Saturday, December 18, 2010

OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011

OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) 

Paris (France), 23-25 February 2011 OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future Paris (France), 23-25 February 2011 Alejandra Torres-Balmont Conference coordinator Administrative, Logistics and Publications Department OIE, 12 rue de Prony , 75017 Paris, France Phone number: +33 (0) 1 44 15 18 88 E-mail: a.balmont@oie.int Thank for your support to the OIE objectives for a safe world.



 Greetings, 

 > Thank for your support to the OIE objectives for a safe world. NOT ! 

 I see again that the OIE has done little to help eradicate all animal TSE from the globe, and in fact in my opinion, have help enhance the spread of BSE and other animal TSE globally by their industry friendly regulations. 

I tried to warn the OIE in 2002 about CWD and the potential, but very real threat of CWD to humans. 

I was told that they were seriously considering this. what happened ? 

NOW, the OIE and the USDA collaborate to make legal the trading of all strains of atypical BSE legal, and in fact have done so with the atypical scrapie, when science has made perfectly clear the risk factors to humans and other species. 

I have said it once (see below), and i will say again ; 

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

 NOW, some history on the failed OIE BSE/TSE policy, and why the OIE allowed BSE and other TSE to spread around the globe $$$ 

OIE Scientific and Technical Review, Vol 11 (2) June 1992 Sub Acute Spongiform Encephalopathies in Animals (CWD???) 

 -------- Original Message -------- 

 Subject: OIE Scientific and Technical Review, Vol 11 (2) June 1992 Sub Acute Spongiform Encephalopathies in Animals (CWD???) 

 Date: Thu, 29 Jan 2004 14:49:03 -0600 

 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy 

 To: BSE-L@uni-karlsruhe.de 

 ######## Bovine Spongiform Encephalopathy ######### 

 Richard Kimberlin has obviously declined and suggested to OIE that Ray Bradley takes on the role of both author of the introductory chapter and coordinator...

http://www.bseinquiry.gov.uk/files/yb/1991/03/04002001.pdf
  

OIE Scientifoc and Technical Review, Vol 11 (2) June 1992 Sub Acute Spongiform Encephalopathies in Animals 91\02.20\1.1-1.2 20/02/91 Minute 

S C Hutchins I Crawford snip... a) I think it would be advisable to include scrapie, BSE and transmissible mink encephalopathy (hence the proposed title)... 

 snip... 


 8. Document (D(iii)) does not follow the OIE Code under 3.2.13.8 IN particular the reference to freedom from any clinical evidence of infectious or contagious disease is a new condition that cannot be met. You will need to refer to the Code in a more general context and find more appropriate wording... 92/11.02/4.1 


and i proposed to OIE years ago to include CWD. but with these new atypical case of TSE showing up in cattle and sheep, it will be interesting to see how the OIE handles the USA demands on weakening the BSE/TSE regs for exporting countries; 

Date: Fri, 12 Jul 2002 16:11:42 -0700 

Reply-To: B S E-l 

Sender: Bovine Spongiform Encephalopathy 

From: TSS 

Subject: CWD/USA -- CWD/OIE? 

 snip... 

 Greetings List Members, 

 speaking with someone at the OIE about my concerns with CWD and the non-testing for TSEs in USA cattle, i find it very sad that the OIE does not follow CWD related issues. BUT, they voice my same concerns and said changes are in the makings. sadly, the changes will take about 2 years? 

 snip... 

 ''I agree with you Dr Terry. The OIE, namely the International Animal Health Code Commission is working on making proposals to Member Countries to change the OIE lists so to avoid some the problems mentioned in you e-mail. This will take at least two years before adoption by the International Committee.'' 

 snip... 

 two years is a very long time, on an issue of such importance to both humans and animals... 

 kind regards, terry 

 snip... 

 PAGE 25 Transmission Studies Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculam (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in _all_ of these species with the shortest incubation period in the ferret... 

 http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf 


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... 



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

snip... 

R. BRADLEY 

http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf 

 
same reason CJD/TSE is not reportable Nationally in the USA. same reason no CJD questionnaire exists in the USA that is issued to all victims and families of victims asking real questions pertaining to route and source of agent. no autopies for all demented of young AND OLD! same reason the USA is steadfast refusing to this day to rapid TSE test all cattle for human/animal consumption. the USA simply does not want to know$ hell, we should just retain it all, and just play like it has not happened for the next 40 years as well. hmm, something else to ponder ; 

5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man so, when/where is our first case transmission study of TSE on man going to be? i wish to witness this and have a few suggestions for our first human guinea-pigs ;-) 

 Terry S. Singeltary Sr.  Bacliff, TEXAS USA 77518 

 ########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############ ----- 

Original Message ----- 

 : "Terry S. Singeltary Sr." 

 To: Sent: Saturday, June 04, 2005 8:07 AM 

 Subject: BSE OIE CHAPTER 2.3.13 (The Weakening of a already terribly flawwed BSE/TSE surveillance system) 

 ##################### Bovine Spongiform Encephalopathy ##################### 

 C H A P T E R 2 . 3 . 1 3 . BOVINE SPONGIFORM ENCEPHALOPATHY Article 2.3.13.1. 

The recommendations in this Chapter are intended to manage the human and animal health risks associated with the presence of the bovine spongiform encephalopathy (BSE) agent in cattle (Bos taurus and B. indicus) only. 

1) When authorising import or transit of the following commodities and any products made from these commodities and containing no other tissues from cattle, Veterinary Administrations should not require any BSE related conditions, regardless of the BSE risk status of the cattle population of the exporting country, zone or compartment: 

 a) milk and milk products; 

 b) semen and in vivo derived cattle embryos collected and handled in accordance with the recommendations of the International Embryo Transfer Society; 

 c) hides and skins; 

 d) gelatin and collagen prepared exclusively from hides and skins; 

 e) protein-free tallow (maximum level of insoluble impurities of 0.15% in weight) and derivatives made from this tallow; 

 f) dicalcium phosphate (with no trace of protein or fat); 

 g) deboned skeletal muscle meat (excluding mechanically separated meat) from cattle 30 months of age or less, which were not subjected to a stunning process, prior to slaughter, with a device injecting compressed air or gas into the cranial cavity, or to a pithing process, and which were subject to ante- and post-mortem inspection and were not suspect or confirmed BSE cases; and which has been prepared in a manner to avoid contamination with tissues listed in Article 2.3.13.13.; 

 h) blood and blood by-products, from cattle which were not subjected to a stunning process, prior to slaughter, with a device injecting compressed air or gas into the cranial cavity, or to a pithing process. 

 2) When authorising import or transit of other commodities listed in this chapter, Veterinary Administrations should require the conditions prescribed in this Chapter relevant to the BSE risk status of the cattle population of the exporting country, zone or compartment. 

 Standards for diagnostic tests are described in the Terrestrial Manual. Article 2.3.13.2. 

 The BSE risk status of the cattle population of a country, zone or compartment should be determined on the basis of the following criteria: 

 1) the outcome of a risk assessment (which is reviewed annually), based on Section 1.3., identifying all potential factors for BSE occurrence and their historic perspective: 

2005 OIE Terrestrial Animal Health Code 2 

 a) Release assessment Release assessment consists of assessing the likelihood that a transmissible spongiform encephalopathy (TSE) agent has been introduced into the cattle population from a pre-existing TSE in the indigenous ruminant population or via commodities potentially contaminated with a TSE agent, through a consideration of the following:

 i) the presence or absence of animal TSE agents in the country or zone or compartment and, if present, their prevalence based on the outcomes of surveillance; 

 ii) meat-and-bone meal or greaves from the indigenous ruminant population; 

 iii) imported meat-and-bone meal or greaves; 

 iv) imported live animals; 

 v) imported animal feed and feed ingredients; 

 vi) imported products of ruminant origin for human consumption, which may have contained tissues listed in Article 2.3.13.13. and may have been fed to cattle; 

 vii) imported products of ruminant origin for in vivo use in cattle. Surveillance and other epidemiological investigations (especially surveillance for BSE conducted on the cattle population) relevant to the above should be taken into account in carrying out the assessment. 

 b) Exposure assessment If the release assessment identifies a risk factor, an exposure assessment should be conducted, consisting of assessing the likelihood of exposure of the BSE agent to cattle, through a consideration of the following: 

 i) recycling and amplification of the BSE agent through consumption by cattle of meat-and bone meal or greaves of ruminant origin, or other feed or feed ingredients contaminated with these; 

 ii) the use of ruminant carcasses (including from fallen stock), by-products and slaughterhouse waste, the parameters of the rendering processes and the methods of animal feed manufacture; 

 iii) the feeding or not of ruminants with meat-and-bone meal and greaves derived from ruminants, including measures to prevent cross-contamination of animal feed; 

 iv) the level of surveillance for BSE conducted on the cattle population to that time and the results of that surveillance. 

 2) on-going awareness programme for veterinarians, farmers, and workers involved in transportation, marketing and slaughter of cattle to encourage reporting of all cases showing clinical signs consistent with BSE in target sub-populations as defined in Appendix 3.8.4.; 

 2005 OIE Terrestrial Animal Health Code 3 

 3) the compulsory notification and investigation of all cattle showing clinical signs consistent with BSE; 

 4) the examination in an approved laboratory of brain or other tissues collected within the framework of the aforementioned surveillance and monitoring system. When the risk assessment (which takes into account the surveillance referred to in the release and exposure assessments above) demonstrates non-negligible risk, the country should conduct Type A surveillance in accordance with Appendix 3.8.4. 

 When the risk assessment (which takes into account the surveillance referred to in the release and exposure assessments above) demonstrates negligible risk, the country should conduct Type B surveillance in accordance with Appendix 3.8.4. Article 2.3.13.3. 

Negligible BSE risk Commodities from the cattle population of a country, zone or compartment pose a negligible risk of transmitting the BSE agent should the following conditions be met: 

 1) a risk assessment, as described in point 1) of Article 2.3.13.2., has been conducted in order to identify the historical and existing risk factors and the country has been demonstrated that appropriate generic measures have been taken for the relevant period of time defined below to manage all risk identified; 

 2) the country has demonstrated that Type B surveillance in accordance with Appendix 3.8.4. is in place, 

 3) EITHER: a) there has been no case of BSE, or any case of BSE has been demonstrated to have been imported and has been completely destroyed, and: 

 i) the criteria in points 2) to 4) of Article 2.3.13.2. have been complied with for at least 7 years; and 

 ii) it has been demonstrated, through an appropriate level of control and audit, that for at least 8 years meat-and-bone meal or greaves derived from ruminants has not been fed to ruminants; OR 

 b) the last indigenous case of BSE was reported more than 7 years ago; and 

 i) the criteria in points 2) to 4) of Article 2.3.13.2. have been complied with for at least 7 years; and 

 ii) it has been demonstrated, thorough an appropriate level of control and audit, that for at least 8 years meat-and-bone meal and greaves derived from ruminants has not been fed to ruminants; and 

 iii) all BSE cases, as well as: - all the progeny of female cases, born within 2 years prior to or after clinical onset of the disease, and 

 2005 OIE Terrestrial Animal Health Code 4 - 

all cattle which, during their first year of life, were reared with the BSE cases during their first year of life, and which investigation showed consumed the same potentially contaminated feed during that period, or - if the results of the investigation are inconclusive, all cattle born in the same herd as, and within 12 months of the birth of, the BSE cases, if alive in the country, zone or compartment, are permanently identified, and their movements controlled, and when slaughtered or at death, are completely destroyed. 

 Article 2.3.13.4. 

 Controlled BSE risk Commodities from the cattle population of a country, zone or compartment pose a controlled risk of transmitting the BSE agent, should the following conditions be met: 

 1) a risk assessment, as described in point 1) of Article 2.3.13.2., has been conducted in order to identify the historical and existing risk factors, and the country has not demonstrated that appropriate generic measures have been taken for the relevant period of time defined below to manage all risks identified; 

 2) the country has demonstrated that Type A surveillance in accordance with Appendix 3.8.4. is in place; 

 3) EITHER a) there has been no case of BSE or any case of BSE has been demonstrated to have been imported and has been completely destroyed, the criteria in points 2) to 4) of Article 2.3.13.2. are complied with, and it can be demonstrated, through an appropriate level of control and audit, that meat-and-bone meal and greaves derived from ruminants has not been fed to ruminants, but at least one of the following two conditions applies: 

 i) the criteria in points 2) to 4) of Article 2.3.13.2. have not been complied with for 7 years; 

 ii) it cannot be demonstrated that controls over the feeding of meat-and-bone meal or greaves derived from ruminants to ruminants have been in place for 8 years; 

 OR b) there has been an indigenous case of BSE reported, the criteria in points 2) to 4) of Article 2.3.13.2. are complied with, and it can be demonstrated, through an appropriate level of control and audit that meat-and-bone meal and greaves derived from ruminants have not been fed to ruminants, but at least one of the following two conditions applies: 

 i) the criteria in points 2) to 4) of Article 2.3.13.2. have not been complied with for 7 years; 

 ii) it cannot be demonstrated that controls over the feeding of meat-and-bone meal and greaves derived from ruminants to ruminants have been in place for 8 years; AND 

 iii) all BSE cases, as well as: - all the progeny of female cases, born within 2 years prior to or after clinical onset of the disease, and 

 2005 OIE Terrestrial Animal Health Code 5 - 

all cattle which, during their first year of life, were reared with the BSE cases during their first year of life, and which investigation showed consumed the same potentially contaminated feed during that period, or - if the results of the investigation are inconclusive, all cattle born in the same herd as, and within 12 months of the birth of, the BSE cases, if alive in the country, zone or compartment, are permanently identified, and their movements controlled, and when slaughtered or at death, are completely destroyed. Article 2.3.13.5. 

 Undetermined BSE risk The cattle population of a country, zone or compartment poses an undetermined BSE risk if it cannot be demonstrated that it meets the requirements of another category. 

 Article 2.3.13.6. 

 When importing from a country, zone or compartment posing a negligible BSE risk, Veterinary Administrations should require: for all commodities from cattle not listed in point 1) of Article 2.3.13.1. the presentation of an international veterinary certificate attesting that the country or zone/compartment complies with the conditions in Article 2.3.13.3. 

 Article 2.3.13.7. 

 When importing from a country, zone or compartment posing a controlled BSE risk, Veterinary Administrations should require: for cattle the presentation of an international veterinary certificate attesting that: 

 1) the country, zone or compartment complies with the conditions in Article 2.3.13.4.; 

 2) cattle selected for export are identified by a permanent identification system enabling them to be traced back to the dam and herd of origin, and are not exposed cattle as described in point 2) c) iii) of Article 2.3.13.4.; 

 3) in the case of a country, zone or compartment with an indigenous case, cattle selected for export were born after the date from which the ban on the feeding of ruminants with meat-and-bone meal and greaves derived from ruminants had been effectively enforced. 

 Article 2.3.13.8. 

 When importing from a country, zone or compartment with an undetermined BSE risk, Veterinary Administrations should require: for cattle the presentation of an international veterinary certificate attesting that: 

 1) the feeding of ruminants with meat-and-bone meal and greaves derived from ruminants has been banned and the ban has been effectively enforced; 

 2005 OIE Terrestrial Animal Health Code 6 

 2) all BSE cases, as well as: 

 a) all the progeny of female cases, born within 2 years prior to or after clinical onset of the disease, and 

 b) all cattle which, during their first year of life, were reared with the BSE cases during their first year of life, and, which investigation showed consumed the same potentially contaminated feed during that period, or 

 c) if the results of the investigation are inconclusive, all cattle born in the same herd as, and within 12 months of the birth of, the BSE cases, if alive in the country, zone or compartment, are permanently identified, and their movements controlled, and when slaughtered or at death, are completely destroyed; 

 3) cattle selected for export: a) are identified by a permanent identification system enabling them to be traced back to the dam and herd of origin and are not the progeny of BSE suspect or confirmed females; 

 b) were born at least 2 years after the date from which the ban on the feeding of ruminants with meat-and-bone meal and greaves derived from ruminants was effectively enforced. 

 Article 2.3.13.9. 

When importing from a country, zone or compartment posing a negligible BSE risk, Veterinary Administrations should require: 

 for fresh meat and meat products from cattle (other than that listed in point 1) of Article 2.3.13.1.) the presentation of an international veterinary certificate attesting that: 

 1) the country, zone or compartment complies with the conditions in Article 2.3.13.3.; 

 2) ante-mortem and post-mortem inspections were carried out on all cattle from which the fresh meat or meat products originate. 

 Article 2.3.13.10. 

 When importing from a country, zone or compartment posing a controlled BSE risk, Veterinary Administrations should require: for fresh meat and meat products from cattle (other than those listed in point 1) of Article 2.3.13.1.) the presentation of an international veterinary certificate attesting that: 

 1) the country, zone or compartment complies with the conditions in Article 2.3.13.4.; 

 2) ante-mortem and post-mortem inspections were carried out on all cattle from which the fresh meat and meat products originate; 

 3) cattle from which the fresh meat and meat products destined for export originate were not subjected to a stunning process, prior to slaughter, with a device injecting compressed air or gas into the cranial cavity, or to a pithing process; 

 4) the fresh meat and meat products do not contain: 

 a) the tissues listed in Article 2.3.13.13., 

 b) mechanically separated meat from the skull and vertebral column from cattle over 30 months of age, all of which have been completely removed in a manner to avoid contamination of the fresh meat and meat products. 

 2005 OIE Terrestrial Animal Health Code 7 

 Article 2.3.13.11. When importing from a country, zone or compartment with an undetermined BSE risk, Veterinary Administrations should require: 

 for fresh meat and meat products from cattle (other than those listed in point 1) of Article 2.3.13.1.) the presentation of an international veterinary certificate attesting that: 

 1) the cattle from which the fresh meat and meat products originate: 

 a) are not suspect or confirmed BSE cases; 

 b) have not been fed meat-and-bone meal or greaves; 

 c) were subjected to ante-mortem and post-mortem inspections; 

 d) were not subjected to a stunning process, prior to slaughter, with a device injecting compressed air or gas into the cranial cavity, or to a pithing process; 

 2) the fresh meat and meat products do not contain: 

 a) the tissues listed in Article 2.3.13.13., 

 b) nervous and lymphatic tissues exposed during the deboning process, 

 c) mechanically separated meat from the skull and vertebral column from cattle over 12 months of age, all of which have been completely removed in a manner to avoid contamination of the fresh meat and meat products. 

 Article 2.3.13.12. 

 Ruminant-derived meat-and-bone meal or greaves, or any commodities containing such products, which originate from a country, zone or compartment defined in Articles 2.3.13.4. and 2.3.13.5. should not be traded between countries. 

 Article 2.3.13.13. 

 1) From cattle of any age originating from a country, zone or compartment defined in Articles 2.3.13.4. and 2.3.13.5., the following commodities, and any commodity contaminated by them, should not be traded for the preparation of food, feed, fertilisers, cosmetics, pharmaceuticals including biologicals, or medical devices: tonsils and distal ileum, and protein products derived thereof. Food, feed, fertilisers, cosmetics, pharmaceuticals or medical devices prepared using these commodities should also not be traded. 

 2) From cattle that were at the time of slaughter over 30 months of age originating from a country, zone or compartment defined in Article 2.3.13.4., the following commodities, and any commodity contaminated by them, should not be traded for the preparation of food, feed, fertilisers, cosmetics, pharmaceuticals including biologicals, or medical devices: brains, eyes, spinal cord, skull, vertebral column and derived protein products. Food, feed, fertilisers, cosmetics, pharmaceuticals or medical devices prepared using these commodities should also not be traded. 

 3) From cattle that were at the time of slaughter over 12 months of age originating from a country, zone or compartment defined in Article 2.3.13.5., the following commodities, and any commodity contaminated by them, should not be traded for the preparation of food, feed, fertilisers, cosmetics, pharmaceuticals including biologicals, or medical devices: brains, eyes, spinal cord, skull, vertebral column and derived protein products. Food, feed, fertilisers, cosmetics, pharmaceuticals or medical devices prepared using these commodities should also not be traded. 

 2005 OIE Terrestrial Animal Health Code 8 

 Article 2.3.13.14. 

 Veterinary Administrations of importing countries should require: for gelatin and collagen prepared from bones and intended for food or feed, cosmetics, pharmaceuticals including biologicals, or medical devices the presentation of an international veterinary certificate attesting that the commodities came from: 

 1) a country, zone or compartment posing a negligible BSE risk; or 

 2) a country, zone or compartment posing a controlled BSE risk; and 

 a) skulls and vertebrae (except tail vertebrae) have been excluded; 

 b) the bones have been subjected to a process which includes all the following steps: 

 i) pressure washing (degreasing), 

 ii) acid demineralisation, 

 iii) prolonged alkaline treatment, 

 iv) filtration, 

 v) sterilisation at =138C for a minimum of 4 seconds, or to an equivalent process in terms of infectivity reduction. 

 Article 2.3.13.15. 

 Veterinary Administrations of importing countries should require: for tallow and dicalcium phosphate (other than protein-free tallow as defined in Article 2.3.13.1.) intended for food, feed, fertilisers, cosmetics, pharmaceuticals including biologicals, or medical devices the presentation of an international veterinary certificate attesting that it originates from: 

 1) a country, zone or compartment posing a negligible BSE risk; or 

 2) a country, zone or compartment posing a controlled BSE risk, and it originates from cattle which have been subjected to ante-mortem and post-mortem inspection and has not been prepared using the tissues listed in point 2 of  Article 2.3.13.13. 

 Article 2.3.13.16. 

 Veterinary Administrations of importing countries should require: for tallow derivatives (other than those made from protein-free tallow as defined in Article 2.3.13.1.) intended for food, feed, fertilisers, cosmetics, pharmaceuticals including biologicals, or medical devices the presentation of an international veterinary certificate attesting that: 

 1) they originate from a country, zone or compartment posing a negligible BSE risk measures; or 

 2) they have been produced by hydrolysis, saponification or transesterification using high temperature and pressure. 

 2005 OIE Terrestrial Animal Health Code 9 APPENDI X 3 . 8 . 4 . 

 SURVEILLANCE FOR BOVINE SPONGIFORM ENCEPHALOPATHY 

 Article 3.8.4.1. 

 Introduction 

 1) Depending on the bovine spongiform encephalopathy (BSE) risk category of a country, zone or compartment, surveillance for BSE may have one or more goals: 

 a) detecting BSE, to a pre-determined design prevalence, in a country, zone or compartment; 

 b) monitoring the evolution of BSE in a country, zone or compartment; 

 c) monitoring the effectiveness of a feed ban and/or other risk mitigation measures, in conjunction with auditing, etc; 

 d) supporting a claimed BSE status; 

 e) gaining or regaining a higher BSE status. 

 2) When the BSE agent is present in a country or zone, the cattle population will comprise the following sectors, in order of decreasing size: 

 a) cattle not exposed to the infective agent; 

 b) cattle exposed but not infected; 

 c) infected cattle, which may lie within one of three stages in the progress of BSE: 

 i) the majority will die or be killed before reaching a stage at which BSE is detectable by current methods; 

 ii) some will progress to a stage at which BSE is detectable by testing before clinical signs appear; 

 iii) the smallest number will show clinical signs. 

 3) The BSE status of a country, zone or compartment cannot be determined only on the basis of a surveillance programme but should be determined in accordance with all the factors listed in Article 2.3.13.2. 

The surveillance programme should take into account the diagnostic limitations associated with the above sectors and the relative distributions of infected cattle among them. 

 4) With respect to the distribution and expression of the BSE agent within the sectors described above, the following four subpopulations of cattle have been identified for surveillance purposes: 

 a) cattle over 30 months of age displaying behavioural or clinical signs consistent with BSE; 

 b) cattle over 30 months of age that are non-ambulatory, recumbent, unable to rise or to walk without assistance; cattle over 30 months of age sent for emergency slaughter or condemned at ante-mortem inspection (casualty, emergency slaughter or downer cattle); 

 2005 OIE Terrestrial Animal Health Code 10 

 c) cattle over 30 months of age which are found dead on farm, during transport or at an abattoir (fallen stock). 

 d) cattle over 36 months of age at routine slaughter. 

 5) A gradient is used to describe the relative value of surveillance applied to each subpopulation. 

Surveillance should focus on the first subpopulation, but investigation of other subpopulations will help to provide an accurate assessment of the BSE situation in the country, zone or compartment. All countries should sample at least three of the four subpopulations. This approach is consistent with Appendix 3.8.1. on surveillance and monitoring of animal health. Article 3.8.4.2. 

 Description of cattle subpopulations 

 1) Cattle over 30 months of age displaying behavioural or clinical signs consistent with BSE Cattle affected by illnesses that are refractory to treatment, and displaying progressive behavioural changes such as excitability, persistent kicking when milked, changes in herd hierarchical status, hesitation at doors, gates and barriers, as well as those displaying progressive neurological signs without signs of infectious illness are candidates for examination. These behavioural changes, being very subtle, are best identified by those who handle animals on a daily basis. Since BSE causes no pathognomonic clinical signs, all countries with cattle populations will observe individual animals displaying clinical signs consistent with BSE. It should be recognised that cases may display only some of these signs, which may also vary in severity, and such animals should still be investigated as potential BSE affected animals. The rate at which such suspicious cases are likely to occur will differ among epidemiological situations and cannot therefore be predicted reliably. This subpopulation, particularly cattle over 30 months of age, is the one exhibiting the highest prevalence. The recognition greatly depends on the owner's awareness and observation of suspect animals. The reporting of these suspect animals when at the farm will depend on the owner's motivation based on cost and socio-economic repercussions. 

 2) Cattle over 30 months of age that are non-ambulatory, recumbent, unable to rise or to walk without assistance; cattle over 30 months of age sent for emergency slaughter or condemned at ante-mortem inspection (casualty or emergency slaughter, or downer cattle) These cattle may have exhibited some of the clinical signs listed above which were not recognised as being consistent with BSE. Experience in countries where BSE has been identified indicates that this subpopulation is the one demonstrating the second highest prevalence. For that reason, it is the second most appropriate population to target in order to detect BSE. 

3) Cattle over 30 months of age which are found dead on farm, during transport or at an abattoir (fallen stock) These cattle may have exhibited some of the clinical signs listed above prior to death, but were not recognised as being consistent with BSE. Experience in countries where BSE has been identified indicates that this subpopulation is the one demonstrating the third highest prevalence. 

 4) Cattle over 36 months of age at routine slaughter Experience in countries where BSE has been identified indicates that this subpopulation is the one demonstrating the lowest prevalence. For that reason, it is the least appropriate population to target in order to detect BSE. However, sampling in this subpopulation may be an aide in monitoring the progress of the epizootic and the efficacy of control measures applied, because it offers continuous access to a cattle population of known class, age structure and geographical origin. Testing of routine slaughter cattle younger than 36 months is of relatively very little value (Table 2). 

 2005 OIE Terrestrial Animal Health Code 11 

 Within each of the above subpopulations, countries may wish to target cattle identifiable as imported from countries or zones not free from BSE, cattle which have consumed potentially contaminated feedstuffs from countries or zones not free from BSE, offspring of BSE affected cows and cattle which have consumed feedstuffs potentially contaminated with other TSE agents. When establishing a surveillance strategy, authorities must take into account inherent difficulties of obtaining samples on farm. These difficulties include higher cost, necessity for education and motivation of owners, counteracting potentially negative socio-economic implication. Authorities must find ways to overcome these difficulties. 

 Article 3.8.4.3. 1) 

Implementation of type A surveillance In order to implement efficiently a surveillance strategy for BSE, a country must use good quality data (or reliable estimates) concerning the age distribution of its adult cattle population and the number of cattle tested for BSE stratified by age and by subpopulation. The application of the following procedure will allow the detection of BSE prevalence of at least one case per 100,000 in the adult cattle population, at a confidence level of 95% in the country, zone or compartment of concern. This Appendix utilises Tables 1 and 2 to determine a desired surveillance point target and the point values of surveillance samples collected. The approach assigns 'point values' to each sample, based on the subpopulation from which it was collected and the likelihood of detecting infected cattle in that subpopulation. The number of points a sample is assigned is determined by the subpopulation from which the sample is collected and the age of the animal sampled. The total points accumulation is then periodically compared to the target number of points for a country, zone or compartment. A country should design its surveillance strategy to ensure that samples are representative of the herd of the country, zone or compartment, and include consideration of demographic factors such as production type and geographic location, and the potential influence of culturally unique husbandry practices. The approach used and the assumptions made should be fully documented, and the documentation retained for 7 years. The points targets and surveillance point values in the appendix were obtained by applying the following factors to a statistical model: 

 a) a prevalence of either at least one case per million or one case per 100,000 of the adult cattle population; 

 b) a confidence level of 95%; 

 c) the pathogenesis, and pathological and clinical expression of BSE: 

 i) sensitivity of diagnostic methods used; 

 ii) relative frequency of expression by age; 

 iii) relative frequency of expression within each subpopulation; 

 iv) interval between clinical pathological change and clinical expression; 

 d) demographics of the cattle population, including age distribution; 

 e) influence of BSE on culling or attrition of animals from the cattle population via the four subpopulations; 

 f) percentage of infected animals in the cattle population which are not detected. 

 2005 OIE Terrestrial Animal Health Code 12 

 Although the procedure accepts very basic information about a cattle population, and can be used with estimates and less precise data, careful collection and documentation of the data significantly enhance their value. Since samples from clinical suspect animals provide many times more information than samples from healthy or dead-of-unknown-cause animals, careful attention to the input data can substantially decrease the procedure's cost and the number of samples needed. The essential input data are: 

 g) cattle population numbers stratified by age; 

 h) the number of cattle tested for BSE stratified by age and by subpopulation. 

 2) Maintenance (type B) surveillance For countries which have demonstrated through risk assessment (including surveillance) that they meet the requirements for 'negligible risk without commodity-specific risk mitigation measures', surveillance should continue at a reduced, maintenance level. In order to implement efficiently a maintenance surveillance strategy for BSE, a country must use good quality data (or reliable estimates) concerning the age distribution of its adult cattle population and the number of cattle tested for BSE stratified by age and by subpopulation. The application of the following procedure will allow the detection of BSE prevalence of at least one case per 50,000 in the adult cattle population, at a confidence level of 95% in the country, zone or compartment of concern. This Appendix utilises Tables 1 and 2 to determine a desired surveillance point target and the point values of surveillance samples collected. Maintenance surveillance should focus on the higher prevalence subpopulations (especially clinical suspects). The number of clinical suspect samples taken annually should approximate the number of samples taken annually from clinical suspect cases during the time taken to reach the country, zone or compartment's BSE status (to a maximum of seven years). Article 3.8.4.4. 1) Selecting the points target The desired surveillance points target is selected from Table 1, which shows target points for adult cattle populations of different sizes. A country's adult cattle population size may be estimated or may be set at one million because, for statistical reasons, one million is the point beyond which sample size does not further increase with population size. The target depends on the design prevalence chosen by the country. 

 2005 OIE Terrestrial Animal Health Code 13 

 Table 1 Points targets for different adult cattle population sizes in a country, zone or compartment which has not identified any BSE cases Target points for country, zone or compartment with 0 cases, 95% confidence Adult Cattle Population Size (24 months and older) *DP 1/100,000 *DP 1/50,000 = 1,000,000 300,000 150,000 800,000 - 1,000,000 240,000 120,000 600,000 - 800,000 180,000 90,000 400,000 - 600,000 120,000 60,000 200,000 - 400,000 60,000 30,000 100,000 - 200,000 30,000 15,000 50,000 - 100,000 15,000 7,500 *DP is the maximum possible prevalence or "design prevalence". 

 2) Determining the point values of samples collected Table 2 can be used to determine the point values of the surveillance samples collected. The approach assigns point values to each sample according to the likelihood of detecting infection based on the subpopulation from which the sample was collected and the age of the animal sampled. This approach takes into account the general principles of surveillance described in Appendix 3.8.1. and the epidemiology of BSE. Because precise aging of the animals that are sampled may not be possible, Table 2 combines point values into five age categories. The point estimates for each category were determined as an average for the age range comprising the group. The age groups were selected on their relative likelihoods of expressing BSE according to scientific knowledge of the incubation of the disease and the world BSE experience. Samples may be collected from any combination of subpopulations and ages but should reflect the demographics of the cattle herd of the country, zone or compartment. In addition, countries should sample at least three of the four subpopulations. The total points for samples collected may be accumulated over a period of a maximum of 7 consecutive years to achieve the target number of points determined in Table 1. 

 2005 OIE Terrestrial Animal Health Code 14 

 Table 2 Surveillance point values for samples collected from animals in the given subpopulation and age category Surveillance subpopulation Routine slaughter * Fallen stock ** Casualty slaughter *** Clinical suspect **** age = 1 year and 0.01 0.2 0.4 N/A Age = 2 years and (young adult) 0.1 0.2 0.4 260 Age = 4 years and (middle adult) 0.2 0.9 1.6 750 Age = 7 years and (older adult) 0.1 0.4 0.7 220 Age = 9 years (aged) 0.0 0.1 0.2 45 * Article 3.8.4.2 4 ** Article 3.8.4.2 3 *** Article 3.8.4.2 2 **** Article 3.8.4.2 1 

 Surveillance points remain valid for 7 years (the 95th percentile of the incubation period). Article 3.8.4.5. 

 To monitor the evolution of BSE in a country, zone or compartment once it is detected To monitor the evolution of BSE in a country, zone or compartment once it is detected, a more intensive sampling method needs to be used to determine disease prevalence. For countries that have determined that BSE exists within their cattle population, the goal of surveillance shifts from one of detection to one of monitoring the extent and evolution of the disease, and monitoring the effectiveness of control measures such as feed bans and SRM removal policies. 

 2005 OIE Terrestrial Animal Health Code 15 

 APPENDI X 3 . 6 . 3 . P R O C E D U R E S F O R T H E R E D UC TI O N O F I N F E C T I V I T Y O F T R A N S M I S S I B L E S P O N G I F O R M E N C E P H A L O P A T H Y A G E N T S 

 Article 3.6.3.1. 

 Meat-and-bone meal The following procedure should be used to reduce the infectivity of any transmissible spongiform encephalopathy agents which may be present during the production of meat-and-bone meal containing ruminant proteins: 

 1. The raw material should be reduced to a maximum particle size of 50 mm before heating. 

 2. The raw material should be heated under saturated steam conditions to a temperature of not less than 133C for a minimum of 20 minutes at an absolute pressure of 3 bar. 

 2005 OIE Terrestrial Animal Health Code www.oie.int/downld/SC/2005/bse_2005.pdf EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA) Publication date: 20 August 2004 Adopted July 2004 (Question N EFSA-Q-2003-083) 

 Report Summary Summary of the Scientific Report The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. 

This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003. 

 The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. 

These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. 

It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties. 

 A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. 

This risk continued to exist, and grew significantly in the mid 90's when domestic cattle, infected by imported MBM, reached processing. 

Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries. 

 EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. 

Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.


CANADA

MEXICO

 What GAO Found United States Government Accountability Office 

 Why GAO Did This Study 

 Highlights Accountability Integrity Reliability 


 To view the full product, including the scope and methodology, click on the link above. 

For more information, contact Robert A. Robinson at (202) 512-3841 or robinsonr@gao.gov. 

 Highlights of GAO-05-101, a report to congressional requesters February 2005 MAD COW DISEASE 

 FDA's Management of the Feed Ban Has Improved, but Oversight Weaknesses Continue to Limit Program Effectiveness FDA has made needed improvements to its management and oversight of the feed-ban rule in response to GAO's 2002 report, but program weaknesses continue to limit the effectiveness of the ban and place U.S. cattle at risk of spreading BSE. Improvements made include FDA establishing a uniform method of conducting compliance inspections and training FDA inspectors, as well as state inspectors who carry out inspections under agreements with FDA, on the new method.

 FDA also implemented new data-entry procedures that are designed to more reliably track feed-ban inspection results. Consequently, FDA has a better management tool for overseeing compliance with the feed-ban rule and a data system that better conforms to standard database management practices.

 However, various program weaknesses continue to undermine the nation's firewall against BSE. For example: 

FDA acknowledges that there are more feed manufacturers and transporters, on-farm mixers, and other feed industry businesses that are subject to the feed ban than the approximately 14,800 firms inspected to date; 

however, it has no uniform approach for identifying additional firms. . 

FDA has not reinspected approximately 2,800, or about 19 percent, of those businesses, in 5 or more years; several hundred are potentially high risk. 

FDA does not know whether those businesses now use prohibited material in their feed. . 

FDA's feed-ban inspection guidance does not include instructions to routinely sample cattle feed to test for potentially prohibited material as part of the compliance inspection. 

Instead, it includes guidance for inspectors to visually examine facilities and equipment and review invoices and other documents. . 

Feed intended for export is not required to carry a caution label 

"Do not feed to cattle or other ruminants," 

when the label would be required if the feed were sold domestically. 

Without that statement, feed containing prohibited material could be inadvertently or intentionally diverted back to U.S. cattle or given to foreign cattle. . 

FDA has not always alerted USDA and states when it learned that cattle may have been given feed that contained prohibited material. 

This lapse has been occurring even though FDA's guidance calls for such communication. . 

Although research suggests that cattle can get BSE from ingesting even a small amount of infected material, inspectors do not routinely inspect or review cleanout procedures for vehicles used to haul cattle feed. 

 More than 5 million cattle across Europe have been killed to stop the spread of bovine spongiform encephalopathy (BSE), commonly called mad cow disease. 

Found in 26 countries, including Canada and the United States, BSE is believed to spread through animal feed that contains protein from BSE-infected animals. 

Consuming meat from infected cattle has also been linked to the deaths of about 150 people worldwide. 

In 1997, the Food and Drug Administration (FDA) issued a feed-ban rule prohibiting certain animal protein (prohibited material) in feed for cattle and other ruminant animals. 

FDA and 38 states inspect firms in the feed industry to enforce this critical firewall against BSE. 

In 2002, GAO reported a number of weaknesses in FDA's enforcement of the feed ban and recommended corrective actions. 

This report looks at FDA's efforts since 2002 to ensure industry compliance with the feed ban and protect U.S. cattle. 

 What GAO Recommends GAO recommends FDA, among other things, develop procedures for finding additional firms subject to the feed-ban and using tests to augment inspections. 

FDA said the study was thorough but disagreed on four of nine recommendations. 

GAO continues to believe that, given the discovery of BSE in North America and the oversight gaps described in the report, the recommended actions are needed to protect U.S. cattle from BSE. 

 3. Mad Cow Disease: FDA's Management of the Feed Ban Has Improved, but Oversight Weaknesses Continue to Limit Program Effectiveness. GAO-05-101, Feb. 25. 



 Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]


 Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2


 PART 2 


 From: Terry S. Singeltary Sr. [flounder@wt.net] 

 Sent: Tuesday, July 29, 2003 1:03 PM 

 To: fdadockets@oc.fda.gov 

 Cc: ggraber@cvm.fda.gov; Linda.Grassie@fda.gov; BSE-L 

 Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION TO DOCKET 2003N-0312] 

 Greetings FDA, 

 snip... 

 PLUS, if the USA continues to flagrantly ignore the _documented_ science to date about the known TSEs in the USA (let alone the undocumented TSEs in cattle), it is my opinion, every other Country that is dealing with BSE/TSE should boycott the USA and demand that the SSC reclassify the USA BSE GBR II risk assessment to BSE/TSE GBR III 'IMMEDIATELY'. 

for the SSC to _flounder_ any longer on this issue, should also be regarded with great suspicion as well. 

NOT to leave out the OIE and it's terribly flawed system of disease surveillance. the OIE should make a move on CWD in the USA, and make a risk assessment on this as a threat to human health. the OIE should also change the mathematical formula for testing of disease. 

this (in my opinion and others) is terribly flawed as well. 

to think that a sample survey of 400 or so cattle in a population of 100 million, to think this will find anything, especially after seeing how many TSE tests it took Italy and other Countries to find 1 case of BSE (1 million rapid TSE test in less than 2 years, to find 102 BSE cases), should be proof enough to make drastic changes of this system. 

the OIE criteria for BSE Country classification and it's interpretation is very problematic. a text that is suppose to give guidelines, but is not understandable, cannot be considered satisfactory. the OIE told me 2 years ago that they were concerned with CWD, but said any changes might take years. 

well, two years have come and gone, and no change in relations with CWD as a human health risk. 

if we wait for politics and science to finally make this connection, we very well may die before any decisions or changes are made. this is not acceptable. 

we must take the politics and the industry out of any final decisions of the Scientific community. 

this has been the problem from day one with this environmental man made death sentence. 

some of you may think i am exaggerating, but you only have to see it once, you only have to watch a loved one die from this one time, and you will never forget, OR forgive...yes, i am still very angry... but the transmission studies DO NOT lie, only the politicians and the industry do... and they are still lying to this day...TSS 


 Gerald Wells: Report of the Visit to USA, April-May 1989 

 snip... 

 The general opinion of those present was that BSE, as an overt disease phenomenon, _could exist in the USA, but if it did, it was very rare. The need for improved and specific surveillance methods to detect it as recognised... 

 snip... 

 It is clear that USDA have little information and _no_ regulatory responsibility for rendering plants in the US... 

 snip... 

 3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a _very low profile indeed_. 

Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be _avoided_ in the US _at all costs_... 

 snip... 

 http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
  

 To be published in the Proceedings of the Fourth International Scientific Congress in Fur Animal Production. Toronto, Canada, August 21-28, 1988 

 Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle 

 R.F. Marsh* and G.R. Hartsough .Department of Veterinary Science, University of Wisconsin-Madison, Madison, Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092 

 ABSTRACT 

Epidemiologic investigation of a new incidence of transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin suggests that the disease may have resulted from feeding infected cattle to mink. This observation is supported by the transmission of a TME-like disease to experimentally inoculated cattle, and by the recent report of a new bovine spongiform encephalopathy in England. 

 INTRODUCTION 

 Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough and Burger who demonstrated that the disease was transmissible with a long incubation period, and that affected mink had a spongiform encephalopathy similar to that found in scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965). Because of the similarity between TME and scrapie, and the subsequent finding that the two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was concluded that TME most likely resulted from feeding mink scrapie-infecied sheep. The experimental transmission of sheep scrapie to mink (Hanson et al., 1971) confirmed the close association of TME and scrapie, but at the same time provided evidence that they may be different. Epidemiologic studies on previous incidences of TME indicated that the incubation periods in field cases were between six months and one year in length (Harxsough and Burger, 1965).

 Experimentally, scrapie could not be transmitted to mink in less than one year. To investigate the possibility that TME may be caused by a (particular strain of scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie agent, including their sheep or goat sources, were inoculated into a total of 61 mink. Only one mink developed a progressive neurologic disease after an incubation period of 22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent from an unidentified source. 

 OBSERVATIONS AND RESULTS 

 A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin reported that many of his mink were "acting funny", and some had died. At this time, we visited the farm and found that approximately 10% of all adult mink were showing typical signs of TME: 

insidious onset characterized by subtle behavioral changes, loss of normal habits of cleanliness, deposition of droppings throughout the pen rather than in a single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over their _backs like squirrels. 

These signs were followed by progressive deterioration of neurologic function beginning with locomoior incoordination, long periods of somnolence in which the affected mink would stand motionless with its head in the corner of the cage, complete debilitation, and death. 

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. 

Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. 

The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. 

Sheep had never been fed. 

 Experimental Transmission. The clinical diagnosis of TME was confirmed by histopaihologic examination and by experimental transmission to mink after incubation periods of four months. 

To investigate the possible involvement of cattle in this disease cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally with a brain suspension from affected mink. 

Each developed a fatal spongiform encephalopathy after incubation periods of 18 and 19 months. 

 DISCUSSION 

These findings suggest that TME may result from feeding mink infected cattle and we have alerted bovine practitioners that there may exist an as yet unrecognized scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). 

A new bovine spongiform encephalopathy has recently been reported in England (Wells et al., 1987), and investigators are presently studying its transmissibility and possible relationship to scrapie. 

Because this new bovine disease in England is characterized by behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be confused with rabies in the United Stales and not be diagnosed. 

Presently, brains from cattle in the United States which are suspected of rabies infection are only tested with anti-rabies virus antibody and are not examined histopathologically for lesions of spongiform encephalopathy. 

We are presently pursuing additional studies to further examine the possible involvement of cattle in the epidemiology of TME. One of these is the backpassage of our experimental bovine encephalopathy to mink. 

Because (here are as yet no agent- specific proteins or nucleic acids identified for these transmissible neuropathogens, one means of distinguishing them is by animal passage and selection of the biotype which grows best in a particular host. 

This procedure has been used to separate hamster- adapted and mink-udapted TME agents (Marsh and Hanson, 1979). 

The intracerebral backpassage of the experimental bovine agent resulted in incubations of only four months indicating no de-adaptation of the Stetsonville agent for mink after bovine passage. 

Mink fed infected bovine brain remain normal after six months. 

It will be essential to demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic association is to be confirmed. 

 ACKNOWLEDGEMENTS 

These studies were supported by the College of Agricultural and Life Sciences, University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United States Department of Agriculture. The authors also wish to acknowledge the help and encouragement of Robert Hanson who died during the course of these investigations. REFERENCES Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and natural transmission. J. Infec. Dis. 115:393-399. Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson, D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861. Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and clinical observations. 3. Infec. Dis. 115:387-392. Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the transmissible mink encephalopathy agent. 3. ViroL 3:176-180. Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460. Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle? Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary Medicine. University of Arizona, pp 20. Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M., Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 121:419-420.  MARSH 

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf 


Greetings, 

 SO, the already terribly flawwed OIE BSE surveillance system is too burdensome for trade. 

Aint that just too bad.

SO, they decide to make it even weaker. 

The damn thing never worked anyway. 

ALL one has to do is look at the documented BSE Countries that went by it. 

Did them a lot of good. 

 TO think that a sample survey of 400 or so cattle in a population of 100 million, to think this will find anything, especially after seeing how many TSE tests it took Italy and other Countries to find 1 case of BSE (1 million rapid TSE test in less than 2 years, to find 102 BSE cases), should be proof enough to make drastic changes of this system. 

the OIE criteria for BSE Country classification and it's interpretation is very problematic. 

a text that is suppose to give guidelines, but is not understandable, cannot be considered satisfactory... 


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

 Terry S. Singeltary Sr. Bacliff, TEXAS USA a.. BSE OIE 

 #################### /lists.aegee.org/bse-l.html #################### 

 === 2010 === 

 2010 PRION DISEASE UPDATE DECEMBER NORTH AMERICA UPDATE 

 There are now two documented strains of CWD, and science is showing that indeed CWD could transmit to humans via transmission studies ; 

 PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent 

 Qingzhong Kong, Shenghai Huang,*Fusong Chen, Michael Payne, Pierluigi Gambetti and Liuting Qing Department of Pathology; Case western Reserve University; Cleveland, OH USA 

*Current address: Nursing Informatics; Memorial Sloan-Kettering Cancer Center; New York, NY USA 

 Key words: CWD, strain, human transmission 

 Chronic wasting disease (CWD) is a widespread prion disease in cervids (deer and elk) in North America where significant human exposure to CWD is likely and zoonotic transmission of CWD is a concern. 

Current evidence indicates a strong barrier for transmission of the classical CWD strain to humans with the PrP-129MM genotype. 

A few recent reports suggest the presence of two or more CWD strains. 

What remain unknown is whether individuals with the PrP-129VV/MV genotypes are also resistant to the classical CWD strain and whether humans are resistant to all natural or adapted cervid prion strains. 

Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP, indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains. 

Preliminary results on CWD transmission in transgenic mice expressing human PrP-129V will also be discussed. 

 Acknowledgement Supported by NINDS NS052319 and NIA AG14359. 

 PPo2-27: Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions 

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

 Prion diseases are infectious neurodegenerative disorders affecting humans and animals that result from the conversion of normal prion protein (PrPC) into the misfolded and infectious prion (PrPSc).

 Chronic wasting disease (CWD) of cervids is a prion disorder of increasing prevalence within the United States that affects a large population of wild and captive deer and elk. CWD is highly contagious and its origin, mechanism of transmission and exact prevalence are currently unclear. 

The risk of transmission of CWD to humans is unknown. Defining that risk is of utmost importance, considering that people have been infected by animal prions, resulting in new fatal diseases. 

To study the possibility that human PrPC can be converted into the infectious form by CWD PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification (PMCA) technique, which mimic in vitro the process of prion replication. 

Our results show that cervid PrPSc can induce the pathological conversion of human PrPC, but only after the CWD prion strain has been stabilized by successive passages in vitro or in vivo. 

Interestingly, this newly generated human PrPSc exhibits a distinct biochemical pattern that differs from any of the currently known forms of human PrPSc, indicating that it corresponds to a novel human prion strain. 

Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids. 

 PPo2-7: Biochemical and Biophysical Characterization of Different CWD Isolates 

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

 Key words: CWD, strains, FT-IR, AFM 

 Chronic wasting disease (CWD) is one of three naturally occurring forms of prion disease. 

The other two are Creutzfeldt-Jakob disease in humans and scrapie in sheep. CWD is contagious and affects captive as well as free ranging cervids. 

As long as there is no definite answer of whether CWD can breach the species barrier to humans precautionary measures especially for the protection of consumers need to be considered. 

In principle, different strains of CWD may be associated with different risks of transmission to humans. 

Sophisticated strain differentiation as accomplished for other prion diseases has not yet been established for CWD. 

However, several different findings indicate that there exists more than one strain of CWD agent in cervids. 

We have analysed a set of CWD isolates from white-tailed deer and could detect at least two biochemically different forms of disease-associated prion protein PrPTSE. 

Limited proteolysis with different concentrations of proteinase K and/or after exposure of PrPTSE to different pH-values or concentrations of Guanidinium hydrochloride resulted in distinct isolate-specific digestion patterns. 

Our CWD isolates were also examined in protein misfolding cyclic amplification studies. 

This showed different conversion activities for those isolates that had displayed significantly different sensitivities to limited proteolysis by PK in the biochemical experiments described above. 

We further applied Fourier transform infrared spectroscopy in combination with atomic force microscopy. 

This confirmed structural differences in the PrPTSE of at least two disinct CWD isolates. 

The data presented here substantiate and expand previous reports on the existence of different CWD strains. 


 UPDATED DATA ON 2ND CWD STRAIN 

 Wednesday, September 08, 2010 CWD PRION CONGRESS SEPTEMBER 8-11 2010 


 P35 ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A WISCONSIN STRAIN OF CWD 

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

 The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. 

Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. 

We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. 

The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. 

Small rodent models are a useful means of identifying prion strains. 

We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. 

These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. 

Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. 

Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region. 


 PPo3-40: Mother to Offspring Transmission of Chronic Wasting Disease 

 Candace K. Mathiason, Amy V. Nalls, Kelly Anderson, Jeanette Hayes-Klug, Nicholas Haley and Edward A. Hoover Colorado State University, Department of Microbiology, Immunology and Pathology, Fort Collins, CO USA 

 Key words: Chronic wasting disease, vertical transmission, muntjac deer 

 We have developed a new cervid model in small Asian muntjac deer (Muntiacus reevesi) to study potential modes of vertical transmission of chronic wasting disease (CWD) from mother to offspring. 

Eight of eight (8/8) muntjac doe orally infected with CWD tested PrPCWD lymphoid positive by 4 months post infection. 

Six fawns were born to these CWD-infected doe. 

Six fawns were born to 6 CWD-infected doe; 4 of the fawns were non-viable. 

The viable fawns have been monitored for CWD infection by immunohistochemistry and sPMCA performed on serial tonsil and rectal lymphoid tissue biopsies. 

PrPCWD has been detected in one fawn as early as 40 days of age. 

Moreover, sPMCA performed on rectal lymphoid tissue has yield positive results on another fawn at 10 days of age. 

In addition, sPMCA assays have also demonstrated amplifiable prions in maternal placental (caruncule) and mammary tissue of the dam. 

 Additional pregnancy related fluids and tissues from the doe as well as tissue from the nonviable fawns are currently being probed for the presence of CWD. 

In summary, we have employed the muntjac deer model, to demonstrate for the first time the transmission of CWD from mother to offspring. 

These studies provide the foundation to investigate the mechanisms and pathways of maternal prion transfer. 

 PRION 2010 Meeting Report International Prion Congress: From agent to disease; September 8–11, 2010; Salzburg, Austria Volume 4, Issue 3 July/August/September 2010


 Monday, November 22, 2010 Mother to Offspring Transmission of Chronic Wasting Disease 


Wednesday, November 17, 2010 

 CWD Update 98 November 10, 2010 


 CHRONIC WASTING DISEASE 


 Sent: Saturday, December 11, 2010 3:17 PM 

 Subject: Species-barrier-independent prion replication in apparently resistant species 

 Species-barrier-independent prion replication in apparently resistant species Pertenece a: UCL University College London Eprints Descripción: 

Transmission of prions between mammalian species is thought to be limited by a "species barrier," which depends on differences in the primary structure of prion proteins in the infecting inoculum and the host, Here we demonstrate that a strain of hamster prions thought to be nonpathogenic for conventional mice leads to prion replication to high levels in such mice but without causing clinical disease. 

Prions pathogenic in both mice and hamsters are produced. 

These results demonstrate the existence of subclinical forms of prion infection with important public health implications, both with respect to iatrogenic transmission from apparently healthy humans and dietary exposure to cattle and other species exposed to bovine spongiform encephalopathy prions, 

Current definitions of the species barrier, which have been based on clinical endpoints, need to be fundamentally reassessed. Autor(es): Hill, AF - Joiner, S - Linehan, J - Desbruslais, M - Lantos, PL - Collinge, J - Id.: 52395313 Versión: 1.0 Estado: Final Palabras clave: 

TRANSMISSIBLE MINK ENCEPHALOPATHY, CREUTZFELDT - JAKOB - DISEASE, FATAL FAMILIAL INSOMNIA, STRAIN VARIATION, TRANSGENIC MICE, SCRAPIE INFECTIVITY, HAMSTER SCRAPIE, VARIANT CJD, BSE AGENT, PROTEIN

 - Tipo de recurso: Article - Tipo de Interactividad: Expositivo Nivel de Interactividad: muy bajo Audiencia: Estudiante - Profesor - Autor - Estructura: Atomic Coste: no Copyright: sí Requerimientos técnicos: Browser: Any - Fecha de contribución: 10-dic-2010 Contacto: 


 for those interested, see more here with comments........ 

 Saturday, December 11, 2010 Species-barrier-independent prion replication in apparently resistant species


 Thursday, November 18, 2010 

 UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS 


 Tuesday, November 02, 2010 

 BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992 


 Rural and Regional Affairs and Transport References Committee 

The possible impacts and consequences for public health, trade and agriculture of the Government’s decision to relax import restrictions on beef Final report June 2010 2.65 

At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 

Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49 

 2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. 

Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo—has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50


 Tuesday, March 16, 2010 

 COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef 

FRIDAY, 5 FEBRUARY 2010 

AUSTRALIA COMMONWEALTH OF AUSTRALIA Proof Committee Hansard RRA&T 2 Senate Friday, 5 February 2010 RURAL AND REGIONAL AFFAIRS AND TRANSPORT [9.03 am] 

 BELLINGER, Mr Brad, Chairman, Australian Beef Association CARTER, Mr John Edward, Director, Australian Beef Association CHAIR—Welcome. Would you like to make an opening statement? Mr Bellinger—Thank you. 

The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. 

His name is Terry Singeltary and he is from Bacliff, Texas. 

He states, and rightfully so: 

 You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heidenhain variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. 

He goes on to state: The OIE— the International Organisation of Epizootics, the arm of the WTO— is a failed global agent that in my opinion is bought off via bogus regulations for global trade and industry reps. I have done this all these years for nothing but the truth. I am a consumer, I eat meat, but I do not have to sit idly by and see the ignorance and greed of it all while countless numbers of humans and animals are being exposed to the TSE agents. All the USA is interested in is trade, nothing else matters. Even Dr Stanley Prusiner, who incidentally won the Nobel Health Prize in 1997 for his work on the prion—he invented the word ‘prion’, or it came from him—states: 

 snip...see full text 110 pages ; 


 *** Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases. 


 for those interested, please see much more here ; 



 Tuesday, July 13, 2010 

 (SEE BEEF PRODUCTS EXPORTED TO AUSTRALIA FROM USA...TSS) 

 AUSTRALIAN QUESTIONNAIRE TO ASSESS BSE RISK (OIE) Terrestrial Animal Health Code, 2009 and USA export risk factor for BSE to Australia


 Saturday, August 14, 2010 

 USA NON-SPECIES CODING SYSTEM (BEEF IMPORT EXPORT BSE RISK THERE FROM) US denies it's illegally sending beef to Australia ? Friday, 13/08/2010 


 Saturday, December 18, 2010 

 First probable human case of mad cow disease in Taiwan was listed posthumously 2010/12/18 21:14:28 


 Sunday, September 6, 2009 

 MAD COW USA 1997 SECRET VIDEO 


 U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom 


Tuesday, December 14, 2010 TAFS1 

 Position Paper on Relaxation of the Feed Ban in the EU SUMMARY © TAFS, Berne, 2010 


 Monday, November 22, 2010 

 Atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control REVIEW ARTICLES 


 Thursday, November 18, 2010 

 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep 


 2008 USAHA Annual Meeting b. Atypical Bovine Spongiform Encephalopathy (BSE) – An ad hoc group on atypical transmissible spongiform encephalopathies (TSEs) concluded that the current science on atypical BSE was not enough to change the current Code Chapter on BSE. c. Atypical Scrapie – The ad hoc group on atypical TSEs also concluded that the available science on atypical scrapie was not sufficient to justify standards specific to atypical scrapie. d. Surveillance – In addition to developing a generic handbook on surveillance, the SC will, as needed, draft disease specific surveillance chapters for placement into the Code, such as those that already exist for BSE, FMD, and classical swine fever. d. Bovine Spongiform Encephalopathy (Chapter 2.3.13) – Several modifications to the chapter that helped clarify some text was adopted; however, the proposal 329 to relax the requirements on gelatin production were rejected. Opposition came primarily from the EU, Japan, Singapore, and Argentina. Without any scientific justification, these countries maintain that there is a risk of BSE transmission when gelatin is made from skulls and vertebrae sourced from countries having controlled or undetermined risk status. 


 Wednesday, April 28, 2010 BSE, Scrapie, CWD, REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL HEALTH STANDARDS COMMISSION Paris, 8-12 February 2010 REPORT OF THE MEETING OF THE OIE TERRESTRIAL ANIMAL


 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.


 4.2.9 A further hypothesis to explain the occurrence of BSE is the emergence or selection of a strain or strains of the scrapie agent pathogenic for cattle. Mutations of the scrapie agent. which can occur after a single passage in mice. have been well documented (9). This phenomenon cannot be dismissed for BSE. but given the form of the epidemic and the geographically widespread occurrence of BSE, such a hypothesis" would require the emergence of a mutant scrapie strain simultaneously in a large . number of sheep flocks, or cattle. throughout the country. Also. if it resulted "from a localised chance transmission of the scrapie strain from sheep to cattle giving rise , . to a mutant. a different pattern of disease would have been expected: its range would '. have increased with time. Thus the evidence from Britain is against the disease being due to a new strain of the agent, but we note that in the United States from 1984 to 1988 outbreaks of scrapie in sheep flocks are reported to have Increased markedly. now being nearly 3 times as high as during any previous period (18).

 If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...



 EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........


 1: J Infect Dis 1980 Aug;142(2):205-8 

 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. 

 Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. 

 Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. 

The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; 

that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. 

Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. 

One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. 

 snip... 

 The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: 6997404


 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


 Nature. 1972 Mar 10;236(5341):73-4. 

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

Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland 

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


 Epidemiology of Scrapie in the United States 1977


 Monday, December 1, 2008 

 When Atypical Scrapie cross species barriers 

 Authors Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France. Content 

 Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence. The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie. Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage. Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. 

Altogether these data indicate. 

 (i) the unsuspected potential abilities of atypical scrapie to cross species barriers 

 (ii) the possible capacity of this agent to acquire new characteristics when crossing species barrier 

 These findings raise some interrogation on the concept of TSE strain and on the origin of the diversity of the TSE agents and could have consequences on field TSE control measures.


P03.141 Aspects of the Cerebellar Neuropathology in Nor98 

 Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National Veterinary Insitute, Sweden; 2National Veterinary Institute, 

 Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. 

The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. 

The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans. 

 ***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.


 PR-26 NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS 

 R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto Superiore di Sanità, Department of Food Safety and Veterinary Public Health, Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, Norway 

 Molecular variants of PrPSc are being increasingly investigated in sheep scrapie and are generally referred to as "atypical" scrapie, as opposed to "classical scrapie". Among the atypical group, Nor98 seems to be the best identified. We studied the molecular properties of Italian and Norwegian Nor98 samples by WB analysis of brain homogenates, either untreated, digested with different concentrations of proteinase K, or subjected to enzymatic deglycosylation. The identity of PrP fragments was inferred by means of antibodies spanning the full PrP sequence. We found that undigested brain homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), truncated at both the C-terminus and the N-terminus, and not N-glycosylated. After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at the highest concentrations, similarly to PrP27-30 associated with classical scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment of 17 kDa with the same properties of PrP11, that was tentatively identified as a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 2% sodium laurylsorcosine and is mainly produced from detergentsoluble, full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a sample with molecular and pathological properties consistent with Nor98 showed plaque-like deposits of PrPSc in the thalamus when the brain was analysed by PrPSc immunohistochemistry. Taken together, our results show that the distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids ~ 90-155. This fragment is produced by successive N-terminal and C-terminal cleavages from a full-length and largely detergent-soluble PrPSc, is produced in vivo and is extremely resistant to PK digestion. 

 *** Intriguingly, these conclusions suggest that some pathological features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease. 119


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

 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*,?? +Author Affiliations *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 (received for review March 21, 2005) 

 Abstract 

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. 


 However these data confirm that, contrary to previous predictions, it is possible that a sheep prion may be transmissible to humans and that BSE from other species may be a public health risk.


 PLEASE SEE ; 

 Thursday, November 18, 2010 

 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep 


 EU COMMENTS AND POSITIONS On the proposed changes to OIE Manual of Diagnostic Tests and Vaccines for Terrestrial Animals 20 CHAPTER 2.4.6: BOVINE SPONGIFORM ENCEPHALOPATHY 

 General comments The changes proposed are generally welcomed by the EU. However, some specific comments detailed below should be taken into account for the final revised version to be adopted in the next General Session. 

 Specific comments LINE 13: 

The words "and possibly spontaneous" should be added as follows: "... suggesting that earlier, undetected indigenous and possibly spontaneous cases may have occurred." 

 LINE 31: The EU would argue for the re-instatement of the deleted phrase [before, or without, the recognition] since fallen stock in particular could be showing some clinical signs which went unrecognised. 

As written, it applies more to the active screening of the healthy slaughter population. 

 Line 228: Replace: "All currently recognized forms of BSE (C, H and L-Type) are detectable by these methods." with: "Classical BSE is recognized by all these methods, while a complete evaluation of the approved BSE rapid tests on atypical forms (C, H and L-Type) was never carried out".




 Scrapie The two Commissions discussed the issue of ‘atypical’ scrapie in terms of notification requirements and the issue of the host genetic resistance. In response to questions of Members, the Code Commission clarified that ‘classical’ scrapie is reportable to the OIE but that ‘atypical’ scrapie is not reportable (in accordance with the recommendations made by the ad hoc Group on Atypical Scrapie and Atypical BSE, which met in November 2007). However, the sharing of scientific information on ‘atypical’ scrapie is encouraged. At this time, the Code Commission considered that more scientific information would be needed to fully address the issues associated with host genotype. EU comment 4 OIE Terrestrial Animal Health Standards Commission / September 2010 The EU takes note of the fact that atypical scrapie is not an OIE listed disease. Nevertheless, it will remain notifiable in the EU. Moreover it must be stressed that any emergence of this disease should be notified to the OIE by Members and that scientific data should continue to be gathered. 

 snip... 

 Zoonotic Potential 

 Has transmission to humans been proven? (with the exception of artificial circumstances) AND Is human infection associated with severe consequences? (death or prolonged illness)

 Monday, November 30, 2009 

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


 Sunday, March 28, 2010 

 Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ? 


 THE O.I.E. and it's junk science continues to emerge, and spread, and put the cart before the horse so to speak about atypical Scrapie with it's may and may not be risk factors, because all science to date shows that in fact the Nor-98 is a risk factor to not only animal health, but human health as well. 

SINCE when did the 'may not' and 'may' become sound science ? 

 "may not be contagious and may, in fact, be a spontaneous degenerative condition of older sheep’ (22)." 

 The OIE Terrestrial Animal Health Code (the Code) does not cover atypical scrapie/Nor 98 because, it states, the condition ‘… is clinically, pathologically, biochemically and epidemiologically unrelated to ‘classical’ scrapie, may not be contagious and may, in fact, be a spontaneous degenerative condition of older sheep’ (22). 22. World Organisation for Animal Health (OIE) (2009). – Terrestrial Animal Health Code. 


 Last year, after examining member country submissions and investigating rigorous scientific research, the OIE (World Organisation for Animal Health) decided that atypical scrapie/Nor 98 should not be listed in its Terrestrial Animal Health Code. The Code sets out trade recommendations or restrictions for listed diseases or conditions, and the OIE determined there was no need for such recommendations around atypical scrapie/Nor 98. 


 Friday, May 7, 2010 

 Identification of atypical scrapie in Canadian sheep Brief Research Reports 


 Friday, August 27, 2010 

 NEW ATYPICAL NOR-98 SCRAPIE CASE DETECTED IDAHO NOW 5 CASES DOCUMENTED 2010 


 Greetings, (Figure 6) including five goat cases in FY 2008 that originated from the same herd in Michigan. This is highly unusual for goats, and I strenuously urge that there should be an independent investigation into finding the common denominator for these 5 goats in the same herd in Michigan with Scrapie. ... Kind Regards, Terry Thursday, January 07, 2010 Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 and FISCAL YEAR 2008 


 Monday, December 14, 2009 

 Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types 


 TRANSMISSION OF SCRAPIE AND ATYPICAL SCRAPIE TO HUMANS, why not ? 

 Sunday, April 18, 2010 

 SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010


 Sunday, October 3, 2010 

 Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America, who's looking ? 


 Monday, November 22, 2010 

 SHEEP WITH MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK 


 Sunday, December 12, 2010 

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


 Seven main threats for the future linked to prions The NeuroPrion network has identified seven main threats for the future linked to prions. First threat The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed. 

 *** Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases. 


 Thursday, August 12, 2010 

 Seven main threats for the future linked to prions 


 AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease. 

 snip...


 Sunday, November 28, 2010 

 Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, a synuclein and AB pathology 


 Docket APHIS-2006-0026 Docket Title Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities Docket Type Rulemaking Document APHIS-2006-0026-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Public Submission APHIS-2006-0026-0012 Public Submission Title Comment from Terry S Singletary


 Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities 

Docket Type Rulemaking Document APHIS-2006-0041-0001 

Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines 

Public Submission APHIS-2006-0041-0028 

Public Submission Title Comment from Terry S Singletary Comment 2006-2007 

USA AND OIE POISONING GLOBE WITH BSE MRR POLICY THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did...


 Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0028.1 Public Submission Title Attachment to Singletary comment January 28, 2007 Greetings APHIS, I would kindly like to submit the following to ; BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01 


 Sent: Friday, April 16, 2010 11:38 AM 

Subject: PRO-MED ATYPICAL SCRAPIE 

 Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG] "The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007. 

 "Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype. 

 "Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage." 

 Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG] The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ] 


 Sunday, April 18, 2010 

 SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010 


 Saturday, June 19, 2010 

 U.S. DENIED UPGRADED BSE STATUS FROM OIE


 see full text and reasons why here ; 


 Atypical BSE in Cattle BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD. To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures. 

 snip...see full text ; 


 14th ICID International Scientific Exchange Brochure - Final Abstract Number: ISE.114 Session: International Scientific Exchange 

 Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009 

 T. Singeltary Bacliff, TX, USA 

 Background: An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades. 

 Methods: 12 years independent research of available data Results: I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. 

 Conclusion: I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. 

I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. page 114 ; 


 The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653 

 BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein 

 Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1 1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to: John Collinge, E-mail: j.collinge@prion.ucl.ac.uk Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002 

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

 Abstract 

 Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure. Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic


 BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein 

 Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: j.collinge@prion.ucl.ac.ukReceived August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002. 


 Friday, December 17, 2010 

 PRION DISEASE Action Plan National Program 103 Animal Health 2012-2017 



THIS is from Ranchers.net, a thread long ago discussing BSE and the OIE...

from the inside looking out ;
 
Quote: Maybe familirise yourself with the OIE. The primary concern is animal health of the world they are the animal version of the WHO. It is a long way down from that ivory tower but here we go, until pressured by the USA representatives a country could not export animals for 6 years after finding a BSE/BASE positive animal so under the old rules the US would not be able to export anywhere in the world for another 4 1/2 years. Who got the risk levels system put in to allow some trade - your US representatives. You guys want to change rules - OK , but you do not get special rules that only apply to the US. As i have told you before Sand h I market all my own slaughter animals and you know that, so don't do the whole holier than thow act.
 
With all due respect, it is obvious that you know little about the OIE and how it actually works. Having been to their offices in Paris and talked personally with the Head of the Animal Test Section, you would choke if you knew how many lobby groups attend that office daily. There is a steady stream of paid lobby groups that have one goal in life and that is to sway the Section Heads of each department within the OIE to suit the needs of different jurisdictions around the world, which curiously enough, also includes the USA and Canada. Anyone can go there and chat with them - providing they can provide valid cause to be let in. To say that the only goal of the OIE is animal health is actually only part of their function. They are more than that and my discussions with Dr. Diaz there has showed me that. But to blindly make a statement regarding what they do when you have no idea what they actually do is like eating the skin of the orange and not knowing what is actually under.
 
Interestingly you state that the US Government applied pressure (to the OIE) I assume and that is a great example of the lobby groups doing their job. So, at the end of the day, one can safely assume that it is the pressure applied by certain influential lobby groups that will determine a likely outcome to an apparent OIE directive. Man alive, isn't it great to live in a democracy wherein the people get to make the choices and not just some "other" interested party or group - say like........Cargill or Tyson for example?
 
So, one last question, question?
 
Who wags the tail of that dog?? And for what reason other than one that is purely associated with trade and international agreements and greed?
 
And you think it is so simply explainable.
 
end

THE ABOVE WAS A QUOTE FROM BSETESTER Ronald Arnold Our company, BSE Prion Solutions Inc, owns the only USA Tested and Proven "Live Animal Infectious Prion Protein Urine Test" anywhere in the world that can identify the presence of PrPsc in as little as 1 ml of urine taken from a living animal.

Ron Arnold, of BSE Prion Solutions bse-tester Well-known member Joined Jul 1, 2005 Messages 517 Reaction score 0 Location Edmonton, Alberta, Canada




 Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net
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