for a self-sustaining epidemic due to endodontic dentistry. A Preliminary Risk Assessment. Unpublished. 2. Smith et al. (2003) Prions and the oral cavity. J. Dent. Res. 82, 769-775. 3. Department of Health. (2003) Risk assessment for vCJD and dentistry. 4. Letters et al. (2005) A study of visual and blood contamination on reprocessed endodontic files from general dental practice. Br. Dent. J. 199, 522-525. 5. Smith et al. (2005) Residual protein levels on reprocessed dental instruments. J. Hosp. Infect. 61, 237-241. 6. Head et al. (2003) Investigation of PrPres in dental tissues in variant CJD. Br. Dent. J. 195, 339-343. 7. SEAC 91 minutes paragraph 9. www.seac.gov.uk/papers/papers.htm 8. Department of Health (2005) Assessing the risk of vCJD transmission via surgery: an interim view. Unpublished. 9. Ingrosso et al. (1999) Transmission of the 263K scrapie strain by the dental route. J. Gen. Virol. 80, 3043-3047. 10. Heikenwalder et al. (2005) Chronic lymphocytic inflammation specifies the organ tropism of prions. Science. 307, 1107-1110. 11. Bishop et al. (2006) Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurology. 12. Hill et al. (2000) Species-barrier-independent prion replication in apparently resistant species. Proc. Natl. Acad. Sci. USA. 97, 10248-10253. 13. SEAC Epidemiology Subgroup (2005) Position statement on the vCJD epidemic. www.seac.gov.uk/statements/state260106subgroup.htm 14. Clarke & Ghani. (2005) Projections of future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility. R. J. Soc. Interface. 15. SEAC (2005) SEAC response to the SEAC Epidemiology Subgroup statement on the vCJD epidemic. www.seac.gov.uk/statements/state260106.htm Page updated: 8th May 2006 http://www.seac.gov.uk/statements/statement0506.htm Greetings, WITH the new atypical TSE in cattle in the USA, new atypical TSE in sheep and goat (USA imported from UK?), real definitions of SRMs now???, this brings into question once again of the ukbsenvcjd only theory, especially in terms of iCJD i.e. 'friendly fire'. what about those 'unknown' strains of sporadic CJD popping up now??? SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here; p.s. please note the 47 PENDING CASES to Sept. 2005 p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ??? p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN??? p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN??? http://www.cjdsurveillance.com/resources-casereport.html TSS Subject: ENDOSCOPY EQUIPMENT (Terminal ileal biopsies should not be used) and CJD (ALL PHENOTYPES) Date: March 8, 2005 at 1:09 pm PST -------- Original Message -------- Subject: Terminal ileal biopsies should not be used to document extent of colonoscopic examination Date: Tue, 8 Mar 2005 09:17:50 -0600 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE ##################### Bovine Spongiform Encephalopathy ##################### Greetings again, seems Bramble et al have failed to take heed to the latest data on atypical TSEs. UNTIL the BSE/nvCJD 'ONLY THEORY' is put to rest once and for all, this agent will continue to spread. with the findings of the testing with CDI from Prusiner et al and Aguzzi continued warnings of muscle tissue and Collinge warnings about sporadic CJD, to continue with this BSE/nvCJD 'ONLY THEORY' should be regarded with great suspicion. WITH many atypical TSEs showing up in cattle, sheep and goats in many different parts of the Globe, with the findings of BASE in cattle in Italy of Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease http://www.pnas.org/cgi/content/abstract/0305777101v1 Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger ] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate; http://www.pnas.org/cgi/content/full/041490898v1 Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471 WITH ALL THIS DATA, TO CONTINUE TO WARN ONLY OF THE nvCJD threat will only allow this agent to continue to spread...TSS Gut 2005;54:566 © 2005 by BMJ Publishing Group Ltd & British Society of Gastroenterology ------------------------------------------------------------------------ LETTER Terminal ileal biopsies should not be used to document extent of colonoscopic examination M D Rutter1 and M G Bramble2 1 University Hospital of North Tees, Stockton-on-Tees, Cleveland, UK 2 James Cook University Hospital, Middlesbrough, Cleveland, UK Correspondence to: Dr M D Rutter University Hospital of North Tees, Stockton-on-Tees, Cleveland, TS19 8PE, UK; matt.rutter@nth.nhs.uk Keywords: ileal biopsies; colonoscopy; guidelines The first 150 words of the full text of this article appear below. We commend the British Society of Gastroenterology and the authors for the excellent publication of guidelines for the management of inflammatory bowel disease in adults (Gut 2004;53(suppl V):v1v16). However, we feel that their recommendation for routine terminal ileal biopsying is inappropriate. Although it is important to biopsy the terminal ileum if there is macroscopic evidence of an abnormality, their statement that "a terminal ileal biopsy performed at colonoscopy documents the extent of examination" is not recommended practice, due to the potential risk of variant Creutzfeld-Jacob disease transmission from prion proteins which are prevalent in the lymphoid tissue of Peyers patches in the ileum. Although the use of disposable forceps may reduce the risk of transmission, there could still be contamination of the intubation channel of the colonoscope and prion protein is resistant to the standard endoscopic cleaning process.1 If the extent of examination needs to . . . [Full text of this article ] http://gut.bmjjournals.com/cgi/content/extract/54/4/566-a -------- Original Message -------- Subject: Re: gutjnl_el;110 Terry S. Singeltary Sr. (22 Aug 2003) ""CJDs (all human TSEs) and Endoscopy Equipment Date: Tue, 26 Aug 2003 15:10:51 -0500 From: "Terry S. Singeltary Sr." To: Robin.Spiller@nottingham.ac.uk hello Professor Spiller and GUT, this has become a real challenge trying to raise my concerns to GUT about sporadic CJD. but i will not give up. you only have to see it once. i hope you take the time to read over all data below...thank you > unsubstantiated opinion and emotion these are not my unsubstantiated opinions, they are backed up by science (transmission studies and or lack of transmissions studies), and i will try and leave my emotions out. > I feel that we need a proper evidence based approach to this. > There is too much unsubstantiated opinion i agree... to categorically state that nv/v CJD is the only risk to endoscopy equipment, while ignoring all other TSEs, is very unsubstantiated. i will try and give more evidence for my concerns below. 1st, there has never been to date any _documented_ transmission of nv/v CJD via the medical surgical arena. this has only been hypothesized... 2nd, However, there has been _documented_ evidence of transmission of sporadic CJD via the medical and surgical arena. TO continue to hide behind the nv/v CJD only theory, when there are over 20 strains of scrapie, most of which transmits to all mammalian species that has been tried upon, CWD which no one knows if or if not it can transmit to humans (to date no transmission studies of any TSEs done on man), but studies have shown transmission to humans as easy as BSE, and does transmit to primate. there are now 6 _documented_ phenotypes of sporadic CJD. with CWD and Scrapie running rampant in the USA, no real active surveillance in cattle for TSEs and no rapid testing done to find BSE agent (48,000+ BSE/TSE test in 14 years TOTAL USA), who knows how these strains of CJD will act and how they will transmit. also, in vitro experiments have demonstrated that scrapie/cwd prions are as efficient as BSE prions in transforming normal human prion protein to PrPSc. this data strongly supports that scrapie/cwd is as infectious as BSE. IN fact, scrapie seems to be more infectious than BSE due to higher concentration of TSE infectious agents in ovine muscle meat and other parts of the sheep, when compared to cattle, and CWD could even be more infectious than all of them, if you consider how it has rapidly spread across the USA. but to categorically state that only nv/v CJD to be of risk to endoscopy equipment when no such documented transmission has ever been documented, while ignoring such similar medical _documented_ transmissions in sporadic CJD, this is very disturbing and most unsubstantiated, and potentially risking TSE exposure to millions and millions due to nothing more than denial and wishful thinking. if i could deny this and wish it away, i would have done this six years ago. but we cant, all we can do is warn the public and the medical community of what we know to date. i am surprised GUT has chosen not to do this, and only to go with the BSE/nv/v/CJD only theory. 85%+ of all CJDs don't just happen without route and source. my fear is a great deal are being mis-diagnosed and un-reported, but being acquired via the medical surgical arena. but one will never know without making all human TSEs reportable in every State, and issuing a CJD Questionnaire to all families of victims of CJD/TSE and asking the _real_ questions that pertain to route and source of agent...TSS REFERENCES snip...end http://neuro-mancer.mgh.harvard.edu/ubb/Forum24/HTML/000145.html FULL TEXT ; http://www.vegsource.com/talk/madcow/messages/93658.html TSS HISTORY OF GUT, ENDOSCOPY, CJD AND TSS http://www.vegsource.com/talk/madcow/messages/93658.html http://www.google.com/search?num=30&hl=en&lr=&edition=us&ie=UTF-8&search=search&tab=wn&scoring=d&q=CJD+ENDOSCOPY+TSS&btnmeta%3Dsearch%3Dsearch=Search+the+Web MAD COW i.e. all TSE 'FRIENDLY FIRE' GETTING SERIOUS (iCJD) ##################### Bovine Spongiform Encephalopathy ##################### CJD WATCH MESSAGE BOARD TSS Detection and Localization of PrPSc in the Skeletal Muscle Thu Mar 2, 2006 10:40 70.110.86.250 © 2006 American Society for Investigative Pathology Detection and Localization of PrPSc in the Skeletal Muscle of Patients with Variant, Iatrogenic, and Sporadic Forms of Creutzfeldt-Jakob Disease Alexander H. Peden, Diane L. Ritchie, Mark W. Head and James W. Ironside From the National Creutzfeldt-Jakob Disease Surveillance Unit and Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom Variant Creutzfeldt-Jakob disease (vCJD) differs from other human prion diseases in that the pathogenic prion protein PrPSc can be detected to a greater extent at extraneuronal sites throughout the body, principally within lymphoid tissues. However, a recent study using a high-sensitivity Western blotting technique revealed low levels of PrPSc in skeletal muscle from a quarter of Swiss patients with sporadic CJD (sCJD). This posed the question of whether PrPSc in muscle could also be detected in vCJD, sCJD, and iatrogenic (iCJD) patients from other populations. Therefore, we have used the same high-sensitivity Western blotting technique, in combination with paraffin-embedded tissue blotting, to screen for PrPSc in muscle tissue specimens taken at autopsy from 49 CJD patients in the United Kingdom. These techniques identified muscle PrPSc in 8 of 17 vCJD, 7 of 26 sCJD, and 2 of 5 iCJD patients. Paraffin-embedded tissue blotting analysis showed PrPSc in skeletal muscle in localized anatomical structures that had the morphological and immunohistochemical characteristics of nerve fibers. The detection of PrPSc in muscle tissue from all forms of CJD indicates the possible presence of infectivity in these tissues, suggesting important implications for assessing the potential risk of iatrogenic spread via contaminated surgical instruments. http://ajp.amjpathol.org/cgi/content/abstract/168/3/927 TSS #################### https://lists.aegee.org/bse-l.html #################### BSE ALSO; PrPSc distribution of a natural case of bovine spongiform encephalopathy Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori- kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa Priori Disease Research Center, National Institute of Animal Health, 3-1-5 Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp Abstract Bovine spongiform encephalopathy (BSE) is a disease of cattle that causes progressive neurodegeneration of the central nervous system. Infectivity of BSE agent is accompanied with an abnormal isoform of prion protein (PrPSc). The specified risk materials (SRM) are tissues potentially carrying BSE infectivity. The following tissues are designated as SRM in Japan: the skull including the brain and eyes but excluding the glossa and the masse- ter muscle, the vertebral column excluding the vertebrae of the tail, spinal cord, distal illeum. For a risk management step, the use of SRM in both animal feed or human food has been prohibited. However, detailed PrPSc distribution remains obscure in BSE cattle and it has caused controversies about definitions of SRM. Therefore we have examined PrPSc distribution in a BSE cattle by Western blotting to reassess definitions of SRM. The 11th BSE case in Japan was detected in fallen stock surveillance. The carcass was stocked in the refrigerator. For the detection of PrPSc, 200 mg of tissue samples were homogenized. Following collagenase treatment, samples were digested with proteinase K. After digestion, PrPSc was precipitated by sodium phosphotungstate (PTA). The pellets were subjected to Western blotting using the standard procedure. Anti-prion protein monoclonal antibody (mAb) T2 conjugated horseradish peroxidase was used for the detection of PrPSc. PrPSc was detected in brain, spinal cord, dorsal root ganglia, trigeminal ganglia, sublingual ganglion, retina. In addition, PrPSc was also detected in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve). Our results suggest that the currently accepted definitions of SRM in 9/13/2005 179 Page 10 of 17 BSE cattle may need to be reexamined. T. Kitamoto (Ed.) PRIONS Food and Drug Safety ================ ALSO from the International Symposium of Prion Diseases held in Sendai, October 31, to November 2, 2004; Bovine spongiform encephalopathy (BSE) in Japan snip... "Furthermore, current studies into transmission of cases of BSE that are atypical or that develop in young cattle are expected to amplify the BSE prion" NO. Date conf. Farm Birth place and Date Age at diagnosis 8. 2003.10.6. Fukushima Tochigi 2001.10.13. 23 9. 2003.11.4. Hiroshima Hyogo 2002.1.13. 21 Test results # 8b, 9c cows Elisa Positive, WB Positive, IHC negative, histopathology negative b = atypical BSE case c = case of BSE in a young animal b,c, No PrPSc on IHC, and no spongiform change on histology International Symposium of Prion Diseases held in Sendai, October 31, to November 2, 2004. Tetsuyuki Kitamoto Professor and Chairman Department of Prion Research Tohoku University School of Medicine 2-1 SeiryoAoba-ku, Sendai 980-8575, JAPAN TEL +81-22-717-8147 FAX +81-22-717-8148 e-mail; kitamoto@mail.tains.tohoku.ac.jp Symposium Secretariat Kyomi Sasaki TEL +81-22-717-8233 FAX +81-22-717-7656 e-mail: kvomi-sasaki@mail.tains.tohoku.ac.ip ================================= 9/13/2005 -------------------------------------------------------------------------------- -------------------------------------------------------------------------------- Page 11 of 17 From: TSS () Subject: Atypical Proteinase K-Resistant Prion Protein (PrPres) observed in an Apparently Healthy 23-Month-Old Holstein Steer Date: August 26, 2005 at 10:24 am PST Atypical Proteinase K-Resistant Prion Protein (PrPres) observed in an Apparently Healthy 23-Month-Old Holstein Steer Jpn. J. Infect. Dis., 56, 221-222, 2003 Laboratory and Epidemiology Communications Atypical Proteinase K-Resistant Prion Protein (PrPres) Observed in an Apparently Healthy 23-Month-Old Holstein Steer Yoshio Yamakawa*, KenÕichi Hagiwara, Kyoko Nohtomi, Yuko Nakamura, Masahiro Nishizima ,Yoshimi Higuchi1, Yuko Sato1, Tetsutaro Sata1 and the Expert Committee for BSE Diagnosis, Ministry of Health, Labour and Welfare of Japan2 Department of Biochemistry & Cell Biology and 1Department of Pathology, National Institute of Infectious Diseases, Tokyo 162-8640 and 2Miistry of Health, Labour and Welfare, Tokyo 100-8916 Communicated by Tetsutaro Sata (Accepted December 2, 2003) *Corresponding author: Mailing address: Department of Biochemistry and Cell Biology, National Institute of Infectious Diseases, Toyama 1-23-1, Shinjuku-ku, Tokyo 1628640, Japan. Tel: +81-3-5285-1111, Fax: +81-3-5285-1157, E-mail: yamakawa@nih.go.jp Since October 18, 2001, 'bovine spongiform encephalopathy (BSE) examination for all cattle slaughtered at abattoirs in the country' has been mandated in Japan by the Ministry of Health, Labour and Welfare (MHLW). 'Plateria' ELISA-kit (Bio-Rad Laboratories, Hercules, Calif., USA) is routinely used at abattoirs for detecting proteinase K (PK)-resistant prion protein (PrPSc) in the obex region. Samples positive according to the ELISA screening are further subjected to Western blot (WB) and histologic and immunohistochemical examination (IHC) at the National Institute of Infectious Diseases (NIID) or Obihiro University. If PrPSc is detected either by WB or by IHC, the cattle are diagnosed as BSE. The diagnosis is approved by the Expert Committee for BSE Diagnosis, MHLW. From October 18, 2001 to September 30, 2003, approximately 2.5 million cattle were screened at abattoirs. A hundred and ten specimens positive according to ELISA were subjected to WB/IHC. Seven showed positive by both WB and IHC, all exhibiting the typical electrophoretic profile of a high content of the di-glycosylated molecular form of PrPSc (1-3) and the distinctive granular deposition of PrPSc in neuronal cells and neuropil of the dorsal nucleus of vagus. An ELISA-positive specimen from a 23 month-old Holstein steer slaughtered on September 29, 2003, in Ibaraki Prefecture (Ibaraki case) was sent to the NIID for confirmation. The animal was reportedly healthy before slaughter. The OD titer in ELISA was slightly higher than the 'cut-off' level given by the manufacturer. The histology showed no spongiform changes and IHC revealed no signal of PrPSc accumulation typical for BSE. However, WB analysis of the homogenate that was prepared from the obex region and used for ELISA revealed a small amount of PrPSc with an electrophoretic profile different from that of typical BSE-associated PrPSc (1-3). The characteristics were (i) low content of the di-glycosylated molecular form of PrPSc, (ii) a faster migration of the non-glycosylated form of PrPSc on SDS-PAGE, and (iii) less resistance against PK digestion as compared with an authentic PrPSc specimen derived from an 83-month-old Holstein (Wakayama case) (Fig. 1). Table 1 summarizes the relative amounts of three distinctive glycoforms (di-, mono, non-glycosylated) of PrPSc calculated by densitometric analysis of the blot shown in Fig. 1. As 2.5 mg wet weight obex-equivalent homogenate of the Ibaraki case (Fig. 1, lane 4) gave slightly stronger band intensities of PrPSc than an 8 mg wet weight obex-equivqlent homogenate of a typical BSE-affected Wakayama case (Fig. 1, lane 2), the amount of PrPSc accumulated in the Ibaraki case was calculated to be 1/500 - 1/1000 of the Wakayama case. In the Ibaraki case, the PrPSc bands were not detectable in the homogenates of the proximal surrounding region of the obex. These findings were consistent with the low OD value in ELISA, i.e., 0.2 -0.3 for the Ibaraki case versus over 3.0 for the Wakayama case. The DNA sequence of the PrP coding region of the Ibaraki case was the same as that appearing in the database (GenBank accession number: AJ298878). More recently, we encountered another case that resembled the Ibaraki case. It was a 21-monthold Holstein steer from Hiroshima Prefecture. WB showed typical BSE-specific PrPSc deposition though IHC did not detect positive signals of PrPSc (data not shown). Though the clinical onset of BSE is usually at around 5 years of age or later, a 20-month-old case showing the clinical signs has been reported (4). Variant forms of BSE similar to our cases, i.e., with atypical histopathological and/or biochemical phenotype, have been recently reported in Italy (5) and in France (6). Such variant BSE was not associated with mutations in the prion protein (PrP) coding region as in our case (5,6). The Ministry of Agriculture, Forestry and Fisheries of Japan (MAFF) announced a ban of feeding ruminants with meat bone meal (MBM) on September 18, 2001, and a complete ban was made on October 15 of the same year. According to the recent MAFF report, the previous seven cases of BSE in Japan were cattle born in 1995 - 1996 and possibly fed with cross-contaminated feed. However, the two cattle in this report were born after the complete ban. Whether contaminated MBM was implicated in the present cases remains to be investigated. REFERENCES Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J. and Hill, A. F. (1996): Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature, 383, 685690. Bruce, M. E., Will, R. G., Ironside, J. W., McConnell, I., Drummond, D., Suttie, A., McCardle, L., Chree, A., Hope, J., Birkett, C., Cousens, S., Fraser, H. and Bostock, C. J. (1997): Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature, 389, 498-501. Hill, A. F., Desbruslais, M., Joiner, S., Sidle, K. C. L., Gowland, I. and Collinge, J. (1997): The same prion strain causes vCJD and BSE. Nature, 389, 448-450. Matravers, W., Bridgeman, J. and Smith, M.-F. (ed.)(2000): The BSE Inquiry. p. 37. vol. 16. The Stationery Office Ltd., Norwich, UK. Casalone, C., Zanusso, G., Acutis, P. L., Crescio, M. I., Corona, C., Ferrari, S., Capobianco, R., Tagliavini, F., Monaco, S. and Caramelli, M. (2003): Identification of a novel molecular and neuropathological BSE phenotype in Italy. International Conference on Prion Disease: from basic research to intervention concepts. Gasreig, Munhen, October 8-10. Bicaba, A. G., Laplanche, J. L., Ryder, S. and Baron, T. (2003): A molecular variant of bovine spongiform encephalopatie. International Conference on Prion Disease: from basic research to intervention concepts. Gasreig, Munhen, October 8-10. Asante, E. A., Linehan, J. M., Desbruslais, M., Joiner, S., Gowland, I., Wood, A. L., Welch, J., Hill, A. F., Lloyd, S. E., Wadsworth, J. D. F. and Collinge, J. (2002). BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J., 21, 6358-6366. 9/13/2005 Page 12 of 17 SEE SLIDES IN PDF FILE; http://www.nih.go.jp/JJID/56/221.pdf http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf AND CWD; Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§ 1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA. *These authors contributed equally to this work. †Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA. ‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland. §To whom correspondence should be addressed: E-mail: gtell2@uky.edu Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD. To test whether skeletal muscle of diseased cervids.........SNIP....END TSS

flounder replied on

##################### Bovine Spongiform Encephalopathy ##################### CJD WATCH MESSAGE BOARD TSS EXPORT of potential USDA CERTIFIED ATYPICAL AND TYPICAL TSE Sat Jun 17, 2006 13:19 71.248.130.63 Greetings, Thought some of you might be interested in the USDA exports of potential USDA CERTIFIED ATYPICAL AND TYPICAL MAD COW BRAINS, SWEETBREADS, BOVINE FROZEN OFFAL, AND LIVE CATTLE. Interestingly, the USA may be the one to blame from there consistent lies and deceit and what they have exported globally for decades, to blame for spreading sporadic CJD around the globe. Looking from stats at ; http://www.fas.usda.gov/ustrade/ustlists/ExCmdty.asp?QI=370619655344&type=1&code=02 then searching here ; http://www.fas.usda.gov/ustrade/USTEXHS10.asp?QI=370619655344 SEEMS that Mexico received from the USA a boat load of potential mad cow brains 0206290030 between 2001 to 2005, Mexico received the most compared to COTE D'IVOIRE which was next in line, followed by ROMANIA, GREECE, SINGAPORE, GERMANY AND SWEDEN. ... NEXT, looking at SWEETBREADS 0206290040 the USA exported, and whatever phenotype of TSE that went along, we have as follows; MEXICO AGAIN receiving a boat load of sweetbreads, followed by ARGENTINA, JAPAN, URUGUAY, COLOMBIA, ISRAEL, BULGARIA, HONG KONG, VENEZUELA, United Arab Emirates, Switzerland, Singapore, Netherlands, The Bahamas, and the Dominican Republic. ... THE LIST for BOVINE OFFAL FROZEN 020629 EXPORTED FROM THE USA ACROSS THE GLOBE IS PHENOMENAL WITH JAPAN RECEIVING THE MOST FROM 1998 TO 2003, FOLLOWED BY MEXICO, and from here the list is staggering along with the amount of potential TSE tainted materials. ... FINALLY, LIVE CATTLE WITH CANADA RECEIVING THE MOST, FOLLOWED BY MEXICO, KOREA REPUBLIC OF, followed by many more countries with smaller amounts. ... WHEN the OIE did away with the BSE GBR risk assessments to ride saddle with GW and his legal tool to trade TSE globally i.e. the BSE MRR policy, 20 years of fighting this disease went down the drain, just so he could trade his precious commodities and futures. THIS BSe about how now the USA having an epidemic of a spontaneous TSE in cattle and humans, as sporadic CJD triples in 3 years in the USA, is simply absurd. nothing is spontaneous about it, there is absolutely no science to back these 'spontaneous' statements up. ... US "Atypical" Mad Cow Threat Was Predicted https://www.prwatch.org/node/4883 TSS #################### https://lists.aegee.org/bse-l.html #################### ##################### Bovine Spongiform Encephalopathy ##################### Subject: DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES Date: June 17, 2006 at 6:56 pm PST Greetings list members, here i go again. i must bring those mad sheep of mad river valley up again. what about those mouse bio-assays? can one of the aphis/usda lurkers on this list, can one of them please comment please? a declaration of emergency was announced ; >> Imported >> Belgium/Netherlands >> Sheep Test Results >> Background >> Factsheet >> Veterinary Services April 2002 >> APHIS > > > > snip... > >> Additional tests will be conducted to determine >> exactly what TSE the animals haveBSE or scrapie. >> These tests involve the use of bioassays that consist >> of injecting mice with tissue from the infected animals >> and waiting for them to develop disease. This testing >> may take at least 2 to 3 years to complete. > > > > http://www.aphis.usda.gov/lpa/pubs/fsheet_faq_notice/fs_ahvtsheeptr.pdf > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES > > http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-31 > > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [2] > > http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32 > > > or if those old urls dont work, go here; > > DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E > (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES > - Terry S. > Singeltary Sr. 7/20/00 (0) > > [Federal Register: July 20, 2000 (Volume 65, Number 140)] [Notices] > [Page 45018] >From the Federal Register Online via GPO Access > [wais.access.gpo.gov] [DOCID:fr20jy00-32] > > ----------------------------------------------------------------------- > > DEPARTMENT OF AGRICULTURE > > Office of the Secretary > > [Docket No. 00-072-1] > > Declaration of Extraordinary Emergency Because of an Atypical > Transmissible Spongiform Encephalopathy (Prion Disease) of Foreign Origin > > A transmissible spongiform encephalopathy (TSE) (prion disease) of > foreign origin has been detected in the United States. It is different > from TSE's previously diagnosed in the United States. The TSE was > detected in the progeny of imported sheep. The imported sheep and > their progeny are under quarantine in Vermont. Transmissible > spongiform encephalopathies are degenerative fatal diseases that can > affect livestock. TSE's are caused by similar, as yet uncharacterized, > agents that usually produce spongiform changes in the brain. > Post-mortem analysis has indicated positive results for an atypical > TSE of foreign origin in four sheep in Vermont. Because of the > potentially serious consequences of allowing the disease to spread to > other livestock in the United States, it is necessary to seize and > dispose of those flocks of sheep in Vermont that are affected with or > exposed to the disease, and their germ plasm. The existence of the > atypical TSE of foreign origin represents a threat to U.S. livestock. > It constitutes a real danger to the national economy and a potential > serious burden on interstate and foreign commerce. The Department has > reviewed the measures being taken by Vermont to quarantine and > regulate the flocks in question and has consulted with appropriate > officials in the State of Vermont. Based on such review and > consultation, the Department has determined that Vermont does not have > the funds to compensate flock owners for the seizure and disposal of > flocks affected with or exposed to the disease, and their germ plasm. > Without such funds, it will be unlikely to achieve expeditious > disposal of the flocks and germ plasm. Therefore, the Department has > determined that an extraordinary emergency exists because of the > existence of the atypical TSE in Vermont. This declaration of > extraordinary emergency authorizes the Secretary to seize, quarantine, > and dispose of, in such manner as he deems necessary, any animals that > he finds are affected with or exposed to the disease in question, and > their germ plasm, and otherwise to carry out the provisions and > purposes of the Act of July 2, 1962 (21 U.S.C. 134-134h). The State of > Vermont has been informed of these facts. > > Dated: This declaration of extraordinary emergency shall become > effective July 14, 2000. Dan Glickman, Secretary of Agriculture. [FR > Doc. 00-18367 Filed 7-19-00; 8:45 am] BILLING CODE 3410-34-P > > http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32 ================================ > [Federal Register: July 20, 2000 (Volume 65, Number 140)] [Notices] > [Page 45018] >From the Federal Register Online via GPO Access > [wais.access.gpo.gov] [DOCID:fr20jy00-31] > > ======================================================================== > Notices Federal Register > ________________________________________________________________________ > > This section of the FEDERAL REGISTER contains documents other than > rules or proposed rules that are applicable to the public. Notices of > hearings and investigations, committee meetings, agency decisions and > rulings, delegations of authority, filing of petitions and > applications and agency statements of organization and functions are > examples of documents appearing in this section. > > ======================================================================== > > [[Page 45018]] > > ----------------------------------------------------------------------- > > DEPARTMENT OF AGRICULTURE > > Office of the Secretary > > [Docket No. 00-072-2] > > Declaration of Emergency Because of an Atypical Transmissible > Spongiform Encephalopathy (Prion Disease) of Foreign Origin > > A transmissible spongiform encephalopathy (TSE) (prion disease) of > foreign origin has been detected in the United States. It is different > from TSE's previously diagnosed in the United States. The TSE was > detected in the progeny of imported sheep. The imported sheep and > their progeny are under quarantine in Vermont. Transmissible > spongiform encephalopathies are degenerative fatal diseases that can > affect livestock. TSE's are caused by similar, as yet uncharacterized, > agents that usually produce spongiform changes in the brain. > Post-mortem analysis has indicated positive results for an atypical > TSE of foreign origin in four sheep in Vermont. Because of the > potentially serious consequences of allowing the disease to spread to > other livestock in the United States, it is necessary to seize and > dispose of those flocks of sheep in Vermont that are affected with or > exposed to the disease, and their germ plasm. The existence of the > atypical TSE of foreign origin represents a threat to U.S. livestock. > It constitutes a real danger to the national economy and a potential > serious burden on interstate and foreign commerce. APHIS has > insufficient funds to carry out the seizure and disposal of animals > and germ plasm necessary to eliminate this disease risk. These funds > would be used to compensate the owners of the animals and germ plasm > for their seizure and disposal in accordance with 21 U.S.C. 134a. > Therefore, in accordance with the provisions of the Act of September > 25, 1981, as amended (7 U.S.C. 147b), I declare that there is an > emergency that threatens the livestock industry of this country and > hereby authorize the transfer and use of such funds as may be > necessary from appropriations or other funds available to agencies or > corporations of the United States Department of Agriculture to seize > and dispose of animals that are affected with or exposed to this TSE, > and their germplasm, in accordance with 21 U.S.C. 134a. > > Dated: This declaration of emergency shall become effective July 14, > 2000. Dan Glickman, Secretary of Agriculture. [FR Doc. 00-18368 Filed > 7-19-00; 8:45 am] BILLING CODE 3410-34-P > > I was told that ; > > > -------- Original Message -------- > Subject: Re: hello Dr. Sutton...question please...scrapie...TSS > Date: Thu, 20 May 2004 14:36:09 -0400 > From: Jim.D.Rogers@aphis.usda.gov > To: flounder@wt.net snip... FULL TEXT AND THREAD BETWEEN TSS, MAFF, USDA AND DR. DETWILER HERE ; https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044eb1f/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument Greetings again BSE-L members, NOW, i cannot for the life of me figure out why we have not heard anything about those mouse bio-assays of those mad sheep of mad river valley, and atypical TSE ? i mean hell, there was a DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES and we never hear of final results, is this not another case of the TEXAS BSE PROTOCOLS of just never confirming anything unless the GAO gets involved? maybe USDA could comment on this now? or is this too like those WMD, just something that never existed? i know Dr. Detwiler is out of the loop on this now, but there are others here that could answer this question if they wanted too and or could??? QUOTE ; 1998 Dr. Detwiler replied. "There is new research which shows that sheep can contract BSE" ......"information I can't divulge".....end WHY, after some 7 years, do we still not have any answers ??? WHERE are those mouse bio-assays ??? PLEASE look on every shelf, maybe same one that those TEXAS MAD COW tissue samples were left on for 7+ months before finally confirming after a Congressional order and or end around, they could be there. ... still disgusted in sunny Bacliff, Texas Terry S. Singeltary Sr. FSA 06/06/03 AGENDA 3.1, 15 JUNE 2006 ATYPICAL SCRAPIE IN SMALL RUMINANTS: CONSIDERATION OF THE CURRENT PRECAUTIONARY RISK MANAGEMENT MEASURES Executive Summary 1. This paper provides information on atypical scrapie (a transmissible spongiform encephalopathy (TSE)) in sheep and goats and the precautionary measures currently in place to protect consumers from the possible risks from TSEs in these species. There are a great many unknowns about atypical scrapie, including the potential implications, if any, for human health. 2. It also reports on the views of stakeholders and consumer focus groups who were asked whether, in the light of this uncertainty, additional precautionary measures were needed and for their views on the Agency’s advice on this subject. 3. The Board is asked to: • note that the Agency’s advice has been reworded to take account of the views of stakeholders and the consumer focus groups and will be tested further • note that the background information on sheep TSEs on the Agency’s website will be reviewed • note that the agricultural departments are planning to review the Ram Genotyping Scheme • note that surveillance for atypical scrapie will be maintained in order to detect any changes in prevalence. • agree that the Agency’s advice and recommendations on precautionary measures should be kept under review and be brought back to the Board if there are significant changes in the understanding of the risk. • agree that developments on atypical scrapie be kept under review to enable contingency policy to be refined as new information emerges. • agree that the Agency should open discussions with the European Commission on the issue of the identification of meat from older sheep or goats and natural sausage casings made from sheep intestines to enable consumer choice. 2 TSE DIVISION Contacts: Alison Gleadle Tel: 020 7276 8303 Email: alison.gleadle@foodstandards.gsi.gov.uk Irene Hill Tel: 020 7276 8324 Email: irene.hill@foodstandards.gsi.gov.uk 3 FSA 06/06/03 AGENDA ITEM 3.1, 15 JUNE 2006 ATYPICAL SCRAPIE IN SHEEP AND GOATS: CONSIDERATION OF THE CURRENT PRECAUTIONARY RISK MANAGEMENT MEASURES Issue 1. To consider whether the Agency should recommend, on the basis of current evidence, that additional precautionary measures are needed to reduce the possible risk to consumers from atypical scrapie....... snip... Conclusions 27. Atypical scrapie is definitely present in the UK flock, and in the flocks of other Member States (MS), and animals with atypical scrapie have, and will be, entering the food supply. However it is not known if this constitutes any risk to human health. Unlike the situation when BSE was first discovered in cattle, precautionary measures are already in place. Based on the limited knowledge of the distribution of infectivity in atypical scrapie, the SEAC Subgroup concluded that the SRM requirements that were put in place on a precautionary basis for BSE in sheep may provide at least a similar level of protection against the possible risk from atypical scrapie. 28. The consideration of the proportionality of any additional precautionary measures is very difficult when the human health risk is unknown, and, as reported by SEAC, there is insufficient data to carry out a risk assessment. 29. Any additional precautionary measures that could be put in place have a high economic cost, are currently highly impractical (see Annex 1 for details) and would impose a cost on industry that would, according to industry stakeholders, be likely to bring into question the economic viability of sheep farming. ... snip... full text ; http://www.food.gov.uk/multimedia/pdfs/fsa060603.pdf FSA 06/06/04 AGENDA ITEM 3.2, 15 JUNE 2006 BSE AND SHEEP CONTINGENCY POLICY Executive Summary 1. This paper asks the Board to agree, for purposes of contingency planning, a possible approach to a graduated strengthening of measures to protect consumers in response to one or more findings of BSE in the current UK sheep flock. 2. The paper also notes the high level of uncertainty around estimates of the possible risk from BSE in sheep and that, if BSE were ever found in a UK sheep, the estimate of the risk to consumers would depend on the accumulated results of surveillance for BSE in sheep up to that time. It therefore recommends that the policy be kept under review and that any policy agreed now on a contingency basis should urgently be reconfirmed taking into account the circumstances at the time of any finding of BSE in a UK sheep. 3. The Board is invited to: • note that, in the event of confirmation of BSE in a sheep, targeted testing of animals in the affected flock or flocks would be carried out to assist in determining the potential spread of the disease and whether it may have entered the food supply (paragraph 9). • agree that an expert group be set up to advise on what additional surveillance should be put in place, if BSE were to be found in a UK sheep, to improve estimates of prevalence of BSE in UK sheep (paragraph 13). • agree that, on current knowledge, it would advise the following graduated response to one or more findings of BSE in the current UK sheep flock: • one finding of BSE in sheep - remove additional SRM; • two findings of BSE in unrelated flocks - exclude sheep aged over 12 months from the food supply and remove the additional SRM from the remaining sheep; • three findings of BSE in unrelated flocks - allow into the food supply only sheep that were either genetically resistant to BSE or semi-resistant and aged under 12 months and remove the additional SRM from those sheep (paragraph 20). 2 • agree that its contingency policy for a finding of BSE in sheep should be kept under review and be urgently reconfirmed should BSE actually be found in a UK sheep (paragraph 22). • comment on the outline handling plan at Annex F and the strategy for the external communication that would be needed (paragraph 30). TSE Division Contacts: Alison Gleadle Tel: 020 7276 8303 (GTN 7276 8303) Email: alison.gleadle@foodstandards.gsi.gov.uk David Carruthers Tel: 020 7276 8305 (GTN 7276 8305) Email: david.carruthers@foodstandards.gsi.gov.uk snip... http://www.food.gov.uk/multimedia/pdfs/fsa060604.pdf Subject: REPORT OF THE COMMITTEE ON SCRAPIE November 9, 2005 USAHA Date: February 12, 2006 at 1:03 pm PST REPORT OF THE COMMITTEE ON SCRAPIE Chair: Dr. Jim Logan, Cheyenne, WY Vice Chair: Dr. Joe D. Ross, Sonora, TX Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN. The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance. The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings. Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories. For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory’s certification was revoked by APHIS-VS. To reduce/eliminate these problems, the Program has placed additional quality requirements on the testing laboratories: additional review of final reports, additional coding systems for testing operations, strict follow-up and reports to NVSL on corrective actions, dual data entry systems, and more frequent inspections. The Agricultural Research Services (ARS) Scrapie Research Update was given by Janet Alverson, USDA- ARS. Dr. Alverson reported on the effect of multiple births and fetal position within the uterus on PrP-Sc accumulation in fetal cotyledons. Fetal cotyledons of fetuses with resistant genotypes can accumulate PrP-Sc when positioned next to a fetus of susceptible genotype with cotyledons positive for PrP-Sc accumulation. Scrapie Surveillance Evaluation Working Group Update was presented by Tracey Lynn, Epidemiologist with the National Surveillance Unit, Center for Epidemiology and Animal Health (CEAH). The presentation provided a background on evaluation, a quick review of analyses completed to date by the scrapie surveillance evaluation working group, and some of the preliminary findings. The process of surveillance system evaluation is undertaken to assist a disease control program with identifying possible improvements to their surveillance system, and includes an assessment of the overall utility of the system, identification of potential gaps in coverage, and an evaluation of the overall performance of the system. The scrapie surveillance evaluation working group reviewed the structure and processes of the scrapie surveillance program, as well as various quality and effectiveness measures. Overall, 98-99% of surveillance samples come from the Regulatory Scrapie Surveillance System (RSSS), so the RSSS system has been the primary focus of the evaluation process. The working group developed a flow chart indicating the flow of sheep through RSSS, which identified potential gaps in surveillance coverage, including custom kill plants and sheep being exported to Mexico. Spatial analyses can assist in identifying areas with high density sheep populations with lower levels of RSSS sampling. Identification compliance is being evaluated by reviewing reports from slaughter plants on the proportion of animals with appropriate identification. Additional analyses remain, including defining the most appropriate economic analyses, and comparing the surveillance system with developing surveillance standards. The working group hopes to have a draft written report for review by the end of the year. Giving the Update on Scrapie Diagnostics and Susceptibility was Katherine O’Roarke, Research Microbiologist, USDA-ARS. "What’s New in Scrapie" -- Biopsy sampling of the third eyelid or tonsillar lymphoid tissue is a useful live animal test for scrapie. The biopsy sample is examined for accumulation of the abnormal prion protein using immunohistochemistry. A joint project conducted by the Veterinary Laboratory Agencies and the Moredun Institute in the United Kingdom has developed an alternative technique in which tissue is collected from the narrow band of lymphoid tissue near the rectal-anal junction. The morphology of the lymphoid follicles is similar in the tonsil, retropharyngeal lymph nodes, third eyelid, and rectal-anal mucosal tissue. A report on more than 300 sheep in the United Kingdom (UK), prepared by Drs. Lorenzo Gonzalez and Jeffrey Martin, will describe the sensitivity, specificity, and optimal collection interval for this technique in a variety of breeds of British sheep. ARS has done a preliminary evaluation of the technique in US sheep. Samples of third eyelid and rectal-mucosal tissue were collected from 56 sheep. Forty-two (42) sheep had negative biopsies at both sites; most of these sheep have been necropsied and no PrP-d was found in retropharyngeal lymph node or tonsil, showing good agreement with the antemortem biopsies. Fourteen (14) sheep had positive rectal biopsy samples; of those, only 12 had positive eyelid biopsies. These sheep will be monitored for disease development. However, the protocol is identical for all samples and it is probable that these sheep represent false negative third eyelid results. Abstracts of reports on the UK studies indicate that sensitivity of the test was 70% in the UK; similar large scale testing on US sheep is necessary. The biopsy tissue is somewhat difficult to handle in the tissue processing laboratory and adaptation to an ELISA format may improve test performance. Alexia McKnight, Assistant Professor of Radiology, University of Pennsylvania, reviewed magnetic resonance imaging (MRI) diagnostics before the committee. A synopsis containing references is attached at the end of this report. Dr. McKnight asked the question, "could MRI be a cost-effective screening test, estimated at $25-30 each with results immediately available." The committee feels that it is not practical as compared to other alternatives currently available. However, the committee expressed interest in future reference to this technology. Dr. Diane Sutton lead the Uniform Methods and Rules (UM&R) and Regulatory Issues Discussion. Several modifications to the UM&R were discussed. Eight issues were identified and communicated to the APHIS scrapie program coordinator. The committee acknowledged that APHIS and the industry is adequately addressing the year-to-year industry concerns. Dr. Kris Petrini representing the North Central United States Animal Health Association District presented five recommendations to the Committee. During the discussions regarding these recommendations it was evident that all five issues had been addressed during the year at this Committee meeting. The Committee approved a recommendation that USDA-APHIS-VS continue to provide indemnity funds for animals that have been designated for testing in Flocks Under Investigation as an alternative to third eyelid testing after consultation with the designated Scrapie Epidemiologist (DSE) and the Regional Area Epidemiologist (RAE). The 2004 Resolutions along with their responses were reviewed by the Committee. A Resolution concerning premises registration and identification was approved by the Committee and forwarded to the Committee on Nominations and Resolutions. Committee on Scrapie Status Report-Fiscal Year 2005: Cooperative State-Federal Scrapie Eradication Program Submitted by Diane Sutton, DVM and Gary Ross, DVM National Center for Animal Health Programs, APHIS, USDA In Fiscal Year 2005 the Scrapie Eradication Program focused on: (1) utilization of a genetic based approach to flock clean-up plans; (2) cleaning up infected and source flocks; (3) tracing and testing exposed animals and flocks; (4) expansion of regulatory slaughter surveillance (RSSS); (5) conducting considtent state reviews, (6) producer education; (7) upgrading of the Scrapie National Generic Database and (8) publishing the updated Scrapie Eradication Uniform Methods and Rules (UM&R). The current Scrapie Eradication UM&R is posted at http://www.aphis.usda.gov/vs/nahps/scrapie/umr-scrapie-erad.pdf. Consistent State Reviews States must meet the requirements in 9 CFR 79.6 in order to move sheep and goats in interstate commerce with minimal restrictions. Twenty seven states have enacted the required identification rules, the remaining states have submitted a work plan that describes the steps that will be taken to comply and provided a timeline for completing significant milestones. USDA is conducting onsite scrapie program consistent state reviews and has completed reviews in 12 states. States must be in full compliance by the end of their current rule making cycle. States not in full compliance at that time will be removed from the consistent state list. Removal from the list would create a significant impact on the interstate movement of sheep and goats from those States. Scrapie Flock Certification Program As of September 30, 2005, there were 1,961 flocks participating in the Scrapie Flock Certification Program (SFCP). Of these flocks 188 were certified flocks, 1,770 were complete monitored flocks, and 3 were selective monitored flocks (figure 2). There were 209 flocks newly enrolled and 53 newly certified (13 with status dates in FY 2005 and 40 with status dates in previous years) in FY 2005 (figure 3). Infected and Source Flocks As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats. Regulatory Scrapie Slaughter Surveillance (RSSS) RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1, 2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below. Scrapie Testing In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9). Animal ID As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises. *This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans. http://www.usaha.org/committees/reports/2005/report-scr-2005.pdf Subject: SCRAPIE USA UPDATE AS of March 31, 2006 2 NEW CASES IN GOAT, 82 INFECTED SOURCE FLOCKS, 19 INFECTED RSSS Date: April 30, 2006 at 4:49 pm PST SCRAPIE USA UPDATE AS of March 31, 2006 2 NEW CASES IN GOAT, 82 INFECTED SOURCE FLOCKS, WITH 4 NEW INFECTED SOURCE FLOCKS IN MARCH, WITH 19 SCRAPIE INFECTED RSSS REPORTED BY NVSL http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html 12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY snip... A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf Published online before print October 20, 2005 Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102 Medical Sciences A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes ( sheep prion | transgenic mice ) Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude * *Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005) Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health. -------------------------------------------------------------------------------- Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper. A.L.D. and V.B. contributed equally to this work. To whom correspondence should be addressed. Hubert Laude, E-mail: laude@jouy.inra.fr www.pnas.org/cgi/doi/10.1073/pnas.0502296102 http://www.pnas.org/cgi/content/abstract/0502296102v1 Of greatest interest today is the BSE agent because it is the presumptive cause of new variant CJD and must be considered a demonstrated risk to human health. The scrapie agent poses a theoretical risk to human health. Today we ask you to consider the implications of two theoretical possibilities: the first, that sheep and goats in BSE countries theoretically might be infected with the BSE agent, and Professor Almond, who headed a subcommittee of the United Kingdom's Spongiform Encephalopathy Advisory Committee, has agreed to review that topic for us today. Then scrapie, which theoretically might be a human pathogen, though there's no hard evidence for that, and of course, some number of sheep and goats in many countries, including the United States, are infected with the scrapie agent. Now, let me say now that no U.S. government regulatory authority would ever knowingly permit humans or animals to be exposed to a product containing the scrapie agent, but considering the nature of the scrapie agent and the disease, we are not so naive as to think that such exposures have not already occurred. ... FULL TEXT ; http://www.fda.gov/ohrms/dockets/ac/99/transcpt/3518t2.rtf http://72.14.209.104/searchq=cache:pKJPlLI2R44J:www.fda.gov/ohrms/dockets/ac/99/transcpt/3518t2.rtf+scrapie+strains+breed+east+friesian&hl=en&gl=us&ct=clnk&cd=23 TSS #################### https://lists.aegee.org/bse-l.html ####################

flounder replied on

##################### Bovine Spongiform Encephalopathy ##################### Subject: PrP(d) accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes Date: June 19, 2006 at 8:11 am PST PrP(d) accumulation in organs of ARQ/ARQ sheep experimentally infected with BSE by peripheral routes. Lezmi S, Ronzon F, Bencsik A, Bedin A, Calavas D, Richard Y, Simon S, Grassi J, Baron T. Agence Francaise de Securite Sanitaire des Aliments (AFSSA), Lyon cedex 07, France. To study the pathogenesis of bovine spongiform encephalopathy infection in small ruminants, two Lacaune sheep with the AA136RR154QQ171 and one with the AA136RR154RR171 genotype for the prion protein, were inoculated with a brain homogenate from a French cattle BSE case by peripheral routes. Sheep with the ARQ/ARQ genotype are considered as susceptible to prion diseases contrary to those with the ARR/ARR genotype. The accumulation of disease-associated prion protein (PrP(d)) was analysed by biochemical and immunohistochemical methods. No PrP(d) accumulation was detected in samples from the ARR/ARR sheep 2 years post inoculation. In the two ARQ/ARQ sheep that had scrapie-like clinical symptoms, PrP(d) was found in the central, sympathetic and enteric nervous systems and in lymphoid organs. Remarkably, PrP(d) was also detected in some muscle types as well as in all peripheral nerves that had not been reported previously thus revealing a widespread distribution of BSE-associated PrP(d) in sheep tissues. snip... Results and Discussion For all samples analysed from the ARR/ARR sheep, no PrPd was detected by any of the three PrPd detection methods used (table 1). This result correlates with the higher genetic resistance to TSE associated with this genotype naturally affected with scrapie (Elsen et al., 1999) or orally infected with the BSE agent (Jeffrey et al., 2001). However, resistance of the ARR/ARR sheep challenged with TSE infection is not considered complete since natural scrapie cases have been reported in sheep with this genotype (Buschmann et al., 2004); (Ikeda et al., 1995) (French surveillance program; unpublished data). Furthermore, BSE has been transmitted to ARR/ARR sheep by the intra-cerebral route (Houston et al., 2003b). In both ARQ/ARQ sheep, the CNS (including retina), the lymphoid system and the autonomous nervous system were identified by each method as major sites of PrPd accumulation (table 1 ; figure 1) and were also described earlier by other groups in experimentally BSE affected sheep (Foster et al., 2001; Jeffrey et al., 2001) as well as in naturally scrapie-affected sheep (Jeffrey et al., 2001; van Keulen et al., 1999). In the CNS, the quantities of abnormal PrP, expressed as equivalent in recPrP, were estimated by ELISA at up to 13000 ng/g of brainstem tissue. Comparatively, the levels found in 13 ARQ/ARQ or ARQ/VRQ sheep clinically affected with natural scrapie averaged 40 000 ± 20 000 ng of PrPd/g of CNS tissues. Lymphoid organs accumulated lower levels of PrPd and large quantities of material were required to detect a signal by WB in the mandibular or iliac medial Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273 - 5 - lymph nodes (LN) of SB3 (Figure 1). In the spleen of SB1 and SB3, 46 and 2 ng equivalent of PrPd/g of tissue were detected, respectively. In the ileum, 232 ng equivalent of PrPd /g of tissue was detected and correlated with a higher number and size of germinal centres when compared to spleen or iliac LN. The mean quantity of PrPd in the CNS was 187 and 36 fold greater than the quantities determined respectively in spleen and in the intestine. Qualitatively, different types of PrPd deposits in the brain were identified from the frontal cortex to the lumbar spinal cord. These PrPd deposits were mainly identical to those previously identified in scrapie- or BSE-affected sheep (Gonzalez et al., 2002; Ryder et al., 2001). In the retina, PrPd accumulation was mainly detected in the ganglionar layer (1), intern (2) and extern (4) plexiform layers (numbers corresponding to the different layers in the retina, figure 2a). Interestingly, in the enteric nervous system of ARQ/ARQ sheep, PrPd was detected associated with neurons (figure 2g) as well as in the coeliac ganglia in which intraand peri-neuronal PrPd deposits were visualized (figure 2h). In the adrenal gland, two types of PrPd accumulation were observed as dense intracellular or synaptic-like deposits (figure 2i). In lymphoid organs, PrPd was detected in germinal centres of secondary lymphoid follicles, in follicular dendritic cells and in tingible body macrophages (figure 2j). PrPd was also detected in cells with a morphology consistent with macrophages in the subcapsular sinus of some lymph nodes (figure 2j, arrowhead, table 1*). These observations are in agreement with previous results obtained both in sheep naturally affected with scrapie (Ersdal et al., 2005); (Jeffrey et al., 2000); (Lezmi et al., 2001) and in experimentally BSE-infected sheep (Lezmi et al., 2001); (Jeffrey et al., 2001). Interestingly, not all germinal centres were labelled for PrPd; this partial absence of labelling in germinal centres (as in tonsils) was not observed in samples from 13 natural scrapie-infected sheep in which all lymphoid germinal centres were positively labelled for PrPd. This agreed with data describing an early and systematic immune system involvement in lambs affected with scrapie (Andreoletti et al., 2000) which was not a Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273 - 6 - feature of BSE agent infection in sheep during the first passage (Jeffrey et al., 2001; Martin et al., 2005). In our study, as opposed to previous published results, in both ARQ/ARQ sheep, PrPd was detected by IHC in all motor nerves and associated with Schwann’s cells (figure 2d, e). PrPd deposits were similarly detected in all other tissue samples containing peripheral nerves, most notably in nerves in muscle samples. This observation was not reported in other studies with sheep BSE (Foster et al., 2001; Jeffrey et al., 2001). However, we observed the same type of deposits in two other sheep (ARQ/VRQ) naturally affected with scrapie (data not shown) and two previous articles report similar data in sheep with natural scrapie (Archer et al., 2004); (Groschup et al., 1999). PrPd presence was also identified in striated muscles for both ARQ/ARQ sheep. These deposits were associated with neuromuscular spindles that are highly innervated structures made of groups of myocytes surrounded by a thin fibrous capsule (figure 2k, l) and are a specialized subset of myocytes implicated in proprioception. In the tongue of sheep, the accumulation of PrPd in these structures was less evident. Only one study reported the PrPd presence in the muscle of sheep affected with scrapie using IHC and ELISA (Andreoletti et al., 2004). Here, sampling and analysis of different muscles were not systematic and thus the ELISA/IHC results were not correlated. However, the accumulation in muscle tissue of PrPd in sheep affected with scrapie is not systematic (Andreoletti et al., 2004). Recently, pathological prion protein was detected in muscles of hamsters and mice infected with rodentadapted BSE or vCJD (Thomzig et al., 2006). Previously, other studies failed to detect prion in nerves and muscles of BSE- or scrapie-infected sheep (Foster et al., 2001); (Hamir et al., 2004) possibly relying on the use of different pre-treatments and antibodies. In conclusion, we have shown that the inoculation of the BSE agent of French origin by peripheral routes to Lacaune sheep lead to the development of the clinical disease only in Copyright @ Acta Biochimica Polonica, Paper in Press, No. 1273 - 7 - ARQ/ARQ sheep. The distribution of PrPd in ARQ/ARQ sheep infected with BSE was very similar to that described in natural scrapie. Overall, we demonstrated for the first time the presence of PrPd in muscles and nerves of sheep infected experimentally with BSE agent, which stresses the potential risk for humans related to consumption of sheep products from sheep naturally infected with BSE. ... snip...end...TSS http://www.actabp.pl/pdf/Preprint/20061273.pdf TSS #################### https://lists.aegee.org/bse-l.html ####################

tshell replied on

I found this excerpt tremendously informative. I understand firewall feeding firsthand, and let me add that this is still ongoing, never will stop because it is cost effective. I related to the last excerpt personally...I am no longer in the biz, but we can all never really let things go and leave them behind.

Terry S. Singel... replied on

Sent: Thursday, November 18, 2010 1:44 PM Subject: UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS 4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 1 of 4 - Page ID #1 FILED U.S. DISTRICT COURT DISTRICT OF NEBRASKA 10 NOV 16 PM 4:16 OFFICE OF THE CLERK IN THE UNITED STATES DISTRICT COURT FOR THE DISTRICT OF NEBRASKA UNITED STATES OF AMERICA, Plaintiff, vs. GALEN J. NIEHUES, Defendant. INDICTMENT 18 U.S.C. § 1001 18 U.S.C. § 1341 The Grand Jury charges that: INTRODUCTION At all times material to this Indictment: I. The U.S. Food and Drug Administration (FDA) was an agency within the U.S. Department of Health and Human Services, a part of the executive branch of the Government of the United States. 2. The FDA provided $250,000.00 in grant funds annually to the Nebraska Department of Agriculture (NDA) to perform inspections at cattle producing sites within the state to detect and identify the possible existence of Bovine Spongiform Encephalopathy (BSE), also known as "Mad Cow Disease". 3. The FDA published a final regulation designed to prevent the spread ofBSE through animal feed. The rule prohibited use of most mammalian protein, such as meat meal, bone meal, and hydrolyzed hair, in the manufacture of animal feeds given to cows, sheep and goats. The regulation also required process and control systems to ensure that feed for these animals does not contain prohibited mammalian tissue. 4. Defendant Galen J. Niehues was employed by NDA between approximately July 1, 2009 and March 31, 2010. Niehues' job duties included identifying cattle producers, performing on -1- 4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 2 of 4 - Page ID # 2 site inspections of the farm site and cattle operations, interviewing cattle producers, taking samples of cattle feed and submitting the feed samples for laboratory analysis for the possible presence of BSE contaminants, and completing reports to document his on-site inspections and feed sample collection. 5. Between approximately July 1,2009 and March 31, 2010, Defendant Galen J. Niehues submitted reports he had completed and feed samples to NDA, for approximately 100 cattle operations within Nebraska as well as travel, per diem, lodging and miscellaneous expenses, by delivering-and placing the reports and related documents and forms to post offices in Lexington, Nebraska and Cozad, Nebraska, to be sent or delivered by the postal service to NDA in Lincoln, Nebraska, and knowingly causing the reports, documents and forms to be delivered by mail. 6. During the time of his employment, Defendant Galen J. Niehues was paid a total of $35,409.65 by NDA in salary and benefits. 7. Title 18 USC §IOOI (False Statement) prohibits anyone, in any matter, within the executive branch of the government of the United States, from making or using any false writing or document, knowing the same to contain any materially false, fictitious, or fraudulent statement or entry. 8. Title 18 USC § 1341 (Mail Fraud) prohibits anyone, having devised or intended to devise a scheme or artifice to defraud, or for obtaining money or property by means of materially false pretenses, representations, or promises, for the purpose of executing such scheme or artifice or attempting to do so, from placing in any post office or authorized depository for mail, any written matter or thing to be sent or delivered by the postal service, or knowingly causing any such matter or thing to be delivered by mail. -2- 4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 3 of 4 - Page ID # 3 COUNT 1 - FALSESTATEMENT 9. Paragraphs 1 - 8 of this Indictment are realleged and incorporated as fully set forth herein. 10. On or about September 10, 2009, in the District of Nebraska, Defendant Galen Niehues, did knowingly and intentionally make, in a matter within the jurisdiction of the U.S. Department of Health and Human Services, a false BSE report, #EIR 3007935397, knowing the same to contain a materially false and fictitious statement in that the information and data contained in the reporthe prepared and submitted to NDA represented he had made contact with and interviewed the cattle owner, inspected the cattle owner's premises, and collected a sample of cattle feed, when in fact, Defendant Niehues, at the time he submitted the report on or about September 10,2009, then and there knew he had not made contact nor interviewed the owner or anyone associated with the cattle operation, nor had he obtained a sample of feed from the cattle operation as he represented in his report. In violation of Title 18 United States Code §1001 COUNT II - MAIL FRAUD 11. Paragraphs 1 - 8 of this Indictment are realleged and incorporated as fully set forth herein. 12. Between approximately July 1, 2009, and March 31, 20 10, in the District of Nebraska, and elsewhere, Defendant Galen J. Niehues, devised and intended to devise a scheme and artifice to defraud, and to obtain money and property by means of material and false pretenses and representations. 13. It was part of the scheme and artifice to defraud, that in connection with his job duties and responsibilities with NDA, Defendant Galen 1. Niehues completed and submitted -3- 4:10-cr-03119-RGK -CRZ Doc # 1 Filed: 11/16/10 Page 4 of 4 Page ID # 4 approximately 92 BSE inspection reports and related documents which he knew contained materially false, fictitious and fraudulent statements. Specifically, the inspection reports were materially false, in that, they represented that the defendant had made contact with and interviewed the cattle owners, inspected the cattle owners' premises, and collected samples of cattle feed, when, in fact, he had not. 14. On or about September 11, 2009, Defendant Galen J. Niehues, as part of his scheme and artifice to defraud, and for the purpose of executing such scheme and artifice, and attempting to do so, placed copies of fabricated BSE reports and related documents in a post office and authorized depository for mail matter, in Cozad, Nebraska, to be sent and delivered by the postal service to NDJ\. in Lincoln, Nebraska, knowing such reports and related documents were to be delivered by mail. In violation of Title 18 United States Code §1341. The United States of America requests that trial of this case be held at Lincoln, Nebraska, pursuant to the rules of this Court. WILLIAM W. MICKLE, II Assistant United States Attorney FILED U.S. DISTRICT COURT DISTRICT OF NEBRASKA 10 NOV 16 PM 4:17 OFFICE OF THE CLERK -4-...END...TSS please see more here, with other bungled and blundered mad cow testing done ; Wednesday, November 17, 2010 MAD COW TESTING FAKED IN USA BY Nebraska INSPECTOR Senator Mike Johanns STATE http://madcowtesting.blogspot.com/2010/11/mad-cow-testing-faked-in-usa-by.html Tuesday, November 02, 2010 IN CONFIDENCE The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW". BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992 http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html Thursday, November 18, 2010 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html Seven main threats for the future linked to prions The NeuroPrion network has identified seven main threats for the future linked to prions. First threat The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed. *** Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases. Second threat In small ruminants, a new atypical form of scrapie currently represents up to 50% of detected cases and even involves sheep selected for resistance to classical scrapie. The consequences for animal and human health are still unknown and there may be a potential connection with atypical BSE. These atypical scrapie cases constitute a second threat not envisioned previously which could deeply modify the European approach to prion diseases. Third threat The species barrier between human and cattle might be weaker than previously expected and the risk of transmission of prion diseases between different species has been notoriously unpredictable. The emergence of new atypical strains in cattle and sheep together with the spread of chronic wasting disease in cervids renders the understanding of the species barrier critical. This constitutes a third threat not properly envisioned previously that could deeply modify the European approach to prion diseases. Fourth threat Prion infectivity has now been detected in blood, urine and milk and this has potential consequences on risk assessments for the environment and food as well as for contamination of surfaces including medical instruments. Furthermore the procedures recommended for decontamination of MBM (Meat and Bone Meal), which are based on older methodologies not designed for this purpose, have turned out to be of very limited efficacy and compromise current policies concerning the reuse of these high value protein supplements (cross-contamination of feed circuits are difficult to control). It should be noted that the destruction or very limited use of MBM is estimated to still cost 1 billion euros per year to the European economy, whereas other countries, including the US, Brazil, and Argentine do not have these constraints. However, many uncertainties remain concerning the guarantees that can be reasonably provided for food and feed safety and scientific knowledge about the causative agents (prions) will continue to evolve. This decontamination and environmental issue is a fourth threat that could modify deeply the European approach to prion diseases. Fifth threat The precise nature of prions remains elusive. Very recent data indicate that abnormal prion protein (PrPTSE) can be generated from the brains of normal animals, and under some conditions (including contaminated waste water) PrPTSE can be destroyed whereas the BSE infectious titre remains almost unchanged, a finding that underlines the possibility of having BSE without any detectable diagnostic marker. These are just two areas of our incomplete knowledge of the fundamental biology of prions which constitute a fifth threat to the European approach to prion diseases. Sixth threat The absence of common methods and standardisation in the evaluation of multiple in vivo models with different prion strains and different transgenic mice expressing PrP from different species (different genotypes of cattle, sheep, cervids, etc) renders a complete and comprehensive analysis of all the data generated by the different scientific groups almost impossible. This deeply impairs risk assessment. Moreover, the possibility of generating PrPTSE de novo with new powerful techniques has raised serious questions about their appropriateness for use as blood screening tests. The confusion about an incorrect interpretation of positive results obtained by these methods constitutes a sixth threat to European approach to prion diseases. Seventh Threat The detection of new or re-emerging prion diseases in animals or humans which could lead to a new crisis in consumer confidence over the relaxation of precautionary measures and surveillance programmes constitutes a seventh threat that could modify the European approach to prion diseases. http://www.neuroprion.org/en/np-neuroprion.html Thursday, August 12, 2010 Seven main threats for the future linked to prions http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html http://prionpathy.blogspot.com/ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation. http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156 http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF Saturday, August 14, 2010 BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS) http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html Wednesday, July 28, 2010 re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010 http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html Monday, August 9, 2010 Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ? http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html PLEASE SEE the dramatic increase in sporadic CJD cases in documented BSE countries, then think, BSE can propagate as nvCJD and sporadic CJD in the lab ; TOTAL CASES OF SPORADIC CJD (DEATHS) DEFINITE AND PROBABLE CASES Australia Austria Canada France Germany Italy Netherlands Slovakia Spain Switzerland UK http://www.eurocjd.ed.ac.uk/sporadic.htm USA 5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded. 2010 PLEASE NOTE REFERENCE LINES 5. AND 6. Monday, August 9, 2010 National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010) Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD 1996 & earlier 51 33 28 5 0 0 1997 114 68 59 9 0 0 1998 88 52 44 7 1 0 1999 120 72 64 8 0 0 2000 146 103 89 14 0 0 2001 209 119 109 10 0 0 2002 248 149 125 22 2 0 2003 274 176 137 39 0 0 2004 325 186 164 21 0 1(3) 2005 344 194 157 36 1 0 2006 383 197 166 29 0 2(4) 2007 377 214 187 27 0 0 2008 394 231 204 25 0 0 2009 425 259 216 43 0 0 2010 204 124 85 20 0 0 TOTAL 3702(5) 2177(6) 1834 315 4 3 1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case; 5 Includes 16 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 21 (19 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded. http://www.cjdsurveillance.com/pdf/case-table.pdf Monday, August 9, 2010 National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010) (please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi) http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html Atypical BSE in Cattle BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD. To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures. snip...see full text ; http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2 14th ICID International Scientific Exchange Brochure - Final Abstract Number: ISE.114 Session: International Scientific Exchange Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009 T. Singeltary Bacliff, TX, USA Background: An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades. Methods: 12 years independent research of available data Results: I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. Conclusion: I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. page 114 ; http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653 BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1 1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to: John Collinge, E-mail: j.collinge@prion.ucl.ac.uk Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002 -------------------------------------------------------------------------------- Abstract Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure. Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic http://www.nature.com/emboj/journal/v21/n23/abs/7594869a.html BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: j.collinge@prion.ucl.ac.ukReceived August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed TSS

Clark replied on

The common denominator in all of these [transmissible mink encephalopathy - TME] outbreaks was either “cattle” or “unknown.” It was possible, of course, to imagine other scenarios, but Marsh believed he had at least strong circumstantial evidence that a TSE similar to mad cow disease already existed in U.S. cattle. “You can trace it back to feed real easy in mink,” Marsh said. “And then you’re left with the question, what was it in the feed that affected them? And what we find is it’s these downer cows that are the common link. You don’t have to be a genius to figure it out.”

Church Construction replied on

Many thanks for taking the time to discuss this, Also, the military apparently knew about this for at least a few years now to because they did some testing on patients in a veterans hospital a few years back that were originally diagnosed as having alzheimer’s disease, because the symptoms are nearly identical to those of alzheimers. If I recall correctly, somewhere around 6% of the patients that they thought had died of alzheimers had actually died of the human form of mad cow disease instead. The reason that this seems to be such an unknown is that there is no set policy for testing alzheimers patients for mad cow disease at the time of their death. For those that don't know, the only definitive test for mad cow is to take a tissue sample from the affected areas and it is quite an expensive procedure. Until the government starts some sort of policy for testing alzheimers patients for mad cow disease at the time of their death, we may never know exactly how prevalent this problem really is in this country. thanks for the sharing / <a href="http://www.bigskyllc.com target=_blank" rel="follow">Church Construction </a>

Terry S. Singel... replied on

How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed. However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons. “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke. BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded. It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said. http://www.washingtonpost.com/national/health-science/case-of-mad-cow-disease-found-in-california-animal-but-food-supply-said-safe/2012/04/24/gIQAtelqfT_story.html counterpart: Creutzfeldt-Jakob disease (CJD). In a statement released on 24 April, Karen Ross, Secretary of the California Department of Food and Agriculture said, “The detection of BSE shows that the surveillance program in place in California and around the country is working.” Food safety advocates such as Yonkers, New York, -based Consumers Union say it’s a warning sign that surveillance is inadequate and needs to be stepped up. Ross’s statement also makes a point of noting a key feature of this particular case: The infected cow carried what is known as ‘L-type’ BSE, a version of the disease that has not been detected before in the US and has so far not been associated with transmission through animal feed. As the policy debate over testing rumbles on, here is a short guide to what is known and not known about this rare strain and its unexpected appearance. http://blogs.nature.com/news/2012/04/california-bse-prion-comes-with-a-different-twist.html STATEMENT FROM CDFA SECRETARY KAREN ROSS ON USDA ANNOUNCEMENT OF DETECTION OF BSE Release #12-016 Print This Release SACRAMENTO, April 24, 2012 – CDFA Secretary Karen Ross issued this statement following the USDA’s announcement of the detection of atypical bovine spongiform encephalopathy (BSE) in a California dairy cow: “The detection of BSE shows that the surveillance program in place in California and around the country is working. Milk and beef remain safe to consume. The disease is not transmitted through milk. Because of the strength of the food protection system, the cow did not enter the food or feed supply. There are numerous safeguards in place to prevent BSE from entering the food chain. “The atypical BSE designation is important because this is a very rare form of BSE not generally associated with an animal consuming infected feed. CDFA veterinarians are working with the USDA to investigate this case and to identify whether additional cows are at risk. Feed restrictions in place in California and around the country for the last 15 years minimize that risk to the greatest degree possible. We will provide additional information about this case as it becomes available.” The USDA announcement may be viewed at: http://www.usda.gov/wps/portal/usda/usdahome?contentid=2012/04/0132.xml&contentidonly=true http://www.cdfa.ca.gov/egov/Press_Releases/Press_Release.asp?PRnum=12-016 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, ... http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006 In an article today for United Press International, science reporter Steve Mitchell writes: Analysis: What that mad cow means By STEVE MITCHELL UPI Senior Medical Correspondent WASHINGTON, March 15 (UPI) -- The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade. The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old. 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. "Everything they did on the Texas cow makes everything they did before 2005 suspect," Brown said. Despite this, Brown said the U.S. prevalence of mad cow, formally known as bovine spongiform encephalopathy, or BSE, did not significantly threaten human or cattle health. "Overall, my view is BSE is highly unlikely to pose any important risk either in cattle feed or human feed," he said. However, Jean Halloran of Consumers Union in Yonkers, N.Y., said consumers should be troubled by the USDA's secrecy and its apparent plan to dramatically cut back the number of mad cow tests it conducts. "Consumers should be very concerned about how little we know about the USDA's surveillance program and the failure of the USDA to reveal really important details," Halloran told UPI. "Consumers have to be really concerned if they're going to cut back the program," she added. Last year the USDA tested more than 300,000 animals for the disease, but it has proposed, even in light of a third case, scaling back the program to 40,000 tests annually. "They seem to be, in terms of actions and policies, taking a lot more seriously the concerns of the cattle industry than the concerns of consumers," Halloran said. "It's really hard to know what it takes to get this administration to take action to protect the public." The USDA has insisted that the safeguards of a ban on incorporating cow tissue into cattle feed (which is thought to spread the disease) and removal of the most infectious parts of cows, such as the brain and spinal cord, protect consumers. But the agency glosses over the fact that both of these systems have been revealed to be inadequately implemented. The feed ban, which is enforced by the Food and Drug Administration, has been criticized by the Government Accountability Office in two reports, the most recent coming just last year. The GAO said the FDA's enforcement of the ban continues to have weaknesses that "undermine the nation's firewall against BSE." USDA documents released last year showed more than 1,000 violations of the regulations requiring the removal of brains and spinal cords in at least 35 states, Puerto Rico and the Virgin Islands, with some plants being cited repeatedly for infractions. In addition, a violation of similar regulations that apply to beef exported to Japan is the reason why Japan closed its borders to U.S. beef in January six weeks after reopening them. Other experts also question the adequacy of the USDA's surveillance system. The USDA insists the prevalence of mad cow disease is low, but the agency has provided few details of its surveillance program, making it difficult for outside experts to know if the agency's monitoring plan is sufficient. "It's impossible to judge the adequacy of the surveillance system without having a breakdown of the tested population by age and risk status," Elizabeth Mumford, a veterinarian and BSE expert at Safe Food Solutions in Bern, Switzerland, a company that provides advice on reducing mad cow risk to industry and governments, told UPI. "Everybody would be happier and more confident and in a sense it might be able to go away a little bit for (the USDA) if they would just publish a breakdown on the tests," Mumford added. UPI requested detailed records about animals tested under the USDA's surveillance plan via the Freedom of Information Act in May 2004 but nearly two years later has not received any corresponding documents from the agency, despite a federal law requiring agencies to comply within 30 days. This leaves open the question of whether the USDA is withholding the information, does not have the information or is so haphazardly organized that it cannot locate it. Mumford said the prevalence of the disease in U.S. herds is probably quite low, but there have probably been other cases that have so far gone undetected. "They're only finding a very small fraction of that low prevalence," she said. Mumford expressed surprise at the lack of concern about the deadly disease from American consumers. "I would expect the U.S. public to be more concerned," she said. Markus Moser, a molecular biologist and chief executive officer of Prionics, a Swiss firm that manufactures BSE test kits, told UPI one concern is that if people are infected, the mad cow pathogen could become "humanized" or more easily transmitted from person to person. "Transmission would be much easier, through all kinds of medical procedures" and even through the blood supply, Moser said. © Copyright 2006 United Press International, Inc. All Rights Reserved http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r http://www.upi.com/Science_News/2003/12/30/Mad-Cow-Linked-to-thousands-of-CJD-cases/UPI-47861072816318/ PAUL BROWN COMMENT TO ME ON THIS ISSUE Tuesday, September 12, 2006 11:10 AM \ "Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." OIE says the animal was sub-clinical ; http://web.oie.int/wahis/public.php?page=single_report&pop=1&reportid=11893 also, officials have confirmed it was a atypical L-type BASE BSE. I am deeply disturbed about the false and terribly misleading information that is being handed out by the USDA FDA et al about this recent case of the atypical L-type BASE BSE case in California. these officials are terribly misinformed (I was told they are not lying), about the risk factor and transmissibility of the atypical L-type BASE BSE. these are very disturbing transmission studies that the CDC PUT OUT IN 2012. I urge officials to come forward with the rest of this story. It is important to reiterate here, even though this animal did not enter the food chain, the fact that the USA now finds mad cow disease in samplings of 1 in 40,000 is very disturbing, and to add the fact that it was an atypical L-type BASE BSE, well that is very disturbing in itself. 1 out of 40,000, would mean that there were around 25 mad cows in the USA annually going by a National herd of 100 million (which now I don’t think the USA herd is that big), but then you add all these disturbing factors together, the documented link of sporadic CJD cases to atypical L-type BASE BSE, the rise in sporadic CJD cases in the USA of a new strain of CJD called ‘classification pending Creutzfeldt Jakob Disease’ cpCJD, in young and old, with long duration of clinical symptoms until death. the USA has a mad cow problem and have consistently covered it up. it’s called the SSS policy. ... see full text with updated transmission studies and science on the atypical L-type BASE BSE Jan. 2012 CDC. ... ***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model ***Infectivity in skeletal muscle of BASE-infected cattle ***feedstuffs- It also suggests a similar cause or source for atypical BSE in these countries. ***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. now, for the rest of the story, the most updated science on the atypical BSE strains, and transmission studies... Thursday, April 26, 2012 Update from USDA Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States WASHINGTON bulletin at 04/26/2012 10:11 PM EDT http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/update-from-usda-regarding-detection-of.html kind regards, terry Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas, USA 77518 flounder9@verizon.net

jerryn replied on

I am starting to think that the cases in Miami, USA and Canada are related. I hope the authorities are smart enough to have a biopsy done to check for a prion infection. Also there was a madman in Warwick ,RI last night that stripped naked, ran around making primal screams in the RI Mall, eventaully swam across the river, ran across 295 until getting caught and is now at a Mental hospital for observation. I think that's a rabies type symptom too. I agree, I think all people who perish from Alzheimer's and Parkinson's disease should should be tested. It makes sense. Who knows, maybe Prion infections are responsible.