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From: TSS ()
Subject: CWD SPREADING IN WYOMING ! what about scrapieTSE and bseTSE ?
Date: November 29, 2005 at 10:35 am PST


From: TSS ()
Subject: CWD SPREADING IN WYOMING !
Date: November 29, 2005 at 9:41 am PST


CWD DETECTED IN NEW HUNT AREA NEAR NEWCASTLE

11/18/2005

CASPER - A white-tailed deer harvested in Hunt Area 9 near Newcastle has tested positive for chronic wasting disease. The disease had not previously been found in deer Hunt Area 9.

The deer was harvested on a ranch southeast of Newcastle in early October. The area is open to hunting for doe and fawn deer until Nov. 30 with 200 doe/fawn licenses, according to Wyoming Game and Fish Department regulations.

“It’s not a surprise that CWD was found in Hunt Area 9,” said Scott Edberg, wildlife supervisor for the Game and Fish’s Casper Region. “The disease has previously been found in the same drainage about 18 miles downstream in South Dakota, and across (U.S.) highway 16 in Hunt Area 6.”

Hunt Area 9 borders the Wyoming/South Dakota state line. Edberg said area landowners are cooperating with CWD research efforts by allowing hunters to harvest antlerless deer and submit them for testing.

The Game and Fish will continue to collect as many samples as possible from deer harvested in the area and killed by vehicles on adjacent highways. CWD surveillance sampling stations will be set up at the Old Mill in Newcastle and at C&A Meats in Sundance Nov. 18-20 from 9 a.m. to 5 p.m.

“We encourage all hunters harvesting deer in northeast Wyoming to stop by a surveillance sampling station and have their animal tested,” Edberg said. Collecting additional samples will help the Game and Fish understand how widespread the disease is in the area.

In addition to the positive test in Deer Area 9, a third deer has tested positive for CWD in Hunt Area 8, southwest of Upton. Although Hunt Area 8 is considered an endemic area (the disease has already been documented in this area), there have been only two positives per year since 2003. The most recent positive sample was from a white-tailed deer. The previous positives were from mule deer.

CWD is a fatal neurological disease that has been diagnosed in wild deer, elk and moose. Animals show no apparent signs of illness throughout much of the course of the disease. In terminal stages of CWD, animals typically are emaciated and display abnormal behavior.

There is no confirmed link between CWD and any human illness.

For more information on chronic wasting disease visit the Game and Fish Web site at http://gf.state.wy.us.

(contact: Robin Kepple (307) 473-3400)

-WGFD-


http://gf.state.wy.us/services/news/pressreleases/05/11/18/051118_3.asp

CWD FOUND IN NEW AREA IN BIG HORN BASIN

10/28/2005

THERMOPOLIS – Two mature mule deer bucks harvested in hunt area 127 immediately northwest of Thermopolis have tested positive for chronic wasting disease (CWD), a fatal brain disease that can affect all members of Wyoming’s deer family. CWD had not previously been detected in this area.

Worland Wildlife Biologist Bart Kroger collected lymph nodes from the deer Oct. 17 as part of the Wyoming Game and Fish Department’s CWD surveillance effort. Both samples were tested at the department’s laboratory in Laramie and tested positive for CWD.

CWD testing is a two-pronged approach, according to Cody Region Wildlife Management Coordinator Kevin Hurley. “The first test is an immunologic test called the ELISA. When a sample tests positive, it is termed a ‘presumptive positive’ until the results from a second IHC (immunohistochemistry) test is known,” Hurley said. “In nearly every case, when the ELISA turns up a presumptive positive, it is confirmed positive by the IHC.”

The two samples from area 127 tested positive for first the ELISA test and then the IHC test on Oct. 28.

In an effort to manage the spread of CWD and to understand how widespread it might be in an area, the department considers taking aggressive actions when cases are found in new areas. In this case, Game and Fish Deputy Director Gregg Arthur has instructed personnel in the Cody region to remove up to 50 deer within a five-mile radius of where the area 127 deer were harvested.

“I have asked our Cody personnel to move forward and collect additional samples. This action is consistent with the best science and the department’s CWD Management Plan,” Arthur said. He added that surveillance in other states has shown that it may be possible to slow down the spread of CWD if new cases of CWD are identified early.

According to Arthur, the additional sampling serves three purposes. First, it allows the Game and Fish to determine the prevalence of CWD in an area. Secondly, it may eliminate CWD in an area and prevent its spread to other areas. And thirdly, it may allow the Game and Fish to locate an area of infection that it can manage aggressively.

“Should more positives turn up, we will expand our efforts,” Arthur said.

The Game and Fish will conduct the removal harvesting both adult males and females between Oct. 27 and mid-November during daytime and nighttime hours. Research has demonstrated that samples taken from adult males and adult females are more likely to indicate if CWD is present than taking samples from younger-aged animals.

All of the animals collected will be field dressed and held in cold storage until the absence or presence of CWD in each is known. The meat from deer testing negative will be donated to individuals and families in need. Carcasses testing positive will be disposed of in an approved landfill in accordance with the Game and Fish CWD transportation regulation.

CWD is a fatal neurological disease that has been diagnosed in wild deer and elk in 10 states and two Canadian provinces. Animals show no apparent signs of illness throughout much of disease course. In terminal stages of CWD, animals typically are emaciated and display abnormal behavior.

There is no confirmed link between CWD and any human illness.

For more information on chronic wasting disease visit the Game and Fish Web site http://gf.state.wy.us

(contact: Dennie Hammer or Kevin Hurley (307) 527-7125)

-WGFD-


http://gf.state.wy.us/services/news/pressreleases/05/10/28/051028_1.asp

CWD FOUND IN DEER AND ELK IN NEW AREA SOUTH OF LARAMIE

10/21/2005

LARAMIE – The first deer and elk to test positive for chronic wasting disease in deer hunt area 77 and elk hunt area 8 south of Laramie were discovered this hunting season by the Wyoming State Veterinary Laboratory and Game and Fish Department.

The discovery was not surprising because CWD has been confirmed in all surrounding hunt areas in both Wyoming and Colorado, according to Bob Lanka, Game and Fish wildlife management coordinator in Laramie.

Both animals testing positive south of Laramie were hunter-killed adult females. The deer was taken on Jelm Mountain Oct. 2 and the elk on Boulder Ridge very near the Colorado border Oct. 3.

“Even though we expected CWD to show up in this area at some time, it is still a little disappointing when we have to add another hunt area to the list of positive areas,” Lanka said.

Hank Edwards, wildlife disease specialist in charge of testing and mapping CWD data, reports his crew has examined the lymph nodes from 1,800 hunter-harvested deer and elk this fall for CWD and expects to test a total of over 4,000 samples this year. No other new area hunt areas in Wyoming have been discovered with CWD this fall.

CWD is a communicable disease of the deer family caused by an abnormal protein or prion. There is no evidence that CWD can infect humans.

As tests are completed the Game and Fish will keep the public informed of any other cases of CWD found in new hunt areas.

(contact: Michelle Zitek (307) 745-4046)


-WGFD-


http://gf.state.wy.us/services/news/pressreleases/05/10/21/051021_2.asp


9/8/2005 DRAFT

WYOMING GAME AND FISH DEPARTMENT

CHRONIC WASTING DISEASE MANAGEMENT PLAN

August 19, 2005

EXECUTIVE SUMMARY


• It is the purpose of this plan to provide flexible and adaptable direction for management of Chronic Wasting Disease (CWD).


• The plan will be reviewed and updated as the CWD situation in Wyoming changes and additional information becomes available.


• The plan consists of four components: Disease Management, Applied Research, Public Information, and Funding.


• Based upon the known epidemiology of CWD in free-ranging deer and elk, eradication currently is not a justified or realistic disease management objective.


• The WGFD will work to minimize the spread of CWD and coordinate CWD management with other state and federal agencies.


• The WGFD will conduct surveillance to determine spatial distribution and prevalence of CWD, and coordinate CWD research with other state and federal agencies.


• The WGFD will provide timely, complete, and accurate information about CWD.


• Although there are concerns or perceptions by some people that CWD could be a livestock or human health threat, there currently is no credible supporting evidence; consequently, this plan addresses CWD as a disease of deer and elk.


• The WGFD will continue to work cooperatively with the Wyoming Department of Public Health and other human health organizations worldwide to monitor current research on CWD and human health and to provide up-to-date information to the public.


• Many very expensive CWD management, research, and public outreach activities are driven by the consideration of CWD as an international disease of concern; therefore, federal funding is appropriate for complete implementation of this plan.

INTRODUCTION


snip...


http://gf.state.wy.us/downloads/pdf/CWD2005Plan8-19-05.pdf


WHAT WILL cwdCJD LOOK LIKE IN HUMANS IN WYOMING ???


Subject: Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus)
Date: October 19, 2005 at 8:33 am PST

0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel
Monkeys (Saimiri sciureus)
Richard F. Marsh,1, Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C.
Bartz4*
Department of Animal Health and Biomedical Sciences, University of
Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of
Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska
68178,4 Department of Veterinary Molecular Biology, Montana State
University, Bozeman, Montana 597183

Received 3 May 2005/ Accepted 10 August 2005

Chronic wasting disease (CWD) is an emerging prion disease of deer and elk.
The risk of CWD transmission to humans following exposure to CWD-infected
tissues is unknown. To assess the susceptibility of nonhuman primates to
CWD, two squirrel monkeys were inoculated with brain tissue from a
CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a
progressive neurodegenerative disease and were euthanized at 31 and 34
months postinfection. Brain tissue from the CWD-infected squirrel monkeys
contained the abnormal isoform of the prion protein, PrP-res, and displayed
spongiform degeneration. This is the first reported transmission of CWD to
primates.


----------------------------------------------------------------------------
----

* Corresponding author. Mailing address: Department of Medical Microbiology
and Immunology, Creighton University, 2500 California Plaza, Omaha, NE
68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail:
jbartz@creighton.edu .

Deceased.


----------------------------------------------------------------------------
----

Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21
0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.


http://jvi.asm.org/cgi/content/abstract/79/21/13794maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=cwd&searchid=1129736446553_4280&stored_search=&FIRSTINDEX=0&volume=79&issue=21&journalcode=jvi

SCRAPIE IN WYOMING


Wyoming State Veterinary Laboratory Newsletter Vol 3(#2):July, 2002

Wyoming State Veterinary Laboratory

1174 Snowy Range Road, Laramie, WY 82070

TEL: (800) 442-8331 FAX: (307) 721-2051

http://wyovet.uwyo.edu/

University of Wyoming


snip...


Scrapie in Wyoming

Scrapie infected flocks are being detected at the rate of about one per month in

Wyoming. Live animal testing reveals that classical signs of scrapie (pruritis; severe weight loss)

are not always present. In fact, we have detected several cases of scrapie in completely

asymptomatic sheep. Practitioners should include scrapie as a rule-out whenever a progressive,

debilitating illness is noted in 3 -5 year old sheep, particularly black-face breeds. Please recall

that goats can be infected with scrapie, too.

Wyoming requires that live scrapie suspects be reported. The USDA, in collaboration

with the Wyoming State Veterinary Laboratory and other state diagnostic laboratories, is doing

research on scrapie infected sheep and goats. This research requires numerous tissues be

collected from live suspects (e.g. eyelid biopsy, blood for genetic testing) or that freshly

harvested tissues (e.g. placentomes, gut lymph nodes) be collected at necropsy. Indemnity is

paid for live scrapie suspects. The USDA will provide reasonable transportation costs for

clinically suspect sheep. Live suspects should be transported to Wyoming State Veterinary

Laboratory for necropsy and collection of samples for the USDA.

Please report live clinical scrapie suspects to USDA @ 772-2186 or WLSB @ 777-7515.

John Duncan


http://wyovet.uwyo.edu/WSVL/archives/V3i2.pdf

http://www.aphis.usda.gov/vs/nahps/scrapie/yearly_report/figure06.gif

http://www.aphis.usda.gov/vs/nahps/scrapie/yearly_report/figure07.gif

1 goat scrapie case Wyoming

http://www.aphis.usda.gov/vs/nahps/scrapie/yearly_report/figure09.gif

RSSS postive samples

http://www.aphis.usda.gov/vs/nahps/scrapie/yearly_report/figure13.gif

BSE TESTING WYOMING ???

http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure4f.html

http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure5f.html

http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure6f.html

http://www.aphis.usda.gov/lpa/issues/bse/surveillance/06bse_descriptionf.html

http://www.fda.gov/ohrms/dockets/ac/04/slides/4019S1_2.ppt

http://www.fsis.usda.gov/PPT/BSE_APHIS_Surveillance_alt/index.asp

SADLY, the june 2004 enhanced BSE surveillance, some 500,000 tests,

many were terribly flawwed, giving much doubt to the credibility of this

program all together;

Daily Update

On, November 28, 2005, no inconclusive test results were reported.

APHIS completed testing of 21,216 clinically normal adult animals as part
of its enhanced surveillance program on Nov. 21. All animals tested
negative.

National Veterinary Services Laboratory (NVSL) Immunohistochemistry (IHC) Testing Summary

The BSE enhanced surveillance program involves the use of a rapid screening test, followed by confirmatory testing for any samples that come back "inconclusive." The weekly summary below captures all rapid tests conducted as part of the enhanced surveillance effort. It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing. This was done to ensure that samples inappropriate for the rapid screen test were still tested, and also to monitor and improve upon IHC testing protocols. Of those 9,200 routine tests, one test returned a non-definitive result on July 27, 2005. That sample underwent additional testing at NVSL, as well as at the Veterinary Laboratories Agency in Weybridge, England, and results were negative.

(please note, WB could not be performed due to BSE protocol not being followed again on the non-definitive of july 27, 2005. ...TSS)

http://www.aphis.usda.gov/lpa/issues/bse_testing/test_results.html

Approaches to extrapolate data to broader cattle population:

There are various methods that could be used to extrapolate the data obtained from our targeted surveillance program to the broader cattle population as a whole. While no decisions have been made about which, if any, of these approaches would be used, these examples can illustrate the options available.

One example of an extrapolation of collected data would be as follows. As previously mentioned, based on summary data from testing within the European Union in 20029, detectable cases of BSE were 29.4 times more common in targeted high-risk animals sampled than in apparently normal animals sampled. This ratio is based on detectable BSE. It is possible that BSE may be present but not at a detectable level. This discussion regarding approaches to estimating prevalence in the broader cattle population assumes detectable BSE.

The ratio based on European data can be used to extrapolate data on expected prevalence in additional populations. If we sample 268,500 targeted high-risk animals and find no cases of BSE, then we are 99 percent confident that at most there are not 5 or more cases in the targeted high-risk population of 446,000. Using the ratio of 29.4, we would then expect 0.15 detectable cases per 446,000 animals in the apparently normal population, or 2.2 cases in the 6.4 million adult animals slaughtered annually. This is an extremely low possible prevalence level, and detection of disease at this level in the apparently normal adult animal population would be extremely difficult. This would require sampling of approximately 4.5 million, 5.1 million, or 5.8 million animals to detect this prevalence level at a confidence level of 90 percent, 95 percent, or 99 percent, respectively.

Without testing a very large sample of normal adult animals, no statistical inferences can be made about the prevalence of BSE in the adult population. Any statements of prevalence after completion of testing of the high-risk population would have to be based on the extrapolation from the high-risk population to the normal adult population. For example, if we sample 268,500 high-risk animals and find no positive animals, then assuming at most 2.2 infected animals in the normal adult population, we could conclude there are no more than 7.2 positive animals in the adult population or about 1 per million (7.2 divided by 6.86 million).

http://www.aphis.usda.gov/lpa/issues/bse/BSEOIG.pdf

THE above was IF the testing was done correctly. WE now know this WAS NOT the case. ...TSS

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.

PMID: 6997404


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

12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie
A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.

The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.

It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.

Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"

Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.

snip...


76/10.12/4.6


http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf


Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*

* Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and Institute for Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000 (received for review October 16, 2000)


Abstract


There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.


snip...

Characterization of the CJD and Scrapie Strains. Controls were set up by transmitting one French and one U.S. scrapie isolate from ruminants as well as French sCJD and iCJD cases from humans. None of these revealed a lesion profile or transmission characteristics similar or close to those of BSE or vCJD, respectively, thus extending to the present French scrapie isolate the previous observation that the BSE agent was different from all known natural scrapie strains (4, 24).

The lesion profiles of sCJD and iCJD differed only slightly in severity of the lesions, but not in shape of the profile, revealing the identity of the causative agents. One of us reported the absence of similarity between sCJD (six cases) and U.K. scrapie (eight cases) in transmission characteristics in mice (4). Herein, we made the striking observation that the French natural scrapie strain (but not the U.S. scrapie strain) has the same lesion profile and transmission times in C57BL/6 mice as do the two human TSE strains studied. This strain "affiliation" was confirmed biochemically. There is no epidemiological evidence for a link between sheep scrapie and the occurrence of CJD in humans (25). However, such a link, if it is not a general rule, would be extremely difficult to establish because of the very low incidence of CJD as well as the existence of different isolates in humans and multiple strains in scrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there is still a possibility that in some instances TSE strains infecting humans do share a common origin with scrapie, as pointed out by our findings.


snip...


Conclusions


From BSE and vCJD transmissions in nonhuman primates, a number of conclusions can be drawn that are of major importance for human health: (i) human-adapted BSE appears to be a variant of the BSE agent that is more virulent for humans than cattle BSE and is efficiently transmitted by the peripheral route; (ii) the detection of vCJD in unusually young patients is probably not because of a lack of diagnosis of cases in older patients, thus raising the question of the source of human contamination with BSE early in life; and (iii) iatrogenic transmissions from patients with vCJD would be readily recognized by using the same diagnostic criteria as those applied to vCJD [clinical and pathological criteria (27) comprising neuronal loss and gliosis in the thalamus correlated with high MRI signal (28, 29)], whether such contaminations had occurred by the central or i.v. route. Primary and iatrogenic cases of vCJD could be distinguished on the basis of the patient's clinical history.

The risk assessment of biological products of human origin, notably those derived from blood, has been deeply modified by the appearance of vCJD. We confirm that the BSE agent has contaminated humans not only in the U.K. and the Republic of Ireland but also in France, and we show that its pathogenic properties for primates are being enhanced by a primary passage in humans. Considering the flow of potentially contaminated bovine-derived products between 1980 and 1996, it is obvious that further vCJD cases may occur outside the U.K. Thus, and in the light of the present study, it is necessary to sustain worldwide CJD surveillance regardless of national BSE incidence and to take all precautionary measures to avoid iatrogenic transmissions from vCJD.


snip...


http://www.pnas.org/cgi/content/full/041490898v1


Scrapie USA August 2005 (documented only cases)

Infected and Source Flocks

As of August 31, 2005, there were 115 scrapie infected and source flocks (figure 3). There were 3 new infected and source flocks reported in August (Figure 4) with a total of 148 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 102 (Figure 6), with 5 flocks released in August. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.69 :
1. In addition, as of August 31, 2005, 574 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 122 were RSSS cases (Figure 7). This includes 55 newly confirmed cases in August 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005.

snip...

full text ;

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html


Published online before print October 20, 2005

Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
Medical Sciences

A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes

( sheep prion | transgenic mice )

Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
*Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway


Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.


--------------------------------------------------------------------------------

Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper.

A.L.D. and V.B. contributed equally to this work.

To whom correspondence should be addressed.

Hubert Laude, E-mail: laude@jouy.inra.fr

www.pnas.org/cgi/doi/10.1073/pnas.0502296102


http://www.pnas.org/cgi/content/abstract/0502296102v1


DIAGNOSTIC CRITERIA FOR sporadic CJDs VS nvCJD ???


Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi

Summary

Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of

electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD

classifications.


snip...


The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species?


http://neurology.thelancet.com

Published online October 31, 2005

IS CJD REPORTABLE IN WYOMING ???


Wyoming Department of Health - Reportable Diseases and Conditions

A report is required by law within 7 days of diagnosis (unless otherwise specified) from both the

attending health care provider/hospital and the laboratory performing diagnostic testing. Send reports to:

Epidemiology Section, Wyoming Department of Health, Hathaway Building - Fourth Floor, Cheyenne,

Wyoming, 82002. Fax reports to our secure fax machine at (307) 777-5573 or phone to (307) 777-3593.


snip...


FAX Creutzfeldt-Jakob Disease


snip...


 Diseases in Red: Immediate Notification at 1-888-996-9104 to Coordinate Epidemiology and Laboratory Response

FAX Reportable within 24 Hours of Diagnosis by Fax Machine or Telephone

LAB Diseases in Blue: Submit an Isolate to the Public Health Laboratory (LAB  = 24 Hour Notification & Lab Isolate)

Send laboratory isolates according to IATA Dangerous Goods Regulations to: State Public Health Laboratory, Hathaway

Building - Fifth Floor, 2300 Capitol Avenue, Cheyenne, Wyoming, 82002. Wyoming laboratories are responsible for

reporting results when a reference laboratory is used.

For additional epidemiology or laboratory information, please see our website at: http://wdh.state.wy.us/epiid

Updated: 02/03/05lf


http://wdh.state.wy.us/epiid/reportlist.pdf


miracles never cease to amaze me. ...


TSS




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