NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009
NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS FROM THE VETERINARY LABORATORIES AGENCY
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.
PREVIOUS CONSIDERATION BY SEAC
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.<<<>>>
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, October 08, 2008 Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
''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
(page 8, bottom paragraph first, then top paragraph at bottom...TSS)
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE MINUTES OF THE 19TH MEETING HELD ON 21 JUNE 1995 AT THE CENTRAL VETERINARY LABORATORY
Idiopathic Brain Stem Neuronal Chromatolysis (IBNC)
29. Mr. Bradley described the results of transmission studies in mice from brains of two cows with IBNC (paper SEAC 19/8). At the previous meeting of SEAC, and at the review of R&D, it had been announced that there was no clinical observation of a scrapie-like disease in mice: this information had proved to be INCORRECT for a number of reasons. Of the mice inoculated with brain tissue from the first cow, there had been mild transient clinical signs, one had shown equivocal lesions of SE but PrP studies had proved negative. From the second cow there were two definite cases of SE though the lesion distribution and incubation period were not the same as seen in mice inoculated with brain from BSE cases or any characterized strain of scrapie. The lesions in these two mice were PrP positive. There was no obvious evidence of any mix up though one possible area of cross-contamination was during the necropsy in the Perth VIC. More evidence would be needed and further transmission studies to validate the results and proposals were put forward for further study.
30. The Committee noted that the results were unusual. They questioned whether there could be coincidental BSE infection or contamination with scrapie. Dr Tyrrell 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.
Agenda Item 7 - Any Other Business: the Hounds Survey
26. Paper SEAC 19/7 responded to a request from the Committee for a re-evaluation of the pathology material in the hounds survey to determine whether anything further could be derived from the available data.
27. In discussion of the options for further work set out in the paper most members felt that the study had been badly carried out and there would be little value in spending more money to try and improve the interpretation of the data. It was particularly significant that no clinical data were available, although the Committee were reminded that most of the hounds were clinically normal culls. Dr Kimberlin was concerned about the lack of results from the study. Any further work would require a control but this could be obtained by exposing hounds to BSE which would also help to answer questions about species sensitivity, thereby serving more than one purpose. The use of immunocytochemistry was fairly robust and would enable the work to be brought to a satisfactory conclusion. Dr Kimberlin's view that this would be necessary was confirmed by an article, circulated at the meeting, showing that the predictive protein sequence was the same in dogs as in cattle. Mr Eddy noted that such an experiment could be expensive and it would be necessary to know what questions were to be addressed.
28. Concluding, Dr Tyrrell said that there was a range of opinions in the Committee from those who thought further work a waste of time to those who wished to do limited further experiments using immunocytochemistry. The Committee did not suggest transmission studies and thought that the lack of clinical data was a major weakness. Hounds were initially studied on the recommendation of the Southwood Committee because they were perceived as a ''high risk'' population exposed to large quantities of potentially infective bovine tissues. SINCE THEN, HOWEVER, A RANGE OF OTHER SPECIES HAD BEEN IDENTIFIED WITH TSEs, AND THE STUDY OF HOUNDS WAS THEREFORE LESS CRITICAL. ...
for the life of me i do not understand that last sentence???
you can see DEFRA comment to me about the Hound Study here, but if anyone claims BSE and or TSE will not transmit to hounds, well, i would beg to differ. ... they don't know, but evidence to date sure looks like something transmitted to the hounds. so why did they halt all scientific investigations of this $$$ same as with the INBC BSE cover-up. they don't want to know, and they don't want you to know. old deep throat warned me about Mr. Ray Bradley 10 years ago. ...TSS
2009 31 March 2009 - A summary of the 102nd SEAC meeting (35 KB) held on 4th March 2009
SEAC noted that IBNC appeared to be a rare disease that occurred in older cattle, predominantly as single cases, although it is possible that surveillance may not detect all cases. Biochemical studies suggested that the prion protein may play a role in the disease. However, it is unclear whether the normal form of the protein or an abnormal form is involved. Studies are required to determine whether IBNC is transmissible or not. SEAC concluded, noting that specified risk material controls are in place to prevent cattle brain from entering the food supply, that current data on IBNC do not suggest it presents a risk to human health.
>>> All of the 15 cattle tested showed that the brains had abnormally accumulated prion protein. <<<
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)
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007
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
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
Wednesday, January 28, 2009
TAFS1 Position Paper on Specified Risk Materials (January, 2009)
TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation
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
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.
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
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, ...
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."
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]
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
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
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.
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."
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:
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  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
USA PRION UNIT BLOG
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)
USA WRITTEN CJD QUESTIONNAIRE ???
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
FINAL AUTOPSY DIAGNOSIS
I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518