Sunday, May 10, 2009

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


Title: Identification and characterization of two bovine spongiform encephalopathy cases diagnosed in the United States


Richt, Juergen Kunkle, Robert Alt, David Nicholson, Eric Hamir, Amirali Czub, Stefanie - NATL BSE REF LAB,MANITOBA Kluge, John - NVSL, APHIS, USDA, AMES, Davis, Arthur - NVSL, APHIS, USDA, AMES, Hall, S Mark - NVSL, APHIS, USDA, AMES,

Submitted to: Journal of Veterinary Diagnostic Investigation Publication Type: Peer Reviewed Journal Publication Acceptance Date: October 20, 2006 Publication Date: March 1, 2007 Citation: Richt, J.A., Kunkle, R.A., Alt, D., Nicholson, E.M., Hamir, A.N., Czub, S., Kluge, J., Davis, A.J., Hall, S.M. 2007. Identification and characterization of two bovine spongiform encephalopathy cases diagnosed in the United States. Journal of Veterinary Diagnostic Investigation. 19(2):142-154.

Interpretive Summary: Bovine spongiform encephalopathy (BSE), also known as "mad cow disease," is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the United Kingdom and subsequently in other countries. Here we report the identification and characterization the two cases of BSE diagnosed in the United States. BSE case 1 (12/2003) and BSE case 2 (11/2004) were identified and characterized using various diagnostic methods specific for BSE (rapid test, Western blot, immunohistochemistry). Sequencing of the prion protein gene of both BSE-positive animals revealed that the sequences of both cattle were similar as previously reported for cattle. These results confirm that two cases of BSE have been identified in the United States so far: one in a cow imported from Canada and one in a cow born and raised in Texas. Technical Abstract: Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy of cattle, first detected in 1986 in the United Kingdom and subsequently in other countries. It is the most likely cause of variant Creutzfeldt-Jakob disease (vCJD) in humans, but the origin of BSE has not been elucidated so far. Here we report the identification and characterization of the two cases of BSE diagnosed in the United States. Case 1 (12/2003) revealed spongiform changes in the obex area of the brainstem and the presence of the abnormal form of the prion protein, PrP**Sc, in the same brain area, by immunohistochemistry and Western blot analysis. Initial suspect diagnosis of BSE for case 2 (11/2004) was made by a rapid ELISA-based BSE test. Case 2 did not reveal unambiguous spongiform changes in the obex area, but PrP**Sc was detected by immunohistochemistry and enrichment Western Blot analysis in the obex. Using Western blot analysis, PrP**Sc from case 1 showed molecular features similar to typical BSE isolates, whereas PrP**Sc from case 2 revealed an unusual molecular PrP**Sc pattern: molecular mass of the unglycosylated and monoglycosylated isoform was higher than that of typical BSE isolates and case 2 was strongly labeled with antibody P4, which is consistent with a higher molecular mass. Sequencing of the prion protein gene of both BSE-positive animals revealed that the sequences of both animals were within the range of the prion protein gene sequence diversity previously reported for cattle.

Project Team

Kunkle, Robert Kehrli, Marcus Nicholson, Eric Greenlee, Justin Hamir, Amirali



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Animal Health (103)


Method For Detecting A Gene Linked To Mad Cow Disease

Last Modified: 05/09/2009

Research Project: Genetic and Biological Determinants of Respiratory Disease Susceptibility Location: Animal Health Systems Research

Title: Association of a bovine prion gene haplotype with atypical BSE


Clawson, Michael

Submitted to: Meeting Abstract Publication Type: Abstract Publication Acceptance Date: December 2, 2008 Publication Date: January 1, 2009 Citation: Clawson, M.L. 2009. Association of a bovine prion gene haplotype with atypical BSE [abstract]. Plant and Animal Genomes XVII Conference. Abstract No. W091. Available:

Technical Abstract: Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a class of fatal neurodegenerative disorders that occur in humans, ruminants, cats, and mink. Three distinct TSEs afflict cattle: classical bovine spongiform encephalopathy (BSE), atypical H-type BSE, and atypical L-type BSE. Classical BSE was identified in the 1980s and is acquired by cattle through the consumption of feed contaminated with the infectious prion agent. Atypical BSEs have only recently been recognized as distinct cattle prion diseases and are extremely rare. The full extent of genetic susceptibilities to atypical BSEs is unknown; however, one atypical H-type case identified in the United States (2006) was most likely caused by a genetic mutation in the prion gene, E211K. We have identified an association of a bovine prion DNA haplotype with atypical BSE that is independent of E211K. The haplotype spans a portion of the prion gene that includes part of intron 2, the entire coding region of exon 3, and part of the three prime untranslated region of exon 3 (13 kb). Despite the low frequency of this haplotype among general cattle populations, it was present in a majority of H- and L-type atypical BSE cases from Canada, France, and the United States. This result indicates that there is a genetic component to atypical BSE susceptibility in addition to E211K.

BSE in an Alabama cow

On March 13, 2006, the U.S. Department of Agriculture (USDA) announced the confirmation of bovine spongiform encephalopathy (BSE) in a cow in Alabama. This is the eighth confirmed case of BSE in North American cattle and the second case since the beginning of 2006. Three cases were detected in the United States and five in Canada. One of the two previous U.S. cases was of Canadian origin, and the first of the five cases detected in Canada was in a cow that had been imported from the United Kingdom.

The newly confirmed case was identified in a non-ambulatory (downer) cow on a farm in Alabama. The animal was euthanized by a local veterinarian and buried on the farm. USDA surveillance is targeted at those animal populations in which BSE would most likely be found. These animals primarily include clinically abnormal or deceased cattle, which would not be expected to enter the human food supply. The cow in Alabama did not enter either the animal or the human food chains, according to the USDA announcement. USDA is working with Alabama animal health officials to conduct an epidemiologic investigation to gather additional information about the age and herd of origin of this animal, which had resided on the Alabama farm for less than a year.

The agency also will work to identify other cows born in the same herd within one year of the affected animal, and any offspring. In addition, USDA is working with Food and Drug Administration (FDA) officials to determine any feed history that may be relevant to the investigation. "Experience worldwide has shown us that it is highly unusual to find BSE in more than one animal in a herd or in an affected animal's offspring. Nevertheless, all animals of interest will be tested for BSE," according to the USDA statement.

BSE is a progressive neurological disorder of cattle that results from infection by an unconventional transmissible agent. Strong evidence indicates that BSE has been transmitted to humans, primarily in the United Kingdom, causing a variant form of Creutzfeldt-Jakob disease (vCJD). As of March 2006, a total of 190 cases of vCJD have been reported worldwide; of these, 160 occurred in the United Kingdom. Two cases have been reported in the United States; for both of these cases, there was clear epidemiologic evidence that the disease was acquired in the United Kingdom.

For additional information about the newly confirmed BSE case, see the USDA announcement available on the USDA website at

Date: March 15, 2006 Content source: National Center for Infectious Diseases


BSE Case Associated with Prion Protein Gene Mutation

Jürgen A. Richt1¤*, S. Mark Hall2

1 National Animal Disease Center, United States Department of Agriculture, Agriculture Research Service, Ames, Iowa, United States of America, 2 National Veterinary Services Laboratories, Pathobiology Laboratory, Animal and Plant Health Inspection Service, United States Department of Agriculture, Ames, Iowa, United States of America

Abstract Top Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy (TSE) of cattle and was first detected in 1986 in the United Kingdom. It is the most likely cause of variant Creutzfeldt-Jakob disease (CJD) in humans. The origin of BSE remains an enigma. Here we report an H-type BSE case associated with the novel mutation E211K within the prion protein gene (Prnp). Sequence analysis revealed that the animal with H-type BSE was heterozygous at Prnp nucleotides 631 through 633. An identical pathogenic mutation at the homologous codon position (E200K) in the human Prnp has been described as the most common cause of genetic CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. A recent epidemiological study revealed that the K211 allele was not detected in 6062 cattle from commercial beef processing plants and 42 cattle breeds, indicating an extremely low prevalence of the E211K variant (less than 1 in 2000) in cattle.

Author Summary Top Bovine spongiform encephalopathy (BSE or Mad Cow Disease), a transmissible spongiform encephalopathy (TSE) or prion disease of cattle, was first discovered in the United Kingdom in 1986. BSE is most likely the cause of a human prion disease known as variant Creutzfeldt Jakob Disease (vCJD). In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in “the approximately 10-year-old cow” carrying the E221K mutation.

Pathog 4(9): e1000156. doi:10.1371/journal.ppat.1000156

Editor: David Westaway, University of Alberta, Canada

Received: June 5, 2008; Accepted: August 15, 2008; Published: September 12, 2008

This is an open-access article distributed under the terms of the Creative Commons Public Domain declaration which stipulates that, once placed in the public domain, this work may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.

Funding: This work was supported by the USDA-ARS-National Animal Disease Center (NADC) and USDA-APHIS-National Veterinary Services Laboratories (NVSL) and by the NIAID-NIH PO1 AI 77774-01 “Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases”.

Competing interests: Patent pending: Dr. Jürgen A. Richt submitted a patent application entitled “Novel Polymorphism in Bovine Prion Protein Gene Sequence” (Docket Number 0078.06; Serial No. 11/787,784) on April 18, 2006.

* E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000388/!

¤ Current address: Kansas State University, College of Veterinary Medicine, DM/P, Manhattan, Kansas, United States of America

Introduction Top Transmissible spongiform encephalopathy (TSE) agents induce fatal neurodegenerative diseases in humans and in some mammalian species [1]. According to the prion-only hypothesis, infectious prions are composed of an abnormal isoform of a host-encoded glycoprotein, called prion protein (PrPc). The disease-associated form, PrPd, is derived from PrPc by a post-translational mechanism that involves conformational change [1]. Human TSEs include Creutzfeldt–Jakob disease (CJD), Gerstmann–Sträussler–Scheinker syndrome, Kuru and Fatal Familial Insomnia [1]. In animals, several distinct TSE diseases are recognized: Scrapie in sheep and goats, transmissible mink encephalopathy in mink, chronic wasting disease in cervids, and bovine spongiform encephalopathy (BSE) in cattle [2]. BSE was first detected in 1986 in the United Kingdom and is the most likely cause of variant CJD (vCJD) in humans. The origin of the original case(s) of BSE still remains an enigma. Hypotheses include (i) sheep- or goat-derived scrapie-infected tissues included in meat and bone meal fed to cattle, (ii) a previously undetected sporadic or genetic bovine TSE contaminating cattle feed or (iii) origination from a human TSE through animal feed contaminated with human remains [3]. This study will provide support to the hypothesis that BSE originated from a previously undetected genetic bovine TSE contaminating cattle feed in the U.K.


Here we report a case of bovine BSE associated with a mutation within the prion protein gene (Prnp) sequence, not previously described for the bovine Prnp. The animal (called “U.S. BSE Alabama”) was an approximately 10 year-old red crossbred (Bos indicus×Bos taurus) hybrid beef cow from Alabama (see Materials and Methods). The ELISA-based BSE test (see Materials and Methods) on brainstem from this animal was repeated five times and revealed a strongly positive reaction with mean optical density (OD) value of 2.40±0.57, whereas the OD value of bovine control obex was <0.04. href="mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000388/!x-usc:"> ). There are several possibilities for the origin of the Prnp K211 allele in animal B14842: (i) it arose de novo in a germ line cell from the U.S. BSE Alabama animal or one of its parents; (ii) it arose as a somatic mutation in the U.S. BSE Alabama animal (rather unlikely), and (iii) it is present in a cattle population or breed yet to be found. Recently, it was determined that the 2-year-old heifer offspring of the U.S. BSE Alabama cow also carries the E211K polymorphism, indicating that the allele is heritable and may persist within the cattle population (11) In a recent epidemiological study which included 6062 cattle from 5 commercial beef processing plants (3892 carcasses) and 2170 registered cattle from 42 breeds., the K211 allele was not detected using a newly developed mass spectrometry assay specific for the E211K variant [12]. These data indicate a rather low prevalence of the E211K variant of less than 1 in 2000 cattle when using Bayesian analysis [12]. This newly developed assay system for K211 [12] will offer the possibility for genetic surveillance of cattle for rare pathogenic mutations that may be associated with BSE.



Texas BSE Investigation Final Epidemiology Report August 2005

Executive Summary

In June 2005, an inconclusive bovine spongiform encephalopathy (BSE) sample from November 2004, that had originally been classified as negative on the immunohistochemistry test, was confirmed positive on SAF immunoblot (Western blot). The U.S. Department of Agriculture (USDA) identified the herd of origin for the index cow in Texas; that identification was confirmed by DNA analysis. USDA, in close cooperation with the Texas Animal Health Commission (TAHC), established an incident command post (ICP) and began response activities according to USDA’s BSE Response Plan of September 2004. Response personnel removed at-risk cattle and cattle of interest (COI) from the index herd, euthanized them, and tested them for BSE; all were negative. USDA and the State extensively traced all at-risk cattle and COI that left the index herd. The majority of these animals entered rendering and/or slaughter channels well before the investigation began. USDA’s response to the Texas finding was thorough and effective.

Background of the Investigation

On June 10, 2005, USDA announced that the November 2004 inconclusive BSE sample tested positive on SAF immunoblot. The SAF immunoblot was run at USDA’s National Animal Disease Center (NADC) upon the recommendation of USDA’s Office of the Inspector General. Samples were sent to a World Organization for Animal Health (OIE) reference laboratory for BSE in Weybridge, England, for confirmatory tests. Farm A, located in Texas, was the suspected farm of origin for the index cow and was placed under hold order on June 20, 2005 pending confirmation of the positive results and DNA analysis of the herd. Weybridge confirmed the BSE positive on June 24, 2005. The carcass of the index cow had been disposed of by incineration in November 2004.

News Release

Texas Animal Health Commission

Box l2966 * Austin, Texas 78711 * (800) 550-8242 * FAX (512) 719-0719

Bob Hillman, DVM * Executive Director

For info, contact Carla Everett, information officer, at 1-800-550-8242, ext. 710, or mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000388/!

For immediate release---

State-Federal Team Responds to Texas BSE Case

The US Department of Agriculture announced June 29 that genetic testing has verified that an aged cow that tested positive for Bovine Spongiform Encephalopathy or BSE originated from a Texas beef cattle herd. Tissues for laboratory testing were initially collected from the animal in November 2004, and the carcass was incinerated and did not enter the human food, animal feed or fertilizer supply system. While tests in November indicated the animal did not have BSE, retesting in England in June confirmed the animal had the disease. The Texas Animal Health Commission (TAHC), the state’s livestock and poultry health regulatory agency, and USDA have jointly assigned a state-federal team to conduct the epidemiological investigation and response.

“The TAHC and US Department of Agriculture’s Veterinary Services are working with a complement of experts from federal and state animal health, food safety, public health and feed regulatory agencies to ensure the continued safety and wholesomeness of our meat supply,” said Dr. Bob Hillman, Texas state veterinarian and executive director of the TAHC, the state’s livestock and poultry health regulatory agency. “Epidemiological investigations are thorough and focus on verifying the herd of origin, and when, where and how the animal and potentially, any herd mates, were exposed to the abnormal prion, or disease agent, that causes BSE. Additionally, epidemiology investigations trace the infected animal’s movement and herd mates. Animals potentially exposed to the disease will be depopulated, with proper disposal. The animals will not be introduced into the human or animal food chain.”

The USDA’s BSE testing protocol requires testing of emaciated or injured cattle, cattle that exhibit central nervous system disorder, cattle unable to rise or to walk normally, and cattle that die of unknown causes. Since June 1, 2004, brain tissue samples from more than 394,000 cattle have been tested in the U.S. and were negative for BSE. Of those, 38,320 were tested in Texas, Dr. Hillman noted. BSE surveillance has been conducted in the U.S. since l990.

The U.S. has taken preventive measures against the introduction of BSE since l989, when prohibitions were placed on cattle and other ruminants from BSE-affected countries, noted Dr. Hillman. In 1997, the importation ban was extended to all of Europe.

Dr. Hillman said the U.S. Food and Drug Administration (FDA) in 1997 banned the use of ruminant-derived protein (from animals such as cattle and sheep) in feed for cattle and other ruminants. There is no evidence that BSE spreads from live animal to animal in the herd, but cattle can be exposed by eating feed that contains rendered protein from infected animals. “These measures taken by the USDA and the FDA are safeguards that work to protect livestock, and ultimately, our meat supply,” he said.


Second BSE case occurred in Texas, USDA says Jun 30, 2005 (CIDRAP News) – The United States' second case of bovine spongiform encephalopathy (BSE) was in a 12-year-old cow that came from a Texas herd and would have been made into pet food if it hadn't been flagged for BSE testing, federal officials announced yesterday evening.

US Department of Agriculture (USDA) officials said the cow was to be processed at a pet food plant in Waco, Tex., when it was diverted for testing because it couldn't walk. Officials didn't name the plant or say exactly where the cow came from. But an Associated Press (AP) report today identified the plant as Champion Pet Food in Waco and said the cow was already dead when brought there last November.

"The source herd is now under a hold order as we identify animals of interest within the herd," USDA Chief Veterinarian John Clifford said in a prepared statement. Investigators will look for cattle born within a year before or after the BSE-infected cow and any of the cow's offspring born within the past 2 years, he explained.

"If the age of the animal cannot be pinpointed, then we may expand our inquiry to include all animals in this herd before the feed ban went into place in 1997," Clifford said. To prevent BSE, the government banned putting cattle protein into cattle feed in August 1997.

The infected cow was incinerated, and no parts were used in human food or animal feed, according to the USDA. "The safety of our food supply is not in question," Clifford stated.

Because of the cow's age, the USDA suspects it became infected by eating contaminated feed before the government ban began in 1997. The USDA and the Food and Drug Administration (FDA) will try to trace the source herd's feed history, officials said.

The FDA will also check whether firms that may have processed meat-and-bone meal from animals from that herd have complied with the 1997 feed ban, Dr. Steve Sundlof, director of the FDA's Center for Veterinary Medicine, said at a news conference last night.

The Texas case is the first US BSE case in a native-born animal; Clifford said the cow lived on one farm all its life. The previous US case, found in December 2003, involved a Canadian-born dairy cow in Washington state.

An initial screening test on the Texas cow last November was inconclusive, and two confirmatory immunohistochemistry tests were negative. But early this month the USDA's inspector general ordered a Western blot test, which came back positive. Further confirmatory tests at an international reference lab in Britain were also positive, prompting the USDA to announce the findings last week.

The USDA waited for the results of DNA tests before announcing that the infected cow came from Texas. The step was necessary because parts of the infected cow were stored with those of four other cattle, causing some uncertainty, officials said.

"We felt that we had the correct herd; we wanted to identify that appropriately with DNA," Clifford said at the news conference. Investigators analyzed DNA from the infected animal and then looked for relatives in the presumed source herd by analyzing DNA from members of the herd, he said. The investigation turned up two cattle that are related to the infected cow, he added.

The AP report said Champion Pet Food is under contract to take samples from animals in poor health. The company's owner, Benjy Bauer, told the AP that his workers took samples from the cow and sent them to the Texas Veterinary Diagnostic Laboratory at Texas A&M University. The lab is one of several the USDA uses to screen cattle for BSE, the story said.

See also:

USDA news release

USDA fact sheet on BSE epidemiologic investiation

USDA press conference transcript

FDA Statement 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.


Correspondence Nature 457, 1079 (26 February 2009) doi:10.1038/4571079b;

Published online 25 February 2009

Rare BSE mutation raises concerns over risks to public health

Malcolm A. Ferguson-Smith1 & Jürgen A. Richt2

Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK Email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000388/! College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA

Atypical forms (known as H- and L-type) of bovine spongiform encephalopathy (BSE) have recently appeared in several European countries as well as in Japan, Canada and the United States. This raises the unwelcome possibility that variant Creutzfeldt–Jakob disease (vCJD) could increase in the human population. ...

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008

Thursday, July 10, 2008

A New Prionopathy update July 10, 2008

Saturday, February 28, 2009

NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009

SEAC 102/2

''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$


page 9 of 14 ;

30. The Committee noted that the results were unusual. the questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr. Tyrell noted that the feeling of the committee was that this did not represent a new agent but it was important to be prepared to say something publicly about these findings. A suggested line to take was that these were scientifically unpublishable results but in line with the policy of openness they would be made publicly available and further work done to test their validity. Since the BSE precautions were applied to IBNC cases, human health was protected. Further investigations should be carried out on isolations from brains of IBNC cases with removal of the brain and subsequent handling under strict conditions to avoid the risk of any contamination.

31. Mr. Bradley informed the Committee that the CVO had informed the CMO about the IBNC results and the transmission from retina and he, like the Committee was satisfied that the controls already in place or proposed were adequate. ...

snip... see full text


18. There is nothing to add to CMO's statement in January 1994, in relation to both the safety of meat and to the diagnosis in the 16 year old girl.




CJD FOURTH FARMER LINE TO TAKE, preparing for media storm ;




* in the USA, a 16 year old in 1978

* in France, a 19 year old in 1982

* in Canada, a 14 year old of UK origin in 1988

* in Poland, cases in people aged 19, 23 and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;


* Creutzfeldt's first patient in 1920 was aged 23



April 20, 2009

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

National Prion Disease Pathology Surveillance Center Cases Examined1

(December 31, 2008)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 42 32 28 4 0 0

1997 115 68 59 9 0 0

1998 93 53 45 7 1 0

1999 115 69 61 8 0 0

2000 151 103 89 14 0 0

2001 210 118 108 9 0 0

2002 258 147 123 22 2 0

2003 273 176 135 41 0 0

2004 335 184 162 21 0 13

2005 346 193 154 38 1 0

2006 380 192 159 32 0 14

2007 370 212 185 26 0 0

2008 383 228 182 23 0 0

TOTAL 30715 17756 1490 254 4 2

1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease acquired in Saudi Arabia; 5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded.

Rev 2/13/09 National


*5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; *6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded.



it would be interesting to know what year these atypical cases occurred, as opposed to lumping them in with the totals only.

are they accumulating ?

did they occur in one year, two years, same state, same city ?

location would be very interesting ?

age group ?

sex ?

how was it determined that nvCJD was ruled out ?

from 1997, the year i started dealing with this nightmare, there were 28 cases (per this report), up until 2007 where the total was 185 cases (per this report), and to date 2008 is at 182. a staggering increase in my opinion, for something that just happens spontaneously as some would have us believe. i don't believe it, not in 85%+ of all sporadic CJD cases. actually, i do not believe yet that anyone has proven that any of the sporadic CJD cases have been proven to be a spontaneous misfolding of a protein. there are many potential routes and sources for the sporadic CJD's. ...TSS

Sunday, April 12, 2009

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

Monday, April 20, 2009

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

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


Docket ID FDA-2002-N-0031 Docket Title Animal Proteins Prohibited in Ruminant Feed Document ID FDA-2002-N-0031-0132 Document Title Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed Delay of Effective Date

Completely Edited Version



page 29

Dr. Linda Detwiler

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. ...

snip...end...Dr. Linda Detwiler

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

Sunday, April 12, 2009

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

Sunday, May 10, 2009

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

Sunday, August 10, 2008 A New Prionopathy OR more of the same old BSe and sporadic CJD

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008


Tissue infectivity and strain typing of the many variants Manuscript 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. ...


Elsevier Editorial System(tm) for The Lancet Infectious Diseases Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary


TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. 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 2007. snip...

see full text 31 pages ;

Saturday, April 04, 2009

An unusually presenting case of sCJD-The VV1 subtype

Saturday, January 24, 2009

Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

2008 Annual Report

Saturday, May 2, 2009


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 ;

Friday, November 30, 2007


Sunday, April 12, 2009 BSE MAD COW TESTING USA 2009 FIGURES Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620

see full text ;

Monday, May 4, 2009

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

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

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