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From: TSS ()
Date: February 28, 2009 at 5:19 pm PST

SEAC 102/2



1. The Department for Environment, Food and Rural Affairs (Defra) has asked SEAC to consider a research article (Annex A) entitled "Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?" produced by the Veterinary Laboratories Agency.

2. Martin Jeffrey, the lead author of the article, will be present at the meeting to present an overview and answer questions.


3. IBNC is a rare1 neurodegenerative disease of adult cattle. This disorder has some clinical similarity to BSE and was initially recognised from histological examination of cattle brains submitted as part of the UK surveillance for BSE diagnosis in 1989. However, the brains of IBNC-affected cattle have pathological features which are clearly different from those seen in BSE. Most cases have been detected in Scotland, but it is not known if this is a true distribution or primarily because Scottish scientists have examined BSE negative cases in more detail. The last reported case of IBNC in an animal presented as a BSE suspect was in 2005, in an animal born in 1992.


4. SEAC first considered IBNC at its 14th meeting (April 1993) and emphasised the importance of defining the new condition in detail with

1 Between the years 1988 and 1991 IBNC occurred in Scotland with an incidence of 7 cases per 100,000 beef suckler cattle over the age of 6 years (from Annex A).

transmission studies and PrP examination. The next discussion was at the 19th meeting (June 1995), when the committee reflected on results of transmission studies in mice (VM, RIII, C57 and C57xVM mice) from brains of two cattle with IBNC. Some mice had shown signs of TSE disease, but it was suggested this could have been due to low level BSE contamination of the samples. The committee recommended that further investigations should be carried out on isolates from brains of IBNC cases with removal of the brain and subsequent handling under conditions that would prevent contamination.

5. At the 49th meeting (March 1998) the committee considered a further IBNC transmission study in which the brain from an IBNC case was removed under aseptic conditions. The mouse strains challenged were RIII, VM, C57BL, C57BL x VM and IM. These experiments ran for between 577 and 631 days and no clinical signs of transmission were evident. The Committee stated2 it was content that, although little was known about IBNC, it did not constitute a health risk to man because suspect IBNC cases would be taken as BSE suspects or caught by the Over Thirty Months (OTM) Scheme.

6. Annex B contains the minutes of the discussions on IBNC at previous SEAC meetings.


7. The research article "Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?" was published in September 2008. The cases studied concerned brains from cattle killed between 1993 and 2005 when they were between 5 and 15 years of age. All of the 15 cattle tested showed that the brains had abnormally accumulated prion protein.

8. Defra has asked that SEAC considers the VLA paper in order to confirm or revise its previous views on this disorder as:

. This is the first time IBNC has been shown to be associated with abnormal expression or accumulation of the prion protein.

. The previous transmission studies conducted in the 1990s were inconclusive and repeat studies are planned.

. IBNC is thought to be rare but the exact prevalence of the disorder is unknown, as IBNC would not be picked up through the

2 At 49th SEAC meeting (9th March 1998), paragraph 52, see Annex B.

active surveillance programme for BSE which uses rapid post-mortem tests to detect proteinase-K resistant PrPSc.

9. Additionally, TSE controls on older cattle have changed since the previous SEAC advice in 1998. For example the OTM Scheme, which was in operation then, has now been replaced with testing of cattle slaughtered for human consumption aged over 48 months. Other controls remain, such as compulsory notification of suspected BSE, ante-mortem inspection, specified risk for cattle slaughtered for human consumption and a ban on cattle born or reared in UK before 1st August 1996 entering the food chain.


10. VLA are hoping to carry out further mouse transmission studies of IBNC cases as part of a larger project, on TSE molecular sciences, about which Defra is currently in advanced negotiations with VLA. If new cases of IBNC occur, it is planned that the brains from 2 cases of IBNC will be obtained and bioassayed in transgenic mouse lines, expressing bovine PrP or ovine PrP (PrP genotype AHQ), developed by the VLA.


11. The committee is asked to consider:

. if the paper changes the previous opinion of SEAC in 1998?

. if members have any comments on the further research planned?




A copy of the paper "Idiopathic Brainstem Neuronal


full text ;

>>>All of the 15 cattle tested showed that the brains had abnormally accumulated prion protein. <<<

SO, what will they call this strain of mad cow disease ???

IF i recall, not too long ago, i said ;

>>>Considering that Mad Cow disease of all documented phenotypes, either the c-BSE, or the atypical h-BSE and or the l-BSE, ALL of which have been documented in North America, how many more, who knows, but they seem to be throwing all there marbles in the pot now by calling the h-type BSE 'familial'. what happens if we come up with another strain ? <<<

Wednesday, February 11, 2009 Atypical BSE North America Update February 2009


Considering that Mad Cow disease of all documented phenotypes, either the c-BSE, or the atypical h-BSE and or the l-BSE, ALL of which have been documented in North America, how many more, who knows, but they seem to be throwing all there marbles in the pot now by calling the h-type BSE 'familial'. what happens if we come up with another strain ? kinda like the sporadic FFI, that's not familial, what's that all about ? considering the many different strains of the typical scrapie 20+, and then the atypical Nor-98 Scrapie, which the USA has documented 6 cases the last i heard, and the thought of more than one strain of CWD in deer and elk, where will the next year, 4 years, 8 years, and beyond take us in the world of human and animal Transmissible Spongiform Encephalopathy and 'sound science' in the USA ? WILL the New Administration see the enfamous enhanced bse surveillance program of 2004 for what it was, a fraud, and have a 'redo' ? WE can hope i suppose. ...TSS

Both of the BSE cases ascertained in the US native-born cattle were atypical cases (H-type), which contributed to the initial ambiguity of the diagnosis. 174, 185 In Canada, there have been 2 atypical BSE cases in addition to the 14 cases of the classic UK strain of BSE2: one was the H-type, and the other was of the L-type.198


source :

Wednesday, October 08, 2008 Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

Sunday, December 28, 2008

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

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

Wednesday, January 28, 2009

TAFS1 Position Paper on BSE in small ruminants (January 2009)


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

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


Wednesday, January 28, 2009

TAFS1 Position Paper on Specified Risk Materials (January, 2009)


(January 2009)

TAFS1 Position Paper on Specified Risk Materials

TAFS1 Position Paper on Testing of Cattle for BSE (Revision January 2009)

Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein

DOI: 10.3201/eid1412.080941

Wednesday, August 20, 2008

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

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.


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

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, ...


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."

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

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP],F2400_P1001_PUB_MAIL_ID:1010,39963

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.

sporadic Fatal Familial Insomnia


Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam


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.

The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003

doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Original TextXavier Bosch

"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."


MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

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?

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

Creutzfeldt Jakob Disease

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008


Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

CJD TEXAS (cjd clusters)


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.

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


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

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

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

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