Commission requests further investigations on three unusual cases of TSE in sheep
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Reference: IP/06/288 Date: 09/03/2006 HTML: EN FR DE EL
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IP/06/288
Brussels 09 March 2006
Commission requests further investigations on three unusual cases of TSE in sheep
An expert panel from the Community Reference Laboratory in Weybridge has informed the European Commission that test results from the brains of two sheep from France and one from Cyprus show an unusual molecular profile that warrants further investigation, and while some data suggest that the samples may not be BSE in sheep, "there is insufficient evidence to definitively rule out BSE". In line with EU legislation, the European Commission has requested that further investigations be carried out. TSEs are Transmissible Spongiform Encephalopathies, a family of diseases characterised by a degeneration of brain tissue such as Creutzfeldt Jackob Disease in humans, scrapie in sheep and BSE in cows. The Commission, together with Member States, is considering a review of the current TSE monitoring programmes in sheep in order to get a better picture on the significance of these three cases. Following the detection of unusual TSE cases in these sheep in France and Cyprus, , the Commission had asked the CRL to continue investigations. The next level of testing, recommended by the CRL expert panel and which has been requested by the Commission , entails the use of the mouse bioassay test and will take between 12 -18 months to complete. BSE has never been found under natural circumstances in sheep, but the first case of BSE in a goat was confirmed last year (IP/05/132). The sheep now being evaluated were detected as part of the EU-wide surveillance programme for TSE strains in small ruminants. Whatever the final test findings show, there is no risk to public health, as the sheep did not enter the food and feed chain and strict animal health measures are applied to all farmed ruminants.
The two French sheep being tested were born in 2000 and 2002, and died on-farm. The Cypriot sheep which developed clinical signs of a TSE-linked disease was two years old. In line with EU requirements for TSE surveillance in small ruminants, all three animals were subject to post-mortem testing. These first test results were positive for TSE and therefore the samples were submitted for further testing to rule out the possibility of BSE (discriminatory molecular testing). The results from the second level of testing indicated an unusual pattern which warrants further investigation, and while some data suggested that the samples may not be BSE in sheep, there was insufficient evidence to definitively rule out the presence of BSE . For this reason, the CRL will now carry out the third and final level of testing (mouse bioassay), in line with EU legislation.
To date, BSE in sheep has been viewed as possible, but has never been detected. Nevertheless, EU measures against BSE have been applied to all farmed ruminants (cattle, goats, and sheep) to ensure the highest possible level of public health protection.
These safety measures include a ban on the use of animal proteins (meat-and-bone meal) in feedstuffs, the removal of specified risk materials (e.g. brain, spinal cord, part of the intestines) from the food and feed chain, the slaughter of sheep and goat herds affected by scrapie, and the application of a TSE surveillance/monitoring programme. This surveillance was intensified in all Member States last year, following the detection of one positive case of BSE in a goat. Over 1 400 000 sheep and 380 000 goats have been tested in the EU since April 2002.
The Commission will work with Member States in considering a review of the current TSE monitoring programmes in sheep in order to get a better picture of the significance of these three cases. . In addition, the European Food Safety Authority (EFSA) was requested to accelerate the ongoing assessments on the risk that different sheep tissues could pose should BSE be confirmed in sheep, and on the current breeding programme which would favour TSE-resistant sheep.
(See MEMO/06/113)
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Reference: MEMO/06/114 Date: 09/03/2006 HTML: EN
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MEMO/06/114
Brussels ,09 March 2006
Questions and Answers on TSEs in Sheep
What are TSEs (Transmissible Spongiform Encephalopathies)?
TSEs are a family of diseases occurring in man and animals that are characterised by a degeneration of brain tissue giving a sponge-like appearance. The family includes diseases such as Creutzfeldt Jakob Disease (CJD) in humans, Bovine Spongiform Encephalopathy (BSE) in cattle and scrapie in sheep and goats. While BSE has only recently been identified, scrapie has been known about for centuries. On the basis of the available data, scrapie is not considered to be transmissible to humans nor to pose a risk to man. However, as a precautionary measure, EU legislation in place to prevent the spread and transmission of BSE also applies to sheep and to goats.
What is the difference between BSE and Scrapie?
Both BSE and Scrapie belong to a family of TSE diseases, caused by prions. Scrapie has existed for over 200 years in sheep without ever showing any signs that it can be transmitted to humans. BSE is a disease affecting cattle which was seen for the first time in 1986. It was discovered 10 years later that it could be transmitted to humans. BSE has never been detected in sheep before.
What EU legislation applies to small ruminants with regard to TSEs?
The EU TSE Regulation 999/2001 lays down the specific testing requirements for TSEs in small ruminants, as well as eradication measures to be carried out when a TSE is detected (e.g. culling of any sheep and goats infected with scrapie). Preventive measures against BSE, such as the feed ban and the removal of specified risk materials, also apply to small ruminants. The TSE Regulation is directly applicable in all Member States.
Why are samples from three sheep now being investigated?
Through the required monitoring programme for small ruminants laid down in EU legislation, three sheep (two in France, one in Cyprus) were found positive for TSE. Any positive TSE cases must be subjected to further tests to ensure that the TSE present is not BSE. In the case of these sheep, the initial "rule-out" tests identified unusual profiles and while some data suggested that the samples may not be BSE in sheep, there was insufficient evidence to definitively rule out the presence of BSE. This means that further "rule-out" tests are required. The definitive test is a mouse bio-assay, which the Community Reference Laboratory (CRL) will now carry out on tissues from the suspected sheep.
How long until the Community Reference Laboratory (CRL) confirms whether these sheep had BSE or Scrapie?
The CRL will now submit the samples to the third level of discriminatory testing (mouse bio-assay). This requires the inoculation of specifically-bred mice with the infected tissue from the affected sheep.
This inoculation will cause disease in the mice which can then be analysed. The analysis will allow the experts to tell definitively whether the disease in the sheep was really BSE or not. However because this type of testing requires the use of mice, it takes time. Normally 12-18 months will be required to conclude the investigations.
What is the process for testing for TSEs/BSE in the EU?
Monitoring for TSEs in domestic animals in Europe is extensive. All healthy slaughtered cattle over thirty months of age are tested at the abattoir for BSE. All animals over 24 months of age that die on farm or are culled for a disease must also be tested at the rendering plant. Finally, animals that display clinical signs of BSE must also be tested.
As for cattle, all sheep that display clinical signs of TSE must be tested. In addition, the EU monitoring programme requires that random samples be taken from the brains of animals both at the abattoir and at the rendering plant. In 2005, up to 10 000 tests per Member State were carried in healthy slaughtered sheep and as many again in fallen stock, depending on the size of the sheep population (statistically-based sampling).
How extensive is TSE testing in small ruminants?
Over 1 560 000 sheep and 410 000 goats have been tested in the EU since April 2002. This testing will now be increased further, so that 800,000 healthy slaughtered sheep in abattoirs and 140,000 dead sheep in rendering plants will be tested in the next 6 months. The tables below indicate the number of samples taken in sheep and goats from 2002-2005, and the number of those which tested positive for TSE:
Table 1: Summary of TSE testing in Sheep in the EU 25 from 2002 to 2005
Sheep
Number of TSE tests 1 560 010
Number of TSE cases 8 093
Number of discriminatory tests (BSE or scrapie; cases from 1998 to 2005) 4 188
Number of discriminatory tests indicating an unusual strain of TSE 3 (2 France, 1 Cyprus)
Table 2: Summary of TSE testing in Goats in the EU 25 from 2002 to 2005
Goats
Number of TSE tests 411 162
Number of TSE cases 1 111
Number of discriminatory tests (BSE or scrapie; cases from 1998 to 2005) 179
Number of discriminatory tests indicating BSE 1 (the French case in January 2005)
Does this finding of an unusual profile of TSE in the 3 sheep hold any threat for the consumer?
No. It should be remembered that the animals in question were detected due to the normal surveillance measures which have been in place for many years. EU measures against BSE have been applied to all farmed ruminants (cattle, goats, and sheep) to ensure the highest possible level of public health protection. These safety measures include a ban on the use of animal proteins (meat-and-bone meal) in feedstuffs, the removal of specified risk materials (e.g. brain, spinal cord, part of the intestines) from the food and feed chain, the slaughter of sheep and goat herds affected by scrapie, and the application of a TSE surveillance/monitoring programme. So, whatever the final findings show, there is no risk to public health, as the sheep did not enter the food and feed chain and strict animal health measures are applied to all farmed ruminants.
Are mutton and lamb still safe to eat?
Yes, mutton and lamb on the EU market can be considered as safe to eat, as stringent EU animal health and food safety rules apply to all meat sold for consumption. As a precautionary measure and following scientific advice, meat from flocks and cohorts which are affected by a BSE case, cannot enter the food chain. After the detection of BSE in a goat in 2005, the European Food Safety Authority (EFSA) was asked to assess the risk from eating the meat and meat products of goats. EFSA considered the risk to be low, due to the risk management measures already in place. There is currently no reason to expect that the risk from sheep meat would be different from goats. Nonetheless, EFSA has already started a quantative risk assessment on sheep meat (which began before these suspected cases even arose), and are due to give their Opinion before autumn 2006.
Are sheep milk and milk products safe for consumption?
After the detection of BSE in a goat in 2005, the EFSA was asked to assess the risk from milk and milk derivatives from small ruminants. EFSA found that these products from sheep and goats are unlikely to present any risk of TSE contamination, provided that milk is sourced from healthy animals. EU food safety rules ensure that only products from healthy animals are allowed to enter the food chain. Therefore, consumers should not be concerned about any health risk from sheep milk and milk products.
What scientific advice has been requested in relation to BSE in sheep?
Research is ongoing on BSE in sheep. Currently EFSA is studying questions on breeding programmes for TSEs in sheep (e.g. how to breed sheep that are more resistant to TSEs). It is also looking at the issue of specified risk materials, and the level of risk different ovine tissues would pose should BSE be confirmed in sheep. EFSA is expected to report their findings on this research in the coming months.
In light of the current findings in the 3 sheep being tested, how do we know that BSE is in not widespread in sheep?
The extensive testing conducted in Europe to date has only detected 3 possible cases of BSE in sheep, which are now being examined further. This indicates a very low prevalence of the disease. Sheep are more commonly affected by scrapie, and are not considered to be a natural host for BSE.
Have there been any more cases of BSE detected in goats since the confirmed case in 2005?
No further positive cases of BSE have been found in goats since the single case detected in January 2005 (see IP/05/132). This is despite increased in testing of goats (see table above), through which 1 111 positive TSE cases have been picked up since 2002, of which 179 were sent for subsequent discriminatory testing.
For more information on TSEs, see:
http://europa.eu.int/comm/food/food/biosafety/bse/index_en.htm
http://europa.eu.int/rapid/pressReleasesAction.do?reference=MEMO/06/114&format=HTML&aged=0&language=EN
The TSE community reference laboratory strain typing expert group (STEG)
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Reference: MEMO/06/113 Date: 09/03/2006
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MEMO/06/113
Brussels, 9 March 2006
The TSE community reference laboratory strain typing expert group (STEG)
Summary of the STEG opinion on three isolates (05-0825 and 06-0017 from France; 204163425 from Cyprus) referred to the group following the identification of unusual molecular profiles on discriminatory Western blot (as required in EU Regulation 36/2005.)
Report drafted by MMS summarising the email consultation on available test data with the European members of the STEG
Executive Summary
Three cases were referred to the STEG following the identification, in each case, of a low molecular weight unglycosylated band in discriminatory Western blot.
A full ring trial has not been possible for any of these cases.
- Fixed material was not collected from the French cases, thereby precluding discriminatory immunohistochemistry. Data has been reviewed from Western blot (AFSSA) and ELISA (CEA) testing.
- Insufficient fresh tissue was available from the Cypriot case to allow full molecular testing without compromising the availability of material for subsequent bioassay. Data has been reviewed from Western blot (VLA) and discriminatory immunostaining (VLA Lasswade).
Absence of a full panel of test results in ring trial means that even if there had been total conformity of interpretation, unequivocal categorization of the isolates would not be possible at this stage. However, it is clear from the results available (see table) that they do not all concur with the 'BSE-like' outcome of the primary differential screening blot, and consequently would not have been categorized as BSE-like even if all test methods had been applied.
Case no 05-0825 06-0017 204163425
Discriminatory WB BSE-like[1] BSE-like1 BSE-like1
ELISA Intermediate scrapie[2] Intermediate scrapie2 Not done
Discriminatory IHC Not done Not done Intermediate[3]
Conclusion
The conclusions of the group, based on the data currently available, are as follows.
It is not possible to compare IHC, WB and ELISA results for each sample using the evaluated discriminatory methods, therefore the STEG cannot classify any of the samples conclusively as "BSE in sheep".
Nevertheless, additional data from ELISA and IHC suggest that the samples may not be BSE in sheep, since they do not conform to our expectations based on currently available data from experimental ovine BSE. However there is insufficient evidence to definitively rule out the presence of BSE, since the actual sensitivity (negative predictive value) of ELISA and IHC alone are not known, and the current data differ from the great majority of classical scrapie isolates.
All three samples appear to fit into a previously unrecognised/undefined category, similar to cases identified in France in 1996, and in the United Kingdom in 2004, that warrants further investigation by bioassay.
[Graphic in PDF & Word format]
Dr Marion M Simmons
On behalf of the EU CRL for TSE (STEG)
8th March 2006
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[1] There are some minor discrepancies between the glycoform profiles obtained from these samples and those of experimental ovine BSE. However, it is the consensus of the group that glycoform profile alone is not a robust discriminatory criterion.
[2] The result does not fit the criteria for 'BSE-like' by this 'test', 05-0825 and 06-0017 isolates show intermediate resistance to PK treatment which is quite common in a population of "classical" scrapie.
[3] The result does not fit the criteria for 'BSE-like' by this 'test', nor does it match those for classical scrapie isolates. It also does not share the properties of 'atypical' scrapie, as defined in the EFSA Opinion.
http://europa.eu.int/rapid/pressReleasesAction.do?reference=MEMO/06/113&format=HTML&aged=0&language=EN
REPORT OF THE COMMITTEE ON SCRAPIE
Chair: Dr. Jim Logan, Cheyenne, WY
Vice Chair: Dr. Joe D. Ross, Sonora, TX
Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN.
The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance.
The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings.
Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories.
For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory's certification was revoked by APHIS-VS.
snip...
Infected and Source Flocks
As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats.
Regulatory Scrapie Slaughter Surveillance (RSSS)
RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1,
2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below.
Scrapie Testing
In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9).
Animal ID
As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises.
*This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans.
http://www.usaha.org/committees/reports/2005/report-scr-2005.pdf
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.
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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: [email protected]
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY
snip...
A The Present Position with respect to Scrapie
A] The Problem
Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.
The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.
It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.
Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"
Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.
snip...
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
What if you can catch old-fashioned CJD by eating meat from a sheep infected
with scrapie?
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number
of campaigners who say that some sCJD, like the variant CJD related to BSE,
is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in ...
The complete article is 889 words long.
full text;
http://www.newscientist.com/article.ns?id=mg16922840.300
Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*,
Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James
Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*
* Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction
des Sciences du Vivant/Département de Recherche Medicale, Centre de
Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc,
BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre
Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de
Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital,
75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western
General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and
Institute for Animal Health, Neuropathogenesis Unit, West Mains Road,
Edinburgh EH9 3JF, United Kingdom
Edited by D. Carleton Gajdusek, Centre National de la Recherche
Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
(received for review October 16, 2000)
Abstract
There is substantial scientific evidence to support the notion that bovine
spongiform encephalopathy (BSE) has contaminated human beings, causing
variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns
about the possibility of an iatrogenic secondary transmission to humans,
because the biological properties of the primate-adapted BSE agent are
unknown. We show that (i) BSE can be transmitted from primate to primate by
intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD
to humans could be readily recognized pathologically, whether it occurs by
the central or peripheral route. Strain typing in mice demonstrates that the
BSE agent adapts to macaques in the same way as it does to humans and
confirms that the BSE agent is responsible for vCJD not only in the United
Kingdom but also in France. The agent responsible for French iatrogenic
growth hormone-linked CJD taken as a control is very different from vCJD but
is similar to that found in one case of sporadic CJD and one sheep scrapie
isolate. These data will be key in identifying the origin of human cases of
prion disease, including accidental vCJD transmission, and could provide
bases for vCJD risk assessment.
http://www.pnas.org/cgi/content/full/041490898v1
The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization
Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease
G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
Smits2
and B. Caughey1,7
1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: [email protected] Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.
Abstract
Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.
snip...
Clearly, it is premature to draw firm conclusions about CWD
passing naturally into humans, cattle and sheep, but the present
results suggest that CWD transmissions to humans would be as
limited by PrP incompatibility as transmissions of BSE or sheep
scrapie to humans. Although there is no evidence that sheep
scrapie has affected humans, it is likely that BSE has caused variant
CJD in 74 people (definite and probable variant CJD cases to
date according to the UK CJD Surveillance Unit). Given the
presumably large number of people exposed to BSE infectivity,
the susceptibility of humans may still be very low compared with
cattle, which would be consistent with the relatively inefficient
conversion of human PrP-sen by PrPBSE. Nonetheless, since
humans have apparently been infected by BSE, it would seem prudent
to take reasonable measures to limit exposure of humans
(as well as sheep and cattle) to CWD infectivity as has been
recommended for other animal TSEs.
snip...
http://www.emboj.org/current.shtml
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.fcgicmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
Transmission of Creutzfeldt-Jakob disease by handling of dura mater.
The Lancet Volume 341(8837) January 9, 1993 pp
123-124
Weber, Thomas; Tumani, Hayrettin; Holdorff, Bernd; Collinge, John; Palmer,
Mark; Kretzschmar, Hans A.; Felgenhauer, Klaus
Sir,- Creutzfeldt-Jakob disease (CJD) can be transmitted iatrogenically by
human pituitary growth hormone, corneal transplants, and dura mater grafts
(1). Possible accidental transmission has been reported in only four
people-a neurosurgeon (2), a pathologist (3), and two laboratory technicians
(4,5) . We have encountered an unusually rapid case of CJD probably acquired
through handling of sheep and human dura mater.
In May, 1992, a 55-year-old orthopaedic surgeon developed paraesthesia of
the left arm. A few days later he had spatial disorientation, apraxia, and
gait ataxia. In June he was admitted and a neurologist suspected CJD on the
basis of the clinical signs, typical electroencephalogram (EEG) pattern, and
history. An EEG in June revealed a typical pattern of periodic biphasic and
triphasic sharp wave complexes. We saw the patient in July, 1992. He was
awake and oriented, with dyscalculia, dysgraphia, disturbed vision, apraxia
mainly of the left side, rigidity of wrists, spasticity of all muscles,
myoclonus of the left arm, increased tendon reflexes, ataxia of limbs and
trunk, and incoordination of left arm. Within 3 weeks he had impaired
consciousness and attention, mildly impaired memory, and threatening visual
hallucinations with restless turning. He had periodic states with movements
of his head and eye-bulbs resembling tonic adversive seizures. During sleep
these motor disturbances stopped. 2 1/2 months later the patient died.
This patient had worked with sheep and human dura mater from 1968 to 1972.
He handled about 150 specimens of ovine origin and at least a dozen human
preparations for research. Handling involved opening skulls with a band saw,
removing dura, and testing them either fresh (usually), preserved, or
lyophilised for mechanical qualities. These specimens were sent to a company
that has sold dura mater preparations by which CJD was transmitted in six
instances. No information was available from the company about a possible
connection with this patient's disease and the earlier cases of transmitted
CJD. Brain biopsy was consistent with diagnosis of CJD. Cerebrospinal fluid
obtained in July showed neuron-specific enolase (NSE) at 82.0 ng/mL,
compared with 16.7 ng/mL in serum of other cases (6). Proton magnetic
resonance spectroscopy of parieto-occipital and temporal grey matter,
parietal white matter, and thalamus revealed a 20-30% reduction of
N-acetylaspartate, as described (7). DNA was genotyped with allele-specific
oligonucleotides (8) and was homozygous for methionine at the polymorphic
codon 129. Subsequent direct DNA sequencing for the PrP gene open-reading
frame demonstrated normal sequence on both alleles, excluding known or novel
pathogenic PrP mutations.
It is tempting to speculate that prions were transmitted to this patient
from sheep or human dura mater through small lacerations of his skin, but
the patient and his wife did not remember any significant injury during his
four years of working with these samples. It cannot be excluded that this
was a case of sporadic CJD although this assumption is unlikely in view of
the clinical course which was similar to iatrogenic CJD transmitted by
peripheral inoculation, such as with human pituitary growth hormone or
gonadotropin or to kuru (1). Iatrogenic cases resulting from intracerebral
inoculation with the transmissible agent, for instance following dura mater
grafts (2-5), present with a dementing picture, as is usual in sporadic CJD,
rather than with ataxia as in this case.
1. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones,
homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27. [Medline
Link] [Context Link]
2. Schoene WC, Masters CL, Gibbs CJ Jr, et al. Transmissible spongiform
encephalopathy (Creutzfeldt-Jakob Disease): atypical clinical and
pathological findings. Arch Neurol 1981; 38: 473-77. [Medline Link] [Context
Link]
3. Gorman DG, Benson DF, Vogel DG, Vinters HV. Creutzfeldt-Jakob disease in
a pathologist. Neurology 1992; 42: 463. [Medline Link] [Context Link]
4. Miller DC. Creutzfeldt-Jakob disease in histopathology technicians. N
Engl J Med 1988; 318: 853-54. [Medline Link] [Context Link]
5. Sitwell L, Lach B, Atack E, Atack D, Izukawa D. Creutzfeldt-Jakob disease
in histopathology technicians. N Engl J Med 1988; 318: 854. [Medline Link]
[Context Link]
6. Wakayama Y, Shibuya S, Kawase J, Sagawa F, Hashizume Y. High
neuron-specific enolase level of cerebrospinal fluid in the early stage of
Creutzfeldt-Jakob disease. Klin Wochenschr 1987; 65: 798-801. [Medline Link]
[Context Link]
7. Bruhn H, Weber T, Thorwirth V, Frahm J. In-vivo monitoring of neuronal
loss in Creutzfeldt-Jakob disease by proton magnetic resonance spectroscopy.
Lancet 1991; 337: 1610-11. [Medline Link] [Context Link]
8. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic
Creutzfeldt-Jakob disease. Lancet 1991; 337: 1444-42. [Medlin
http://www.mad-cow.org/jul99_late_news.html#ggg
Subject: U.K. SHEEP/GOAT EXPORTS GLOBAL 1988-2001
Date: November 19, 2002 at 12:24 pm PST
In Reply to: EMERGING SHEEP RESEARCH FINDINGS ON BSE IN SHEEP !!! posted by TSS on November 19, 2002 at 10:37 am:
i know this looks like hcll, but will try to get a better version
online later. dang xcel file.......tss
0208h023: UK exports of sheep, goats and sheep/goat meats and meat products (1988 - 2001) 1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 Product EC / Non-EC Country Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Live Sheep EC France 15,175 21,303 17,645 22,355 18,733 22,760 26,943 31,941 43,250 52,482 25,806 31,373 24,812 32,487 15,973 25,012 12,484 21,390 7,188 11,485 8,372 9,022 9,332 8,845 3,815 4,562 1,177 1,491 Netherlands 22 31 54 50 66 65 272 458 1,223 1,593 1,429 1,527 3,122 3,806 2,527 3,833 2,693 3,822 1,318 2,193 829 804 1,232 1,066 2,266 2,255 848 1,012 Belgium-Luxembourg 342 521 981 1,039 258 331 1,568 1,909 3,071 3,328 3,834 4,000 4,064 4,771 1,442 3,584 674 1,215 275 419 43 46 78 9 Irish Republic 2,071 2,438 2,909 3,143 3,133 2,430 3,704 2,180 2,540 1,035 102 129 419 544 16 29 36 48 Germany 132 219 467 664 143 168 1,830 2,094 1,615 1,683 2,771 4,017 2,345 4,250 195 248 379 493 1,503 1,326 2,251 3,079 872 1,441 281 287 Italy 62 85 357 631 20 65 333 495 26 21 409 447 55 73 616 823 1,000 1,662 777 815 2,117 2,409 563 823 9 22 Spain 1 2 7 4 6 8 7 8 38 66 340 380 286 363 192 276 541 922 249 335 138 140 221 212 78 89 23 2 Greece 10 13 13 13 10 10 209 211 1,127 946 386 342 Portugal 4 22 8 26 1 3 53 150 2 2 17 22 36 46 Austria 2 16 3 2 EC Total 17,812 24,637 22,436 27,924 22,217 25,662 33,023 37,310 51,994 60,635 33,534 39,539 35,555 46,094 23,094 37,778 17,604 29,287 9,448 14,975 11,870 12,365 16,281 16,557 8,015 9,557 2,417 2,823 Non-EC Poland 20 77 8 50 Peru 18 34 U.S.A. 16 55 Turkey 13 17 Brazil 12 10 South Korea 8 11 Hungary 1 10 1 9 5 40 1 1 Iran 1 1 6 2 Japan 0 2 0 4 0 7 2 26 0 3 1 8 Dubai 2 4 1 8 Colombia 2 10 Switzerland 1 12 1 4 Canada 2 25 0 2 St. Helena 0 3 0 2 0 5 0 7 1 5 0 3 Kenya 1 13 Israel 1 4 Bahrain 1 6 0 1 Trinidada & Tobago 1 4 0 1 Seychelles 1 4 Oman 0 3 0 6 Falkland Islands 0 3 0 2 Montserrat 0 4 Iceland 0 2 Malta 0 2 Non-EC Total 24 104 14 109 8 68 16 62 57 76 3 38 1 10 1 8 0 6 2 8 3 16 0 2 Live Sheep Total 17,835 24,741 22,451 28,033 22,225 25,730 33,040 37,373 52,051 60,710 33,537 39,577 35,556 46,103 23,095 37,787 17,604 29,293 9,448 14,975 11,870 12,365 16,283 16,565 8,018 9,573 2,417 2,825 Live Goats EC Belgium-Luxembourg 0 1 1 1 3,514 355 128 15 Germany 1,162 58 156 15 France 15 15 0 7 3 14 5 25 0 4 0 0 2 2 850 28 102 11 Italy 1 2 5 19 510 47 Spain 7 5 227 21 Irish Republic 7 8 13 4 7 4 2 1 0 0 Portugal 1 2 5 20 Netherlands 0 2 0 1 0 4 Denmark 0 7 EC Total 23 25 14 17 8 37 12 31 9 25 0 0 2 2 7 5 0 4 6,263 509 386 40 Non-EC Tanzania 1 3 1 4 3 7 9 12 6 10 11 57 9 78 10 93 Trinidada & Tobago 0 1 1 7 11 66 0 2 Kenya 2 8 4 29 3 24 Ethiopia 1 6 3 8 2 11 1 7 Saudi Arabia 0 1 1 6 1 5 2 15 Switzerland 0 6 0 1 1 7 3 19 Barbados 2 20 1 9 Kuwait 0 1 1 8 1 8 0 2 0 2 Dominica 1 6 2 10 0 7 Rwanda 3 17 Jamaica 2 13 St Lucia 0 3 1 22 Abu Dhabi 1 5 1 1 St Vincent 1 5 1 6 Brazil 1 12 Qatar 1 3 Thailand 1 9 Uganda 1 5 Antigua & Barbuda 1 4 Grenada 0 4 St Kitts, Nevis 0 8 Seychelles 0 3 Cyprus 0 1 St. Helena 0 1 0 1 Czech Republic 0 2 India 0 2 Yugoslavia 0 1 U.S.A. 0 0 Non-EC Total 6 42 5 24 11 66 3 23 5 31 7 35 5 30 8 41 13 47 11 56 11 58 14 117 13 122 0 2 Live Goats Total 29 66 18 41 20 103 15 54 14 55 7 35 6 32 8 41 19 52 11 56 11 62 14 117 6,276 631 386 42 Sheepmeat (Fresh or Chilled) EC France 55,651 123,685 66,053 137,378 54,681 114,629 51,887 106,840 69,644 133,413 74,841 153,683 77,529 164,972 107,761 207,181 90,578 219,901 75,449 177,336 76,278 147,207 76,476 135,411 68,275 136,668 22,635 58,948 Belgium-Luxembourg 8,068 20,508 8,539 19,483 8,936 22,715 9,132 21,221 9,281 20,300 10,741 25,372 10,963 24,000 9,610 23,129 9,305 24,714 8,148 20,308 8,430 18,529 8,209 18,582 8,322 17,080 1,651 4,399 Germany 2,714 6,929 3,340 8,591 2,784 7,236 2,118 5,466 4,338 11,208 4,618 11,345 5,425 12,796 7,687 17,149 7,976 20,647 7,912 19,413 7,007 14,710 6,193 12,334 6,597 12,733 1,087 2,700 Spain 1,572 3,641 2,853 7,950 4,203 11,558 5,545 15,016 7,715 19,871 6,267 15,207 7,587 17,717 7,455 17,433 3,498 9,016 2,097 4,748 1,641 3,339 2,633 4,846 1,053 2,124 45 106 Italy 2,264 5,214 2,359 5,478 3,015 7,659 4,198 10,724 4,267 10,559 3,710 8,737 5,850 14,185 5,944 13,325 3,768 8,905 1,978 4,497 4,018 9,163 4,732 9,929 4,040 8,862 995 2,368 Portugal 305 665 569 1,344 360 836 454 1,024 894 2,108 2,104 4,760 3,044 7,013 3,305 7,924 4,865 12,742 3,872 9,011 3,739 6,850 2,963 5,799 2,070 4,024 543 1,312 Greece 53 127 61 122 131 291 490 1,059 1,106 2,433 1,065 2,364 1,993 4,277 1,810 3,788 2,265 4,970 1,764 3,727 743 1,545 1,275 2,023 948 1,418 1 1 Austria 11 24 14 28 46 116 302 648 885 1,896 1,725 4,061 1,576 4,020 1,217 3,322 1,078 2,227 755 1,542 659 1,392 27 60 Netherlands 17 43 89 271 228 716 416 1,298 338 1,190 414 1,675 565 1,914 771 2,286 902 3,237 654 2,518 562 1,548 845 4,355 957 4,624 170 1,004 Irish Republic 68 131 58 95 110 183 72 127 280 446 239 469 313 551 65 112 266 432 221 751 689 875 250 711 310 1,007 378 871 Denmark 1 1 54 133 48 133 48 79 24 46 2 10 129 361 341 678 375 424 86 231 65 128 74 154 6 23 0 1 Sweden 14 39 9 20 14 41 7 17 42 89 14 15 0 3 213 387 99 193 5 10 107 204 187 384 139 291 3 9 Finland 49 83 0 0 60 207 8 26 4 6 14 30 Guadeloupe 11 25 Canary Islands 1 3 Luxembourg 1 4 0 1 EC Total 70,738 161,005 84,047 180,976 74,555 166,114 74,379 162,896 97,928 201,662 104,317 224,285 114,283 249,684 146,688 297,457 125,534 309,408 103,411 245,900 104,356 206,326 104,594 196,069 93,382 190,257 27,550 71,808 Non-EC Switzerland 1,984 4,535 2,177 5,019 1,915 4,682 1,882 4,441 1,557 4,099 1,844 5,003 2,199 5,680 2,112 5,813 1,435 4,700 1,321 4,079 1,077 3,004 906 2,600 1,025 3,162 159 574 Ghana 10 30 11 19 13 44 51 156 94 275 22 58 3 8 0 1 0 2 2 8 6 26 Gibraltar 14 30 7 15 10 35 14 52 30 88 10 52 8 46 4 24 8 28 1 7 1 6 5 10 1 1 Brazil 10 25 0 1 54 112 24 38 17 38 1 3 1 6 0 1 China 22 11 66 54 U.S.A. 1 8 35 73 27 48 4 10 0 2 Turkey 15 33 21 37 3 7 25 39 Niger 49 92 Falkland Islands 32 68 4 3 0 2 2 11 11 32 Bermuda 1 7 5 28 0 1 2 10 2 9 4 19 5 19 9 33 11 56 2 10 Norway 28 55 0 3 11 26 0 1 Japan 0 3 0 1 37 60 1 4 Egypt 10 25 13 33 Abu Dhabi 7 13 0 4 6 7 3 7 3 6 Croatia 3 16 14 28 1 1 Hong Kong 5 13 2 15 5 20 2 13 1 6 0 1 1 6 0 2 0 4 0 2 Cyprus 0 1 16 33 0 1 Canada 1 1 4 8 5 11 4 12 Qatar 13 32 0 2 0 1 0 1 Nigeria 8 7 1 4 0 2 Pakistan 8 16 Faroe Islands 8 10 Iran 2 4 5 14 0 2 Bahamas 1 1 7 13 Hungary 7 12 Israel 3 18 3 7 0 1 Andorra 6 11 Bahrain 0 1 3 8 0 1 1 3 1 6 0 1 Malta 0 1 1 4 1 5 1 8 1 5 0 2 0 2 0 5 0 4 0 3 1 5 Bulgaria 2 4 3 6 Uganda 5 10 Botswana 4 10 Singapore 0 2 2 10 1 5 0 3 0 4 St. Helena 1 1 0 1 3 14 Romania 3 22 Congo (Republic) 3 9 Soviet Union 0 1 3 6 New Zealand 3 4 Gambia 1 3 2 4 Trinidada & Tobago 2 5 Sierra Leone 0 1 0 1 0 2 1 1 0 1 Lebanon 0 1 2 4 0 7 0 1 Nauru 2 4 Antigua & Barbuda 2 2 Burundi 2 2 Grenada 2 7 Czech Republic 2 7 Kazakhstan 1 1 1 3 San Marino 1 5 South Africa 1 1 Kuwait 0 1 1 2 Azerbaijan 1 1 0 1 Jamaica 1 2 Belize 0 1 0 1 0 1 Guatemala 0 2 0 1 0 1 Iceland 1 1 Poland 1 3 Yemen 1 1 Bangladesh 1 3 Vatican City 0 1 Thailand 0 2 0 1 Tanzania 0 1 Mali 0 2 0 1 Oman 0 1 0 1 Jordan 0 1 Argentina 0 1 Burma 0 1 Brunei 0 1 Ivory Coast 0 1 Morocco 0 1 Non-EC Total 2,105 4,803 2,226 5,162 2,096 5,084 2,010 4,775 1,804 4,680 1,986 5,381 2,300 5,864 2,137 5,895 1,456 4,791 1,354 4,182 1,089 3,055 922 2,650 1,046 3,261 191 642 Sheepmeat (Fresh or Chilled) Total 72,843 165,808 86,273 186,138 76,651 171,198 76,389 167,671 99,732 206,343 106,303 229,666 116,583 255,547 148,826 303,352 126,990 314,199 104,765 250,081 105,445 209,381 105,516 198,720 94,427 193,517 27,741 72,451 Sheepmeat (Frozen) EC France 974 1,862 349 743 548 1,316 1,496 2,581 2,766 5,091 1,479 2,951 1,768 3,647 2,069 4,068 2,868 5,699 1,616 3,405 1,481 2,472 1,609 2,606 2,080 3,560 1,197 2,482 Spain 256 359 969 1,752 1,020 2,238 658 1,159 665 1,328 121 265 717 1,540 450 949 305 697 141 417 434 940 208 397 325 751 187 338 Germany 340 604 322 674 199 432 150 281 544 817 119 276 141 324 510 1,046 169 656 382 809 526 1,051 619 1,371 343 1,167 153 400 Italy 532 776 109 232 487 1,197 279 704 357 871 537 1,453 797 1,831 273 841 197 602 124 352 59 181 133 357 224 479 157 357 Irish Republic 0 1 20 44 23 55 68 177 130 227 113 248 62 120 97 185 249 453 299 697 679 1,126 347 760 257 455 503 1,076 Netherlands 3 5 17 24 13 18 107 160 302 583 78 277 204 766 492 1,445 153 434 267 676 381 1,356 161 509 111 562 155 735 Portugal 804 1,118 737 1,029 163 333 214 317 90 124 97 247 1 1 0 0 110 223 71 163 4 22 2 7 0 0 Denmark 15 26 75 125 245 552 247 496 94 211 0 1 107 234 82 138 70 142 50 112 221 382 204 379 86 197 40 108 Belgium-Luxembourg 30 63 130 291 166 508 230 678 334 705 83 254 105 216 182 565 56 234 8 21 9 30 79 193 74 140 10 38 Finland 15 77 17 41 0 1 252 636 160 550 33 107 45 98 59 121 66 109 Sweden 21 94 40 126 12 35 1 1 41 124 28 83 64 267 58 140 163 374 96 202 34 56 Greece 21 23 23 58 13 16 3 4 0 0 1 3 22 52 1 3 2 8 30 77 Canary Islands 1 1 49 97 1 4 11 21 1 5 Austria 1 1 0 2 23 43 0 0 Guadeloupe 15 22 EC Total 2,977 4,840 2,765 4,995 2,962 6,933 3,489 6,679 5,315 10,043 2,640 5,992 3,902 8,680 4,448 10,001 4,413 9,870 3,056 7,027 3,897 7,800 3,584 7,083 3,693 7,698 2,437 5,590 Non-EC Gibraltar 11 23 8 19 20 53 28 47 15 36 24 46 8 22 22 44 69 85 24 66 68 62 17 42 28 63 24 55 Croatia 23 36 75 222 41 69 23 35 19 32 11 21 1 2 Switzerland 0 1 26 93 13 47 1 3 15 30 2 7 0 2 14 47 5 22 8 26 59 181 31 107 Ghana 4 21 10 53 4 20 13 69 4 19 3 5 1 6 3 14 6 39 9 65 14 79 15 79 17 70 Cyprus 1 1 9 21 3 7 7 11 14 26 19 37 5 10 4 9 1 2 7 22 6 18 11 9 Tajikistan 81 4 1 2 1 2 Faroe Islands 18 24 1 1 24 32 30 54 6 10 Falkland Islands 0 1 0 1 21 52 25 73 18 44 1 4 10 33 Lebanon 3 5 34 62 25 49 10 27 4 7 Slovenia 41 52 22 28 0 1 0 0 South Africa 18 6 35 17 1 1 0 0 Congo (Republic) 52 58 0 1 Malta 2 2 22 38 1 1 1 2 1 4 1 1 2 8 1 5 10 8 2 9 2 21 Kazakhstan 5 12 1 2 1 1 32 51 1 3 3 9 0 1 Yemen 2 3 2 1 16 22 7 11 5 11 6 12 1 2 2 4 1 1 Argentina 0 1 41 60 Bermuda 9 49 7 32 7 36 4 23 0 3 0 1 0 1 3 20 3 19 3 16 4 10 0 2 Bulgaria 1 2 21 28 15 22 Bosnia & Herzegovina 0 1 10 18 19 42 Barbados 1 2 5 15 13 40 7 16 China 3 5 22 16 Azerbaijan 1 4 4 15 14 37 2 7 2 5 Russia 1 2 2 7 19 8 Gambia 3 6 3 8 6 16 2 6 2 7 2 5 0 1 0 1 1 3 1 2 Belize 3 4 6 13 12 26 Egypt 12 27 8 8 Norway 1 1 0 1 0 1 17 30 1 1 Chad 18 17 Australia 1 1 17 22 New Zealand 1 1 1 2 16 22 Sierra Leone 1 4 1 1 2 3 0 1 0 1 9 11 4 9 Trinidada & Tobago 17 13 Angola 0 1 6 9 7 9 0 1 1 1 0 0 3 4 St. Helena 1 1 1 2 1 2 0 1 4 11 2 8 3 6 2 2 1 1 2 2 1 2 Antigua & Barbuda 0 2 2 4 3 16 1 7 0 2 6 25 2 7 1 4 Mali 0 2 16 32 0 1 Oman 1 4 14 42 Kyrgyzstan 5 8 10 19 0 1 Mozambique 6 10 7 13 Nigeria 1 2 3 9 0 1 2 5 1 2 1 2 1 6 1 4 Sao Tome-Principe 1 2 1 2 1 2 0 0 2 4 3 9 5 12 Libya 4 7 3 4 0 1 4 10 St Lucia 7 4 1 2 2 7 1 2 Indonesia 0 1 8 9 Hong Kong 3 4 0 2 2 7 1 3 1 2 2 4 0 2 Abu Dhabi 1 2 0 1 4 8 3 6 FYR Macedonia 1 4 6 13 Jamaica 3 9 3 9 1 5 U.S.A. 1 2 0 1 1 1 1 4 0 0 1 2 3 8 Seychelles 6 7 Algeria 6 10 Jordan 5 9 1 3 Colombia 1 3 5 14 Djibouti 2 4 1 2 1 1 2 5 St Kitts, Nevis 4 2 0 1 0 1 Morocco 0 1 0 1 3 7 Pakistan 4 5 Iraq 1 3 2 5 1 1 India 0 2 3 6 0 2 Singapore 1 6 0 2 1 5 0 1 1 1 Syria 1 1 2 4 1 1 0 0 Yugoslavia 3 8 Ethiopia 0 1 3 6 Turkey 0 1 0 1 1 1 1 2 1 1 Saudi Arabia 2 5 0 20 0 1 0 1 Japan 2 6 0 0 Bangladesh 1 6 0 1 0 2 0 2 Taiwan 2 5 Honduras 1 4 1 3 Polar Regions 1 1 1 1 1 3 Venezuela 1 2 0 1 0 1 0 1 Guinea 1 3 Brunei 1 3 Niger 1 4 Soviet Union 1 3 Sudan 1 3 Rwanda 1 2 Poland 1 3 Armenia 1 1 Brazil 1 5 Andorra 1 1 Belarus 1 2 Gabon 1 1 Ivory Coast 0 2 0 1 Grenada 0 1 Yemen (Aden) 0 1 Bahrain 0 1 0 1 Liberia 0 1 South Korea 0 1 Uganda 0 1 Qatar 0 1 0 1 Greenland 0 1 Antarctica 0 1 Bolivia 0 1 San Marino 0 1 Dominica 0 1 Benin 0 1 Ukraine 0 1 Kenya 0 1 Canada 0 0 Mauritius 0 1 Iceland 0 2 Non-EC Total 165 300 123 337 85 272 82 218 102 167 61 134 170 256 335 469 248 590 182 452 162 315 130 348 216 576 175 378 Sheepmeat (Frozen) Total 3,142 5,140 2,887 5,332 3,046 7,205 3,571 6,897 5,417 10,210 2,701 6,126 4,073 8,936 4,783 10,470 4,661 10,460 3,237 7,478 4,059 8,115 3,714 7,431 3,909 8,275 2,612 5,968 Goatmeat (Fresh, Chilled or Frozen) EC France 4 16 16 13 7 8 9 23 11 19 8 14 44 44 15 27 0 0 Irish Republic 1 1 17 20 0 0 0 1 0 6 Spain 17 39 Netherlands 7 41 Germany 3 7 0 0 0 0 3 0 0 0 Italy 1 2 0 0 0 0 Portugal 0 0 0 0 Belgium-Luxembourg 0 0 0 1 Sweden 0 0 EC Total 5 18 1 1 16 13 24 28 9 23 38 106 8 15 44 44 15 28 0 0 0 6 3 0 0 0 Non-EC Switzerland 0 2 Angola 0 2 Non-EC Total 0 2 0 2 Goatmeat (Fresh, Chilled or Frozen) Total 5 18 1 1 16 13 24 28 9 23 39 108 8 15 44 44 15 28 0 2 0 6 3 0 0 0 Sheep and Goat Offal (Fresh or Chilled) EC France 232 213 390 385 151 177 179 163 353 336 408 355 280 276 247 180 132 94 62 48 580 475 702 365 442 208 57 32 Germany 19 25 15 21 13 17 20 11 19 35 186 155 289 175 207 167 209 139 173 124 228 216 179 168 125 111 113 47 Belgium-Luxembourg 4 6 2 2 3 5 134 20 83 19 20 26 23 43 180 350 36 60 19 22 15 18 63 123 68 116 12 7 Greece 62 41 20 7 135 148 31 18 18 5 16 12 51 53 0 0 10 9 1 1 Netherlands 20 16 2 2 29 25 4 3 9 7 16 17 3 4 1 1 131 119 22 9 33 13 26 5 Austria 0 1 1 1 64 53 46 25 19 20 29 24 32 16 24 5 1 0 Irish Republic 11 11 2 1 13 8 22 19 3 3 40 28 4 3 0 0 0 1 13 12 59 44 4 2 Spain 1 1 11 16 25 54 13 52 7 16 5 10 77 31 14 21 1 1 0 0 Italy 7 12 5 11 10 27 0 0 5 5 6 4 18 10 0 0 8 5 18 5 4 1 Denmark 14 33 1 0 1 1 Portugal 1 1 1 1 3 1 0 0 1 0 Finland 2 1 EC Total 348 315 446 444 330 401 409 317 541 483 643 567 779 618 738 795 455 341 274 215 985 853 1,020 699 770 502 218 95 Non-EC Russia 17 12 Brazil 16 12 Lebanon 7 7 Turkey 3 16 3 3 Seychelles 1 13 U.S.A. 1 2 Gibraltar 0 2 Switzerland 0 1 Non-EC Total 3 16 16 12 3 3 1 2 17 12 0 2 8 20 0 1 Sheep and Goat Offal (Fresh or Chilled) Total 351 331 462 456 332 404 410 320 541 483 643 567 796 630 738 797 463 360 274 216 985 853 1,020 699 770 502 218 95 Sheep or Goat Offal (Frozen) EC France 37 14 125 113 100 150 78 86 582 340 455 366 332 353 556 345 168 167 27 23 8 17 28 31 Belgium-Luxembourg 4 4 4 47 4 37 2 2 1 2 22 16 133 151 0 0 383 46 411 39 141 73 Irish Republic 69 52 103 104 46 79 71 56 187 50 96 30 117 80 159 121 118 134 5 2 44 42 35 73 12 8 20 16 Greece 234 354 269 346 178 190 15 8 39 23 53 51 2 2 0 0 Netherlands 73 80 20 4 18 16 25 13 12 26 13 2 149 32 0 0 Germany 13 12 20 21 19 27 9 10 9 9 30 17 6 8 34 28 4 3 16 7 64 82 19 3 1 1 0 0 Spain 17 65 8 44 13 71 12 38 2 7 12 47 16 70 25 115 7 22 13 33 17 18 5 3 14 9 Denmark 5 6 19 17 5 6 3 4 40 9 0 0 Italy 1 11 15 7 1 3 0 0 0 0 0 1 Portugal 3 4 1 0 0 0 0 0 5 3 Austria 0 0 3 2 1 0 Sweden 1 0 3 2 EC Total 426 512 566 667 393 580 233 294 846 493 636 471 483 494 827 588 602 606 58 56 129 175 487 175 430 51 175 98 Non-EC Lithuania 86 38 Russia 38 33 Hong Kong 5 9 5 18 21 11 Iran 18 12 Singapore 1 2 1 3 1 2 15 8 Gibraltar 2 2 1 1 1 3 2 2 1 1 Yemen 7 11 Japan 6 29 Kazakhstan 3 2 U.S.A. 1 3 0 1 1 7 Abu Dhabi 1 2 2 2 Malta 0 1 2 1 Falkland Islands 1 42 0 1 Turkey 1 8 Oman 0 1 1 2 Algeria 1 1 Canada 0 1 St Kitts, Nevis 0 1 New Zealand 0 0 Non-EC Total 1 1 15 21 2 4 1 1 24 40 0 1 48 64 3 49 1 2 23 14 1 2 2 9 89 43 16 9 Sheep or Goat Offal (Frozen) Total 426 513 581 688 395 584 235 294 870 534 637 472 532 558 831 637 602 608 81 71 130 177 489 184 519 93 191 107 Ó Crown Copyright Source: H M Customs and Excise Data prepared by Statistics (Commodities & Food) Accounts and Trade, ESD, DEFRA 2001/2 EU data is provisional and subject to amendment.
http://www.vegsource.com/articles/sheep_exports_files/sheet001.htm
Other US BSE risks: the imported products picture
http://www.mad-cow.org/00/jul00_dont_eat_sheep.html#hhh
-------- Original Message --------
Subject: DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE (similar to exp. BSE in sheep)
Date: Wed, 7 Apr 2004 08:56:36 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: [email protected]
######## Bovine Spongiform Encephalopathy #########
Date: April 07, 2004 Time: 13:45
DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE
The Veterinary Laboratories Agency (VLA) have informed Defra, the
Devolved Administrations and the Food Standards Agency of a type of
scrapie not previously seen in the UK.
The VLA and other European laboratories with expertise in scrapie-like
diseases have now applied several rapid diagnostic methods to tissue
samples from a sheep with suspected scrapie. Some of the methods have
indicated that the case does not appear to resemble previously
recognised cases of scrapie and, although there were differences, it had
some characteristics similar to experimental BSE in sheep and also to an
experimental strain of sheep scrapie. More importantly, though,
microscopic analysis of brain material showed that the case neither
resembled previously recognised types of scrapie or experimental BSE in
sheep.
A meeting of the scientific experts who performed these analyses, held
on the 30th March, concluded that this case could not be considered to
be BSE in sheep, although it does not behave like known types of scrapie
either. Further investigation will be needed before more can be said
about how this unusual result should be described.
Defra's Chief Scientific Adviser, Professor Howard Dalton, said "The UK,
and especially the VLA, have played an important part in improving the
diagnostic methods available for identifying TSEs in sheep. As we
continue to assess more samples with these improved methods it is likely
that we will continue to find samples, such as this, which fall outside
our current knowledge of the disease. Defra, as it does with all
research, will continue to consult scientific experts to ensure that we
are investigating these cases using the best available techniques and
methods."
The National Scrapie Plan remains unaffected by this new result and SEAC
will be consulted in the near future.
Notes to editors
1. Scrapie is a fatal neurological sheep disease belonging to a group of
diseases called transmissible spongiform encephalopathies (TSEs),
including BSE in cattle and CJD in humans. It has been present in the
national flock for over 250 years. It is not considered to be
transmissible to humans.
2. There is a theoretical risk that BSE could be present in sheep,
masked by scrapie, but it has not been found naturally occurring in sheep.
3. There is as yet no definitive diagnostic method that can rapidly
distinguish between different TSEs for example scrapie from BSE.
Consequently, from time to time the scrapie surveillance programmes in
EU member states throw up unusual results that merit further
investigations (Defra press release 371/03 refers
http://www.defra.gov.uk/news/2003/030911a.htm)
4. The VLA have applied several different methods to the sample to
compare it to a wide range of previously detected scrapie cases,
experimental BSE in sheep and an experimental strain of scrapie, termed
CH1461. Two main methods have been used in this analysis:-
a. Western blot (WB)
This involves taking a sample of the brain and treating it with an
enzyme proteinase k to destroy the normal prion protein (PrPC). The
diseased form of the protein (PrPSc) is able to withstand this treatment
and is then separated from other cellular material on a gel. A blot is
taken of the gel and the PrPSc is visualised using specific antibodies.
b. Immunohistochemistry (IHC)
This involves taking thin slices of the brain, and by using special
(antibody) markers to detect the PrPSc it is possible to see disease
specific patterns of PrPSc distribution in the brain under a microscope.
The Western blot method found that the sample did not appear to resemble
previously recognised cases of scrapie and, although there were some
differences, some characteristics were similar to experimental BSE in
sheep and also the experimental strain of sheep scrapie, CH1461. IHC
found that it neither resembled previously recognised types of scrapie
or experimental BSE in sheep
5. The tissue sample has now been analysed using a total of 5 different
diagnostic methods claiming to be able to differentiate between scrapie
and experimental BSE in sheep. Two were performed at the VLA and three
were performed in other European laboratories.
6. The VLA is the European Reference Laboratory for TSEs and is
responsible for co-ordinating such investigations into unusual cases.
Their findings will be considered by the European Food Safety
Authority's committee of TSE experts and in the UK by the Spongiform
Encephalopathy Advisory Committee (SEAC).
7. The genotype of the suspect sheep was ARQ/ARQ which is known to be
susceptible to some strains of scrapie and, in experiments, to BSE.
Background information on scrapie, scrapie genotyping, and the National
Scrapie Plan is published on the Defra internet at www.defra.gov.uk/nsp.
8. For information and advice on BSE in sheep from the FSA please
consult their web site at www.foodstandards.gov.uk
Public enquiries 08459 335577;
Press notices are available on our website
www.defra.gov.uk
Defra's aim is sustainable development
End
Nobel House
17 Smith Square
London SW1P 3JR
Website www.defra.gov.uk
http://www.wired-gov.net/
TSS
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
TSE in Sheep Contingency Planning Assessment of Risk due to BSE
Infectivity from Disposal of Sheep A report for DEFRA November 2001
Management Summary It has been recognised for a considerable time that
sheep in the United Kingdom may have been infected with BSE. To date no
evidence has been found to demonstrate that the national flock is
actually infected with the disease. DEFRA have prepared a draft
contingency plan in the event that BSE were to be identified in UK
sheep. The worst case scenario under this plan is the disposal of the
entire UK flock, some 40 million sheep and lambs. This study has
estimated the potential exposure of the UK population to BSE infectivity
present in sheep in the event that this plan had to be put into effect.
http://www.defra.gov.uk/animalh/bse/bse-publications/seac/DNVReport.pdf
but who would have guessed that such an important experiment/study would
have gotton so screwed up, by not being able to tell a sheep brain from
a cow brain;
© DEFRA 2002 Item 3- Scrapie Brain pool experiments- Update on current
position and audits of samples 3.1 Members were updated on experiments
conducted at the Institute of Animal Health (IAH) to examine a pool of
scrapie brains collected in the early 1990s for evidence of BSE. SEAC
had previously recommended that the material should be examined by DNA
analys
--------------------------------------------------------------------------------
Reference: IP/06/288 Date: 09/03/2006 HTML: EN FR DE EL
PDF: EN FR DE EL
DOC: EN FR DE EL
IP/06/288
Brussels 09 March 2006
Commission requests further investigations on three unusual cases of TSE in sheep
An expert panel from the Community Reference Laboratory in Weybridge has informed the European Commission that test results from the brains of two sheep from France and one from Cyprus show an unusual molecular profile that warrants further investigation, and while some data suggest that the samples may not be BSE in sheep, "there is insufficient evidence to definitively rule out BSE". In line with EU legislation, the European Commission has requested that further investigations be carried out. TSEs are Transmissible Spongiform Encephalopathies, a family of diseases characterised by a degeneration of brain tissue such as Creutzfeldt Jackob Disease in humans, scrapie in sheep and BSE in cows. The Commission, together with Member States, is considering a review of the current TSE monitoring programmes in sheep in order to get a better picture on the significance of these three cases. Following the detection of unusual TSE cases in these sheep in France and Cyprus, , the Commission had asked the CRL to continue investigations. The next level of testing, recommended by the CRL expert panel and which has been requested by the Commission , entails the use of the mouse bioassay test and will take between 12 -18 months to complete. BSE has never been found under natural circumstances in sheep, but the first case of BSE in a goat was confirmed last year (IP/05/132). The sheep now being evaluated were detected as part of the EU-wide surveillance programme for TSE strains in small ruminants. Whatever the final test findings show, there is no risk to public health, as the sheep did not enter the food and feed chain and strict animal health measures are applied to all farmed ruminants.
The two French sheep being tested were born in 2000 and 2002, and died on-farm. The Cypriot sheep which developed clinical signs of a TSE-linked disease was two years old. In line with EU requirements for TSE surveillance in small ruminants, all three animals were subject to post-mortem testing. These first test results were positive for TSE and therefore the samples were submitted for further testing to rule out the possibility of BSE (discriminatory molecular testing). The results from the second level of testing indicated an unusual pattern which warrants further investigation, and while some data suggested that the samples may not be BSE in sheep, there was insufficient evidence to definitively rule out the presence of BSE . For this reason, the CRL will now carry out the third and final level of testing (mouse bioassay), in line with EU legislation.
To date, BSE in sheep has been viewed as possible, but has never been detected. Nevertheless, EU measures against BSE have been applied to all farmed ruminants (cattle, goats, and sheep) to ensure the highest possible level of public health protection.
These safety measures include a ban on the use of animal proteins (meat-and-bone meal) in feedstuffs, the removal of specified risk materials (e.g. brain, spinal cord, part of the intestines) from the food and feed chain, the slaughter of sheep and goat herds affected by scrapie, and the application of a TSE surveillance/monitoring programme. This surveillance was intensified in all Member States last year, following the detection of one positive case of BSE in a goat. Over 1 400 000 sheep and 380 000 goats have been tested in the EU since April 2002.
The Commission will work with Member States in considering a review of the current TSE monitoring programmes in sheep in order to get a better picture of the significance of these three cases. . In addition, the European Food Safety Authority (EFSA) was requested to accelerate the ongoing assessments on the risk that different sheep tissues could pose should BSE be confirmed in sheep, and on the current breeding programme which would favour TSE-resistant sheep.
(See MEMO/06/113)
http://europa.eu.int/rapid/pressReleasesAction.do?reference=IP/06/288&format=HTML&aged=0&language=EN
Reference: MEMO/06/114 Date: 09/03/2006 HTML: EN
PDF: EN
DOC: EN
MEMO/06/114
Brussels ,09 March 2006
Questions and Answers on TSEs in Sheep
What are TSEs (Transmissible Spongiform Encephalopathies)?
TSEs are a family of diseases occurring in man and animals that are characterised by a degeneration of brain tissue giving a sponge-like appearance. The family includes diseases such as Creutzfeldt Jakob Disease (CJD) in humans, Bovine Spongiform Encephalopathy (BSE) in cattle and scrapie in sheep and goats. While BSE has only recently been identified, scrapie has been known about for centuries. On the basis of the available data, scrapie is not considered to be transmissible to humans nor to pose a risk to man. However, as a precautionary measure, EU legislation in place to prevent the spread and transmission of BSE also applies to sheep and to goats.
What is the difference between BSE and Scrapie?
Both BSE and Scrapie belong to a family of TSE diseases, caused by prions. Scrapie has existed for over 200 years in sheep without ever showing any signs that it can be transmitted to humans. BSE is a disease affecting cattle which was seen for the first time in 1986. It was discovered 10 years later that it could be transmitted to humans. BSE has never been detected in sheep before.
What EU legislation applies to small ruminants with regard to TSEs?
The EU TSE Regulation 999/2001 lays down the specific testing requirements for TSEs in small ruminants, as well as eradication measures to be carried out when a TSE is detected (e.g. culling of any sheep and goats infected with scrapie). Preventive measures against BSE, such as the feed ban and the removal of specified risk materials, also apply to small ruminants. The TSE Regulation is directly applicable in all Member States.
Why are samples from three sheep now being investigated?
Through the required monitoring programme for small ruminants laid down in EU legislation, three sheep (two in France, one in Cyprus) were found positive for TSE. Any positive TSE cases must be subjected to further tests to ensure that the TSE present is not BSE. In the case of these sheep, the initial "rule-out" tests identified unusual profiles and while some data suggested that the samples may not be BSE in sheep, there was insufficient evidence to definitively rule out the presence of BSE. This means that further "rule-out" tests are required. The definitive test is a mouse bio-assay, which the Community Reference Laboratory (CRL) will now carry out on tissues from the suspected sheep.
How long until the Community Reference Laboratory (CRL) confirms whether these sheep had BSE or Scrapie?
The CRL will now submit the samples to the third level of discriminatory testing (mouse bio-assay). This requires the inoculation of specifically-bred mice with the infected tissue from the affected sheep.
This inoculation will cause disease in the mice which can then be analysed. The analysis will allow the experts to tell definitively whether the disease in the sheep was really BSE or not. However because this type of testing requires the use of mice, it takes time. Normally 12-18 months will be required to conclude the investigations.
What is the process for testing for TSEs/BSE in the EU?
Monitoring for TSEs in domestic animals in Europe is extensive. All healthy slaughtered cattle over thirty months of age are tested at the abattoir for BSE. All animals over 24 months of age that die on farm or are culled for a disease must also be tested at the rendering plant. Finally, animals that display clinical signs of BSE must also be tested.
As for cattle, all sheep that display clinical signs of TSE must be tested. In addition, the EU monitoring programme requires that random samples be taken from the brains of animals both at the abattoir and at the rendering plant. In 2005, up to 10 000 tests per Member State were carried in healthy slaughtered sheep and as many again in fallen stock, depending on the size of the sheep population (statistically-based sampling).
How extensive is TSE testing in small ruminants?
Over 1 560 000 sheep and 410 000 goats have been tested in the EU since April 2002. This testing will now be increased further, so that 800,000 healthy slaughtered sheep in abattoirs and 140,000 dead sheep in rendering plants will be tested in the next 6 months. The tables below indicate the number of samples taken in sheep and goats from 2002-2005, and the number of those which tested positive for TSE:
Table 1: Summary of TSE testing in Sheep in the EU 25 from 2002 to 2005
Sheep
Number of TSE tests 1 560 010
Number of TSE cases 8 093
Number of discriminatory tests (BSE or scrapie; cases from 1998 to 2005) 4 188
Number of discriminatory tests indicating an unusual strain of TSE 3 (2 France, 1 Cyprus)
Table 2: Summary of TSE testing in Goats in the EU 25 from 2002 to 2005
Goats
Number of TSE tests 411 162
Number of TSE cases 1 111
Number of discriminatory tests (BSE or scrapie; cases from 1998 to 2005) 179
Number of discriminatory tests indicating BSE 1 (the French case in January 2005)
Does this finding of an unusual profile of TSE in the 3 sheep hold any threat for the consumer?
No. It should be remembered that the animals in question were detected due to the normal surveillance measures which have been in place for many years. EU measures against BSE have been applied to all farmed ruminants (cattle, goats, and sheep) to ensure the highest possible level of public health protection. These safety measures include a ban on the use of animal proteins (meat-and-bone meal) in feedstuffs, the removal of specified risk materials (e.g. brain, spinal cord, part of the intestines) from the food and feed chain, the slaughter of sheep and goat herds affected by scrapie, and the application of a TSE surveillance/monitoring programme. So, whatever the final findings show, there is no risk to public health, as the sheep did not enter the food and feed chain and strict animal health measures are applied to all farmed ruminants.
Are mutton and lamb still safe to eat?
Yes, mutton and lamb on the EU market can be considered as safe to eat, as stringent EU animal health and food safety rules apply to all meat sold for consumption. As a precautionary measure and following scientific advice, meat from flocks and cohorts which are affected by a BSE case, cannot enter the food chain. After the detection of BSE in a goat in 2005, the European Food Safety Authority (EFSA) was asked to assess the risk from eating the meat and meat products of goats. EFSA considered the risk to be low, due to the risk management measures already in place. There is currently no reason to expect that the risk from sheep meat would be different from goats. Nonetheless, EFSA has already started a quantative risk assessment on sheep meat (which began before these suspected cases even arose), and are due to give their Opinion before autumn 2006.
Are sheep milk and milk products safe for consumption?
After the detection of BSE in a goat in 2005, the EFSA was asked to assess the risk from milk and milk derivatives from small ruminants. EFSA found that these products from sheep and goats are unlikely to present any risk of TSE contamination, provided that milk is sourced from healthy animals. EU food safety rules ensure that only products from healthy animals are allowed to enter the food chain. Therefore, consumers should not be concerned about any health risk from sheep milk and milk products.
What scientific advice has been requested in relation to BSE in sheep?
Research is ongoing on BSE in sheep. Currently EFSA is studying questions on breeding programmes for TSEs in sheep (e.g. how to breed sheep that are more resistant to TSEs). It is also looking at the issue of specified risk materials, and the level of risk different ovine tissues would pose should BSE be confirmed in sheep. EFSA is expected to report their findings on this research in the coming months.
In light of the current findings in the 3 sheep being tested, how do we know that BSE is in not widespread in sheep?
The extensive testing conducted in Europe to date has only detected 3 possible cases of BSE in sheep, which are now being examined further. This indicates a very low prevalence of the disease. Sheep are more commonly affected by scrapie, and are not considered to be a natural host for BSE.
Have there been any more cases of BSE detected in goats since the confirmed case in 2005?
No further positive cases of BSE have been found in goats since the single case detected in January 2005 (see IP/05/132). This is despite increased in testing of goats (see table above), through which 1 111 positive TSE cases have been picked up since 2002, of which 179 were sent for subsequent discriminatory testing.
For more information on TSEs, see:
http://europa.eu.int/comm/food/food/biosafety/bse/index_en.htm
http://europa.eu.int/rapid/pressReleasesAction.do?reference=MEMO/06/114&format=HTML&aged=0&language=EN
The TSE community reference laboratory strain typing expert group (STEG)
--------------------------------------------------------------------------------
Reference: MEMO/06/113 Date: 09/03/2006
HTML: EN
PDF: EN
DOC: EN
MEMO/06/113
Brussels, 9 March 2006
The TSE community reference laboratory strain typing expert group (STEG)
Summary of the STEG opinion on three isolates (05-0825 and 06-0017 from France; 204163425 from Cyprus) referred to the group following the identification of unusual molecular profiles on discriminatory Western blot (as required in EU Regulation 36/2005.)
Report drafted by MMS summarising the email consultation on available test data with the European members of the STEG
Executive Summary
Three cases were referred to the STEG following the identification, in each case, of a low molecular weight unglycosylated band in discriminatory Western blot.
A full ring trial has not been possible for any of these cases.
- Fixed material was not collected from the French cases, thereby precluding discriminatory immunohistochemistry. Data has been reviewed from Western blot (AFSSA) and ELISA (CEA) testing.
- Insufficient fresh tissue was available from the Cypriot case to allow full molecular testing without compromising the availability of material for subsequent bioassay. Data has been reviewed from Western blot (VLA) and discriminatory immunostaining (VLA Lasswade).
Absence of a full panel of test results in ring trial means that even if there had been total conformity of interpretation, unequivocal categorization of the isolates would not be possible at this stage. However, it is clear from the results available (see table) that they do not all concur with the 'BSE-like' outcome of the primary differential screening blot, and consequently would not have been categorized as BSE-like even if all test methods had been applied.
Case no 05-0825 06-0017 204163425
Discriminatory WB BSE-like[1] BSE-like1 BSE-like1
ELISA Intermediate scrapie[2] Intermediate scrapie2 Not done
Discriminatory IHC Not done Not done Intermediate[3]
Conclusion
The conclusions of the group, based on the data currently available, are as follows.
It is not possible to compare IHC, WB and ELISA results for each sample using the evaluated discriminatory methods, therefore the STEG cannot classify any of the samples conclusively as "BSE in sheep".
Nevertheless, additional data from ELISA and IHC suggest that the samples may not be BSE in sheep, since they do not conform to our expectations based on currently available data from experimental ovine BSE. However there is insufficient evidence to definitively rule out the presence of BSE, since the actual sensitivity (negative predictive value) of ELISA and IHC alone are not known, and the current data differ from the great majority of classical scrapie isolates.
All three samples appear to fit into a previously unrecognised/undefined category, similar to cases identified in France in 1996, and in the United Kingdom in 2004, that warrants further investigation by bioassay.
[Graphic in PDF & Word format]
Dr Marion M Simmons
On behalf of the EU CRL for TSE (STEG)
8th March 2006
--------------------------------------------------------------------------------
[1] There are some minor discrepancies between the glycoform profiles obtained from these samples and those of experimental ovine BSE. However, it is the consensus of the group that glycoform profile alone is not a robust discriminatory criterion.
[2] The result does not fit the criteria for 'BSE-like' by this 'test', 05-0825 and 06-0017 isolates show intermediate resistance to PK treatment which is quite common in a population of "classical" scrapie.
[3] The result does not fit the criteria for 'BSE-like' by this 'test', nor does it match those for classical scrapie isolates. It also does not share the properties of 'atypical' scrapie, as defined in the EFSA Opinion.
http://europa.eu.int/rapid/pressReleasesAction.do?reference=MEMO/06/113&format=HTML&aged=0&language=EN
REPORT OF THE COMMITTEE ON SCRAPIE
Chair: Dr. Jim Logan, Cheyenne, WY
Vice Chair: Dr. Joe D. Ross, Sonora, TX
Dr. Deborah L. Brennan, MS; Dr. Beth Carlson, ND; Dr. John R. Clifford, DC; Dr. Thomas F. Conner, OH; Dr. Walter E. Cook, WY; Dr. Wayne E. Cunningham, CO; Dr. Jerry W. Diemer, TX; Dr. Anita J. Edmondson, CA; Dr. Dee Ellis, TX; Dr. Lisa A. Ferguson, MD; Dr. Keith R. Forbes, NY; Dr. R. David Glauer, OH; Dr. James R. Grady, CO; Dr. William L. Hartmann, MN; Dr. Carolyn Inch, CAN; Dr. Susan J. Keller, ND; Dr. Allen M. Knowles, TN; Dr. Thomas F. Linfield, MT; Dr. Michael R. Marshall, UT; Dr. Cheryl A. Miller, In; Dr. Brian V. Noland, CO; Dr. Charles Palmer, CA; Dr. Kristine R. Petrini, MN; Mr. Stan Potratz, IA; Mr. Paul E. Rodgers, CO; Dr. Joan D. Rowe, CA; Dr. Pamela L. Smith, IA; Dr. Diane L. Sutton, MD; Dr. Lynn Anne Tesar, SD; Dr. Delwin D. Wilmot, NE; Dr. Nora E. Wineland, CO; Dr. Cindy B. Wolf, MN.
The Committee met on November 9, 2005, from 8:00am until 11:55am, Hershey Lodge and Convention Center, Hershey, Pennsylvania. The meeting was called to order by Dr. Jim Logan, chair, with vice chairman Dr. Joe D. Ross attending. There were 74 people in attendance.
The Scrapie Program Update was provided by Dr. Diane Sutton, National Scrapie Program Coordinator, United States Department of Agriculture (USDA), Animal and Plant Health Inspection Services (APHIS), Veterinary Services (VS). The complete text of the Status Report is included in these Proceedings.
Dr. Patricia Meinhardt, USDA-APHIS-VS-National Veterinary Services Laboratory (NVSL) gave the Update on Genotyping Labs and Discrepancies in Results. NVSL conducts investigations into discrepancies on genotype testing results associated with the Scrapie Eradication Program. It is the policy of the Program to conduct a second genotype test at a second laboratory on certain individual animals. Occasionally, there are discrepancies in those results. The NVSL conducts follow-up on these situations through additional testing on additional samples from the field and archive samples from the testing laboratories.
For the period of time from January 1, 2005, until October 15, 2005, there were 23 instances of discrepancies in results from 35 flocks. Of those 23 instances, 14 were caused by laboratory error (paperwork or sample mix-up), 3 results from field error, 5 were not completely resolved, and 1 originated from the use of a non-approved laboratory for the first test. As a result of inconsistencies, one laboratory's certification was revoked by APHIS-VS.
snip...
Infected and Source Flocks
As of September 30, 2005, there were 105 scrapie infected and source flocks. There were a total of 165** new infected and source flocks reported for FY 2005. The total infected and source flocks that have been released in FY 2005 was 128. The ratio of infected and source flocks cleaned up or placed on clean up plans vs. new infected and source flocks discovered in FY 2005 was 1.03 : 1*. In addition 622 scrapie cases were confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2005, of which 130 were RSSS cases. Fifteen cases of scrapie in goats have been reported since 1990. The last goat case was reported in May 2005. Approximately 5,626 animals were indemnified comprised of 49% non-registered sheep, 45% registered sheep, 1.4% non-registered goats and 4.6% registered goats.
Regulatory Scrapie Slaughter Surveillance (RSSS)
RSSS was designed to utilize the findings of the Center for Epidemiology and Animal Health (CEAH) Scrapie: Ovine Slaughter Surveillance (SOSS) study. The results of SOSS can be found at http://www.aphis.usda.gov/vs/ceah/cahm/Sheep/sheep.htm . RSSS started April 1,
2003. It is a targeted slaughter surveillance program which is designed to identify infected flocks for clean-up. During FY 2005 collections increased by 32% overall and by 90% for black and mottled faced sheep improving overall program effectiveness and efficiency as demonstrated by the 26% decrease in percent positive black faced sheep compared to FY 2004. Samples have been collected from 62,864 sheep since April 1, 2003, of which results have been reported for 59,105 of which 209 were confirmed positive. During FY 2005, 33,137 samples were collected from 81 plants. There have been 130 NVSL confirmed positive cases (30 collected in FY 2004 and confirmed in FY 2005 and 100 collected and confirmed in FY 2005) in FY 2005. Face colors of these positives were 114 black, 14 mottled, 1 white and 1 unknown. The percent positive by face color is shown in the chart below.
Scrapie Testing
In FY 2005, 35,845 animals have been tested for scrapie: 30,192 RSSS; 4,742 regulatory field cases; 772 regulatory third eyelid biopsies; 10 third eyelid validations; and 129 necropsy validations (chart 9).
Animal ID
As of October 04, 2005, 103,580 sheep and goat premises have been assigned identification numbers in the Scrapie National Generic Database. Official eartags have been issued to 73,807 of these premises.
*This number based on an adjusted 12 month interval to accommodate the 60 day period for setting up flock plans.
http://www.usaha.org/committees/reports/2005/report-scr-2005.pdf
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: [email protected]
www.pnas.org/cgi/doi/10.1073/pnas.0502296102
http://www.pnas.org/cgi/content/abstract/0502296102v1
12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY
snip...
A The Present Position with respect to Scrapie
A] The Problem
Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.
The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.
It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.
Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"
Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.
snip...
76/10.12/4.6
http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
What if you can catch old-fashioned CJD by eating meat from a sheep infected
with scrapie?
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number
of campaigners who say that some sCJD, like the variant CJD related to BSE,
is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in ...
The complete article is 889 words long.
full text;
http://www.newscientist.com/article.ns?id=mg16922840.300
Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*,
Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James
Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*
* Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction
des Sciences du Vivant/Département de Recherche Medicale, Centre de
Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc,
BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre
Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de
Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital,
75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western
General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and
Institute for Animal Health, Neuropathogenesis Unit, West Mains Road,
Edinburgh EH9 3JF, United Kingdom
Edited by D. Carleton Gajdusek, Centre National de la Recherche
Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
(received for review October 16, 2000)
Abstract
There is substantial scientific evidence to support the notion that bovine
spongiform encephalopathy (BSE) has contaminated human beings, causing
variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns
about the possibility of an iatrogenic secondary transmission to humans,
because the biological properties of the primate-adapted BSE agent are
unknown. We show that (i) BSE can be transmitted from primate to primate by
intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD
to humans could be readily recognized pathologically, whether it occurs by
the central or peripheral route. Strain typing in mice demonstrates that the
BSE agent adapts to macaques in the same way as it does to humans and
confirms that the BSE agent is responsible for vCJD not only in the United
Kingdom but also in France. The agent responsible for French iatrogenic
growth hormone-linked CJD taken as a control is very different from vCJD but
is similar to that found in one case of sporadic CJD and one sheep scrapie
isolate. These data will be key in identifying the origin of human cases of
prion disease, including accidental vCJD transmission, and could provide
bases for vCJD risk assessment.
http://www.pnas.org/cgi/content/full/041490898v1
The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization
Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease
G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
Smits2
and B. Caughey1,7
1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: [email protected] Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.
Abstract
Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.
snip...
Clearly, it is premature to draw firm conclusions about CWD
passing naturally into humans, cattle and sheep, but the present
results suggest that CWD transmissions to humans would be as
limited by PrP incompatibility as transmissions of BSE or sheep
scrapie to humans. Although there is no evidence that sheep
scrapie has affected humans, it is likely that BSE has caused variant
CJD in 74 people (definite and probable variant CJD cases to
date according to the UK CJD Surveillance Unit). Given the
presumably large number of people exposed to BSE infectivity,
the susceptibility of humans may still be very low compared with
cattle, which would be consistent with the relatively inefficient
conversion of human PrP-sen by PrPBSE. Nonetheless, since
humans have apparently been infected by BSE, it would seem prudent
to take reasonable measures to limit exposure of humans
(as well as sheep and cattle) to CWD infectivity as has been
recommended for other animal TSEs.
snip...
http://www.emboj.org/current.shtml
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.fcgicmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
Transmission of Creutzfeldt-Jakob disease by handling of dura mater.
The Lancet Volume 341(8837) January 9, 1993 pp
123-124
Weber, Thomas; Tumani, Hayrettin; Holdorff, Bernd; Collinge, John; Palmer,
Mark; Kretzschmar, Hans A.; Felgenhauer, Klaus
Sir,- Creutzfeldt-Jakob disease (CJD) can be transmitted iatrogenically by
human pituitary growth hormone, corneal transplants, and dura mater grafts
(1). Possible accidental transmission has been reported in only four
people-a neurosurgeon (2), a pathologist (3), and two laboratory technicians
(4,5) . We have encountered an unusually rapid case of CJD probably acquired
through handling of sheep and human dura mater.
In May, 1992, a 55-year-old orthopaedic surgeon developed paraesthesia of
the left arm. A few days later he had spatial disorientation, apraxia, and
gait ataxia. In June he was admitted and a neurologist suspected CJD on the
basis of the clinical signs, typical electroencephalogram (EEG) pattern, and
history. An EEG in June revealed a typical pattern of periodic biphasic and
triphasic sharp wave complexes. We saw the patient in July, 1992. He was
awake and oriented, with dyscalculia, dysgraphia, disturbed vision, apraxia
mainly of the left side, rigidity of wrists, spasticity of all muscles,
myoclonus of the left arm, increased tendon reflexes, ataxia of limbs and
trunk, and incoordination of left arm. Within 3 weeks he had impaired
consciousness and attention, mildly impaired memory, and threatening visual
hallucinations with restless turning. He had periodic states with movements
of his head and eye-bulbs resembling tonic adversive seizures. During sleep
these motor disturbances stopped. 2 1/2 months later the patient died.
This patient had worked with sheep and human dura mater from 1968 to 1972.
He handled about 150 specimens of ovine origin and at least a dozen human
preparations for research. Handling involved opening skulls with a band saw,
removing dura, and testing them either fresh (usually), preserved, or
lyophilised for mechanical qualities. These specimens were sent to a company
that has sold dura mater preparations by which CJD was transmitted in six
instances. No information was available from the company about a possible
connection with this patient's disease and the earlier cases of transmitted
CJD. Brain biopsy was consistent with diagnosis of CJD. Cerebrospinal fluid
obtained in July showed neuron-specific enolase (NSE) at 82.0 ng/mL,
compared with 16.7 ng/mL in serum of other cases (6). Proton magnetic
resonance spectroscopy of parieto-occipital and temporal grey matter,
parietal white matter, and thalamus revealed a 20-30% reduction of
N-acetylaspartate, as described (7). DNA was genotyped with allele-specific
oligonucleotides (8) and was homozygous for methionine at the polymorphic
codon 129. Subsequent direct DNA sequencing for the PrP gene open-reading
frame demonstrated normal sequence on both alleles, excluding known or novel
pathogenic PrP mutations.
It is tempting to speculate that prions were transmitted to this patient
from sheep or human dura mater through small lacerations of his skin, but
the patient and his wife did not remember any significant injury during his
four years of working with these samples. It cannot be excluded that this
was a case of sporadic CJD although this assumption is unlikely in view of
the clinical course which was similar to iatrogenic CJD transmitted by
peripheral inoculation, such as with human pituitary growth hormone or
gonadotropin or to kuru (1). Iatrogenic cases resulting from intracerebral
inoculation with the transmissible agent, for instance following dura mater
grafts (2-5), present with a dementing picture, as is usual in sporadic CJD,
rather than with ataxia as in this case.
1. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones,
homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27. [Medline
Link] [Context Link]
2. Schoene WC, Masters CL, Gibbs CJ Jr, et al. Transmissible spongiform
encephalopathy (Creutzfeldt-Jakob Disease): atypical clinical and
pathological findings. Arch Neurol 1981; 38: 473-77. [Medline Link] [Context
Link]
3. Gorman DG, Benson DF, Vogel DG, Vinters HV. Creutzfeldt-Jakob disease in
a pathologist. Neurology 1992; 42: 463. [Medline Link] [Context Link]
4. Miller DC. Creutzfeldt-Jakob disease in histopathology technicians. N
Engl J Med 1988; 318: 853-54. [Medline Link] [Context Link]
5. Sitwell L, Lach B, Atack E, Atack D, Izukawa D. Creutzfeldt-Jakob disease
in histopathology technicians. N Engl J Med 1988; 318: 854. [Medline Link]
[Context Link]
6. Wakayama Y, Shibuya S, Kawase J, Sagawa F, Hashizume Y. High
neuron-specific enolase level of cerebrospinal fluid in the early stage of
Creutzfeldt-Jakob disease. Klin Wochenschr 1987; 65: 798-801. [Medline Link]
[Context Link]
7. Bruhn H, Weber T, Thorwirth V, Frahm J. In-vivo monitoring of neuronal
loss in Creutzfeldt-Jakob disease by proton magnetic resonance spectroscopy.
Lancet 1991; 337: 1610-11. [Medline Link] [Context Link]
8. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic
Creutzfeldt-Jakob disease. Lancet 1991; 337: 1444-42. [Medlin
http://www.mad-cow.org/jul99_late_news.html#ggg
Subject: U.K. SHEEP/GOAT EXPORTS GLOBAL 1988-2001
Date: November 19, 2002 at 12:24 pm PST
In Reply to: EMERGING SHEEP RESEARCH FINDINGS ON BSE IN SHEEP !!! posted by TSS on November 19, 2002 at 10:37 am:
i know this looks like hcll, but will try to get a better version
online later. dang xcel file.......tss
0208h023: UK exports of sheep, goats and sheep/goat meats and meat products (1988 - 2001) 1988 1989 1990 1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 Product EC / Non-EC Country Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Tonnes £ thousand Live Sheep EC France 15,175 21,303 17,645 22,355 18,733 22,760 26,943 31,941 43,250 52,482 25,806 31,373 24,812 32,487 15,973 25,012 12,484 21,390 7,188 11,485 8,372 9,022 9,332 8,845 3,815 4,562 1,177 1,491 Netherlands 22 31 54 50 66 65 272 458 1,223 1,593 1,429 1,527 3,122 3,806 2,527 3,833 2,693 3,822 1,318 2,193 829 804 1,232 1,066 2,266 2,255 848 1,012 Belgium-Luxembourg 342 521 981 1,039 258 331 1,568 1,909 3,071 3,328 3,834 4,000 4,064 4,771 1,442 3,584 674 1,215 275 419 43 46 78 9 Irish Republic 2,071 2,438 2,909 3,143 3,133 2,430 3,704 2,180 2,540 1,035 102 129 419 544 16 29 36 48 Germany 132 219 467 664 143 168 1,830 2,094 1,615 1,683 2,771 4,017 2,345 4,250 195 248 379 493 1,503 1,326 2,251 3,079 872 1,441 281 287 Italy 62 85 357 631 20 65 333 495 26 21 409 447 55 73 616 823 1,000 1,662 777 815 2,117 2,409 563 823 9 22 Spain 1 2 7 4 6 8 7 8 38 66 340 380 286 363 192 276 541 922 249 335 138 140 221 212 78 89 23 2 Greece 10 13 13 13 10 10 209 211 1,127 946 386 342 Portugal 4 22 8 26 1 3 53 150 2 2 17 22 36 46 Austria 2 16 3 2 EC Total 17,812 24,637 22,436 27,924 22,217 25,662 33,023 37,310 51,994 60,635 33,534 39,539 35,555 46,094 23,094 37,778 17,604 29,287 9,448 14,975 11,870 12,365 16,281 16,557 8,015 9,557 2,417 2,823 Non-EC Poland 20 77 8 50 Peru 18 34 U.S.A. 16 55 Turkey 13 17 Brazil 12 10 South Korea 8 11 Hungary 1 10 1 9 5 40 1 1 Iran 1 1 6 2 Japan 0 2 0 4 0 7 2 26 0 3 1 8 Dubai 2 4 1 8 Colombia 2 10 Switzerland 1 12 1 4 Canada 2 25 0 2 St. Helena 0 3 0 2 0 5 0 7 1 5 0 3 Kenya 1 13 Israel 1 4 Bahrain 1 6 0 1 Trinidada & Tobago 1 4 0 1 Seychelles 1 4 Oman 0 3 0 6 Falkland Islands 0 3 0 2 Montserrat 0 4 Iceland 0 2 Malta 0 2 Non-EC Total 24 104 14 109 8 68 16 62 57 76 3 38 1 10 1 8 0 6 2 8 3 16 0 2 Live Sheep Total 17,835 24,741 22,451 28,033 22,225 25,730 33,040 37,373 52,051 60,710 33,537 39,577 35,556 46,103 23,095 37,787 17,604 29,293 9,448 14,975 11,870 12,365 16,283 16,565 8,018 9,573 2,417 2,825 Live Goats EC Belgium-Luxembourg 0 1 1 1 3,514 355 128 15 Germany 1,162 58 156 15 France 15 15 0 7 3 14 5 25 0 4 0 0 2 2 850 28 102 11 Italy 1 2 5 19 510 47 Spain 7 5 227 21 Irish Republic 7 8 13 4 7 4 2 1 0 0 Portugal 1 2 5 20 Netherlands 0 2 0 1 0 4 Denmark 0 7 EC Total 23 25 14 17 8 37 12 31 9 25 0 0 2 2 7 5 0 4 6,263 509 386 40 Non-EC Tanzania 1 3 1 4 3 7 9 12 6 10 11 57 9 78 10 93 Trinidada & Tobago 0 1 1 7 11 66 0 2 Kenya 2 8 4 29 3 24 Ethiopia 1 6 3 8 2 11 1 7 Saudi Arabia 0 1 1 6 1 5 2 15 Switzerland 0 6 0 1 1 7 3 19 Barbados 2 20 1 9 Kuwait 0 1 1 8 1 8 0 2 0 2 Dominica 1 6 2 10 0 7 Rwanda 3 17 Jamaica 2 13 St Lucia 0 3 1 22 Abu Dhabi 1 5 1 1 St Vincent 1 5 1 6 Brazil 1 12 Qatar 1 3 Thailand 1 9 Uganda 1 5 Antigua & Barbuda 1 4 Grenada 0 4 St Kitts, Nevis 0 8 Seychelles 0 3 Cyprus 0 1 St. Helena 0 1 0 1 Czech Republic 0 2 India 0 2 Yugoslavia 0 1 U.S.A. 0 0 Non-EC Total 6 42 5 24 11 66 3 23 5 31 7 35 5 30 8 41 13 47 11 56 11 58 14 117 13 122 0 2 Live Goats Total 29 66 18 41 20 103 15 54 14 55 7 35 6 32 8 41 19 52 11 56 11 62 14 117 6,276 631 386 42 Sheepmeat (Fresh or Chilled) EC France 55,651 123,685 66,053 137,378 54,681 114,629 51,887 106,840 69,644 133,413 74,841 153,683 77,529 164,972 107,761 207,181 90,578 219,901 75,449 177,336 76,278 147,207 76,476 135,411 68,275 136,668 22,635 58,948 Belgium-Luxembourg 8,068 20,508 8,539 19,483 8,936 22,715 9,132 21,221 9,281 20,300 10,741 25,372 10,963 24,000 9,610 23,129 9,305 24,714 8,148 20,308 8,430 18,529 8,209 18,582 8,322 17,080 1,651 4,399 Germany 2,714 6,929 3,340 8,591 2,784 7,236 2,118 5,466 4,338 11,208 4,618 11,345 5,425 12,796 7,687 17,149 7,976 20,647 7,912 19,413 7,007 14,710 6,193 12,334 6,597 12,733 1,087 2,700 Spain 1,572 3,641 2,853 7,950 4,203 11,558 5,545 15,016 7,715 19,871 6,267 15,207 7,587 17,717 7,455 17,433 3,498 9,016 2,097 4,748 1,641 3,339 2,633 4,846 1,053 2,124 45 106 Italy 2,264 5,214 2,359 5,478 3,015 7,659 4,198 10,724 4,267 10,559 3,710 8,737 5,850 14,185 5,944 13,325 3,768 8,905 1,978 4,497 4,018 9,163 4,732 9,929 4,040 8,862 995 2,368 Portugal 305 665 569 1,344 360 836 454 1,024 894 2,108 2,104 4,760 3,044 7,013 3,305 7,924 4,865 12,742 3,872 9,011 3,739 6,850 2,963 5,799 2,070 4,024 543 1,312 Greece 53 127 61 122 131 291 490 1,059 1,106 2,433 1,065 2,364 1,993 4,277 1,810 3,788 2,265 4,970 1,764 3,727 743 1,545 1,275 2,023 948 1,418 1 1 Austria 11 24 14 28 46 116 302 648 885 1,896 1,725 4,061 1,576 4,020 1,217 3,322 1,078 2,227 755 1,542 659 1,392 27 60 Netherlands 17 43 89 271 228 716 416 1,298 338 1,190 414 1,675 565 1,914 771 2,286 902 3,237 654 2,518 562 1,548 845 4,355 957 4,624 170 1,004 Irish Republic 68 131 58 95 110 183 72 127 280 446 239 469 313 551 65 112 266 432 221 751 689 875 250 711 310 1,007 378 871 Denmark 1 1 54 133 48 133 48 79 24 46 2 10 129 361 341 678 375 424 86 231 65 128 74 154 6 23 0 1 Sweden 14 39 9 20 14 41 7 17 42 89 14 15 0 3 213 387 99 193 5 10 107 204 187 384 139 291 3 9 Finland 49 83 0 0 60 207 8 26 4 6 14 30 Guadeloupe 11 25 Canary Islands 1 3 Luxembourg 1 4 0 1 EC Total 70,738 161,005 84,047 180,976 74,555 166,114 74,379 162,896 97,928 201,662 104,317 224,285 114,283 249,684 146,688 297,457 125,534 309,408 103,411 245,900 104,356 206,326 104,594 196,069 93,382 190,257 27,550 71,808 Non-EC Switzerland 1,984 4,535 2,177 5,019 1,915 4,682 1,882 4,441 1,557 4,099 1,844 5,003 2,199 5,680 2,112 5,813 1,435 4,700 1,321 4,079 1,077 3,004 906 2,600 1,025 3,162 159 574 Ghana 10 30 11 19 13 44 51 156 94 275 22 58 3 8 0 1 0 2 2 8 6 26 Gibraltar 14 30 7 15 10 35 14 52 30 88 10 52 8 46 4 24 8 28 1 7 1 6 5 10 1 1 Brazil 10 25 0 1 54 112 24 38 17 38 1 3 1 6 0 1 China 22 11 66 54 U.S.A. 1 8 35 73 27 48 4 10 0 2 Turkey 15 33 21 37 3 7 25 39 Niger 49 92 Falkland Islands 32 68 4 3 0 2 2 11 11 32 Bermuda 1 7 5 28 0 1 2 10 2 9 4 19 5 19 9 33 11 56 2 10 Norway 28 55 0 3 11 26 0 1 Japan 0 3 0 1 37 60 1 4 Egypt 10 25 13 33 Abu Dhabi 7 13 0 4 6 7 3 7 3 6 Croatia 3 16 14 28 1 1 Hong Kong 5 13 2 15 5 20 2 13 1 6 0 1 1 6 0 2 0 4 0 2 Cyprus 0 1 16 33 0 1 Canada 1 1 4 8 5 11 4 12 Qatar 13 32 0 2 0 1 0 1 Nigeria 8 7 1 4 0 2 Pakistan 8 16 Faroe Islands 8 10 Iran 2 4 5 14 0 2 Bahamas 1 1 7 13 Hungary 7 12 Israel 3 18 3 7 0 1 Andorra 6 11 Bahrain 0 1 3 8 0 1 1 3 1 6 0 1 Malta 0 1 1 4 1 5 1 8 1 5 0 2 0 2 0 5 0 4 0 3 1 5 Bulgaria 2 4 3 6 Uganda 5 10 Botswana 4 10 Singapore 0 2 2 10 1 5 0 3 0 4 St. Helena 1 1 0 1 3 14 Romania 3 22 Congo (Republic) 3 9 Soviet Union 0 1 3 6 New Zealand 3 4 Gambia 1 3 2 4 Trinidada & Tobago 2 5 Sierra Leone 0 1 0 1 0 2 1 1 0 1 Lebanon 0 1 2 4 0 7 0 1 Nauru 2 4 Antigua & Barbuda 2 2 Burundi 2 2 Grenada 2 7 Czech Republic 2 7 Kazakhstan 1 1 1 3 San Marino 1 5 South Africa 1 1 Kuwait 0 1 1 2 Azerbaijan 1 1 0 1 Jamaica 1 2 Belize 0 1 0 1 0 1 Guatemala 0 2 0 1 0 1 Iceland 1 1 Poland 1 3 Yemen 1 1 Bangladesh 1 3 Vatican City 0 1 Thailand 0 2 0 1 Tanzania 0 1 Mali 0 2 0 1 Oman 0 1 0 1 Jordan 0 1 Argentina 0 1 Burma 0 1 Brunei 0 1 Ivory Coast 0 1 Morocco 0 1 Non-EC Total 2,105 4,803 2,226 5,162 2,096 5,084 2,010 4,775 1,804 4,680 1,986 5,381 2,300 5,864 2,137 5,895 1,456 4,791 1,354 4,182 1,089 3,055 922 2,650 1,046 3,261 191 642 Sheepmeat (Fresh or Chilled) Total 72,843 165,808 86,273 186,138 76,651 171,198 76,389 167,671 99,732 206,343 106,303 229,666 116,583 255,547 148,826 303,352 126,990 314,199 104,765 250,081 105,445 209,381 105,516 198,720 94,427 193,517 27,741 72,451 Sheepmeat (Frozen) EC France 974 1,862 349 743 548 1,316 1,496 2,581 2,766 5,091 1,479 2,951 1,768 3,647 2,069 4,068 2,868 5,699 1,616 3,405 1,481 2,472 1,609 2,606 2,080 3,560 1,197 2,482 Spain 256 359 969 1,752 1,020 2,238 658 1,159 665 1,328 121 265 717 1,540 450 949 305 697 141 417 434 940 208 397 325 751 187 338 Germany 340 604 322 674 199 432 150 281 544 817 119 276 141 324 510 1,046 169 656 382 809 526 1,051 619 1,371 343 1,167 153 400 Italy 532 776 109 232 487 1,197 279 704 357 871 537 1,453 797 1,831 273 841 197 602 124 352 59 181 133 357 224 479 157 357 Irish Republic 0 1 20 44 23 55 68 177 130 227 113 248 62 120 97 185 249 453 299 697 679 1,126 347 760 257 455 503 1,076 Netherlands 3 5 17 24 13 18 107 160 302 583 78 277 204 766 492 1,445 153 434 267 676 381 1,356 161 509 111 562 155 735 Portugal 804 1,118 737 1,029 163 333 214 317 90 124 97 247 1 1 0 0 110 223 71 163 4 22 2 7 0 0 Denmark 15 26 75 125 245 552 247 496 94 211 0 1 107 234 82 138 70 142 50 112 221 382 204 379 86 197 40 108 Belgium-Luxembourg 30 63 130 291 166 508 230 678 334 705 83 254 105 216 182 565 56 234 8 21 9 30 79 193 74 140 10 38 Finland 15 77 17 41 0 1 252 636 160 550 33 107 45 98 59 121 66 109 Sweden 21 94 40 126 12 35 1 1 41 124 28 83 64 267 58 140 163 374 96 202 34 56 Greece 21 23 23 58 13 16 3 4 0 0 1 3 22 52 1 3 2 8 30 77 Canary Islands 1 1 49 97 1 4 11 21 1 5 Austria 1 1 0 2 23 43 0 0 Guadeloupe 15 22 EC Total 2,977 4,840 2,765 4,995 2,962 6,933 3,489 6,679 5,315 10,043 2,640 5,992 3,902 8,680 4,448 10,001 4,413 9,870 3,056 7,027 3,897 7,800 3,584 7,083 3,693 7,698 2,437 5,590 Non-EC Gibraltar 11 23 8 19 20 53 28 47 15 36 24 46 8 22 22 44 69 85 24 66 68 62 17 42 28 63 24 55 Croatia 23 36 75 222 41 69 23 35 19 32 11 21 1 2 Switzerland 0 1 26 93 13 47 1 3 15 30 2 7 0 2 14 47 5 22 8 26 59 181 31 107 Ghana 4 21 10 53 4 20 13 69 4 19 3 5 1 6 3 14 6 39 9 65 14 79 15 79 17 70 Cyprus 1 1 9 21 3 7 7 11 14 26 19 37 5 10 4 9 1 2 7 22 6 18 11 9 Tajikistan 81 4 1 2 1 2 Faroe Islands 18 24 1 1 24 32 30 54 6 10 Falkland Islands 0 1 0 1 21 52 25 73 18 44 1 4 10 33 Lebanon 3 5 34 62 25 49 10 27 4 7 Slovenia 41 52 22 28 0 1 0 0 South Africa 18 6 35 17 1 1 0 0 Congo (Republic) 52 58 0 1 Malta 2 2 22 38 1 1 1 2 1 4 1 1 2 8 1 5 10 8 2 9 2 21 Kazakhstan 5 12 1 2 1 1 32 51 1 3 3 9 0 1 Yemen 2 3 2 1 16 22 7 11 5 11 6 12 1 2 2 4 1 1 Argentina 0 1 41 60 Bermuda 9 49 7 32 7 36 4 23 0 3 0 1 0 1 3 20 3 19 3 16 4 10 0 2 Bulgaria 1 2 21 28 15 22 Bosnia & Herzegovina 0 1 10 18 19 42 Barbados 1 2 5 15 13 40 7 16 China 3 5 22 16 Azerbaijan 1 4 4 15 14 37 2 7 2 5 Russia 1 2 2 7 19 8 Gambia 3 6 3 8 6 16 2 6 2 7 2 5 0 1 0 1 1 3 1 2 Belize 3 4 6 13 12 26 Egypt 12 27 8 8 Norway 1 1 0 1 0 1 17 30 1 1 Chad 18 17 Australia 1 1 17 22 New Zealand 1 1 1 2 16 22 Sierra Leone 1 4 1 1 2 3 0 1 0 1 9 11 4 9 Trinidada & Tobago 17 13 Angola 0 1 6 9 7 9 0 1 1 1 0 0 3 4 St. Helena 1 1 1 2 1 2 0 1 4 11 2 8 3 6 2 2 1 1 2 2 1 2 Antigua & Barbuda 0 2 2 4 3 16 1 7 0 2 6 25 2 7 1 4 Mali 0 2 16 32 0 1 Oman 1 4 14 42 Kyrgyzstan 5 8 10 19 0 1 Mozambique 6 10 7 13 Nigeria 1 2 3 9 0 1 2 5 1 2 1 2 1 6 1 4 Sao Tome-Principe 1 2 1 2 1 2 0 0 2 4 3 9 5 12 Libya 4 7 3 4 0 1 4 10 St Lucia 7 4 1 2 2 7 1 2 Indonesia 0 1 8 9 Hong Kong 3 4 0 2 2 7 1 3 1 2 2 4 0 2 Abu Dhabi 1 2 0 1 4 8 3 6 FYR Macedonia 1 4 6 13 Jamaica 3 9 3 9 1 5 U.S.A. 1 2 0 1 1 1 1 4 0 0 1 2 3 8 Seychelles 6 7 Algeria 6 10 Jordan 5 9 1 3 Colombia 1 3 5 14 Djibouti 2 4 1 2 1 1 2 5 St Kitts, Nevis 4 2 0 1 0 1 Morocco 0 1 0 1 3 7 Pakistan 4 5 Iraq 1 3 2 5 1 1 India 0 2 3 6 0 2 Singapore 1 6 0 2 1 5 0 1 1 1 Syria 1 1 2 4 1 1 0 0 Yugoslavia 3 8 Ethiopia 0 1 3 6 Turkey 0 1 0 1 1 1 1 2 1 1 Saudi Arabia 2 5 0 20 0 1 0 1 Japan 2 6 0 0 Bangladesh 1 6 0 1 0 2 0 2 Taiwan 2 5 Honduras 1 4 1 3 Polar Regions 1 1 1 1 1 3 Venezuela 1 2 0 1 0 1 0 1 Guinea 1 3 Brunei 1 3 Niger 1 4 Soviet Union 1 3 Sudan 1 3 Rwanda 1 2 Poland 1 3 Armenia 1 1 Brazil 1 5 Andorra 1 1 Belarus 1 2 Gabon 1 1 Ivory Coast 0 2 0 1 Grenada 0 1 Yemen (Aden) 0 1 Bahrain 0 1 0 1 Liberia 0 1 South Korea 0 1 Uganda 0 1 Qatar 0 1 0 1 Greenland 0 1 Antarctica 0 1 Bolivia 0 1 San Marino 0 1 Dominica 0 1 Benin 0 1 Ukraine 0 1 Kenya 0 1 Canada 0 0 Mauritius 0 1 Iceland 0 2 Non-EC Total 165 300 123 337 85 272 82 218 102 167 61 134 170 256 335 469 248 590 182 452 162 315 130 348 216 576 175 378 Sheepmeat (Frozen) Total 3,142 5,140 2,887 5,332 3,046 7,205 3,571 6,897 5,417 10,210 2,701 6,126 4,073 8,936 4,783 10,470 4,661 10,460 3,237 7,478 4,059 8,115 3,714 7,431 3,909 8,275 2,612 5,968 Goatmeat (Fresh, Chilled or Frozen) EC France 4 16 16 13 7 8 9 23 11 19 8 14 44 44 15 27 0 0 Irish Republic 1 1 17 20 0 0 0 1 0 6 Spain 17 39 Netherlands 7 41 Germany 3 7 0 0 0 0 3 0 0 0 Italy 1 2 0 0 0 0 Portugal 0 0 0 0 Belgium-Luxembourg 0 0 0 1 Sweden 0 0 EC Total 5 18 1 1 16 13 24 28 9 23 38 106 8 15 44 44 15 28 0 0 0 6 3 0 0 0 Non-EC Switzerland 0 2 Angola 0 2 Non-EC Total 0 2 0 2 Goatmeat (Fresh, Chilled or Frozen) Total 5 18 1 1 16 13 24 28 9 23 39 108 8 15 44 44 15 28 0 2 0 6 3 0 0 0 Sheep and Goat Offal (Fresh or Chilled) EC France 232 213 390 385 151 177 179 163 353 336 408 355 280 276 247 180 132 94 62 48 580 475 702 365 442 208 57 32 Germany 19 25 15 21 13 17 20 11 19 35 186 155 289 175 207 167 209 139 173 124 228 216 179 168 125 111 113 47 Belgium-Luxembourg 4 6 2 2 3 5 134 20 83 19 20 26 23 43 180 350 36 60 19 22 15 18 63 123 68 116 12 7 Greece 62 41 20 7 135 148 31 18 18 5 16 12 51 53 0 0 10 9 1 1 Netherlands 20 16 2 2 29 25 4 3 9 7 16 17 3 4 1 1 131 119 22 9 33 13 26 5 Austria 0 1 1 1 64 53 46 25 19 20 29 24 32 16 24 5 1 0 Irish Republic 11 11 2 1 13 8 22 19 3 3 40 28 4 3 0 0 0 1 13 12 59 44 4 2 Spain 1 1 11 16 25 54 13 52 7 16 5 10 77 31 14 21 1 1 0 0 Italy 7 12 5 11 10 27 0 0 5 5 6 4 18 10 0 0 8 5 18 5 4 1 Denmark 14 33 1 0 1 1 Portugal 1 1 1 1 3 1 0 0 1 0 Finland 2 1 EC Total 348 315 446 444 330 401 409 317 541 483 643 567 779 618 738 795 455 341 274 215 985 853 1,020 699 770 502 218 95 Non-EC Russia 17 12 Brazil 16 12 Lebanon 7 7 Turkey 3 16 3 3 Seychelles 1 13 U.S.A. 1 2 Gibraltar 0 2 Switzerland 0 1 Non-EC Total 3 16 16 12 3 3 1 2 17 12 0 2 8 20 0 1 Sheep and Goat Offal (Fresh or Chilled) Total 351 331 462 456 332 404 410 320 541 483 643 567 796 630 738 797 463 360 274 216 985 853 1,020 699 770 502 218 95 Sheep or Goat Offal (Frozen) EC France 37 14 125 113 100 150 78 86 582 340 455 366 332 353 556 345 168 167 27 23 8 17 28 31 Belgium-Luxembourg 4 4 4 47 4 37 2 2 1 2 22 16 133 151 0 0 383 46 411 39 141 73 Irish Republic 69 52 103 104 46 79 71 56 187 50 96 30 117 80 159 121 118 134 5 2 44 42 35 73 12 8 20 16 Greece 234 354 269 346 178 190 15 8 39 23 53 51 2 2 0 0 Netherlands 73 80 20 4 18 16 25 13 12 26 13 2 149 32 0 0 Germany 13 12 20 21 19 27 9 10 9 9 30 17 6 8 34 28 4 3 16 7 64 82 19 3 1 1 0 0 Spain 17 65 8 44 13 71 12 38 2 7 12 47 16 70 25 115 7 22 13 33 17 18 5 3 14 9 Denmark 5 6 19 17 5 6 3 4 40 9 0 0 Italy 1 11 15 7 1 3 0 0 0 0 0 1 Portugal 3 4 1 0 0 0 0 0 5 3 Austria 0 0 3 2 1 0 Sweden 1 0 3 2 EC Total 426 512 566 667 393 580 233 294 846 493 636 471 483 494 827 588 602 606 58 56 129 175 487 175 430 51 175 98 Non-EC Lithuania 86 38 Russia 38 33 Hong Kong 5 9 5 18 21 11 Iran 18 12 Singapore 1 2 1 3 1 2 15 8 Gibraltar 2 2 1 1 1 3 2 2 1 1 Yemen 7 11 Japan 6 29 Kazakhstan 3 2 U.S.A. 1 3 0 1 1 7 Abu Dhabi 1 2 2 2 Malta 0 1 2 1 Falkland Islands 1 42 0 1 Turkey 1 8 Oman 0 1 1 2 Algeria 1 1 Canada 0 1 St Kitts, Nevis 0 1 New Zealand 0 0 Non-EC Total 1 1 15 21 2 4 1 1 24 40 0 1 48 64 3 49 1 2 23 14 1 2 2 9 89 43 16 9 Sheep or Goat Offal (Frozen) Total 426 513 581 688 395 584 235 294 870 534 637 472 532 558 831 637 602 608 81 71 130 177 489 184 519 93 191 107 Ó Crown Copyright Source: H M Customs and Excise Data prepared by Statistics (Commodities & Food) Accounts and Trade, ESD, DEFRA 2001/2 EU data is provisional and subject to amendment.
http://www.vegsource.com/articles/sheep_exports_files/sheet001.htm
Other US BSE risks: the imported products picture
http://www.mad-cow.org/00/jul00_dont_eat_sheep.html#hhh
-------- Original Message --------
Subject: DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE (similar to exp. BSE in sheep)
Date: Wed, 7 Apr 2004 08:56:36 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: [email protected]
######## Bovine Spongiform Encephalopathy #########
Date: April 07, 2004 Time: 13:45
DEFRA INVESTIGATES AN UNUSUAL SCRAPIE CASE
The Veterinary Laboratories Agency (VLA) have informed Defra, the
Devolved Administrations and the Food Standards Agency of a type of
scrapie not previously seen in the UK.
The VLA and other European laboratories with expertise in scrapie-like
diseases have now applied several rapid diagnostic methods to tissue
samples from a sheep with suspected scrapie. Some of the methods have
indicated that the case does not appear to resemble previously
recognised cases of scrapie and, although there were differences, it had
some characteristics similar to experimental BSE in sheep and also to an
experimental strain of sheep scrapie. More importantly, though,
microscopic analysis of brain material showed that the case neither
resembled previously recognised types of scrapie or experimental BSE in
sheep.
A meeting of the scientific experts who performed these analyses, held
on the 30th March, concluded that this case could not be considered to
be BSE in sheep, although it does not behave like known types of scrapie
either. Further investigation will be needed before more can be said
about how this unusual result should be described.
Defra's Chief Scientific Adviser, Professor Howard Dalton, said "The UK,
and especially the VLA, have played an important part in improving the
diagnostic methods available for identifying TSEs in sheep. As we
continue to assess more samples with these improved methods it is likely
that we will continue to find samples, such as this, which fall outside
our current knowledge of the disease. Defra, as it does with all
research, will continue to consult scientific experts to ensure that we
are investigating these cases using the best available techniques and
methods."
The National Scrapie Plan remains unaffected by this new result and SEAC
will be consulted in the near future.
Notes to editors
1. Scrapie is a fatal neurological sheep disease belonging to a group of
diseases called transmissible spongiform encephalopathies (TSEs),
including BSE in cattle and CJD in humans. It has been present in the
national flock for over 250 years. It is not considered to be
transmissible to humans.
2. There is a theoretical risk that BSE could be present in sheep,
masked by scrapie, but it has not been found naturally occurring in sheep.
3. There is as yet no definitive diagnostic method that can rapidly
distinguish between different TSEs for example scrapie from BSE.
Consequently, from time to time the scrapie surveillance programmes in
EU member states throw up unusual results that merit further
investigations (Defra press release 371/03 refers
http://www.defra.gov.uk/news/2003/030911a.htm)
4. The VLA have applied several different methods to the sample to
compare it to a wide range of previously detected scrapie cases,
experimental BSE in sheep and an experimental strain of scrapie, termed
CH1461. Two main methods have been used in this analysis:-
a. Western blot (WB)
This involves taking a sample of the brain and treating it with an
enzyme proteinase k to destroy the normal prion protein (PrPC). The
diseased form of the protein (PrPSc) is able to withstand this treatment
and is then separated from other cellular material on a gel. A blot is
taken of the gel and the PrPSc is visualised using specific antibodies.
b. Immunohistochemistry (IHC)
This involves taking thin slices of the brain, and by using special
(antibody) markers to detect the PrPSc it is possible to see disease
specific patterns of PrPSc distribution in the brain under a microscope.
The Western blot method found that the sample did not appear to resemble
previously recognised cases of scrapie and, although there were some
differences, some characteristics were similar to experimental BSE in
sheep and also the experimental strain of sheep scrapie, CH1461. IHC
found that it neither resembled previously recognised types of scrapie
or experimental BSE in sheep
5. The tissue sample has now been analysed using a total of 5 different
diagnostic methods claiming to be able to differentiate between scrapie
and experimental BSE in sheep. Two were performed at the VLA and three
were performed in other European laboratories.
6. The VLA is the European Reference Laboratory for TSEs and is
responsible for co-ordinating such investigations into unusual cases.
Their findings will be considered by the European Food Safety
Authority's committee of TSE experts and in the UK by the Spongiform
Encephalopathy Advisory Committee (SEAC).
7. The genotype of the suspect sheep was ARQ/ARQ which is known to be
susceptible to some strains of scrapie and, in experiments, to BSE.
Background information on scrapie, scrapie genotyping, and the National
Scrapie Plan is published on the Defra internet at www.defra.gov.uk/nsp.
8. For information and advice on BSE in sheep from the FSA please
consult their web site at www.foodstandards.gov.uk
Public enquiries 08459 335577;
Press notices are available on our website
www.defra.gov.uk
Defra's aim is sustainable development
End
Nobel House
17 Smith Square
London SW1P 3JR
Website www.defra.gov.uk
http://www.wired-gov.net/
TSS
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
TSE in Sheep Contingency Planning Assessment of Risk due to BSE
Infectivity from Disposal of Sheep A report for DEFRA November 2001
Management Summary It has been recognised for a considerable time that
sheep in the United Kingdom may have been infected with BSE. To date no
evidence has been found to demonstrate that the national flock is
actually infected with the disease. DEFRA have prepared a draft
contingency plan in the event that BSE were to be identified in UK
sheep. The worst case scenario under this plan is the disposal of the
entire UK flock, some 40 million sheep and lambs. This study has
estimated the potential exposure of the UK population to BSE infectivity
present in sheep in the event that this plan had to be put into effect.
http://www.defra.gov.uk/animalh/bse/bse-publications/seac/DNVReport.pdf
but who would have guessed that such an important experiment/study would
have gotton so screwed up, by not being able to tell a sheep brain from
a cow brain;
© DEFRA 2002 Item 3- Scrapie Brain pool experiments- Update on current
position and audits of samples 3.1 Members were updated on experiments
conducted at the Institute of Animal Health (IAH) to examine a pool of
scrapie brains collected in the early 1990s for evidence of BSE. SEAC
had previously recommended that the material should be examined by DNA
analys