CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008 (BSE)

CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008


PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS


BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START

A decade ago FDA launched what would become an unprecedented inspectional program. We set out to enforce the BSE feed rule, adopted in 1997, to prevent the establishment and amplification of bovine spongiform encephalopathy (BSE) by prohibiting the feeding of mammalian protein (“prohibited materials”) to ruminant animals. The regulation had national and international attention, because of then-recent evidence from the UK that humans could contract a fatal disease (variant Creutzfeldt-Jakob Disease) related to BSE by consuming meat products derived from BSE-infected cattle.

Enforcing the regulation presented a unique challenge. BSE had not been reported in the United States, and there were no practical tests to detect the presence of the causative agent during the preclinical state in live animals. Nor were there practical tests to determine the species origin of protein products found in animal feed. In addition, the regulation reached the activities of thousands of firms in a number of diverse industries – including renderers, protein blenders, and feed manufacturers. Many of these firms had no experience complying with regulations like the BSE feed rule and were not in FDA’s inventory of regulated firms.

Nevertheless, CVM set a goal of 100 percent compliance with the regulation. We wanted compliance not only with the regulation’s paperwork requirements, but also actual compliance – that is, prohibited materials would in fact not be fed to ruminants. We would achieve the goal through a campaign to educate regulated groups and by inspecting 100 percent of the regulated firms in major segments of the affected industries.

During FY 1998, the first year of enforcement, we inspected slightly more than 2,600 firms, approximately 50 percent of all renderers, but only 15 percent of all feed manufacturers. And we found that 15-50 percent of the inspected firms were not in compliance with all aspects of the regulation.

ACCOMPLISHMENTS AT THE MIDWAY POINT

By the end of FY 2003, we had achieved our goal of inspecting 100 percent of the nation’s renderers, protein blenders, and feed manufacturers – more than 6,600 firms – for compliance with the regulation. We had also inspected an additional 6,900 firms including ruminant feeders, on-farm mixers, pet food manufacturers, animal feed salvagers, and animal feed transporters.

In addition, we had nearly reached our goal of 100 percent compliance with the regulation’s requirements; less than 1 percent of the firms known to handle prohibited material had violations serious enough to require official action at their latest inspection. But that was still too many violations – we issued seven Warning Letters and instituted 14 product recalls for violations of the regulation during the year.

5 YEARS LATER – NO SIGNIFICANT VIOLATIONS

FDA and State investigators conducted more than 7,500 inspections during FY 2008. We found no serious violations; thus, no enforcement action was required. From the start of the program in FY 1998 until the end of the fiscal year 2008, we had conducted more than 66,000 inspections at more than 24,000 different facilities.

A decade ago we set a goal of 100 percent actual compliance with the rule. Although there is no way to determine definitively that the goal has been met, inspection results from recent years and the absence of any feed-related BSE cases in the United States suggests that we have effectively achieved full compliance with the feed ban.

A TEAM EFFORT

These achievements were possible only through the efforts of a number of groups and individuals, including FDA District Offices and State agencies, whose investigators have conducted the thousands of inspections; industry associations, who have educated their member firms; and CVM and Agency Headquarters staff, including CVM’s Office of Research scientists, who have done groundbreaking work in developing rapid and efficient test methods for prohibited protein materials.

THE YEARS AHEAD

This year, we turn a page in the history of enforcing the BSE rule. A 2008 amendment of the rule, to expand the scope of prohibited material, presents new enforcement challenges. We describe the rule change, and our efforts to ensure compliance, in the section “Controlling Risk from Bovine Spongiform Encephalopathy (BSE).”

A MESSAGE FROM THE DIRECTOR CVM Director, Dr. Bernadette Dunham

A Tribute to Dr. Stephen Sundlof

Dr. Steve Sundlof, my predecessor who left CVM to become Director of the Center for Food Safety and Applied Nutrition, is my mentor and friend. It was his vision that led to the publication of the CVM Annual Report, and I am proud to continue the series. Dr. Sundlof was Center Director for more than 13 years; during those years, he most capably led the Center through a number of highly visible and sometimes controversial issues. He initiated a number of undertakings, both within and outside the Center, that have contributed much to the Center’s productivity and contributions to public and animal health. His legacy with CVM is a great one, and we thank him for all that he did.

Celebrating out Accomplishments

This has been an extraordinary year for recognition from outside the Center of the work of our CVM staff members. The details of the recognition are in the opening vignette at the front of this report, in the various sections that describe our accomplishments in various areas, and in the Awards Appendix. I congratulate those on our staff whose efforts bring great credit to themselves, the Center, and the Agency.

But I want also to acknowledge the work of the many staff members whose names do not appear on awards lists or who do not have prestigious assignments to national and international groups. Their efforts – working hard day in and day out to accomplish their assigned tasks, supporting the work of others in many cases – are vitally necessary to the success of the Center’s programs. We thank them for what they – the unsung heroes – do to help the Center accomplish its mission of protecting public and animal health.

We have an enormously talented staff in CVM, with accomplishments that extend well beyond their contributions to their work assignments. We see an example in the excellent set of photographs, taken by staff members, that are displayed throughout this report. It is regrettable that we were unable to publish all of the excellent photographs in this year’s report. However, you can look for them in CVM’s future Annual Reports. I thank everyone who took the time to submit their photographic work.

HIGHLIGHTS OF FY 2008: ACCOMPLISHMENTS THROUGH THE WORK OF OUR PEOPLE

The pages of this report contain descriptions of a great number of accomplishments made by our people during the year, often with the collaboration of our partners and stakeholders. I have selected the following as examples of public and animal health significance, public interest, and level of achievement during the year. You will find more details on each accomplishment elsewhere in this report.

Animal Health Literacy Campaign

We want people to be knowledgeable about current animal health issues, to learn how to apply that knowledge for the benefit of their animals and their own health, and to know that CVM is an important source of relevant information. So when the idea for an Animal Health Literacy Campaign came from staff members within CVM – a result of our efforts to be a high performance organization – Center management enthusiastically endorsed the project. The program got off to a good start in FY 2008, and we intend to expand the program to include strategic interaction with industry groups in the future. As with the “One Health” initiative, veterinarians are key players in increasing animal health literacy levels, and we encourage their involvement in the literacy campaign.

Animal Feed Safety

The melamine-contaminated pet food recall of 2007, and several contamination incidents involving human food, changed the way consumers, Congress, and regulators think about food safety. The incidents highlighted the fact that the United States is part of a global economy, and that imports are a significant part of the human food and animal feed chain. FDA responded with its Food Protection Plan (food = feed) and an Import Safety Action Plan. These plans encompass animal feed, including pet food, which are within CVM’s responsibility. Among other actions, the Agency began the process during the year to establish offices in five foreign locations, starting with China. CVM will be part of this international presence.

The Food and Drug Administration Amendments Act of 2007 (FDAAA) requires the Agency to establish a range of standards, databases and notification systems to protect pets and other animals from unsafe food. We went right to work on this ambitious agenda in FY 2008, holding public meetings, reviewing public comment, drafting documents, and taking other actions leading toward implementation of the Congressional mandates. Our work including taking a close look at a “Pet Event Tracking Network,” a concept proposed during the 50-State Gateway to Food Protection Meeting convened to facilitate implementation of the Food Protection Plan. The Pet Event Tracking Network would be an early warning system to detect companion animal feedborne outbreaks and would provide Federal regulators the opportunity to partner with State and local health organizations.

Our research staff developed a method for detecting melamine in pet food in FY 2007. Then, in FY 2008, with the news of melamine contamination of infant formula in China, our scientists quickly modified the method so that it could be used in detecting melamine in infant formula – this time protecting human health!

Extension and Expansion of User Fees to Expedite New Animal Drug Approvals

Building on the success of the Animal Drug User Fee Act of 2003 (ADUFA I) – kudos to our Office of New Animal Drug Evaluation, which has met every ADUFA goal so far – and acknowledging the need to speed the approval process for generic new animal drugs, Congress passed two laws this past year: the Animal Drug User Fee Amendments of 2008 (ADUFA II), and the Animal Generic Drug User Fee Act of 2008. CVM participated actively with industry associations in the negotiations leading to the passage of these new laws. When ADUFA II went into effect October 1, 2008, the Office of New Animal Drug Evaluation was ready with updated procedures and training for implementation of the law’s new features that will make the program even better for the industry and CVM. With the new laws, we plan to do more to improve the efficiency and effectiveness of our drug review program, both for pioneer and generic applications.

Guidance on Controversial Pre-Market Decisions

When we set about preparing and releasing guidance on two controversial subjects – genetically engineered animals and animal clones – we knew that we needed to utilize the best science, develop a clear regulatory policy, and communicate the relevant information to the public in an understandable manner. Working with experts elsewhere in the Agency, our staff did a superb job.

Our draft guidance document on genetically engineered animals provides timely information on the regulatory environment – including specific guidance on pre-market approval requirements – to genetically engineered animal sponsors who are approaching the commercialization stage. Importantly, the guidance also describes to the consumer the protections in place to ensure the safe use of this technology.

The number of comments we received on the draft animal cloning risk assessment – 30,000 – highlighted the level of public concern and controversy on this topic. We knew that release of the final draft – which confirmed our position that clones and their progeny from several animal species are as safe as food from conventional animals and therefore do not need pre-market approval – would need to be done with great care. So we made it clear to the public as we released the risk assessment that we based all our decisions about food and animal safety on the best science available, using extensive amounts of data, and that we were working openly and using only publicly available information. We worked with communications experts on FDA’s staff to make sure we delivered complete, accurate, and understandable information to consumers.

During the year we continued to be at the forefront of the rapidly developing One Health Initiative. One Health is the collaborative effort of multiple disciplines – working locally, nationally, and globally – to obtain optimal health for people, animals, and our environment. Nearly two dozen Federal and State agencies and professional and scientific organizations – including the American Medical Association and the American Veterinary Medical Association – are participants.

The increasing threats posed by emerging zoonotic diseases (those that can be transmitted from animal to humans), food and waterborne diseases, and environmental change led to the creation of the initiative.

Because the scope of One Health includes animal health, CVM is squarely within the parameters of the initiative. Here are three examples:

Avian influenza.Our scientists made progress during the year in developing methodology to detect use of certain antiviral drugs in poultry. The methodology will be used to enforce CVM’s order prohibiting the use of those drugs in poultry. The goal is to preserve the drugs’ efficacy for human use in the event of an influenza pandemic. Foodborne antimicrobial resistance.This past year brought renewed attention to the effort, long led by CVM, to reduce resistance-related risk to human health from the use of antimicrobials in animals. For example, Congress included in ADUFA II a reporting provision related to the marketing of antimicrobial drugs, and Congress held hearings on the subject of antimicrobial resistance during the year. Our scientists continued to provide leadership and scientific expertise on this issue, on a national and international basis. They worked not only to help other nations cope with resistance issues, but also to ensure that decisions of other nations and international bodies will be based on objective evidence for determining the degree of risk. Bovine spongiform encephalopathy (BSE). Scientific evidence strongly suggests that humans can contract variant Cruetzfeldt-Jakob Disease by consuming meat products derived from BSE-infected cattle. Compliance during the past decade with our BSE feed rule, which is aimed at protecting both animal and human health, is one of the most impressive regulatory accomplishments in which CVM has participated. The success is a result of a joint effort involving FDA, State regulatory agencies, livestock production industries, the feed and rendering industries, and others. This year, we began gearing up for a new level of public protection – we have been training inspectors, coordinating with industry on education programs, and otherwise preparing to implement the amended BSE feed rule that expands the prohibition on use of cattle protein in animal food.

SNIP...

Controlling Risk from BSE THE CHALLENGE

BSE is a chronic, degenerative, always fatal neurological disease affecting the central nervous system of cattle. BSE belongs to a family of diseases known as transmissible spongiform encephalopathies that include several ruminant and nonruminant animal diseases. Laboratory and epidemiological evidence strongly suggests that people can contract a human transmissible spongiform encephalopathy, variant Cruetzfeldt-Jakob Disease, by consuming meat products derived from BSE-infected cattle. In the absence of adequate controls, BSE could spread among the cattle population through feed ingredients derived from infected cattle.

FY 2008 ACCOMPLISHMENTS

Much of our effort to combat BSE in 2008 focused on enforcing and strengthening our BSE feed regulation, which prohibits the use of certain mammalian-origin proteins in ruminant feed. A major initiative involved an amendment of the BSE feed rule to strengthen public health protection. The goal is to prevent the establishment and amplification of BSE in the United States through animal feed. Following are highlights of some of our achievements that accomplished FDA’s strategic goal of consumer protection, and the Department-wide objective of improving the safety of food products.

U.S. AND WORLDWIDE BSE DETECTION

As in the previous fiscal year, FY 2008 did not see the discovery of any BSE-infected cattle in the United States. During the same time period, four new BSE cases were detected in Canada. Worldwide, the incidence of BSE continued to decline significantly.

STRENGTHENING THE BSE FEED REGULATION

Overview of Rule Revision

FDA’s 1997 BSE feed rule prohibits the use of mammalian-origin proteins in feed fed to ruminants,19 while allowing the use of these materials in feed for non-ruminant animals.20 In April 2008, the Agency revised the regulation to strengthen further the feed-related safeguards against BSE in the United States. The revised rule prohibits the use of the highest risk cattle materials in the food or feed of all animals, ruminants and non-ruminants, including pet food.

Need for Revision

Compliance with the 1997 rule has been extremely high. No cases of BSE in animals born after the introduction of the ban have been detected, and no new BSE cases have been reported in the United States since March 2006. Nevertheless, the FDA concluded that additional protections were needed. One reason was that inspections of feed manufacturing firms had identified a small number of instances of inadequate cleanout procedures, mislabeling, and recordkeeping deficiencies. Although few in number, such lapses could allow food and feed intended for nonruminants to get into the food supply for ruminants. In addition, the amount of feed material needed to transmit the disease is so small that it is possible that the procedures followed by a seemingly compliant firm may not be sufficiently robust to prevent cross-contamination of cattle feed with enough infectious material to cause new cases of BSE. This scenario best explains why cases of BSE continued to be found in cattle born in the United Kingdom after implementation of that country’s ruminant-to-ruminant feed ban.

Details of the Revised Rule

The new rule is intended to mitigate compliance failures and prevent the potential transmission of the BSE agent through cross-contamination or on-farm misfeeding of prohibited material to ruminants. Thus, the new rule is expected to further reduce risk of any ruminant exposure to the BSE agent not eliminated by the 1997 feed rule.

Revisions to definition of prohibited material. Scientific data indicate that roughly 90 percent of BSE infectivity in cattle is contained in the brain and spinal cord of older cattle.21 For this reason, the new rule focuses primarily on removing the brains and spinal cords of older cattle from all animal feed. Specifically, the new rule prohibits the use of the following cattle materials in the food or feed of all animals, including pet food:

The entire carcass of BSE-positive cattle; The brains and spinal cords from cattle 30 months of age and older; The entire carcass of cattle not inspected and passed for human consumption that are 30 months of age or older and from which brains and spinal cords were not removed; Tallow that is derived from BSE-positive cattle; tallow that is derived from other materials prohibited by this rule that contains more than 0.15 percent insoluble impurities; and Mechanically separated beef that is derived from the materials prohibited by this rule. We refer to these materials as “cattle materials prohibited in animal feed,” or CMPAF.

The final rule allows renderers to process the entire carcasses of dead stock cattle22 under 30 month of age for use in nonruminant feed. (The risk of BSE in cattle less than 30 months of age is considered to be exceedingly low.) The proposed rule would have required removal of the brain and spinal cord from all cattle, regardless of age, because of FDA concerns that government inspectors are not routinely available in rendering plants to verify the ages of dead cattle. The Agency changed the final rule, based on comments that age determination of dead stock cattle in the rendering plants is feasible. The regulation allows renderers the option of determining the age of the cattle and processing cattle under 30 months of age without removing the brain and spinal cord. However, the final rule requires renderers to develop and maintain written procedures for determining the age of and/or removing the brain and spinal cord from dead cattle.

FDA also based its proposal on European surveillance data that showed that cattle not inspected and passed for human consumption (dead stock) were included among the cattle at highest risk of BSE. However, the comments supported the conclusion that very little risk reduction is gained by excluding material from such cattle that are less than 30 months of age.

Inspections and record-keeping. During inspections at rendering facilities, FDA intends to verify that renderers maintain records sufficient to demonstrate that material rendered for use in animal feed does not contain CMPAF. In addition to written procedures for determining the age of cattle, the regulations also specify written procedures for effectively excluding the brain and spinal cord from animals 30 months of age and older. Investigators will be verifying that actual practices are effective in providing compliance with the regulation. The Agency revised the final rule to clarify that a renderer’s records must include certification from each supplier, or other documentation acceptable to FDA, that CMPAF has been excluded from materials to be rendered for use in animal feed.

Disposing of CMPAF. As a result of this final rule, a large volume of byproducts from the beef and cattle industries will no longer be allowed to be rendered for animal feed use. Alternative means of disposing of this material include landfill, composting, incineration, alkaline hydrolysis, and burial.

Country exception. In response to comments to the proposed rule, FDA revised the final rule so that the Agency may designate a country as not subject to the new requirements; that is, animal protein from the country would not have to have CMPAF removed if it is intended for consumption by nonruminant animals. Any country seeking such a designation must submit a written request to the Director of CVM, providing information about that country’s BSE case history, risk factors, measures to prevent the introduction and transmission of BSE, and any other information relevant to determining the country’s BSE status.

Conclusion

Two and a half years passed from the time of the proposed rule (October 2005) until publication of the final rule (April 2008). During that time, FDA reviewed more than 800 comments, including comments from industry, State and local governments, trade associations, academia, and consumers. Among other time-consuming steps, the Agency reviewed comments challenging FDA’s estimate of the cost of the new regulation. This review required an extensive reanalysis of the economic impact of the rule. We believe that the time and effort required, including that needed to draft changes to the proposed rule, were worthwhile. The revised regulation provides an additional margin of safety by reducing the consequences of inadvertent cross-contamination or on-farm misfeeding, thereby helping to ensure that the U.S. beef supply remains safe.

We plan to publish a Guidance for Industry to assist the industry in complying with the revised regulation. More information about BSE and the BSE rule is available on the CVM Web site at


http://www.fda.gov/AnimalVeterinary/GuidanceComplianceEnforcement/ComplianceEnforcement/BovineSpongiformEncephalopathy.htm





INTERNATIONAL INVOLVEMENT

We provided information to trading partners and the World Organisation for Animal Health that included feed ban enforcement data and updates on FDA’s science-based enhancements to animal feed controls for preventing transmission of BSE through feed.

A CVM staff member, Dr. Dragan Momcilovic, continued to serve as the only U.S. member of the Board of SAFEED-PAP,23 a European project that is aimed at the development of improved testing methods for detection of prohibited animal protein in feed. The participants, mostly regulatory scientists, include representatives of national laboratories and several universities. A large number of European nations, in addition to Japan, China, and the United States, participate in the project.

TRAINING, EDUCATION, AND ENFORCEMENT

We continued to train inspection personnel, so that the BSE feed rule will be enforced effectively and efficiently. We also continued to provide educational outreach to the regulated industry.

Specifically, during FY 2008 we conducted a formal, full-day training course for inspection personnel. We also provided training/outreach information to feed regulators and industry personnel at a number of other meetings. CVM will increase training activities in FY 2009 to ensure proper training for inspectors for the implementation of the 2008 amendment to the BSE rule.

Additionally, we provided updates on BSE inspections and enforcement, as well as information about the new BSE rule, at several meetings sponsored by the Association of American Feed Control Officials; two of the workshops were open to industry representatives. We presented the same information at a meeting sponsored by FDA’s Division of Federal-State Relations for representatives of States that have contracts to do inspection work for FDA. We will continue educational outreach to industry during FY 2009 to help with compliance with the revised feed rule.

During FY 2008, the FDA district offices and State agencies conducted more than 7,889 inspections for BSE feed rule compliance at renderers, feed manufacturers, and other firms. More than 98 percent of the inspections were found to be NAI (no action indicated), meaning that the firms were in compliance. No firms were classified OAI (official action indicated); that is, no serious violations were found. We did classify approximately 0.8 percent of the inspections as VAI (voluntary action indicated). Typically VAI involves minor recordkeeping violations, which can be corrected at the time of the inspection. As a result, no formal enforcement actions (Warning Letters, injunctions, or seizures of violative products, for example) were taken during FY 2008.

DEVELOPING ANALYTICAL METHODS FOR DETECTING PROHIBITED PROTEIN

During the year, we completed peer validation of the real time polymerase chain reaction (PCR) method developed by CVM scientists to detect prohibited animal proteins in animal feed. The method will detect cattle, sheep, and goat materials produced under processing conditions used in the United States and European Union. The real time PCR method represents a significant improvement over the current PCR method, because a single analyst can analyze 12 samples in a little more than 2 hours. The current method requires 8 hours to analyze these same 12 samples. At the end of the fiscal year, we were working actively with the FDA field laboratories to transfer the method to those laboratories for use in surveillance and compliance activities. The process of transferring the method included training in the methodology prior to releasing it for use by the laboratories.

With the help of a visiting scientist, CVM scientists during the year were able to modify further the real-time PCR method into a multiplex real-time PCR method, which permits the detection of cattle, sheep, goat, deer, and elk in a single reaction tube. This test will also permit detection of ruminant proteins rendered in the European Union. One goal of this effort is to create an assay in which both the DNA extraction and PCR assay reagents are contained in two ready-to-use, commercially available kits. By having all the assay components ready to use from commercial kits, the FDA field laboratories will not need to make up any component, nor will they need to constantly conduct quality control checks of reagents that they need to prepare. Eliminating variation in reagents between different testing laboratories avoids a potential source of error when performing this method.

SNIP...END OF BSe ;

full text ;


http://www.fda.gov/AboutFDA/CentersOffices/CVM/ucm185152.htm#BSEFeedRuleEnforcement:ADecadeofSuccess






NOW, LETS GET TO THE FACTS, and or the truth ;


10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007

CODE

Cattle feed delivered between 01/12/2007 and 01/26/2007

RECALLING FIRM/MANUFACTURER

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON

Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

42,090 lbs.

DISTRIBUTION

WI

___________________________________

PRODUCT

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

REASON

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE

9,997,976 lbs.

DISTRIBUTION

ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html


NEW URL

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



PLEASE NOTE, THE FDA URLS FOR OLD WARNING LETTERS ARE OBSOLETE AND DO NOT WORK IN MOST CASES. I LOOKED UP THE OLD ONE ABOVE AND FOUND IT, BUT HAVE NOT DONE THAT FOR THE OTHERS TO FOLLOW. THE DATA IS VALID THOUGH!


Thursday, March 19, 2009

MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA WITH ONGOING 12 YEARS OF DENIAL NOW, WHY IN THE WORLD DO WE TO TALK ABOUT THIS ANYMORE $$$

http://madcowfeed.blogspot.com/2009/03/millions-and-millions-of-pounds-of-mad.html



Subject: MAD COW PROTEIN IN COMMERCE USA 2006 RECALL UPDATE From: "Terry S. Singeltary Sr." <[log in to unmask]> Reply-To: SAFETY <[log in to unmask]> Date: Mon, 9 Oct 2006 14:10:37 -0500 Content-Type: text/plain Parts/Attachments: text/plain (558 lines) Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS IN COMMERCE AL, TN, AND WV Date: September 6, 2006 at 7:58 am PST

PRODUCT a) EVSRC Custom dairy feed, Recall # V-130-6; b) Performance Chick Starter, Recall # V-131-6; c) Performance Quail Grower, Recall # V-132-6; d) Performance Pheasant Finisher, Recall # V-133-6. CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.

REASON Dairy and poultry feeds were possibly contaminated with ruminant based protein.

VOLUME OF PRODUCT IN COMMERCE 477.72 tons DISTRIBUTION AL

______________________________

snip...

http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html



Subject: MAD COW FEED RECALLS ENFORCEMENT REPORT FOR AUGUST 9, 2006 KY, LA, MS, AL, GA, AND TN 11,000+ TONS Date: August 16, 2006 at 9:19 am PST RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE - CLASS II

______________________________

snip...

______________________________

PRODUCT Bulk custom dairy pre-mixes, Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete.

REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.

VOLUME OF PRODUCT IN COMMERCE 350 tons DISTRIBUTION AL and MS

______________________________

PRODUCT a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb. bags, Recall # V-121-6; b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6; c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6; d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6; e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6; f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6; g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6 CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.

REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".

VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags

DISTRIBUTION AL, GA, MS, and TN

END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006

###

http://www.fda.gov/bbs/topics/ENFORCE/2006/ENF00964.html



Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS

Products manufactured from 02/01/2005 until 06/06/2006 Date: August 6, 2006 at 6:16 pm PST PRODUCT a) CO-OP 32% Sinking Catfish, Recall # V-100-6; b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6; c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6; d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6; e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6; f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6; g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6; h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6; i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6; j) CO-OP LAYING CRUMBLES, Recall # V-109-6; k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6; l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6; m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE Product manufactured from 02/01/2005 until 06/06/2006 RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.

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

VOLUME OF PRODUCT IN COMMERCE 125 tons DISTRIBUTION AL and FL

END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html



MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II

______________________________

PRODUCT a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6; b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6; c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6; d) Feather Meal, Recall # V-082-6 CODE a) Bulk b) None c) Bulk d) Bulk RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.

REASON Possible contamination of animal feeds with ruminent derived meat and bone meal.

VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons

DISTRIBUTION Nationwide

END OF ENFORCEMENT REPORT FOR July 12, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html



look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;

Risk of oral infection with bovine spongiform encephalopathy agent in primates Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.

snip...

BSE bovine brain inoculum 100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg Primate (oral route)* 1/2 (50%) Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%) RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%) PrPres biochemical detection The comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal. Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inocula

Published online January 27, 2005

http://www.thelancet.com/journal/journal.isa



It is clear that the designing scientists must also have shared Mr Bradley's surprise at the results because all the dose levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s145d.pdf



2) Infectious dose:

To cattle: 1 gram of infected brain material (by oral ingestion)

http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml



http://list.uvm.edu/cgi-bin/wa?A2=SAFETY;pbpHeQ;20061009141037-0500B



Office of Inspector General Semiannual Report to Congress FY 2007 - 2nd Half

Two Texas Companies Sentenced and Fined for Misbranding Meat Products In April 2007, two closely held and related Texas companies pled guilty in Federal court and were sentenced to 12 months of probation and ordered to pay $10,250 in fines for misbranding meat products. One of the companies sold adulterated meat products to a retail store in New Mexico. Additionally, portions of the invoices failed to properly and consistently identify the meat products as being from cattle more than 30 months old at time of slaughter. This information is required to be disclosed because of bovine spongiform encephalopathy (BSE, or "mad cow disease") concerns. No adulterated meat reached consumers.

http://www.usda.gov/oig/webdocs/sarc071212.pdf



Saturday, August 29, 2009

FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009

http://madcowfeed.blogspot.com/2009/08/foia-request-feed-recall-2009-product.html



Friday, September 4, 2009

FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009

http://madcowfeed.blogspot.com/2009/09/foia-request-on-feed-recall-product.html



Sunday, September 6, 2009

MAD COW USA 1997 SECRET VIDEO

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html



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

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN

http://maddeer.org/video/embedded/prusinerclip.html



2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html



Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html



Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ? August 20, 2008

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html



Monday, May 11, 2009

Rare BSE mutation raises concerns over risks to public health

http://bse-atypical.blogspot.com/2009/05/rare-bse-mutation-raises-concerns-over.html



Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

see full text ;

http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html



Sunday, June 07, 2009

L-TYPE-BSE, H-TYPE-BSE, C-TYPE-BSE, IBNC-TYPE-BSE, TME, CWD, SCRAPIE, CJD, NORTH AMERICA

http://bse-atypical.blogspot.com/2009/06/l-type-bse-h-type-bse-c-type-bse-ibnc.html



Sunday, May 10, 2009

Identification and characterization of bovine spongiform encephalopathy cases diagnosed and NOT diagnosed in the United States

http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.html



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

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801e47e1



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, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure. ...

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8151



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

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f8152&disposition=attachment&contentType=msw8



Sunday, August 10, 2008

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html



SEAC OCTOBER 2009

• Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility

http://www.seac.gov.uk/pdf/hol-response091008.pdf



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html



>>> In the papers, the government alleges the meatpacking plant slaughtered and processed downer cows for nearly four years - from January 2004 to September 2007 - <<<>95%) downer or dead dairy cattle and a few horses. She had never been fed.

We believe that these findings may indicate the presence of a previously unrecognized scrapie-like disease in cattle and wish to alert dairy practitioners to this possibility.

snip...

PROCEEDINGS OF THE SEVENTH ANNUAL WESTERN CONFERENCE FOR FOOD ANIMAL VETERINARY MEDICINE, University of Arizona, March 17-19, 1986

http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf



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



IS THERE A SCRAPIE-LIKE DISEASE IN CATTLE ?

YOU BET THERE IS, AND HAS BEEN, AND WE BEEN FEEDING THE MOST HIGH RISK I.E. DEAD STOCK DOWNER COWS TO OUR CHILDREN FOR DECADES, who will follow these children for human TSE from mad cow disease here in the USA in the years, decades to come, and how many will they expose from the 'pass it forward' friendly fire modes ???

http://downercattle.blogspot.com/2008/12/evaluation-of-fsis-management-controls.html



Saturday, May 2, 2009

U.S. GOVERNMENT SUES WESTLAND/HALLMARK MEAT OVER USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM

http://downercattle.blogspot.com/2009/05/us-government-sues-westlandhallmark.html



Thursday, May 1, 2008 DEAD STOCK DOWNER COW BAN i.e. non-ambulatory policy still not changed by USDA May 1, 2008

http://downercattle.blogspot.com/2008/05/dead-stock-downer-cow-ban-ie-non.html



Sunday, May 17, 2009

WHO WILL WATCH THE CHILDREN ? SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/






Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

MAD COW USA 1997 VIDEO

According to the print story on the CBC News site ;

http://www.cbc.ca/story/world/national/2005/04/12/usbse050412.html

The United States did not properly analyze two suspected cases of mad cow disease in 1997, years before it showed up in Canada and devastated this country's beef industry, a CBC News investigation suggests.

Dr. Masuo Doi (a former USDA vet) ... is now retired and speaking for the first time about his concerns. "I don't want to carry on off to my retirement," he told CBC's Investigative Unit. "I want to hand it over to someone to continue, to find out. I think it's very, very important ... "How many did we miss?"

Documents obtained by CBC show that the samples tested by the department did not contain parts of the animal's brain critical for an accurate diagnosis.

1997 video from New York shows stricken cow The scientists' comments raise new questions about how the U.S. industry has been able to essentially escape BSE when Canada's much smaller industry, observing almost identical safety and testing practices, has had four cases in the past two years. Part of the answer could be in a slaughterhouse in Oriskany Falls, N.Y., which eight years ago may have become the home of the first American case of mad cow.
The suspect cow was recorded on USDA videotape, which has been obtained by CBC News. CBC News has now learned that key areas of the brain where signs of BSE would be most noticeable were never tested. The most important samples somehow went missing. That information was contained in a USDA lab report that was left out of the documents officially released by the department. It proves that the scientist in charge of the case knew his investigation was limited because of the missing brain tissue.

With questions about the first cow still lingering, a second American cow showed up at the same plant three months later with suspicious symptoms. Videotape of that animal shows its head was bobbing and it was unable to rise to its feet, setting off warning bells for mad cow disease. The second cow's brain was also sent for testing. Officials were later told verbally that the samples had tested negative for BSE. Doi made repeated requests for documentary proof of the negative tests. To this day, he has seen nothing. "How many are buried?" he wonders of other possible cases of BSE in the United States. "Can you really trust our inspection [system]?" For weeks, the USDA told CBC that it had no records for the second cow suspected of having BSE in 1997. Then just a few days ago, it suddenly produced documents that it says proves that a cow was tested and that the tests were negative for mad cow disease. But the documents also prove, once again, that there were problems with the testing. This time, so much brain tissue was missing that it compromised the examination. 2nd suspected mad cow, from a USDA video. The problems were so severe that one USDA scientist wrote that his own examination was of "questionable validity" because he couldn't tell what part of the cow's brain he was looking at. ...SNIP...END

DON'T be surprised at this video. THE USDA et al have been hiding mad cows since before this video was made, and they continue to this day. NOW, what will the USDA et al do about this past 2 decades of cover-up of mad cow disease in the USA ??? probably the same old BSe. ...TSS

""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."

THIS WAS DONE FOR A REASON!

THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS

USDA 2003

We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip.............

Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip...

FULL TEXT;

Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

2005

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

CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006

The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.

The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...

http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm

PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."

end...TSS

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm

http://madcowtesting.blogspot.com/2009/02/report-on-testing-ruminants-for-tses-in.html

FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

####

http://www.fda.gov/bbs/topics/news/2004/new01061.html

USDA: In 9,200 cases only one type of test could be used

WASHINGTON (AP)--The U.S. Department of Agriculture acknowledged Aug. 17 that its testing options for bovine spongiform encephalopathy were limited in 9,200 cases despite its effort to expand surveillance throughout the U.S. herd.

In those cases, only one type of test was used--one that failed to detect the disease in an infected Texas cow.

The department posted the information on its website because of an inquiry from The Associated Press.

Conducted over the past 14 months, the tests have not been included in the department's running tally of BSE tests since last summer. That total reached 439,126 on Aug. 17.

"There's no secret program," the department's chief veterinarian, John Clifford, said in an interview. "There has been no hiding, I can assure you of that."

Officials intended to report the tests later in an annual report, Clifford said.

These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC.

In the Texas case, officials had declared the cow free of disease in November after an IHC test came back negative. The department's inspector general ordered an additional kind of test, which confirmed the animal was infected.

Veterinarians in remote locations have used the preservative on tissue to keep it from degrading on its way to the department's laboratory in Ames, Iowa. Officials this year asked veterinarians to stop using preservative and send fresh or chilled samples within 48 hours.

The department recently investigated a possible case of BSE that turned up in a preserved sample. Further testing ruled out the disease two weeks ago.

Scientists used two additional tests--rapid screening and Western blot--to help detect BSE in the country's second confirmed case, in a Texas cow in June. They used IHC and Western blot to confirm the first case, in a Washington state cow in December 2003.

"The IHC test is still an excellent test," Clifford said. "These are not simple tests, either."

Clifford pointed out that scientists reran the IHC several times and got conflicting results. That happened, too, with the Western blot test. Both tests are accepted by international animal health officials.

Date: 8/25/05

http://www.hpj.com/archives/2005/aug05/aug29/BSEtestoptionswerelimited.cfm

""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."

THIS WAS DONE FOR A REASON!

As for lowering standards, R-CALF has referenced the OIE (World Organization for Animal Health) as the authority on animal health issues. That's fine, as far as it goes. Trouble is, the OIE does not set standards, as R-CALF has claimed. Further, the OIE does not recommend countries ban meat imported - with SRMs removed - from countries with low or high BSE risk, contrary to R-CALF's implication.

In addition, there are no standards recognized for importing meat from minimal- or low-risk BSE countries. The U.S. is trying to set standards as precedent for trade, based on nearly 20 years of science. R-CALF wants trade only with countries who have never had a BSE case. They have not explained how many years they want the rest of the world to sit around and wait until it's okay to trust science and begin trading. Or how they would justify keeping imports out if ever a BSE case was discovered in the U.S. or export again ever.

http://www.mad-cow-facts.com/News-Commentary/r-calf-bullard-4-4-05.htm

JUST ABOUT EVERY COUNTRY THAT WENT BY THOSE FAILED OIE BSE GUIDELINES WENT DOWN WITH BSE. ...TSS

OH, NOT TO FOGET ;

Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program

An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.

snip...

4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half

http://www.usda.gov/oig/webdocs/sarc070619.pdf

http://madcowtesting.blogspot.com/2009/04/bse-mad-cow-testing-usa-2009-figures.html

Tuesday, August 18, 2009BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html

Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PSTWHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html

Monday, June 01, 2009

Biochemical typing of pathological prion protein in aging cattle with BSESOMETHING TO PONDER ???

O.K. confusious asks, IF all these new atypical BSEs i.e. new strains of mad cow disease is just an 'OLD COW PRION DISEASE', why then can not the 'old human prion disease' such as the sporadic CJD, be from an 'old cow prion disease', same as the nvCJD 'young people mad cow disease' (which also happens in 74 year old), but why cannot the 'old cow prion diseases', i.e. l-BSE, h-BSE, and ibncBSE, cause the 'old people prion disease', which looks like sporadic CJD. seems that is what some of the pathology is showing ???OH, that probably makes too much sense, and that the only answer could be that it's all just a happenstance of bad luck and or a spontaneous event, that just happens out of the clear blue sky $$$IF this is the case, then where are all the SPONTANEOUS BSE CASES OF MAD COW DISEASE IN THE U.S.A., AND WHERE HAVE THEY BEEN BURIED IN THE USA OVER THE LAST 25 YEARS ???

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html

SEE THIS DAMNING VIDEO AT BOTTOM OF ;

Monday, July 27, 2009

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html

Monday, May 4, 2009

Back to the Past With New TSE Testing Agricultural Research/May-June 2009

http://madcowtesting.blogspot.com/2009/05/back-to-past-with-new-tse-testing.html

Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html

TSS

BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

Greetings,

I wish to send this old interview from bbc radio 4 back in 1999 i believe. but most importantly is some of the late Dr. Joe Gibbs and sporadic CJD, and pathology and links of illness, young people with CJD, all right here in the USA. there is much misspelling, which i left the way it was, i am not too good at spelling either :-)

1999

BSE-The Untold Story

Richard Uridge investigates reports that a new version of the brain disease CJD is taking hold in young people across the Atlantic, and asks whether American Scientists are right to question Britain's strategy for dealing with Mad Cow Disease, and it's equivalent in humans.

Richard Uridge : Mad Cow Disease.Three simple words with an almost humorous ring, but there's nothing remotely funny about Bovine Spungiform Encephelopathy, so far it's claimed 40 lives in Britain,and no one's sure how many more people will die.

[Compare the 40 dead people to the thousands who die of lung cancer from smoking,or the number involved in drink driving accidents or from alcohol damage to the brain or liver and, purely in terms of numbers and risk,one wonders why. Perhaps because it was perceived that it would permeate the human food chain? BSE is such a scare story -LB]

"I think we were in real difficulty with these diseases, they're biologically obscure, they have very long incubation periods and we simply didn't have some of the information that people would have liked us to have and we still don't."

Richard Uridge : BSE has cost British tax payers a staggering 4 billion pounds.More than 3 million cattle have been slaughtered, and British farming ears to recover. Yet there's the possibility that our whole response to the disease is will take many ybased on an incorrect hypothesis. This programme investigates the challenges to the pillars of conventional wisdom on BSE, and its human equivalent new variant Creuzfeld-Jacob disease.

"If we're wrong, and the route of transmission is not through eating beef, then we have decimated an industry, and we have frightened millions of people totally unnecessarily"

Richard Uridge : In fact, there's a serious body of scientific opinion that believes BSE got into people through the blood stream, not the stomach. Right or wrong, last week the British government started importing one blood product, plasma from the US, where officially there is no BSE, and no new variant of CJD. But American experts have told us that Britain could be making a big mistake because there's mounting concern that a new strain of CJD is taking hold on the other side of the Atlantic. "We have our own BSE problem here.You know we're facing what the UK faced a few years ago."

"Is it a time bomb, is it just something that a few people seem susceptible to, it makes for a very bad science-fiction movie."

Richard Uridge : So our story begins in America. Older people have been sporadically and apparently spontaneously contracting CJD for may years, but now younger Americans are being affected, and bearing in mind that it was young people getting CJD here that alerted Britain to a possible link with mad cows, the trend is worrying an increasing number of people across the Atlantic. Andrew Kimbrel is a lawyer in Washington DC, and Director of the Centre for Food Safety. His particular concern is that the picture is confused, because no one is monitoring the disease properly, as the case of Douglas McKewan suggests.

Andrew Kimbrel : He was one of 7 cases in a cluster in Utah, in the past year. Now when I spoke to Tracy McKewan, his wonderful wife, the story that she told, what it took for her to get this disease diagnosed was extra ordinary, and they put them through every test you can imagine. He was a man in his 30s, young kids, good job, just a regular guy. Comes home feeling sick, feeling disorientated, not sure what's going on with him. They decide that he's mentally ill. Well she doesn't believe it. She thinks something's terribly wrong. So she happens to see a guy on TV talking about one of the other CJD cases in their area, and she says "That's what my husband has, that's what he has". So she calls her doctor,a nd he says, you know, "Darling if I had to listen to everybody who sees things on TV, and says "That's what I have" or "My husband has"...".She says "No,that's what he has", and she then has to go to another hospital, they won't do it, she then has to go to Washington here, where she finally gets some NIH people to do the proper tests, and of course she has the variant CJD, and he died. For all we know there could be 50 or 100, but 7 who had enough courage, enough, to get the special test, to go the next step, to figure out what's going on. This is a very fast .....people are dying in months, they waste away in months.

[It sounds like Roy Neary in Close Encounters -LB]

Richard Uridge : Jay Whitlock is wasting away from CJD in Oklahoma.doctors say it's a sporadic case. His wife Julie is not so sure. One - because he's just 27 years old. Two - because the symptoms she describes are almost identical to those who've suffered from new variant CJD in this country. It's a critical distinction, and one which she outlined for us in this, her first ever media interview.

Julie Whitlock : Right now, he's in a field nursing facility. He's uncomprehensive [I think she means incomprehensible -LB], having some hallucinations, incontinent. He still does, know me, he will hug me and hold me, use names. He started having memory trouble in January of this year 1999. He was forgetting phone numbers, and then when we would be heading somewhere to go eat, he'd go past the restaurant,a nd I'd say "What are you doing?" and he'd say "Well where are we going?", and I'd tell him again, and we'd be heading home and he'd take different routes to get home, and I'd say "What are you doing?" and he'd say, "If you want to drive, you get over here and drive". So you know, the next time he'd take different directions somewhere, I would think "Okay, he's testing me", you know? I'd think it was just a man thing, he was... you know, just doing that on purpose,and then... that was through January, and it was February 1st, his employer talked to me, and said, "You know, he's forgetting... are you having trouble?", Jay said, "Well he's forgetting some stuff", he said "Well he's doing it at work too", and I didn't have any idea about that. So that's when I got him to the doctor the next day. Most times, I feel like we're living a nightmare, and, you know, the children, Zachary, he's... the youngest is 3, and he knows daddy's sick, he doesn't understand any more. But the oldest, he knows there's no medicine to make daddy better, and he will pray to God to find the medicine [A lot of good that will do -LB] to make his daddy better. You know, so the kids are.. you know it's just continuous stress, worry and heartache.

Richard Uridge : Sadly there is no cure for CJD, and proof of whether Mr Whitlock does indeed have the new variant will have to wait until after his death. Only then can tests be conclusive. But if the tests are positive, how might he and the other American victims have contracted the disease? One theory is that it comes from eating deer or elk meat infected with a Spungiform Encephelopathy, similar to Mad Cow Disease, but it is only a theory. Even in Britain there is still no absolute proof, that eating mad cows gave humans CJD, and that's something that bothers June Goodfield, a zoologist, and campaigner for the public understanding of science.

June Goodfield : It isn't enough just to say, "In the absence of any other viable hypothesis, this is the best one". It isn't enough, because we have a number of things, including the fact that we have not reproduced it experimentally in non-hum, an primates, weighing against it. It is vitally important we find out why. It's important because, if we're wrong, and the route of transmission is not through eating beef, then we have decimated an industry, and we have frightened millions of people totally unnecessarily, and I believe we could nail it down.

Richard Uridge : She says that there are experiments that could establish this one way or another, and she argues that if eating meat was the cause, we should have seen many more cases by now.

June Goodfield : If you think of the tons of BSE infected hamburgers that have been fed, probably, to British children, if eating BSE infected meat was the cause of new variant CJD in human beings, we would would be expecting to see evidence of this in that age group and we are not seeing it. [Not if it has very long incubation periods-LB] That is a very significant epidemiological fact.

Richard Uridge : The mainstream consensus among British experts that is despite the lack of absolute proof, the government had to act on what remains a very probable explanation. Alan Dickinson, carried out much of the ground breaking work on Scrapie, the sheep brain disease, which many people believe was the cause of BSE in cows. He thinks eating infected material from mad cows is almost certainly the way humans got new variant CJD. His careful not to blame per se, he says the more likely culprit is infected brains, spinal chord or offal, which then got into pies, sausages and burgers.

[If this is true why aren't dogs and cats suffering from CJD or BSE as, those products are more likely to end up in dog and cat food? -LB]

Alan Dickinson : The fact that 20-30 people have got BSE, I think is indisputable.The fact that they have got it from something that you would be likely to buy in a supermarket or a butchers shop, I think is a very,very high, probability. Alternative probabilities, I see no reasonable ones for accepting. If you have a hit-and-run accident, and the car is never found, you are in a reasonable position to conclude that it was a car that did it, for a variety of reasons. I think we're in this same position, of saying, a bovine product of some sort..... a bovine food product of some sort, has been the origin of these cases in humans.

Richard Uridge : Our search for clues now takes us back to the late 1950s, when the American Nobel Prize winner Carlton Gadusek, was working with the Foray people in Papua New Guinea. He was intrigued by a brain disease called "kuru". Why, he wanted to know, did only the women and children of the tribe suffer from it. June Goodfield is one of Dr Gadusek's contemporaries.

June Goodfield : The men of the Foray were the ones who were responsible for going hunting, and they went off, and they would sort of kill a few wild pig or what have you. But they kept the spoils of the hunting for themselves. On the whole, the women of the Foray ate vegetables, roots and tubers and what have you, the occasional rodent. But often had very little meat, and they would from time to time, as they prepared the bodies for burial, put slivers of the meat from the flesh, and eat them and give them to the children, or they would put the brain tissue into a little bamboo and steam it and eat it. But anybody who says that Carlton Gadusek believes that cannibalism was the route, is misrepresenting what Carlton Gadusek thinks, and I know because I've asked him, and I've quoted it in my book, and I know because I've asked Joe Gibbs, and I've quoted it in my book.

[There was a TV programme that claimed that a tribe, maybe the same one, had the women and children eat the brains of the dead males, maybe this was the misrepresentation -LB]

Richard Uridge : Gibbs worked with Dr Gadusek, so we spoke to him in his office in Maryland, and asked him about his experiments with chimpanzees to test the theory that eating brains spread kuru in the Foray tribe.

Joe Gibbs : The first experiments we did were to put stomach tubes into the animals, as you would for a gastric analysis, and through those tubes, we introduced copious amounts of highly infectious, known to be transmissible kuru, CJD, and nothing has ever happened to those animals.

June Goodfield : Faced with the fact that experimentally they could not reproduce this route of transmission, that is eating, in the laboratory, they were faced with the fact, "How on Earth did the young children and the women of the Foray get the infected agent?". The only way that Gadusek and Gibbs could experimentally reproduce this,was by inoculation in some form.

Joe Gibbs : Somewhat later, in the course of our studies, I designed an experiment, in which I took squirrel monkeys and starved them for two or three days, then allowed them to handle the infected tissues, from other primates that had developed the diseases, kuru, CJD and Scrapie and in doing so, they consumed the infected tissues. I think in most cases it was upwards of around 80 grams of tissue and all of these squirrel monkeys, both, for kuru, CJD and Scrapie, all developed disease.They have positive pathology and we extracted the prion protein from their brain. So it was, yes we transmitted orally, but letting the animals handle the tissue and consume the tissue, rather than putting it directly into the stomach of chimpanzees and other small monkeys.

June Goodfield : And they finally came to the conclusion that if you looked at the kids and the women, you know they're not living in antiseptic, sterile conditions, they're living in close contact with the Earth, where they have cuts and sores on their hands, where if they are scrabbling with their hands in the bodies or the brain tissue, their inoculating themselves through open wounds or they've been handling a bit of material and they rub their eyes, and it's coming in through the mucus membrane, or they're picking their noses and and it's going in through the mucus membrane there. They believe it was self inoculation. it's inoculation through some way getting into the blood stream.

Joe Gibbs : And they had multiple, multiple, multiple, routes of inoculation. Lesions on their skin, in their eyes, in their mouths, and other lesions caused by puni grass cuts and by leeches on their bodies and so forth, mosquito bites. All these provided a ample means of infectivity.

Richard Uridge : Put simply, these experiments suggest that primates are infected while eating, but not by eating. In other words by inoculation rather than by ingestion. But "so what if it does?", says Dr Ros Ridley, of the School of Vetinary Medicine in Cambridge.

Ros Ridley : Whether the infectious material got in through cuts in the skin, cuts in the mouth, perhaps with bad teeth or whatever, or whether it was because they swallowed it, is, in a sense, immaterial. They contaminated each other with the brains of their dead relatives by handling that material. Now if we look across now to the new variant CJD and we look at those people who have become infected, they are absolutely, perfectly normal people leading perfectly normal lives in Britain.They have not inoculated themselves with some strange material. They don't have strange professions. They don't work in abattoirs, or they don't dissect animals up, or anything of that sort. They're just ordinary people. What they have done,o f course,i s to eat beef in many forms, not just the recognisable meat form, but prepared foods of various types, everything from sausages onwards, potentially contains beef from various parts of the carcass, and so in their case, the likelihood that it is eating, rather than some other means of introducing bovine material into the body, seems overwhelming, because they are such ordinary people.

Richard Uridge : In the course of making this programme, we discovered that despite the proclaimed ordinariness of its victims, an abattoir worker, Jason Keat, has died of new variant CJD, and if transmission is through blood, as we have already heard that some scientists believe, could vaccines or or other medicines containing things like calf serum, have played a part? The year long BSE enquiry which was due to have reported its findings next month, has looked into that possibility, and sections of the food industry have perhaps naturally, been keen to cast doubt on the infected meat theory. So could it have been vaccines? Professor Robert Will heads Britain's CJD Surveillance unit based in Edinburgh.

Robert Will : I personally believe that's highly unlikely, because if there was any infectivity in calf serum, it will have been at a very,very low level.

[People are worried about the very very low level of microwaves in cell phones which may be producing brain tumours. Level is not the question. In principle is the question. As Chaos theory shows, small things can have big effects. So how low the level is may not be relevant. Although longer time exposure and power level may be relevant in cell phones, so maybe level is important - LB]

Whereas I think that we know there probably was a high level of exposure orally to BSE infection through the food chain.T he other issue is that when vaccines are given, they are only given in a very tiny dosage, once, and so the level of exposure, as far as I'm concerned is likely to be minute, compared to other types of exposure that almost certainly did take place. There is further concern, however, about the issue of blood and perhaps surgical instruments. As far as I'm concerned from the evidence we have on classical CJD, I don't think there is any good evidence that this form of CJD has been transmitted through blood or blood products. We can't be absolutely certain about that, which is why more work is being done, epidemiologically, and also to do with transmission studies. However, new variant CJD might be different, and the reason for that is that I believe it's due to a different infectious agent, and we have evidence from Dr Ironside's work that the tissue distribution of infectivity in the body foe example, in the lymph nodes and spleen, is likely to be much higher in new variant CJD than it is in the ordinary form of CJD, and that does raise legitimate concerns about the possibility of blood or blood products resulting in on ward transmission of new variant CJD from an individual who is incubating it to someone who receives the product, and for that reason, although there are enormous uncertainties about the type of risk

[Ref:Video:BB14:RI 3; N30:The Numbers Game;OB4 Equinox {Living Dangerously}; Protext Files; Green File Mindfld.wri;Red File: Reith992.wri],

that's involved, or the level of risk, a number of actions have been taken, for example in the UK plasma products, that are derived from blood, will be sourced from plasma... derived from outside the UK, from the middle of this year, and there's also plans to remove the white cells from all blood donations throughout the UK. So, I think, although this is a theoretical risk of onward transmission, it's something that has been taken very seriously, and action has been taken.

Room for a Moo?

Rural crisis: Broadbottom farmer David Gould says life is near to impossible for dairy farmers in the wake of the BSE problems. Economics dictate he can't keep calfs like this one.

A FARMER hit hard by the rural crisis is giving away his cows to anyone who can offer them a good home. Broadbottom farmer David Gould says plummeting market prices are making life near impossible for dairy farmers. Prices have dropped so low that some farmers routinely shoot calves dead just after birth in an attempt to stem spiralling losses. Farmer Gould refuses to do this but says such a 'hand to mouth existence' cannot go on without the help of some Good Samaritans. So he's now calling on anyone with enough spare land to give him a helping hand and adopt 'a farm-yard friend'. Economics "The economics of the situation leads to some farmers shooting them - but I can't do that," he said. "If there are any readers out there who can take one or more off my hands and give them a good home I would be willing to do it. "You don't necessarily have to be a farmer to look after a cow but someone with a smallholding with a couple of acres of land." The Little Hill Farm owner says the number of farms with cows has dropped to a paltry 10 locally. But such a situation - which sees a farmer collect less than nine pence per pint of milk - has arisen due to the strength of the pound and the BSE crisis. "The powers that be say the prices are to do with the strength of the pound," he said. "But as far as I know we don't export milk, or if we do it must be very little." Betrayed He added: "The BSE crisis has been the main factor though and we do feel betrayed by the way the government handled the whole issue. "It's the government's fault in the first place for relaxing the sterilisation process. "Through all the years we were punished but the farmers themselves never let their standards slip." If you have the resources to 'home a cow', contact The Advertiser on 0161 339 7611 and we will pass on your details. Mark Travis [It's nice to know that some of our "countryside guardians" look after their animals so well that they don't shoot them in cold blood because they don't turn a profit. Well done farmer Gould for putting life before money -LB]

A FARMER who will give away bull calves to a caring home has had several offers to" help - including sponsorship from the Hindu community. The Advertiser reported how Broadbottom farmer David Gould is offering to take a novel approach to falling market prices. Farmers countrywide face prices which have dropped so low that some consider shooting calves dead just after birth in attempt to stem spiralling losses Farmer Gould won't do that and instead is proposing to hand over any bull calf to someone who can offer a suitable home. We have already had a number of offers for help from a few unlikely sources. A proposed urban farm and a land owner have said they wish to help by taking them off David's hands. David is especially grateful to those offering help because another imminent calving spell is set to signal new additions in Broadbottom. "We have none left now but we will start calving next week and if it's practical for them we hope to do something soon in the coming weeks," he said. "We will have to think through what is best for the cows but we are definitely keen for others to come forward if they wish." Sponsor Ashton man Vishnu Mohandas proposed sponsoring the calves, a scheme which he and his friends have already done at a Sanctuary in Watford. "I read this in the paper and was very pleased with how Mr Gould is handling the situation Mr Mohandas said . "I have sent money to India for a similar scheme while I, and many others nearby, have contributed money to a sponsorship scheme in Watford. Anyone interested in adopting a bull calf must have plenty of land. [The Advertiser 27/9/2000]

MP speaks out

MP Andrew Bennett has slammed farmers involved in recent petrol protests - saying "they've got a cheek". Mr Bennett described last week's crisis as very sad, adding that farmers got their fuel virtually for free. The MP went on to reveal his thoughts about the haulage industry and how, in order for the majority of lorry drivers to he satisfied, there would have to he cuts. "We've got a third more lorry drivers than we need - there should be some restructuring,"he said. "I appreciate many are self-employed and buy vehicles by mortgaging their houses, but they shouldn't take it out on the general public,it doesn't help." Mr Bennett says motorists should consider smaller cars and recommended working and shopping locally.

[Whilst I agree with Andrew,and the Farmers and hauliers have no right to break the rule of law "for the benefit of the country" we've probably got a third too many politicians too,and they're probably paid a third too much -LB]

Richard Uridge : The process of leuko - depletion - filtering out white cells, in which the infective agent is thought to be more concentrated, won't start until November. But the Department of Health told us that the decision to source plasma from countries without BSE came into effect just last week. Then they told us that the imports are coming from the US, where as we've been hearing, there are growing concerns that CJD is spreading to much younger people than normal, 27 in at least one case. but Professor Will says there's no evidence of a new variant of CJD that can't be traced to Britain, and anyway these are only precautions against a theoretical risk. Alan Dickinson agrees, although he did find the infective agent of the sheep brain disease Scrapie, in blood during his research.

Alan Dickinson : You're not dealing with solid tissues, you're dealing with serum and blood which can have small amounts of infectivity. I think I was the first person to show that. I regarded finding it on a small proportion of occasions , in heart, blood, as really an index that my crude method of extracting blood from a heart, was sufficient to incorporate quite a lot of heart tissue and blood cell lining tissue. I'm sure I was doing it far more crudely, and one would hope that more is known now about this..... you can hear me perhaps doubting this, more is recognised and appreciated about the ease of cross contamination of these kinds of products when people think they are dealing in clean room situations with safety cabinets. These words are dangerous in their own right.

Richard Uridge : There are other doubts about the official British position on CJD, and again they arise from the investigations into kuru, in the Foray tribe of Papua New Guinea, and later work by Joe Gibbs. Essentially the question boils down to how new is new variant CJD? Twenty years ago a neurosurgeon died in Boston, apparently of sporadic CJD, but as Joe Gibbs explains, later examination of his brain, revealed the classic symptoms of the new variant.

Joe Gibbs : I maintain that the so-called florid or daisy plaques that they refer to in new variant, occurs outside of the UK in cases, and I've shown them in kuru, chronic wasting disease of deer and elk are massive plaques with vaccules around them, and then the paediatric neurosurgeon in Boston who died. he was first diagnosed has having a very rare skin disease called Almeyer Dago's Disease, and that disease he did have, but then he started to develop neurological signs, and ultimately died and we received the autopsy tissues on him. We demonstrated that he had the prion protein in brain and in lung tissue and it turned out to be the very clear sporadic type of CJD. Now my argument here is that, I'm not sure what are the restrictive clinical and pathological characteristics of so-called new variant, because I can show a number of these things in sporadic cases. I can show people that have gone for psychiatric evaluation, then they developed ataxia and had an extended period of clinical progressive disease over a period of 18 months - 2 years, but they turned out not to have new variant,according to the UK description.

Richard Uridge : British CJD experts have also examined the Boston neurosurgeon's brain tissue. Professor Robert Will believes Joe Gibb's theory's based on a misunderstanding.

Robert Will : Well I think there's a whole wealth of evidence now, that suggest that new variant CJD is a new disease. For example, when the original paper was published in April 1996, one of the things we put in that paper, was the importance of reviewing other neuropathological information from the past, in other countries, to see whether this disease had been seen before elsewhere, and since then we have not been informed or been able to identify, any other case which fulfils the neuropathological appearances of new variant CJD. Now I think there's a slight misunderstanding here, because although florid plaques are a very important component of the diagnosis, neuropathologically, the fact that there is a single or one or two, florid plaques,i s not the issue. The issue is that these plaques are widely distributed throughout the brain. So it is true that occasionally, a type of florid plaque has been seen other cases, you know single or one or two florid plaques, for example, in the case you mentioned, of the neurosurgeon, and also in another case, with dura mater related CJD, but these cases have been reviewed by Dr Ironside and by other neuropathologists, and the view I think, is clearly that the occasional florid plaque does happen sometimes, but the overall neuropathological appearances do not look at all like new variant CJD, and the general consensus among the neuropathological community is, according to my understanding, that new variant CJD is indeed a new disease.

Richard Uridge : Another of the commonly held beliefs about BSE concerns the role played by the animal feed industry. However unsavoury it sounds, renderers turned animals carcasses into animal feed. Sheep were fed to cows, and cows were fed to cows.

[Then if the farming community was decimated they have only their greedy exploitative selves to blame -LB]

Everything went into the pot and was cooked to produce meat and bone meal, then the temperature of the whole process was reduced, ostensibly to save on fuel, and the assumption is it was no longer hot enough to kill off the infective agent. More recent experiments have shown that's probably not true, because even the higher temperature doesn't work. Only one condition does. It's called "pressure cooking", and is now widely used in mainland Europe. Paul Foxcroft is Sales Director of the rendering company Prospa de Molder. He doesn't dispute that BSE was spread by infected feed, but now thinks a combination of things caused BSE to explode in Britain and only in Britain.

Paul Foxcroft : You've got more, unknown to anybody, more active Scrapie agent in the meat and bone meal, year upon year, as more and more sheep are slaughtered. Perhaps a sudden boost of active Scrapie agents within the meat and bone meal again as if there was an increase in the incidence of Scrapie within the sheep flock. You have more meat and bone meal, more then of that meat and bone meal going into the dairy feed, you then have more of that dairy feed being put into the cow. Now I don't believe that combination of circumstances has existed anywhere else in the world, at that same time. We could be perhaps a little mischievous and raise the possibility of organophosphates as maybe a predisposing factor. There certainly could will be genetic factors in terms of genetic susceptibility, as the gene pool is narrowed if you like, with artificial insemination over the last several decades, where we've perhaps used bulls with a susceptibility, or lack of resistance, whichever way you want to term it, into the herd... national herd. Therefore, it's almost a lottery, we've hit this horrendous jackpot, at least we got all the numbers. There are plenty of other countries out there that have perhaps only got 1, 2, or 3, or 4 of the numbers and for that they get nothing.

Richard Uridge : So why are the numbers coming up in America now? What special combination of circumstances exists across the Atlantic? Michael Hanson of the US Consumers Association believes his governments rules about what goes into animal feed are far too lax, and that they just aren't looking hard enough into the problem of CJD.

Michael Hanson : There is the problem of "if you don't look, you don't find". They tend to be concentrating on supposedly looking for a British style BSE case, they're not focusing on intensively sampling animals that might be at risk. So the brain chemistry, the neuropathology that they're looking at, they're looking for the very obvious signs, like you see in BSE cattle in Britain. They're not looking for more subtle signs, and even though there is a quotes "surveillance system", we think it's highly flawed, and while there's no direct evidence, I think there's indirect evidence that there might be a TSE existing in food animals, in the US.

Richard Uridge : Andrew Kimbrel, the lawyer from the Centre for Food Safety, who told us that America was having its own BSE crisis has a shopping list for his government.

Andrew Kimbrel : We just filed a formal legal petition saying "make CJD a reportable disease" which it is not in this country,e ven though we've seen explosions of cases, for goodness sake "stop feeding ruminants to ruminants". We're asking exactly for the UK regulations on feeding animals to animals, which we don't have in this country. "Blood Supply", and an investigation on why in the world our wildlife is coming down with TSEs, at such an alarming rate.

Richard Uridge : So what are we to make of the developments in America? And what can they tell Britain about its pillars of conventional wisdom on mad cows and CJD? I went to see the man who advises the British government on these issues, the chairman of the Spungiform Encephelopathy Advisory Committee or CEAC, Professor Sir John Patterson. I asked him first about the cases of CJD in younger people, and the claims that America now has a new variant of the disease.

John Patterson : It's very difficult to comment on that usefully. All my experience on the UK CEAC tells me that you really need to know all the details of any cases or any problems that are being posed before commenting on them. But if it's true that there are some cases of human Spungiform Encephelopathy in unusually young people, then I think now we have some clear definitions of the clinical state of those patients, we have some modern imaging techniques, for doing MRI scans, and most particularly we have some laboratory based biochemical tests that can be applied to material from these patients which should be able to define the situation much more clearly, and I think you need to get all those details first, before judging what the significance might be.

Richard Uridge : But it wouldn't surprise you, or perhaps it would, if there were 40 cases eventually, in America over the next 2-3 years?

John Patterson : Oh I think it would surprise me enormously if that were the case, and that's why one would want to know all the details of any such case, let alone 40, before commenting on it.

Richard Uridge : One of the conventions, with BSE is that it was caused by people ingesting the infected agent... infected meat, yet some experiments carried out by Carlton Gadusek in he 1950 showed that it might actually be through blood contact. Has his work been disregarded in building the current hypothesis?

John Patterson : No, not at all, I mean it's clearly recognised that there are a number of ways of transmitting Spungiform Encephelopathies.You refer of course, to the peripheral route of inoculation, blood to blood, and you would have to say that in that context, the most vulnerable people would be those that are occupationally exposed because they work in abattoirs, and to date, there have been no cases of variant CJD in abattoir workers which would link the occupation with the exposure.

Richard Uridge : What about the death of Jason Keat? He was an abattoir worker in his 20s, on his death certificate it says that he died from new variant CJD?

John Patterson : You, you... I mean I .... It may surprise you to know of course that I can't relate individual names to individual cases. It is true that there are one or two people that who've classical CJD who've worked in abattoirs, and if you tell me that Jason Keat was one of those with variant CJD who also worked in an abattoir, then I accept that, but then there 39 other cases, who didn't and it's very difficult to unify those 39 cases with a hypothesis about blood to blood transmission. Whatever his occupation he probably, like the other 39 cases, was a consumer of beef products, which would be the most likely hypothesis for the way in which BSE was contracted.

Richard Uridge : You don't think 1 in 40 or 1 in 41 is significant then?

John Patterson : Er no, I don't think it is, in the same way that one of the cases we know had been a vegetarian for many years, but if you trace back the history of that individual, to the point at which she became a very strict vegetarian, then it's most likely we think, that she was exposed to beef products before she became a strict vegetarian and the occurrence of one case we don't feel necessitates altering our proposal about the most likely route of transmission.

Richard Uridge : But it is just a hypothesis, at this stage. Is it possible that another hypothesis would explain transmission and that is that is that vaccines have somehow played a role?

John Patterson : I think that the CJD surveillance unit and Professor Will in quite a long and extensive questionnaire to the families of each of these cases, and looking all the time for occupational exposure, geographic clustering, medical histories, histories of operations which might unify all these 40 cases, then you cannot find such evidence.

Richard Uridge : And in the absence of any other evidence, the current hypothesis is the best one, in your view?

John Patterson : I think it is, yes.

Richard Uridge : If I can turn to the use of blood and blood products. Is there a concern that BSE or new variant CJD should I say, could be spread through blood products?

John Patterson : Yes, and this is going back a little while now, but of course CEAC made some recommendations to the government about that, particularly in relation to removing the white cells from blood transfusion, and the technique which is called leuko-depletion is being extended to become comprehensive in the UK blood transfusion service as soon as possible.

Richard Uridge : Because the white cells actually carry a greater proportion of the infective agent?

John Patterson : Again the evidence is very incomplete in this, but if you think about it intuitively, and you say "we must apply the precautionary principle", then it seems logical to remove the white cells, as the most likely component of blood which would transmit the infection.

Richard Uridge : Why is it that precautionary measures are being taken only now?

John Patterson : Well it takes quite a long time of course to set some of these processes in train....

Richard Uridge : That's 3 years potentially in which people could have been infected person to person.

John Patterson : There is a possibility that if variant CJD is transmissible by blood or blood products, and that some of the cases we know have been blood donors, that it might have been transfused from one individual to another, that's exactly the sort of eventuality that one is trying to prevent, from people who are currently incubating variant CJD, but haven't actually manifested it, yet. That's why those precautions are being taken.

Richard Uridge : Might it not have been more sensible to take that precautionary measure at the outset? It didn't seem that it was a problem taking precautionary measures as far as the beef industry was concerned quickly, so why not the medical industry?

John Patterson : I think that's exactly right, because of course the proposition was that the source of cases of variant CJD was oral exposure to beef products which contained some of the specified offals, most notably brain and spinal chord. So it was important really to shut that door as quickly as possible. There was then a need to look at the evidence about the transmission of classical CJD via blood transfusion,and there the evidence is conflicting, and by and large there, the conclusion has to be that classical sporadic CJD is only very rarely transmitted by blood transfusion, if at all. However, it then became clear that variant CJD was behaving differently, particularly in relation to the phase of the infection in humans, in the lymphoid tissues. You can find the agent of variant CJD in the spleen and in the tonsils and in the lymphoid tissue in the appendix,and so one had to recognise that it was possible that circulating lymphocytes also contained this agent. There was also a developing body of knowledge about animal experimentation using mice, which led one, taking all this into account, to recommend to the government that leuko-depletion would be a sensible precautionary measure. Now that all took time to develop, time to think about and then of course, once you've made the decision, time to implement. So that's where you get the apparent delay from.

Richard Uridge : Finally, are we now any nearer to breaking the deafening silence on the crucial question? How many more people will die from variant CJD in Britain? We've seen estimates prepared for the Royal Society, based on the number of cases so far. Depending on how you interpret them, the projections are either good news or chilling reading. They indicate there could be as few as a dozen or so more deaths,or as many as 13 million.

John Patterson : That was one of the major questions that was asked immediately on March 20th 1996, and the answer then was that there was a very broad range of possibilities. Three years later and fortunately the range of possibilities still remains very wide, and so, to be honest I think the only that we will tell, is to wait for another 2 or 3 or 5 years before we can answer... we can narrow down that range usefully for you.


http://www.fortunecity.com/emachines/e11/86/bse.html



http://www.radiolistings.co.uk/candc/uridge_richard.html



http://www.radiolistings.co.uk/programmes/bse___the_untold_story.html



Wednesday, May 19, 1999 Published at 06:45 GMT 07:45 UK

Sci/Tech

BSE fears over US blood imports

But a BBC programme says some experts fear a new kind of Creutzfeld-Jakob Disease (CJD), the human equivalent of BSE, is killing young Americans.

The programme, "BSE - The Untold Story", was broadcast on Radio 4.

CJD is normally a disease of elderly people, but the type linked to eating the meat of BSE cattle - new variant CJD - attacks young patients.

CJD is not a notifiable disease in the USA, so there are no accurate figures on the number of cases or the ages of patients.

A Washington lawyer, Andrew Kimbrell, told the programme: " We've seen explosions of cases.We're facing what the UK faced a few years ago".

"Is it a time bomb? Is it just something that a few people seem susceptible to?"

http://news.bbc.co.uk/2/hi/science/nature/347137.stm



MADCOW USDA the untold story

http://madcowusda.blogspot.com/



NOW, remember what was said about the FIRST TEN, i.e., what i call the chosen ones ;

Lancet 1996; 347: 921- 25

A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith

Summary

snip...

Discussion

The ten cases of CJD in this report are remarkable in that they have a specific neuropathological profile which, to our knowledge, has not been described previously[6,8] and which is so consistent that neuropathological samples from the cases are virtually indistinguishable. The cases are further characterised by having remarkably low ages at onset for CJD and other atypical features, including a generally protracted and unusual clinical course and absence of EEG changes typical of CJD. These findings raise the possibility that the cases represent a new clinicopathological variant of CJD.

Effect of age

It is possible that the unusual neuropathological profile of these cases is due to their young age. Review of published reports on previous young patients worldwide did not reveal any descriptions of neuropathology similar to these UK cases. In 14 cases of CJD aged less than 30 years previously reported outside the UK, plaques are described in only one, and in this report the possible diagnosis of Gerstmann- Straussler- Scheinker syndrome was raised. In four of these cases,[9-12] pathological reports have been reviewed and there was no evidence of PrP plaques (Paul Brown, personal communication). We did immunocytochemical staining on another of these cases of CJD aged 27 years from Poland (courtesy of Professor Kulczycki) and on a 16- year- old patient from the UK dying of CJD in 1980, and there was no evidence of plaque formation in either case. We also did immunocytochemical staining on 11 cases of CJD developing after administration of human growth hormone (mean age 27.5 years) and although PrP plaques were present predominantly in the cerebellum, the neuropathological features in these cases'3 were otherwise quite distinct from the young patients in this report. We emphasise that plaque distribution and spongiform change in these ten young cases were clearly apparent on routine light microscopy. Current evidence suggests, therefore, that the pathological profile in these cases is unlikely to be simply an age- related feature.

CJD has been described previously in young patients, but these are usually isolated case reports[9-12] and in systematic surveys the identification of CJD in patients aged less than 30 years old is exceptional. In the UK, only one such case was identified between 1970 and 1989. In France, between 1968 and 1982[14], only two patients aged less than 30 years old were identified; only one was identified in Japan between 1975 and 1977; and none at all in Israel between 1963 and 1987. Additional cases aged less than 40 years have been identified through the European surveillance project on CJD (1993- 95); two cases aged 22 and 34 years old were found in the Netherlands; two aged 31 and 33 years old in Germany; two aged 26 and 37 years old in France; and one aged 37 years old in Italy. Six of these cases are judged on clinical evidence not to be similar to the cases described in this report. Neuropathological information is available on two of these six cases, neither of which showed the characteristic changes. In the remaining case, full neuropathological information will be available shortly.

Case ascertainment

The overall incidence of CJD has risen in the UK in the 1990s[15], although this is due mainly to an increase in the incidence of CJD in those aged over 75 years (these cases have a typical clinicopathological profile). The most likely explanation for this is improved ascertainment of CJD in the elderly, with the possible implication that the identification of young cases of CJD may be due to similar improved case ascertainment due in part to the publicity surrounding the BSE epidemic. It is noteworthy that three of the ten cases in this report were notified to the CJD Surveillance Unit as suspect cases of CJD only after biopsy samples had been examined. In the absence of neuropathological examination, these cases might not have come to the attention of the CJD Surveillance Unit. It seems likely, however, that patients of this age dying of a progressive neurological condition would have undergone necropsy in the past. Two cases came to the attention of the CJD Surveillance Unit through unconventional means (through a newspaper report and after a clinical presentation of other cases) which led to their notification earlier than would otherwise have been the case. All of the ten cases were identified over 10 months and although there was extensive publicity surrounding two young cases in late 1995, there has been considerable publicity regarding CJD and BSE since 1990. Other European countries have undertaken systematic surveillance of CJD over a similar period and there has been no obvious increase in the incidence of CJD in young patients despite detailed investigation.

There is a possibility that the diagnosis of such atypical cases may previously have been previously missed. Three of the 14 cases discussed above were from Poland, aged 19, 23, and 27 years, and were identified in the course of a study of subacute sclerosing panencephalitis (SSPE)[16] A recent review of the clinical details of suspect but unconfirmed cases of SSPE held by the SSPE register in the UK has provided no evidence that cases of CJD were misdiagnosed as SSPE in the UK. Although improved ascertainment remains a potential explanation for the identification of the young patients we report, such information as is available does not support this interpretation.

Possible link with BSE

The first aim of the CJD Surveillance Unit has been to identify any changes in CJD that might be attributable to the transmission of BSE to the human population. Although the small number of cases in this report cannot be regarded as proof, the observation of a potentially new form of CJD in the UK is consistent with such a link. The common neuropathological picture may indicate infection by a common strain of the causative agent, as in sheep scrapie in which strains of the disease have been identified which can be distinguished on the basis of diseaseincubation period and distinctive neuropathological profile in mouse models[17]. Exposure of the human population to the BSE agent is likely to have been greatest in the 1980s, and especially towards the end of that decade, before the ban on the use of specified bovine offal was introduced. This would be consistent with an incubation period of between 5 and 10 years for these cases.

If the present cases are due to exposure to the BSE agent and this accounts for the distinctive neuropathological appearance, it is not clear why this previously unrecognised variant of the disease has been found only in persons under the age of 45 years. The absence of this variant in older persons could be due to age- related exposure to the agent; to reduced susceptibility among older persons; or to misdiagnosis of this variant of the disease in older age- groups, especially in those in which dementia is more common.

We were alerted earlier to a possible link between CJD and BSE by our finding of an apparent excess of CJD among cattle farmers[15]. Our interpretation of this was tempered by observations of high rates among cattle farmers in other European countries in which BSE was either very rare or had not been reported. None of the four farmers showed the neuropathological features described here, and all were consistent with previous experience of sporadic cases of CJD.

Conclusions

We believe that our observation of a previously unrecognised variant of CJD occurring, to date, only in persons under the age of 45 years is a cause for great concern. That it is due to exposure to the BSE agent is perhaps the most plausible interpretation of our findings. However, we emphasise that we do not have direct evidence of such a link and other explanations are possible. That these cases have been observed now because of improved ascertainment cannot be completely dismissed. It seems unlikely, however, that such a distinctive neuropathological pattern would have been missed previously, especially among persons dying at a young age. It is essential to obtain information on the clinical and neuropathological characteristics of young patients with CJD in Europe and elsewhere, and historically in the UK, but proof of an association between BSE and CJD may depend on animal transmission studies and continued epidemiological vigilance. If there is a causal link then, given the potentially long and widespread exposure to the BSE agent, further cases of this new variant of CJD are likely to arise.

We thank J Mackenzie for data management, P Brown for reviewing an early version of the manuscript, J Collinge for assistance with the molecular analysis, and W B Matthews who initiated CJD surveillance in the UK in the 1980 for advice. The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home and Health Department and suported by BBSRC (grant no 15/BS204814). The Concerted Action on CJD Surveillance in Europe was funded through the EC Biomed I Programme. The epidemiological surveillance of CJD would not be possible without the collaboration of neurologists and neuropathologists throughout the UK and Europe.

References

snip.....

http://www.cjd.ed.ac.uk/lancet.htm



sporadic CJD, the big lie

Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD, the big lie) Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk.

Accepted 15 April 2007

Abstract

Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1



Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.

POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie OCCUPATIONAL EXPOSURE

Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

snip...

see full text sporadic CJD the big lie;

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276



Thursday, July 10, 2008

A New Prionopathy, or the same old BSe

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html



Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

Saturday, July, 18, 2009

Greetings,

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. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.

The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.

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. 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. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

please see history, and the ever evolving TSE science to date ;

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



SEE THE DAMNING VIDEO NOW AT THE BOTTOM OF THE BLOG BELOW ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html



Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie. Emerg Infect Dis. 2009 Aug; [Epub ahead of print]

http://nor-98.blogspot.com/2009/07/transgenic-mice-expressing-porcine.html



Transmissible mink encephalopathy - review of the etiology

http://transmissible-mink-encephalopathy.blogspot.com/2009/07/transmissible-mink-encephalopathy.html



Wednesday, July 1, 2009

Nor98 scrapie identified in the United States J Vet Diagn Invest 21:454-463 (2009)

http://nor-98.blogspot.com/2009/07/nor98-scrapie-identified-in-united.html



Monday, June 01, 2009 Biochemical typing of pathological prion protein in aging cattle with BSE

SOMETHING TO PONDER ???

O.K. confusious asks, IF all these new atypical BSEs i.e. new strains of mad cow disease is just an 'OLD COW PRION DISEASE', why then can not the 'old human prion disease' such as the sporadic CJD, be from an 'old cow prion disease', same as the nvCJD 'young people mad cow disease' (which also happens in 74 year old), but why cannot the 'old cow prion diseases', i.e. l-BSE, h-BSE, and ibncBSE, cause the 'old people prion disease', which looks like sporadic CJD. seems that is what some of the pathology is showing ???

OH, that probably makes too much sense, and that the only answer could be that it's all just a happenstance of bad luck and or a spontaneous event, that just happens out of the clear blue sky $$$

IF this is the case, then where are all the SPONTANEOUS BSE CASES OF MAD COW DISEASE IN THE U.S.A., AND WHERE HAVE THEY BEEN BURIED IN THE USA OVER THE LAST 25 YEARS ???

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html



Tuesday, August 04, 2009

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/08/susceptibilities-of-nonhuman-primates.html



Thursday, July 23, 2009

UW Hospital warning 53 patients about possible exposure to rare brain disease

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html



Wednesday, August 05, 2009 Rate of CWD infection increases in core area WISCONSIN

http://chronic-wasting-disease.blogspot.com/2009/08/rate-of-cwd-infection-increases-in-core.html



Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



2009


NOW, let's analyze some data since 1999. ...TSS

Sunday, August 3, 2008 The conference celebrating ‘The end of kuru: 50 years of research into an extraordinary disease’

1: Related Articles Collinge J, Alpers MP.

Introduction. Philos Trans R Soc Lond B Biol Sci. 2008 Jul 31. [Epub ahead of print] No abstract available. PMID: 18672464 [PubMed - as supplied by publisher]

http://journals.royalsociety.org/content/j104810238060l8w/fulltext.pdf



In our field studies, we have interviewed many individuals who participated in traditional mortuary feasting or who described the participation of family members from the preceding generation. These detailed descriptions will be published elsewhere but have reaffirmed the oral histories of endocannibalism in the Fore recorded previously12,22–24 and that this practice ceased abruptly at the time of Australian administrative control over the kuru areas. Although isolated events might have occurred for a few years after this prohibition, we are confident that new exposures of individuals to kuru at mortuary feasts would not have occurred after 1960. Not only have no cases of kuru been recorded in people born after 1959 (and only nine were recorded in those born after 1956); but also all the 11 last recorded cases of kuru that we report here were born before 1950. If any source of infection remained, whether from surreptitious cannibalism, possible ground contam-ination with human prions at sites where food was prepared, or other lateral routes, we would expect individuals born after this period to have kuru—especially since children are thought to have had shorter incubation periods than adults. However, no such cases have been observed. Additionally, although a fraction of hamster-adapted scrapie prions have been shown to survive in soil for at least 3 years,25 the mortuary feast practices (during which the entire body would be consumed) were undertaken so that any substantial contamination of soil would not have occurred, and traditional bamboo knives and leaf plates were burned after the feast.

snip...see full text ;

http://kuru-tse.blogspot.com/2008/08/conference-celebrating-end-of-kuru-50.html



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

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

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404

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



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

snip...

A The Present Position with respect to Scrapie

A1 The Problem

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

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

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

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

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

snip...

76/10.12/4.6

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



Epidemiology of Scrapie in the United States 1977

http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf



http://scrapie-usa.blogspot.com/



Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284

FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in ...

http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html




Nor98 scrapie identified in the United States

J Vet Diagn Invest 21:454-463 (2009)

Christie M. Loiacono,1 Bruce V. Thomsen, S. Mark Hall, Matti Kiupel, Diane Sutton, Katherine O'Rourke, Bradd Barr, Lucy Anthenill, Delwyn Keane

Abstract.

A distinct strain of scrapie identified in sheep of Norway in 1998 has since been identified in numerous countries throughout Europe. The disease is known as Nor98 or Nor98-like scrapie, among other names. Distinctions between classic scrapie and Nor98 scrapie are made based on histopathology and immunodiagnostic results. There are also differences in the epidemiology, typical signalment, and likelihood of clinical signs being observed. In addition, sheep that have genotypes associated with resistance to classic scrapie are not spared from Nor98 disease. The various differences between classic and Nor98 scrapie have been consistently reported in the vast majority of cases described across Europe. The current study describes in detail the pathologic changes and diagnostic results of the first 6 cases of Nor98 scrapie disease diagnosed in sheep of the United States.

Key words: Histopathology; Nor98; PrP immunolabeling; scrapie; sheep.

snip...

The current study describes the diagnostic findings of the first 6 cases of Nor98 scrapie in sheep of the United States.

snip...

Results

Case 1

snip...see full text ;

Wednesday, July 1, 2009

Nor98 scrapie identified in the United States J Vet Diagn Invest 21:454-463 (2009)

http://nor-98.blogspot.com/2009/07/nor98-scrapie-identified-in-united.html



http://nor-98.blogspot.com/



http://scrapie-usa.blogspot.com/



Sunday, May 18, 2008

MAD COW DISEASE BSE CJD CHILDREN VACCINES

http://bseinquiry.blogspot.com/2008/05/mad-cow-disease-bse-cjd-children.html



Monday, August 17, 2009 Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.html



Monday, May 19, 2008 S

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS

http://bseinquiry.blogspot.com/2008/05/sporadic-cjd-in-farmers-farmers-wives.html



Sunday, May 18, 2008

BSE, CJD, and Baby foods (the great debate 1999 to 2005)

http://bseinquiry.blogspot.com/2008/05/bse-cjd-and-baby-foods-great-debate.html



Sunday, May 18, 2008

BSE Inquiry DRAFT FACTUAL ACCOUNT DFA

http://bseinquiry.blogspot.com/2008/05/bse-inquiry-draft-factual-account-dfa.html



Monday, August 17, 2009

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.html



PRODUCT Red Blood Cells Leukocytes Reduced. Recall # B-1148-09 CODE Unit: 3291680 RECALLING FIRM/MANUFACTURER Florida's Blood Centers, Inc, Orlando, FL, by telephone on January 12, 2009 and by letter dated January 13, 2009. Firm initiated recall is complete. REASON Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION FL

___________________________________


PRODUCT 1) Recovered Plasma. Recall # B-1151-09; 2) Red Blood Cells Leukocytes Reduced. Recall # B-1152-09 CODE 1) and 2) Unit: 6585642 RECALLING FIRM/MANUFACTURER Carter BloodCare/ WE & Lela I Stewart Blood Center, Inc, Tyler, TX, by fax on April 23, 2007 and March 11, 2009. Firm initiated recall is complete. REASON Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TX and Switzerland

___________________________________


END OF ENFORCEMENT REPORT FOR JULY 1, 2009

#

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm170185.htm



PRODUCT 1) Red Blood Cells, Leukocytes Reduced. Recall B-1180-09; 2) Recovered Plasma. Recall # B-1181-09 CODE 1) and 2) Unit: 27LT64128 RECALLING FIRM/MANUFACTURER ARC Greater Alleghenies, Johnstown, PA, by telephone or electronic notification on December 1, 2008 and by letter dated December 3, 2008. Firm initiated recall is complete. REASON Blood products, collected from a donor who may have been at risk for Creutzfeldt-Jakob disease (CJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION CA, WV

___________________________________


PRODUCT 1) Recovered Plasma. Recall # B-1214-09; 2) Red Blood Cells. Recall # B-1215-09 CODE 1) and 2) Unit: KS25920 RECALLING FIRM/MANUFACTURER Inova Health Care Services, Blood Donor Services, Sterling, VA, by letter dated November 23, 2007. Firm initiated recall is complete. REASON Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION VA, NJ

___________________________________


http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm170893.htm



PRODUCT Source Plasma. Recall # B-1206-09 CODE Units: 07YARF0702, 07YARE9866, 07YARE9175, 07YARE8806, 07YARE6013, 07YARE6743, 07YARE7284, 07YARE3054, 07YARE1421, 07YARE1044, 07YARE0168, 07YARD9701, 07YARD8977, 07YARD8152, 07YARD7695, 07YARD6945, 07YARD6722, 07YARD5923, 06YARE3812, 06YARE4248, 06YARE4943, 06YARE5522, 06YARE6898, 06YARE7196, 07YARA0218, 07YARA0575, 07YARA1157, 07YARA1574, 07YARA2145, 07YARA2571, 07YARA5682, 07YARA8317, 07YARA9272, 07YARA9637, 07YARB0583, 07YARB1028, 07YARB1861, 07YARB2231, 07YARB2855, 07YARB3489, 07YARB4656, 07YARB5193, 07YARB5849, 07YARB6358, 07YARB7036, 07YARB7618, 07YARB8311, 07YARB8853, 07YARB9492, 07YARC0024, 07YARC0639, 07YARC1138, 07YARC1799, 07YARC2236, 07YARC3313, 07YARC4218, 07YARC4928, 07YARC5294, 07YARC5924, 07YARC7298, 07YARC8991, 07YARC9252, 07YARD3794, 07YARD4519, 07YARD5590, 07YARF3027

RECALLING FIRM/MANUFACTURER BioLife Plasma Services LP, Fayetteville, AR, by fax on January 14, 2008. Firm initiated recall is complete. REASON Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 66 units DISTRIBUTION CA

PRODUCT 1) Red Blood Cells. Recall # B-1250-09; 2) Fresh Frozen Plasma. Recall # B-1251-09 CODE 1) and 2) Unit: 4054709 RECALLING FIRM/MANUFACTURER Wellmont Health System dba Marsh Regional Blood Center, Kingsport, TN, by letter dated October 4, 2007. Firm initiated recall is complete. REASON Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 2 units DISTRIBUTION TN

___________________________________


PRODUCT Source Plasma. Recall # B-1254-09 CODE Units: 363035924, 363035761, 363034642, 363034463, 363034153, 363033965, 363033670, 363033509, 363033093, 363032543, 363032383, 363032099, 363031873, 363031593, 363031321, 363031001, 363030738, 363030235, 363030057, 363029728, 363028978, 363028657, 363028438, 363028031, 363027863, 363027492, 363027314, 363026918, 363026688, 363026356, 363026123, 363025728, 363025483, 363025047, 363024873, 363024318, 363023960, 363023655, 363023279, 363022828, 363022612, 363020673, 363020392, 363021363, 363021144, 363019633, 363019419, 363018827, 363018647, 363016935, 363016561, 363016170, 363015723, 363014906, 363014413, 363014104, 363013235, 363012843, 363012402, 363011999, 363011601, 363011195, 363008854, 363008420, 363008054, 363007611, 363007268, 363006814, 363006486, 363006074, 363005753, 363005310, 363004961, 363004496, 363004188, 363003610, 363000147, I74029391, I74028830, I74028628, I74025361, I74024797, I74024560, I74024220, I74024002, I74023653, I74023404, I74023044, I74022854, I74022445, I74022288, I74021933, I74021763, I74021452, I74021274, I74020724, I74019586, I74025147, 363015212 RECALLING FIRM/MANUFACTURER Talecris Plasma Resources Inc, Fort Worth, TX, by facsimile on March 13, 2007. Firm initiated recall is complete. REASON Blood products, collected from a donor who was at risk for Creutzfeldt-Jakob Disease (CJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 99 units DISTRIBUTION NC

___________________________________


END OF ENFORCEMENT REPORT FOR JULY 22, 2009

###

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm173256.htm



PRODUCT 1) Red Blood Cells Leukocytes Reduced. Recall # B-1281-09; 2) Platelets Leukocytes Reduced. Recall # B-1282-09; 3) Recovered Plasma. Recall # B-1283-09 CODE 1) Units: 1974540, W044108025910; 2) Unit: W044108025910; 3) Units: W044108025910, 1974540 RECALLING FIRM/MANUFACTURER Siouxland Community Blood Bank, Sioux City, IA, by letter and email on February 3, 2009. Firm initiated recall is complete. REASON Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 5 units DISTRIBUTION Austria, IA

___________________________________


END OF ENFORCEMENT REPORT FOR JULY 29, 2009

###

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm174738.htm



2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html



note that some states only make cjd reportable in <> THAT HAVE THOSE PROCEDURES............sorry, i get angry at all the stupidity, and greed, because all the ignorance is born from the fear of $$$ with TSEs and ramifications of a link with livestock producing animals. for petes sake, to date, all iCJD cases have been from sporadic CJD strains. that's all iCJD is, is sporadic CJD, until a route and source found, but the pathology is the same. except for those 4 from nvCJD blood.

vCJD-related abnormal prion protein in a person with haemophilia - an update

Following the finding of evidence of the abnormal prion protein that causes variant Creutzfeldt-Jakob Disease (vCJD) in a haemophilia patient at post mortem, a risk assessment of the possible routes of exposure for this individual has been published by the Department of Health [1].

The vCJD-related abnormal prion protein was detected only in the individual's spleen but the finding was the first of the agent in a haemophilia patient [2].

To date, no haemophilia or bleeding disorder patients have been diagnosed with or died from clinical vCJD.

The investigations into the possible routes of infection considered, assuming that the abnormal prion protein did indicate vCJD infection, four possible infection routes: dietary exposure to BSE; surgical procedures; transfusions with several units of red cells; and treatment with large amounts of UK sourced Factor VIII. This included two batches of Factor VIII 8Y that were sourced from plasma pools which included plasma from a single donor who later developed clinical vCJD.

The calculations involved in this risk assessment depend on the likely prevalence of subclinical vCJD infections among the UK population, the infectivity of plasma products and blood components - both of which are subject to great uncertainties - and the number of donors contributing to the plasma pools. The risk assessment concludes that in scenarios based on current evidence, the most likely source of this patient's infection was treatment with UK sourced clotting factors.

This haemophilia patient had been treated in the 1990s with over 390,000 units of UK-sourced Factor VIII, including over 9,000 units from two implicated batches of Factor VIII 8Y (linked to a donor who later developed clinical vCJD).

The risk assessment concluded that, based on plausible assumptions, the patient was more likely to have been infected by a batch of Factor VIII that was not sourced from a pool containing plasma from the donor known to have vCJD (a non-implicated batch), than by one of the two implicated batches that were linked to this donor.

This is because far more units of non-implicated than implicated batches were administered to this patient. Each batch of plasma product is sourced from many thousands of donors (around 20,000), any one of whom could have had an asymptomatic abnormal prion protein infection. The relative risks from implicated and non-implicated batches will only be clarified further by long-term follow-up of patients.

The CJD Incidents Panel considered the risk assessment, together with other information, and concluded that this evidence confirms its existing advice to patients already notified as at increased risk of vCJD. There is therefore currently no reason to change that advice, nor to notify any new groups of patients. There is no change in the public health vCJD "at risk" status of any patients with bleeding disorders.

All patients with bleeding disorders [3] who have been treated with UK-sourced pooled factor concentrates or antithrombin [4] between 1980 and 2001 [5] are classified as "at risk of vCJD for public health purposes". Special infection control precautions and other safety measures apply to these patients.

All haemophilia centre doctors were informed of the Panel's decision on Tuesday, 9 June, 2009 and were asked to send a letter to their patients who have been notified as at increased risk of vCJD. Further information is available at http://www.hpa.org.uk/vcjdplasmaproducts.

Notes

1. vCJD risk assessment calculations for a patient with multiple routes of exposure. Department of Health, 9 June 2009. Available at:

http://www.dh.gov.uk/en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/DH_100357



2. HPA. Post mortem finding of asymptomatic variant Creutzfeldt-Jakob Disease abnormal prion protein in a person with haemophilia, Health Protection Report [serial online] 2009; 3 (10): news. Available at:

http://www.hpa.org.uk/hpr/archives/2009/news0709.htm#vcjd



3. Defined here as congenital and acquired haemophilia (Haemophilia A and Haemophilia B), Von Willebrand Disease, other congenital bleeding disorders and congenital antithrombin III deficiency.

4. ie clotting factors and antithrombin made from pooled plasma. These include factor VIII, factor IX, factor VII, factor XI, factor XIII and prothrombin complex concentrates as well as antithrombin.

5. The start date of 1980 is when BSE is thought to have entered the human food chain. The end date of 2001 is the last possible expiry date of any product manufactured by the UK fractionators that was sourced from UK donors until 1998.

http://www.hpa.org.uk/hpr/archives/2009/news2309.htm#cjd



Variant CJD and plasma products Introduction

Earlier notification of patients who had received implicated plasma products

Asymptomatic vCJD abnormal prion protein in a haemophilia patient

Introduction Certain plasma products, manufactured using plasma from donors who later developed vCJD, may have exposed people who received them to infectivity and an increased risk of developing vCJD. The level of risk is unknown, and likely to be very low. The risk in such circumstances is in addition to a general risk for many people in the UK from past exposure to the BSE agent from eating beef and beef products.

The conclusions of a vCJD Blood Risk Assessment carried out by Det Norske Vertis Consulting have been accepted by the Spongiform Encephalopathy Advisory Committee (SEAC), the Committee on the Microbiological Safety of Blood and Tissue (now the Advisory Committee on the Safety of Blood Tissues and Organs - SaBTO), and by the Committee on Safety of Medicines.

As a consequence, all patients with bleeding disorders 1 who have been treated with UK-sourced pooled factor concentrates or antithrombin 2 between 1980 and 2001 3 are classified as at risk of vCJD for public health purposes. These patients should follow advice to reduce the risk of spreading vCJD to other patients.

Please also see Information leaflets for patients and healthcare professionals.

Earlier notification of patients who had received implicated plasma products In 2004 the HPA, the UK Haemophilia Centre Doctors' Organisation (UKHCDO) and colleagues notified patients who had received plasma products manufactured using plasma from donors who had subsequently developed vCJD. That notification dealt with plasma donations which had been used to manufacture factor VIII, factor IX, antithrombin, intravenous immunoglobulin G, albumin, intramuscular human normal immunoglobulin and anti-D.

Patients treated with these plasma products were managed according to an assessment of potential vCJD infectivity carried out by the Health Protection Agency with the CJD Incidents Panel.

The UKHCDO and patient representatives, the CJD Incidents Panel and UK Health Departments agreed that it was likely that many patients with bleeding disorders would have had sufficient exposure to these implicated plasma products to put them 'at risk of vCJD for public health purposes'. It was also thought likely that further batches of UK-sourced plasma products would be implicated in the future as more cases of vCJD arose.

In 2004 all patients with bleeding disorders were told whether they had received UK-sourced pooled factor concentrate or antithrombin between 1980 and 2001. Those who had were informed that special precautions needed to be taken to reduce the chance of any further spread of vCJD and were asked to follow public health advice.

Asymptomatic vCJD abnormal prion protein in a haemophilia patient A person with haemophilia was recently found to have evidence of the agent (abnormal prion protein) that causes vCJD only in his spleen at post mortem.

The post mortem was carried out as part of a study jointly co-ordinated by the UK Haemophilia Centre Doctors Organisation and the National CJD Surveillance Unit. This was the first time that vCJD abnormal prion protein had been found in a patient with haemophilia.

Up to the present, no haemophilia or bleeding disorder patient has been diagnosed with or died from clinical vCJD This haemophilia patient had been treated with several batches of UK-sourced clotting factor Factor VIII. This includes two batches of Factor VIII manufactured using plasma from a single donor who developed clinical vCJD 6 months after donating the plasma. These batches are called 'implicated' batches because they are linked to a donor who subsequently developed vCJD.

The haemophilia patient was in his 70s when he died of a condition unrelated to vCJD, 11 years and one month after receiving the second batch of implicated Factor VIII. He had no signs or symptoms of vCJD or other neurological disease when alive.

The patient had also received several transfusions of red cells, and had undergone surgical procedures in the past. A recent statistical assessment of the available information has concluded that the most likely source of vCJD infection was treatment with UK plasma-sourced clotting factors.

The risk assessment, together with other information, has been considered by the CJD Incidents Panel. The Panel has concluded that this evidence confirms its existing advice to patients already notified as at increased risk of vCJD. There is therefore currently no reason to change that advice, or to notify any new groups of patients.

There is no change in the pubic health vCJD 'at risk' status of any patients with bleeding disorders.

The CJD Incidents Panel keeps all new information about potentially infected products under close and regular review and advises doctors and their patients about the implications of all new evidence as it emerges.

Patients who are unsure about their vCJD at risk status, or who would like more information, should contact their haemophilia centre.

1. Defined here as congenital and acquired haemophilia (Haemophilia A and Haemophilia B), Von Willebrand Disease, other congenital bleeding disorders and congenital antithrombin III deficiency. 2. ie. clotting factors and antithrombin made from pooled plasma. These include factor VIII, factor IX, factor VII, factor XI, factor XIII and prothrombin complex concentrates as well as antithrombin. 3. The start date of 1980 is when BSE is thought to have entered the human food chain. The end date of 2001 is the last possible expiry date of any product manufactured by the UK fractionators that was sourced from UK donors until 1998.

Last reviewed: 10 June 2009

http://www.hpa.org.uk/webw/HPAweb&HPAwebStandard/HPAweb_C/1195733818681



Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html



Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

Saturday, July, 18, 2009

Greetings,

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. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.

The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.

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. 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. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

please see history, and the ever evolving TSE science to date ;

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

2003

Neurology 2003;60:176-181 © 2003 American Academy of Neurology

-------------------------------------------------------------------------------- Views & Reviews

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.

Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.

Transmissible spongiform encephalopathies (TSEs) attracted increased attention in the mid-1980s because of the emergence among UK cattle of bovine spongiform encephalopathy (BSE), which has been shown to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD). The BSE outbreak has been reported in 19 European countries, Israel, and Japan, and human cases have so far been identified in four European countries, and more recently in a Canadian resident and a US resident who each lived in Britain during the BSE outbreak. To monitor the occurrence of emerging forms of CJD, such as vCJD, in the United States, the Centers for Disease Control and Prevention has been conducting surveillance for human TSEs through several mechanisms, including the establishment of the National Prion Disease Pathology Surveillance Center. Physicians are encouraged to maintain a high index of suspicion for vCJD and use the free services of the pathology center to assess the neuropathology of clinically diagnosed and suspected cases of CJD or other TSEs.

http://www.neurology.org/cgi/content/abstract/60/2/176



RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176



Reply to Singletary 26 March 2003

Ryan A. Maddox, MPH

Mr. Singletary raises several issues related to current Creutzfeldt- Jakob disease (CJD) surveillance activities. Although CJD is not a notifiable disease in most states, its unique characteristics, particularly its invariably fatal outcome within usually a year of onset, make routine mortality surveillance a useful surrogate for ongoing CJD surveillance.[1] In addition, because CJD is least accurately diagnosed early in the course of illness, notifiable-disease surveillance could be less accurate than, if not duplicative of, current mortality surveillance.[1] However, in states where making CJD officially notifiable would meaningfully facilitate the collection of data to monitor for variant CJD (vCJD) or other emerging prion diseases, CDC encourages the designation of CJD as a notifiable disease.[1] Moreover, CDC encourages physicians to report any diagnosed or suspected CJD cases that may be of special public health importance (e.g., vCJD, iatrogenic CJD, unusual CJD clusters).

As noted in our article, strong evidence is lacking for a causal link between chronic wasting disease (CWD) of deer and elk and human disease,[2] but only limited data seeking such evidence exist. Overall, the previously published case-control studies that have evaluated environmental sources of infection for sporadic CJD have not consistently identified strong evidence for a common risk factor.[3] However, the power of a case-control study to detect a rare cause of CJD is limited, particularly given the relatively small number of subjects generally involved and its long incubation period, which may last for decades. Because only a very small proportion of the US population has been exposed to CWD, a targeted surveillance and investigation of unusual cases or case clusters of prion diseases among persons at increased risk of exposure to CWD is a more efficient approach to detecting the possible transmission of CWD to humans. In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

Mr. Singletary also expresses concern over a recent publication by Asante and colleagues indicating the possibility that some sporadic CJD cases may be attributable to bovine spongiform encephalopathy (BSE).[4] The authors reported that transgenic mice expressing human prion protein homozygous for methionine at codon 129, when inoculated with BSE prions, developed a molecular phenotype consistent with a subtype of sporadic CJD. Although the authors implied that BSE might cause a sporadic CJD-like illness among persons homozygous for methionine, the results of their research with mice do not necessarily directly apply to the transmission of BSE to humans. If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication).

References

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.

2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003;60:176-181.

3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283-314.

4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002;21:6358-6366.

5. The UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics. Available at: http://www.cjd.ed.ac.uk/figures.htm. Accessed February 18, 2003.


http://www.neurology.org/cgi/eletters/60/2/176



http://www.neurology.org/cgi/eletters/60/2/176#535



http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT




http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/%20fulltext




http://www.bmj.com/cgi/eletters/320/7226/8/b#6117




http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406




Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html




Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P
.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

snip...

COMMENT

snip...see full text ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html




Monday, June 01, 2009 Biochemical typing of pathological prion protein in aging cattle with BSE

SOMETHING TO PONDER ???

O.K. confusious asks, IF all these new atypical BSEs i.e. new strains of mad cow disease is just an 'OLD COW PRION DISEASE', why then can not the 'old human prion disease' such as the sporadic CJD, be from an 'old cow prion disease', same as the nvCJD 'young people mad cow disease' (which also happens in 74 year old), but why cannot the 'old cow prion diseases', i.e. l-BSE, h-BSE, and ibncBSE, cause the 'old people prion disease', which looks like sporadic CJD. seems that is what some of the pathology is showing ???

OH, that probably makes too much sense, and that the only answer could be that it's all just a happenstance of bad luck and or a spontaneous event, that just happens out of the clear blue sky $$$

IF this is the case, then where are all the SPONTANEOUS BSE CASES OF MAD COW DISEASE IN THE U.S.A., AND WHERE HAVE THEY BEEN BURIED IN THE USA OVER THE LAST 25 YEARS ???

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html




Saturday, August 01, 2009

Cases of Early-Onset Sporadic Creutzfeld-Jakob Disease in Michigan

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cases-of-early-onset-sporadic.html




Monday, July 27, 2009

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



Monday, August 17, 2009

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.html




WE must make all human TSE reportable in every state, and internationally, of ALL age groups, and this must be mandatory.

WE must test all livestock producing animals for animal and human consumption for Transmissible Spongiform Encephalopathy, and let the chips fall where they may. This masquerade of BSE testing in the USA, and the mad cow feed ban that never was must stop. ...


My Mother autopsy as follows ;

Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html




with kindest regards,

I am sincerely,

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY UPDATE (the silent pandemic) AND Agricultural Bioterrorism

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY UPDATE (the silent pandemic) AND Agricultural Bioterrorism

From: Terry S. Singeltary Sr. [flounder9@verizon.net] Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

Greetings,

i would like to kindly submit to this docket and warn of the potential for biological 'suitcase bombs' from civilian air-traffic populations from known BSE/FMD and other exotic animal disease pathogens coming into the USA.

please be warned;

snip...

Greetings list members,

i just cannot accept this;

23 kg of meat in a suitcase (suitcase bomb...TSS)

The data do not provide a species of origin code for these

products, therefore they may not contain any ruminant product.

what kind of statement is this?

how stupid do they think we are?

it could also very well mean that _all_ of it was ruminant based products !

snip...

Greetings FDA and public,

if you go to the below site, and search all BSE known countries and check out their air traffic illegal meat they have confiscated, and check out the low number checked, compared to actual passenger traffic, would not take too much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.

[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.]]

if they were to have questioned the terrorist that bombed the Twin Towers with jets, if they were to have questioned them at flight school in the USA, i am sure that they would have said they did not intend to visit the Twin Towers as a flying bomb either. what am i thinking, they probably did ask this? stupid me.

snip...

*** Czech Republic 74 472

Bovine Spongiform Encephalopathy, Czech Republic

Impact Worksheet, June 14, 2001

Summary: The Czech Republic confirmed on June 8, 2001 the first case of bovine spongiform encephalopathy (BSE) in a native-born cow. This is the first confirmed case of BSE in a native-born animal outside of western Europe. The Czech Republic has less than 1% of world cattle stocks, and less than 0.1% of sheep and goats. The country's exports of beef and veal accounted for less than 0.1% of world beef and veal exports in 1999 and are negligible in terms of world trade in these products. In 2001, the Czech Republic exported beef and live cattle to Austria, Germany, France, Greece, Bulgaria, Italy, Portugal, Slovakia, and Russia.

In December 1997, the USDA, APHIS enacted regulations which prohibited the importation of live ruminants and ruminant meat from Europe, including the Czech Republic. These import restrictions also applied to bone meal, blood meal, meat meal, offal, fat, glands, and serum from ruminants. In December 2000, APHIS expanded its import restrictions regarding BSE by prohibiting all imports of rendered animal protein products, regardless of species, from Europe.

How extensive is the situation in the affected country and what was the country's disease status prior to the outbreak? On June 8, 2001 the Czech Republic reported to the OIE the first case of bovine spongiform encephalopathy (BSE) in a native-born cow. The initial two positive tests in the Czech Republic were subsequently confirmed on June 14, 2001 by the German BSE Reference Center. This confirmation of BSE is the first confirmed occurrence of BSE in a native-born

Page 33 of 98 8/3/2006

animal outside of western Europe.

The six year old cow with clinical signs of BSE came from a breeding herd of 138 cows owned by a cooperative farm. No other animals on the farm showed clinical signs of BSE. As a result of the current case, all susceptible animals in the herd will be destroyed and tested. In addition, the Czech government will extend BSE testing to all slaughtered bovine animals older than 30 months. Before this case, the Czech government had randomly tested around 11 thousand slaughtered bovines this year, all with negative results.

The source of the infectious agent is not yet known. The Czech government banned feeding all meat-and-bone meal to cattle in 1991. However, the Czech government has initiated an investigation to determine if the contamination could have happened through feeding of imported milk feed substitutes in which milk fat was replaced by rendering-plant fat. In April 2001 the European Commission listed the Czech Republic as a Category III country "likely to present a BSE risk", based on assessments of the amount of live cattle and feed of animal origin imported into countries in question. Other countries listed in the same category were Poland, Hungary, Estonia, Lithuania, Slovakia, Cyprus, and Switzerland.

Source: OIE; Reuters; ProMED

What is the country's production and trade in affected animals and animal products? The Czech Republic has less than 0.2% of the world cattle stocks. In 1999 live cattle exports accounted for less than 0.6% of world cattle exports (Table A). No official data are available pertinent to the destination of exported cattle for that time period. However, recent news reports stated that this year the Czech Republic exported beef and live cattle to Austria, Germany, France, Greece, Bulgaria, Italy, Portugal, Slovakia, and Russia. Production and trade in live sheep and goats are very small, making up less than 0.1% of world production and trade in these animals.

Table A: Stocks and Trade in Live Animals, Czech Republic

Live Animal 2000 Stocks Trade 1999 Exports 1999 Imports Head % World Head % World Head % World Cattle 1,573,530 <0.2% 53,880 <0.6% 13,228 <0.2% Page 34 of 98 8/3/2006 Sheep 84,108 <0.1% 791 <0.1% 93 <0.1% Goats 31,988 <0.1% 115 <0.1% 21 <0.1% The Czech Republic's exports of beef and veal accounted for less than 0.1% of world beef and veal exports in 1999 and are negligible in terms of world trade in these products (Table B). In addition, no mutton, lamb, or goat meats were exported in 1999. Table B: Production and Trade in Relevant Products, Czech Republic Products 2000 Production, provisional Trade 1998 Exports 1998 Imports Metric ton % World Metric ton % World Metric ton % World Beef and veal 110,261 <0.2% 2,249 <0.1% 3,325 <0.1% Mutton and lamb 2,950 <0.1% Page 35 of 98 8/3/2006 0 0.0% 179 <0.1% Goat meat 290 <0.1% 0 0.0% 0 0.0% Note: Sheep and goats were included here as 'affected' animals because APHIS has included all ruminants and ruminant products in restrictions pertaining to BSE. Sources: UN Food and Agriculture Organization, Reuters. Did the US have restrictions on ruminant imports from the Czech Republic prior to the current case? In December 1997, APHIS prohibited the importation of live ruminants and most ruminant products from all of Europe including the Czech Republic until a thorough assessment of the risks of introduction of BSE into the US could be made. Prior to December 1997, import restrictions were applied only to those countries which had reported cases of BSE in native animals. In addition, the importation of ruminant meat from BSE-affected countries was permitted if the meat was deboned and free of visually identifiable lymphatic and nervous tissue and if it met other restrictions. The import regulations enacted in December 1997 extended the import restrictions to countries that had not had a declared case of BSE, yet had risk factors for the occurrence of BSE. These regulatory changes also removed the provisions which allowed the importation of ruminant meat from the restricted countries, essentially prohibiting the importation of ruminant meat from all of Europe. These import restrictions also applied to bone meal, blood meal, meat meal, offal, fat, glands, and serum from ruminants. Additionally, in December 2000, APHIS expanded its import restrictions regarding BSE by prohibiting all imports of rendered animal protein products, regardless of species, from Europe. Source: USDA, APHIS, VS What are the US imports of affected animals or animal products from the country? In accordance with the 1997 ban on the importation of live ruminants and most ruminant products including meat from Europe, the World Trade Atlas data show no such imports from the Czech Republic in 2000 or January - March 2001. The Czech Republic has two meat processing establishments approved to ship pork products to the US. However, according to available data, during 2000 and January - March 2001 no product from these plants was exported to the US. The US imports some dairy products such as butter and cheese from the Czech Republic. These products are unlikely sources of BSE. Source: World Trade Atlas; USDA, APHIS, VS; USDA, FSIS. Page 36 of 98 8/3/2006 What is the level of passenger traffic arriving in the United States from the affected country? A total of 45,438 passengers arrived in the US on direct flights from the Czech Republic in fiscal year 2000. It is likely that additional passengers originating in the Czech Republic traveled to the US on non-direct flights. As part of APHIS-PPQ's Agriculture Quarantine Inspection Monitoring, 238 air passengers from the Czech Republic were inspected for items of agricultural interest in fiscal year 2000. Of these, 10, or 4.2%, were found to be carrying a total of 17 kg of items that could potentially present a risk for BSE. None of the passengers with items reported plans to visit or work on a farm or ranch while in the US. Source: US Department of Transportation, and APHIS-PPQ Agricultural Quarantine Inspection data base CEI's plans for follow-up: CEI has no further plans regarding this case. However, if you seek more information or wish to comment on this worksheet, please reply to this message or contact Milo Muller at (970) 490-



http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



SEE BSE threat from air passenger traffic, country by country ;


APHIS-2007-0033-0002.1

Docket APHIS-2007-0033 Docket Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Docket Type Rulemaking Document APHIS-2007-0033-0001 Document Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Public Submission APHIS-2007-0033-0002.1 Public Submission Title Attachment to Singeltary comment Views



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf



http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=090000648027c28e



Docket Management Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305 Comment Number: EC -254 Accepted - Volume 11

2003-04-08 10:36:55



http://www.fda.gov/OHRMS/DOCKETS/DOCKETS/02n0276/02N-0276-EC-254.htm



http://madcowfeed.blogspot.com/2008/07/docket-aphis-2007-0033-docket-title.html


Voluntary - Public Clearance Office: Office of Scientific and Technical Affairs (OSTA) Date: 4/6/2009 GAIN Report Number: EZ9003

Czech Republic Post:

Prague BSE Confirmed in a Cow in the Region of Liberec in the Czech Republic

Report Categories: Livestock and Products Pest/Disease Occurrences Approved By: Eric Wenberg Prepared By: Jana Mikulasova Report Highlights: The 29th case of BSE in the Czech Republic was confirmed on March 25, 2009 by the State Veterinary Administration. The cow was on farm in Roprachtice in the Region of Liberec in a northern part of the country. General Information: On March 25, 2009, the State Veterinary Administration (SVA) confirmed the 29th case of BSE in a cow in the Czech Republic since 2001. The cow located on a farm in Roprachtice, approximately 100 km to the northeast of Prague, nearing Polish border, was 66 months old. It has been the youngest infected animal that was born after the ban of meat-bone meal use in feeds. According to the SVA, the cow may have gotten contaminated feed, but it's difficult to locate the source of the problem since the contaminated feed would have been consumed five years ago. There were 82 cows identified in the same herd that will be killed within 90 days. The affected farmers will receive a compensation for these animals. The Czech Republic has been fully in line with the EC Regulation no. 999/2001 of the European Parliament and of the Council of 22 May 2001, laying down rules for the prevention, control and eradication of transmissible spongiform encephalopathies and it has been recognized as having a "controlled BSE risk" in accordance with Chapter 11.6. of the Terrestrial Code of The World Organization for Animal Health (OIE). The last case of BSE in the Czech Republic occurred in December 2007. Author Defined:



http://gain.fas.usda.gov/Recent%20GAIN%20Publications/BSE%20Confirmed%20in%20a%20Cow%20in%20the%20Region%20of%20Liberec%20in%20the%20Czech%20Republic_Prague_Czech%20Republic_4-17-2009.pdf




Saturday, April 25, 2009

Prague BSE Confirmed in a Cow in the Region of Liberec in the Czech Republic



http://docket-aphis-2006-0041.blogspot.com/2009/04/prague-bse-confirmed-in-cow-in-region.html



Monday, April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)



http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.html



Sunday, April 12, 2009

BSE MAD COW TESTING USA 2009 FIGURES

Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620



http://www.aphis.usda.gov/newsroom/hot_issues/bse/surveillance/ongoing_surv_results.shtml


http://madcowtesting.blogspot.com/2009/04/bse-mad-cow-testing-usa-2009-figures.html



PLEASE SEE MY FULL COMMENT SUBMISSION IN THE PDF ATTACHMENT, OR GO HERE


Thursday, April 9, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed



http://madcowfeed.blogspot.com/2009/04/docket-no-fda2002n0031-formerly-docket.html



http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-problem-dont.html#comments



Sunday, April 12, 2009 r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada



http://prionunitusaupdate2008.blogspot.com/2009/04/r-calf-and-usa-mad-cow-problem-dont.html



http://prionunitusaupdate2008.blogspot.com/2009/04/cjd-foundation-sides-with-r-calfers-no.html#comments



ALSO, please note ;

SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)SEACAgenda102nd Meeting on Wednesday 4 March 2009Room 808, Nobel House, 17 Smith Square, Defra, London SW1P 3JR10.05 Approval of draft minutes from SEAC 101

snip...

ITEM 3 - CURRENT ISSUES 8.

SEAC was informed about the following issues: .

A mother and son in Spain had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. As both the mother and son lived in a region of Spain with a history of BSE, had frequently shared meals of cattle brain, and as no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure. Furthermore, the similar times of onset of disease of the cases did not suggest transmission had occurred from one to the other.snip...Thursday, February 26, 2009SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)see full text ;



http://www.seac.gov.uk/papers/102-1.pdf




http://seac992007.blogspot.com/2009/02/seac-102nd-meeting-on-wednesday-4-march.html




Tuesday, August 12, 2008Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)




http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html




Tuesday, April 21, 2009

Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN



http://cjdusa.blogspot.com/2009/04/doctor-antonio-ruiz-villaespesa.html



Alzheimer's and TSE


http://betaamyloidcjd.blogspot.com/



kind regards, terry

MADCOW USDA the untold story

I have wasted a decade researching this as a lay person, and believe me, there are some in the industry that will make sure you are aware of that, but that's another story. My mother was murdered by what I call corporate and political homicide. whether or not is oral consumption or friendly fire, second, third, or fourth passage, via someone that consumed something years ago, I am here to tell you we have all been exposed to the mad cow agent, no matter what species you wish to speak of, and I am speaking of the Transmissible Spongiform Encephalopathies, and i could not have spelled that ten years ago, much less the Heidenhain Variant of Creutzfeldt Jakob Disease, and my spell checker does not have that one either. IT is an exceedingly rare strain of the CJD i.e. sporadic, spontaneous, of which neither one has ever been proven to be either. There is a route, and source.

Mad Cow disease has been in the USA for some time, of which has been systematically covered up, and I can and have proven this. The media does not care, and I am going to stop beating the drums on the 10 year anniversary of my mothers homicide December 14, 2007. I send this data to you as a last resort of sorts, you use as you wish. I have no PhDs, I am no doctor, and I am President and CEO of nothing. I simply wanted the truth. I have wasted a decade. But i am hear to tell you the UKBSEnvCJD only theory is and was bogus from the beginning. Its a long long story, one you cannot explain in a few minutes, hours, or even days. But you must become aware of the fact that the USA MAD COW STRAIN i.e. the h-BASE, is more virulent to humans, and mirrors that of some sub-types of the sporadic CJD. I have tried to warn the public, industry and health officials for a decade only to be called a high school flunky. but if you would please take the time to read the data, i believe you will see i was proven correct. and that is disturbing.

The following is from someone that wanted to interview me recently but i said ................

well see for yourself, but please take the time///

Thank you,

I am sincerely,

Terry S. Singeltary Sr.

PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in ''TYPE UNKNOWN''. ...TSS

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,

26 from 2007)

http://www.cjdsurveillance.com/pdf/case-table.pdf



BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS


http://madcowtesting.blogspot.com/



----- Original Message ----- From: Terry S. Singeltary Sr. To: xxxxxxxxxxxx Sent: Saturday, October 06, 2007 4:49 PM Subject: Re: xxxxxxxxxxxxxxxxxxxxx

October 6, 2007 16:40 hours central

Hello Mr. xxxxxx,

Glad to meet you sir. I have to admit, I am a bit shocked at your fact finding mission now, since I am retiring and have not been giving interviews i.e. radio, tv, or anymore people at my house for a while. I am winding down after a decade as of there abouts 12/14/07 i.e. mom's dod of the Heidenhain Variant of Creutzfeldt Jakob disease. you see, this is why I do/did this, maybe you are aware of that. I am/was only vested in one thing, the truth. no, i don't pretend to have all the answers. there's still many unanswered questions, but the answers we do have, we have been ignoring, we must start taking them serious i.e. amplification and transmission of the TSEs, and the list is growing, and they are becoming more virulent. I have answered the questions I needed, and i tried to educate the public and the industries involved the best I could. you must know, I did not choose to do this. it chose me, and it killed my mother. it, i.e. transmissible spongiform encephalopathy. oral route, or friendly fire, both are 100% fatal once clinical, there is much more to this than what I call the UKBSEnvCJD only theory, of a disease, that was supposedly only in one part of the world, in only one geographical location, only involving one group of cattle, and only one group of young humans, when documented BSE farmers and there wives were dropping dead from the sporadic CJDs i.e. one of six phenotypes now documented, but the list is growing there too, and questionable (we will get to all this in a minute). IF you believe in the UKBSEnvCJD only theory, BSE/TSE tainted MBM was imported into the USA and the rest of the world, and or IF you also believe in the fact that the USA and North America have also recycled there own super strain (you do know we shipped the continuous rendering technology to the UK some 5 years, BEFORE WE STARTED USING IT). The USA did both. so, it's a double whammy so to speak, and super strain due to the fact we have been feeding our food producing animals for consumption, not only all the strains of sheep scrapie typical and atypical, but also the cwd and there different phenotypes of deer and elk, along I am afraid with a few TME cases, I dont think you can rule that out. and nobody even checks for TSE in dogs and cats. so the USA is in a Unique situation. I have files for all this if you want it. sorry, I am rambling now. BUT for anyone to think that Canada having a worse BSE problem than the USA, or Mexico, is not looking at all the data. feed alone trading back and forth, cross contamination of vehicles, live cattle, sheep, deer and elk trades, crossing borders, legal and illegal, and yes, we see that now BSE does transmit to red deer. CWD transmitting to cattle and sheep. all this transmitting to primates. I could go on. with that said, i said long ago, the BSE MRR policy is nothing more than a legal tool to trade TSE globally. the BSE GBR risk assessments must be adhered to, the BSE MRR repealed, killed, buried. the OIE even says the BSE MRR ratings are only as good as what the countries supplying the info are giving them to go by. and if you look at what the top TSE scientist have said about the infamous june 2004 enhanced BSE surveillance program, it was flawed from the beginning, and results should not be trusted, as with what was later found out about the Harvard BSE study, but to get to the just of that, you must read the PEER REVIEW of the Harvard BSE study. I have suppled sources for all this below. ...

Mr. xxxxxx wrote;

> I do care and I'm trying to get all sides of the issue covered.

this is good news! it's about time somebody did. 10 YEARS post voluntary feed ban, and were still feeding cows to cows i.e. srm. however, I am afraid my view does not matter much, I am only the messenger. I am not a cattle baron. not educated much, this is what they keep telling me anyway (this is funny really, see below). what needs to happen is that you Sir read and understand the TSE science, and you tell your readers what really is and has been going on. if your waiting on the USDA to tell you, your gonna have a long wait. you have the USDA cowtailing to the big ag buisiness like the big feeders, packers, renders etc. all feeding off one thing, money. i don't really blame the ranchers, most were pretty much oblivious to the long term severity of TSE. the USDA and FDA did, and they failed to enforce there very own rules and to educate. I found this out sitting in on the Jan. 2001 USDA emergency 50 state BSE conference call ;

Subject: USDA/APHIS response to BSE-L--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001 Date: Wed, 10 Jan 2001 14:04:21 -0500 From: Gomez, Thomas M. Reply-To:


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html



Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
Date: Tue, 9 Jan 2001 16:49:00 -0800
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To:

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

Greetings List Members,

I was lucky enough to sit in on this BSE conference call today and even managed to ask a question. that is when the trouble started.

I submitted a version of my notes to Sandra Blakeslee of the New York Times, whom seemed very upset, and rightly so.

"They tell me it is a closed meeting and they will release whatever information they deem fit. Rather infuriating."

and i would have been doing just fine, until i asked my question. i was surprised my time to ask a question so quick.

(understand, these are taken from my notes for now. the spelling of names and such could be off.)

[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.

[TSS] yes, thank you,

U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]

[host Richard] could you repeat the question?

[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[not sure whom ask this] what group are you with?

[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.

[not sure who is speaking] could you please disconnect Mr. Singeltary

[TSS] you are not going to answer my question?

[not sure whom speaking] NO

from this point, i was still connected, got to listen and tape the whole conference. at one point someone came on, a woman, and ask again;

[unknown woman] what group are you with?

[TSS] CJD Watch and my Mom died from hvCJD we are trying to tract down CJD and other human TSE's world wide. i was invited to sit in on this from someone inside the USDA/APHIS and that is why i am here. do you intend on banning me from this conference now?

at this point the conference was turned back up, and i got to finish listening. They never answered or even addressed my one question, or even addressed the issue. BUT, i will try and give you a run-down for now, of the conference.

IF i were another Country, I would take heed to my notes, BUT PLEASE do not depend on them. ask for transcript from;

[log in to unmask] 301-827-6906

he would be glad to give you one ;-)

Rockville Maryland, Richard Barns Host

snip...full text ;

http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html



NOW, almost 7 years later, look where were at, and what about the atypicals and the fact there are now 4 documented blood transfusion related nvCJD cases.

FDA Proposes Barring Certain Cattle Material From Medical Products As BSE Safeguard

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0701&L=sanet-mg&P=5318



http://bloodindex.org/view_news_zone.php?id=206



http://www.bseinquiry.gov.uk/files/yb/1988/11/04003001.pdf



8. The Secretary of State has a number of licences. We understand that the inactivated polio vaccine is no longer being used. There is a stock of smallpox vaccine. We have not been able to determine the source material. (Made in sheep very unlikely to contain bovine ingredients).

http://www.bseinquiry.gov.uk/files/yb/1989/02/14010001.pdf


http://www.bseinquiry.gov.uk/files/yb/1989/02/14011001.pdf


although 176 products do _not_ conform to the CSM/VPC guidelines.

http://www.bseinquiry.gov.uk/files/yb/1989/09/06011001.pdf


> Looks like you have the facts at hand. Would you mind being the subject of next week's interview?

negative, I do not have all the facts. I only try to stay informed with the up to date sound science. and I try to give this to the public and industry. a great deal of time the industry and most media choose to ignore the ever so evolving science, and they choose take the rubber stamped TSE data that the USDA feeds them, which most of the time is ancient data. again, this is almost 2008, and we are still feeding cows to cows i.e. banned srms ;

October 2007 Update on Feed Enforcement Activities to Limit the Spread of BSE

http://www.phxnews.com/fullstory.php?article=53149


http://www.fda.gov/cvm/BSE1007.htm


10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html


MORE 2006 FEED BAN VIOLATIONS BELOW, ''IN COMMERCE'' ;

Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS IN COMMERCE AL, TN, AND WV Date: September 6, 2006 at 7:58 am PST

PRODUCT a) EVSRC Custom dairy feed, Recall # V-130-6; b) Performance Chick Starter, Recall # V-131-6; c) Performance Quail Grower, Recall # V-132-6; d) Performance Pheasant Finisher, Recall # V-133-6. CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete. REASON Dairy and poultry feeds were possibly contaminated with ruminant based protein. VOLUME OF PRODUCT IN COMMERCE 477.72 tons DISTRIBUTION AL ______________________________ PRODUCT a) Dairy feed, custom, Recall # V-134-6; b) Custom Dairy Feed with Monensin, Recall # V-135-6. CODE None. Bulk product RECALLING FIRM/MANUFACTURER Recalling Firm: Burkmann Feed, Greeneville, TN, by Telephone beginning on June 28, 2006. Manufacturer: H. J. Baker & Bro., Inc., Albertville, AL. Firm initiated recall is complete. REASON Possible contamination of dairy feeds with ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 1,484 tons DISTRIBUTION TN and WV

http://www.fda.gov/bbs/topics/enforce/2006/ENF00968.html


Subject: MAD COW FEED RECALLS ENFORCEMENT REPORT FOR AUGUST 9, 2006 KY, LA, MS, AL, GA, AND TN 11,000+ TONS Date: August 16, 2006 at 9:19 am PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE - CLASS II ______________________________ PRODUCT Bulk custom made dairy feed, Recall # V-115-6 CODE None RECALLING FIRM/MANUFACTURER Hiseville Feed & Seed Co., Hiseville, KY, by telephone and letter on or about July 14, 2006. FDA initiated recall is ongoing. REASON Custom made feeds contain ingredient called Pro-Lak which may contain ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE Approximately 2,223 tons DISTRIBUTION KY

______________________________ PRODUCT Bulk custom made dairy feed, Recall # V-116-6 CODE None RECALLING FIRM/MANUFACTURER Rips Farm Center, Tollesboro, KY, by telephone and letter on July 14, 2006. FDA initiated recall is ongoing. REASON Custom made feeds contain ingredient called Pro-Lak which may contain ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 1,220 tons DISTRIBUTION KY

______________________________ PRODUCT Bulk custom made dairy feed, Recall # V-117-6 CODE None RECALLING FIRM/MANUFACTURER Kentwood Co-op, Kentwood, LA, by telephone on June 27, 2006. FDA initiated recall is completed. REASON Possible contamination of animal feed ingredients, including ingredients that are used in feed for dairy animals, with ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 40 tons DISTRIBUTION LA and MS

______________________________ PRODUCT Bulk Dairy Feed, Recall V-118-6 CODE None RECALLING FIRM/MANUFACTURER Cal Maine Foods, Inc., Edwards, MS, by telephone on June 26, 2006. FDA initiated recall is complete. REASON Possible contamination of animal feed ingredients, including ingredients that are used in feed for dairy animals, with ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 7,150 tons DISTRIBUTION MS

______________________________ PRODUCT Bulk custom dairy pre-mixes, Recall # V-119-6 CODE None RECALLING FIRM/MANUFACTURER Walthall County Co-op, Tylertown, MS, by telephone on June 26, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 87 tons DISTRIBUTION MS

______________________________ PRODUCT Bulk custom dairy pre-mixes, Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 350 tons DISTRIBUTION AL and MS

______________________________ PRODUCT a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb. bags, Recall # V-121-6; b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6; c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6; d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6; e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6; f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6; g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6 CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing. REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants". VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags DISTRIBUTION AL, GA, MS, and TN

END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006

###

http://www.fda.gov/bbs/topics/ENFORCE/2006/ENF00964.html


Subject: MAD COW FEED RECALL MI MAMMALIAN PROTEIN VOLUME OF PRODUCT IN COMMERCE 27,694,240 lbs Date: August 6, 2006 at 6:14 pm PST PRODUCT Bulk custom dairy feds manufactured from concentrates, Recall # V-113-6 CODE All dairy feeds produced between 2/1/05 and 6/16/06 and containing H. J. Baker recalled feed products. RECALLING FIRM/MANUFACTURER Vita Plus Corp., Gagetown, MI, by visit beginning on June 21, 2006. Firm initiated recall is complete. REASON The feed was manufactured from materials that may have been contaminated with mammalian protein. VOLUME OF PRODUCT IN COMMERCE 27,694,240 lbs DISTRIBUTION MI

END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html


Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006 Date: August 6, 2006 at 6:16 pm PST PRODUCT a) CO-OP 32% Sinking Catfish, Recall # V-100-6; b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6; c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6; d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6; e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6; f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6; g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6; h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6; i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6; j) CO-OP LAYING CRUMBLES, Recall # V-109-6; k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6; l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6; m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE Product manufactured from 02/01/2005 until 06/06/2006 RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete. REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants". VOLUME OF PRODUCT IN COMMERCE 125 tons DISTRIBUTION AL and FL

END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html


Subject: MAD COW FEED RECALL KY VOLUME OF PRODUCT IN COMMERCE ????? Date: August 6, 2006 at 6:19 pm PST PRODUCT Bulk custom made dairy feed, Recall # V-114-6 CODE None RECALLING FIRM/MANUFACTURER Burkmann Feeds LLC, Glasgow, KY, by letter on July 14, 2006. Firm initiated recall is ongoing. REASON Custom made feeds contain ingredient called Pro-Lak, which may contain ruminant derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE ????? DISTRIBUTION KY END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html


CJD WATCH MESSAGE BOARD TSS MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II ______________________________ PRODUCT a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6; b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6; c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6; d) Feather Meal, Recall # V-082-6 CODE a) Bulk b) None c) Bulk d) Bulk RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing. REASON Possible contamination of animal feeds with ruminent derived meat and bone meal. VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons DISTRIBUTION Nationwide

END OF ENFORCEMENT REPORT FOR July 12, 2006

###

http://www.fda.gov/bbs/topics/enforce/2006/ENF00960.html


Subject: MAD COW FEED BAN WARNING LETTER ISSUED MAY 17, 2006 Date: June 27, 2006 at 7:42 am PST Public Health Service Food and Drug Administration

New Orleans District 297 Plus Park Blvd. Nashville, TN 37217

Telephone: 615-781-5380 Fax: 615-781-5391

May 17, 2006

WARNING LETTER NO. 2006-NOL-06

FEDERAL EXPRESS OVERNIGHT DELIVERY

Mr. William Shirley, Jr., Owner Louisiana.DBA Riegel By-Products 2621 State Street Dallas, Texas 75204

Dear Mr. Shirley:

On February 12, 17, 21, and 22, 2006, a U.S. Food & Drug Administration (FDA) investigator inspected your rendering plant, located at 509 Fortson Street, Shreveport, Louisiana. The inspection revealed significant deviations from the requirements set forth in Title 21, Code of Federal Regulations, Part 589.2000 [21 CFR 589.2000], Animal Proteins Prohibited in Ruminant Feed. This regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE). You failed to follow the requirements of this regulation; products being manufactured and distributed by your facility are misbranded within the meaning of Section 403(a)(1) [21 USC 343(a)(1)] of the Federal Food, Drug, and Cosmetic Act (the Act).

Our investigation found you failed to provide measures, including sufficient written procedures, to prevent commingling or cross-contamination and to maintain sufficient written procedures [21 CFR 589.2000(e)] because:

You failed to use clean-out procedures or other means adequate to prevent carryover of protein derived from mammalian tissues into animal protein or feeds which may be used for ruminants. For example, your facility uses the same equipment to process mammalian and poultry tissues. However, you use only hot water to clean the cookers between processing tissues from each species. You do not clean the auger, hammer mill, grinder, and spouts after processing mammalian tissues.

You failed to maintain written procedures specifying the clean-out procedures or other means to prevent carryover of protein derived from mammalian tissues into feeds which may be used for ruminants.

As a result . the poultry meal you manufacture may contain protein derived from mammalian tissues prohibited in ruminant feed. Pursuant to 21 CFR 589.2000(e)(1)(i), any products containing or may contain protein derived from mammalian tissues must be labeled, "Do not feed to cattle or other ruminants." Since you failed to label a product which may contain protein derived from mammalian tissues with the required cautionary statement. the poultry meal is misbranded under Section 403(a)(1) [21 USC 343(a)(1)] of the Act.

This letter is not intended as an all-inclusive list of violations at your facility. As a manufacturer of materials intended for animal feed use, you are responsible for ensuring your overall operation and the products you manufacture and distribute are in compliance with the law. You should take prompt action to correct these violations, and you should establish a system whereby violations do not recur. Failure to promptly correct these violations may result in regulatory action, such as seizure and/or injunction, without further notice.

You should notify this office in writing within 15 working days of receiving this letter, outlining the specific steps you have taken to bring your firm into compliance with the law. Your response should include an explanation of each step taken to correct the violations and prevent their recurrence. If corrective action cannot be completed within 15 working days, state the reason for the delay and the date by which the corrections will be completed. Include copies of any available documentation demonstrating corrections have been made.

Your reply should be directed to Mark W. Rivero, Compliance Officer, U.S. Food and Drug Administration, 2424 Edenborn Avenue, Suite 410, Metairie, Louisiana 70001. If you have questions regarding any issue in this letter, please contact Mr. Rivero at (504) 219-8818, extension 103.

Sincerely,

/S

Carol S. Sanchez Acting District Director New Orleans District

http://www.fda.gov/foi/warning_letters/g5883d.htm


look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;

Risk of oral infection with bovine spongiform encephalopathy agent in primates

Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.

snip...

BSE bovine brain inoculum

100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg

Primate (oral route)* 1/2 (50%)

Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%)

RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)

PrPres biochemical detection

The comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was

inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of

bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal.

Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inocula

Published online January 27, 2005

http://www.thelancet.com/journal/journal.isa


It is clear that the designing scientists must

also have shared Mr Bradley's surprise at the results because all the dose

levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s145d.pdf


2

6. It also appears to me that Mr Bradley's answer (that it would take less than say 100

grams) was probably given with the benefit of hindsight; particularly if one

considers that later in the same answer Mr Bradley expresses his surprise that it

could take as little of 1 gram of brain to cause BSE by the oral route within the

same species. This information did not become available until the "attack rate"

experiment had been completed in 1995/96. This was a titration experiment

designed to ascertain the infective dose. A range of dosages was used to ensure

that the actual result was within both a lower and an upper limit within the study

and the designing scientists would not have expected all the dose levels to trigger

infection. The dose ranges chosen by the most informed scientists at that time

ranged from 1 gram to three times one hundred grams. It is clear that the designing

scientists must have also shared Mr Bradley's surprise at the results because all the

dose levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s147f.pdf


Re: BSE .1 GRAM LETHAL NEW STUDY SAYS via W.H.O. Dr Maura Ricketts

[BBC radio 4 FARM news]

http://www.maddeer.org/audio/BBC4farmingtoday2_1_03.ram


http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm


2) Infectious dose:

To cattle: 1 gram of infected brain material (by oral ingestion)

http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0611&L=sanet-mg&P=3715


> I'd love to give you the opportunity to point out what you've detailed in your message to me.

DO you know how long that would take, and what it curtails ? I will try to do this below as short as I can, but to understand all this you will have to go back to early days, then work your way to date. ill try, this is long winded, but this is one reason I don't give interviews anymore, for one thing, it's not about me, and another, if you are really looking for the truth, you cannot give it in a 10 minute one page interview. but if you do really want the facts, as I have come to know them, consider this an interview, and I will try to explain below, and you can use as you wish, in part, or whole. I hope you do, I have wasted 10 years trying to explain that there is more to this story than the UKBSEnvCJD hamburger eating adolescent only theory, and the rest of the 85 to 90 % of dead from CJD, i.e. sporadic, well, that just happens, no route, no source, just spontaneous happening of bad luck. THE spontaneous theory has never been proven. THE only spontaneous TSE they developed in the lab, does not look like natural field TSE. IN fact, most scientist say this cannot even be proven i.e spontaneous TSE in humans, cattle, or any other species. AND the few that do believe in it (without proof), are mostly big ag and industry, and they just try to avoid communicating it at all cost, remember that, 'AT ALL COST'. ...

STRICTLY PRIVATE AND CONFIDENTIAL 25, AUGUST 1995

snip...

To minimise the risk of farmers' claims for compensation from feed compounders.

To minimise the potential damage to compound feed markets through adverse publicity.

To maximise freedom of action for feed compounders, notably by maintaining the availability of meat and bone meal as a raw material in animal feeds, and ensuring time is available to make any changes which may be required.

snip...

THE FUTURE

4..........

MAFF remains under pressure in Brussels and is not skilled at handling potentially explosive issues.

5. Tests _may_ show that ruminant feeds have been sold which contain illegal traces of ruminant protein. More likely, a few positive test results will turn up but proof that a particular feed mill knowingly supplied it to a particular farm will be difficult if not impossible.

6. The threat remains real and it will be some years before feed compounders are free of it. The longer we can avoid any direct linkage between feed milling _practices_ and actual BSE cases, the more likely it is that serious damage can be avoided. ...

SEE full text ;

http://www.bseinquiry.gov.uk/files/yb/1995/08/24002001.pdf


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

snip...

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

snip...

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

snip...

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a _very low profile indeed_. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be _avoided_ in the US _at all costs_...

snip...

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


YOU SEE SIR, it never was about sound science.

LETS start with the UKBSEnvCJD only theory, lets look at UK exports to USA, Canada, and Mexico. the imported only theory. ...

1994 UK EXPORTS BEEF VEAL USA , MEXICO $ CANADA ONLY other Countries list in PDF file)

USA -------- TOTALS ''8'' TONS CANADA -- TOTALS ''29'' TONS

1995 UK EXPORT BEEF AND VEAL TO USA AND CANADA

USA ------- TOTALS ''358'' TONS

CANADA --TOTALS ''24'' TONS

BONE-IN BEEF AND VEAL

USA-------- TOTALS ''10'' TONS (i think this is part of the 358 tons above?)

UK EXPORT OF LIKE CATTLE TO USA AND CANADA

1986 TO 1996 USA TOTAL = 1297

1986 TO 1996 CAN TOTAL = 299

http://www.bseinquiry.gov.uk/files/mb/m11f/tab10.pdf

UK EXPORT MEAT OR OFFAL OF BOVINE ANIMALS DEC 1987

CANADA -- 64,526 KG

UK EXPORT OFFALS OF BOVINE ANIMALS FRESH CHILLED OR FROZEN OTHER THAN LIVER DEC 1987 YTD

USA -- 45,943 KG

UK EXPORT MEAT OF BOVINE ANIMAL WITH BONE IN 1988

CANADA -- 4,163 KG

PREP OR PRES MEAT OR OFFAL OF BOVINE ANIMALS CUMULATIVE TO DEC 1988

USA -------- 28,609 KG CANADA -- 22,044 KG

MEAT OF BOVINE ANIMALS WITH BONE IN CUMULATIVE TO ANUAL 1989

USA -------- 17,880 KG MEXICO---- 33,444 KG

BONELESS MEAT OF BOVINE 1989

USA --------111,953 KG CANADA---1,800 KG MEXICO --- 1,143,387 KG

EDIBLE OFFAL OF BOVINE ANIMALS 1989

USA -------- 19,980 KG MEXICO--- 31,244 KG

MORE........

MEAT OF BOVINE ANIMALS BONELESS 1990

USA 146,443

http://www.bseinquiry.gov.uk/files/mb/m11g/tab05.pdf


UK Exports of Live Cattle by Value 1986-96

USA 697 LIVE CATTLE

CANADA 299 LIVE CATTLE

http://www.bseinquiry.gov.uk/files/mb/m11f/tab11.pdf


UK TABLE of Exports of meal of meat and meat offal; greaves 1979 - 1995

USA 24 TONS

CANADA 83 TONS

http://www.bseinquiry.gov.uk/files/mb/m12/tab12.pdf


HOWEVER, my files show 44 tons of greaves for USA. ...TSS

Subject: Re: exports from the U.K. of it's MBM to U.S.??? From: S.J.Pearsall@esg.maff.gsi.gov.uk Date: Tue, 8 Feb 2000 14:03:16 +0000 To: flounder@wt.net (Receipt Notification Requested) (Non Receipt Notification Requested)

Terry Meat and bonemeal is not specifically classified for overseas trade purposes. The nearest equivalent is listed as flours and meals of meat or offals (including tankage), unfit for human consumption; greaves. UK exports of this to the US are listed below: Country Tonnes

1980
1981 12
1982
1983
1984 10
1985 2
1986
1987
1988
1989 20
1990

Data for exports between 1975 and 1979 are not readily available. These can be obtained (at a charge) from data retailers appointed by HM Customs and Excise: BTSL (Tel: 01372 463121) or Abacus (01245 252222). Best wishes Simon Pearsall Overseas trade statistics Stats (C&F)C

====================================== END...TSS

BSE GBR RISK ASSESSMENTS, USA, CANADA, AND MEXICO

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA)

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.

http://www.efsa.europa.eu/en/science/tse_assessments/gbr_assessments/573.html

http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/573.Par.0004.File.dat/sr03_biohaz02_usa_report_v2_en1.pdf


EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of Canada

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 to provide an up-to-date scientific report on the GBR in Canada, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Canada. This scientific report addresses the GBR of Canada as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into the country middle of the eighties and could have reached domestic cattle in the early nineties. 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 90s. It is possible that imported meat and bone meal (MBM) into Canada reached domestic cattle and led to an internal challenge in the early 90s.

A certain risk that BSE-infected cattle entered processing in Canada, and were at least partly rendered for feed, occurred in the early 1990s when cattle imported from UK in the mid 80s could have been slaughtered. 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 Canada is III, i.e. it is confirmed at a lower level that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as the system remains unstable, it is expected that the GBR continues to grow, even if no additional external challenges occur.

http://www.efsa.europa.eu/en/science/tse_assessments/gbr_assessments/564.html


http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/564.Par.0001.File.dat/sr02_biohaz02_canada_report_v2_en1.pdf


EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of Mexico

Last updated: 8 September 2004 Publication Date: 20 August 2004

Adopted July 2004 (Question N° EFSA-Q-2003-083)

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 Mexico, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Mexico. This scientific report addresses the GBR of Mexico as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into Mexico and could have reached domestic cattle. These cattle imported could have been rendered and therefore led to an internal challenge in the mid to late 1990s. It is possible that imported meat and bone meal (MBM) into Mexico reached domestic cattle and leads to an internal challenge around 1993.

It is likely that BSE infectivity entered processing at the time of imported 'at - risk' MBM (1993) and at the time of slaughter of imported live 'at - risk' cattle (mid to late 1990s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable. Thus it is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.

EFSA concludes that the current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.

http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/565.Par.0003.File.dat/sr04_biohaz02_mexico_report_summary_en1.pdf


http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/565.Par.0004.File.dat/sr04_biohaz02_mexico_report_v2_en1.pdf


O.K. NOW Mr. xxxxxx, for the North America Home Grown TSE, amplification, and transmission, and what about the atypical h-base bse, and the nor-98 scrapie cases popping up in the USA and why isn't anyone speaking much about it, and what are the potential ramifications, what does the Nobel Prize Winner Stanely Prusiner say about atypical scrapie, lets look at that first ;

Published online before print October 20, 2005

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

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

( sheep prion transgenic mice )

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

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

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

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

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

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

To whom correspondence should be addressed.

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

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

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


OF COURSE, the USDA et al once was concerned for human health from typical scrapie, and rightly so, typical scrapie transmits to primates by there NON-FORCED ORAL CONSUMPTION ;

76/10.12/4.6

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


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


ALSO,

The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.

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


SO, in my opinion, the _myth_ that typical scrapie does not transmit to humans, is just that, a myth. there have never been transmission studies done on man, and I would bet my last bottom dollar, anyone making the statement, would not eat a handful of scrapie infected brains. you do know about james alford don't you, whether or not it was the sheep brains he was fed in the middle east, or something else, this war hero has cjd, and he is very young. this was a tragic story too. dont hear much of that now either. ...

http://www.blackfive.net/main/2004/10/a_dying_hero_ss.html


http://www.blackfive.net/main/2004/03/ssg_james_alfor.html


NOW, why in the world is no one much speaking about the lates 3 cases of the NOR-98 case in the USA, and what are the potential ramifications thereof;

Subject: Aspects of the Cerebellar Neuropathology in Nor98 Date: September 26, 2007 at 4:06 pm PST

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.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


From: "Terry S. Singeltary Sr."

Sent: Tuesday, August 21, 2007 9:50 AM

Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringing total to 3 cases to date

Infected and Source Flocks As of June 30, 2007, there were .....

snip...

One field case and one validation case were consistent with Nor-98 scrapie.

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps


IN the February 2007 Scrapie report it only mentions ;

''One case was consistent with Nor98 scrapie.''

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/


(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS USDA, APHIS, VS ET AL. ...TSS)

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553


An evaluation of scrapie surveillance in the United States

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427


FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451


MR. Jolley, you see why this cannot be done in a 10 minute interview, or a one page story,

SO, lets move on from typical and atypical scrapie, to CWD in deer and elk, and why are

either important to the cattle producer and humans? Because they all transmit to each other

in the lab, and there are potential ramifications from the environmental transmission of these

TSE, and as they have mutated, they become more virulent, so the potential for some TSE

to transmit via the environment is very real i.e. casual contact between sheep, goat, deer, elk,

with cattle in the field. This has never been documented in cattle, yet,

to date. lets look at some of the data there to date ;

P04.61

Survival of PrPSc during Simulated Wastewater Treatment Processes

Pedersen, J1; Hinckley, G1; McMahon, K2; McKenzie, D3; Aiken, JM3 1University of Wisconsin, Soil Science/Civil and Environmental Engineering, USA; 2University of Wisconsin, Civil and Environmental Engineering, USA; 3University of Wisconsin, Comparative Biosciences, USA

Concern has been expressed that prions could enter wastewater treatment systems through sewer and/or septic systems (e.g., necropsy laboratories, rural meat processors, private game dressing) or through leachate from landfills that have received TSE-contaminated material. Prions are highly resistant to degradation and many disinfection procedures raising concern that they could survive conventional wastewater treatment. Here, we report the results of experiments examining the partitioning and survival of PrPSc during simulated wastewater treatment processes including activated and mesophilic anaerobic sludge digestion. We establish that PrPSc can be efficiently extracted from activated and anaerobic digester sludges with 1% sodium dodecyl sulfate, 10% sodium undecyl sulfate, and 1% sodium N-lauryl sarcosinate. Activated sludge digestion does not result in significant degradation of PrPSc. The protein partitions strongly to the activated sludge solids and is expected to enter biosolids treatment processes. A large fraction of PrPSc survived simulated mesophilic anaerobic sludge digestion. Our results suggest that if prions were to enter municipal waste water treatment systems, most of the agent would partition to activated sludge solids, survive mesophilic anaerobic digestion, and be present in treated biosolids. Land application of biosolids containing prions could represent a route for their unintentional introduction into the environment. Our results argue for excluding inputs of prions to municipal wastewater treatment facilities that would result in unacceptable risk of prion disease transmission via contaminated biosolids.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


P04.01

Chronic Wasting Disease in a Captive White-Tailed Deer Farm

Keane, D1; Barr, D1; Bochsler, P1; Hall, M2; Gidlewski, T3; O'Rourke, K4; Spraker, T5 1University of Wisconsin, USA; 2US Department of Agriculture, USA; 3US Department of Agriculture, USA; 4USDA ARS-ADRU, Washington State University, USA; 5Veterinary Diagnostic Laboratory, Colorado State University, USA

A white-tailed deer farm in Portage, Wisconsin, was depopulated in January 2006, after chronic wasting disease (CWD) had been initially discovered on the property in September 2002. Prior to the depopulation, a total of 22 positive animals had been removed from the property: one in 2002, six in 2003, ten in 2004, four in 2005 and one in 2006. At the time of depopulation a total of 76 animals remained: 47 females and 29 males. Age was assessed by visual examination of teeth at the time of death and revealed 26 adult, 8 fawn and 42 yearling animals. The following tissues were examined by immunohistochemistry for PrPCWD using Ab99/97.6.1: obex, tonsil, retropharyngeal, submandibular, parotid, prescapular, axillary, inguinal, prefemoral and popliteal lymph nodes, recto-anal mucosal tissue and eye. Seventy-nine percent of animals (sixty) were found to be positive in at least one tissue; 49 were obex positive, 58 retropharyngeal positive, 56 tonsil positive, 48 recto-anal mucosal associated lymphoid tissue positive and 4 animals were positive for PrPCWD in the retina. Prion genotype was determined for all animals.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


P01.47

Quantifying the Species Barrier in Chronic Wasting Disease by a Novel in vitro Conversion Assay

Li, L1; Coulthart, MB2; Balachandran, A3; Chakrabartty, A4; Cashman, NR1 1University of British Columbia, Brain Research Centre, Canada; 2Public Health Agency of Canada, National Microbiology Laboratory, Canada; 3Animal Diseases Research Institute, Canada Food Inspection Agency, National Reference Laboratory for Scrapie and CWD, Canada; 4Ontario Cancer Institute and Department of Medical Biophysics, University of Toronto, Canada

Background: Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that can affect North American cervids (deer, elk, and moose). Although the risk of CWD crossing the species barrier and causing human disease is still unknown, however, definite bovine spongiform encephalopathy (BSE) transmission to humans as variant CJD (vCJD), it would seem prudent to limit the exposure of humans to CWD.

Aim: In view of the fact that BSE can be readily transmitted to non-bovid species, it is important to establish the species susceptibility range of CWD.

Methods: In vitro conversion system was performed by incubation of prions with normal brain homogenates as described before, and protease K (PK) resistant PrP was determined by immunoblotting with 6H4 monoclonal prion antibody.

Results: Our results demonstrate that PrPC from cervids (including moose) can be efficiently converted to a protease-resistant form by incubation with elk CWD prions, presumably due to sequence and structural similarities between these species. Interestingly, hamster shows a high conversion ratio by PrPCWD. Moreover, partial denaturation of substrate PrPC can apparently overcome the structural barriers between more distant species.

Conclusions: Our work correctly predicted the transmission of CWD to a wild moose. We find a species barrier for prion protein conversion between cervids and other species, however, this barrier might be overcome if the PrPC substrate has been partially denatured in a cellular environment. Such an environment might also promote CWD transmission to non-cervid species, *** including humans. Acid/GdnHCl-treated brain PrPC was a superior substrate for the in vitro conversion than PrPC treated at physiological pH. This has implications for the process by which the prion protein is converted in disease.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


Subject: Clinical Observations of BSE Infection in Red Deer Date: October 4, 2007 at 9:05 am PST

P04.80

Clinical Observations of BSE Infection in Red Deer

Steele, P1; Martin, S2; Jeffrey, M2; González, L2; Sisó, S2; Finlayson, J1; Hamilton, S1; Eaton, Samatha L1; Reid, Hugh W1; Todd, R1; Pang, Y1; Chianini, F1; Dagleish, MP1 1Moredun Research Institute, UK; 2Veterinary Laboratory Agency, Lasswade, UK

Observation of clinical signs is often the first step in the diagnosis of TSE diseases in experimental, farmed and wild animals. Clinical presentation of chronic wasting disease (CWD) infected deer varies widely as disease progresses and many clinical signs observed can be non-specific to TSE infection, however by terminal stage the majority of cases involve behavioural changes and loss of body condition. We present here the first description of clinical disease in deer experimentally infected with BSE. These data are part of the results of an ongoing project to investigate the susceptibility of UK red deer (Cervus elaphus elaphus) to BSE infection either by alimentary or intra-cerebral infection. Eighteen European red deer calves (mean 64 days old) were challenged intragastrically with 25g of BSE-infected bovine brain. Six challenged and 2 control deer were culled at 6 and 12 month post infection. These animals showed no clinical signs and no disease-specific PrP (PrPd) on immunohistochemistry (IHC) examination of a wide range of tissues collected at post-mortem. Six BSE-dosed and 4 negative control deer are still alive at time of writing (1384 dpi). Subsequently, 6 red deer of the same cohort (mean 341 days old) were challenged with 0.05g of BSE positive bovine brain material and 2 with sterile saline by the intracerebral route. Currently (1106 dpi), five of the six challenged animals have developed clinical signs and terminal disease confirmed by IHC and western blot detection of PrPd. Clinical signs similar to CWD cases have been observed including behavioral change, wide stance, lowered head, and excessive salivation. All animals had significant weight loss attributed to inability or unwillingness to feed, with inhalation pneumonia occurring in the case of one animal which is commonly observed in CWD cases. The first animal to show clinical signs was markedly different to the four subsequent cases. This animal had to be culled following several behavioral episodes causing physical injury. Our results prove for the first time that UK red deer are susceptible to intra-cerebral BSE infection and shows that the clinical presentation of disease shares many similarities to that recorded for CWD.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil Particles

Christopher J. Johnson1,2, Joel A. Pedersen3, Rick J. Chappell4, Debbie McKenzie2, Judd M. Aiken1,2*

1 Program in Cellular and Molecular Biology, University of Wisconsin-Madison, Madison, Wisconsin, United States of America, 2 Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison, Madison, Wisconsin, United States of America, 3 Department of Soil Science and Molecular and Environmental Toxicology Center, University of Wisconsin-Madison, Madison, Wisconsin, United States of America, 4 Biostatistics and Medical Informatics, University of Wisconsin Medical School, Madison, Wisconsin, United States of America

Soil may serve as an environmental reservoir for prion infectivity and contribute to the horizontal transmission of prion diseases (transmissible spongiform encephalopathies [TSEs]) of sheep, deer, and elk. TSE infectivity can persist in soil for years, and we previously demonstrated that the disease-associated form of the prion protein binds to soil particles and prions adsorbed to the common soil mineral montmorillonite (Mte) retain infectivity following intracerebral inoculation. Here, we assess the oral infectivity of Mte- and soil-bound prions. We establish that prions bound to Mte are orally bioavailable, and that, unexpectedly, binding to Mte significantly enhances disease penetrance and reduces the incubation period relative to unbound agent. Cox proportional hazards modeling revealed that across the doses of TSE agent tested, Mte increased the effective infectious titer by a factor of 680 relative to unbound agent. Oral exposure to Mte-associated prions led to TSE development in experimental animals even at doses too low to produce clinical symptoms in the absence of the mineral. We tested the oral infectivity of prions bound to three whole soils differing in texture, mineralogy, and organic carbon content and found soil- bound prions to be orally infectious. Two of the three soils increased oral transmission of disease, and the infectivity of agent bound to the third organic carbon-rich soil was equivalent to that of unbound agent. Enhanced transmissibility of soil-bound prions may explain the environmental spread of some TSEs despite the presumably low levels shed into the environment. Association of prions with inorganic microparticles represents a novel means by which their oral transmission is enhanced relative to unbound agent.

snip...

Discussion These experiments address the critical question of whether soil particle­bound prions are infectious by an environmentally relevant exposure route, namely, oral ingestion. Oral infectivity of soil particle­bound prions is a conditio sine qua non for soil to serve as an environmental reservoir for TSE agent. The maintenance of infectivity and enhanced transmissibility when TSE agent is bound to the common soil mineral Mte is remarkable given the avidity of the PrPTSE­Mte interaction [22]. One might expect the avid interaction of PrPTSE with Mte to result in the mineral serving as a sink, rather than a reservoir, for TSE infectivity. Our results demonstrate this may not be the case. Furthermore, sorption of prions to complex whole soils did not diminish bioavailability, and in two of three cases promoted disease transmission by the oral route of exposure. While extrapolation of these results to environmental conditions must be made with care, prion sorption to soil particles clearly has the potential to increase disease transmission via the oral route and contribute to the maintenance of TSE epizootics.

Two of three tested soils potentiated oral prion disease transmission. The reason for increased oral transmissibility associated with some, but not all, of the soils remains to be elucidated. One possibility is that components responsible for enhancing oral transmissibility were present at higher levels in the Elliot and Bluestem soils than in the Dodge soil. The major difference between the Dodge soil and the other two soils was the extremely high natural organic matter content of the former (34%, [22]). The Dodge and Elliot soils contained similar levels of mixed-layer illite/smectite, although the contribution of smectite layers was higher in the Dodge soil (14%­16%, [22]). The organic matter present in the Dodge soil may have obstructed access of PrPTSE to sorption sites on smectite (or other mineral) surfaces.

The mechanism by which Mte or other soil components enhances the oral transmissibility of particle-bound prions remains to be clarified. Aluminosilicate minerals such as Mte do not provoke inflammation of the intestinal lining [39]. Although such an effect is conceivable for whole soils, soil ingestion is common in ruminants and other mammals [25]. Prion binding to Mte or other soil components may partially protect PrPTSE from denaturation or proteolysis in the digestive tract [22,40] allowing more disease agent to be taken up from the gut than would otherwise be the case. Adsorption of PrPTSE to soil or soil minerals may alter the aggregation state of the protein, shifting the size distribution toward more infectious prion protein particles, thereby increasing the specific titer (i.e., infectious units per mass of protein) [41]. In the intestine, PrPTSE complexed with soil particles may be more readily sampled, endocytosed (e.g., at Peyer's patches), or persorbed than unbound prions. Aluminosilicate (as well as titanium dioxide, starch, and silica) microparticles, similar in size to the Mte used in our experiments, readily undergo endocytotic and persorptive uptake in the small intestine [42­44]. Enhanced translocation of the infectious agent from the gut lumen into the body may be responsible for the observed increase in transmission efficiency.

Survival analysis indicated that when bound to Mte, prions from both BH and purified PrPTSE preparations were more orally infectious than unbound agent. Mte addition influenced the effective titer of infected BH to a lesser extent than purified PrPTSE. Several nonmutually exclusive factors may explain this result: (1) other macromolecules present in BH (e.g., lipids, nucleic acids, other proteins) compete with PrPTSE for Mte binding sites; (2) prion protein is more aggregated in the purified PrPTSE preparation than in BH [45], and sorption to Mte reduces PrPTSE aggregate size, increasing specific titer [41]; and (3) sorption of macromolecules present in BH to Mte influences mineral particle uptake in the gut by altering surface charge or size, whereas the approximately 1,000-fold lower total protein concentration in purified PrPTSE preparations did not produce this effect.

We previously showed that other inorganic microparticles (kaolinite and silicon dioxide) also bind PrPTSE [22]. All three types of microparticles are widely used food additives and are typically listed as bentonite (Mte), kaolin (kaolinite), and silica (silicon dioxide). Microparticles are increasingly included in Western diets. Dietary microparticles are typically inert and considered safe for consumption by themselves, do not cause inflammatory responses or other pathologies, even with chronic consumption, and are often sampled in the gut and transferred from the intestinal lumen to lymphoid tissue [39,46,47]. Our data suggest that the binding of PrPTSE to dietary microparticles has the potential to enhance oral prion disease transmission and warrants further investigation.

In conclusion, our results provide compelling support for the hypothesis that soil serves as a biologically relevant reservoir of TSE infectivity. Our data are intriguing in light of reports that naïve animals can contract TSEs following exposure to presumably low doses of agent in the environment [5,7­9]. We find that Mte enhances the likelihood of TSE manifestation in cases that would otherwise remain subclinical (Figure 3B and 3C), and that prions bound to soil are orally infectious (Figure 5). Our results demonstrate that adsorption of TSE agent to inorganic microparticles and certain soils alter transmission efficiency via the oral route of exposure.

snip...

full text is here:

http://pathogens.plosjournals.org/perlserv/?request=get-document&doi=10.1371/journal.ppat.0030093


http://pathogens.plosjournals.org/perlserv/?request=get-pdf&file=10.1371_journal.ppat.0030093-L.pdf


http://pathogens.plosjournals.org/perlserv/?request=get-pdf&file=10.1371_journal.ppat.0030093-S.pdf


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0611&L=sanet-mg&T=0&F=&S=&m=1742&P=7260


From: "Terry S. Singeltary Sr." Subject: CWD UPDATE 88 AUGUST 31, 2007

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&P=450


Date: Wed, 29 Aug 2007 21:13:08 -0500

From: "Terry S. Singeltary Sr."

Subject: CWD NEW MEXICO RECORDS IT'S 19 CASE (near Texas border again)

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=26079


Monitoring the Potential Transmission of Chronic Wasting Disease to Humans Using a Hunter Registry Database in Wyoming (405 lines)

From: Terry S. Singeltary Sr. Date: Thu, 30 Aug 2007 21:23:42 -0500

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&F=&S=&P=27654


J Biol Chem. 2007 Aug 20; : 17709374

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

####################################

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

###################################

FULL TEXT ;

http://www.jbc.org/cgi/

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=21267


From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was

attached

to your email), we did not say CWD in humans will present like variant

CJD.

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

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

-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Aguzzi warns of CWD danger

The TSE family of diseases also includes chronic wasting disease (CWD) in deer, a condition that has spread in the US in recent years (Nature 416, 569; 2002). Speaking at the Days of Molecular Medicine conference in La Jolla in March, prion expert Adriano Aguzzi issued a strong warning against underestimating this form of TSE.

"For more than a decade, the US has by-and-large considered mad cows to be an exquisitely European problem. The perceived need to protect US citizens from this alien threat has even prompted the deferral of blood donors from Europe," he said. "Yet the threat-from-within posed by CWD needs careful consideration, since the evidence that CWD is less dangerous to humans than BSE is less-than-complete. Aguzzi went on to point out that CWD is arguably the most mysterious of all prion diseases.

"Its horizontal spread among the wild population is exceedingly efficient, and appears to have reached a prevalence unprecedented even by BSE in the UK at its peak. The pathogenesis of CWD, therefore, deserves a vigorous research effort. Europeans also need to think about this problem, and it would be timely and appropriate to increase CWD surveillance in Europe too." Aguzzi has secured funding from the National Institutes of Health to investigate CWD, and the effort will be lead by Christina Sigurdson in his department at the University of Zurich. KAREN BIRMINGHAM, LONDON

This quote from Dr. Gambetti is especially significant since he is the rather cautious TSE researcher under contract with the Centers for Disease Control to examine the brains of individuals who have died of CJD. -----------------

Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, said all deer should be tested for chronic wasting disease before any processing is done.

"There is no way around it," he said. "Nobody should touch that meat unless it has been tested." --------------------------------------

http://www.ledger-enquirer.com/mld/...ion/3954298.htm ================================================== ====

'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.'

http://www.bse.org.uk/files/yb/1991/01/04004001.pdf


see full text ;

Re: Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES)

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165


doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." ............................

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext


http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf


see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408


MR. xxxxxxx, I strongly urge you to read in full and uderstand the following about the TWO atyipcal H-BASE mad cow cases in Texas and Alabama ;


FC5.5.1

BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc C-terminal Truncated Fragments

Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux, MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4; Monaco, S3 1University of Verona, of Neurological and Visual Sciences, Italy; 2CEA, IMETI/SEPIA, France; 3University of Verona, Neurological and Visual Sciences, Italy; 4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps Research Insitute, USA

The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human prion disease, remains still unknown. The marked disease phenotype heterogeneity observed in sCJD is thought to be influenced by the type of proteinase K-resistant prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V) codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE) and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type, distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all genotypes,; (ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V- 2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD subtype M/V-2 shared molecular and pathological features with an atypical form of BSE, named BASE, thus suggesting a potential link between the two conditions. This connection was further confirmed after 2D-PAGE analysis, which showed an identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally inoculated with brain homogenates from BASE. Samples were separated by using a twodimensional electrophoresis (2D-PAGE) followed by immunoblotting. We here show that the PrPSc pattern obtained in infected primates is identical to BASE and sCJD MV-2 subtype. These data strongly support the link, or at least a common ancestry, between a sCJD subtype and BASE.

This work was supported by Neuroprion (FOOD-CT-2004-506579)

FC5.5.2

Transmission of Italian BSE and BASE Isolates in Cattle Results into a Typical BSE Phenotype and a Muscle Wasting Disease

Zanusso, G1; Lombardi, G2; Casalone, C3; D'Angelo, A4; Gelmetti, D2; Torcoli, G2; Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3; Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2 1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER, Italy; 3IZSPLVA, Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta, Italy

The clinical phenotype of bovine spongiform encephalopathy has been extensively reported in early accounts of the disorder. Following the introduction of statutory active surveillance, almost all BSE cases have been diagnosed on a pathological/molecular basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance system has uncovered atypical BSE cases, which are characterized by distinct conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose clinicopathological phenotypes remain unknown. We recently reported two Italian atypical cases with a PrPSc type similar to BSE-L, pathologically characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A major limitation of transmission studies to mice is the lack of reliable information on clinical phenotype of BASE in its natural host. In the present study, we experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates by i.c. route. BASE infected cattle showed survival times significantly shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with previous observations in TgBov mice. Clinically, BSE-infected cattle developed a disease phenotype highly comparable with that described in field BSE cases and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle developed an amyotrophic disorder accompanied by mental dullness. The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.

P01.34

Pathological Interaction Between Protein Misfolding Disorders: Prions and Alzheimer's Disease

Morales, R; Estrada, L; Castilla, J; Soto, C University of Texas Medical Branch, Neurology, USA

Protein Misfolding Disorders (PMD) include several diverse diseases, such Alzheimer's, Parkinson's, Transmissible Spongiform Encephalopathies, Diabetes Type II and various systemic amyloidosis. The central event in these diseases is the accumulation of a misfolded, ß-sheet rich aggregated form of a naturally expressed protein. In vitro studies have shown that protein misfolding and aggregation follows a seedingnucleation mechanism similar to the process of crystallization. In this model, the limiting step is the formation of small oligomeric intermediates that act as seeds to catalyze the polymerization process. The seeding-nucleation model provides a rationale and plausible explanation for the infectious nature of prions. Infectivity lies on the capacity of preformed stable misfolded oligomeric proteins to act as a seed to catalyze the misfolding and aggregation process. The mechanism of misfolding and aggregation is similar in all PMD suggesting that misfolded aggregates have an inherent capability to be transmissible. Moreover, it has been shown that oligomeric seeds formed by one protein can accelerate the misfolding and aggregation of another protein, by a process termed cross-seeding. Our current study aims to assess the potential molecular cross-talk among PMD in vivo. For this purpose we inoculated with prions a transgenic mice model of Alzheimer's disease (tg2576) that develops typical amyloid plaques over time. 45, 303 and 365 days old transgenic and wild type mice were inoculated intraperitoneally with RML prions. We found significant diminution in prion incubation periods for tg2576 mice compared to age matched wild type controls. Moreover, a time dependent effect was observed, where the shorter incubation period was observed in animals containing larger number of amyloid plaques. Inoculation of tg2576-RML prions into wild type mice showed incubation periods similar to the original infectious material, suggesting that strains characteristics are maintained. In vitro data showed cross-seeding aggregation between PrPSc and Aß. Our findings suggest an interaction between Alzheimer's and prion pathologies, indicating that one protein misfolding process may be an important risk factor for the development of a second perhaps more prevalent disease.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


Subject: Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route Date: September 29, 2007 at 12:50 pm PST

P04.27

Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route

Holznagel, E1; Yutzy, B1; Deslys, J-P2; Lasmézas, C2; Pocchiari, M3; Ingrosso, L3; Bierke, P4; Schulz-Schaeffer, W5; Motzkus, D6; Hunsmann, G6; Löwer, J1 1Paul-Ehrlich-Institut, Germany; 2Commissariat à l´Energie Atomique, France; 3Instituto Superiore di Sanità, Italy; 4Swedish Institute for Infectious Disease control, Sweden; 5Georg August University, Germany; 6German Primate Center, Germany

Background:

In 2001, a study was initiated in primates to assess the risk for humans to contract BSE through contaminated food. For this purpose, BSE brain was titrated in cynomolgus monkeys.

Aims:

The primary objective is the determination of the minimal infectious dose (MID50) for oral exposure to BSE in a simian model, and, by in doing this, to assess the risk for humans. Secondly, we aimed at examining the course of the disease to identify possible biomarkers.

Methods:

Groups with six monkeys each were orally dosed with lowering amounts of BSE brain: 16g, 5g, 0.5g, 0.05g, and 0.005g. In a second titration study, animals were intracerebrally (i.c.) dosed (50, 5, 0.5, 0.05, and 0.005 mg).

Results:

In an ongoing study, a considerable number of high-dosed macaques already developed simian vCJD upon oral or intracerebral exposure or are at the onset of the clinical phase. However, there are differences in the clinical course between orally and intracerebrally infected animals that may influence the detection of biomarkers.

Conclusions:

Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate. The difference in the incubation period between 5 g oral and 5 mg i.c. is only 1 year (5 years versus 4 years). However, there are rapid progressors among orally dosed monkeys that develop simian vCJD as fast as intracerebrally inoculated animals.

The work referenced was performed in partial fulfilment of the study "BSE in primates" supported by the EU (QLK1-2002-01096).

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


Subject: Transmission of BSE to Cynomolgus Macaque, a Non-human Primate; Development of Clinical Symptoms and Tissue Distribution of PrPSC Date: September 29, 2007 at 12:45 pm PST

P03.137

Transmission of BSE to Cynomolgus Macaque, a Non-human Primate; Development of Clinical Symptoms and Tissue Distribution of PrPSC

Yamakawa, Y1; Ono, F2; Tase, N3; Terao, K3; Tannno, J3; Wada, N4; Tobiume, M5; Sato, Y5; Okemoto-Nakamura, Y1; Hagiwara, K1; Sata, T5 1National Institure of Infectious diseases, Cell biology and Biochemistry, Japan; 2Corporation for Production and Research Laboratory Primates., Japan; 3National Institure of Biomedical Innovation, Tsukuba Primate Reserch Center, Japan; 4Yamauchi Univ., Veterinary Medicine, Japan; 5National Institure of Infectious diseases, Pathology, Japan

Two of three cynomolgus monkeys developed abnormal neuronal behavioral signs at 30-(#7) and 28-(#10) months after intracerebral inoculation of 200ul of 10% brain homogenates of BSE affected cattle (BSE/JP6). Around 30 months post inoculation (mpi), they developed sporadic anorexia and hyperekplexia with squeal against environmental stimulations such as light and sound. Tremor, myoclonic jerk and paralysis became conspicuous during 32 to 33-mpi, and symptoms become worsened according to the disease progression. Finally, one monkey (#7) fell into total paralysis at 36-mpi. This monkey was sacrificed at 10 days after intensive veterinary care including infusion and per oral supply of liquid food. The other monkey (#10) had to grasp the cage bars to keep an upright posture caused by the sever ataxia. This monkey was sacrificed at 35-mpi. EEG of both monkeys showed diffuse slowing. PSD characteristic for sporadic CJD was not observed in both monkeys. The result of forearm movement test showed the hypofunction that was observed at onset of clinical symptoms. Their cognitive function determined by finger maze test was maintained at the early stage of sideration. However, it was rapidly impaired followed by the disease progression. Their autopsied tissues were immunochemically investigated for the tissue distribution of PrPSc. Severe spongiform change in the brain together with heavy accumulation of PrPSc having the type 2B/4 glycoform profile confirmed successful transmission of BSE to Cynomolgus macaques. Granular and linear deposition of PrPSC was detected by IHC in the CNS of both monkeys. At cerebral cortex, PrPSC was prominently accumulated in the large plaques. Sparse accumulation of PrPSc was detected in several peripheral nerves of #7 but not in #10 monkey, upon the WB analysis. Neither #7 nor #10 monkey accumulated detectable amounts of PrPSc in their lymphatic organs such as tonsil, spleen, adrenal grands and thymus although PrPSc was barely detected in the submandibular lymph node of #7 monkey. Such confined tissue distribution of PrPSc after intracerebral infection with BSE agent is not compatible to that reported on the Cynomolgus macaques infected with BSE by oral or intra-venous (intra-peritoneal) routs, in which PrPSc was accumulated at not only CNS but also widely distributed lymphatic tissues.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


P04.49

Case Report of Variant Creutzfeldt-Jakob Disease in a Macaque after Blood Transfusion

Lescoutra-Etchegaray, N1; Ruchoux, MM1; Correia, E1; Jolit, A1; Freire, S1; Lasmezas, CI2; Deslys, JP1; Comoy, E1 1CEA/DSV/IMETI/SEPIA, France; 2Scripps Florida, USA

A fourth human case of probable transmission of vCJD through transfusion has now been reported but a number of features affecting transfusion-related infection remain imprecise, including infectious dose, length of incubation period and critical infectious window of blood donors.

We report here the first case of experimental transmission of vCJD in primates by blood transfusion. Experimental infection of Cynomolgus macaque has been demonstrated to be a sensitive model for the investigation of human prion diseases, inducing similar distribution of infectivity in peripheral lymphoid tissues and equivalent brain pathology. In our study, transfusion was performed with 40 ml of whole blood drawn from a vCJD-infected macaque at the terminal stage of the disease. Clinical symptoms of vCJD appeared in the recipient animal after five years of incubation. The total amount of infectivity in the transfused blood was approximately 106 fold lower than in the brain (titration still in progress). In several animals infected intravenously with brain homogenate, the presence of PrPres in serial lymph nodes biopsies and in other organs at autopsy was examined and results will be presented.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


P04.51

Atypical Presentation of Variant Creutzfeldt-Jakob Disease in a 73 Year Old Blood Transfusion Recipient

Wroe, S1; Pal, S1; Webb, T1; Alner, K2; Hewitt, P3; Brander, S4; Wadsworth, JD5; Collinge, J1 1National Hospital for Neurology and Neurosurgery, National Prion Clinic, UK; 2National Hospital for Neurology and Neurosurgery, Department of Neuropsychology, UK; 3Health Protection Agency, UK; 4National Hospital for Neurology and Neurosurgery, Department of Neuropathology, UK; 5Institute of Neurology, UCL, UK

We report atypical presentation of variant Creutzfeldt-Jakob Disease (vCJD) identified ante-mortem in a 73 year-old recipient of blood products. This patient was transfused following orthopaedic surgery in December 1997. Tracing of blood products identified a single unit of non-leucodepleted red cells from an individual who developed neuropathologically confirmed vCJD eleven months after donation. Nine years post transfusion, this individual was referred to the National Prion Clinic for specialist investigation. Six years post transfusion the recipient complained of fluctuating fatigue and impaired concentration. At this time neurological examination and MRI brain (T1/T2 weighted/DWI) were normal. Progressive symptoms emerged six months later with imbalance and deteriorating cognition. Examination two months after onset of neurological symptoms demonstrated cognitive deficits, dyspraxia or visuospatial dysfunction and normal motor, sensory and gait examination. Six weeks later cognitive impairment was identified alongside tremulousness, impaired manual dexterity and limb ataxia. Serological investigations were normal. MRI (T1/T2 weighted/FLAIR/DWI) demonstrated prominent signal change throughout the dorsal thalamus, consistent with vCJD. PRNP genotyping revealed no mutations and homozygosity for methionine at codon 129. The prolonged incubation period of vCJD and possibility of asymptomatic carrier states pose major public health concerns. This case highlights the significant risk encountered by recipients of contaminated blood products and the necessity for their specialist monitoring.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


P04.36

Enhanced Surveillance of Persons Identified as at Increased Risk of CJD Due to Blood Transfusion or Healthcare Procedures

Brookes, D1; Chow, Y1; Ward, HJT2; Will, RG2; Hewitt, P3; Gill, ON1 1HPA, CJD, UK; 2National CJD Surveillance Unit, UK; 3Colindale, NHS Blood and Tissue, UK

Introduction:

Reports of four iatrogenic transmissions of variant-CJD (vCJD) infection in the UK (all due to transfusion of blood from donors who later developed vCJD), evidence from iatrogenic transmissions of sporadic CJD and experimental work on CJD infectivity in tissues and on healthcare instruments have given rise to concern about the risks of iatrogenic transmission of CJD. This risk warrants a) certain public health precautions, and b) follow-up of individuals with identified risks in order to gain evidence about their risks and ensure appropriate management of these risks. Evidence of transmission via iatrogenic routes is important to inform public health measures and so prevent ongoing transmission of CJD.

Methods:

The Health Protection Agency and Health Protection Scotland holds details of persons identified as 'at-risk' of vCJD due to blood transfusion and of persons identified as 'at-risk' of CJD (of any type) from other healthcare procedures. The GPs/clinicians of all persons identified as 'at-risk' for public health purposes are provided with: information; risk assessment updates; advice on

public health precautions and advice on referral to specialist care. Procedures are being established to obtain enhanced surveillance data on these individuals, including: clinical status updates, date and cause of death, surplus tissue and blood specimens, and postmortem investigations.

Results:

Persons 'at-risk' of CJD have experienced a range of exposures. Estimated risks are uncertain and overlapping. Some individuals - recipients of vCJD implicated blood components - are considered to be at a clearly higher risk of infection: active follow-up is currently conducted for these individuals. In time, the enhanced surveillance of persons at increased risk of CJD will provide estimates of transmission risks and of the impact of iatrogenic exposures on mortality. Conclusion: Knowledge about iatrogenic transmission of CJD is being gained by the follow-up of individuals who have been identified as 'at-risk' of CJD in the UK. This enhanced surveillance may need to be sustained for many years.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



Prions in Plasma of Patients with Sporadic Creutzfeldt-Jakob Disease Safar, J.G1,3, Geschwindm M.D2,3, Letessier, F1, Kuo, A1, Pomeroy, K1, Deering, C1, Serban, A1, Groth, D1, Miller, B.L2,3, DeArmond, S.J1,4, Prusiner, S.B1,3,5 1Institute for Neurodegenerative Diseases, 2Memory and Aging Center, Departments of 3Neurology, 4Pathology, and 5Biochemistry and Biophysics, University of California, San Francisco, California 94143

http://www.prion2007.com/pdf/Prion%20Friday.pdf


http://www.prion2007.com/pdf/Prion%20Thursday.pdf


http://www.prion2007.com/pdf/Prion%20Wednesday.pdf


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

Date: Mon, 24 Sep 2007 21:31:55 -0500

I suggest that you all read the data out about h-BASE and sporadic CJD, GSS, blood, and some of the other abstracts from the PRION2007. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744


*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OF POKER INDEED GOES UP. ...TSS

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were ''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007 11:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779


BRITISH MEDICAL JOURNAL

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... Terry S Singeltary (2 January 2000)

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries." ...snip...END

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


Full Text Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama


Suppressed peer review of Harvard study October 31, 2002.

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf


(this is funny really, see below)

a funny thing happened. I was honored with having my name added to this list. they slammed for being an uneducated scientist, on a list with scientists that seemed to be interested in my thoughts, or the data I was putting out, and they also said that I was a high school drop flunky, quoting from an article I had writing and it published in the journal of neurology some years ago, and the sad part is, I was correct. most everything in that article has come to pass. you can take a look here ;

Terry Singletary -- A retired machinist and high school dropout, Terry Singletary suffered the tragic loss of his mother to "sporadic" Creutzfeldt-Jakob disease (CJD) in 1997. Desperate to find an explanation for his mother's death, he has devoted himself to the sad and fruitless task of connecting her death to her diet. Various reports confirm that Mrs. Singletary's life was claimed by the most common sub-type of CJD (one that accounts for 70 percent of "sporadic" cases). Sporadic CJD, unlike its newer "variant," is not linked to meat.

As the self-appointed international coordinator of CJD Watch, an organization he co-founded with social worker Deborah Oney, Singletary is cited in media reports as an apparent expert on tracking mad cow disease. This despite his lack of formal education and the absence for support from any credible academic, medical or scientific authority. His sensationalist allegations about the safety of U.S. beef have found their way into hundreds of newspapers and broadcasts. Singletary moderates a mad-cow discussion forum run by a vegetarian activist group; his contributions account for more than half the traffic on the "BSE-L" mailing list, which is generally read by real scientists. Animal rights activists and other food-scare artists frequently refer to him as "Dr. Terry Singletary," apparently an honorary degree as he has yet to finish high school.

Like many activists, Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef. Relying entirely on shallow circumstantial evidence and frequent repetition of claims which have been publicly refuted as false, he also blindly insists upon a mad-cow with Alzheimer's, Parkinson's, and Lou Gehrig 's disease. His specific allegations have been clearly refuted by Centers for Disease Countrol and Prevention scientists in the journal Neurology.

http://www.consumerfreedom.com/article_detail.cfm/article/138


ARTICLE IN QUESTION ABOVE FOLLOWS ;

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


3 MONTHS LATER IN A BOOK INTERVIEW, VENDICATION OF SORTS

THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations.

ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded

that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/


IN REALITY, sporadic CJD is 1 in 9,000 in 50 years of age and above, and that's with a inadequate or what I call passive surveillance system. see below ;

Dr. William Shulaw, a veterinarian with The Ohio State University extension service, is involved in a nationwide program to eradicate scrapie, the form of BSE found in sheep.

Shulaw said the chances of a person getting sporadic Creutzfeldt-Jakob disease is about one in a million. But that's the total population, infants, children, adults and the elderly. Chances increase to one in 9,000 when the group is restricted to those age 50 and older.

http://www.pjstar.com/stories/082207/REG_BE523NH6.049.php

FURTHER into my journal of neurology article, some years later, again, sadly, I was proven correct ;

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


AND YES, to the na Sayers, you are more likely to be killed by a car wreck than by CJD, for now, but my mother, and my neighbors mother, neither one were killed in a car wreck, they both died from CJD, confirmed, my mother dying from an exceedingly, rare phenotype of the sporadic CJDs i.e hvCJD, and there are others, and the numbers are increasing for sporadic CJD. but most all of us have been exposed to the TSE agent, one way or the other. however, the zillion dollar questions are still the same, why do only some humans and animals go clinical and die
from the TSE agent, and of course the origin, which to me is not as important as what we do know, amplification and transmission, i cannot stress this enough, the transmission studies do not lie.

I guess if I could say one thing about this Canadian vs USA mad cow situation, I would say this would have been, should have been resolved long ago. but instead, everyone ignored it, and now, all they want to do is sit around and point fingers at each other. my suggestion, test, test, test, I would say at least 1 million head a year, for five years.

the june 2004 enhanced bse surveillance does not even count (see why bio-rad, tss below). and that's not really enough, i would like to see 100% testing for consumption of either human or pet food for TSE. then you might have an answer to what you really have in relations to BSE and BASE H or L type. but the testing protocols and sampling protocols must be adhered too, this did not happen in the 2004 enhanced cover-up, and they could not even get that right. enforce the feed ban, srm removal (in fact you may have to enhance that with the atypicals, due to more tissue/organs tissue infectivity).

NOW, about that June 2004 enhanced BSE cover-up program ;

United States District Court, District of Arizona

USA v Roland Emerson Farabee 2: 07-wi-0001-01-EHC

Proceeding Type: Waiver/Plea

Judge: Honorable Earl H Carroll Courtroom: Phoenix Courtroom #501, 5th Floor

Date: 01/17/2007 Court Reporter: Candy Potter Time: 01:30 PM Courtroom Deputy: Bobbi Hightower

Counts: (may not reflect all counts) 18:641 and 2 (Theft of Government Money and Adiding and Abetting) 1 18: 1341 and 2 (Mail Fruad and Aiding and Abetting) 2 18:1343 and 2 (Wire Fraud and Aiding and Abetting) - 3

U.S. Attorney: Long, Robert

---------------------------------------------------------------------------- ---- Defense Attorney(s): Attorney Phone: Attorney Designation: McDonald, Jr., A. Melvin retained

http://www.azd.uscourts.gov/azd/callive.nsf/552b822403e5434a07256d6c007cc3ef/256244d68b5f47780725725e006aac31?OpenDocument&Click=


if my siphering is correct, that would be about another 2600 potential mad cows that went into the food chain. add that to these ;

It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing.< href="http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html">http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html

and add these in ; UPI previously reported that from 2001 to 2003 the USDA collected the wrong part of the brain in more than 200 cows that were being screened as part of its BSE surveillance program. The USDA documents also indicate the agency never was able to identify or test 52 cows that came into the United States in 2001 along with the Washington cow that tested positive in 2003. Of these, 11 were considered to be "high risk" because they were born within a year and on the same premises as the infected cow. These cows may have gone into the food supply and been consumed by people. The concern is humans can contract a fatal brain disease from eating beef products contaminated with the mad cow pathogen.

http://www.terradaily.com/upi/2005/WWN-UPI-20050721-17422400-bc-us-madcow.html

NOT to forget what Paul Brown TSE expert at CDC said ; THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ; CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006 The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade. The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old. These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen. "The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that." Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive. USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general. "Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...

http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm

PAUL BROWN COMMENT TO ME ON THIS ISSUE Tuesday, September 12, 2006 11:10 AM "Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."

OR, what the Honorable Phyllis Fong of the OIG found ; Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III Report No. 50601-10-KC January 2006 Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

Greetings list members, IF you remember correctly, i posted this ;

Subject: Re: USDA/APHIS JUNE 2004 'ENHANCED' BSE/TSE COVER UP UPDATE DECEMBER 19, 2004 USA Date: Thu, 30 Dec 2004 12:27:06 -0600 From: "Terry S. Singeltary Sr. BSE-L

snip...

OH, i did ask Bio-Rad about this with NO reply to date; -------- Original Message -------- Subject: USA BIO-RADs INCONCLUSIVEs Date: Fri, 17 Dec 2004 15:37:28 -0600 From: "Terry S. Singeltary Sr." To: susan_berg@bio-rad.com

Hello Susan and Bio-Rad, Happy Holidays! I wish to ask a question about Bio-Rad and USDA BSE/TSE testing and there inconclusive. IS the Bio-Rad test for BSE/TSE that complicated, or is there most likely some human error we are seeing here? HOW can Japan have 2 positive cows with No clinical signs WB+, IHC-, HP- , BUT in the USA, these cows are considered 'negative'? IS there more politics working here than science in the USA? What am I missing?

-------- Original Message -------- Subject: Re: USDA: More mad cow testing will demonstrate beef's safety Date: Fri, 17 Dec 2004 09:26:19 -0600 From: "Terry S. Singeltary Sr."

snip...end

Experts doubt USDA's mad cow results

snip...END

WELL, someone did call me from Bio-Rad about this, however it was not Susan Berg. but i had to just about take a blood oath not to reveal there name. IN fact they did not want me to even mention this, but i feel it is much much to important. I have omitted any I.D. of this person, but thought I must document this ; Bio-Rad, TSS phone conversation 12/28/04 Finally spoke with ;

Bio-Rad Laboratories 2000 Alfred Nobel Drive Hercules, CA 94547 Ph: 510-741-6720 Fax: 510-741-5630 Email: XXXXXXXXXXXXXXXXXX at approx. 14:00 hours 12/28/04, I had a very pleasant phone conversation with XXXX XXXXX about the USDA and the inconclusive BSE testing problems they seem to keep having. X was very very cautious as to speak directly about USDA and it's policy of not using WB. X was very concerned as a Bio-Rad official of retaliation of some sort. X would only speak of what other countries do, and that i should take that as an answer. I told X I understood that it was a very loaded question and X agreed several times over and even said a political one. my question;

Does Bio-Rad believe USDA's final determination of False positive, without WB, and considering the new atypical TSEs not showing positive with -IHC and -HP ??? ask if i was a reporter. i said no, i was with CJD Watch and that i had lost my mother to hvCJD. X did not want any of this recorded or repeated. again, very nervous, will not answer directly about USDA for fear of retaliation, but again said X tell me what other countries are doing and finding, and that i should take it from there. "very difficult to answer" "very political" "very loaded question" outside USA and Canada, they use many different confirmatory tech. in house WB, SAF, along with IHC, HP, several times etc. you should see at several talks meetings (TSE) of late Paris Dec 2, that IHC- DOES NOT MEAN IT IS NEGATIVE. again, look what the rest of the world is doing. said something about Dr. Houston stating; any screening assay, always a chance for human error. but with so many errors (i am assuming X meant inconclusive), why are there no investigations, just false positives? said something about ''just look at the sheep that tested IHC- but were positive''. ... TSS

------- Original Message --------
Subject: Your questions Date: Mon, 27 Dec 2004 15:58:11 -0800 From: To: flounder@wt.net

Hi Terry: ............................................snip Let me know your phone number so I can talk to you about the Bio-Rad BSE test. Thank you Regards Bio-Rad Laboratories 2000 Alfred Nobel Drive Hercules, CA 94547 Ph: 510-741-6720 Fax: 510-741-5630 Email: ================================= END...TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########

THE SEVEN SCIENTIST REPORT *** http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle 03-025IFA 03-025IFA-2 Terry S. Singeltary 9/13/2005

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

ALL IN ALL, it's a terribly failed system of TSE surveillance and attempted erradication of this agent in the USA, a failed policy driven by industry greed and ignorance. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&F=P&P=10277

THAT TEJAS MAD COW THAT WAS COVERED-UP FOR ABOUT 7 PLUS MONTHS, i confirmed 7 months previously, but the other Tejas mad cow, got away, went to the render without testing at all ; TSS REPORT ON 2ND TEJAS MAD COW Mon, 22 Nov 2004 17:12:15 -0600 (the one that did NOT get away, thanks to the Honorable Phyllis Fong)

-------- Original Message --------

Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ??? Date: Mon, 22 Nov 2004 17:12:15 -0600 From: "Terry S. Singeltary Sr." To: Carla Everett References: <[log in to unmask]> <[log in to unmask] us>

Greetings Carla,still hear a rumor;

Texas single beef cow not born in Canada no beef entered the food chain?

and i see the TEXAS department of animal health is ramping up forsomething, but they forgot a url for update?I HAVE NO ACTUAL CONFIRMATION YET...can you confirm???terry

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

-------- Original Message --------
Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ??? Date: Fri, 19 Nov 2004 11:38:21 -0600 From: Carla Everett To: "Terry S. Singeltary Sr." References: <[log in to unmask]>

The USDA has made a statement, and we are referring all callers to the USDA web site. We have no informationabout the animal being in Texas. CarlaAt 09:44 AM 11/19/2004,

you wrote:>Greetings Carla,>>i am getting unsubstantiated claims of this BSE 'inconclusive' cow is from>TEXAS. can you comment on this either way please?>>thank you,>Terry S. Singeltary Sr.>> ===================

-------- Original Message -------- Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ??? Date: Mon, 22 Nov 2004 18:33:20 -0600 From: Carla Everett To: "Terry S. Singeltary Sr." References: <[log in to unmask]> <[log in to unmask] us> <[log in to unmask]> <[log in to unmask] us> <[log in to unmask]>

our computer department was working on a place holder we could postUSDA's announcement of any results. There are no results to be announced tonightby NVSL, so we are back in a waiting mode and will post the USDA announcementwhen we hear something.At 06:05 PM 11/22/2004,

you wrote:>why was the announcement on your TAHC site removed?>>Bovine Spongiform Encephalopathy:>November 22: Press Release title here >>star image More BSE information>>>>terry>>Carla Everett wrote:>>>no confirmation on the U.S.' inconclusive test...>>no confirmation on location of animal.>>>>>>========================== ==========================

THEN, 7+ MONTHS OF COVER-UP BY JOHANN ET AL! no doubt about it now $$$

NO, it's not pretty, hell, im not pretty, but these are the facts, take em or leave em, however, you cannot change them.

with kindest regards,

I am still sincerely disgusted and tired in sunny Bacliff, Texas USA 77518

Terry S. Singeltary Sr.

FULL 130 LASHINGS TO USDA BY OIG again

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


Link: TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&T=0&P=23557


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&T=0&P=720


MR. xxxxxxxxx, THAT MAD COW IN TEXAS WOULD HAVE NEVER BEEN CONFIRMED IF NOT FOR THE HONORABLE

PHYLLIS FONG OF THE OIG.

I know I have missed something, but not sure what it is. I hope if nothing else, I hope you take the time to read all of this,

I could even go further into this and try and explain about Mission Texas and the late Richard Marsh findings, and the BSE farmers

and there wives with sporadic CJD, and that part of the equation to this long term nightmare, but I am afraid I have been too long

winded already. IF not, and your still with me, i have documented here ;

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf


NOW BACK TO THOSE FARMERS WITH BSE HERDS THAT DIED FROM SPORADIC CJD

CJD FARMERS WIFE 1989

http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf


http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf


cover-up of 4th farm worker ???

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf


http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf


CONFIRMATION OF CJD IN FOURTH FARMER

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf


now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf


4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf


SEE FULL TEXT ;

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276


Mr. xxxxx,

I hope you study this, I hope it helps answer a few questions you might not have answered yourself.

I tried/did help r-calf Dr. Thornsberry et al, but they were only interested in one thing, themselves.

the history of that is here ;

*** http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=12


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=24


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=36


http://ranchers.net/forum/viewtopic.php?t=15704&postdays=0&postorder=asc&start=48


EXPORTATION AND IMPORTATION OF ANIMALS AND ANIMAL PRODUCTS: BSE; MRR AND IMPORTATION OF COMMODITIES, 65758-65759 [E6-19042]

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0701&L=sanet-mg&T=0&P=3854


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0611&L=sanet-mg&T=0&P=3381


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=498


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&T=0&P=10277


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0701&L=sanet-mg&T=0&P=9972


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=4492


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=2583


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=2470


BOTTOM LINE ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............

http://www.oie.int/eng/Session2007/RF2006.pdf


----- Original Message -----
From: Terry S. Singeltary Sr. To: Terry S. Singeltary Sr. Sent: Thursday, March 29, 2007 3:44 PM Subject: BSE; MRR; Importation of Live Bovines and Products Derived from Bovines Commodities

Sent: Sunday, January 28, 2007 9:12 PM Subject: BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01 COMMENT SUBMISSION

----- Original Message ----- From: Terry S. Singeltary Sr. To: Terry S. Singeltary Sr. Sent: Thursday, March 29, 2007 3:27 PM Subject: BSE; MRR; Importation of Live Bovines and Products Derived from Bovines Commodities

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=19960


Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type RULE Document ID APHIS-2006-0041-0397

COMMENT FROM TERRY S. SINGELTARY SR. 1/09/2007

snip...

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, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???

go figure....

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

see full text ;

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3412&disposition=attachment&contentType=crtext


http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8152



ATTACHMENT TO SINGELTARY COMMENT 1/09/2007

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8


http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f3412


England worried briefly about infecting other countries 27 Aug 00 confidential correspondence obtained by Terry S. Singeltary Sr. http://www.mad-cow.org/00/aug00_last_news.html#fff

Sun, 3 Sep 2000. Unpublished Inquiry documents obtained by CJD activist Terry S. Singeltary Sr. of Bacliff, Texas

http://www.mad-cow.org/00/sep00_news.html#hhh


Other US BSE risks: the imported products picture 24 Jul 00 Trade Statistics: UK to US Compiled by Terry S.Singeltary Sr of Bacliff, Texas

http://www.mad-cow.org/00/jul00_dont_eat_sheep.html#hhh


http://www.mad-cow.org/00/may00_news.html


http://www.mad-cow.org/00/may00_news.html#aaa


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&P=21108


THE VETERINARY RECORD 516 No 47. Vol. 58 November 23rd, 1946

NATIONAL VETERINARY MEDICAL ASSOCIATION OF GREAT BRITAIN AND IRELAND

ANNUAL CONGRESS, 1946

The annual Congress, 1946, was held at the Royal Veterinary College, Royal College Street, London, N.W.I. from September 22nd to September 27th.

Opening Meeting

[skip to scrapie vaccine issue...tss]

Louping-ill vaccine incident ;

http://www.whale.to/v/singeltary.html


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


In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

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


Mr. xxxxxx SIR,

You will never know how much time and money i........we i.e. myself and my wife, have spent doing this, nobody will. but that's not why i did it. i made a promise to mom, i would find the complete truth, and i would make a promise to myself to get cjd reportable reportable in every state, with a cjd questionnaire asking real questions pertaining to route and source of TSE agent. i failed.

IN close, I hope you find time to study this. see for yourself what i have tried to do is/was in good faith.

YOU might wonder why I have wasted almost a decade, daily, hourly almost, doing this for nothing.

I cannot answer that myself, if you figure it out, let me know, and my wife, she would love to know.

IF nothing else, I hope I have at least brought more awareness to this disease, and helped change the

industries involved that are responsible in the spreading of this TSE agent, to change there ways.

who knows, someone might have listened to some of it.

I have attached a PDF file, that is my hypothesis on the diagnostic criteria differentiating the human TSEs,

and what I have said was wrong with it from day one. IT was never published by Lancet. but I have no PhDs.

I am only a lay person as they say when trying to get something published with no PhD. should have listened

to mom, and stayed in school. ...

kindest regards,

I am sincerely,

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

----- Original Message ----- From: xTo: Terry S. Singeltary Sr. Sent: Friday, October 05, 2007 3:19 PM Subject: x

I do care and I'm trying to get all sides of the issue covered. Looks like you have the facts at hand. Would you mind being the subject of next week's interview? I'd love to give you the opportunity to point out what you've detailed in your message to me.

x

---------------------------------------------------------------------------- ---- Date: Fri, 5 Oct 2007 14:52:23 -0500 From: flounder9@verizon.net Subject: x To: x> CC: x

Jolley: Five Minutes With Ray Rodriguez

A. The US has no reported cases born after the ban was in effect. We have 1 atypical case, 1 imported cow from Canada and another with the same prion type as the Europe-Canada prion.

http://www.cattlenetwork.com/content.asp?contentid=165979


make that 2 H-BASE ATYPICALS. plus, one we will never know in Texas because they sent that stumbling suspect mad cow straight to the render, no test, no nothing, just get rid of it, and the faster the better. FYI, i am not sure if you are aware of this, or even if you care, but some disturbing data coming out about the h-BASE. ...terry

----- Original Message -----
From: xxxxxxxxx To: flounder9@verizon.net Sent: Wednesday, August 22, 2007 11:52 PM Subject: Re: HELLO DR. DETWILER... question please ... TSS

Mr. Singeltary,

Yes, both US cases (TX and AL) were H atypicals. I will check if details on the tests have been published.

Linda

Greetings Dr. Detwiler,

I must be slipping here, but i forget, did both those cows in Texas and Alabama have the H-type atypical TSE i.e. base/bse or whatever the be nice they are calling it now

Has this been documented on paper yet, i cannot find it now?

were both the Texas and Alabama BASE H-type ?

Texas ???

Alabama ???

any pathological reports on both those cows on the web yet ?

i saw those 2 further cases of the Nor-98 like/type/whatever reported in the june scrapie report. interesting!

snip...END...TSS

October 2007 Update on Feed Enforcement Activities to Limit the Spread of BSE

http://www.phxnews.com/fullstory.php?article=53149


Transmissible Spongiform Encephalopathy UPDATE USA OCTOBER 2007

http://www.phxnews.com/fullstory.php?article=53128


http://www.phxnews.com/


***ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

Date: Mon, 24 Sep 2007 21:31:55 -0500

I suggest that you all read the data out about h-BASE and sporadic CJD, GSS, blood, and some of the other abstracts from the PRION2007. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744


*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OF POKER INDEED GOES UP. ...TSS

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were ''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007 11:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779


see full text 143 pages ;

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


From: "Terry S. Singeltary Sr." Sent: Tuesday, August 21, 2007 9:50 AM Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringing total to 3 cases to date

Infected and Source Flocks

As of June 30, 2007, there were .....

snip...

One field case and one validation case were consistent with Nor-98 scrapie.

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps


IN the February 2007 Scrapie report it only mentions ;

''One case was consistent with Nor98 scrapie.''

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/


(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS USDA, APHIS, VS ET AL. ...TSS)

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553


An evaluation of scrapie surveillance in the United States

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427


(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............

http://www.oie.int/eng/Session2007/RF2006.pdf

Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program ­ Phase II and Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


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:

[Hyperlink, http://www.gao.gov/cgi-bin/getrpt?GAO-05-101]:


http://www.gao.gov/htext/d05101.html


http://www.gao.gov/highlights/d05101high.pdf


January 2002 MAD COW DISEASE Improvements in the Animal Feed Ban and Other Regulatory Areas Would Strengthen U.S. Prevention Efforts GAO-02-183

http://www.gao.gov/new.items/d02183.pdf


http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

####

http://www.fda.gov/bbs/topics/news/2004/NEW01061.html


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&P=720


THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;

CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006

The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.

The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

http://www.upi.com/

CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...

http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm

PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." ........TSS

BRITISH MEDICAL JOURNAL

SOMETHING TO CHEW ON

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2


BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1


THE PATHOLOGICAL PROTEIN

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/


INTRODUCTION

http://www.thepathologicalprotein.com/_wsn/page3.html


Yam Philip Yam News Editor Scientific American www.sciam.com http://www.thepathologicalprotein.com/

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively

SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535


JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


THE PATHOLOGICAL PROTEIN Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/


doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." ............................

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext


http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf


ya'll gonna miss me ;-)...tss

PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in ''TYPE UNKNOWN''. ...TSS

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,

26 from 2007)

http://www.cjdsurveillance.com/pdf/case-table.pdf


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 Posted by Terry S. Singeltary Sr. at 12:57 PM 3 comments:


Terry S. Singeltary Sr. said... Mr. xxxxxx,

I figured since you were studying this, i thought i might send you this too. i am sure it will be just explained off as another one of those strange coincidences, and i am just biased due to the circumstances i.e. the mom dod hvcjd card, i find it very interesting however that about the same time the atypicals were showing up, the feds were wanting to destroy decades of archives of brains samples of cjd victims and primate victims, donated for research $$$ i know its just another coincidence, and i am tainted/biased, but i thought i might send this to you if you were not aware of this factor. ...terry

Mad Cow Cover-Up: NIH Plans to Destroy Brain Samples of Humans Afflicted with Mad Cow-Like CJD

Steve Mitchell of UPI has been providing excellent, ongoing coverage of mad cow and its threats in the US. Here is his latest piece. One more strong piece of evidence of the cover-up ‹ it now deserves that label ‹ of mad cow risks here in the US under an administration beholden to big beef.

John Stauber -

NIH may destroy human brain collection http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm


Washington Times - Washington,DC,USA NIH may destroy human brain collection

By Steve Mitchell Medical Correspondent

Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.

Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.

Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.

"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE).

The collection is badly in need of organization and no one is certain how many brains or other tissue samples it contains, said Brown, who worked with the collection since its inception in the 1960's until his retirement last year. There could be brains, blood, spinal fluid and various other tissues from 1,000 people or more, he said. Some of the specimens would be of scientific use today, he said.

"This collection has the unique value of stretching back to the beginning of when these diseases were discovered," Brown told UPI, noting that the first samples were obtained in 1963. "It would be as though you had in your hands the possibility of finding out when AIDS started."

Bruce Johnson, a former technician at the CNSS lab who worked extensively with the collection before he retired in 2003, told UPI he was told "in two years they (NIH officials)are going to destroy it, if nobody wants it."

Eugene Major, acting director of the basic neuroscience program at the NIH, said no specific timeframe had been established.

"We have not set a firm deadline date," Major told UPI. "We are working very hard with investigators that we know in order to be able to make sure that whatever we deem is valuable is potentially kept here." Some samples already have been determined not to have any research value and have been "removed and disposed of," he said.

Others samples have been given out to Dr. David Asher at the Food and Drug Administration and Pierluigi Gambetti at the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio.

Major maintained the remaining collection was not particularly valuable for research. "Whatever had been collected here that has not already been distributed to responsible investigators who could use them really has very little remaining value," he said.

Neither Asher nor Gambetti returned phone calls from UPI, but Brown said he thought Asher had received only a dozen or two samples at most and Gambetti had not received much at all.

Neil Cashman, a brain-disease researcher at the University of Toronto's Center for Research in Neurodegenerative Diseases -- who has tried to obtain the collection from the NIH -- said it was priceless.

"It would be like destroying an art museum," Cashman told UPI. "There's all this information and insight that's locked up in these tissues and if it's destroyed it will be lost forever."

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, United Kingdom and France, also thinks the brain collection is invaluable.

"It is the opinion of the Board of Directors ... of The MIND Inc., that the ... brain bank should not be broken up nor destroyed," said Harry E. Peery, MIND's executive director, in a letter to UPI. "We believe that this collection is of inestimable research value and should be kept intact."

The institute, at the University of Saskatchewan in Saskatoon, applied for possession of the collection in early 2004, but received a letter from the NINDS indicating the fate of the collection had not yet been determined.

"We have heard nothing further since that time" and continue to be interested in acquiring the complete collection, Peery said.

CJD belongs to a group of rare, brain-wasting disorders that are little understood, incurable and fatal. This includes mad cow disease in cows, chronic wasting disease in deer and elk. The most infamous of these illnesses in humans is variant CJD, which people can contract from eating beef products infected with the mad-cow pathogen.

Although vCJD has infected more than 154 people worldwide, only one case has ever been detected in the United States -- in a Florida woman who is thought to have contracted the disease while living in the United Kingdom. However, the NIH brain samples have never been screened for vCJD -- something Johnson thinks is critically important.

"No one has ever looked to see if any American (in the collection) in the past had variant CJD," Johnson said. "You think it would be required that they do that. You think it would be a Congressional mandate that they test these brains: 'Let's see if we've got this disease in our country.'"

Johnson noted at least one brain in the collection he personally had examined -- from a French woman collected in 1971 -- showed evidence of possible vCJD infection, but the sample needed further study to be sure.

Other samples in the collection include the brains of patients who were only 16 years old when they were diagnosed with CJD. This would be unusual for sporadic CJD, because generally it strikes those over age 60. Variant CJD, on the other hand, typically occurs in patients in their 20s or younger.

"I thought it was absolutely vital (to test these brains)," Johnson said. "Maybe there's a dozen cases in there of variant CJD."

Major disagreed. "There's really no reason to do that," he said. "The effort it would take to screen those samples ... would not give us any new insights into variant CJD beyond what it is we already know."

Johnson said he was frustrated with the NIH administration's lack of interest in preserving the collection or testing for vCJD. "They don't understand," he said, "they honest-to-god don't understand what it's all about."

Patient advocates also objected to the possible destruction of the brains.

Terry Singeltary, whose mother died of a type of CJD called Heidenhain variant in 1997, said he is outraged and families of other CJD victims probably will be, too.

"A lot of these families went through a lot of heartache and a lot of trouble to get these brain samples to the NIH," Singeltary told UPI. "Now they're just going to discard them because they're not of scientific use? That's just asinine. That stuff is valuable information."

Graham Steel, vice-chair of the Human BSE Foundation in the United Kingdom, told UPI, "The potential loss of such important tissue samples would be a massive blow for TSE (the group of diseases that includes CJD and BSE) research in the United States. This should not be allowed to happen."

Singeltary noted there currently is no cure for these diseases. "If you don't have any answers yet, why would you throw these specimens away?" he asked.

He added that more sensitive tests are just becoming available and could help determine the origin of some of the CJD cases. "We've all been sitting around waiting for more sensitive tests to get validated because we want answers," he said.

"You know, it must be an embarrassment," Johnson said. "Some Senator is going to eventually say 'What is NIH doing about mad cow disease?' And people are going to scratch their heads and say 'not much'." He added, "What's going to happen (is) one of these senators or their wife is going to develop spontaneous CJD one day and ... there's going to be hell raised and they're going to ask, 'Why isn't NIH working on this?'"

--

E-mail sciencemail@upi.com

NIH sends mixed signals on CJD brains

By Steve Mitchell Medical Correspondent

Washington, DC, Apr. 7 (UPI) -- A National Institutes of Health official who told United Press International the agency might destroy its collection of brains from human patients afflicted with a condition similar to mad cow disease reportedly has told the head of a patient-advocate group the collection would be preserved.

The official, Eugene Major, acting director of the basic neuroscience program at the NIH, has not responded to e-mail or a phone call from UPI seeking clarification of his remarks, and the official status of the collection remains unknown.

As reported by UPI on March 24, the collection is stored in freezers by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. It contains brains and other tissue samples from hundreds of people who died from the brain-wasting illness Creutzfeldt Jakob disease, as well as tissues from an untold number of experimental animals.

The consensus of scientists in this field is the collection, which dates back to 1963, is invaluable for research and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

Florence Kranitz, president of the non-profit advocacy group CJD Foundation, told UPI she had "a very long conversation" with Major, in which he told her the remaining tissues in the collection would not be destroyed.

"He reassured me in no uncertain terms," Kranitz said, noting constituents of the foundation and other CJD advocacy groups had been expressing concerns to her the tissues would be destroyed.

Kranitz, who has personal reasons for wanting the collection preserved -- her husband died of CJD in 2000 -- said she plans to meet with Major at the end of April to discuss the issue further.

CJD belongs to a group of diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep. All TSEs are incurable and fatal.

Major previously told UPI some samples already have been destroyed and others have been given to researchers at the Food and Drug Administration and the National Prion Disease Pathology Surveillance Center in Cleveland.

Major said the remaining collection "has very little remaining value" and could be destroyed if another entity does not claim them.

Bruce Johnson, a former NIH scientist who retired at the end of 2003, said he had been told the collection would be destroyed in two years if no one took the samples from the NIH.

In response to hearing that Major had failed to confirm to UPI the brain collection would not be destroyed, Patricia Ewanitz, who lives in Port Jefferson Station, N.Y., and is founder of the advocacy group CJD Voice, said, "The brain tissue might not be indispensable to the National Institutes of Health but it is absolutely necessary to the families who thought enough of science to donate the brains, brain tissue and blood in hopes of someday finding an answer to why their loved one died."

Ewanitz, whose husband died of CJD in 1997, added, "It now seems like such a joke."

Terry Singeltary, whose mother passed away from a type of CJD in 1997, said the NIH should use the samples for scientific research, not just store them in freezers.

Both Singeltary and Ewanitz said they would feel more reassured if Major verified in writing the collection will not be destroyed.

"I would go further and ask Major what he plans to do with them," Singeltary said. "If the samples are just going to sit up there and go bad, then they should give them out to researchers looking for cause and cure."

The revelation the NIH might destroy part or all of the collection sparked an outcry from patient advocates, consumer groups and scientists.

Advocates have been contacting their members of Congress, urging them to investigate and prevent the NIH from destroying the brains. Consumer groups also have gotten involved and scientists have taken steps to obtain the collection or have urged Major not to destroy the samples.

Felicia Nestor, who serves as a consultant to Public Citizen, told UPI she had contacted certain legislators and at least one was considering looking into the situation. Nestor asked the legislator's name be withheld.

Kranitz said Major also told her he plans "to advertise in professional neurological journals and by whatever means necessary to make it known" to researchers in the field the tissues are available.

Major previously said, however, that efforts to inform researchers of the availability of the collection were already underway and included informing NIH grantees. He added he had personally notified researchers at scientific meetings, but no TSE researcher contacted by UPI was aware of this.

"I was never informed," said Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University. She said the first she had heard of the situation was in UPI's March 24 report.

Manuelidis also said she contacted Major, expressing interest in the specimens, but so far has not received a response.

"I sent a letter to (Major) on (March 25) about our interest in these specimens, but he has not replied," she told UPI in an e-mail.

Neil Cashman, a TSE expert at the University of Toronto, who said he was not aware the samples might be destroyed, has lobbied colleagues at the University of British Columbia -- where Cashman is scheduled to move to this summer -- to help draft a letter requesting the collection.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, the United Kingdom and France, requested the collection in January, 2004. So far, the institute has not been informed of a decision by the NIH.

Asked if Major had told him whether the collection would be preserved, MIND Executive Director Harry Peery said, "We have heard nothing further from Eugene Major or anyone else at the NIH regarding the brain collection."

--

E-mail: sciencemail@upi.com

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

NIH says it will preserve CJD brains By STEVE MITCHELL

WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.

That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.

"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.

Cornyn had inquired about the status of the collection in April.

Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.

Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.

Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.

"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.

CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.

Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.

Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.

"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.

"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.

"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.

"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.

Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.

Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.

Steve Mitchell is UPI's Medical Correspondent. E-mail: sciencemail@upi.com

Copyright 2005 by United Press International. All Rights Reserved.

http://washingtontimes.com/upi-brea...50800-6771r.htm


http://www.sciencedaily.com/upi/ind...s-cjdbrains.xml


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

JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005 Mr. Terry SingeltaryP.O. Box 42Bacliff, Texas 77518 Dear Mr. Singeltary: In response to your recent request for my assistance, I have contacted the National Institutes ofHealth. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be ofservice in this matter. Sincerely,

JOHN CORNYN United States Senator JC:djl

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

JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 May 18,2005 Mr. Terry SingeltaryP.O. Box 42Bacliff, Texas 77518 Dear Mr. Singeltary: Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,

JOHN CORNYN United States Senate JC:djl Enclosure

DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of HealthNational Institute of NeurologicalDisorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: sll22c@nih.gov

May 10, 2005

The Honorable John CornynUnited States SenatorOccidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about thepreservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by theNational Institute of Neurological Disorders and Stroke (NINDS) Intramural Research programfor many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand hisdesire that any tissues that could help investigators unravel the puzzle of this deadly disease arepreserved. I hope he will be pleased to learn that all the brains and other tissues with potential tohelp scientists learn about CJD are, and will continue to be, conserved. (The tissues that arediscarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate thatthey have a compelling research or public health need for such materials. For example, sampleshave been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National PrionDiseases Pathology Surveillance Center at Case Western Reserve University in Ohio and workswith the Centers for Disease Control and Prevention to monitor all cases of CJD in the UnitedStates. Dr. Gambetti studies the tissues to learn about the formation, physical and chemicalproperties, and pathogenic mechanisms of prion proteins, which are believed to be involved inthe cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food andDrug Administration, for use in assessing a potential diagnostic test for CJD.

Page 2 - The Honorable John Cornyn

in closing, we know that donating organs and tissue from loved ones is a very difficult andpersonal choice that must often be made at the most stressful of times. We at the NINDS aregrateful to those stalwart family members who make this choice in the selfless hope that it willhelp others afflicted with CJD. We also know the invaluable contribution such donations maketo the advancement of medical science, and we are dedicated to the preservation of all of thetissue samples that can help in our efforts to overcome CJD.

I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,

Story C. Landis, Ph.D. Director, National Institute ofNeurological Disorders and Stroke ================================== END...TSS

SEE FULL TEXT where NIH wanted to destroy all CJD brains donated for research after BSE scandal broke in USA ;

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&P=12771



The cow in the 2003 case was born in Canada, but the last two -- ***found in Texas in June 2005, and Alabama in March 2006 -- were born and raised in the U.S. USDA scientists did not find any definite spongiform lesions on the brains of the Texas and Alabama cows. Also, abnormal forms of prion proteins -- which are thought to cause BSE -- were larger in these cows than those described in most European BSE cases, and they reacted differently to antibodies.

http://www.agriculture.com/ag/story.jhtml?storyid=/templatedata/ag/story/data/1150135629418.xml&catref=ag1001



tss

October 24, 2007 1:03 PM Terry S. Singeltary Sr. said... Subject: BSE TSE NORTH AMERICA THE REGULATORY POINT OF VIEW or the lack of Date: October 22, 2007 at 3:12 pm PST

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

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

Completely Edited Version

PRION ROUNDTABLE

pages 36 -53. ...tss

THE REGULATORY POINT OF VIEW

Dr. Linda Detwiler: I'm going to take a different approach. I'm going to talk about scientific findings and how they're pertinent and why they're important to regulatory policy. We know there have been about 150 deaths from variant CJD from 1986. These are very tragic indeed, however, there are a lot more deaths from the flu and other diseases, yet this is what's made headlines and created a panic.

The objective is to review the research significant to regulatory issues and what's know and—equally important—what's not known. We'll look at the BSE case in Canada and what happened there and what may have happened in their feed cycle. We'll look at implications for the US and possible next steps.

It's important for us to know the similarities, and equally important to know the differences. The differences between species for control purposes, eradication, and prevention are so critical. If you don't know the differences in regards to transmission, pathogenesis, etc., you won't be able to control the diseases appropriately. For example you cannot control Scrapie like you do BSE with a feed ban.

My mantra is that you have to remember these long incubation diseases with limits to the pre-clinical. You have to be thinking. If you knew the disease was going to be here tomorrow, what should you have done yesterday? If you wait until the first time you find the disease, you are years too late. You might be one incubation cycle too late or multiple incubation cycles too late. The population you're concerned with, be it cows or sheep or elk or humans, it's already been infected and you're just going to live out the clinical manifestation of the disease.

Prevention is best, but if you know it’s been introduced, what can you do to limit the transmission? Europe has had to play it out. The UK in 1988 put the ruminant-to-ruminant feed ban on, but they did not ban the total use. It's banned only for ruminants, so they had too much to use. It's not prohibited to go other places, so the meat and bone meal is sold to the continent. It dumps into France and starts to move throughout Europe. Add another five years incubation period, plus surveillance, and you see the manifestation of clinical detection in the Benelux countries.

Europe then started to restrict the use of meat and bone meal, and sale of it went on to other countries. There are a lot of countries that probably have received product, but are not doing surveillance. This is something the US needs to watch out for. We don't classify countries like we do for other diseases, like foot and mouth. For BSE, we'll stop trade if a country is not okay, which is just the opposite from the policy for foot and mouth. This is something the US needs to change. We need to evaluate the countries of the world. Canada has done this and Canada will trade only if the BSE risk is at an acceptable level.

We can't forget other animal species are susceptible, such as cats. Domestic cats and large zoo cats are susceptible to the BSE agent, as well as TSEs of exotic ruminants. If exposed to the BSE agent, bison are susceptible.

Why is the distribution of infectivity important for us? There are several reasons from a regulatory standpoint. If you know where the infectivity is, it helps you eliminate high risk tissue, especially ones that might be a zoonotic risk. That is, What tissues might be more infectious to humans if it’s a zoonotic disease or out of the rations of other animlas if they’re susceptible. Also, it provides direction as to what tissues you might target for diagnostic purposes. In natural cases of BSE, infectivity was in retina, brain and the spinal cord.

In the experimental challenges, calves were fed 100 grams of BSE-infected brain tissue. At certain intervals, some were sacrificed. Forty-some tissues from these calves were put into mice to be bio-assayed. The British were criticized, saying the mouse bio-assay is not as sensitive because of the species barrier, and that you should go back and repeat this in cattle—at least for the most important tissues. This study in cattle had actually generated some additional data { that is additional tissues where infectivity has been found}. The trigeminal ganglia, the dorsal ganglia, the distal ileum and the bone marrow were all from the original mouse pathogenesis study. Recently, tonsil has been found to have infectivity by a calf bio-assay.

Pre-clinically, the distal ileum was six months post-inoculation, and the tonsil was about 10 months post-inoculation. To date, cattle muscle samples have been put into mice and also back into cattle, and no infectivity has been found.

Scrapie infectivity has been identified in brain, spinal cord, tonsil, the peripheral lymph nodes, nasal mucosa, placenta, liver, and the length of the intestine from the esophagus to the rectum. Nora Hunter has published work where both whole blood and buffy coat were taken from sheep and inoculated back into sheep from New Zealand, and those sheep did come down with TSE.

Dr. Spraker: How did they demonstrate nasal mucosa?

Dr. Detwiler: Bill Hadwell collected nasal mucosa from Suffolk sheep and inoculated it into mice.

In baby ruminants, the cells of the distal ileum are a different cell type up until about nine months of age. The cell type is such that they allow the passage of large molecules to transported across the intestine. By about nine months, that function wanes. It's probably for colostral purposes, for colostral antibodies to transfer over. But some have asked if that's why there's an ease of transmission across the gut at a younger age. Horrigan's work at Mission, Texas, showed that after nine months, it appeared to be more difficult to transmit Scrapie. It may be there's a correlation with the change of cell types.

This is a chart I made for the OIE, the Office of International Epizootics to look at secretions and excretions in sheep and goats where, to date, no infectivity had been found in feces, urine, saliva, colostrum, milk and semen. Intra-cranial inoculation into mice resulted in no infectivity. But I want to point out that I don't think we should stop looking. Even with the blood. If you had asked someone prior to 2000 if infectivity was ever found in sheep's blood, he would have said "no," because work done to that time had not shown infectivity in sheep blood. But that work was all blood intra-cranially into mice. How much blood, feces and saliva can you put intra-cranially into a mouse?

Dr. Bartz: With BSE into mice, they estimated a 104 difference in sensitivity between the cow and the mouse.

Dr. Detwiler: How about nasal discharge? Is that a possibility for shed of the agent? We have to keep asking these questions, even for cattle. If it's in the lymphoid tissue of the distal ileum, the tonsil, or the lymphoid tissue of the third eyelid, would it not make sense that, at least at some low level, there might be some lymphoreticular distribution similar to the other animals with TSE. I think that's a valid question to ask, and the calf pathogenesis studies are not finished. I think we have to be careful definitively in saying it's limited to only two three peripheral tissues.

Dr. Detwiler: Dr. Prusiner is examing muscle from cattle with BSE, and looking for presence of PRP and probably infectivity.

Dr. Thornsberry: He's very concerned about muscle tissue.

Dr. Bartz: There's one comment I'd like to make on the muscle infectivity studies. We need to remember that a lot of these are taking a biopsy. If you take a biopsy of muscle, what's in that? It's obviously muscle cells, but it's also nervous tissue and LRS tissue. It's in a muscle homogenate, which is consumed, but the question remains what cell type in that homogenate is the agent really in?

Dr. Detwiler: I asked that of Dr. Prusiner. How did they ascertain that it was muscle cells versus nerve tissue, etc. He said they were very careful in how they took the biopsy.

Dr. Thornsberry: Doesn't every muscle biopsy have some neural tissue in it?

Dr. Detwiler: There's some innervation going to those muscle groups.

Dr. Detwiler: Parenterally, BSE does go into pigs. It was through three routes: intra-cranial, IV and IP with incubation times of 69 to 150 weeks. Seven out of 10 came down with BSE. Orally, it did not go after 84 months. It's important to note that infectivity was not confined only to the CNS, but also in stomach, jejunum, distal ileum and pancreas.

With chickens, both parenterally and orally, they did not find evidence of disease. They tried to sub-passage the chickens by taking brain from animals that had been exposed, to see if it adapted. I think you may have to do this multiple times. What the research in the UK did do was look at high-risk tissues from pigs and chickens that had been exposed to BSE, and put them into susceptible mice. Again, we have to be careful to say there's no residual infectivity at all. If continually fed to a species over time , there may be some kind of passage or adaptation over time. That's why, in other countries, they take out high-risk tissues and don't allow them to be fed at all. In Europe, they've gone to the extreme of taking out all animal tissue being fed to food-producing animals, to break this cycle.

Lateral Transmission

Scrapie and CWD spread animal to animal. In both embryo and semen studies, infectivity for transmission of BSE was not detected. Two cases of disease with fairly significant numbers have been caused by making vaccines from tissues from animals incubating the disease. Just recently, a Mycoplasma aglacia vaccine that was given to sheep and goats in Italy caused infection in 600 to 700 premises.

Contaminated feed appears to be the primary if not sole source of infectivity. Animals that are incubating the disease get slaughtered, rendered, and fed back to other cattle, and they get sick in an average of three to six years.

This is significant to me when we talk about the British attack rate studies. In this experiment, infected brain was given orally. One gram killed seven of 10, so the British knew that had to go down to lower doses. They've now looked at a tenth of a gram and a hundredth and a thousandth of a gram. This is still underway, but at about 60 months, a tenth of gram has killed three of 15 animals affected with BSE, and a hundredth of a gram has killed one of 15 thus far at a 50-plus-month incubation. It doesn’t take too much brain material. The British message is to not underestimate the possibility of cross-contamination in the feed cycle. In the United States we allow mills and plant to use the same equipment when processing ruminant and non-ruminant material. Although a system of cleaning is required by the FDA, the science questions how effective the flush methodologies could really be. The same trucks may haul ruminant and non-ruminant feeds. Those are potentials for cross-contamination.

Dr. Keller: What about risk from feather meal/poultry litter?

Dr. Detwiler: Feather meal can pick up infectivity from other feeds.

Dr. Keller: Whenever you feed a ruminant product to chickens and then the feathers/litter are collected, they're collecting the ruminant material directly in the spillage.

Dr. Detwiler: Hold on to that thought and I'll address it later. A big unknown for the whole world but important to answer involves sheep. BSE goes into sheep orally and maintains BSE characteristics. In research, it looks like Scrapie clinically and histologically. To date, the only valid way to differentiate BSE and Scrapie in sheep is a mouse bio-assay. Would it spread? If it does go naturally into sheep, would it spread like Scrapie through some kind of contagious nature? So far, the distribution of infectivity is looking identical to Scrapie. The significance of this is that the worst case scenario would be BSE in sheep naturally. You would have a potentially zoonotic disease that could spread from sheep to sheep and could not be differentiated from a common endemic disease, would have a widespread tissue distribution, peripheral nerve, blood, so no tissue of the sheep would really be safe. What would countries do if they found this? That's about 40 million sheep.

Dr. Bartz: Do you know if anyone is taking material from the BSE-infected sheep and inoculating non-human primates, to see if it looks like variant CJD?

Dr. Detwiler: It seems a good idea, but I'm not aware of it.

Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test.

There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don’t get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an ‘official’ test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren’t they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

Why Do We Do Surveillance?

Is the disease present? You absolutely have to do surveillance to find out if the disease is present. It will also tell you if your prevention has been successful. If you have the disease, it will also give you indication that your controls are working. That's how Britain knew the feed ban had a big effect. They continued to monitor disease and they saw it peak and they saw it come down about five years after the implementation of the feed ban.

You also need surveillance for buyer confidence and for trade confidence with our international partners. BSE is found in older animals, over 24 months of age, high risk, those exposed to contaminated feed, neurologically ill cattle, any fallen stock and emergency slaughter. This is important to collect samples through active surveillance as owners will sometimes miss subtle signs.

In regards to inactivation, Paul Brown did some work and had to repeat it because people didn't believe it. He found that, at 600° degrees Celius, there was survival in the ash. However, there was none in air emission or the residue. At 1000° Celius, he found no survival.

Canada has actually had two cases of BSE. The first was an imported case in 1993—a UK import. This animal had a broken leg, but because it was a UK import, the owner called the government veterinary staff and they had it looked it. The Canadians had to do extensive tracings on all their UK imports because of this case.

The native case was, of course, in May 2003. This animal was six years old, an Angus type, presented for slaughter as down with signs of pneumonia. It was condemned at slaughter, so it did not go for human consumption. The head was taken for BSE surveillance, and the rest of the carcass went to rendering. The 2003 animal was not related to any of the imports. Was it spontaneous, or was it a case of chronic wasting disease Epidemiologically, the animal was born in the spring of 1997 and was most likely infected at that time. Canada did import 182 head of cattle from the United Kingdom between 1981 and 1990, and there were 11 in the high-risk period, possibly incorporated into the animal feed chain. They also did a risk assessment, which showed they had a low risk of introduction.

They did trace-backs, and that helped to keep the public calm. There was no crisis. In fact, beef consumption went up in Canada. But they tried to find the herd of birth, which is really key in BSE. They narrowed it down to three herds, and all of these were put under quarantine. The cattle in those groups were pretty much depopulated.

When the positive animal was identified, CFIA did trace-backs, and that helped to keep the public calm. There was no crisis. In fact, beef consumption went up in Canada. But they tried to find the herd of birth, which is really key in BSE. They narrowed it down to three herds, and all of these were put under quarantine. The cattle in those groups were pretty much depopulated.

They do have a national ID system, but it had been in place for only two years. So they had to use other types of identification to trace back the animal. Subsequent to the depopulations, one herd was identified as the herd of birth.

One thing the OIE requires is that, for a country to resume trade in animals and animal products, they should depopulate progeny if it's a BSE-infected cow, along with birth cohorts. They had to depopulate over 100 head in these trace-forward herds.

The positive cow itself went to the slaughter plant and was condemned. The head went to the lab and the remaining carcass went to a renderer. From the renderer, the material could have gone to three farms. Two of these were poultry farms. The poultry farms also had cattle, and there was an admission that the cattle were fed the same material as the poultry. Again this is prohibited by the feed ban, but it happens on the farm. These three farms were depopulated in their eintirety.

This renderer did not have dedicated lines, so he made ruminant feed and non-ruminant, prohibited and non-prohibited. They used a barley flush. After the positive cow was processed, the barley flush went to one of these farms and could have been fed to cattle. That was another breakdown in the feed system. That's not supposed to happen, but it did.

It went to two pet-food plants. Material from the renderer at the time the animal might have been processed also went to a feed mill that had 1800 clients, but none of these was depopulated. There were violations. The message is that there are not a lot of resources for checking on-farm compliance with the feed ban. The rubber hits the road on the farm.

I already talked about the epidemiological investigation, the trace-forwards and the trace-backs. CFIA depopulated 2700 animals. They tested approximately 2000 of those. They also brought in an international review team made up of scientists from Switzerland, the US and New Zealand, who reviewed the epi investigation and found it to be thorough and complete. They made recommendations that Canada really had to get an SRM ban to take the high-risk materials out of the human good chain now. Canada has done that.

The committee told CFIA they needed to increase surveillance which examined high risk cattle in large numbers. The review team also said they had to find mechanisms to prevent this cross contamination in the animal food chain. Take out high-risk materials from the animal food chain period. Have dedicated mills, plants, etc.

The media was kind. There was no panic. However, rendering, slaughter and the producer level impacts are still significant. Initially, there was no place for the meat and bonemeal to go. The whole system started to back up. The renderers couldn’t sell it, so they filled their silos. The feed-mills filled their silos. They stopped slaughtering older cows because the system couldn’t take any more. The government started to help, and some things started to go to landfill. Canada did put the SRM ban in place for public protection, but not yet for a nimals. CFIA is working on increasing surveillance, and they need to look at changes in the feed rule.

More Surveillance

North America needs Canada to increase surveillance. Right now, it's a black box. We need to know if it's a single, isolated case or not. With the way BSE works, that's not likely. Or are the ones that were exposed dead and long gone? If there were to be more cases, how many? And very important, if there are additional cases, how old are they? If they were all born prior to the feed ban, that lets us know the feed ban has been very effective. If not, we have had leaks. This year (2003) for surveillance in Canada, they'll come to about the same level they had last year, about 3700 brains tests. They really need to kick this up. The recommendation by the international committee and even by their own government is up to the level of about 40,000.

Think about the whole task of on-farm compliance. You have hundreds of thousands of farms. How do you get 100% compliance? That's very difficult. You can’t be on all farms at all times. My philosophy is choke infectivity out high. If you take your infectivity out of the system at the highest point, what leaks through on the farm won’t matter. Take your high-risk material, your brains and spinal cords and your deads and downs, and get them completely out of the animal food chain. If people inadvertently feed the wrong material on the farm, it won't have the infectivity to keep the cycle going.

It doesn’t take much to introduce BSE into a country. Identification is extremely important. We don’t have a national ID system in place. We're moving in that direction, but how about if tomorrow we found a case of BSE here and we couldn't trace it back? We couldn’t stand up in front of the American public and say, "We don’t know where this animal came from." What would be the reaction?

Disposal itself can cause emergency situations and we need an infrastructure—not only for TSEs but for all animal diseases. The US and Canada both need some kind of infrastructure for proper animal disposal, for animal health and public health reasons.

Can it happen here in the US? Would we find it? To me, we could not have been more fortunate. We got a warning shot right in our neighbor's back yard. We now know that before May 20, we thought we had escaped the bullet of the agent coming to North America. May 20 showed us we were wrong. We know the agent entered North America in the indigenous population. We now have time to re-evaluate the risk, to look the lessons not only in Canada, but around the world. We can do it without having this crisis upon us.

We have import regulations, feed bans, etc. We've had import regulations in place since 1989 on live ruminants and ruminant products. This shouldn't be down-played. This is significant. Look at the way it spread through Europe. That was from imports of high-risk products.

Surveillance: We've looked at all the groups you're supposed to look at for BSE in all the high-risk categories of the downers, etc. We've looked at about 20,000 for the last two years. We should go higher. We've had the same feed ban date as Canada. We do have a few different exceptions.

We've done formal risk assessments—one by the USDA, one by the European Union, and one by Harvard. Harvard's assessment was the most extensive. They looked at the potential pathways. They found that ,although US is resistant to BSE, there is still a potential for a low animal and human exposure. We still allow the use of high-risk material in the US—brain, spinal cord, and advanced meat recovery. Advanced meat recovery is the process where the bones will go through a machine to remove more muscle tissue. The allows some potential exposure if you have BSE infectivity in the system because the spinal column (even if you remove the spinal cord) still has dorsal ganglia that could be incorporated into AMR. That can still introduce infectivity into the AMR product.

Harvard found the feed ban is key to protection and that the leaks will allow amplification, at least in pockets. Why is the risk not zero? Science, trading patterns, existing regulations and human error all constitute a risk. The US, like Canada, does not have dedicated facilities or transport.

The UK imports into the US. There were 496 total, and 173 of the UK imports could have entered the US feed system. People don't like to hear this, but it's possible that one of the UK imports in the US entered the animal feed system and was exported to Canada. That's a possibility, because they import 50% of their feed from the US.

From 1994, we imported 11 million head of cattle from Canada. Most of these were feedlot animals for slaughter, but there were about 500,000 breeding animals. A number of Canada’s cull cows were slaughtered here and could have introduced infectivity into our system. Even today we have Canadian imports in the country, breeding animals that were brought in prior to the ban and reside here.

We have feed ban exemptions: plate waste, poultry litter. We still allow that if it comes off a human plate, or if it's trimmings, it can be palletized and fed to ruminants. That might be a small amount, but it could allow spinal cord in certain cuts to be fed back to ruminants. Poultry litter or feather meal could be significant. Poultry is getting quite a bit of ruminant material in the US because it cannot go back to ruminants. Poultry and pigs are getting a substantial amount. Poultry litter is not only what passes through the chicken, but think about how chickens eat. They spill a lot on the floor. That stuff is still allowed to be fed back to cattle. That's a direct break in the ban, except that it's legal. Ruminants are getting ruminant material.

Unfiltered tallow: tallow is a lipid material. However, if it's not filtered, there are protein residues. That's meat and bone meal. That's allowed to be fed, so that's another legal exception where you can feed ruminant meat and bone meal through unfiltered tallow. We don't have an SRM ban and the 40 animals are the ones that if you have the agent, they introduce the most infectivity back into the animal food chain when they're rendered.

What's our on-farm compliance? We really don't know.

Dr. Thornsberry: What about blood meal?

Dr. Detwiler: Although there's not been infectivity found in cattle blood, the one thing we have to be concerned about is the stunning method. You can have micro-emboli of brain material in blood, and we do allow the legal feeding of blood meal. You could have a contamination from these emboli. Also the brain drips into the blood.

In the past a stun gun that injected air into the cranium had been used. It was so high pressure that it scrambled the brain, pushed it through the foramen magnum, rippled the spinal cord and went down into the azygous venous system. Big parts of the brain and spinal cord were being found in the heart, the lungs, the liver. The industry has gone away from using this stun gun, but there's no regulation that prohibits it.

Firewalls

Identification and traceability aren’t going to stop the disease, but they are essential for locating herd cohorts and identifying other animals that may have been exposed. OIE requires birth cohorts and progeny of a BSE positive cow to be removed.

Canada implemented what the international scientific committee told them to do: they're taking all these tissues out of the food supply for people. You can't remove the dorsal ganglia, the trigeminal ganglia out of the bony tissues so you have both the skull and veretabral column be SRM. The distal ileum is coming out of all ages.

AMR is one thing the US needs to look at. It's a wide dissemination of nervous tissue in the meat product, especially if the process is not done correctly. Even if you remove the spinal cord, you still have the dorsal ganglia associated with the vertebral column and the dura that protects the spinal cord. It would be prudent for the government to move now on restricting ARM.

Harvard's first model did 1000 runs and found the mean ID-50s to humans was 35. Eleven of these were from brain and spinal cord. Twenty—more than half—were from AMR. In the recent runs with Canada, they had some where the ID-50s were up in the hundreds and, in the worst-case scenario- over 1000. Half of those were from AMR. So even if you remove brain and spinal cord from the human food chain, AMR will still deliver more potential doses of infectivity to the public than brain and spinal cord combined.

Dr. Bartz: Exactly what is AMR? Why does it increase infectivity?

Dr. Detwiler: You put the vertebral column through a machine that beats everything off of it.

Dr. Bartz: So you’re saying that if you pull out the spinal cord, when it goes through the ARM, it's pulling off DRGs and the dura.

Dr. Detwiler: And then that gets incorporated into ground beef and it's widely disseminated. In the United States, food has to be labeled. You wouldn't go to a store and buy something that had brain and spinal cord in it. It would have to be labeled so you would know you were eating it.

Dr. Thornsberry: I don't remember the percentage, but it was over half of all advanced meat recovery material we were manufacturing was going into the commodity-type systems.

Dr. Detwiler: That doesn't have to be labeled AMR, so you don’t know what's in and what's not. That could be incorporated into any mixed meat product. The public would feel betrayed and they wouldn't know what products to avoid because of the labeling requirements. McDonald's has never allowed it in their product, even before BSE risk. They continue to not allow it. I don’t know about the other fast-food chains.

Dr. Thornsberry: It looks like a paste. It doesn't have a consistency and it's got a lot of water in it. It's a paste of junk. You combine that and make a sausage or a salami. The average person wouldn't get a piece of AMR meat, but might get some specialty meat that had AMR in it.

Dr. Detwiler: If it's in ground beef, it has to be done in a smaller percentage. It technically could, in a lower percent, go into some ground beef. Some of these large dairies are looking for protein sources and they mix their own on-farm. Two practitioners in California told me their dairies are feeding pet food. What goes into pet food? A lot of the down cows. It never crosses their mind that this could be ruminant material.

The 4D risk is significant. In the Harvard study the model showed that if BSE was circulating in the cattle production system, 1500 ID-50s would come from healthy cattle at slaughter, and 37,000 from the 4D. Think about it. If a cow dies from BSE it is at the end stage disease where the level of infectivity is highest. If there are leaks in the feed ban, this population re-introduce the highest level of infectivity back to your cattle population.

Dr. Spraker: What are the four Ds?

Dr. Detwiler: Dead, dying, down, diseased. They're a lump sum of the animals that don’t make it for human consumption.

LESSONS LEARNED

We have to be careful that the absence of evidence is not evidence of absence, and when we say there's absolutely no scientific evidence. We need to be very careful when we talk to the public, and even producers, to make sure there is scientific evidence, and if there's not, to tell that to the public. A case in point is the UK with the link to human disease. In 1989, some experts said that there was absolutely no scientific evidence that the disease could go to humans. That was a correct statement then, however, it was not the entire story. What should have followed was that there is no evidence to definitively state that BSE could not cross species. The bottom line was that we don’t have much evidence period. We have to be careful of predictions from TSE’s in other species, as these long incubations dieeases are like wolves in sheep’s clothing.

Prevention with adverse action or significant cost is very difficult to sell. I spent my whole career with APHIS trying to sell prevention—first with Scrapie and then with BSE. If BSE didn't go to humans, I don't think we'd ever have sold the feed ban. Now the industry is glad we did. Failure to prevent is an easy target, but how do you measure success? Regulations: if people believe it will protect them, you don't need much compliance. If people aren't cognizant or don't realize how it will protect them, you need a lot of compliance. Driving is a good example. People believe seat belts will at least reduce fatality. You don't need a cop in your car to wear your seatbelt. But speeding is a different story. How many people drive the speed limit? By statistics, the higher the speed limit, the more you increase your chance of fatality. We need to know how much your producers and clients believe in the feed ban.

Disposal: positive solutions for one problem may create more expensive problems. It's easy to say "ban everything," but then what do you do with it? That's something we need to educate the public on. It's not as easy as it appears. You create environmental problems.

Dr. Keller: Do you know what they're doing with the rendered product? We heard at one time they were using it in the UK to make asphalt.

Dr. Detwiler: There are a lot of different things now. Some places are combining it with concrete and asphalt because it's a binder. Most of it gets rendered first and then the rendered material is used. They're using it as a fuel source—the meat and bone meal and the tallow. The tallow, apparently, burns well. Some of the rendering plants in California converted their systems when energy prices rose and ran off their own tallow. There's a cement company in the US that wants to burn meat and bone meal in their kilns. Temperatures will go up into the thousands of degrees. Regaining scientific credibility and public confidence may require extreme measures. That's why I think it's so important for the countries not to lose public confidence. Japan lost it, and they've had to take extreme measures, like testing every cow for human consumption.

Dr. Thornsberry: Two years ago, I worked as a consultant in Missouri and I had a couple of clients who owned large swine operations. They were cleaning out their swine feed bins and feeding it to their cows. They were feeding for a large corporate entity, and that entity would remove a group of hogs and they might leave two or three tons of hog feed. The hog feed contained meat and bone meal. It wasn't a real high level. Sometimes it was porcine meal and sometimes it was bovine, but it was in there. I got in contact with the state veterinarian and it went up to the FDA level concerning the feeding of this and the feeding of poultry litter. I asked if they'd made any rulings about the feeding of poultry litter because I knew that 5% of our feed is lost without ever being consumed. The FDA veterinarian’s comment to me was, "Dr. Thornsberry, until there are dead bodies lying in the street, you will not see a change made on the feeding of poultry litter." I said, "You know there's a potential epidemiological link between the spread of this disease and the feeding of poultry litter, and you're telling me you have to have dead bodies before you make a ruling on it."

He said yes. What's going on with our government?

Dr. Thornsberry: We need to put the pressure on.

Dr. Detwiler: That's right, and there are different entities that are saying this to the government. The Food Marketing Institute, the chain restaurants, do have a position statement they've put together that say they'd like to see the exemptions gone. They feel there are reasons to close the loopholes.

Dr. Thornsberry: I got a memo this summer that Canada agreed not to use AMR from that point on, but it is not prohibited in Canada.

Dr. Detwiler: Right, and that's crucial. Vertebral column is considered a risk material and it cannot be used. The United States has not done that. We're testing products to see if evidence of nervous tissue can be found, but that's not every product. It's done on a random basis by FSIS. We are still allowing AMR.

THE STATE REGULATORY VIEWPOINT

Dr. Keller: My focus is mainly on questions I have from a state perspective. Although I had about 10 years of veterinary practice experience, my education, with respect to prions, began when I started working for the State of North Dakota in ~1997. Serving as ND’s Designated Scrapie Epidemiologist (DSE) and working with their mandatory Chronic Wasting Disease (CWD) surveillance program, has further increased my appreciation for regulatory challenges associated with TSEs.

Recently, state animal health officials have had the task of drafting comments for the proposed BSE rule, which attempts to address ‘minimal risk countries.’

Background: In ND, the North Dakota Board of Animal Health is charged with protecting the health of domestic animals and also non-traditional livestock. In our state, that includes everything from captive birds to farmed cervids, to lions and tigers and bears. When in a state regulatory position, these responsibilities are not optional, they are required of animal health officials by statute. We are also to prevent escape and release of animals injurious or competitive with agriculture, horticulture, forestry, and other natural resource interests; thereby addressing potential environmental concerns.

We're also charged with taking any steps necessary to control, suppress, eradicate, any and all contagious and infectious diseases. Since prion diseases are indeed considered infectious, they fall under that statute. The State Veterinarian also has the responsibility, if warranted, to quarantine domestic animals and non-traditional livestock. He is to regulate and prohibit arrival or departure from our state of any animals that may be exposed or infected with the disease. Where this could get interesting, is when a state doesn't think the federal regulations are protecting the state’ s animal health or is not responsive enough in addressing urgent concerns. Also, it is very difficult trying to regulate prion diseases when you have limited ‘official live animal tests’ available, such as are available for TB and Brucellosis where you can get more definitive answers quickly. In that situation, prevention is always the best policy.

The industry groups and state agencies need to have a clearer understanding of the prion agent, its pathogenicity, and how it's transmitted so that more useful comments can be submitted on proposed rules.

We need to be concerned about what is an ‘acceptable risk’ of having possible disease transmission to other animals. We also cannot over look the economic damage done by diseases. Look at what one case of BSE did to Canada from an economic standpoint! Another area of responsibility we are charged with, is addressing zoonotic diseases. BSE is one prion disease that is currently considered to be zoonotic.

We have zero tolerance in this country for fecal contamination of our food, so what are we going to determine is an ‘acceptable risk’ for BSE contamination? And if we do allow animals in from countries where there is a risk, what are the mitigating factors or measures that need to be taken? It is my opinion that we need to have extensive measures in place to prevent the introduction of the disease and to maintain the identity of animals from other countries, even if they are only considered to be of ‘minimal risk’.

When we talk about identifying a country as minimal risk because they've had only one case, could this then be extrapolated to other countries? We may end up comparing apples and oranges though. For example, if we have a country in Europe that has one case of BSE, it's not really the same because it's on a different continent with different neighbors. We may not even be fully familiar with the extent of their country’s animal movements or the quality of their surveillance.

Dr. Detwiler: That's a very good point. This rule will allow other countries to apply for minimal-risk status, and I can tell you there are countries other than Canada that have only one case—Greece, Austria, Finland—that have much higher surveillance levels and have had measures in place a lot longer. So this is a reality that this situation could happen.

Dr. Keller: Those countries are watching the situation closely and may be waiting to make their application should the proposed BSE rule be approved as is. So what would happen if the US found one case of BSE? In our department, there would be immediate attention to what we would say to the producers and to the consuming public. We would need to have factual information available to address food safety concerns. How would we do that? We would need to be able to say we were doing a thorough epidemiologic investigation, and quickly get information back to consumers and our international trading partners as it became available. Two critical actions need to be done: country of origin labeling through maintenance of import identification of live animal and products and identification of individual animals. Another question that needs to be posed is which is easier and more cost effective to do first? There is identity on animals that come into this country, but when we've looked into that, there's no requirement for anyone to maintain that identity. In the past, this country has relied on the use of the brucellosis tags, auction-market back tags and brand inspection. But this does not allow for complete tracking of animals. Especially as the Brucellosis program winds down. There is an obvious need for individual animal identification.

The tattoos that have been suggested as a way to track animals long-term are not a fail-safe method. Everyone has their own idea of what a tattoo should be, what type of ink to use, etc. The reality is, you could have a very good tattoo but it’s not user-friendly. We'd have trouble getting compliance if we asked feedlots to rely on running every animal through a chute to read a tattoo. Running multiple animals down an alley and using RFID readers to collect their information as they run by, might be a workable system. A second form of ID will be needed in case of ID failure.

What's the international standard when we talk about BSE? OIE does not necessarily require seven years since the last case before you can move animals, but OIE has certain requirements for regions to qualify as minimal-risk regions in countries where BSE was identified fewer than seven years ago. For a minimal-risk zone or in a country, these are the things that have to be done: It has to have been based on fewer than one case per million during each of the last four consecutive 12-month periods, within the cattle population that’s over 24 months. These are very specific requirements. Whenever we talk about being consistent with OIE, we really need to consider that, since other countries usually recognize the status assigned by the OIE.

What are the consequences of being more lenient than international standards? It could be used in the marketplace against us and thus lead to a loss of consumer confidence. Other countries would be quick to speak up if they have more stringent standards in avoiding SRMs. When we have a case, it may become a marketing tool for another country.

When our standards are lower, we tend to be the country that would be exposed to the movement of animals that are at risk for bringing in BSE. We could have a ripple effect where we would have loss of consumer confidence, loss of markets, and devastation to the US cattle industry. If we have another country that's not using SRM and we're looking at identifying that country as a minimal risk area, but we bring in animals that they're not using SRMs from, and the US is still allowing use of SRMS, will that not possibly affect consumer confidence in this country or give our trading partners a reason to question the rationale?

Dr. Detwiler: How about the animals we have now? As I mentioned, if breeding animals were north of the border and they went to slaughter like the cull dairy cows, they'd have their SRMs removed to protect the Canadian public. Below the border, those same animals do not have their SRMs removed.

Dr. Keller: I agree. The number of Canadian birth cohorts you refer to that have entered the US is significant and has not yet been addressed by APHIS. There again, another country could be quick to point out the lack of attention by our epidemiologists to that group of animals, should we ever have a case of BSE in a US born and raised bovine. The main point is that we need to have a standard. The list of SRMs has expanded through research, and I think we’re hearing today that it’s possible even more SRMs may be added. Whatever the list of SRMs is, it needs to be consistent among countries, and the OIE needs to define what the SRMs are.

Obviously there's going to be resistance to taking additional precautions due to costs, but the costs will be even greater and farther reaching if we don’t. We need to be proactive on this issue, not reactive.

The feeding of feather meal is also a major concern. Does feather meal risk increase when you’re moving animals in that are from minimal-risk countries? I believe it would be a potential route of amplification of the prion in our ruminant feed supply. The Harvard Risk Assessment warned against it.

Dr. Schuler, the ND State Veterinarian discovered that in the proposed BSE rule, where they use the terminology "designated feedlot," that it means only the feedlot name listed on a health certificate. It does not mean that the animals going to a designated feedlot are actually going to go to slaughter immediately under a verified protocol. When animals from another country go to a designated feedlot, there's nothing to prevent their tags from being removed and animals being deviated out of the slaughter channel they were originally intended for.

Dr. Detwiler: How did they ever identify what animals ___________________

Dr. Keller: That's a very good question, and it goes back to "what is a designated feedlot?" and "why doesn’t the ID have to be maintained on those animals?" Actions must be taken to address the need for maintenance of ID.

Dr. Thornsberry: We're required in the state of Nebraska to write on the health certificate that these animals are for feeding purposes only and must go to slaughter. But that doesn’t mean that they do.

Dr. Detwiler: A lot of the feedlots will come in and remove the ear-tags and put their own lot tags in order to make a consistent lot, so you lose that ID. That was a problem when the US wanted to investigate what animals were already in feedlots. There's no regulation for that animal to retail that ID.

Dr. Thornsberry: I thought there was, for disease control.

Dr. Detwiler: There might be for Mexican steers, but not others.

Dr. Keller: Most people are not aware that all state animal health officials know from the health certificates, is the first point of destination. For example, feedlot heifers might be brought in from another country and when the feedlot manager discovers some of them are bred, they may be pulled out to be sold as breeding animals and no one may ever know.

snip...

FULL TEXT;

Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

The roundtable presentations and discussions were recorded. A transcript will be made available to the Academy of Veterinary Consultants, the American Association of Bovine Practitioners, and the Colleges of Veterinary Medicine throughout the United States and Canada. A condensed version translated for the livestock industry will be made available to educate livestock producers about prion related diseases.

http://www.r-calfusa.com/Newsletter/2004January.pdf




2005

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

The BSE enhanced surveillance program involves the use of a rapid screening test, followed by confirmatory testing for any samples that come back "inconclusive." The weekly summary below captures all rapid tests conducted as part of the enhanced surveillance effort. It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing.

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



*** Suppressed peer review of Harvard study October 31, 2002 ***

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf



Owens, Julie From: Terry S. Singeltary Sr. [flounder9@verizon.net] Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98 8/3/2006

Greetings FSIS,

I would kindly like to comment on the following ;

[Federal Register: July 12, 2006 (Volume 71, Number 133)] [Notices] [Page 39282-39283] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr12jy06-35] ----------------------------------------------------------------------- DEPARTMENT OF AGRICULTURE Food Safety and Inspection Service [Docket No. FSIS-2006-0011] Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Update; Notice of Availability and Technical Meeting

snip...

http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-10928.htm


MY comments/questions are as follows ;

1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk assessment and will this assessment include the Atypical TSE and SRM issues ?

*** Suppressed peer review of Harvard study October 31, 2002 ***

http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf



2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our feed bans continue to fail in 2006, and continue to fail today ?

snip...

full text 98 pages ;


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf




BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS

http://madcowtesting.blogspot.com/




PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in ''TYPE UNKNOWN''. ...TSS

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, *** 26 from 2007)

http://www.cjdsurveillance.com/pdf/case-table.pdf




USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf



3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp




SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html



There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



or this ;

FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

####

http://www.fda.gov/bbs/topics/news/2004/NEW01061.html



What Do We Feed to Food-Production Animals? A Review of Animal Feed

Ingredients and Their Potential Impacts on Human Health

Amy R. Sapkota,1,2 Lisa Y. Lefferts,1,3 Shawn McKenzie,1 and Polly Walker1 1Johns Hopkins Center for a Livable Future, Bloomberg School of Public Health, Baltimore, Maryland, USA; 2Maryland Institute for Applied Environmental Health, College of Health and Human Performance, University of Maryland, College Park, Maryland, USA; 3Lisa Y. Lefferts Consulting, Nellysford, Virginia, USA

snip...

Table 1. Animal feed ingredients that are legally used in U.S. animal feeds

Animal

Rendered animal protein from Meat meal, meat meal tankage, meat and bone meal, poultry meal, animal the slaughter of food by-product meal, dried animal blood, blood meal, feather meal, egg-shell production animals and other meal, hydrolyzed whole poultry, hydrolyzed hair, bone marrow, and animal animals digest from dead, dying, diseased, or disabled animals including deer and elk Animal waste Dried ruminant waste, dried swine waste, dried poultry litter, and undried processed animal waste products

snip...

Conclusions

Food-animal production in the United States has changed markedly in the past century, and these changes have paralleled major changes in animal feed formulations. While this industrialized system of food-animal production may result in increased production efficiencies, some of the changes in animal feeding practices may result in unintended adverse health consequences for consumers of animal-based food products. Currently, the use of animal feed ingredients, including rendered animal products, animal waste, antibiotics, metals, and fats, could result in higher levels of bacteria, antibioticresistant bacteria, prions, arsenic, and dioxinlike compounds in animals and resulting animal-based food products intended for human consumption. Subsequent human health effects among consumers could include increases in bacterial infections (antibioticresistant and nonresistant) and increases in the risk of developing chronic (often fatal) diseases such as vCJD. Nevertheless, in spite of the wide range of potential human health impacts that could result from animal feeding practices, there are little data collected at the federal or state level concerning the amounts of specific ingredients that are intentionally included in U.S. animal feed. In addition, almost no biological or chemical testing is conducted on complete U.S. animal feeds; insufficient testing is performed on retail meat products; and human health effects data are not appropriately linked to this information. These surveillance inadequacies make it difficult to conduct rigorous epidemiologic studies and risk assessments that could identify the extent to which specific human health risks are ultimately associated with animal feeding practices. For example, as noted above, there are insufficient data to determine whether other human foodborne bacterial illnesses besides those caused by S. enterica serotype Agona are associated with animal feeding practices. Likewise, there are insufficient data to determine the percentage of antibiotic-resistant human bacterial infections that are attributed to the nontherapeutic use of antibiotics in animal feed. Moreover, little research has been conducted to determine whether the use of organoarsenicals in animal feed, which can lead to elevated levels of arsenic in meat products (Lasky et al. 2004), contributes to increases in cancer risk. In order to address these research gaps, the following principal actions are necessary within the United States: a) implementation of a nationwide reporting system of the specific amounts and types of feed ingredients of concern to public health that are incorporated into animal feed, including antibiotics, arsenicals, rendered animal products, fats, and animal waste; b) funding and development of robust surveillance systems that monitor biological, chemical, and other etiologic agents throughout the animal-based food-production chain “from farm to fork” to human health outcomes; and c) increased communication and collaboration among feed professionals, food-animal producers, and veterinary and public health officials.

REFERENCES...snip...end

Sapkota et al. 668 VOLUME 115 NUMBER 5 May 2007 • Environmental Health Perspectives

http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1867957&blobtype=pdf




Brief review on the epidemiology of transmissible spongiform encephalopathies (TSE) Marcus G. Doherr Department of Clinical Veterinary Medicine, Vetsuisse Faculty, University of Bern, Bremgartenstrasse 109a, P.O. Box 8466, 3001 Bern, Switzerland Received 25 June 2006; accepted 30 October 2006 Available online 13 November 2006

snip...

As a consequence of the studies linkingMBM use in cattle feed to BSE, the inclusion of MBM into ruminant feed was banned in the UK in July 1988, and in Switzerland in December 1990. These bans in both countries resulted in a significant reduction of new infections in cattle born after their implementation, thereby highlighting the importance of controlling this exposure route [34].However, the relatively large number of BSE cases born after those bans indicated that, despite the feed ban in place, BSE infectivity — to some extent – was still reaching cattle. These cases, subsequently denoted as born-after-the-ban (BAB) and born-after-the-reinforced (UK in 1996) ban (BARB), documented the presence of other infection routes besides direct (legal) inclusion of MBM in cattle concentrates. Cross-contamination of cattle feed with feed for pigs and poultry during production, transportation or storage, and cross-exposure of cattle to pig or poultry feed on mixed-species farms were suspected as additional routes [35–37]. More recently, insufficiently heated bone meal and tallow used in concentrate feeds and milk replacers has been suggested as an additional source of BSE infectivity [38,39]. The measures such as the MBM bans currently implemented in Europe, as shown by intensive surveillance with over 40 million animals (clinical suspects, emergencyslaughtered and fallen cattle as well as healthy-slaughtered adult cattle) tested between January 2002 and September 2005, have resulted in a constant decline of the BSE epidemic at least in the old EU member states (Fig. 2) [18]. It is to be expected that the epidemic will phase out in countries with sufficiently implemented control measures. This, due to the long incubation time of the disease, will require time.

snip...end

October 2007 Update on Feed Enforcement Activities to Limit the Spread of BSE

http://www.phxnews.com/fullstory.php?article=53149


http://www.fda.gov/cvm/BSE1007.htm


* GAO-05-51 October 2004 FOOD SAFETY (over 500 customers receiving potentially BSE contaminated beef) - TSS 10/20/04

October 2004 FOOD SAFETY USDA and FDA Need to Better Ensure Prompt and Complete Recalls of Potentially Unsafe Food

snip...

Page 38 GAO-05-51 Food Recall Programs To examine the voluntary recall of beef products associated with the December 2003 discovery of an animal infected with BSE, we analyzed the distribution lists USDA collected from companies and the verification checks it conducted to develop a diagram illustrating the location and volume of recalled beef that reached different levels of the distribution chain. We compared the distribution lists and verification checks to identify how many customers listed on the distribution lists did not receive the recalled beef and the number of customers not listed on distribution lists that received the recalled beef. We interviewed USDA and FDA staff involved with the recall to understand the timing of recall actions and the challenges encountered during the recall. To develop information on the 2002 recall of ground beef by a ConAgra plant in Greeley, Colorado, we reviewed USDA s recall file and other documents on the recall. We