Gov Walker, deer czar Kroll, some hunting groups become passive over brain rotting disease-

It’s aim for the lowest common denominator in management of Wisconsin’s huge whitetail population. The incidence of chronic wasting disease (CWD), the prion-based malady that turns deer (and elk and moose) brains into sponges has gone from a half per cent in 2002 to 5 per cent (10x increase) today.

The spread of infectious diseases requires a public strategy, but public is an idea foreign to right wing governor Scott Walker and his “deer czar” James (“public deer management is Communism”) Kroll.

We have written many stories on CWD, a close relative to mad-cow disease. So far CWD is not transmitted to humans. While venison eaters seem safe for now, the CWD causal agent is a prion. This is a misfolded protein that corrupts healthy neural proteins into more prions. The end result is disgustingly sick, terminal deer. CWD prions are a kind of ultimate pollution as well, lying in the soil for an indefinite (permanent?) time waiting for a deer or other cervid to come along. They are very resistant to natural decay.

Wisconsin outdoor writer Patrick Durkin has been sounding the alarm at Walker’s termination of the state’s CWD-eradication program. Ironically, this change leaves the state’s wolf population as the only thing in the outdoors now serving to control the spread of the disease. Wolves tend to kill the weaker animals in a herd, thus culling to a greater or lesser degree some of the CWD-addled deer.

In present day Wisconsin, like a number of other states (mostly “red” ones), it seems the number of deer, regardless of their health, is the only thing that matters politically.

The disastrous effects of this indifferent approach to the horrible wildlife disease will never be fully reversed. While it must be stressed there is no evidence that humans have ever gotten this disease, these non-living prions do change over time, and they can suddenly cross species barriers. That is what probably happened with mad-cow disease jumping to humans. A spark of hope is that newspapers in the Dairy State are covering and so magnifying alarm sounded by Durkin and others.

The ideological approach to wildlife management always results in death and decay.

– – – –

A gallery of Patrick Durkin’s books.

His article. Patrick Durkin: CWD’s spreading and herd, hunters deserve better.

 

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About The Author

Ralph Maughan

Dr. Ralph Maughan is professor emeritus of political science at Idaho State University with specialties in natural resource politics, public opinion, interest groups, political parties, voting and elections. Aside from academic publications, he is author or co-author of three hiking/backpacking guides.

33 Responses to Chronic wasting disease epidemic spreads in Wisconsin deer

  1. avatar Paul says:

    Ralph,

    Durkin makes some good points here, but don’t count on him to ever say anything positive about wolves. He is a supporter of every killing method imaginable and even wrote a column supporting the hounding of wolves. In that column he claimed that wolf/dog fights are no worse than a “goose wing-beating a Labrador.” One or two good columns aside, this guy is no friend to wildlife unless it benefits the kill everything crowd in Wisconsin. Just my opinion.

    • avatar Ralph Maughan says:

      Paul,

      I did know that about him, and in a way, it makes his criticism of the lack of action on chronic wasting disease more credible because some “hunting” groups in Wisconsin seem to have opted for “we don’t care if they (the deer) are sick, as long as there are a lot of them.”

      • avatar Paul says:

        I agree. It seems like he has multiple personalities though. A couple of weeks ago he came out with a column absolutely eviscerating the Wisconsin DNR and how they pander to the lobbying groups led by Bob “Connecticut Effect” Welch. For the first time ever I agreed with everything he had to say. I even pointed out on my site that if a “kill everything” person like Durkin is complaining about how the the WDNR operates then something must be very wrong with that agency. Then the next week he was whining about how a couple of “non-hunters” were elected to the “Conservation Congress” as if their election was some kind of “threat” to the 80 plus year hunter/trapper control of that group and their 300 plus delegates. We now have CWD in areas where it has never been,including in wolf habitat, yet the WDNR is still obsessed with getting wolves here down to that magical “350” number. This wolf hating agency is cutting off their nose to spite their face and I hope it bites them in the ass hard.

  2. avatar Carl says:

    Ralph,

    At the present time the wolves are not having an impact on the chronic wasting infected deer.
    Unfortunately the wolves are in the northern part of the state with a few packs in the central, while the disease outbreak is in the southern part of the state with a few showing up in Illinois.

    • avatar Ralph Maughan says:

      Carl,

      It has not been proven that wolves retard CWD at all, anywhere, although it seems likely that under some circumstances they could because infected deer that are showing symptoms, stagger and are not alert.

      My statement about wolves in the context of Wisconsin’s “CWD management” is mostly intended to be sarcastic — their disease management is so bad that the state’s wolves are the bright spot in it (a statement intended to sting).

      • Monday, March 26, 2012
        CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE
        OR-09 15:10 – 15:25 CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE David

        http://www.prion2012.com/program/final-program

        OR-09: Canine spongiform encephalopathy—A new form of animal prion disease

        Monique David, Mourad Tayebi UT Health; Houston, TX USA

        It was also hypothesized that BSE might have originated from an unrecognized sporadic or genetic case of bovine prion disease incorporated into cattle feed or even cattle feed contaminated with prion-infected human remains.1 However, strong support for a genetic origin of BSE has recently been demonstrated in an H-type BSE case exhibiting the novel mutation E211K.2 Furthermore, a specific prion protein strain causing BSE in cattle is believed to be the etiological agent responsible for the novel human prion disease, variant Creutzfeldt-Jakob disease (vCJD).3 Cases of vCJD have been identified in a number countries, including France, Italy, Ireland, the Netherlands, Canada, Japan, US and the UK with the largest number of cases. Naturally occurring feline spongiform encephalopathy of domestic cats4 and spongiform encephalopathies of a number of zoo animals so-called exotic ungulate encephalopathies5,6 are also recognized as animal prion diseases, and are thought to have resulted from the same BSE-contaminated food given to cattle and humans, although and at least in some of these cases, a sporadic and/or genetic etiology cannot be ruled out. The canine species seems to display resistance to prion disease and no single case has so far been reported.7,8

        Here, we describe a case of a 9 week old male Rottweiler puppy presenting neurological deficits; and histological examination revealed spongiform vacuolation characteristic of those associated with prion diseases.9 Initial biochemical studies using anti-PrP antibodies revealed the presence of partially proteinase K-resistant fragment by western blotting. Furthermore, immunohistochemistry revealed spongiform degeneration consistent with those found in prion disease and displayed staining for PrPSc in the cortex.

        Of major importance, PrPSc isolated from the Rottweiler was able to cross the species barrier transmitted to hamster in vitro with PMCA and in vivo (one hamster out of 5). Futhermore, second in vivo passage to hamsters, led to 100% attack rate (n = 4) and animals displayed untypical lesional profile and shorter incubation period.

        In this study, we show that the canine species might be sensitive to prion disease and that PrPSc isolated from a dog can be transmitted to dogs and hamsters in vitro using PMCA and in vivo to hamsters.

        If our preliminary results are confirmed, the proposal will have a major impact on animal and public health and would certainly lead to implementing new control measures for ‘canine spongiform encephalopathy’ (CSE).

        References

        1. Colchester AC, Colchester NT. The origin of bovine spongiform encephalopathy: the human prion disease hypothesis. Lancet 2005; 366:856-61; PMID:16139661; http:// dx.doi.org/10.1016/S0140-6736(05)67218-2.

        2. Richt JA, Hall SM. BSE case associated with prion protein gene mutation. PLoS Pathog 2008; 4:e1000156; PMID:18787697; http://dx.doi.org/10.1371/journal. ppat.1000156.

        3. Collinge J. Human prion diseases and bovine spongiform encephalopathy (BSE). Hum Mol Genet 1997; 6:1699-705; PMID:9300662; http://dx.doi.org/10.1093/ hmg/6.10.1699.

        4. Wyatt JM, Pearson GR, Smerdon TN, Gruffydd-Jones TJ, Wells GA, Wilesmith JW. Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats. Vet Rec 1991; 129:233-6; PMID:1957458; http://dx.doi.org/10.1136/vr.129.11.233.

        5. Jeffrey M, Wells GA. Spongiform encephalopathy in a nyala (Tragelaphus angasi). Vet Pathol 1988; 25:398-9; PMID:3232315; http://dx.doi.org/10.1177/030098588802500514.

        6. Kirkwood JK, Wells GA, Wilesmith JW, Cunningham AA, Jackson SI. Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu (Tragelaphus strepsiceros). Vet Rec 1990; 127:418-20; PMID:2264242.

        7. Bartz JC, McKenzie DI, Bessen RA, Marsh RF, Aiken JM. Transmissible mink encephalopathy species barrier effect between ferret and mink: PrP gene and protein analysis. J Gen Virol 1994; 75:2947-53; PMID:7964604; http://dx.doi.org/10.1099/0022-1317- 75-11-2947.

        8. Lysek DA, Schorn C, Nivon LG, Esteve-Moya V, Christen B, Calzolai L, et al. Prion protein NMR structures of cats, dogs, pigs, and sheep. Proc Natl Acad Sci U S A 2005; 102:640-5; PMID:15647367; http://dx.doi.org/10.1073/pnas.0408937102.

        9. Budka H. Neuropathology of prion diseases. Br Med Bull 2003; 66:121-30; PMID:14522854; http://dx.doi.org/10.1093/bmb/66.1.121.

        http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf

        Monday, March 8, 2010

        Canine Spongiform Encephalopathy aka MAD DOG DISEASE

        http://caninespongiformencephalopathy.blogspot.com/

        TSE in dogs have not been documented simply because OF THE ONLY STUDY, those brain tissue samples were screwed up too. see my investigation of this here, and to follow, later follow up, a letter from defra, AND SEE SUSPICIOUS BRAIN TISSUE SAF’s. …TSS

        http://www.mad-cow.org/00/aug00_late_news.html#ggg

        TSE & HOUNDS

        GAH WELLS (very important statement here…TSS)

        HOUND STUDY

        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://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

        76 pages on hound study;

        snip…

        http://web.archive.org/web/20030327022236/http://www.bseinquiry.gov.uk/files/sc/seac16/tab04.pdf

        The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.

        38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on ‘hound ataxia’ mirrored those in material from Robert Higgins’ hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in ‘blind’ examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.

        39.Hound ataxia had reportedly been occurring since the 1930’s, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.

        40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.

        41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.

        Histopathological support to various other published MAFF experiments

        42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).

        http://www.bseinquiry.gov.uk/witness/htm/stat067.htm

        It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.

        snip…

        http://www.bseinquiry.gov.uk/files/ws/s324.pdf

        NEW URL ;

        http://collections.europarchive.org/tna/20080102174910/http://www.bseinquiry.gov.uk/files/ws/s324.pdf

        2005

        DEFRA Department for Environment, Food & Rural Affairs

        Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk

        GTN: FAX:

        Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518

        21 November 2001

        Dear Mr Singeltary

        TSE IN HOUNDS

        Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.

        As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government’s independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.

        Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.

        Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less

        critical. For more details see- http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf

        As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.

        Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK

        You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.

        I hope this is helpful

        Yours sincerely 4

        HUGH MCDONAGH BSE CORRESPONDENCE SECTION

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

        HOUND SURVEY

        I am sorry, but I really could have been a co-signatory of Gerald’s minute.

        I do NOT think that we can justify devoting any resources to this study, especially as larger and more important projects such as the pathogenesis study will be quite demanding.

        If there is a POLITICAL need to continue with the examination of hound brains then it should be passed entirely to the VI Service.

        J W WILESMITH Epidemiology Unit 18 October 1991

        Mr. R Bradley

        cc: Mr. G A H Wells

        http://collections.europarchive.org/tna/20081106102318/http://www.bseinquiry.gov.uk/files/yb/1991/10/18001001.pdf

        3.3. Mr R J Higgins in conjunction with Mr G A Wells and Mr A C Scott would by the end of the year, indentify the three brains that were from the ”POSITIVE” end of the lesion spectrum.

        http://collections.europarchive.org/tna/20080103034308/http://www.bseinquiry.gov.uk/files/yb/1993/12/06001001.pdf

        SEE FULL TEXT ;

        http://caninespongiformencephalopathy.blogspot.com/

        Monday, February 14, 2011

        THE ROLE OF PREDATION IN DISEASE CONTROL: A COMPARISON OF SELECTIVE AND NONSELECTIVE REMOVAL ON PRION DISEASE DYNAMICS IN DEER

        NO, NO, NOT NO, BUT HELL NO !

        Journal of Wildlife Diseases, 47(1), 2011, pp. 78-93 © Wildlife Disease Association 2011

        http://chronic-wasting-disease.blogspot.com/2011/02/role-of-predation-in-disease-control.html

        http://www.thewildlifenews.com/2011/11/03/jackson-hole-newsguide-retired-biologist-stop-feeding-make-elk-migrate/

        Thursday, December 25, 2008

        Lions and Prions and Deer Demise

        snip…

        Greetings,

        A disturbing study indeed, but even more disturbing, the fact that this very study shows the potential for transmission of the TSE agent into the wild of yet another species in the USA. Science has shown that the feline is most susceptible to the TSE agent. Will CWD be the demise of the mountain lions, cougars and such in the USA? How many have ever been tested in the USA? I recall there is a study taking place ; Review A prion disease of cervids: Chronic wasting disease Christina J. Sigurdson et al ;

        Mountain lion (Puma concolor) susceptibility to experimental feeding of CWD prions is currently under investigation (M. Miller and L. Wolfe, personal communication).

        WHAT about multiple strains of CWD ?

        0C7.04

        North American Cervids Harbor Two Distinct CWD Strains

        snip…

        http://chronic-wasting-disease.blogspot.com/2008/12/lions-and-prions-and-deer-demise.html

        SNIP…SEE ;

        http://chronic-wasting-disease.blogspot.com/2009/07/deer-carcass-decomposition-and.html

        SEE FULL TEXT ;

        http://caninespongiformencephalopathy.blogspot.com/2012/03/canine-spongiform-encephalopathy-new.html

        Monday, March 26, 2012

        CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE

        http://caninespongiformencephalopathy.blogspot.com/2012/03/canine-spongiform-encephalopathy-new.html

        http://caninespongiformencephalopathy.blogspot.com/

        FELINE SPONGIFORM ENCEPHALOPATHY FSE

        http://felinespongiformencephalopathyfse.blogspot.com/2009_04_01_archive.html

        http://felinespongiformencephalopathyfse.blogspot.com/

        http://betaamyloidcjd.blogspot.com/2008/05/fecal-transmission-of-aa-amyloidosis-in.html

        Friday, November 09, 2012

        *** Chronic Wasting Disease CWD in cervidae and transmission to other species

        http://chronic-wasting-disease.blogspot.com/2012/11/chronic-wasting-disease-cwd-in-cervidae.html

        Sunday, November 11, 2012

        *** Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November 2012

        http://chronic-wasting-disease.blogspot.com/2012/11/susceptibilities-of-nonhuman-primates.html

        Friday, December 14, 2012

        Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005 – December 14, 2012

        http://chronic-wasting-disease.blogspot.com/2012/12/susceptibility-chronic-wasting-disease.html

        kind regards,
        terry

        • avatar jdubya says:

          These cases can also arise spontaneously…at least with humans so can assume other species as well. There are “three” ways to die of prion disease: genetic, spontaneous and consumption of prions.

          • avatar Vicki Fossen says:

            We’ve made strides in methods to detect the diseases in wildlife. Humans though, still require necropsy to be certain.

            Many of the symptoms could mimic other diseases in humans, so I makes me wonder if it is more prevalent than surmised.

  3. avatar Immer Treue says:

    Had a young buck, couldn’t have been more than ten months old, did not make it through the last snow and it’s aftermath up here. All the signs of starvation, with a few characteristics that looked liked CWD. As CWD does not occur in NE MN, DNR had some up the next day. Performed afield necropsy, and it was indeed starvation.

    Throat was cut open to remove lymph nodes, and check for any type of impaction. Surprise guests were botfly larva. Don’t really affect deer, but having those hitch hikers can’t be all that comfortable.

    All said, with all the wolves up here supposedly killing all the deer, how come they could not find this weak little fawn. Heck, my dog walked right up to it.

  4. avatar Vicki Fossen says:

    I’ve been able to communicate with some Colorado researchers. It does seem that cats do kill affected animals prior to our being able to see signs of disease. I am sure wolves are the same. The up-side to that is, the sooner they are dead (afflicted animals), the less they expose others. CWD is a prolific spreader.

    Although we are not aware of CWD spreading to people, we are not certain it can’t. Considering it’s close relationship and similarities to CVJ, I would steer clear of eating deer until tests show no evidence of disease.

    But hey, I’d rather be around to study than be dead and being studied.

  5. avatar jdubya says:

    Vicki is right, anyone who willfully eats a CWD animal is dumb. Who wants to be the first human diagnosed with CWD?

    Not having had the disease but based upon accounts of the human counterpart, some neural sensory deprivation is obvious early. While this can be managed in a human health care setting, if you are a deer or elk in the wild with lions, bears and wolves hunting you down, even the first stages of CWD could be all it it would take to cull you from the herd.

  6. avatar WM says:

    A quick and authoritative history of events on the detection and apparent distribution of CWD:

    http://www.cwd-info.org/index.php/fuseaction/about.timeline

    The site is a joint project of the Boone and Crockett Club, Mule Deer Foundation, and Rocky Mountain Elk Foundation. These non-profit wildlife conservation organizations formed the Chronic Wasting Disease Alliance in January 2002 to address CWD. Other organizations have since joined the Alliance.

    The story is, unfortunately, far from complete. No real progress on controlling it or its geographic distribution. Very disturbing.

    • avatar Immer Treue says:

      Rott of CWD certainly does point to captive/game farm origins.

      But now we have this:

      “While the study reveals that prions are shed in feces of symptomatic deer as well, the discovery that the infected deer shed prions (PREE-ons) in their feces many months before they show clinical symptoms has particularly provocative implications, according to the research team, at University of California, San Francisco and the Colorado Division of Wildlife’s Wildlife Research Center.”

      Who’d a ever thought that them deer and elk would be spreading more fear and death than those vermin carrying wolves? Sorry, just couldn’t resist.

      • avatar Mark L says:

        I HAVE seen it as a theory in causing some dinosaur species’ die offs, oddly enough.

  7. avatar Nancy says:

    “While it must be stressed there is no evidence that humans have ever gotten this disease, these non-living prions do change over time, and they can suddenly cross species barriers”

    Maybe CWD has already made the jump (like that disease transmitted from cats to humans, where they become cat horders) showing up in some hunters, forcing them to go on blogs, posting pictures and bragging about maiming, torturing and killing wolves “for the good of mankind ” 🙂

    • avatar alf says:

      “While it must be stressed there is no evidence that humans have ever gotten this disease, these non-living prions do change over time, and they can suddenly cross species barriers”

      Creuzfelt-Jakob (spelling ?) disease is the human equivalent of BSE, CWD, etc., and it does indeed jump species.

      In all its forms — CWD, BSE, Canine SE, etc., including C-Jv, there are occasional but very rare spontaneous cases of spongiform encephalitis.

      BSE has been pretty conclusively traced back to feeding cattle ground up scrapie-suffering sheep brains and spinal cords in Crysalix (spelling ?) and other such “supplements” (The corporate industrial agribusiness complex at work, serving YOU !).

      Scrapie was first identified is sheep hundreds of years ago, and although it’s still rare, it (spongiform encephalitis) probably spread more quickly among sheep than in other species because of sheep’s herding instinct.

      As stated above, SE occurs spontaneously, but is facilitated by consuming nerve tissue of infected animals.

      • avatar Ida Lupine says:

        (The corporate industrial agribusiness complex at work, serving YOU !).

        Yes, I remember it well. It was just the push I needed to get me to stop eating red meat for good. I couldn’t believe how many animals had to be destroyed, and I did not and do not want to contribute to it.

        Did CWD occur naturally or I thought I read somewhere where it was introduced by farmed deer and elk? Probably from feeding them the same ungodly stuff that nature never intended them to eat?

        • pens, pens, PENS ???

          *** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.

          http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

          now, decades later ;

          2012

          PO-039: A comparison of scrapie and chronic wasting disease in white-tailed deer

          Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; Agricultural Research Service, National Animal Disease Center; Ames, IA USA

          Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. The purpose of these experiments was to determine susceptibility of white-tailed deer (WTD) to scrapie and to compare the resultant clinical signs, lesions, and molecular profiles of PrPSc to those of chronic wasting disease (CWD). We inoculated WTD intracranially (IC; n = 5) and by a natural route of exposure (concurrent oral and intranasal (IN); n = 5) with a US scrapie isolate. All deer were inoculated with a 10% (wt/vol) brain homogenate from sheep with scrapie (1ml IC, 1 ml IN, 30 ml oral). All deer inoculated by the intracranial route had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues as early as 7 months-post-inoculation (PI) and a single deer that was necropsied at 15.6 months had widespread distribution of PrPSc highlighting that PrPSc is widely distributed in the CNS and lymphoid tissues prior to the onset of clinical signs. IC inoculated deer necropsied after 20 months PI (3/5) had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. The results of this study suggest that there are many similarities in the manifestation of CWD and scrapie in WTD after IC inoculation including early and widespread presence of PrPSc in lymphoid tissues, clinical signs of depression and weight loss progressing to wasting, and an incubation time of 21-23 months. Moreover, western blots (WB) done on brain material from the obex region have a molecular profile similar to CWD and distinct from tissues of the cerebrum or the scrapie inoculum. However, results of microscopic and IHC examination indicate that there are differences between the lesions expected in CWD and those that occur in deer with scrapie: amyloid plaques were not noted in any sections of brain examined from these deer and the pattern of immunoreactivity by IHC was diffuse rather than plaque-like. After a natural route of exposure, 100% of WTD were susceptible to scrapie. Deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 months PI. Tissues from these deer were positive for PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer exhibited two different molecular profiles: samples from obex resembled CWD whereas those from cerebrum were similar to the original scrapie inoculum. On further examination by WB using a panel of antibodies, the tissues from deer with scrapie exhibit properties differing from tissues either from sheep with scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are strongly immunoreactive when probed with mAb P4, however, samples from WTD with scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4 or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly immunoreactive and samples from WTD with scrapie are strongly positive. This work demonstrates that WTD are highly susceptible to sheep scrapie, but on first passage, scrapie in WTD is differentiable from CWD.

          http://www.landesbioscience.com/journals/prion/03-Prion6-2-Transmission-and-strains.pdf

          2011

          *** After a natural route of exposure, 100% of white-tailed deer were susceptible to scrapie.

          http://www.usaha.org/Portals/6/Reports/2011/report-cwal-2011.pdf

          Scrapie in Deer: Comparisons and Contrasts to Chronic Wasting Disease (CWD)

          Justin J. Greenlee of the Virus and Prion Diseases Research Unit, National Animal Disease Center, ARS, USDA, Ames, IA provided a presentation on scrapie and CWD in inoculated deer. Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. We inoculated white-tailed deer intracranially (IC) and by a natural route of exposure (concurrent oral and intranasal inoculation) with a US scrapie isolate. All deer inoculated by the intracranial route had evidence of PrPSc accumulation and those necropsied after 20 months post-inoculation (PI) (3/5) had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. A single deer that was necropsied at 15.6 months PI did not have clinical signs, but had widespread distribution of PrPSc. This highlights the facts that 1) prior to the onset of clinical signs PrPSc is widely distributed in the CNS and lymphoid tissues and 2) currently used diagnostic methods are sufficient to detect PrPSc prior to the onset of clinical signs. The results of this study suggest that there are many similarities in the manifestation of CWD and scrapie in white-tailed deer after IC inoculation including early and widespread presence of PrPSc in lymphoid tissues, clinical signs of depression and weight loss progressing to wasting, and an incubation time of 21-23 months. Moreover, western blots (WB) done on brain material from the obex region have a molecular profile consistent with CWD and distinct from tissues of the cerebrum or the scrapie inoculum. However, results of microscopic and IHC examination indicate that there are differences between the lesions expected in CWD and those that occur in deer with scrapie: amyloid plaques were not noted in any sections of brain examined from these deer and the pattern of immunoreactivity by IHC was diffuse rather than plaque-like. After a natural route of exposure, 100% of white-tailed deer were susceptible to scrapie. Deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 months PI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. While two WB patterns have been detected in brain regions of deer inoculated by the natural route, unlike the IC inoculated deer, the pattern similar to the scrapie inoculum predominates.

          Committee Business:

          The Committee discussed and approved three resolutions regarding CWD. They can be found in the report of the Reswolutions Committee. Essentially the resolutions urged USDA-APHIS-VS to:

          Continue to provide funding for CWD testing of captive cervids

          Finalize and publish the national CWD rule for Herd Certification and Interstate Movement

          Evaluate live animal test, including rectal mucosal biopsy, for CWD in cervids

          http://www.usaha.org/Portals/6/Reports/2011/report-cwal-2011.pdf

          2011 Annual Report

          Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research Unit

          2011 Annual Report

          In Objective 1, Assess cross-species transmissibility of transmissible spongiform encephalopathies (TSEs) in livestock and wildlife, numerous experiments assessing the susceptibility of various TSEs in different host species were conducted. Most notable is deer inoculated with scrapie, which exhibits similarities to chronic wasting disease (CWD) in deer suggestive of sheep scrapie as an origin of CWD.

          snip…

          4.Accomplishments 1. Deer inoculated with domestic isolates of sheep scrapie. Scrapie-affected deer exhibit 2 different patterns of disease associated prion protein. In some regions of the brain the pattern is much like that observed for scrapie, while in others it is more like chronic wasting disease (CWD), the transmissible spongiform encephalopathy typically associated with deer. This work conducted by ARS scientists at the National Animal Disease Center, Ames, IA suggests that an interspecies transmission of sheep scrapie to deer may have been the origin of CWD. This is important for husbandry practices with both captive deer, elk and sheep for farmers and ranchers attempting to keep their herds and flocks free of CWD and scrapie.

          http://ars.usda.gov/research/projects/projects.htm?ACCN_NO=411467&showpars=true&fy=2011

          White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection

          Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS

          Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. Previous experiments demonstrated that white-tailed deer are susceptible to sheep-derived scrapie by intracranial inoculation. The purpose of this study was to determine susceptibility of white-tailed deer to scrapie after a natural route of exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal (1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with scrapie. Non-inoculated deer were maintained as negative controls. All deer were observed daily for clinical signs. Deer were euthanized and necropsied when neurologic disease was evident, and tissues were examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) due to an injury. At that time, examination of obex and lymphoid tissues by IHC was positive, but WB of obex and colliculus were negative. Remaining deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by potential natural routes of inoculation. In-depth analysis of tissues will be done to determine similarities between scrapie in deer after intracranial and oral/intranasal inoculation and chronic wasting disease resulting from similar routes of inoculation.

          see full text ;

          http://www.usaha.org/Portals/6/Reports/2010/report-cwal-2010.pdf

          how many states have $465,000., and can quarantine and purchase there from, each cwd said infected farm, but how many states can afford this for all the cwd infected cervid game ranch type farms ???

          ? game farms in a state X $465,000., do all these game farms have insurance to pay for this risk of infected the wild cervid herds, in each state ???

          how many game farms, are too many game farms ?

          when you have states handing out shooting pen permits like candy on halloween, just to advance their coffers, then other states wanting to do the same thing, with most all of them ignoring the science on shooting pens and cwd, what do you expect is going to happen.

          when is enough, enough ?

          Tuesday, December 20, 2011

          CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) Farm Update DECEMBER 2011

          The CWD infection rate was nearly 80%, the highest ever in a North American captive herd.

          RECOMMENDATION: That the Board approve the purchase of 80 acres of land for $465,000 for the Statewide Wildlife Habitat Program in Portage County and approve the restrictions on public use of the site.

          Form 1100-001

          (R 2/11)

          NATURAL RESOURCES BOARD AGENDA ITEM

          SUBJECT: Information Item: Almond Deer Farm Update

          FOR: DECEMBER 2011 BOARD MEETING

          TUESDAY

          TO BE PRESENTED BY TITLE: Tami Ryan, Wildlife Health Section Chief

          SUMMARY:

          http://dnr.wi.gov/about/nrb/2011/december/12-11-2b2.pdf

          http://chronic-wasting-disease.blogspot.com/2011/12/chronic-wasting-disease-cwd-wisconsin.html

          SEE MORE USAHA REPORTS HERE, 2012 NOT PUBLISHED YET…TSS

          http://www.usaha.org/Portals/6/Proceedings/USAHAProceedings-2010-114th.pdf

          http://www.usaha.org/Portals/6/Proceedings/2009_USAHA_Proceedings.pdf

          http://portals5.gomembers.com/portals/6/proceedings/2008_usaha_proceedings.pdf

          Wednesday, November 14, 2012

          PENNSYLVANIA 2012 THE GREAT ESCAPE OF CWD INVESTIGATION MOVES INTO LOUISIANA and INDIANA

          http://chronic-wasting-disease.blogspot.com/2012/11/pennsylvania-2012-great-escape-of-cwd_14.html

          Pennsylvania CWD number of deer exposed and farms there from much greater than first thought

          Published: Wednesday, October 17, 2012, 10:44 PM Updated: Wednesday, October 17, 2012, 11:33 PM

          http://chronic-wasting-disease.blogspot.com/2012/10/pennsylvania-cwd-number-of-deer-exposed.html

          Tuesday, October 23, 2012

          PA Captive deer from CWD-positive farm roaming free

          http://chronic-wasting-disease.blogspot.com/2012/10/pa-captive-deer-from-cwd-positive-farm.html

          HERE, we see why these shooting pen owners some much like the USDA oversight of these game farms ;

          USDA TO PGC ONCE CAPTIVES ESCAPE “it‘s no longer its business.”

          problem solved $$$…TSS

          Sunday, January 06, 2013

          USDA TO PGC ONCE CAPTIVES ESCAPE “it‘s no longer its business.”

          http://chronic-wasting-disease.blogspot.com/2013/01/usda-to-pgc-once-captives-escape-its-no.html

          what happened to the PA deer from the CWD index heard that went to Louisiana ???

          Monday, April 15, 2013

          Deer farmers in the state of Louisiana are under a quarantine due to Chronic Wasting Disease CWD

          http://chronic-wasting-disease.blogspot.com/2013/04/deer-farmers-in-state-of-louisiana-are.html

          Friday, September 28, 2012

          Stray elk renews concerns about deer farm security Minnesota

          http://chronic-wasting-disease.blogspot.com/2012/09/stray-elk-renews-concerns-about-deer.html

          Monday, June 11, 2012

          OHIO Captive deer escapees and non-reporting

          http://chronic-wasting-disease.blogspot.com/2012/06/ohio-captive-deer-escapees-and-non.html

          Friday, October 12, 2012

          Texas Animal Health Commission (TAHC) is Now Accepting Comments on Rule Proposals for “Chronic Wasting Disease (CWD)”

          TO: comments@tahc.state.tx.us;

          Texas Animal Health Commission (TAHC)

          http://chronic-wasting-disease.blogspot.com/2012/10/texas-animal-health-commission-tahc-is.html

          please note, I do not know how much of this 125 TONS of banned mad cow protein was part of the ;

          e) “Big Jim’s” BBB Deer Ration, Big Buck Blend, Recall # V-104-6;

          bbbut, this was about 10 years post mad cow feed ban from 1997. 10 years later, and still feeding banned mad cow protein to cervids???

          considering that .005 gram is lethal to several bovines, and we know that the oral consumption of CWD tainted products is very efficient mode of transmission of CWD.

          Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006

          Date: August 6, 2006 at 6:16 pm PST

          PRODUCT

          a) CO-OP 32% Sinking Catfish, Recall # V-100-6;

          b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6;

          c) Pro 40% Swine Conc Meal — 50 lb, Recall # V-102-6;

          d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;

          ***e) “Big Jim’s” BBB Deer Ration, Big Buck Blend, Recall # V-104-6;

          f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;

          g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox — 0.0055%, Recall # V-106-6;

          h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6;

          i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;

          j) CO-OP LAYING CRUMBLES, Recall # V-109-6;

          k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6;

          l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;

          m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6

          CODE

          Product manufactured from 02/01/2005 until 06/06/2006

          RECALLING FIRM/MANUFACTURER

          Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.

          REASON

          Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as “Do not feed to ruminants”.

          VOLUME OF PRODUCT IN COMMERCE

          125 tons

          DISTRIBUTION

          AL and FL

          END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

          ###

          http://www.fda.gov/Safety/Recalls/EnforcementReports/2006/ucm120413.htm

          10,000,000 lbs banned blood laced meat and bone meal mbm 2007

          http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm

          ——– Original Message ——–

          Subject: DOCKET– 03D-0186 — FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

          Date: Fri, 16 May 2003 11:47:37 –0500

          From: “Terry S. Singeltary Sr.”

          To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000096/!x-usc:mailto:fdadockets@oc.fda.gov

          Greetings FDA,

          i would kindly like to comment on; Docket 03D-0186FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;

          snip…

          Oral transmission and early lymphoid tropism of chronic wasting diseasePrPres in mule deer fawns (Odocoileus hemionus ) These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.

          snip…

          http://vir.sgmjournals.org/cgi/content/full/80/10/2757

          now, just what is in that deer feed? _ANIMAL PROTEIN_

          Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES

          Date: Sat, 25 May 2002 18:41:46 -0700 From: “Terry S. Singeltary Sr.” Reply-To: BSE-LTo: BSE-L

          8420-20.5% Antler DeveloperFor Deer and Game in the wildGuaranteed Analysis Ingredients / Products Feeding Directions

          snip…

          _animal protein_

          http://www.surefed.com/deer.htm

          snip…

          DEPARTMENT OF HEALTH & HUMAN SERVICESPUBLIC HEALTH SERVICEFOOD AND DRUG ADMINISTRATIONApril 9, 2001 WARNING LETTER01-PHI-12CERTIFIED MAILRETURN RECEIPT REQUESTED

          Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145

          PHILADELPHIA DISTRICT

          Tel: 215-597-4390

          Dear Mr. Raymond:Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23,2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials. The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 – Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).Our investigation found failure to label your swine feed with the required cautionary statement “Do Not Feed to cattleor other Ruminants” The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser.

          In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal.Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain.The above is not intended to be an all-inclusive list of deviations fromthe regulations. As a manufacturer of materials intended for animalfeed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance withthe law. We have enclosed a copy of FDA’s Small Entity Compliance Guideto assist you with complying with the regulation… blah, blah, blah…

          http://www.fda.gov/foi/warning_letters/g1115d.pdf

          snip…end…full text ;

          2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

          EMC 1 Terry S. Singeltary Sr. Vol #: 1

          http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm

          http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm

          see my full text submission here ;

          http://madcowfeed.blogspot.com/2008/07/docket-03d-0186-fda-issues-draft.html

          Sunday, April 21, 2013

          Politicians ignore alarming CWD spike in Wyoming valley Wisconsin

          http://chronic-wasting-disease.blogspot.com/2013/04/politicians-ignore-alarming-cwd-spike.html

          Tuesday, April 16, 2013

          Cervid Industry Unites To Set Direction for CWD Reform and seem to ignore their ignorance and denial in their role in spreading Chronic Wasting Disease

          http://chronic-wasting-disease.blogspot.com/2013/04/cervid-industry-unites-to-set-direction.html

          Thursday, May 02, 2013

          Chronic Wasting Disease (CWD) Texas Important Update on OBEX ONLY TEXTING

          http://chronic-wasting-disease.blogspot.com/2013/05/chronic-wasting-disease-cwd-texas.html

          kind regards,
          terry

          • avatar Ralph Maughan says:

            So after reading this, “A comparison of scrapie and chronic wasting disease in white-tailed deer.” Should I conclude that scrapie and thus sheep are the cause (or just a cause) of CWD?

            Then I have a question. What becomes of humans that eat scrapie-infected mutton, or worse, brains?

            • Ralph Maughan says:

              May 4, 2013 at 11:01 pm

              So after reading this, “A comparison of scrapie and chronic wasting disease in white-tailed deer.” Should I conclude that scrapie and thus sheep are the cause (or just a cause) of CWD?

              IN MY OPINION, YES. …TSS

              Then I have a question. What becomes of humans that eat scrapie-infected mutton, or worse, brains?

              1: 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.

              snip…

              The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

              PMID: 6997404

              http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

              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://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

              Nature. 1972 Mar 10;236(5341):73-4.

              Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

              Gibbs CJ Jr, Gajdusek DC.

              Nature 236, 73 – 74 (10 March 1972); doi:10.1038/236073a0

              Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

              C. J. GIBBS jun. & D. C. GAJDUSEK

              National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

              SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

              http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html

              http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html

              why do we not want to do TSE transmission studies on chimpanzees $

              5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

              snip…

              R. BRADLEY

              http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf

              Wednesday, February 16, 2011

              IN CONFIDENCE

              SCRAPIE TRANSMISSION TO CHIMPANZEES

              IN CONFIDENCE

              http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html

              Sunday, December 12, 2010

              EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010

              http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsa-reviews-bsetse-infectivity-in.html

              Sunday, April 18, 2010

              SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

              http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html

              Thursday, December 23, 2010

              Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009

              Volume 17, Number 1 January 2011

              http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/molecular-typing-of-protease-resistant.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

              Monday, April 25, 2011

              Experimental Oral Transmission of Atypical Scrapie to Sheep

              Volume 17, Number 5-May 2011

              http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html

              Friday, February 11, 2011

              Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

              http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html

              Thursday, March 29, 2012

              atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012

              NIAA Annual Conference April 11-14, 2011San Antonio, Texas

              http://nor-98.blogspot.com/2012/03/atypical-nor-98-scrapie-has-spread-from.html

              RESEARCH

              Emerging Infectious Diseases • http://www.cdc.gov/eid • Vol. 17, No. 5, May 2011

              Experimental Oral Transmission of Atypical Scrapie to Sheep

              Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos

              To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specifi c prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These fi ndings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain.

              SNIP…

              Although we do not have epidemiologic evidence that supports the effi cient spread of disease in the fi eld, these data imply that disease is potentially transmissible under fi eld situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing fi nding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown.

              How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantifi ed, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confi rmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected.

              Emerging Infectious Diseases • http://www.cdc.gov/eid • Vol. 17, No. 5, May 2011

              http://wwwnc.cdc.gov/eid/article/17/5/pdfs/10-1654.pdf

              Monday, November 30, 2009

              USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE

              http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html

              IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.

              I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.

              JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. …

              Tuesday, July 17, 2012

              O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 – 25 May 2012

              http://transmissiblespongiformencephalopathy.blogspot.com/2012/07/oie-bse-cwd-scrapie-tse-prion-disease.html

              Thursday, December 20, 2012

              OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE

              http://transmissiblespongiformencephalopathy.blogspot.com/2012/12/oie-group-recommends-that-scrape-prion.html

              kind regards, terry

              • avatar Ralph Maughan says:

                Terry,

                This is a very long answer with a lot of documentation, but in my judgment I don’t want to eat any lamb or mutton, nor venison from places where CWD is prevalent.

              • avatar WM says:

                Terry S.,

                If the risk is so high, and the literature so filled with studies, hypotheses, and maybe even scientifically convincing data that CJ, CWD, etc., has the ability to jump a species, or be successfully artificially introduced, why is the Center for Communicable Disease in Atlanta not all over this with its own studies, warnings, prohibitions and the like?

                By the way, I am not at all disagreeing with you, but genuinely curious that if there is substantial, cogent and convincing risk it is perplexing (maybe even condemning) that warning bells are not going off in a VERY LOUD volume with state and federal health authorities in the US, as well as around the world. Are we too early, at the low end of the learning curve, too dumb,or is it categorical denial and politics, because of potential economic ramifications?

                • WM says:
                  May 5, 2013 at 1:11 pm
                  Terry S.,

                  If the risk is so high, and the literature so filled with studies, hypotheses, and maybe even scientifically convincing data that CJ, CWD, etc., has the ability to jump a species, or be successfully artificially introduced, why is the Center for Communicable Disease in Atlanta not all over this with its own studies, warnings, prohibitions and the like?

                  By the way, I am not at all disagreeing with you, but genuinely curious that if there is substantial, cogent and convincing risk it is perplexing (maybe even condemning) that warning bells are not going off in a VERY LOUD volume with state and federal health authorities in the US, as well as around the world. Are we too early, at the low end of the learning curve, too dumb,or is it categorical denial and politics, because of potential economic ramifications?

                  =======================my reply as follows, sorry to be so long winded, but I think all of you need all the science, and then you all have to make your won minds up. please see source references ;

                  Monday, May 23, 2011

                  CDC Assesses Potential Human Exposure to Prion Diseases Travel Warning

                  Public release date: 23-May-2011

                  Contact: Francesca Costanzo adajmedia@elsevier.com 215-239-3249 Elsevier Health Sciences

                  CDC assesses potential human exposure to prion diseases Study results reported in the Journal of the American Dietetic Association Philadelphia, PA, May 23, 2011 – Researchers from the Centers for Disease Control and Prevention (CDC) have examined the potential for human exposure to prion diseases, looking at hunting, venison consumption, and travel to areas in which prion diseases have been reported in animals. Three prion diseases in particular – bovine spongiform encephalopathy (BSE or “Mad Cow Disease”), variant Creutzfeldt-Jakob disease (vCJD), and chronic wasting disease (CWD) – were specified in the investigation. The results of this investigation are published in the June issue of the Journal of the American Dietetic Association.

                  “While prion diseases are rare, they are generally fatal for anyone who becomes infected. More than anything else, the results of this study support the need for continued surveillance of prion diseases,” commented lead investigator Joseph Y. Abrams, MPH, National Center for Emerging and Zoonotic Infectious Diseases, CDC, Atlanta.”But it’s also important that people know the facts about these diseases, especially since this study shows that a good number of people have participated in activities that may expose them to infection-causing agents.”

                  Although rare, human prion diseases such as CJD may be related to BSE. Prion (proteinaceous infectious particles) diseases are a group of rare brain diseases that affect humans and animals. When a person gets a prion disease, brain function is impaired. This causes memory and personality changes, dementia, and problems with movement. All of these worsen over time. These diseases are invariably fatal. Since these diseases may take years to manifest, knowing the extent of human exposure to possible prion diseases could become important in the event of an outbreak.

                  CDC investigators evaluated the results of the 2006-2007 population survey conducted by the Foodborne Diseases Active Surveillance Network (FoodNet). This survey collects information on food consumption practices, health outcomes, and demographic characteristics of residents of the participating Emerging Infections Program sites. The survey was conducted in Connecticut, Georgia, Maryland, Minnesota, New Mexico, Oregon, and Tennessee, as well as five counties in the San Francisco Bay area, seven counties in the Greater Denver area, and 34 counties in western and northeastern New York.

                  Survey participants were asked about behaviors that could be associated with exposure to the agents causing BSE and CWD, including travel to the nine countries considered to be BSE-endemic (United Kingdom, Republic of Ireland, France, Portugal, Switzerland, Italy, the Netherlands, Germany, Spain) and the cumulative length of stay in each of those countries. Respondents were asked if they ever had hunted for deer or elk, and if that hunting had taken place in areas considered to be CWD-endemic (northeastern Colorado, southeastern Wyoming or southwestern Nebraska). They were also asked if they had ever consumed venison, the frequency of consumption, and whether the meat came from the wild.

                  The proportion of survey respondents who reported travel to at least one of the nine BSE endemic countries since 1980 was 29.5%. Travel to the United Kingdom was reported by 19.4% of respondents, higher than to any other BSE-endemic country. Among those who traveled, the median duration of travel to the United Kingdom (14 days) was longer than that of any other BSE-endemic country. Travelers to the UK were more likely to have spent at least 30 days in the country (24.9%) compared to travelers to any other BSE endemic country. The prevalence and extent of travel to the UK indicate that health concerns in the UK may also become issues for US residents.

                  The proportion of survey respondents reporting having hunted for deer or elk was 18.5% and 1.2% reported having hunted for deer or elk in CWD-endemic areas. Venison consumption was reported by 67.4% of FoodNet respondents, and 88.6% of those reporting venison consumption had obtained all of their meat from the wild. These findings reinforce the importance of CWD surveillance and control programs for wild deer and elk to reduce human exposure to the CWD agent. Hunters in CWD-endemic areas are advised to take simple precautions such as: avoiding consuming meat from sickly deer or elk, avoiding consuming brain or spinal cord tissues, minimizing the handling of brain and spinal cord tissues, and wearing gloves when field-dressing carcasses.

                  According to Abrams, “The 2006-2007 FoodNet population survey provides useful information should foodborne prion infection become an increasing public health concern in the future. The data presented describe the prevalence of important behaviors and their associations with demographic characteristics. Surveillance of BSE, CWD, and human prion diseases are critical aspects of addressing the burden of these diseases in animal populations and how that may relate to human health.”

                  ###

                  The article is “Travel history, hunting, and venison consumption related to prion disease exposure, 2006-2007 FoodNet population survey” by Joseph Y. Abrams, MPH; Ryan A. Maddox, MPH; Alexis R Harvey, MPH; Lawrence B. Schonberger, MD; and Ermias D. Belay, MD. It appears in the Journal of the American Dietetic Association, Volume 111, Issue 6 (June 2011) published by Elsevier.

                  In an accompanying podcast CDC’s Joseph Y. Abrams discusses travel, hunting, and eating venison in relation to prion diseases. It is available at http://adajournal.org/content/podcast.

                  http://www.eurekalert.org/pub_releases/2011-05/ehs-cap051811.php

                  Thursday, May 26, 2011

                  Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

                  Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.

                  Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

                  Joseph Y. Abrams, MPH, Ryan A. Maddox, MPH , Alexis R. Harvey, MPH , Lawrence B. Schonberger, MD , Ermias D. Belay, MD

                  Accepted 15 November 2010. Abstract Full Text PDF References .

                  Abstract

                  The transmission of bovine spongiform encephalopathy (BSE) to human beings and the spread of chronic wasting disease (CWD) among cervids have prompted concerns about zoonotic transmission of prion diseases. Travel to the United Kingdom and other European countries, hunting for deer or elk, and venison consumption could result in the exposure of US residents to the agents that cause BSE and CWD. The Foodborne Diseases Active Surveillance Network 2006-2007 population survey was used to assess the prevalence of these behaviors among residents of 10 catchment areas across the United States. Of 17,372 survey respondents, 19.4% reported travel to the United Kingdom since 1980, and 29.5% reported travel to any of the nine European countries considered to be BSE-endemic since 1980. The proportion of respondents who had ever hunted deer or elk was 18.5%, and 1.2% had hunted deer or elk in a CWD–endemic area. More than two thirds (67.4%) reported having ever eaten deer or elk meat. Respondents who traveled spent more time in the United Kingdom (median 14 days) than in any other BSE-endemic country. Of the 11,635 respondents who had consumed venison, 59.8% ate venison at most one to two times during their year of highest consumption, and 88.6% had obtained all of their meat from the wild. The survey results were useful in determining the prevalence and frequency of behaviors that could be important factors for foodborne prion transmission.

                  http://www.adajournal.org/article/S0002-8223(11)00278-1/abstract

                  CDC

                  Saturday, February 18, 2012

                  Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease

                  CDC Volume 18, Number 3—March 2012

                  SNIP…

                  Interspecies transmission of CWD to noncervids has not been observed under natural conditions. CWD infection of carcass scavengers such as raccoons, opossums, and coyotes was not observed in a recent study in Wisconsin (22). In addition, natural transmission of CWD to cattle has not been observed in experimentally controlled natural exposure studies or targeted surveillance (2). However, CWD has been experimentally transmitted to cattle, sheep, goats, mink, ferrets, voles, and mice by intracerebral inoculation (2,29,33).

                  CWD is likely transmitted among mule, white-tailed deer, and elk without a major species barrier (1), and other members of the cervid family, including reindeer, caribou, and other species of deer worldwide, may be vulnerable to CWD infection. Black-tailed deer (a subspecies of mule deer) and European red deer (Cervus elaphus) are susceptible to CWD by natural routes of infection (1,34). Fallow deer (Dama dama) are susceptible to CWD by intracerebral inoculation (35). Continued study of CWD susceptibility in other cervids is of considerable interest.

                  Reasons for Caution There are several reasons for caution with respect to zoonotic and interspecies CWD transmission. First, there is strong evidence that distinct CWD strains exist (36). Prion strains are distinguished by varied incubation periods, clinical symptoms, PrPSc conformations, and CNS PrPSc depositions (3,32). Strains have been identified in other natural prion diseases, including scrapie, BSE, and CJD (3). Intraspecies and interspecies transmission of prions from CWD-positive deer and elk isolates resulted in identification of >2 strains of CWD in rodent models (36), indicating that CWD strains likely exist in cervids. However, nothing is currently known about natural distribution and prevalence of CWD strains. Currently, host range and pathogenicity vary with prion strain (28,37). Therefore, zoonotic potential of CWD may also vary with CWD strain. In addition, diversity in host (cervid) and target (e.g., human) genotypes further complicates definitive findings of zoonotic and interspecies transmission potentials of CWD.

                  Intraspecies and interspecies passage of the CWD agent may also increase the risk for zoonotic CWD transmission. The CWD prion agent is undergoing serial passage naturally as the disease continues to emerge. In vitro and in vivo intraspecies transmission of the CWD agent yields PrPSc with an increased capacity to convert human PrPc to PrPSc (30). Interspecies prion transmission can alter CWD host range (38) and yield multiple novel prion strains (3,28). The potential for interspecies CWD transmission (by cohabitating mammals) will only increase as the disease spreads and CWD prions continue to be shed into the environment. This environmental passage itself may alter CWD prions or exert selective pressures on CWD strain mixtures by interactions with soil, which are known to vary with prion strain (25), or exposure to environmental or gut degradation.

                  Given that prion disease in humans can be difficult to diagnose and the asymptomatic incubation period can last decades, continued research, epidemiologic surveillance, and caution in handling risky material remain prudent as CWD continues to spread and the opportunity for interspecies transmission increases. Otherwise, similar to what occurred in the United Kingdom after detection of variant CJD and its subsequent link to BSE, years of prevention could be lost if zoonotic transmission of CWD is subsequently identified, CWD will likely continue to emerge in North America. …

                  SNIP…

                  http://wwwnc.cdc.gov/eid/article/18/3/11-0685_article.htm

                  http://wwwnc.cdc.gov/eid/article/18/3/11-0685_article.htm

                  Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

                  Our results have far-reaching implications for human health, since they indicate that cervid PrPSc can trigger the conversion of human PrPC into PrPSc, suggesting that CWD might be infectious to humans. Interestingly our findings suggest that unstable strains from CWD affected animals might not be a problem for humans, but upon strain stabilization by successive passages in the wild, this disease might become progressively more transmissible to man.

                  http://www.jbc.org/content/early/2011/01/04/jbc.M110.198465.full.pdf

                  Our results also have profound implications for understanding the mechanisms of the prion species barrier and indicate that the transmission barrier is a dynamic process that depends on the strain and moreover the degree of adaptation of the strain. If our findings are corroborated by infectivity assays, they will imply that CWD prions have the potential to infect humans and that this ability progressively increases with CWD spreading.

                  http://www.jbc.org/content/286/9/7490

                  http://www.jbc.org/content/286/9/7490.full.pdf+html

                  P35

                  ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A WISCONSIN STRAIN OF CWD

                  Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5

                  The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region.

                  http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf

                  PPo3-7:

                  Prion Transmission from Cervids to Humans is Strain-dependent

                  Qingzhong Kong, Shenghai Huang,*Fusong Chen, Michael Payne, Pierluigi Gambetti and Liuting Qing Department of Pathology; Case western Reserve University; Cleveland, OH USA *Current address: Nursing Informatics; Memorial Sloan-Kettering Cancer Center; New York, NY USA

                  Key words: CWD, strain, human transmission

                  Chronic wasting disease (CWD) is a widespread prion disease in cervids (deer and elk) in North America where significant human exposure to CWD is likely and zoonotic transmission of CWD is a concern. Current evidence indicates a strong barrier for transmission of the classical CWD strain to humans with the PrP-129MM genotype. A few recent reports suggest the presence of two or more CWD strains. What remain unknown is whether individuals with the PrP-129VV/MV genotypes are also resistant to the classical CWD strain and whether humans are resistant to all natural or adapted cervid prion strains. Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP, indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains. Preliminary results on CWD transmission in transgenic mice expressing human PrP-129V will also be discussed.

                  Acknowledgement Supported by NINDS NS052319 and NIA AG14359.

                  PPo2-27:

                  Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions

                  Marcelo A. Barria,1 Glenn C. Telling,2 Pierluigi Gambetti,3 James A. Mastrianni4 and Claudio Soto1 1Mitchell Center for Alzheimer’s disease and related Brain disorders; Dept of Neurology; University of Texas Houston Medical School; Houston, TX USA; 2Dept of Microbiology, Immunology & Molecular Genetics and Neurology; Sanders Brown Center on Aging; University of Kentucky Medical Center; Lexington, KY USA; 3Institute of Pathology; Case western Reserve University; Cleveland, OH USA; 4Dept of Neurology; University of Chicago; Chicago, IL USA

                  Prion diseases are infectious neurodegenerative disorders affecting humans and animals that result from the conversion of normal prion protein (PrPC) into the misfolded and infectious prion (PrPSc). Chronic wasting disease (CWD) of cervids is a prion disorder of increasing prevalence within the United States that affects a large population of wild and captive deer and elk. CWD is highly contagious and its origin, mechanism of transmission and exact prevalence are currently unclear. The risk of transmission of CWD to humans is unknown. Defining that risk is of utmost importance, considering that people have been infected by animal prions, resulting in new fatal diseases. To study the possibility that human PrPC can be converted into the infectious form by CWD PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification (PMCA) technique, which mimic in vitro the process of prion replication. Our results show that cervid PrPSc can induce the pathological conversion of human PrPC, but only after the CWD prion strain has been stabilized by successive passages in vitro or in vivo. Interestingly, this newly generated human PrPSc exhibits a distinct biochemical pattern that differs from any of the currently known forms of human PrPSc, indicating that it corresponds to a novel human prion strain. Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.

                  PPo2-7:

                  Biochemical and Biophysical Characterization of Different CWD Isolates

                  Martin L. Daus and Michael Beekes Robert Koch Institute; Berlin, Germany

                  Key words: CWD, strains, FT-IR, AFM

                  Chronic wasting disease (CWD) is one of three naturally occurring forms of prion disease. The other two are Creutzfeldt-Jakob disease in humans and scrapie in sheep. CWD is contagious and affects captive as well as free ranging cervids. As long as there is no definite answer of whether CWD can breach the species barrier to humans precautionary measures especially for the protection of consumers need to be considered. In principle, different strains of CWD may be associated with different risks of transmission to humans. Sophisticated strain differentiation as accomplished for other prion diseases has not yet been established for CWD. However, several different findings indicate that there exists more than one strain of CWD agent in cervids. We have analysed a set of CWD isolates from white-tailed deer and could detect at least two biochemically different forms of disease-associated prion protein PrPTSE. Limited proteolysis with different concentrations of proteinase K and/or after exposure of PrPTSE to different pH-values or concentrations of Guanidinium hydrochloride resulted in distinct isolate-specific digestion patterns. Our CWD isolates were also examined in protein misfolding cyclic amplification studies. This showed different conversion activities for those isolates that had displayed significantly different sensitivities to limited proteolysis by PK in the biochemical experiments described above. We further applied Fourier transform infrared spectroscopy in combination with atomic force microscopy. This confirmed structural differences in the PrPTSE of at least two disinct CWD isolates. The data presented here substantiate and expand previous reports on the existence of different CWD strains.

                  http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

                  2012

                  Envt.06:

                  Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

                  Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Aru Balachandran,2 Jürgen Richt,3 Vincent Beringue,4 Juan-Maria Torres,5 Paul Brown,1 Bob Hills6 and Jean-Philippe Deslys1

                  1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Canadian Food Inspection Agency; Ottawa, ON Canada; 3Kansas State University; Manhattan, KS USA; 4INRA; Jouy-en-Josas, France; 5INIA; Madrid, Spain; 6Health Canada; Ottawa, ON Canada

                  †Presenting author; Email: emmanuel.comoy@cea.fr

                  The constant increase of chronic wasting disease (CWD) incidence in North America raises a question about their zoonotic potential. A recent publication showed their transmissibility to new-world monkeys, but no transmission to old-world monkeys, which are phylogenetically closer to humans, has so far been reported. Moreover, several studies have failed to transmit CWD to transgenic mice overexpressing human PrP. Bovine spongiform encephalopathy (BSE) is the only animal prion disease for which a zoonotic potential has been proven. We described the transmission of the atypical BSE-L strain of BSE to cynomolgus monkeys, suggesting a weak cattle-to-primate species barrier. We observed the same phenomenon with a cattleadapted strain of TME (Transmissible Mink Encephalopathy). Since cattle experimentally exposed to CWD strains have also developed spongiform encephalopathies, we inoculated brain tissue from CWD-infected cattle to three cynomolgus macaques as well as to transgenic mice overexpressing bovine or human PrP. Since CWD prion strains are highly lymphotropic, suggesting an adaptation of these agents after peripheral exposure, a parallel set of four monkeys was inoculated with CWD-infected cervid brains using the oral route. Nearly four years post-exposure, monkeys exposed to CWD-related prion strains remain asymptomatic. In contrast, bovinized and humanized transgenic mice showed signs of infection, suggesting that CWD-related prion strains may be capable of crossing the cattle-to-primate species barrier. Comparisons with transmission results and incubation periods obtained after exposure to other cattle prion strains (c-BSE, BSE-L, BSE-H and cattle-adapted TME) will also be presented, in order to evaluate the respective risks of each strain.

                  Envt.07:

                  Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

                  Martin L. Daus,1,† Johanna Breyer,2 Katjs Wagenfuehr,1 Wiebke Wemheuer,2 Achim Thomzig,1 Walter Schulz-Schaeffer2 and Michael Beekes1 1Robert Koch Institut; P24 TSE; Berlin, Germany; 2Department of Neuropathology, Prion and Dementia Research Unit, University Medical Center Göttingen; Göttingen, Germany †Presenting author; Email: dausm@rki.de

                  Chronic wasting disease (CWD) is a contagious, rapidly spreading transmissible spongiform encephalopathy (TSE) occurring in cervids in North America. Despite efficient horizontal transmission of CWD among cervids natural transmission of the disease to other species has not yet been observed. Here, we report a direct biochemical demonstration of pathological prion protein PrPTSE and of PrPTSE-associated seeding activity in skeletal muscles of CWD-infected cervids. The presence of PrPTSE was detected by Western- and postfixed frozen tissue blotting, while the seeding activity of PrPTSE was revealed by protein misfolding cyclic amplification (PMCA). The concentration of PrPTSE in skeletal muscles of CWD-infected WTD was estimated to be approximately 2000- to 10000-fold lower than in brain tissue. Tissue-blot-analyses revealed that PrPTSE was located in muscle- associated nerve fascicles but not, in detectable amounts, in myocytes. The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

                  http://www.landesbioscience.com/journals/prion/Prion5-Supp-PrionEnvironment.pdf?nocache=1333529975

                  UPDATED DATA ON 2ND CWD STRAIN

                  Wednesday, September 08, 2010

                  CWD PRION CONGRESS SEPTEMBER 8-11 2010

                  http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html

                  CJD9/10022

                  October 1994

                  Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

                  Dear Mr Elmhirst,

                  CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

                  Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

                  The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

                  The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

                  The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

                  I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

                  http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

                  now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????

                  “Our conclusion stating that we found no strong evidence of CWD transmission to humans”

                  From: TSS (216-119-163-189.ipset45.wt.net)

                  Subject: CWD aka MAD DEER/ELK TO HUMANS ???

                  Date: September 30, 2002 at 7:06 am PST

                  From: “Belay, Ermias”

                  To:

                  Cc: “Race, Richard (NIH)” ; ; “Belay, Ermias”

                  Sent: Monday, September 30, 2002 9:22 AM

                  Subject: RE: TO CDC AND NIH – PUB MED- 3 MORE DEATHS – CWD – YOUNG HUNTERS

                  Dear Sir/Madam,

                  In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

                  That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that “no-one has ever been infected with prion disease from eating venison.” Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

                  Ermias Belay, M.D. Centers for Disease Control and Prevention

                  —–Original Message—–

                  From:

                  Sent: Sunday, September 29, 2002 10:15 AM

                  To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

                  Subject: TO CDC AND NIH – PUB MED- 3 MORE DEATHS – CWD – YOUNG HUNTERS

                  Sunday, November 10, 2002 6:26 PM ……snip……..end…………..TSS

                  Thursday, April 03, 2008

                  A prion disease of cervids: Chronic wasting disease

                  2008 1: Vet Res. 2008 Apr 3;39(4):41

                  A prion disease of cervids: Chronic wasting disease

                  Sigurdson CJ.

                  snip…

                  *** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

                  snip…

                  full text ;

                  http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

                  http://chronic-wasting-disease.blogspot.com/2006_12_01_archive.html

                  Sunday, January 22, 2012

                  Chronic Wasting Disease CWD cervids interspecies transmission

                  http://chronic-wasting-disease.blogspot.com/2012/01/chronic-wasting-disease-cwd-cervids.html

                  Friday, November 09, 2012

                  *** Chronic Wasting Disease CWD in cervidae and transmission to other species

                  http://chronic-wasting-disease.blogspot.com/2012/11/chronic-wasting-disease-cwd-in-cervidae.html

                  Friday, November 09, 2012

                  *** Chronic Wasting Disease CWD in cervidae and transmission to other species

                  http://chronic-wasting-disease.blogspot.com/2012/11/chronic-wasting-disease-cwd-in-cervidae.html

                  Sunday, November 11, 2012

                  *** Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November 2012

                  http://chronic-wasting-disease.blogspot.com/2012/11/susceptibilities-of-nonhuman-primates.html

                  Friday, December 14, 2012

                  Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005 – December 14, 2012

                  http://chronic-wasting-disease.blogspot.com/2012/12/susceptibility-chronic-wasting-disease.html

                  *** NOR IS THE FDA recalling this CWD positive elk meat for the well being of the dead elk ;

                  Wednesday, March 18, 2009 Noah’s Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

                  ___________________________________

                  PRODUCT

                  a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;

                  b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;

                  c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;

                  d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;

                  e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;

                  f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9;

                  CODE

                  Elk Meats with production dates of December 29, 30, and 31

                  RECALLING FIRM/MANUFACTURER

                  Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009.

                  Manufacturer: Noah’s Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing.

                  REASON

                  Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD).

                  VOLUME OF PRODUCT IN COMMERCE

                  Unknown

                  DISTRIBUTION

                  NV, CA, TX, CO, NY, UT, FL, OK

                  ___________________________________

                  http://www.fda.gov/bbs/topics/ENFORCE/2009/ENF01099.html

                  Monday, February 09, 2009

                  Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

                  snip…

                  Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain

                  Date: August 25, 2007 at 12:42 pm PST

                  our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.

                  http://www.jbc.org/

                  Wednesday, March 18, 2009

                  Noah’s Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

                  http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

                  kind regards, terry

  8. Tuesday, April 16, 2013

    Cervid Industry Unites To Set Direction for CWD Reform and seem to ignore their ignorance and denial in their role in spreading Chronic Wasting Disease

    http://chronic-wasting-disease.blogspot.com/2013/04/cervid-industry-unites-to-set-direction.html

    Saturday, March 10, 2012

    CWD, GAME FARMS, urine, feces, soil, lichens, and banned mad cow protein feed CUSTOM MADE for deer and elk

    http://chronic-wasting-disease.blogspot.com/2012/03/cwd-game-farms-urine-feces-soil-lichens.html

    Monday, July 16, 2012

    Persistence of the bovine spongiform encephalopathy infectious agent in sewage

    http://bseusa.blogspot.com/2012/07/persistence-of-bovine-spongiform.html

    Friday, February 25, 2011

    Soil clay content underlies prion infection odds Soil clay content underlies prion infection odds

    http://chronic-wasting-disease.blogspot.com/2011/02/soil-clay-content-underlies-prion.html

    Friday, February 08, 2013

    *** Behavior of Prions in the Environment: Implications for Prion Biology

    http://chronic-wasting-disease.blogspot.com/2013/02/behavior-of-prions-in-environment.html

    kind regards, terry

    • avatar Ralph Maughan says:

      Terry S. Singeltary Sr.,

      Thank you for all the information about CWD and its relationship to Mad-cow (BSE) and the human analogue to BSE, CJD, etc.

      If you want to write something additional for our newspaper — something in addition to what you have copied and pasted, I would appreciate it. In the meantime, I am reading your material.

      You might not know that we have covered this a number of times in the past — CWD, the menace of game farms, the relationship between prionic diseases, etc.

      Ralph Maughan
      webmaster

      (also sent by email)

  9. avatar DavePA says:

    Interesting to see this in relation to the recent discussions on intelligent life destroying itself, bird flu stories, antibiotic resistance, etc. Sometimes glad I’m not younger. Of course my Dad told me that he always thought the world would destroy itself in the 50’s, so you never know what the future will bring. Dave

  10. avatar Mark L says:

    And notice that all the species that are mentioned/grouped by the prions issues are at artificially higher numbers than in millenia (or eons) past. Maybe it’s just a price paid for being on top of a population wave?

  11. jdubya says:

    May 6, 2013 at 8:33 am

    These cases can also arise spontaneously…

    at least with humans so can assume other species as well. There are “three” ways to die of prion disease:

    genetic, spontaneous and consumption of prions.

    =====================hello jdubya,

    actually, the infamous _spontaneous_ theory has never been proven in any wild TSE in any species. there was one case they thought might have arisen spontaneously, but even this case was never proven. sporadic CJD in humans is not a single strain, my many strains, from many potential routes and sources. in fact, all iatrogenic CJD is, is sporadic CJD before route and source is proven. I can assure you, 85%+ of all human TSE prion disease i.e. sporadic CJD, does not happen spontaneously. please see ;

    Prions: Protein Aggregation and Infectious Diseases

    ADRIANO AGUZZI AND ANNA MARIA CALELLA

    Institute of Neuropathology, University Hospital of Zurich, Zurich, Switzerland

    snip…

    3. Sporadic Creutzfeldt-Jakob disease Approximately 85% of all human prion diseases are sporadic forms of CJD. For sCJD, there is no association with a mutant PRNP allele, nor is there any epidemiological evidence for exposure to a TSE agent through contact with people or animals infected with TSEs. sCJD cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the size and glycoform ratio of proteaseresistant prion protein identified on western blot (type 1 or type 2) (174). Heterozygosity (Met/Val) at PrP codon 129 appears to be associated with a lower risk (378) and/or prolonged incubation time (119, 387). The lack of routine laboratory testing for preclinical diagnosis makes the search for agent sources and other risk factors extremely difficult. At present, the means of acquisition of a TSE agent in these patients remains a mystery. *** So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE. In humans, the peak age incidence of sporadic CJD is 55-60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

    snip…

    Physiol Rev . VOL 89 . OCTOBER 2009 . http://www.prv.org

    http://physrev.physiology.org/cgi/content/abstract/89/4/1105

    http://creutzfeldt-jakob-disease.blogspot.com/2010/01/darwinian-evolution-of-prions-in-cell.html

    *** So far, there is no evidence for spontaneous PrPSc formation in any animal or human TSE. In humans, the peak age incidence of sporadic CJD is 55–60 years. However, if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age because more time would allow more opportunity for rare misfolding events.

    http://molpath.ucsd.edu/PDF's_PATH225/2011%20PDFs%20for%20225/Sigurdson_annurev.pathmechdis.3.121806.pdf

    *** So far, there is no evidence for spontaneous PrPres formation in any animal or human TSE disease. Moreover, in New Zealand and Australia where scrapie has been eradicated, there is no evidence of spontaneous occurrence of sheep scrapie. In addition, in humans the peak age incidence of sporadic CJD is 55–60 years, and if spontaneous misfolding were the primary event, one might expect a continuously increasing incidence with age, since more time might allow more opportunity for rare misfolding events.

    http://bmb.oxfordjournals.org/content/66/1/1.full.pdf

    *** 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

    *** Conclusion/Significance: Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

    http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0003017

    http://www.plosone.org/annotation/listThread.action?root=363

    http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143

    http://madcowspontaneousnot.blogspot.com/2010/07/spontaneous-generation-of-mammalian.html

    SPONTANEOUS TSE

    Perspectives BIOMEDICINE: A Fresh Look at BSE Bruce Chesebro*

    Mad cow disease, or bovine spongiform encephalopathy (BSE), is the cattle form of a family of progressive brain diseases. These diseases include scrapie in sheep, Creutzfeldt-Jakob disease (CJD) in humans, and chronic wasting disease (CWD) in deer and elk. They are also known as either “prion diseases” because of the association of a misfolded cellular prion protein in pathogenesis or “transmissible spongiform encephalopathies” (TSEs) because of the spongelike nature of the damaged brain tissue (1).

    The recent discovery of two BSE-infected cows, one in Canada and one in the United States, has dramatically increased concern in North America among meat producers and consumers alike over the extent to which BSE poses a threat to humans as well as to domestic and wild animals. The European BSE epidemic of the late-1980s seems to have been initiated a decade earlier in the United Kingdom by changes in the production of meat and bone meal (MBM) from rendered livestock, which led to contamination of MBM with the BSE infectious agent. Furthermore, the fact that UK farmers fed this rendered MBM to younger animals and that this MBM was distributed to many countries may have contributed to the ensuing BSE epidemic in the United Kingdom and internationally (2).

    Despite extensive knowledge about the spread of BSE through contaminated MBM, the source of BSE in Europe remains an unsolved mystery (2). It has been proposed that BSE could be derived from a cross-species infection, perhaps through contamination of MBM by scrapie-infected sheep tissues (see the figure). Alternatively, BSE may have been an endemic disease in cattle that went unnoticed because of its low level of horizontal transmission. Lastly, BSE might have originated by “spontaneous” misfolding of the normal cellular prion protein into the disease-associated abnormal isoform (3), which is postulated to be the infectious agent or “prion.”

    Five possible sources of BSE in North American cattle. Sheep, deer, and elk could spread prion diseases (TSEs) to cattle through direct animal contact or contamination of pastures. Endemic BSE has not been proven to exist anywhere in the world, but it is difficult to exclude this possibility because of the inefficient spread of BSE infectivity between individual animals (2). ___BSE caused by spontaneous misfolding of the prion protein has not been proven___. CREDIT: KATHARINE SUTLIFF/SCIENCE

    snip…

    Nevertheless, the idea that BSE might originate due to the spontaneous misfolding of prion proteins has received renewed interest in the wake of reports suggesting the occurrence of atypical BSE (9-11). These results imply that new strains of cattle BSE might have originated separately from the main UK outbreak. Where and how might such strains have originated? Although such rare events cannot be studied directly, any number of sources of the original BSE strain could also explain the discovery of additional BSE strains in cattle (see the figure). However, it would be worrisome if spontaneous BSE were really a valid etiology because such a mechanism would be impossible to prevent–unlike other possible scenarios that could be controlled by large-scale eradication of TSE-positive animals.

    Another way to look at this problem is to examine evidence for possible spontaneous TSE disease in other animals besides cattle. Spontaneous BSE would be extremely difficult to detect in cattle, where horizontal spread is minimal. However, in the case of the sheep TSE disease, scrapie, which spreads from ewes to lambs at birth as well as between adults, spontaneous disease should be detectable as new foci of clinical infection. In the early 1950s scrapie was eradicated in both Australia and New Zealand, and the mainland of both these countries has remained scrapie-free ever since. This scrapie-free status is not the result of selection of sheep resistant to scrapie because sheep from New Zealand are as susceptible as their UK counterparts to experimental scrapie infection (12). These experiments of man and nature appear to indicate that spontaneous clinical scrapie does not occur in sheep. Similarly, because CWD is known to spread horizontally, the lack of CWD in the deer or elk of eastern North America but its presence in western regions would also argue against a spontaneous disease mechanism. This is particularly noteworthy in New Zealand, where there are large numbers of deer and elk farms and yet no evidence of spontaneous CWD. If spontaneous scrapie does not occur in sheep or deer, this would suggest that spontaneous forms of BSE and sporadic Creutzfeldt-Jakob disease (sCJD) are unlikely to be found in cattle or humans. The main caveat to this notion is that spontaneous disease may arise in some animal species but not others. In humans, sCJD–which is considered by some researchers to begin by spontaneous misfolding of the prion protein–usually takes more than 50 years to appear. Thus, in animals with a shorter life-span, such as sheep, deer, and cattle, an analogous disease mechanism might not have time to develop.

    What can we conclude so far about BSE in North America? Is the BSE detected in two North American cows sporadic or spontaneous or both? “Sporadic” pertains to the rarity of disease occurrence. “Spontaneous” pertains to a possible mechanism of origin of the disease. These are not equivalent terms. The rarity of BSE in North America qualifies it as a sporadic disease, but this low incidence does not provide information about cause. For the two reported North American BSE cases, exposure to contaminated MBM remains the most likely culprit. However, other mechanisms are still possible, including cross-infection by sheep with scrapie or cervids with CWD, horizontal transmission from cattle with endemic BSE, and spontaneous disease in individual cattle. Based on our understanding of other TSEs, the spontaneous mechanism is probably the least likely. Thus, “idiopathic” BSE–that is, BSE of unknown etiology–might be a better term to describe the origin of this malady. …

    snip…full text ;

    http://www.sciencemag.org/cgi/content/full/sci;305/5692/1918

    DR. DEHAVEN: “All right. I think we’ve got three different questions in there, and I’ll try to touch on each one of them.

    “First of all, let me correct just a technical issue, and that is you mentioned 1 in 10,000. And actually our surveillance system currently is designed, the one that we have in place now is designed to detect 1 positive in 1 million cattle, and I gave some numbers between 200,000 and 268,000 that would allow us to detect 1 in 10 million as opposed to 1 in 10,000.

    “So we would, if we were able to collect in the ballpark of those numbers of samples then we with increasing numbers of samples have an increasingly statistically valid sample from which to determine, one, whether or not the disease exists and, if so, at what prevalence level.

    “So our real emphasis is to test as many of those animals as we can, ensure that we get an appropriate geographical distribution, but not setting a specific number as far as a target. Again, consistent with the recommendation from the International Review Team, their recommendation was to test all of them.

    “So that’s consistent with where we’re going is to test as many as we possibly can.

    *** “As far as spontaneous cases, that is a very difficult issue. **___There is no evidence to prove that spontaneous BSE occurs in cattle___; but here again it’s an issue of proving a negative. We do know that CJD, the human version of the disease, does occur spontaneously in humans at the rate of about 1 in 1 million. We don’t have enough data to definitively say that spontaneous cases of BSE in cattle occur or do not occur.

    “Again, it’s a very difficult situation to prove a negative.

    “So a lot of research is ongoing. Certainly if we do come up with any positive samples in the course of this surveillance we will be looking at that question in evaluating those samples but no scientifically hard evidence to confirm or refute whether or not spontaneous cases of BSE occur.

    snip…

    http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2004/03/0106.html

    http://madcowspontaneousnot.blogspot.com/2010/07/spontaneous-generation-of-mammalian.html

    Thursday, August 12, 2010

    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

    snip…

    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/

    What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”

    The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health.

    “(The agency) has no foundation on which to base that statement.”

    “We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.

    In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said

    Saturday, May 26, 2012

    Are USDA assurances on mad cow case ‘gross oversimplification’?

    http://bseusa.blogspot.com/2012/05/are-usda-assurances-on-mad-cow-case.html

    http://www.nature.com/nature/journal/v485/n7398/full/485279b.html

    Saturday, August 4, 2012

    Final Feed Investigation Summary – California BSE Case – July 2012

    http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/final-feed-investigation-summary.html

    http://www.promedmail.org/direct.php?id=20120501.1119136

    P.9.21

    Molecular characterization of BSE in Canada

    Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

    Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

    Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

    Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

    Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. ***It also suggests a similar cause or source for atypical BSE in these countries.

    http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

    Monday, March 25, 2013

    Minnesota Firm Recalls Bone-In Ribeye That May Contain Specified Risk Materials Recall Release CLASS II RECALL FSIS-RC-024-2013

    http://madcowusda.blogspot.com/2013/03/minnesota-firm-recalls-bone-in-ribeye.html

    Saturday, December 15, 2012

    Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle — an update 5 December 2012

    http://bse-atypical.blogspot.com/2012/12/bovine-spongiform-encephalopathy-effect.html

    Tuesday, March 5, 2013

    Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

    FDA believes current regulation protects the public from BSE but reopens comment period due to new studies

    http://transmissiblespongiformencephalopathy.blogspot.com/2013/03/use-of-materials-derived-from-cattle-in_6452.html

    kind regards,

    terry

  12. avatar Richie G. says:

    Many years ago on WBAI New York A.M. at 12:00 P.M. ,a person named Gary Null was on the air for one hour. He wrote many books on eating everything but meat, he ran a running club in Central Park on Sunday mornings. He made many claims from mercury in our vaccine’s caused many of our childhood disease’s of today. He also spoke about mad cow disease many years before being on the news in America. One thing I remember he said that the cows were feeding on other fallen cows or disease cattle. He also said that these cows were found with plastic six pack holders in their stomach, they were eating every disgusting thing. Now go to a controlled game farm, I can just think how these animals are treated, it’s easy to see how this can spread. Besides who cares there is money to be made on these controlled hunts, again Ralph no where do I read about these topics. What I did hear today the Democrats are trying to define a law that reads what makes a journalist, what a joke. They do not define as Tom Hartman spoke about journalist were throwing out pathlets during the revolutionary war. The democrats definition would throw you out Ralph ,which is a joke they are just trying to protect themselves from the real truth. I have learned more interesting topics from everyone who writes on this site for or against my beliefs, again thank you and everyone very much.

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