SEAC 99th meeting on Friday 14th December 2007

SEAC August 2010 Drayton Farm report update and more

2010-08-12T15:48:00.000-07:00

2010 10 August 2010 -A report of SEAC’s 17 June discussion about a risk assessment on Drayton farm, Warwickshire is available.

Spongiform Encephalopathy Advisory Committee

JOINT MEETING OF UK TSE COMMITTEES, 17th JUNE 2010. SUMMARY

Horizon scanning

The Committee Chairs identified the main issues they would be dealing with as follows:

ACDP TSE Working Group

• Infection control guidance is under constant review.

• Much progress has been made in making endoscopy safer through development of less invasive approaches and techniques to reduce risk of potential contamination, but more remains to be done.

• Work is beginning with surgeons on risk reduction in relation to other invasive procedures where instruments are difficult to decontaminate. Advisory Committee on Decontamination Science & Technology

• Quality assurance for decontamination procedures.

• Ways of keeping used surgical instruments wet/damp to maximise removal of protein.

• Introduction of anti-prion agents into validated decontamination cycles. Manufacturers need to know what is required and Trusts need to recognise vCJD transmission risks from conventionally decontaminated surgical instruments.

• Ways of minimising the number of discarded endoscopes and other instruments. Advisory Committee on the Safety of Blood Tissues and Organs

• Risk assessment and risk management of a wide spectrum of issues from blood to liver transplantation.

• Achieving a more consistent approach to consent for blood transfusion across the UK following the recent public consultation. Spongiform Encephalopathy Advisory Committee

• Obtaining more data on vCJD prevalence.

• Preventing new TSE epidemics.

• Ensuring that cost saving measures do not result in prevention of TSE transmission being compromised. CJD Incidents Panel

• Applying new prevalence data when available.

• Engagement of people who need to change practice on the ground.

• Coping with changes caused by cost pressures.

Conclusions

It was agreed that the presentations/discussions had achieved an interesting and useful day for participants. Whilst the purpose of the meeting was not necessarily to reach conclusions or provide advice for Government, there was consensus on the following:

• The TSE committees work well together.

• Advice should continue to be based on the available scientific evidence.

• Infection control and decontamination practice had improved widely as a result of the work on prevention of TSE

http://www.seac.gov.uk/summaries/tse-meeting-summary.pdf

10 August 2010 - A report of the 17th June 2010 Joint Meeting of the UK Committees (the Advisory Committee on Dangerous Pathogens TSE Working Group, the Advisory Committee on Decontamination Science and Technology, the Advisory Committee on the Safety of Blood Tissues and Organs, the CJD Incidents Panel and SEAC) that advise Government on TSEs is available.

2010

RESIDUAL TSE RISK AT DRAYTON FARM

Drayton farm (in Warwickshire) has been a site of research projects involving experimentally infected TSE animals. Due to the long incubation periods and on site farm bio-security, the animals were kept such that they had access to pasture for most of the year. Infected animals were allowed to graze on pasture and pasture contamination from infected grazing animals contributes to the potential TSE risk of infection for the farm.

The experiments at Drayton have now ended and Defra asked the Veterinary Laboratories Agency to perform a quantitative risk assessment on the residual risks associated with the land where infected animals grazed, or were subject to composted manure or the release of wastewater. This work is of wider interest than just Drayton farm as there are several farms in the UK where such experimentally infected animals were kept.

Defra and the Devolved Administrations asked the advice of SEAC on the risk assessment.

SEAC considered this on 17 June 2010 in London.

Further background material on this subject can be found at:

http://randd.defra.gov.uk/Default.aspx?Menu=Menu&Module=More&Location=None&ProjectID=16637&FromSearch=Y&Publisher=1&SearchText=SE0256&SortString=ProjectCode&SortOrder=Asc&Paging=10#Description

http://www.seac.gov.uk/papers/drayton-background.pdf

Spongiform Encephalopathy Advisory Committee

17 JUNE 2010.

Drayton Farm Risk Assessment

The Committee was presented with a Risk Assessment prepared by the Veterinary Laboratories Agency, which assesses the residual TSE risk at Drayton Farm due to the presence of experimental animals and field cases on the site, and the land spreading of composted manure and wastewater. SEAC last considered this issue at its 53rd Meeting on 21-22 September 1998.

Several Members commented that the methodology of the Risk Assessment was sound. However, concerns were raised that some of the specific input values to the risk assessment are problematic because of the large number of assumptions on which these are based.

A Member suggested that the outputs of the Risk Assessment needed to be checked against the available data to ascertain whether these were consistent. For example, there are data from Iceland and the Ripley flock which clearly show that there is a risk from re-introducing livestock onto land which has previously held a scrapie infected flock. This should have raised questions in the minds of the authors about why the model comes out with a very low risk.

Additionally, a Member suggested that more consideration should be given to whether the theoretical assumptions based on regulations, actually correlate with the real life activity on the farm.

A Member noted that the Risk Assessment adds little to SEAC’s previous advice on this issue provided in 1998, and the Chair stated that because there are no additional data available, it is difficult for SEAC to update its previous advice on this issue.

The Committee agreed that:

• The methodology of the Risk Assessment is sound; however:

• there are serious problems with the input assumption to the model, given the lack of data;

• The outputs of the model are therefore uncertain; and

• There is no need to revise SEAC’s advice of 21-22 September 1998. Extract from Minutes of SEAC 53, 21/22 September 1998.

Item 9 - Disposal of excreta from cattle experimentally infected with BSE (paper SEAC 53/6)

42. The Committee had previously considered options for disposal of waste from cattle exposed to BSE in January 1998. Members had before them paper SEAC 53/6 providing details of possible disposal options for excreta from two long term experiments which were starting at Drayton Experimental Husbandry Farm. Alternative measures to incineration had been examined due to the practical and cost problems of pursuing this option for such a long period. Further information about the farm and methods of disposal which would comply with Good Agricultural Practice were provided. The key change was the proposal to utilise crops from the farm and the Committee were asked to note the concerns of a neighbouring farmer with a watercourse on his property which ran through the experimental farm about possible run off.

43. The Committee noted that the animals were being held in brand new buildings and that there were arrangements for separation of solid and liquid waste. The housing arrangements and husbandry procedures would ensure rigorous separation of the different groups of animals. It was reported that the Committee’s views would be applied to disposal arrangements for excreta from the sheep experimentally exposed to BSE at the Institute for Animal Health.

44. The Committee confirmed their previous advice that the waste from challenged animals should be incinerated for the first 28 days (which represented an extended “safe” clearance time from cattle intestines) and that, thereafter, the excreta should be composted for one year. They agreed that there was no scientific basis why composted material should not be spread on land as fertiliser prior to planting crops which could then be used for human consumption or animal feed, although coppicing may present the most desirable option from the point of view of public perception. However, the Committee felt it would be prudent not to spread the material on pasture which would be grazed by cattle, and that the experimental animals which were part of the cattle bioassay experiment should not be given the food from the farm. This was in order to prevent any future challenge of a positive result that contaminated food may have been a route of exposure which might lead to a consequent claim that the tissue being assayed was not really positive.

http://www.seac.gov.uk/papers/drayton-report.pdf

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area

T.A. Nichols,1,2 Bruce Pulford,1 A. Christy Wyckoff,1,2 Crystal Meyerett,1 Brady Michel,1 Kevin Gertig,3 Edward A. Hoover,1 Jean E. Jewell,4 Glenn C. Telling5 and Mark D. Zabel1,*

1Department of Microbiology, Immunology and Pathology; College of Veterinary Medicine and Biomedical Sciences; Colorado State University; Fort Collins, CO USA; 2National Wildlife Research Center; Wildlife Services; United States Department of Agriculture; Fort Collins, CO USA; 3Fort Collins Utilities; Fort Collins; CO USA; 4Department of Veterinary Sciences; Wyoming State Veterinary Laboratory; University of Wyoming; Laramie, WY USA; 5Department of Microbiology, Immunology, Molecular Genetics and Neurology; Sanders Brown Center on Aging; University of Kentucky; Lexington, KY USA Key words: prions, chronic wasting disease, water, environment, serial protein misfolding cyclic amplification Abbreviations: CWD, chronic wasting disease; sPMCA, serial protein misfolding cyclic amplification; PrPC, cellular prion protein; PrPSc, disease-related, misfolded murine PrP; PrPCWD, disease-related, misfolded cervid PrP; PrPRES, protease-resistant PrP; FCWTF, Fort Collins water treatment facility

Chronic wasting disease (CWD) is the only known transmissible spongiform encephalopathy affecting free-ranging wildlife. Although the exact mode of natural transmission remains unknown, substantial evidence suggests that prions can persist in the environment, implicating components thereof as potential prion reservoirs and transmission vehicles.1-4 CWD-positive animals may contribute to environmental prion load via decomposing carcasses and biological materials including saliva, blood, urine and feces.5-7 Sensitivity limitations of conventional assays hamper evaluation of environmental prion loads in soil and water. Here we show the ability of serial protein misfolding cyclic amplification (sPMCA) to amplify a 1.3 x 10-7 dilution of CWD-infected brain homogenate spiked into water samples, equivalent to approximately 5 x 107 protease resistant cervid prion protein (PrPCWD) monomers. We also detected PrPCWD in one of two environmental water samples from a CWD endemic area collected at a time of increased water runoff from melting winter snow pack, as well as in water samples obtained concurrently from the flocculation stage of water processing by the municipal water treatment facility. Bioassays indicated that the PrPCWD detected was below infectious levels. These data demonstrate detection of very low levels of PrPCWD in the environment by sPMCA and suggest persistence and accumulation of prions in the environment that may promote CWD transmission.

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CWD has been endemic in the area for forty years, and it remains unclear how long prions can persist in the environment. If persistent for at least several years, CWD prions deposited into the environment from thousands of infected carcasses may accumulate on soil and vegetation such that it can be washed into surface water draining the basin during snowmelt or rainstorms. Symptomatic and asymptomatic positive animals can also contribute to environmental CWD load via biological materials such as saliva, blood, urine and feces.5-7,32,36,38 Deer and elk defecate approximately 900,000 kg of feces and urinate approximately 14 million liters of urine in the area immediately surrounding the Cache la Poudre river per year.39-42 Although urine and feces likely contain much lower prion loads than blood or saliva, the sheer amount of excreta may contribute significantly to overall environmental prion contamination. The data presented here demonstrate that sPMCA can detect low levels of PrPCWD in the environment, corroborate previous biological and experimental data suggesting long term persistence of prions in the environment2,3 and imply that PrPCWD accumulation over time may contribute to transmission of CWD in areas where it has been endemic for decades. This work demonstrates the utility of sPMCA to evaluate other environmental water sources for PrPCWD, including smaller bodies of water such as vernal pools and wallows, where large numbers of cervids congregate and into which prions from infected animals may be shed and concentrated to infectious levels.

see full text ;

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf

Wednesday, October 14, 2009

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area

http://chronic-wasting-disease.blogspot.com/2009/10/detection-of-protease-resistant-cervid.html

Fifth threat The precise nature of prions remains elusive. Very recent data indicate that abnormal prion protein (PrPTSE) can be generated from the brains of normal animals, and under some conditions (including contaminated waste water) PrPTSE can be destroyed whereas the BSE infectious titre remains almost unchanged, a finding that underlines the possibility of having BSE without any detectable diagnostic marker. These are just two areas of our incomplete knowledge of the fundamental biology of prions which constitute a fifth threat to the European approach to prion diseases.

http://www.neuroprion.org/en/np-neuroprion.html

The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion. These findings emphasize the critical importance of understanding tissue distribution of L-type BSE prions in cattle because, among the current administrative measures for BSE controls, the specified risk materials removal policy plays a crucial role in consumer protection.

In Japan, atypical BSE was detected in an aged Japanese Black cow (BSE/JP24) (8). We recently reported the successful transmission of BSE/JP24 prions to cattle and showed that the characteristics of these prions closely resemble those of L-type BSE prions found in Italy (9). In this study, we report the peripheral distribution of L-type BSE prions in experimentally challenged cattle.

http://www.cdc.gov/EID/content/16/7/1151.htm

Thursday, August 12, 2010

Seven main threats for the future linked to prions

http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html

http://prionpathy.blogspot.com/

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

SEAC Agenda 104th meeting on Friday 5th March 2010

2010-02-27T18:23:00.000-08:00

SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

Agenda 104th meeting on Friday 5th March 2010

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009 at Nobel House, 17 Smith Square, London SW1P 3JR.

ITEM 3 – CURRENT ISSUES 6. SEAC was updated on the Strain Typing Expert Group report on a suspected BSE case in a historic Scottish goat. The goat had originally been diagnosed with scrapie in 1990. However, retrospective tests have now confirmed that the goat isolate was indistinguishable from BSE. The results do not affect the SEAC position statement on the Potential Human Health Risks from Changes to Classical Scrapie Controls published in February 2008. That statement concluded that if BSE was confirmed, the fact that the animal was born prior to the introduction of the ruminant feed ban meant it could have been exposed to BSE contaminated feed.

snip...

7. Atypical scrapie has been confirmed in a sheep from the New Zealand (NZ) national flock. NZ authorities have stated that because atypical scrapie is distinct from classical scrapie, they consider that NZ remains “free from scrapie.”

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8. The Advisory Committee on the Safety of Blood, Tissues and Organs has recommended that blood prion filtration should be used to treat patients with no prior evidence of dietary exposure to BSE. SEAC noted that the Department of Health Ministers were currently considering this recommendation.

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10. In light of the recent high profile dismissal of a Government scientific advisor, the Chair said he would be concerned if any member of a Scientific Advisory Committee (SAC) were to be dismissed for expressing scientific opinions whether or not these contradict Government policy. It was added that SEAC adheres to the principles of the Philips’ report and clearly recognises the distinction between risk management and risk assessment: this distinction may not be so clearly defined in the work of other SACs. The Chair said that there was to be a meeting of scientists engaged in work on government committees and that Professor Graham Medley would be representing SEAC.

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ITEM 4 – UPDATE ON CJD EPIDEMIOLOGY

11. Professor Richard Knight (National CJD Surveillance Unit) provided the Committee with the latest figures for the number of clinical vCJD and sporadic CJD (sCJD) cases. To date there had been 170 definite or probable clinical cases of vCJD in the UK - 167 from probable dietary infection with BSE and three from probable vCJD infection via transfusion of blood from donors who later developed vCJD. Of the 150 cases tested all were codon 129MM. Four cases are still alive. The number of deaths from vCJD peaked at 28 in 2000 and had since declined with two known deaths so far in 2009. The median age of death is 30 years of age.

12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case.

13. Professor Knight explained that one MV genotype case had been classified as possible vCJD as clinical features were consistent with the disease. However, it had not been possible to undertake neuropathological examination post mortem so the diagnosis could not be confirmed. The clinical profile of this MV case was consistent with that observed for MM cases.

14. Professor Knight summarised data on sCJD cases stating that from May 1990 to September 2009, 1080 cases of sCJD had been identified in the UK with a mean age at death of 67 years and genotype distribution of 63% MM, 19% MV and 18% VV at codon 129 of the prion protein gene.

15. Professor Knight also provided a brief report on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). The initial eleven cases described by Gambetti2 exhibited a mean age of onset of 62 years and mean disease duration of 20 months. Eight out of ten had a family history of dementia and were codon 129VV. Cases had minimal spongiform change and minimal immunohistochemical stained PrP deposits with distinct patterns in the cortex and cerebellum. Western Blot (WB) also shows a minimal amount of PrPres present. Further studies by Gambetti have now identified codon 129MV and MM cases which have a longer disease duration and exhibit some PK resistance. The cases did not have clinical profiles typical for sCJD. A UK case and a Dutch case have also been identified, with characteristics not inconsistent with the Gambetti studies.

16. Professor Knight added that due to the unique clinical presentation of the disease it was likely that at least some cases of disease would not be identified for referral, making it hard to obtain complete data on this disease. However, it was likely that a case would be identified as a prion disease at autopsy and the WB currently used would be able to identify the unique profile which categorises this disease. A retrospective review of the NCJDSU brain bank is underway to look for more cases.

17. A Member asked whether the recent review of neuropathology archives in the UK would have identified PSPr. Professor Knight responded that it would be dependent on the type of WB used at the time which is currently not known. The use of appropriate WB methodology would be an issue in accurately identifying the relevant characteristics.

18. One Member was not convinced by the characterisation of this disease, adding that clinical cases classified as Alzheimer’s Disease have shown similar laddering profiles in WB, protease resistant fragments and the presence of abnormal PrP. The disease has, to date, not been shown to be transmissible which means it should not yet be categorised a prion disease under the current terminology.

19. Summing up, the Chair noted that it was clear that more information was required to fully characterise and fill knowledge gaps regarding this disease. It was important that its unique pathology be more widely recognised to enable future diagnosis and enable tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise the disease further.

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ITEM 74 – CATEGORY 3 ANIMAL PRODUCTS IN FERTILISERS (SEAC 103/3)

29. The Chair reminded Members that in 2005 the Committee had considered a release assessment which evaluated the amount of potential infectivity available in the soil of non-pasture land following the application of Category 3-derived5 fertiliser.

European Regulations are now being renegotiated and Defra are considering whether it is appropriate to seek a relaxation of rendering requirements (see paper 103/3). In order to inform this consideration, Defra commissioned a full Risk Assessment (RA) which was completed in 2008 which SEAC is now invited to consider.

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ITEM 8 – FUTURE OPERATION OF SEAC (SEAC 103/4)

40. The Chair reminded Members that he had recently written to them about proposals on the future operation of SEAC. The Chair asked Members of the Committee for their views on the suggestion that SEAC should henceforth aim to conduct the majority of its business in correspondence, only meeting when there was a major new development, or a significant amount of business over a short period.

41. Some Members were in favour of what was being proposed and suggested that business conducted by e-mail could usefully be confined to a scheduled period, or periods, in the year.

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http://www.seac.gov.uk/papers/104-1.pdf

MODELLING BSE SURVEILLANCE STRATEGIES IN CATTLE ISSUE

http://www.seac.gov.uk/papers/104-4.pdf

SEAC 104/2

ESTIMATING THE PREVALENCE OF SUBCLINICAL vCJD

ISSUE

1. The prevalence of subclinical vCJD in the UK is highly uncertain. Current estimates are based on the Hilton et al data on abnormal prion protein (PrPvCJD) in stored appendix samples, however the National Anonymous Tonsil Archive (NATA) study is on-going, and a further study of appendices has been commissioned. In addition, a pilot to assess the feasibility of a post mortem study to test spleen tissue has also been commissioned. The NATA data are currently within the confidence intervals of the Hilton data, but there remains a possibility that once completed, the data from these studies might be discrepant.

2. All samples in the NATA survey (over 80,000) have tested negative by EIA. However, one of 10,000 samples re-tested by IHC has given a positive result in one follicle. Extensive further testing of this sample has produced negative results.

3. Prevalence of infective material in subclinical individuals is a key factor determining the risks of secondary vCJD transmission via surgery, or donated blood, tissues or organs. To assess these risks, presence of PrPvCJD has been used as a surrogate indicator of infectivity, though the relationship between the two is not fully established.

4. In addition, risk assessments have assumed that in principle, the same “prevalence” would drive all transmission risks. For example, if the prevalence of sub-clinical infection was “1 in x”, then 1 in every x surgical procedures encountering any lymphoid tissue (e.g. tonsil, appendix or spleen) would meet with infective material. Similarly, 1 in every x blood donations would be infective. At present, this means that assessments are based primarily on the Hilton et al appendix results. This approach may not be appropriate if the presence of PrPvCJD varies markedly by site, and possibly over time. Nor would one necessarily expect different “prevalence studies” to be mutually consistent.

5. The Committee is asked to consider the following questions:

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http://www.seac.gov.uk/papers/104-2.pdf

SEAC104/3

VCJD TRANSMISSION VIA BLOOD COMPONENTS: CAN A MORE PLAUSIBLE RANGE OF SCENARIOS BE ESTABLISHED?

ISSUE

1. The Department of Health (DH) uses a wide range of possible scenarios for blood borne transmission of vCJD, to support risk management. However, a number of those scenarios overpredict the number of clinical cases of vCJD that have resulted so far from blood-borne transmission. DH analysts have drafted the paper attached at Annex A, which assesses the possibility of revising these scenarios so as to be more consistent with the available “positive” and “negative” evidence on human transmission, as well as with the findings of animal studies.

2. DH would like SEAC’s advice on the establishment of a more plausible range of scenarios. SEAC is asked to consider:

* Acknowledging the crude nature of the calculations offered, have any major factors been misrepresented or overlooked?

* Are members aware of any more sophisticated analysis that addresses the consistency of blood-borne transmission scenarios with observed case numbers?

* Given the apparent consistency problem, can the existing range of inputs on infectivity, prevalence of infective donors and susceptibility to disease be reconciled with the data by invoking plausible further hypotheses - and if so, what are they?

* If not, should some of the existing input ranges now be regarded as implausible - singly or in combination - and if so, which?

* Should additional scenarios now be regarded as plausible - and if so, how should the current input ranges be extended?

* Can the Committee suggest any further lines of investigation that could be implemented relatively quickly, and could throw further light on the numbers of vCJD cases likely to result from blood-borne transmission?

THE DH SCENARIOS

3. The current scenarios are based on three main inputs:

• the prevalence of infective donors;

• vCJD infectivity (levels and timing) in blood components; and

• susceptibility of recipients to clinical disease.

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http://www.seac.gov.uk/papers/104-3.pdf

Greetings,

HAVE we come to a point to where sub-clinical disease is an acceptable factor ???

LEGALLY, is it o.k. to be sub-clinically infected from a contaminated product ???

IF SO, what is the legality from the second passage infection from that sub-clinical host to clinical infection via the pass it forward and or friendly fire mode of transmission for any iatrogenic Transmissible Spongiform encephalopathy to second, third, fourth passage ???

I THOUGHT also, it would be nice if SEAC would have included the figures on sporadic CJD in there report, which they failed to do. they seem to come up with all these mathematical formulas, but fail to show you the numbers. so please allow me to show you some numbers on sporadic CJD ;

IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ;

However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52).

IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76

http://www.eurocjd.ed.ac.uk/sporadic.htm

CJD RISING SWITZERLAND

CJD is a predominantly sporadic disorder but can also occur as a dominantly inherited or infective condition. Only one of the 26 most recent confirmed cases was identified as carrying a disease related mutation of the PRNP gene, none had identifiable iatrogenic exposure, and none resembled variant CJD. Thus 25 of the 26 cases appear to be sporadic cases. Sporadic CJD is distributed worldwide with a reported incidence of about one in a million per year. Raised awareness of the disease in recent years could account for an increase in reported cases of CJD, although neither an increase in the average age of patients nor more frequent recognition of CJD amongst residents of nursing homes (where dementing illness is prevalent and misdiagnosis might be expected) were seen in the Swiss cases. Moreover, improved ascertainment as an explanation for the observed increase would imply levels of under-reporting in countries other than Switzerland, which appear implausible. The authors of the Lancet report suggest that the rise in cases might be due to some form of unidentified iatrogenic transmission or to exposure to a zoonotic source of infection, though cases do not resemble variant Creutzfeldt-Jakob disease (vCJD). The ongoing surveillance of CJD in Switzerland and the rest of Europe is essential to monitor the situation to see if this rise is sustained in Switzerland, and if a similar rise occurs in other countries (see http://www.eurocjd.ed.ac.uk).

http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1921

Prion data suggest BSE link to sporadic CJD Declan Butler

Predicting the number of cases of Creutzfeldt-Jakob disease (CJD) in people as a result of transmission of bovine spongiform encephalopathy (BSE) has just got more difficult.Whereas it was thought that BSE only caused a new form of the disease called variant CJD (vCJD), a study in mice from a team led by John Collinge at University College London suggests that it may also cause a disease indistinguishable from the commonest form of classical, or 'sporadic', CJD (E.

http://www.nature.com/nature/journal/v420/n6915/full/420450a.html

Mouse model sheds new light on human prion disease

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Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.

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http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm

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

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

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed

http://www.nature.com/emboj/journal/v21/n23/full/7594869a.html

What about CJD in the USA ?

USA sporadic CJD cases rising ;

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

CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

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

WHY DO FARMERS AND THEIR WIVES WITH BSE HERDS, ONLY HAVE SPORADIC CJD ???

Monday, May 19, 2008

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

http://bseinquiry.blogspot.com/

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 HAS STILL FAILED TO EXPLAIN THIS ;

Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl

Journal European Journal of Epidemiology Publisher Springer Netherlands ISSN 0393-2990 (Print) 1573-7284 (Online) Issue Volume 1, Number 1 / March, 1985

P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron

(1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France

Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology

P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron

(1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France

Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology

http://www.springerlink.com/content/j344470112792q50/

http://www.springerlink.com/content/j344470112792q50/fulltext.pdf?page=1

2. Sporadic CJD normally occurs in people in their 50s and 60s although it can occur more rarely in younger age groups. Until this year the youngest case of sporadic CJD in the UK had been in a 34 year old. Other countries, howver, have reported sporadic CJD in teenagers. Those we know about are;

* 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.

full text ;

http://collections.europarchive.org/tna/20081106132604/http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.PDF

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

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

ALSO, SEAC STATES HERE ;

20. The pathogenesis of BSE and classical scrapie in sheep and BSE in non-human primates is generally considered to be a reasonable model for the pathogenesis of vCJD in humans....

IF THAT is the case, then why is it not the same for sporadic CJD and typical scrapie, because typical scrapie transmits to primates by their non-forced oral consumption of infectious material, and or the atypical Nor-98 Scrapie (cause it's either scarpie or bse, you have to call it something, or name it something else), which is very similar and is encoded by distinct prion types very similar to sporadic CJD type 1 ?

Monday, December 14, 2009

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

http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html

OH, and about the figures SEAC presented for nvCJD in the USA, what's that all about ?

12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case...

I DON'T understand WHY SEAC would state three in the USA, and then stipulate that TWO were presumed to have occured in the UK, BUT fail to stipulate that the 3rd was either SAUDI ARABIA linked, or USA linked ? it was just odd to me how it was worded. SEAC made sure the UK source for the USA was the UK, but fail to explain that for the Saudi Arabia case of nvCJD...see next link below;

Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

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

update October 2009

T. Singeltary

Bacliff, TX, USA

Background:

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

Methods:

12 years independent research of available data

Results:

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

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf

International Society for Infectious Diseases Web: http://www.isid.org

http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html

http://transmissiblespongiformencephalopathy.blogspot.com/

Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html

my comments to PLosone here ;

http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd

Sunday, February 14, 2010

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html

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

http://seac992007.blogspot.com/

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009

2010-02-04T13:52:00.000-08:00

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009

snip...

ITEM 4 – UPDATE ON CJD EPIDEMIOLOGY

11. Professor Richard Knight (National CJD Surveillance Unit) provided the Committee with the latest figures for the number of clinical vCJD and sporadic CJD (sCJD) cases. To date there had been 170 definite or probable clinical cases of vCJD in the UK - 167 from probable dietary infection with BSE and three from probable vCJD infection via transfusion of blood from donors who later developed vCJD. Of the 150 cases tested all were codon 129MM. Four cases are still alive. The number of deaths from vCJD peaked at 28 in 2000 and had since declined with two known deaths so far in 2009. The median age of death is 30 years of age.

12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case.

13. Professor Knight explained that one MV genotype case had been classified as possible vCJD as clinical features were consistent with the disease. However, it had not been possible to undertake neuropathological examination post mortem so the diagnosis could not be confirmed. The clinical profile of this MV case was consistent with that observed for MM cases.

14. Professor Knight summarised data on sCJD cases stating that from May 1990 to September 2009, 1080 cases of sCJD had been identified in the UK with a mean age at death of 67 years and genotype distribution of 63% MM, 19% MV and 18% VV at codon 129 of the prion protein gene.

15. Professor Knight also provided a brief report on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). The initial eleven cases described by Gambetti2 exhibited a mean age of onset of 62 years and mean disease duration of 20 months. Eight out of ten had a family history of dementia and were codon 129VV. Cases had minimal spongiform change and minimal immunohistochemical stained PrP deposits with distinct patterns in the cortex and cerebellum. Western Blot (WB) also shows a minimal amount of PrPres present. Further studies by Gambetti have now identified codon 129MV and MM cases which have a

longer disease duration and exhibit some PK resistance. The cases did not have clinical profiles typical for sCJD. A UK case and a Dutch case have also been identified, with characteristics not inconsistent with the Gambetti studies.

16. Professor Knight added that due to the unique clinical presentation of the disease it was likely that at least some cases of disease would not be identified for referral, making it hard to obtain complete data on this disease. However, it was likely that a case would be identified as a prion disease at autopsy and the WB currently used would be able to identify the unique profile which categorises this disease. A retrospective review of the NCJDSU brain bank is underway to look for more cases.

17. A Member asked whether the recent review of neuropathology archives in the UK would have identified PSPr. Professor Knight responded that it would be dependent on the type of WB used at the time which is currently not known. The use of appropriate WB methodology would be an issue in accurately identifying the relevant characteristics.

18. One Member was not convinced by the characterisation of this disease, adding that clinical cases classified as Alzheimer’s Disease have shown similar laddering profiles in WB, protease resistant fragments and the presence of abnormal PrP. The disease has, to date, not been shown to be transmissible which means it should not yet be categorised a prion disease under the current terminology.

19. Summing up, the Chair noted that it was clear that more information was required to fully characterise and fill knowledge gaps regarding this disease. It was important that its unique pathology be more widely recognised to enable future diagnosis and enable tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise the disease further.

http://www.seac.gov.uk/minutes/draftminutes103.pdf

>>>12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case.<<<

>>>Two of the three U.S. cases, two of the four cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom. One of the 25 French cases may also have been infected in the United Kingdom.<<<

>>>There has never been a case of vCJD that did not have a history of exposure within a country where the cattle disease, BSE, was occurring.<<<

>>>vCJD Cases Reported in the US Three cases of vCJD have been reported from the United States. By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. There is strong evidence that suggests that two of the three cases were exposed to the BSE agent in the United Kingdom and that the third was exposed while living in Saudi Arabia.<<<

http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm

Heaven forbid any human mad cow disease coming from the U.S.A. ???

hmmm, i don't recall any madcows in Saudi Arabia ???

Eurosurveillance, Volume 11, Issue 49, 07 December 2006 Articles Editorial team1

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

Citation style for this article: Editorial team. Third case of vCJD reported in the United States. Euro Surveill. 2006;11(49):pii=3091. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3091 Date of submission:

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

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

Third case of vCJD reported in the United States

Editorial Team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office

A clinical diagnosis of variant Creutzfeldt Jakob Disease (vCJD) was confirmed after brain biopsy investigations in a United States (US) resident and reported in November [1]. The patient is a young man who grew up in Saudi Arabia and lived in the US since late 2005. Before that he visited the US once in 1989 and several times after 2001. He has never visited any country in Europe or received a blood transfusion nor has he undergone any neurosurgical procedure. This vCJD case is the third in a US resident. The previous two patients both grew up in the United Kingdom (UK), and this is where they were believed to have been infected [2].

In Saudi Arabia, the first and only previous case of vCJD was reported in 2005. This was suspected to be related to consumption of meat contaminated with the prion agent which causes bovine spongiform encephalitis in cattle (BSE). The European Food Safety Authority (http://www.efsa.org) has not published a geographical BSE risk assessment for Saudi Arabia [3] and there have been no cases of BSE in cattle reported by Saudi Arabia to the World Organisation for Animal Health (http://www.oie.int). Although the UK is not the only potential beef exporter to have had a BSE epidemic, it remains plausible, subject to Saudi Arabia's import policy, that contaminated beef was inadvertently imported from the UK to Saudi Arabia in the period before 1996 (when the EU banned the export of UK beef and cattle).

Based on this patient's history, the occurrence of a previously reported case of vCJD in Saudi Arabia, and the expected length of the incubation period for food-related vCJD, the most likely source of infection is thought to be contaminated meat products the patient consumed as a child when living in Saudi Arabia. The patient has no known history of donating blood, and investigations have identified no risk of onwards transmission within the US.

Variant Creutzfeldt-Jakob disease was first identified in the United Kingdom in the mid-1990s. As of November 2006, worldwide there have been 200 vCJD cases: 164 patients in the United Kingdom, 21 in France, four in Ireland, three in the US (including the present case), two in the Netherlands and one each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain [4]. All patients, except 10 (including the present case) had lived either in the United Kingdom (170 cases) or in France (20 cases). Evidence so far indicates that the most probable source of infection in most cases was consumption of meat products contaminated with the prion agent causing BSE.

References: 1.Centers for Disease Control and Prevention. Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East. (http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm) 2.Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354. 3.European Food Safety Authority . Geographical BSE Risk (GBR) assessments covering 2000-2006. List of countries and their GBR level of risk as assessed by the Scientific Steering Committee and the (EFSA). 1 August 2006. (http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/summary_list_countries.Par.0001.File.dat/GBR_assessments_table_Overview_assessed_countries_2002-2006.pdf) 4.Variant Creuzfeldt-Jakob disease. Current data – December 2006. (http://www.cjd.ed.ac.uk/vcjdworld.htm)

http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3091

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.

http://www.isid.org/14th_icid/

http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf

http://www.isid.org/publications/ICID_Archive.shtml

From: xxxx
To: Terry Singeltary
Sent: Saturday, December 05, 2009 9:09 AM
Subject: 14th ICID - abstract accepted for 'International Scientific Exchange'

Your preliminary abstract number: 670

Dear Mr. Singeltary,

On behalf of the Scientific Committee, I am pleased to inform you that your abstract

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

WAS accepted for inclusion in the INTERNATIONAL SCIENTIFIC EXCHANGE (ISE) section of the 14th International Congress on Infectious Diseases. Accordingly, your abstract will be included in the "Intl. Scientific Exchange abstract CD-rom" of the Congress which will be distributed to all participants.

Abstracts accepted for INTERNATIONAL SCIENTIFIC EXCHANGE are NOT PRESENTED in the oral OR poster sessions.

Your abstract below was accepted for: INTERNATIONAL SCIENTIFIC EXCHANGE

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Author: T. Singeltary; Bacliff, TX/US

Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange

This abstract has been ACCEPTED.

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Authors: T. Singeltary; Bacliff, TX/US

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

Body: Background

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

Methods

12 years independent research of available data

Results

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

Conclusion

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

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

Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion

http://www.isid.org/14th_icid/

http://www.isid.org/publications/ICID_Archive.shtml

http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

snip...

I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;

.....snip

''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete.

Thanks for your interest.''

Best regards,

Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA

END...TSS

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html

for those interested, please see full text ;

Friday, January 29, 2010 14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)

http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html

Monday, February 01, 2010

Import Alert 17-04 BSE CJD HIGH RISK TISSUES, Nutritional Supplements and Cosmetics

http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-17-04-bse-cjd-high-risk.html

Monday, February 01, 2010

Import Alert 57-20 and 84-03 Human Dura Mater and risk factors there from due to Creutzfeldt Jakob Disease (CJD)

http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-57-20-and-84-03-human-dura.html

Wednesday, February 3, 2010

Import Alert 62-07 Sygen Injectable (Bovine-Extracted GMI Monosialoganglioside) manufactured from bovine brain starting material

http://bseusa.blogspot.com/2010/02/import-alert-62-07-sygen-injectable.html

Wednesday, February 3, 2010

Import Alert 71-02 Detention Without Physical Examination Of Animal Feeds And Feed Ingredients That May Contain Ingredients Of Animal Origin Import Alert 71-02

http://madcowfeed.blogspot.com/2010/02/import-alert-71-02-detention-without.html

Wednesday, February 3, 2010

Import Alert 99-25 Detention Without Physical Examination of Animal Feed...BSE...and Not the Subject of a Valid USDA Import Permit Import Alert 99-25

http://madcowfeed.blogspot.com/2010/02/import-alert-99-25-detention-without.html

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

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

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed

USA sporadic CJD cases rising ;

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

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

CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

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

Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html

my comments to PLosone here ;

http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd

CJD RISING SWITZERLAND

CJD is a predominantly sporadic disorder but can also occur as a dominantly inherited or infective condition. Only one of the 26 most recent confirmed cases was identified as carrying a disease related mutation of the PRNP gene, none had identifiable iatrogenic exposure, and none resembled variant CJD. Thus 25 of the 26 cases appear to be sporadic cases. Sporadic CJD is distributed worldwide with a reported incidence of about one in a million per year. Raised awareness of the disease in recent years could account for an increase in reported cases of CJD, although neither an increase in the average age of patients nor more frequent recognition of CJD amongst residents of nursing homes (where dementing illness is prevalent and misdiagnosis might be expected) were seen in the Swiss cases. Moreover, improved ascertainment as an explanation for the observed increase would imply levels of under-reporting in countries other than Switzerland, which appear implausible. The authors of the Lancet report suggest that the rise in cases might be due to some form of unidentified iatrogenic transmission or to exposure to a zoonotic source of infection, though cases do not resemble variant Creutzfeldt-Jakob disease (vCJD). The ongoing surveillance of CJD in Switzerland and the rest of Europe is essential to monitor the situation to see if this rise is sustained in Switzerland, and if a similar rise occurs in other countries (see http://www.eurocjd.ed.ac.uk).

http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1921

Prion data suggest BSE link to sporadic CJD Declan Butler

Predicting the number of cases of Creutzfeldt–Jakob disease (CJD) in people as a result of transmission of bovine spongiform encephalopathy (BSE) has just got more difficult.Whereas it was thought that BSE only caused a new form of the disease called variant CJD (vCJD), a study in mice from a team led by John Collinge at University College London suggests that it may also cause a disease indistinguishable from the commonest form of classical, or 'sporadic', CJD (E.

http://www.nature.com/nature/journal/v420/n6915/full/420450a.html

IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ;

However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52).

IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76

http://www.eurocjd.ed.ac.uk/sporadic.htm

Switzerland sporadic CJD ;

Swiss rise in CJD raises concerns over possible BSE link [LONDON] THE LANCET

Plaque attack: Swiss patients have spongiform patterns in the brain typical of sporadic CJD. The number of people dying from Creutzfeldt-Jakob disease (CJD) has risen sharply in Switzerland -- sparking fears of a possible link with bovine spongiform encephalopathy (BSE).

BSE is thought to be the cause of a distinctive form of the brain-wasting disease known as variant CJD. The Swiss cases, in contrast, are standard 'sporadic' CJD. Each year between 1997 and 2000, no more than 11 Swiss people developed CJD. But 19 cases were reported in 2001, and seven were recorded in the first quarter of this year. This is some four times higher than the incidence elsewhere, reports a team led by Adriano Aguzzi of the University Hospital Zurich (M. Glatzel et al. Lancet 360, 139-141; 2002).

The increase could be a mere statistical blip, or it may be due to increased awareness of the disease leading to more diagnoses. More disturbing is the possibility that the cases are linked to the consumption of BSE-infected meat products -- which would mean that the BSE agent can cause two distinct forms of CJD.

Possible links between the Swiss CJD cases and BSE will now be explored by strain-typing experiments in which the disease is transmitted to mice. These tests will take at least a year to complete. "It's the best way to establish or exclude any suspected link," says Moira Bruce of the UK Institute for Animal Health's Neuropathogenesis Unit in Edinburgh.

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

Experiences in England and Switzerland -- two countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since mad cow was first detected in British herds in 1986.

Switzerland discovered last year its CJD rate was twice that of any other country in the world. Switzerland had been seeing about eight to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.

http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r

Mouse model sheds new light on human prion disease

snip...

Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.

snip...

http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm

Monday, May 19, 2008

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

http://bseinquiry.blogspot.com/

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

Saturday, December 12, 2009

103RD MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

http://seac992007.blogspot.com/2009/12/103rd-meeting-of-spongiform.html

Thursday, January 31, 2008

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION

40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base

13 © SEAC 2007

cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”

41. A member considered that this question ............

http://www.seac.gov.uk/minutes/99.pdf

http://seac992007.blogspot.com/2008/01/spongiform-encephalopathy-advisory.html

Wednesday, November 18, 2009

R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission

http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.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 see video

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

Sunday, January 17, 2010

BSE USA feed inspection violations 01/01/2009 to 01/17/2010 FDA BSE/Ruminant Feed Inspections Firms Inventory Report

http://madcowfeed.blogspot.com/2010/01/bse-usa-feed-inspection-violations.html

Tuesday, January 19, 2010

CVM's OR Develops New PCR-Based Method for Testing Animal Feed

http://madcowfeed.blogspot.com/2010/01/cvms-or-develops-new-pcr-based-method.html

Thursday, January 07, 2010

Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 and FISCAL YEAR 2008

http://scrapie-usa.blogspot.com/2010/01/scrapie-and-nor-98-scrapie-november.html

Monday, December 14, 2009

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

http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html

TSS

103RD MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

2009-12-12T20:06:00.000-08:00

103RD MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 103rd Meeting in London on 24th November 2009, and discussed the following:

CURRENT ISSUES

SEAC was informed about:

• Confirmation of BSE in a Scottish goat originally diagnosed with scrapie in 1990; the goat culled in 1990 was born prior to the introduction of the ruminant feed ban and could have been exposed to contaminated feed.

• The first case of atypical scrapie discovered in a sheep from New Zealand.

• A recommendation to Health Ministers by the Advisory Committee on the Safety of Blood Tissues and Organs that prion filtration should be introduced for blood used to treat patients who were not exposed to BSE through their diet.

• Recently published research1 on the discovery in Papua New Guinea of a novel protective prion protein variant.

CJD UPDATE

SEAC was updated on the number of clinical cases of variant Creutzfeldt-Jakob Disease (vCJD) and sporadic CJD (sCJD). To date there have been 170 definite or probable UK cases of clinical vCJD. 167 of these are associated with probable dietary exposure to BSE and three arose after transfusions of blood from donors who later developed vCJD. The mean age of death was 30 years. There have been no cases of vCJD in individuals born after 1989.

1 A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure by S Mead, J Whitfield, M Poulter, P Shah, J Uphill, T Campbell, H Al-Dujaily, H Hummerich, J Beck, C A Mein, C Verzilli, J Whittaker, M P Alpers & J Collinge. New Eng J Med., 2009, 361, 2056-65.

2 © SEAC 2009

Elsewhere in the world, 47 vCJD cases have been reported: 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan.

A brief report was provided on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). Eleven cases have been reported with a mean age of onset of 62 years and disease duration of 20 months. The committee thought it was important that the unique pathology of this disease be more widely recognised to enable future diagnosis and tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise this disease further.

EFFECT OF AGE ON THE PATHOGENESIS OF TSEs Most clinical cases of variant CJD have occurred in young adults, the median age at onset of disease being 26 years and the median age at death 28 years (the comparable ages for sporadic CJD are both 67). The reasons behind this apparent age-related susceptibility are uncertain. A recent paper2 reports the findings of a study in mice, which suggest that the age related decline in the functioning of follicular dendritic cells might impair TSE pathogenesis. The Committee agreed that a competent immune system was required for efficient replication of TSEs in the host. It was noted that older people are more immuno-compromised than the young, but there is very little data on the effect of ageing on the human immune system. The Committee concluded that there are insufficient data to suggest that this might provide an explanation for the young age of vCJD patients.

UP-DATE ON vCJD PREVALENCE STUDIES

The committee received an update on progress with the vCJD prevalence studies being conducted by the Health Protection Agency. This covered the National Anonymous Tonsil Archive (NATA), the post mortem archive and a new study of appendices. To date, NATA has tested approximately 80,000 pairs of tonsils and none were positive. A pilot for a study of spleens obtained

2 The effects of host age on follicular dendritic cell status dramatically impair scrapie agent neuroinvasion in aged mice. K L Brown, G J Wathne, J Sales, M E Bruce, and N A Mabbott, the Journal of Immunology 2009, doi:10.4049/jimmunol.0802695. 3 © SEAC 2009

from post mortems, that will assess a number of potential methodologies, will start in early 2010, and report on the efficacy of these methodologies in June 2010. The new study of 30,000 appendices, to be tested by immunohistochemistry, will also start in early 2010. The Committee noted progress, and commented that in order to have sufficient power, the spleen study would have to test approximately 50,000 samples.

CATEGORY 3 ANIMAL PRODUCTS IN FERTILISERS

Building on previous work in 2005, Defra has commissioned a full risk assessment, which SEAC was invited to consider, to evaluate the amount of potential infectivity available in the soil of nonpasture land following the application of Category 3-derived3 fertiliser.

The committee considered that the methodology used in the risk assessment was scientifically valid, but felt that too many worst case assumptions had been made in addressing the paucity of experimental data, that certain temporal aspects could have been addressed differently and that the possibility of regulatory failure had not been sufficiently considered.

FUTURE OPERATION OF SEAC

The Committee discussed the future organisation of SEAC business and the desirability of streamlining the decision making process, so that Ministers could obtain advice more quickly. In particular, SEAC considered a proposal that in future much of its business could be dealt with by correspondence, rather than in face-to-face meetings.

In discussion the committee recognised that some SEAC business could be carried out more efficiently by e-mail correspondence. The process would need to be structured, perhaps at fixed times of the year and the discussion would be summarised and recorded on the SEAC Website so that the decision-making process was transparent and available for public scrutiny. However, it was important that the committee still aimed to meet in person twice or three times per year.

3 Category 3 is low risk material, most of which is fit for human consumption, but not intended for human consumption.

3 © SEAC 2009

http://www.seac.gov.uk/pdf/seac103_summary.pdf

RE--Protease-Sensitive Prionopathy (PSPr) SEE ;

Protease-Sensitive Prionopathy (PSPr)

http://www.mhlw.go.jp/shingi/2009/02/dl/s0210-8d_0030.pdf

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

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

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

see sporadic CJD, the big lie, POLICY IN CONFIDENCE; CONFIDENTIAL, coming full circle below ;

http://neuropathologyblog.blogspot.com/2008/07/new-prion-disease.html

http://www.wellsphere.com:86/cjd-article/a-novel-human-disease-with-abnormal-prion-protein-sensitive-to-protease-prionopathy/641206

Thursday, July 10, 2008

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

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.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 — at the average rate of one every six weeks...<<<

http://downercattle.blogspot.com/2009/09/suit-meatpacker-used-downer-cows-for-4.html

do you actually believe all these schools recalled this meat because of a few cattle being abused,

see list ;

FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program

March 24, 2008

School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008

http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf

I have tried to get these papers through the court, but no luck. they want me to pay to retrieve the papers, and i am not going to pay for something I know happened. about like the last two FOIA on suspect mad cow feed going into commerce in the USA in 2009. I knew it had, but wanted them to say it. and they finally did via the FOIA.

Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.

http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

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

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

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Thursday, November 05, 2009 9:25 PM Subject: [BSE-L] re-FOIA REQUEST ON FEED RECALL PRODUCT contaminated with prohibited material Recall # V-258-2009 and Recall # V-256-2009

http://madcowfeed.blogspot.com/2009/11/re-foia-request-on-feed-recall-product.html

PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

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

http://downercattle.blogspot.com/

please see full text here ;

Tuesday, November 17, 2009

SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2

http://downercattle.blogspot.com/2009/11/seac-effect-of-age-on-pathogenesis-of.html

Wednesday, November 18, 2009

R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission

http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.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 see video

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

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

http://madcowfeed.blogspot.com/2008/06/texas-firm-recalls-cattle-heads-that.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

Saturday, December 05, 2009

Molecular Model of Prion Transmission to Humans

http://creutzfeldt-jakob-disease.blogspot.com/2009/12/molecular-model-of-prion-transmission.html

Monday, November 23, 2009

BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.

http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html

Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html

Tuesday, August 18, 2009

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

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

Atypical BSE North America Update February 2009

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

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

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

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

http://seac992007.blogspot.com/

TSS

SaBTO Advisory Committee on the Safety of Blood, Tissues and Organs 2nd Public Meeting 27 October 2009

2009-10-24T15:12:00.000-07:00

SaBTO

Advisory Committee on the Safety of Blood, Tissues and Organs

2nd Public Meeting

“Blood Donation – Selection, Deferral and Exclusion”

27 October 2009

Royal Horticultural Halls, London SW1P

At their inaugural meeting in January 2008, members of SaBTO decided that a public meeting should be held every year, focusing on a particular issue within the committee’s remit. The 2009 Public Meeting will focus on the issue of donor selection. Selection of donors is key to the delivery of a safe blood supply. Many prospective donors may be turned down for a variety of reasons, including age, sexual history and medical concerns. As a scientific advisory committee, SaBTO is concerned that exclusion or deferral of prospective blood donors is done for justifiable reasons and in the best interests of blood recipients.

The format of the afternoon will include an introduction to SaBTO and donor selection, and an open forum with the opportunity for the audience to comment and ask questions.

The committee hopes that the audience will reflect a wide range of stakeholders and interest groups. Should the event be oversubscribed, there will be a limit of one delegate per organisation. If you are interested in attending. please e-mail SaBTO@dh.gsi.gov.uk or telephone 020 7972 4750 by 13 October 2009 (this deadline has been extended). Please note that this event is free of charge for all delegates.

The Advisory Committee on the Safety of Blood, Tissues, and Organs (SaBTO) is a Non-Departmental Public Body, with an independent Chair and members selected by the Appointments Commission for their specific areas of expertise. SaBTO provides independent advice to the UK Government and the Devolved Administrations on the most appropriate ways to ensure the safety of blood, cells, tissues, and organs for transfusion/transplantation. SaBTO also provides advice on the microbiological safety of gametes and stem cells, and considers both risk assessment and risk management options for Ministers and UK Health Departments to consider.

Further detailed information about SaBTO and its remit can be found at

http://www.dh.gov.uk/ab/SaBTO/index.htm

http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/digitalasset/dh_104789.pdf

see other SaBTO meetings minutes here ;

2009 Download summary of seventh meeting, 14-15 July 2009 (PDF, 65K)

Download minutes of sixth meeting, 28 April 2009 (PDF, 104K)

Download summary of sixth meeting, 28 April 2009 (PDF, 64K)

Download minutes of fifth meeting, 20 January 2009 (PDF, 102K)

Download agenda for the fifth meeting, 20 January 2009 (PDF, 15K)

2008

snip...end

http://www.dh.gov.uk/ab/SaBTO/DH_089412

Tuesday, November 11, 2008 SaBTO Summary of 1st Public Meeting – variant CJD and blood Tuesday 21st October 2008, 2pm-4pm

http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40.

The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

snip...

http://www.seac.gov.uk/minutes/99.pdf

http://seac992007.blogspot.com/2008/07/seac-draft-minutes-of-100th-meeting.html

http://seac992007.blogspot.com/

>>>There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.<<<

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip... see full text ;

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

Thursday, July 10, 2008

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

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008

A New Prionopathy update July 10, 2008

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

Communicated by: Terry S. Singeltary Sr. <:flounder9@verizon.net>

[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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

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

Friday, October 23, 2009

Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008

http://cjdtexas.blogspot.com/2009/10/creutzfeldt-jakob-disease-surveillance.html

O.2.2

vCJD infection in an asymptomatic UK haemophilic patient

Alexander Peden1, Graham Fairfoul1, Suzanne Lowrie1, Linda McCardle1, Mark Head1, Seth Love2, Hester Ward1, Simon Cousens3, David Keeling4, Carolyn Millar5, FGH Hill6, James Ironside1 1University of Edinburgh, UK; 2Frenchay Hospital, Bristol, UK; 3London School of Hygiene and Tropical Medicine, UK; 4Churchill Hospital, Oxford, UK; 5Imperial College London, UK; 6Birmingham Children’s Hospital, Birmingham, UK

We describe a study of 17 UK patients with haemophilia considered to be at increased risk of vCJD through exposure to UK plasma products. 10 autopsy cases and 7 biopsy cases were analysed for disease- associated, protease-resistant prion protein (PrPres). The tissues available from each case were variable, ranging from a single biopsy sample to a wide range of autopsy tissues. A single specimen from the spleen of one autopsy case gave a strong positive result on repeated testing for PrPres by Western blot analysis. This tissue came from a 73 year-old male with no history of neurological disease, who was heterozygous (methionine/valine) at codon 129 in the prion protein gene. He had received over 9,000 units of Factor VIII concentrate prepared from plasma pools known to include donations from a vCJD-infected donor, and some 400,000 units not known to include donations from vCJD-infected donors. He had also received 14 units of red blood cells and had undergone several surgical and invasive endoscopic procedures. Estimates of the relative risks of exposure though diet, surgery, endoscopy, blood transfusion and receipt of UK plasma products suggest that by far the most likely route of infection was receipt of UK plasma products.

O.2.3

Detection of prion particles in body fluids of humans and animals

Detlev Riesner Institut fur Physikalische Biologie, Heinrich-Heine- Universitat Dusseldorf, Germany

A major structural difference between the cellular isoform of the prion protein PrPC and the pathogenic isoform PrPSc is the much higher state of molecular aggregation of PrPSc. PK-resistance as normally used for PrPSc-characterization is not reliably valid for all species, strains and sources of prions. The method of suface-FIDA (fluorescence-intensity distribution analysis) was developed avoiding PK-digestion and detecting specifically disease related PrP-aggregates (Birkmann et al 2007, J. Vet. Microbiol 123, 294-304). After partial purification PrP-aggregates are bound to a chip covered with the captureantibody SAF32, labeled with two types of antibodies against two different epitopes and carrying two different fluorescent dyes. A dual-color laser beam is scanning the chip surface, and the fluorescence signals are evaluated in respect to local coincidence, intensities and particle size. The method was applied to brain homogenate of sCJD-victims (post mortem), to CSF-samples of BSE-afflicted cattle (ante mortem) and blood plasma of scrapie sheep (ante mortem). Particles of PrP-aggregates could be detected in all samples, exhibiting a diameter range of 300 nm (optical resolution) to 1ƒÊm. PrP-aggregates were detected with 100% sensitivity in the sCJD-brain homogenate samples; studies on CSF are ongoing. PrP-aggregates were detected in CSF of BSE-cattle, but the number of samples was too little to evaluate the sensitivity. Only one antibody was available to detect scrapie-PrP-aggregates from blood plasma; the sensitivity of 60% will be improved in the ongoing experiments with a second antibody. PrP-aggregates can be used as seeds for fibril formation with recombinant PrP as template (Stohr et al., 2008, PNAS 105, 2409-14). This system will be used as amplification of the particle detection method.

O.2.4

Detection of prions in blood leucocytes

Linda A. Terry, Laurence Howells, Jeremy Hawthorn, Sally Everest, Sarah Jo Moore, Jane C. Edwards Veterinary Laboratories Agency, UK

Background: Infected human blood has been implicated in the iatrogenic transmission of vCJD in four reported cases. Experimental transmission studies have demonstrated that blood from scrapie and BSE infected sheep also contains infectivity. Rodent models of prion disease implicated both cellular and plasma fractions. However, direct detection of PrPsc from blood in the absence of in vitro amplification or bioassay has proved difficult. Methods for the direct detection of PrPsc in blood would be advantageous for the study of the pathogenesis of TSEs and as a basis for a blood test. Objectives: To develop a method for the direct detection of PrPsc in blood cells from scrapie and BSE infected sheep; to study the temporal distribution of PrPsc in blood and to determine the identity of the cells bearing prions in blood. Methods: Peripheral blood mononuclear cells (PBMC) were isolated from sheep naturally infected with scrapie or experimentally infected with BSE at the clinical stage of disease and from scrapie infected sheep from 3 months of age through to clinical end-point. PBMCs were tested for PrPsc content by a direct immunoassay based on the IDEXX CWD HerdChek kit. Different subsets of PBMCs were isolated by subset specific cell surface markers and magnetic bead separation and analysed for PrPsc content. Results: PrPSc was detected in 54% of sheep with clinical scrapie and 71% of sheep with clinical BSE. A longitudinal study of the temporal distribution of blood PBMC associated PrPsc showed that the detection rate increases during the course of disease and is more likely to be observed during the second half of the incubation period. Additionally detection is more likely in scrapie infected sheep if they carry the PRNP genotype of VRQ/VRQ. Cell separation studies showed that the PrPsc is associated with a specific cell subset implicating a subset of B lymphocytes. Discussion. This is the first report of the direct detection of PrPsc in cells isolated from sheep blood in the absence of in vitro amplification or bioassay. Since PrPsc can be detected from as early as 3 months of age in sheep naturally infected with scrapie, correlating with initial replication in the gut-associated lymphoid tissue, the assay could be the basis of a preclinical test. The identification of the cell subset carrying PrPsc progresses our understanding of the pathogenesis of the disease. However, it remains unclear whether this cell subset is responsible for the dissemination of prions or in clearance of circulating PrPsc. Funded by defra, UK and IDEXX.

O.2.6

Human urine and PrP

Silvio Notari1*, Liuting Qing1*, Ayuna Dagdanova1*, Sergei Ilchenko1, Mark E. Obrenovich1, Wen-Quan Zou1, Maurizio Pocchiari2, Pierluigi Gambetti1, Qingzhong Kong1, Shu G. Chen1 1Case Western Reserve University, USA; 2Istituto Superiore di Sanità, Italy

Background: The presence and the characteristics of prion protein (PrP) in human urine under normal conditions are controversial. Similarly, there are no definite data on the presence of infectivity in urine in the course of naturally occurring human prion diseases. Objectives: 1) To definitely determine the presence and characteristics of PrPC in normal urine. 2) To evaluate the prion infectivity in human urine in sporadic Creutzfeldt-Jakob disease (sCJD), we have carried out a set of bioassays in humanized transgenic mouse with urine samples collected from sCJD subjects. Methods: 1) Advanced mass spectrometry and experimental treatments have been used to demonstrate the presence, primary structure and posttranslational modifications of purified urinary PrPC (uPrP). 2) Bioassays were performed by intracerebral inoculation of 100 times concentrated and dialyzed urine, collected from three sCJD-MM1 cases to humanized transgenic mice and from appropriate controls. Results: We found that human urine contains significant amount of PrP (approximately 10 ng/ml) that is truncated with the major N-terminus at residue 112 as the PrPC fragment identified as C1, and it carries an anchor, which is soluble because likely lacks the phosholipid component. None of the humanized transgenic mice inoculated with sCJD concentrated urine had evidence of prion disease during a period of over 700 days (their normal life expectancy) leading to the conclusion that prion infectivity in sCJD urine, if present, must be less than 6 infectious units/100ml. Discussion: The issues raised in the discussion will include: 1) The origin of the truncated uPrP; 2) How the present data compare with the experimental studies published to date that indicate presence of infectivity; 3) The practical implications of our findings. *

These authors contributed equally

O.4.6

All separated components, prepared from BSE-infected sheep blood, are infectious upon transfusion

Sandra McCutcheon1, Anthony Richard Alejo Blanco1, Christopher de Wolf1, Boon Chin Tan1, Nora Hunter1, Valerie Hornsey2, Christopher Prowse2, Marc Turner2, Martin H Groschup3, Dietmar Becher4, Fiona Houston5, Jean C Manson1 1The Roslin Institute and R (D) SVS, University of Edinburgh, UK; 2Scottish National Blood Transfusion Service, UK; 3FLIFederal Research Institute for Animal Health, Germany; 4Micromun, Germany; 5University of Glasgow, UK

Background: The possibility that vCJD may be transmitted by blood transfusion is serious public health issue, of which 4 probable (3 clinical) cases have been attributed. Recently a case of asymptomatic vCJD infection was identified in a haemophiliac; following treatment with clotting factors from UK plasma pools. Sheep orally infected with BSE provide a suitable model, to assess vCJD infection in humans & risk reduction methods, as the distribution of PrPSc & infectivity in lymphoid tissues resembles that of vCJD patients.

Objectives: To determine qualitative and quantitative data on the changes in infectivity in blood and its clinically relevant components with time, to assess the effect of leucodepletion of such products and the potential for secondary transmission by blood transfusion.

Methods: We orally infected sheep with bovine BSE brain homogenate and collected two full-sized donations of whole blood, before the onset of clinical signs. The following components were transfused into naive recipients: whole blood, buffy coat and leucoreduced and non leucoreduced plasma, platelets and red cells. A sub sample of all components was inoculated into TgShpXI mice for determination of infectivity titers. A unit of whole blood from selected primary recipients was transfused into secondary recipients. We are creating a blood archive throughout this study.

Results: 33% of the infected donors have been confirmed as having BSE. We have 4 transmissions of BSE-infectivity following the transfusion of whole blood, buffy coat and plasma. Short incubation times were recorded in these recipients (468, 513, 567 and 594 days) & were similar to those seen in their respective donors (534, 628, 614 and 614 days). The donor of buffy coat also donated both leucodepleted and non leucodepleted blood components to other recipients.

Discussion: Our study will provide invaluable data on the safety of blood products, in relation to TSE infection, used in human medicine (DoH 007/0162)

O.8.1

Variant CJD and plasma products

Robert G. Will National CJD Surveillance Unit, Edinburgh, UK

Evidence from the Transfusion Medicine Epidemiology Review (TMER) project indicates that variant CJD is transmissible through transfusion of labile blood components. The question as to whether plasma products sourced from vCJD contaminated plasma pools has been addressed by a number of risk assessments, with conflicting conclusions. Recently a case of possible vCJD infection in an individual with haemophilia has been described and analysis has suggested that infection may have been related to prior treatment with vCJD implicated Factor VIII. The details of this case will be described together with an analysis of plasma product exposures in UK clinical cases of vCJD.

O.8.2

Blood safety: from screening tests to prion removal

Marc Turner Scottish National Blood Transfusion Service and Department of Haematology, Royal Infirmary, Edinburgh, UK

Although the number of clinical cases of variant CJD continues to fall, concern remains within UK and Western European Blood Services in relation to the risk of transmission of variant CJD due to the estimated prevalence of sub-clinical infection in the general population and the clinical cases of transmission of variant CJD prions by blood components and plasma products. The UK Advisory Committee on the Safety of Blood, Tissues and Organs (SaBTO) has considered a number of further precautionary measures including reducing exposure to blood transfusion, importation of blood components, implementation of prion assays and prion reduction for red cell concentrates. The latter two technologies are currently under independent evaluation and it is expected that contingent on the outcome of these an initial decision on whether or not to recommend implementation of these technologies will be made by SaBTO in Autumn 2009.

O.9.3

Updated risk assessment of variant Creutzfeldt-Jakob disease (vCJD) risks for recipients of plasma-derived blood clotting products in the U.S.

Hong Yang, Richard Forshee, Mark Walderhaug, Steven Anderson US Food and Drug Administration, USA

Background: A recent announcement by UK health authorities of a case of vCJD infection in a >70 year old person with hemophilia has prompted the US Food & Drug Administration (FDA) to re-evaluate vCJD risks in the U.S. via plasma-derived Factor VIII (pdFVIII) and to update its 2006 risk assessment. As of May 2009, confirmed vCJD deaths have occurred in persons who are homozygous methionine (MM) at codon 129 of the PRP gene. Several reports in the last few years have indicated signs of vCJD infection in persons of methionine-valine (MV) and homozygous valine (VV) genotypes. FDA updated risk assessment by assuming all genotypes are susceptible to vCJD and modeling the incubation periods for all three genotypes.

Objectives: To evaluate the vCJD risk for pdFVIII recipients with severe hemophilia and vonWillebrand diseases.

Methods: The model assumed equal susceptibility of three genotypes, a median incubation period of 12 years for the MM and 32 years for MV and VV genotypes, and vCJD infectivity was present in the blood of infected donors during the last 50% to 90% of incubation period. Model used statistical distributions for inputs including susceptibility to the disease, donation rates, frequency and duration of travel to the UK, France and other countries in Europe since 1980, the effectiveness of donor deferral policies and infectivity clearance during manufacturing processes.

Results: For severe hemophilia patients at the highest risk (prophylaxis, with inhibitor, with immune tolerance) the model estimated annual mean exposure to be ~7 x 10-8 iv ID50 or ~1 in 270,000 with the lower prevalence (4 per million) assumption, and ~1 x 10-4 iv ID50 or ~1 in 12,000 with the higher prevalence (1 per 4,225) assumption. Donor deferral policies reduce the risk by >92%.

Discussion: Due to limited data and knowledge of vCJD, the model estimates are uncertain. However, it suggests the risk is small, and donor deferral and manufacturing processes greatly reduce the risk.

P.10.7

Serial passage of sCJD in humanised transgenic mice indicates two major transmission strains associated with PrPSc of either type 1 or 2

Matthew Bishop, Robert Will, Enrico Cancellotti, Jean Manson University of Edinburgh, UK

Background: Questions remain about the aetiology of sporadic CJD and whether phenotypic variation is solely controlled by factors such as codon 129 genotype and biochemistry of PrPC. Variation in infective strain has not been clearly demonstrated in sCJD.

Objectives: By serial passage of sCJD in transgenic mice expressing human prion protein with MM, MV, and VV codon 129 genotypes we aimed to understand strain transmission characteristics for the three most commonly observed phenotypes of sCJD.

Methods: We performed intracerebral inoculation of humanised transgenic mice with brain homogenates derived from similar mice previously inoculated with frontal cortex from sCJD patients of subgroups MM1, MV2, and VV2. These mice were assessed for clinical TSE signs, for TSE vacuolation, and deposition of PrPSc.

Results: sCJD(MM1) passage via all mice showed transmission profiles similar to primary inoculation. sCJD(MV2) passage via HuMM and HuVV mice showed a transmission profile similar to primary inoculation. Passage via a HuMV mouse showed transmission properties similar to not only the primary inoculum but also sCJD(MM1). sCJD(VV2) passage via HuMV and HuVV mice showed transmission profiles similar to the primary inoculation. Passage via a HuMM mouse showed transmission properties similar to not only the sCJD(VV2) primary inoculum but also sCJD(MM1). Cluster analysis of the lesion profile data showed that three clusters seen after primary inoculation were reduced to two following second passage, identified by the biochemical type of PrPSc (1 or 2) found in the host mice.

Discussion: Serial passage of sCJD subgroups MM1, MV2, and VV2 shows that PrPSc type and mouse codon 129 genotype determine the secondary transmission profile, independently of the originating inoculum strain. There are associations between type 1 PrPSc and C129-Met, and type 2 PrPSc and C129-Val. This should allow us to investigate further the relationship between PrPSc, genotype, infection, and pathology.

P.5.1

Detection of cellular prion protein (PrPc) in plasma from healthy cynomolgus monkeys (Macaca fascicularis) and changes observed after BSE infection

Barbara Yutzy, Edgar Holznagel, Johannes Löwer Paul-Ehrlich-Institut, Germany

Background: Orally BSE-dosed cynomolgus monkeys represent a valuable model to examine the kinetic of blood infectivity and to assess the risk of blood-borne transmission of variant Creutzfeldt-Jacob disease (vCJD).

Methods: Blood samples were collected monthly from BSE-infected (n = 18) and non-infected female cynomolgus monkeys (n = 8) over a period of up to 9 years. PrPc concentrations were retrospectively analyzed in plasma samples by a dot blot assay and by a sandwich ELISA using a highly sensitive dissociation- enhanced lanthanide fluoro-immunoassay (DELFIA) for detection. Different blood preparation protocols were evaluated to obtain plasma.

Objective: To detect changes in the levels of soluble plasmaderived PrPc. Results: Different blood preparation protocols had a significant effect on the measured plasma PrPc concentrations. In non-infected macaques, concentrations of soluble, plasmaderived PrPc were at least 10-fold lower compared to plasma concentrations in healthy humans. Levels of plasma PrPc increased 6 – 12 months after experimental BSE infection, remained high during the asymptomatic phase, and dropped towards the clinical phase. Soluble, plasma-derived PrPc molecules were PK-sensitive in BSE-infected macaques.

Discussion: There is a species-specific difference in the PrPc concentrations between human and macaque. At least a part of the plasma-derived PrPc fraction originates from blood cells. Andfinally, BSE infection caused an increase in plasma PrPc levels during the asymptomatic phase of infection. Blood transfusion studies have been initiated to examine whether these PK-sensitive PrP molecules carry infectivity.

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.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

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

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

Friday, October 23, 2009

Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008

http://cjdtexas.blogspot.com/2009/10/creutzfeldt-jakob-disease-surveillance.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/

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 see video

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

Saturday, August 22, 2009

FREE Kim Min-sun, she is correct about mad cow fears from USDA BEEF

http://usdavskorea.blogspot.com/2009/08/free-kim-min-sun-she-is-correct-about.html

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

THIS recall is not confusing ;

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

Thursday, March 19, 2009

MILLIONS AND MILLIONS OF POUNDS OF MAD COW FEED IN COMMERCE USA WITH ONGOING 12 YEARS OF DENIAL

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

Sunday, October 18, 2009

Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, October 17, 2009

http://madcowfeed.blogspot.com/2009/10/wisconsin-firm-recalls-beef-tongues.html

Thursday, October 15, 2009

Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, Oct 15, 2009

http://madcowfeed.blogspot.com/2009/10/nebraska-firm-recalls-beef-tongues-that.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

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

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

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE SEAC giving up, giving in, or just dreaming ???

2009-03-02T09:03:00.000-08:00

SEAC

The 2008 review (PDF 100KB)

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

Light-touch review Summary and recommendations

1. The Spongiform Encephalopathy Advisory Committee (SEAC) has played an outstanding role in evaluating the scientific evidence relating to Transmissible Spongiform Encephalopathies (TSEs), assessing the risk they represent for human health and responding to the concerns of the public.

2. The Committee works well and is well supported by a highly efficient and effective secretariat. While there are several changes of detail that could be made to improve current arrangements, there is a clear consensus that they are broadly about right.

3. As required in any Cabinet Office review, the options of winding up the Committee and of providing its functions in a different way have been investigated. No sensible alternative arrangements have been identified.

4. That said, it is now clear that the risks from TSEs are low, although further work is needed in some areas. Most who gave evidence to this review could see a time in the not far distant future when SEAC would no longer be needed.

5. Better relations with European institutions are needed, especially with the European Food Safety Authority (EFSA); and greater clarity about the roles and accountabilities of the different bodies is needed.

6. It is heartening to see that virtually all the recommendations from the previous review have been implemented. The Committee has made a strong commitment to openness and has followed it through with rigour. It is essential

snip... see full text ;

http://www.seac.gov.uk/pdf/review-2008.pdf

The response to the 2008 review (PDF 21KB)

http://www.seac.gov.uk/pdf/review-2008-response.pdf

The “wiring diagram” described in the 2008 response (PDF 100KB)

http://www.seac.gov.uk/pdf/wiring-diagrams080623.pdf

> 4. That said, it is now clear that the risks from TSEs are low, although further work is needed in some areas. Most

> who gave evidence to this review could see a time in the not far distant future when SEAC would no longer be

> needed.

Greetings,

I strongly disagree !

I would like to know by each scientist and or politician that made such a crude decision, what scientific rational they based this crude decision on ???

to me, it's like your in a 9 inning baseball game, in the 3rd inning tied up, and someone calls the game.

TWO plus decades into the BSE crisis, and we are still in the prehistoric realms of science in terms of Transmissible Spongiform Encephalopathies, if you look at the overall picture i.e. all species and the different TSEs, sources, routes, environmental risk factors, AND YES, I still believe the United States of America is covering up cases of Transmissible Spongiform Encephalopathy in the USA bovine, i.e. FOR PROFIT $$$

With the ever so emerging infectious TSE, in many species of the wild, game, and livestock, to humans, and espeically international trading of the TSE's due to the OIE and the USDA don't look, don't speak, don't tell policy, I strongly urge SEAC reconsider. It is of my opinion that SEAC's work has just begun, you cannot quit in the 3rd inning of a 9 inning game, one of which may have extra innings. ...

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 7751

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

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html

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

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

Saturday, January 24, 2009 Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html

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

http://madcowtesting.blogspot.com/2009/02/tafs1-position-paper-on-testing-of.html

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

(January 2009)

TAFS1 Position Paper on Specified Risk Materials

http://madcowspontaneousnot.blogspot.com/2009/01/tafs1-position-paper-on-specified-risk.html

November 25, 2008

Update On Feed Enforcement Activities To Limit The Spread Of BSE

http://madcowfeed.blogspot.com/2008/11/november-2008-update-on-feed.html

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 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

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

full text ;

http://madcowspontaneousnot.blogspot.com/2008/02/specified-risk-materials-srm.html

http://madcowspontaneousnot.blogspot.com/

Thursday, February 26, 2009

'Harmless' prion protein linked to Alzheimer's disease Non-infectious form of prion protein could cause brain degeneration

http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html

Sunday, February 15, 2009

Scientists warn of first ever case of human mad cow disease from blood plasma

http://vcjdtransfusion.blogspot.com/2009/02/scientists-warn-of-first-ever-case-of.html

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

When Atypical Scrapie cross species barriers

Authors

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

Content

Atypical scrapie is a TSE occurring in small ruminants and harbouring peculiar clinical, epidemiological and biochemical properties. Currently this form of disease is identified in a large number of countries. In this study we report the transmission of an atypical scrapie isolate through different species barriers as modeled by transgenic mice (Tg) expressing different species PRP sequence.

The donor isolate was collected in 1995 in a French commercial sheep flock. inoculation into AHQ/AHQ sheep induced a disease which had all neuro-pathological and biochemical characteristics of atypical scrapie. Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate retained all the described characteristics of atypical scrapie.

Surprisingly the TSE agent characteristics were dramatically different v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and biochemical characteristics similar to those of atypical BSE L in the same mouse model. Moreover, whereas no other TSE agent than BSE were shown to transmit into Tg porcine mice, atypical scrapie was able to develop into this model, albeit with low attack rate on first passage.

Furthermore, after adaptation in the porcine mouse model this prion showed similar biological and biochemical characteristics than BSE adapted to this porcine mouse model. Altogether these data indicate.

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

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

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

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

SCRAPIE USA

INFECTED AND SOURCE FLOCKS

There were 20 scrapie infected and source flocks with open statuses (Figure 3) as of April, 30, 2008. Twenty eight new infected and source flocks have been designated in FY 2008 (Figure 4); three source flocks were reported in April. ...snip

POSITIVE SCRAPIE CASES

As of April 30, 2008, 122 new scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL) in FY 2008 (Figure 6). Of these, 103 were field cases and 19* were Regulatory Scrapie Slaughter Surveillance (RSSS) cases (collected in FY 2008 and reported by May 20, 2008). Positive cases reported for April 2008 are depicted in Figure 7. Eighteen cases of scrapie in goats have been confirmed by NVSL since implementation of the regulatory changes in FY 2002 (Figure 8). The most recent positive goat case was confirmed in February 2008 and originated from the same herd in Michigan as the other FY 2008 goat cases. ...snip

CAPRINE SCRAPIE PREVALENCE STUDY (CSPS)

snip...

However, four positive goats have been identified this fiscal year through field investigations. One was a clinical suspect submitted for testing and the other three originated from the birth herd of the clinical case.

ANIMALS SAMPLED FOR SCRAPIE TESTING

As of April 30, 2008, 26,703 animals have been sampled for scrapie testing: 23,378 RSSS, 1,517 goats for the CSPS study, 1,466 regulatory field cases, 270 regulatory third eyelid biopsies, and 72 regulatory rectal biopsies (chart 8).

TESTING OF LYMPHOID TISSUE OBTAINED BY RECTAL BIOPSY WAS APPROVED BY USDA AS AN OFFICIAL LIVE-ANIMAL TEST ON JANUARY 11, 2008. ...

PLEASE NOTE, (FIGURE 6), Scrapie Confirmed Cases in FY 2008 MAP, PA 3, 1**, Two cases-state of ID UNKNOWN, 1 case Nor98-like**

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

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

P03.141

Aspects of the Cerebellar Neuropathology in Nor98

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

Norway Nor98 is a prion disease of old sheep and goats. This atypical form of scrapie was first described in Norway in 1998. Several features of Nor98 were shown to be different from classical scrapie including the distribution of disease associated prion protein (PrPd) accumulation in the brain. The cerebellum is generally the most affected brain area in Nor98. The study here presented aimed at adding information on the neuropathology in the cerebellum of Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A panel of histochemical and immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers for phagocytic cells were conducted. The type of histological lesions and tissue reactions were evaluated. The types of PrPd deposition were characterized. The cerebellar cortex was regularly affected, even though there was a variation in the severity of the lesions from case to case. Neuropil vacuolation was more marked in the molecular layer, but affected also the granular cell layer. There was a loss of granule cells. Punctate deposition of PrPd was characteristic. It was morphologically and in distribution identical with that of synaptophysin, suggesting that PrPd accumulates in the synaptic structures. PrPd was also observed in the granule cell layer and in the white matter. The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

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

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

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.

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

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

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007

http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html

Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

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

Monday, December 1, 2008 When Atypical Scrapie cross species barriers

http://nor-98.blogspot.com/2008/12/when-atypical-scrapie-cross-species.html

Wednesday, January 28, 2009 TAFS1 Position Paper on BSE in small ruminants (January 2009)

http://scrapie-usa.blogspot.com/2009/01/tafs1-position-paper-on-bse-in-small.html

Monday, September 1, 2008 RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1] September 1, 2008

http://foiamadsheepmadrivervalley.blogspot.com/2008/09/re-foia-of-declaration-of-extraordinary.html

OIE amending the Annex to Decision 2007/453/EC establishing the BSE status of Member States or third countries or regions thereof according to their BSE risk

http://docket-aphis-2006-0041.blogspot.com/2009/01/oie-amending-annex-to-decision.html

Thursday, January 31, 2008

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION

40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base

13 © SEAC 2007

cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”

41. A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA. There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human prion diseases. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important that to keep a watching brief on new developments on TSEs.

snip...

snip...end...full text ;

http://www.seac.gov.uk/minutes/99.pdf

SEAC stated ;

There is no evidence that sCJD is increasing in the USA

i respectfully beg to differ. from 26 documented sCJD cases in 1996 rising to 157 documented sCJD cases in 2004, with 152 cases in 2005, to 143 in 2006, this would seem to be a rise in documented sporadic CJD cases from 1996 to me. ...TSS

CJD Cases examined ---------------------- Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD

1996 / 42 / 32 / 26 / 4 / 0 / 0 1997 / 115 / 68 / 57 / 9 / 0 / 0 1998 / 93 / 53 / 45 / 7 / 1 / 0 1999 / 114 / 69 / 61 / 8 / 0 / 0 2000 / 151 / 103 / 89 / 14 / 0 / 0 2001 / 208 / 116 / 106 / 9 / 0 / 0 2002 / 255 / 143 / 118 / 23 / 2 / 0 2003 / 272 / 174 / 132 / 41 / 0 / 0 2004 / 334 / 183 / 157 / 21 / 0 / 1* 2005 / 352 / 195 / 152 / 37 / 1 / 0 2006 / 372 / 186 / 143 / 30 / 0 / 1** 2007 / 120 / 68 / 35 / 7 / 0 / 0

A ProMED-mail post

snip...

[2] USA: National Prion Disease Pathology Surveillance Center Date: June 2007 Source: National Prion Disease Pathology Surveillance Center (USA) [edited]

CJD Cases examined ---------------------- Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD

1996 / 42 / 32 / 26 / 4 / 0 / 0 1997 / 115 / 68 / 57 / 9 / 0 / 0 1998 / 93 / 53 / 45 / 7 / 1 / 0 1999 / 114 / 69 / 61 / 8 / 0 / 0 2000 / 151 / 103 / 89 / 14 / 0 / 0 2001 / 208 / 116 / 106 / 9 / 0 / 0 2002 / 255 / 143 / 118 / 23 / 2 / 0 2003 / 272 / 174 / 132 / 41 / 0 / 0 2004 / 334 / 183 / 157 / 21 / 0 / 1* 2005 / 352 / 195 / 152 / 37 / 1 / 0 2006 / 372 / 186 / 143 / 30 / 0 / 1** 2007 / 120 / 68 / 35 / 7 / 0 / 0 TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2

*Acquired in UK ** Acquired in Saudi Arabia *** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007. **** 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).

Notes:

-- Cases are listed based on the year of death when available. If the year of death is not available, the year of sample receipt is used.

-- Referrals: Cases with possible or probable prion disease from which brain tissue or blood in the case of familial disease were submitted.

-- Inconclusive: Cases in which the samples were not sufficient to make a diagnosis.

-- Non-vCJD type unknown are cases in which the tissue submitted was adequate to establish the presence but not the type; in all cases, vCJD could be excluded.

-- 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]

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

full text ;

http://seac992007.blogspot.com/

Saturday, February 14, 2009 10:18 AM

A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004

http://creutzfeldt-jakob-disease.blogspot.com/2009/02/case-control-study-of-sporadic.html

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

SEAC 102nd Meeting on Wednesday 4 March 2009

2009-02-26T18:29:00.000-08:00

SEAC

Agenda

102nd Meeting on Wednesday 4 March 2009

Room 808, Nobel House, 17 Smith Square, Defra, London SW1P 3JR

10.05 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...

21. A member asked if it was possible to translate the estimates of infectivity into a number of potential additional cases of vCJD. Dr Comer explained that such estimates would rely upon a number of assumptions where there would be large uncertainties, producing results with very large uncertainties. Human exposure expressed as bovine oral ID50 may be more easily understood, aiding decision making. A member noted that the possibility of human-to-human transmission made estimating the potential number of vCJD cases for a given level of dietary exposure to BSE more complex. Members agreed that it was important to communicate to consumers the reasons why such estimates are so difficult to make.

snip...

24. Dr Victor Del Rio Vilas (VLA) presented an overview of the study conducted between July 2005 and November 2007. The aim was to characterise cases of atypical scrapie, particularly with regard to their holdings of origin and to examine any association between variables related to the holdings and the occurrence of atypical scrapie. Forty case holdings and 119 control holdings were analysed. Data were collected from interviews with farmers with a questionnaire, producing 260 analytical variables. A statistical analysis of the data collected showed that a small number of variables, including certain animal feeds, were associated with an increase in the odds of atypical scrapie being present. Some variables appeared protective against atypical scrapie.

snip...

see full text ;

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

10.50

New results on idiopathic brainstem neuronal chromatolysis Dr M Jeffrey (VLA)

SEAC 102/2

NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS FROM THE VETERINARY LABORATORIES AGENCY

ISSUE

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.

BACKGROUND

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.

NEW RESULTS

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.

FUTURE RESEARCH

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.

ADVICE SOUGHT

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?

SEAC SECRETARIAT

FEBRUARY 2009

ANNEX A

A copy of the paper “Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?”

ANNEX B

FROM MINUTES OF 14TH SEAC MEETING – 22 APRIL 1993

12. The Committee emphasised the importance of defining this new condition in detail with transmission studies and PrP examination (3 had already been examined for PrP, all negative). The total number of cases was now 50 with still only one in England.

FROM MINUTES OF 18TH SEAC MEETING – 10 FEBRUARY 1995

16. A Member told the Committee that no infectivity (by bioassay in mice) nor PrP had been found in the brains of idiopathic brainstem chromatolysis and hippocampal sclerosis cases. It is thought that the condition might be caused by a dietary deficiency, or some other metabolic disease.

FROM MINUTES OF 19TH SEAC MEETING – 21 JUNE 1995

29. A Member 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 characterised 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. The Chair 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. A Member 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.

FROM MINUTES OF 42ND SEAC MEETING – 23 MAY 1997

62. The Committee were advised that the paper had been circulated for information, and that no further action was proposed until further results were available unless the Committee felt otherwise. The Committee noted the paper. FROM MINUTES OF 49TH SEAC MEETING – 9 MARCH 1998

52. The Committee had expressed concern last year that IBNC could be a transmissible disease. Mouse assays from cases had been undertaken and SEAC 49/8 was an update on information given to the Committee last year. The positive results obtained from the earlier transmission experiments were now thought probably to have been due to BSE strain 301V contamination in the laboratory. Consequently no firm conclusion could be drawn from them on whether IBNC is transmissible. The latest transmission study, had been running for between 577 and 631 days with no evidence of transmission to date. The Committee were informed that the IBNC cases had tested negative by immunohistochemistry. The Committee were content that, although little was known about IBNC, it did not constitute a health risk to man. Suspect IBNC cases would be taken as BSE suspects or caught by the Over Thirty Months Scheme.

http://www.seac.gov.uk/papers/102-2.pdf

12.00 DH risk assessment on sourcing and pooling plasma Dr S Dobra (DH)

SEAC 102/3 (PDF)

Annex (PDF)

SEAC 102/3

SEAC

Spongiform Encephalopathy Advisory Committee

Comparing the relative risk of vCJD transmission via single unit and pooled plasma from UK and non-UK sources. ISSUE

1. The Department of Health (DH) has asked SEAC to provide advice on a risk assessment methodology, for assessing the risks of using single unit plasma with respect to pooled plasma, from the UK or a range of non-UK source countries. The risk assessment is attached at annex 1.

RISK ASSESSMENT METHODOLOGY

2. The DH risk assessment quantifies the risks to public health of pooled plasma under a number of different scenarios. The purpose of the risk assessment is to allow the potential vCJD risks of different plasma products to be quantified and compared to inform recommendations about their use. Firstly, it presents a comparison of the risk of single unit plasma with respect to pooled plasma of various pool sizes from the same source country. It then compares the relative risk of single unit plasma to pooled plasma (for various pool sizes) from a different source country. This is done on a scenario basis, using different assumed values for the risk reduction from sourcing from outside the UK.

3. Due to the uncertainty of a number of key parameters associated with vCJD, the risk assessment uses a scenario-based approach for two of the main parameters: vCJD prevalence in the UK donor population and the level of infectivity of blood. Under different infectivity and prevalence scenarios, it assesses the risk of transmission of vCJD via plasma in a pooled product, compared to the baseline risk of single unit UK plasma.

4. The paper also presents a methodology for estimating the relative vCJD prevalence of a source country with respect to the UK, through comparisons of vCJD and BSE incidence, adjusted to take into account their relative degrees of active and passive surveillance.

5. Finally, the paper compares the relative risk of single unit UK plasma to pooled plasma (for various pool sizes) that has been prion filtered.

© SEAC 2009

1

KEY ASSUMPTIONS USED IN THE DH RISK ASSESSMENT

6. The key assumptions used in the risk assessment are:

Two scenarios for the infectivity of whole blood are used for the purposes of this model: a 'low' scenario (0.1 ID/ml i.v. transmission) and a ‘high’ scenario (30 ID/ml i.v. transmission).

50% of the infectivity of whole blood is removed by leucodepletion and the remaining 50% of infectivity resides within the residual plasma.

Two scenarios for prevalence of vCJD in the UK are used: a 'low' scenario (1 in 20,000) and a ‘high’ scenario (1 in 4,000). In the absence of any further evidence, these will also be used for the UK donor population and applied irrespective of age or genotype.

The dose-response follows a Poisson distribution.

100% of the population is susceptible to infection and developing clinical symptoms of vCJD. The rationale for these assumptions is explained in the assessment.

PREVIOUS SEAC ADVICE

7. Previous SEAC advice on the pooling of blood, as well as the level, and distribution, of infectivity in blood is summarised in the risk assessment, which also includes the SEAC position statement on TSE infectivity in blood at Annex A. Other SEAC advice pertinent to consideration of the risk assessment on the geographical BSE risk categorisation and estimates of the prevalence of subclinical vCJD are given below.

8. SEAC provided advice on the sub-clinical prevalence of vCJD in its 2008 position statement on Prevalence of Subclinical variant Creutzfeldt Jakob Disease Infections 2. The statement concluded that it would be prudent to consider that the estimate provided by the Hilton et al study of 1 in 4,000, provides a reasonable, pragmatic and precautionary working scenario for the prevalence of subclinical infections.

9. SEAC has not previously provided advice on sourcing plasma from countries with known BSE risks. However, the committee provided advice on the Geographical BSE Risk (GBR) system for assessing sourcing risks as part of its discussion on medical implants containing bovine material, in February 2006. The committee considered that the GBR system gives a very imprecise indication of BSE risk. In relative terms, the BSE risk was likely to be lower in a GBR I country compared with a GBR III country, but the difference in risk cannot be quantified. In terms of a more robust risk analysis, the Committee considered that it is important to obtain a more reliable estimate of the prevalence of BSE in a country than simply GBR status, and to have confidence in the quality of the surveillance data. SEAC concluded that the GBR status of a country gives an imprecise indication of BSE risk and that it would be better to use an estimated prevalence of BSE in a country based on data from a robust surveillance system 3.

© SEAC 2009

2

ADVICE SOUGHT FROM SEAC

10. The Committee is asked to comment on:

whether the assumptions used in the risk assessment are reasonable; and

the appropriateness of the methodology of the risk assessment to quantify and compare the potential vCJD risk that may be associated with plasma products.

Notes

1. Position Statement on TSE Infectivity in Blood, July 2006,

http://www.seac.gov.uk/statements/statement0806.htm

2. Position Statement on Prevalence of Subclinical variant Creutzfeldt Jakob Disease Infections, August 2008,

http://www.seac.gov.uk/statements/statement0806.htm

3. Medical Implants Containing Bovine Material, SEAC 91, 24 February 2006.

© SEAC 2009

http://www.seac.gov.uk/papers/102-3.pdf

A comparison of the relative risk of vCJD transmission via single unit and pooled plasma from UK and non-UK sources

Sonya Crowe, Stephen Dobra and Jenny Ball, Health Protection Analytical Team, Department of Health

January 2009

PART I: BACKGROUND TO RISK ASSESSMENT

1. Introduction

This paper concerns the possible risk of person-to-person vCJD transmission via transfusion of Fresh Frozen Plasma (FFP). We propose a risk assessment methodology to assess such risks for pooled and imported FFP products relative to the baseline risk associated with UK sourced single unit FFP. We also assess the implications of different assumptions for the reduction in infectivity due to the manufacturing process. An assessment of the claimed reductions in infectivity through processing is outside the scope of this paper. The risk assessment concentrates on four potential supply options of FFP:

1. UK-sourced single unit FFP (as a baseline)

2. Non-UK-sourced single unit FFP

3. Non-UK-sourced pooled FFP

4. Non-UK-sourced prion-reduced pooled FFP

The analysis presented in this paper is concerned only with vCJD transmission, but the sourcing of plasma has a wide range of other implications, from microbiological risk to clinical efficacy and safety.

snip...

1.2 Background to Part III: Risk assessment of country sourcing There remains considerable uncertainty about the underlying prevalence of vCJD in the UK population, and populations elsewhere in the world. However, it is important when considering the risk reduction of a policy such as importation of plasma that an estimate is made of the relative prevalence of the non-UK source country to that of the UK. This allows, as detailed in part II of this paper, an assessment of the risk reduction associated with sourcing single unit or pooled plasma from non-UK sources with respect to the baseline of single unit UK plasma.

We present a methodology for estimating the relative vCJD prevalence of a source country with respect to the UK, through comparisons of vCJD and BSE incidence, adjusted to take into account their relative degrees of active and passive surveillance. Again, we ask SEAC to asses the general methodology proposed.

snip...

see full text 50 pages ;

http://www.seac.gov.uk/papers/102-3annex.pdf

In the afternoon of the 4th of March 2009, SEAC will discuss unpublished research relating to the finding of abnormal prion protein at post mortem in the spleen of a person with haemophilia (as reported recently by the Health Protection Agency). This part of the meeting will be held in closed session to allow discussion of unpublished data. This is in accordance with the SEAC Code of Practice.

END...TSS

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

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html

40,000 human heart valves a year from BSE herds

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

[Opinion (webmaster): The US has focused for years on tracing, containing, and eradicating live animal imports from the UK or other countries with acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep. This strategy does not acknowledge imports of rendered bovine products from England during the BSE period nor secondary products such as surgical catgut, which is to say surgical cowgut, or dairy cattle embryos, vaccines for veterinarian and human medicines. What has become of these? Mr. Singeltary, who lost his mother to CJD of unexplained origin a few years back and went on to became a well-known TSE activist, has tracked down voluminous pertinent import data through correspondence with UK officials and searches of government web sites. Imports of such products are frequently cited by Europeans in rating BSE risks in the US and in shutting out US exports.

Many people's eyes glaze over when reviewing reams of sometimes older trade statistics. There is no proof that any of the imported products was contaminated with BSE nor if so, any evidence that any BSE product lead to infection in US livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr. Singeltary establish that an appalling variety and tonnage of products that were imported by the US from the UK and othr BSE-affected countries during the peak of the BSE epidemic years.]

10 January 1990 COMMERCIAL IN CONFIDENCE

NOT FOR PUBLICATION

COMMITTEE ON SAFETY OF MEDICINES WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY

2.1 At the first meeting of the Working Party on Bovine Spongiform Encephalopathy on 6 September 1989, detailed consideration was given to XXXXX Surgical Catgut. This arose from the Company's response to the Letter to Licence Holders, indicating that the bovine small intestine source material was derived from UK cattle, unlike 8 other licenced catgut sutures. In contrast XXXXX Surgical Catgut was stated to hold over 90% share of the market for catgut sutures, and to constitute approximately 83% of all sutures used in U.K. IMPORTS OF SUTURES FROM THE KNOWN BSE COUNTRY;

3006.10.0000: STERILE SURGICAL CATGUT, SIMILAR STERILE SUTURE MATERIALS AND STERILETISSUE ADHESIVES FOR SURGICAL WOUND CLOSURE; AND SIMILAR STERILE MATERIAL U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

<--- Dec 1998 ---> <--- 1998 YTD ---> Country Quantity Value Quantity Value

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

WORLD TOTAL . . . . . . . 10,801 3,116 143,058 40,068

Belgium . . . . . . . . . --- --- 107 14 France . . . . . . . . . 81 49 2,727 1,132 Switzerland . . . . . . . --- --- 1,357 1,693 United Kingdom . . . . . 1,188 242 35,001 5,564

U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date Subheading 300210: ANTISERA AND OTHER BLOOD FRACTIONS, AND MODIFIED IMMUNOLOGICAL PRODUCTS

3002.10.0010: HUMAN BLOOD PLASMA U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

<--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value

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

WORLD TOTAL . . . . . . . 25,740 1,827 270,357 20,476 Belgium . . . . . . . . . 14 8 145 60 France . . . . . . . . . --- --- 134 60 Netherlands . . . . . . . --- --- 11 5 Switzerland . . . . . . . 10,462 597 86,101 5,894 United Kingdom . . . . . --- --- 335 62 3002.10.0020: NORMAL HUMAN BLOOD SERA, WHETHER OR NOT FREEZE-DRIED U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) <--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value

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

WORLD TOTAL . . . . . . . 1,039 817 19,056 22,678 Austria . . . . . . . . . --- --- 9,194 18,707 Belgium . . . . . . . . . --- --- 22 15 Netherlands . . . . . . . 353 2 6,733 41 Switzerland . . . . . . . 374 218 1,084 440 United Kingdom . . . . . --- --- 1 4 3002.10.0030: HUMAN IMMUNE BLOOD SERA U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) <--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 1,926 461 14,484 3,563 ... United Kingdom . . . . . 2 8 464 192 3002.10.0040: FETAL BOVINE SERUM (FBS) U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) <--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 2,727 233 131,486 8,502 ... Belgium . . . . . . . . . --- --- 17 32 United Kingdom . . . . . 329 82 743 756 3002.10.0090: OTHER BLOOD FRACTIONS NOT ELSEWHERE SPECIFIED OR INCLUDED U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) <--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 88,467 27,343 944,412 309,947 ... United Kingdom . . . . . 1,887 2,300 26,823 23,585 ===================================================================

http://www.ita.doc.gov/industry/otea/Trade-Detail/Latest-December/Imports/30/300290.html

U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date

Subheading 300290: HUMAN BLOOD; ANIMAL BLOOD PREPARED FOR THERAPEUTIC, ETC. USES; TOXINS, CULTURES OF MICRO-ORGANISMS (EXCLUDING YEASTS) AND SIMILAR PRODUCTS NESOI

<--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 36,178 643 250,982 11,604 ... United Kingdom . . . . . 584 39 11,292 588

http://www.ita.doc.gov/industry/otea/Trade-Detail/Latest-Month/Imports/05/051199.html

U.S. Imports for Consumption: March 1999 and 1999 Year-to-Date

Subheading 051199: ANIMAL PRODUCTS, NESOI; DEAD HORSES AND OTHER EQUINE ANIMALS, BOVINE ANIMALS, SHEEP, GOATS AND POULTRY, UNFIT FOR HUMAN CONSUMPTION, NESOI

0511.99.2000: PARINGS AND SIMILAR WASTE OF RAW HIDES OR SKINS; GLUE STOCK, NOT ELSEWHERE SPECIFIED OR INCLUDED U.S. Imports for Consumption: March 1999 and 1999 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

0511.99.4024: DAIRY CATTLE EMBRYOS U.S. Imports for Consumption: March 1999 and 1999 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Number)

<--- Mar 1999 --- <--- 1999 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . --- --- 53 16 Canada . . . . . . . . . --- --- 9 3 France . . . . . . . . . --- --- 44 13 0511.99.4050: ANIMAL PRODUCTS NOT ELSEWHERE SPECIFIED OR INCLUDED; DEAD ANIMALS OF CHAPTER 1, UNFIT FOR HUMAN CONSUMPTION U.S. Imports for Consumption: March 1999 and 1999 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms) <--- Mar 1999 --- <--- 1999 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 718,476 2,313 2,206,867 4,739 Belgium . . . . . . . . . --- --- 13 18 France . . . . . . . . . 1,088 14 1,489 20 United Kingdom . . . . . 11 3 38 9

http://www.ita.doc.gov/industry/otea/Trade-Detail/Latest-December/Imports/30/300220.html

U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date

Subheading 300220: VACCINES FOR HUMAN MEDICINE

3002.20.0000: VACCINES FOR HUMAN MEDICINE U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

<--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 25,702 26,150 550,258 378,735 Belgium . . . . . . . . . 14,311 12,029 248,041 199,036 France . . . . . . . . . 3,902 4,859 87,879 92,845 Switzerland . . . . . . . 716 353 9,303 4,271 United Kingdom . . . . . 4,075 1,172 162,960 47,148 ==================================================================

http://www.ita.doc.gov/industry/otea/Trade-Detail/Latest-December/Imports/30/300230.html

U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date

Subheading 300230: VACCINES FOR VETRINARY MEDICINE

List of (6-digit) Subheadings in this (2-digit) Chapter Next (6-Digit) Subheading ... Descending ... Ascending

Latest Monthly Data

Switch from U.S. Imports to U.S. Exports

About These Trade Data Tables

3002.30.0000: VACCINES FOR VETRINARY MEDICINE U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

<--- Dec 1998 --- <--- 1998 YTD --- Country Quantity Value Quantity Value ================================================================= WORLD TOTAL . . . . . . . 6,528 237 87,149 2,715 Canada . . . . . . . . . --- --- 2,637 305 Federal Rep. of Germany --- --- 104 5 Netherlands . . . . . . . 138 64 472 192 New Zealand . . . . . . . 6,390 173 83,882 1,895 United Kingdom . . . . . --- --- 54 318 =================================================================

http://www.ita.doc.gov/industry/otea/Trade-Detail/Latest-December/Imports/30/300610.html

U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date

Subheading 300610: STERILE SURGICAL CATGUT, SIMILAR STERILE SUTURE MATERIALS AND STERILE TISSUE ADHESIVES FOR SURGICAL WOUND CLOSURE; STERILE HAEMOSTATICS, ETC.

3006.10.0000: STERILE SURGICAL CATGUT, SIMILAR STERILE SUTURE MATERIALS AND STERILETISSUE ADHESIVES FOR SURGICAL WOUND CLOSURE; AND SIMILAR STERILE MATERIAL U.S. Imports for Consumption: December 1998 and 1998 Year-to-Date (Customs Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

Belgium . . . . . . . . . --- --- 107 14 Federal Rep. of Germany 1,795 356 16,878 3,741 France . . . . . . . . . 81 49 2,727 1,132

Subject: Re: exports from the U.K. of it's MBM to U.S.??? Date: Tue, 8 Feb 2000 14:03:16 +0000 From: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:S.J.Pearsall@esg.maff.gsi.gov.uk To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto: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

Subject: Re: Imports of MBM or Ruminants to the U.S. from foreign Countries with the potential risk of BSE... Date: Tue, 28 Dec 1999 17:19:15 -0500 From: Linda Detwiler To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:flounder@wt.net (Receipt Notification Requested)

I have attached the file ibov96.txt containing all of the bovine imports for 1996.

Subject: [Fwd: IMPORTED UK AND NETHERLANDS BEEF?] -Reply Date: Thu, 3 Sep 1998 6:54:00 -0400 From: Linda Detwiler To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:flounder@wt.net (Receipt Notification Requested)

I will check on this as I had not heard about the UK. The Netherlands would not have suprised me as they did not have a case until March 1997. ... now my question would be, how many of these animals that fed on MBM's from these countries, were imported to the United States, via 3rd country routes??? i will give you that answer below...TSS

Marva Thompson Foreign Trade Reference Room 202/482-2185

"The U.S. is apparently still importing beef, pork, sheep, and lamb from countries in which BSE is found [this is probably completely legal under regulations applicable at time of import-- webmaster]:

Bovine anmls bnlss ex prcssd frozen/U.S. Imports for Consumption 1997 year to date (custom value, in thousands of dollars) (units of quantity: kilograms)

United Kingdom 37,122 kilograms, 43 thousand dollars Netherlands 56,260 kilograms, 413 thousand dollars Canada 18,141,481 kilograms, 23,914 million dollars

Livers of bovine animals, edible, frozen. U.S. Imports for consumption

Netherlands 19,230 kilograms, 25 thousand dollars Canada 160,632 kilograms, 147 thousand dollars

Tongues of bovine animals, edible, frozen U.S. Imports for consumption

Netherlands 1,047 kilograms, 4 thousand dollars Canada 767,859 kilograms, 2,028 million

Hi-qulty beef cuts w/bone in prcssd f/c u.S. Imports for consumption

Canada 25,332 kilograms, 37 thousand dollars

Beef cuts w/bone in excpt prcdssd fr/ch u.S. Imports for consumption

Netherlands 5,276 kilograms, 30 thousand dollars Canada 117,142 kilograms, 353 thousand dollars

Meat bovine anmls cuts w/bone ex prrocssd fr us imports for consumption

Netherlands 51,836 kilograms, 444 thousand dollars Canada 120,955,010 kilograms, 253,199 million

Cattle hides, whole, fresh or wet-salt u.S. Imports for consumption

Belgium 1,270 pieces, 112 thousand dollars United kingdom 36 pieces, 3 thousand dollars Ireland 12,797 pieces, 839 thousand dollars Italy 50 pieces, 10 thousand dollars Fr germany 2,500 pieces, 36 thousand dollars Canada 1,405,430 pieces, 67,320 million dollars

Hides/skins bovine anmls nesoi whole frh/wet-saltd u.S. Imports for consumption

United kingdom 13 pieces, 1 thousand dollars Italy 4 pieces, 4 thousand dollars Germany 9,455 pieces, 139 thousand dollars Canada 567,816 pieces, 17,196 million dollars

Cattle hides, whole, fresh or wet-salted u.S. Imports for consumption

1998 year to date Italy 7 pieces, 2 thousand dollars Ireland 1,408 pieces, 85 thousand dollars France 25 pieces 2 thousand dollars Canada 965,355 pieces, 37,244 million dollars

Hides and skins of bovine animals, whole, nesoi, fresh or wet-salted U.S. Imports for consumption

United kingdom 18 pieces, 3 thousand dollars Sweden 1 pieces, 1 thousand dollars Italy 2 pieces, 2 thousand dollars Germany 5,565 pieces, 72 thousand dollars Canada 84,327 pieces, 2,257 million dollars

Sheep, lamb skins, no wool, nesoi, pickled not split, u.S. Imports for Consumption

United kingdom 9,504 pieces, 88 thousand dollars Sheep, lamb skins, no wool, nesoi, pickled, split u.S. Imports for Consumption

United Kingdom 149,580 pieces, 1,212 million dollars Netherlands 50,400 pieces, 267 thousand dollars Italy 4,175 pieces, 64 thousand dollars France 13,644 pieces, 57 thousand dollars Canada 131,642 pieces, 241 thousand dollars

Flawed inspection of food is a danger, senate panel told 9-11-98 Knight Rider Tribune News

The government's current system to check food imports for possible health dangers is dangerously flawed, experts in the food business told a Senate subcommittee Thursday. U.S. inspectors check only 2 percent of all foreign shipments and consistently issue low penalties to importers who break the rules, experts said. Unscrupulous importers typically import large amounts of products that will not pass (Food and Drug Administration) inspection, said a former West Coast customs broker.

He said importers easily bypass inspections by docking at high-volume ports, such as Los Angeles-Long Beach and New York, where the inspection force is stretched thin. Inspections are so low there they virtually pass right through.

Subject: MBM/U.K. imports of MBM to the U.S./BSE Inquiry http://www.bse.org.uk/dfa/dfa25.htm Date:Mon, 10 Apr 2000 15:14:21 -0700 From: "Terry S. Singeltary Sr." To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:flounder@wt.net

69. On 14 February 1990, Mr Meldrum wrote a letter to the Chief Veterinary Officers of a number of countries. [76] On 15 February 1990, Mrs Attridge and other officials were sent a copy of the letter of 14 February 1990 and a list of the countries to which it had been sent. They were stated to be the countries which had imported ruminant based meat and bone meal from the United Kingdom. The countries listed were Norway; Sweden, Switzerland, Czechoslovakia, Hungary, Nigeria, Thailand, South Africa, Malaysia, Taiwan, Hong Kong, South Korea, Japan, Canada, USA, Turkey, Kenya, Malta, Libera, Lebanon, Saudi Arabia, Sri Lanka, Puerto Rico, Curacao, Finland.[77] The letter from Mr Meldrum included the following: Although we have kept the Office Internationale des Epizooties (OIE) fully informed about this new disease, and they will shortly be disseminating information and recommendations to member countries, I am writing to you on a personal basis to ensure that you are aware of all the developments in relation to BSE, including its likely cause. The majority of our findings have now been published in the Veterinary Record.?[78] 70. On 20 February 1990, Dr Pickles wrote to Ms Verity (APS/CMO). Dr Pickles? minute included the following: 1. Mr Meldrum is arguing that MAFF have already taken all the necessary and responsible steps to warn importing countries of the BSE dangers in UK meat and bone meal. Yet the action taken so far overseas suggest the message has not got through, or where it has this has been late. The first nation that woke up to the danger did so a year after our own feed ban. It seems even now several EC countries neither ban our imports or the general feeding of ruminant protein. It also seems the OIE and CVO have yet to inform the rest of the world. 2. I do not see how this can be claimed to be responsible?. We do not need an expert group of the Scientific Veterinary Committee to tell us British meat and bone meal is unsafe for ruminants. I fail to understand why this cannot be tackled from the British end which seems to be the only sure way of doing it, preferably by banning exports. As CMO says in his letter of 3 January surely it is short sighted for us to risk being seen in future as having been responsible for the introduction of BSE to the food chain in other countries.??[79]

http://www.mad-cow.org/00/jul00_dont_eat_sheep.html#hhh

The documents below were provided by Terry S. Singeltary Sr on 8 May 2000. They are optically character read (scanned into computer) and so may contain typos and unreadable parts.

TIP740203/l 0424 CONFIDENTIAL

http://www.mad-cow.org/00/may00_news.html#aaa

5.3.3 The greatest risk, in theory, would be from parenteral injection of material derived from bovine brain or lymphoid tissue. Medicinal products for injection or surgical implantation which are prepared from bovine tissues, or which utilise bovine serum albumin or similar agents in their manufacture, might also be capable of transmitting infectious agents. All medicinal products are licensed under the Medicines Act by the Licensing Authority following guidance, for example from the Committee on Safety of Medicines (CSM), the Committee on Dental and Surgical Materials (CDSM) and their subcommittees. The Licensing Authority have been alerted to potential concern about BSE in medicinal products and will ensure that scrutiny of source materials and manufacturing processes now takes account of BSE agent.

http://www.bseinquiry.gov.uk/files/ib/ibd1/tab02.pdf

BEFORE the BSE Inquiry went online, i was requesting the daily hearings and submissions, and they were sending them to me via air mail. then, when the BSE Inquiry finally went online, i was then able to go back and match up some of what i had with the YB numbers (above), with the official documents. ...TSS

BSE offals used in cosmetics, toiletry and perfume industry Sun, 3 Sep 2000. Unpublished Inquiry documents obtained by CJD activist Terry S. Singeltary Sr. of Bacliff, Texas Miss Marion Kelly Cosmetic, Toiletry and Perfumery Association 35 Dover Street London W1X3RA

Department of Trade and Industry 10-18 Victoria Street London SW1H ONN Enquiries 01-215 5000 Telex 8811074 DTHQ G 01 215 3324 1 February 1990

http://www.mad-cow.org/00/sep00_news.html#bbb

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

>>>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...

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

GREETINGS ! NOW that the USA is doing the same thing the U.K. did, i.e. exporting there TSE tainted product into commerce, and thanks to GW, USDA, OIE et al, it's legal now, but since the poor mother and son that died from nvCJD from eating there favorite food 'cow brain', let's look at some of the countries now getting USDA certified mad cow brains ; bovine brain is used in Mexican food, specifically Jan. 7--The Mexican menu at Taqueria El Rodeo so authentic that you'll find cesos de res - cow brains -- on the meat list, right between tongue and headcheese.

http://www.allbusiness.com/food-beverage/restaurants-food-service/10251896-1.html

A fried-brain sandwich is generally a sandwich with sliced calves' brains on sliced bread. Thinly sliced fried slabs on white toast became a ubiquitous menu item in St. Louis, Missouri, after the rise of the city's stockyards in the late 1880s, although demand there has so dwindled that only a handful of eateries still offer them. But they remain popular in the Ohio River valley, where they are served heavily battered on hamburger buns. In Evansville, Indiana, it is not uncommon to find them on many restaurant menus and they are a major seller at the annual Fall Festival. Evansville is a city located in Vanderburgh County, Indiana. ... An Italian sandwich. ... Beef brains and calf or veal (juvenile beef) brains are used in certain types of ethnic cooking, such as French cuisine and Mexican cuisine (known as sesos in Spanish), often seen in tacos and burritos. ... Percentages are relative to US recommendations for adults. ... Frying is the cooking of food with cooking oil as the heat transfer medium. ... This page is about the food toast, for other meanings, see Toast (disambiguation). ... Nickname: Gateway City, Gateway to the West, or Mound City Location in the state of Missouri Coordinates: Country United States State Missouri County Independent City Mayor Francis G. Slay (D) Area - City 66. ... // Development and commercial production of electric lighting Development and commercial production of gasoline-powered automobile by Karl Benz, Gottlieb Daimler and Maybach First commercial production and sales of phonographs and phonograph recordings. ... The Ohio River is the largest tributary by volume of the Mississippi River. ... Hamburgers often contain lettuce, onions, and other toppings, as shown here. ... Nickname: River City Location in the state of Indiana Country United States State Indiana County Vanderburgh Mayor Jonathan Weinzapfel Area - City 105. ...

CNN Cow brain sandwiches still on the menu. Associated Press, January 16, 2004.

http://www.nationmaster.com/encyclopedia/Brain-sandwiches

Where in Mexico City are you going to stay? And what type of food? Obviously mexican food (any region) is great but you will be surprised with the quality of french, spanish, italian and steak houses. There are thousands of restaurant all around the city and its suburbs. I am familiar with the south part of Mexico City. Assuming that you are more interested in mexican food San Angel Inn is a great place for food and ambiance. I used to get the "sesos a la mantequilla negra" {cow brain cooked in black butter} that was before the boom of cattle CNS viral infections. The dish is similar to sweetbreads. I still order it if they have it on the menu.

For great "cocina poblana" Las Cazuelas de la abuela located in a strip mall in San Jeronimo. The "sopa de medula" (spinal cord soup) is to die for. Also try the chalupitas and the "enchiladas de leche" (milk enchiladas); pretty much anything on the menu is tasty and not expensive.

http://forums.winespectator.com/eve/forums/a/tpc/f/958106983/m/186107164

Subject: What if TSEs are in USA cattle herds? (EXPORTS OF USA BOVINE BRAINS/SWEATBREADS) From: "Terry S. Singeltary Sr." <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:flounder@WT.NET> Reply-To: Bovine Spongiform Encephalopathy <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:BSE-L@UNI-KARLSRUHE.DE> Date: Wed, 14 Nov 2001 15:39:04 -0800 Content-Type: text/plain Parts/Attachments: text/plain (244 lines)

######## Bovine Spongiform Encephalopathy <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:BSE-L@UNI-KARLSRUHE.DE> #########

U.S. Trade Quick-Reference Tables: August 2001 Exports

Subheading 020629: OFFAL OF BOVINE ANIMALS, EDIBLE, NESOI, FROZEN

snip...

0206.29.0030: BRAINS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: August 2001 and 2001 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

August 2001 2001, through August Quantity Value Quantity

Value WORLD TOTAL 23,052 35

125,160

192 Federal Rep. of Germany 0

3,962

4 Mexico 15,147 28

103,611

170 Norway 7,905 8

7,905

8 Singapore 0

9,682

10

0206.29.0040: SWEATBREADS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: August 2001 and 2001 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms)

August 2001 2001, through August Quantity Value Quantity

Value WORLD TOTAL 616,391 749

1,563,588

2,079 Argentina 148,806 242

969,496

1,328 Colombia 24,037 16

24,037

16 Japan 22,045 49

75,946

186 Mexico 410,663 427

481,956

523 Singapore 0

633

6 United Arab Emirates 0

680

6 Venezuela 10,840 14

10,840

14

snip...

http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-Month/Exports/02/020629.html

Subject: USDA December 2006 Exports POTENTIAL MAD COW BOVINE BRAINS, HEARTS, KIDNEYS, SWEATBREADS, LIPS, OFFAL, TONGUES, AND MORE From: "Terry S. Singeltary Sr." <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:flounder9@VERIZON.NET> Reply-To: Bovine Spongiform Encephalopathy <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG> Date: Fri, 15 Dec 2006 16:04:43 -0600 Content-Type: text/plain Parts/Attachments: text/plain (1173 lines)

Subject: USDA December 2006 Exports POTENTIAL MAD COW BOVINE BRAINS, HEARTS, KIDNEYS, SWEATBREADS, LIPS, OFFAL, TONGUES, AND MORE

Date: December 15, 2006 at 1:27 pm PST Greetings,

i thought it might be a good time at the end of 2006 and compare what country, if any, would still be foolish enough to import potentially certified USDA MAD COW BRAINS FOR HUMAN CONSUMPTION. last time i looked this up was in 2004 and was shocked at the countries still doing this. seems the USDA et al would gladly poison any third world country that is willing to take this BSe, but only two take them now i.e. Gabon and the Ivory Coast are still accepting USDA certified potentially mad cow brains for human consumption. a sad day when they know that these brains could very likely contain the TSE agent, but yet still export them to other countries. sadly, the USA is no better than the UK when in came/comes to knowingly exporting there BSE/TSE. just gotta love that BSE MRR policy of GWs and the OIE, the legal trading of all strains of TSE globally. ...TSS

0206290030: BRAINS OF BOVINES, EDIBLE, FROZEN

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram)

January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 0 0 0 0 48,988 27 0 0 0 0 0 0 0 0 0 0 0 0 0 0 48,988 27 Gabon 0 0 0 0 24,494 14 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,494 14 Ivory Coast 0 0 0 0 24,494 14 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,494 14

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206290030&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

THAT compared to 2004 USDA EXPORT OF POTENTIAL MAD COW BRAINS ;

0206290030: BRAINS OF BOVINE ANIMALS, EDIBLE, FROZEN U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value

.World 37,727 33 363,222 344

Mexico 37,727 33 338,475 326 Romania 0 0 24,747 19

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020629.html

0206290040: SWEATBREADS OF BOVINES, EDIBLE, FROZEN

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 1,837 3 0 0 0 0 17,690 40 55,641 74 19,264 34 0 0 5,739 7 32,760 43 0 0 132,931 200 Bahamas 0 0 0 0 0 0 0 0 0 0 0 0 0 0 5,739 7 0 0 0 0 5,739 7 Dominican Republic 1,837 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1,837 3 Mexico 0 0 0 0 0 0 17,690 40 55,641 74 19,264 34 0 0 0 0 32,760 43 0 0 125,355 191

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206290040&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

0206210000: TONGUES OF BOVINES, EDIBLE, FROZEN

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 743,244 2,712 770,499 2,661 689,994 1,924 576,134 1,357 898,542 1,947 1,105,844 2,649 490,803 1,325 658,963 1,893 789,509 2,330 657,647 1,858 7,381,179 20,656 Belgium 0 0 0 0 0 0 23,000 20 8,723 25 0 0 0 0 0 0 0 0 0 0 31,723 45 Bulgaria 0 0 0 0 19,156 70 23,492 64 47,091 127 23,357 62 22,902 38 24,295 72 81,698 240 0 0 241,991 672 Colombia 0 0 0 0 0 0 300 3 0 0 0 0 0 0 0 0 0 0 0 0 300 3 Costa Rica 0 0 0 0 0 0 22,415 60 0 0 0 0 0 0 0 0 0 0 0 0 22,415 60 Germany 0 0 305 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 305 3 Greece 0 0 0 0 0 0 0 0 3,414 10 22,936 37 0 0 0 0 0 0 0 0 26,350 47 Honduras 1,470 6 0 0 0 0 0 0 0 0 0 0 4,504 13 0 0 0 0 0 0 5,974 19 Hong Kong 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 50,294 118 50,294 118 Japan 0 0 0 0 0 0 0 0 0 0 0 0 0 0 595 9 0 0 0 0 595 9 Latvia 0 0 0 0 0 0 73,454 165 0 0 0 0 0 0 0 0 0 0 0 0 73,454 165 Mexico 741,774 2,706 770,194 2,658 670,838 1,854 410,277 978 839,314 1,785 1,058,200 2,546 463,397 1,274 634,073 1,813 707,811 2,090 606,366 1,737 6,902,244 19,442 Netherlands 0 0 0 0 0 0 23,196 67 0 0 0 0 0 0 0 0 0 0 0 0 23,196 67 Philippines 0 0 0 0 0 0 0 0 0 0 1,351 3 0 0 0 0 0 0 987 3 2,338 6

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206210000&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

0206290010: HEARTS OF BOVINES, EDIBLE, FROZEN

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 767,425 589 516,379 411 838,340 690 365,331 269 223,401 138 273,330 194 655,828 503 516,732 308 857,063 528 1,023,945 660 6,037,774 4,290 Angola 54,449 41 54,431 45 54,430 46 0 0 0 0 0 0 0 0 0 0 0 0 27,215 26 190,525 157 China 0 0 0 0 0 0 0 0 0 0 0 0 57,420 36 0 0 0 0 0 0 57,420 36 Costa Rica 0 0 9,525 10 9,607 7 0 0 0 0 9,525 9 15,431 15 0 0 9,525 11 0 0 53,613 53 Egypt 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 18,111 12 19,051 12 37,162 24 Korea 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 39,468 34 12,208 18 51,676 52 Mexico 712,976 548 452,423 356 765,222 629 356,250 260 214,320 129 181,242 108 452,360 322 502,186 291 772,136 450 953,124 589 5,362,239 3,683 Nicaragua 0 0 0 0 9,081 8 9,081 9 9,081 8 9,081 8 0 0 0 0 17,823 21 0 0 54,147 54 Peru 0 0 0 0 0 0 0 0 0 0 73,482 68 130,617 130 14,546 17 0 0 12,347 15 230,992 230

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206290010&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

0206220000: LIVERS OF BOVINES, EDIBLE, FROZEN

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 7,728,862 9,464 7,263,710 8,918 6,170,310 6,943 8,429,810 9,979 8,536,550 8,231 9,634,156 9,271 10,820,962 10,020 8,225,706 7,977 7,758,088 7,180 9,663,403 8,489 84,231,557 86,473 Angola 70,761 27 270,847 239 136,213 119 784,365 538 497,820 383 381,219 312 574,363 481 279,872 237 810,888 680 659,739 571 4,466,087 3,587 Armenia 0 0 0 0 0 0 0 0 0 0 0 0 0 0 46,000 50 0 0 0 0 46,000 50 Bahamas 0 0 0 0 0 0 0 0 1,336 3 0 0 0 0 5,443 4 0 0 0 0 6,779 7 Bahrain 0 0 0 0 18,152 7 25,322 19 36,640 28 23,660 19 85,656 56 22,410 10 34,100 23 41,300 61 287,240 223 Canada 29,101 87 31,124 89 43,858 119 37,887 93 11,218 25 48,868 57 23,171 38 51,570 60 79,836 79 136,436 121 493,069 768 Congo 0 0 0 0 0 0 0 0 46,000 46 0 0 94,988 96 23,000 25 0 0 0 0 163,988 167 Costa Rica 41,347 34 28,572 26 28,572 28 13,265 15 18,152 25 9,321 10 6,552 7 0 0 48,843 51 19,849 18 214,473 212 Cuba 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 47,829 26 47,829 26 Dominican Republic 3,484 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 3,484 3 Ecuador 0 0 4,721 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 4,040 3 8,761 7 Egypt 4,774,076 7,024 4,028,075 6,211 4,077,426 5,168 5,723,286 7,831 6,563,392 6,827 7,789,768 7,906 9,444,848 8,902 6,984,806 6,997 5,795,342 5,569 8,082,712 7,166 63,263,731 69,601 France 0 0 0 0 0 0 0 0 0 0 0 0 0 0 9,437 7 0 0 0 0 9,437 7 Gabon 0 0 0 0 0 0 26,331 10 0 0 4,876 3 11,143 8 24,494 25 0 0 0 0 66,844 46 Greece 0 0 0 0 0 0 0 0 0 0 0 0 12,310 7 0 0 0 0 0 0 12,310 7 Guinea 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,506 18 24,506 18 Honduras 168,233 138 82,904 92 28,561 16 18,294 13 24,439 17 6,751 5 29,294 19 27,831 19 37,645 27 19,066 19 443,018 364 Iraq 319,719 233 0 0 0 0 0 0 0 0 0 0 0 0 0 0 54,210 41 0 0 373,929 273 Ivory Coast 0 0 0 0 0 0 23,610 8 0 0 0 0 0 0 0 0 0 0 0 0 23,610 8 Jamaica 69,308 36 109,377 58 36,638 19 124,772 60 44,521 28 83,587 54 35,525 23 38,997 27 28,208 20 128,114 84 699,047 409 Jordan 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 13,123 9 0 0 13,123 9 Korea 0 0 0 0 0 0 0 0 0 0 23,616 16 0 0 0 0 51,270 44 12,240 17 87,126 77 Kuwait 49,898 39 0 0 48,995 37 0 0 99,668 78 0 0 0 0 0 0 0 0 0 0 198,561 155 Malaysia 0 0 0 0 0 0 0 0 0 0 0 0 0 0 46,310 21 0 0 47,764 80 94,074 101 Mexico 412,895 277 368,053 200 421,650 269 374,343 193 537,381 351 614,174 428 246,284 191 380,638 259 208,595 150 289,508 187 3,853,521 2,506 Netherlands 78,000 75 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 78,000 75 Nicaragua 18,943 15 18,807 15 41,651 33 43,216 32 34,563 26 47,199 35 0 0 36,595 26 18,883 20 38,375 31 298,232 233 Peru 0 0 0 0 0 0 0 0 0 0 263,209 167 29,806 19 77,603 57 193,669 161 87,431 67 651,718 470 Poland 1,593,796 1,306 2,216,196 1,896 1,174,658 957 926,766 899 444,095 266 24,494 25 24,944 23 47,494 50 70,000 75 0 0 6,522,443 5,498 Republic of South Africa 0 0 0 0 0 0 25,348 36 0 0 0 0 10,945 8 0 0 0 0 24,494 21 60,787 65 Russia 0 0 0 0 0 0 150,869 82 0 0 108,903 99 0 0 0 0 0 0 0 0 259,772 181 Saudi Arabia 99,301 171 54,803 52 40,951 109 106,412 140 163,955 118 128,905 92 178,020 134 100,206 80 234,490 170 0 0 1,107,043 1,066 Senegal 0 0 0 0 49,389 18 0 0 13,370 9 13,353 9 0 0 0 0 12,349 9 0 0 88,461 45 Sierra Leone 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,000 22 0 0 24,000 22 Singapore 0 0 25,737 10 0 0 25,724 9 0 0 0 0 13,113 9 0 0 0 0 0 0 64,574 29 Spain 0 0 0 0 0 0 0 0 0 0 0 0 0 0 23,000 23 0 0 0 0 23,000 23 United Arab Emirates 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 42,637 31 0 0 42,637 31 United Kingdom 0 0 24,494 25 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,494 25 Vietnam 0 0 0 0 23,596 45 0 0 0 0 0 0 0 0 0 0 0 0 0 0 23,596 45 Zaire 0 0 0 0 0 0 0 0 0 0 62,253 33 0 0 0 0 0 0 0 0 62,253 33

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206220000&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

0206100000: OFFAL OF BOVINES, EDIBLE, FRESH OR CHILLED

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram)

January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 151,702 598 142,843 727 276,270 896 188,421 543 362,729 970 116,820 472 185,245 638 297,623 832 271,498 640 178,816 637 2,171,967 6,953 Anguilla 0 0 0 0 87 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 87 3 Bahamas 0 0 1,273 3 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1,273 3 Bahrain 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 Canada 21,379 15 0 0 46,853 71 18,452 17 171,620 279 9,451 15 29,694 46 139,002 222 206,428 318 64,805 103 707,684 1,086 China 0 0 0 0 0 0 0 0 0 0 0 0 0 0 19,704 66 9,206 31 0 0 28,910 97 Hong Kong 28,166 136 14,490 21 27,855 41 17,875 27 0 0 0 0 6,759 23 0 0 0 0 0 0 95,145 248 Japan 550 8 0 0 0 0 0 0 0 0 0 0 0 0 4,132 14 0 0 0 0 4,682 22 Mexico 78,027 424 127,080 702 201,475 781 152,094 499 191,109 691 107,369 457 148,792 569 125,593 480 55,864 292 114,011 535 1,301,414 5,430 Philippines 0 0 0 0 0 0 0 0 0 0 0 0 0 0 9,192 50 0 0 0 0 9,192 50 Singapore 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 United Kingdom 23,580 15 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 23,580 15

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206100000&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

0206290090: OFFAL OF BOVINES, EDIBLE, FROZEN, NESOI

U.S. Domestic Exports: 2006 and 2006 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) January February March April May June July August September October Through October Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value Quantity Value .World 3,317,168 4,027 3,016,529 4,700 3,438,524 5,024 3,193,299 3,613 2,833,026 4,158 1,968,683 2,972 1,368,243 2,201 1,437,708 2,335 1,481,508 2,677 1,627,962 2,664 23,682,650 34,371 Albania 0 0 0 0 0 0 0 0 0 0 0 0 24,390 47 0 0 0 0 0 0 24,390 47 Angola 2,025 3 0 0 0 0 0 0 0 0 0 0 20,310 30 21,501 34 14,590 27 1,361 4 59,787 99 Antigua Barbuda 0 0 0 0 0 0 0 0 13,255 31 0 0 0 0 0 0 0 0 3,402 4 16,657 35 Barbados 0 0 0 0 0 0 1,644 15 0 0 0 0 0 0 0 0 0 0 0 0 1,644 15 Belgium 0 0 0 0 24,494 25 24,494 25 0 0 0 0 0 0 24,494 25 24,000 25 48,988 50 146,470 150 Bulgaria 0 0 23,596 23 0 0 0 0 16,568 25 16,568 25 8,284 12 0 0 47,655 39 24,494 21 137,165 145 Canada 855,976 567 614,312 449 831,285 574 1,080,210 670 502,611 611 395,999 547 217,288 315 390,223 591 480,802 813 332,017 472 5,700,723 5,611 Congo 0 0 0 0 0 0 0 0 0 0 23,610 23 0 0 0 0 0 0 0 0 23,610 23 Cyprus 0 0 0 0 0 0 0 0 0 0 0 0 0 0 24,494 25 0 0 0 0 24,494 25 Ecuador 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 28,046 39 28,046 39 Egypt 690,000 875 320,000 500 146,964 150 0 0 0 0 0 0 0 0 0 0 0 0 0 0 1,156,964 1,525 Gabon 47,220 46 70,830 69 48,988 50 165,270 161 94,440 92 73,288 75 24,494 25 71,894 71 70,610 73 24,000 23 691,034 685 Germany 0 0 24,494 25 24,420 25 0 0 0 0 0 0 0 0 0 0 0 0 0 0 48,914 50 Greece 0 0 0 0 24,426 21 48,988 24 24,734 38 25,350 38 0 0 0 0 47,628 40 48,988 43 220,114 204 Guatemala 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 7,143 12 7,143 12 Guyana 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 13,578 13 13,578 13 Honduras 0 0 0 0 0 0 0 0 0 0 0 0 19,051 18 19,051 18 0 0 0 0 38,102 37 Hong Kong 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 43,143 65 43,143 65 Italy 0 0 0 0 0 0 0 0 552 4 0 0 0 0 0 0 0 0 0 0 552 4 Ivory Coast 285,088 280 188,880 184 192,416 192 118,050 115 330,430 324 94,440 92 94,440 92 213,702 204 48,104 44 165,882 174 1,731,432 1,701 Jamaica 210 4 55,425 97 63,179 87 56,267 29 80,773 123 32,838 58 0 0 10,458 16 18,824 86 9,531 14 327,505 515 Japan 182,627 287 148,426 227 207,558 336 147,611 209 278,269 378 163,490 213 145,112 189 72,775 95 13,874 21 0 0 1,359,742 1,956 Korea 0 0 0 0 0 0 0 0 18,035 35 0 0 0 0 0 0 0 0 0 0 18,035 35 Macao 0 0 47,163 119 92,937 218 70,740 168 47,170 87 23,586 58 70,753 168 0 0 0 0 0 0 352,349 819 Mali 0 0 0 0 0 0 0 0 23,610 23 0 0 0 0 0 0 0 0 24,494 25 48,104 48 Mexico 863,030 1,567 1,364,663 2,785 1,620,522 3,099 1,199,058 1,899 1,264,144 2,124 980,786 1,661 661,690 1,223 570,222 1,241 700,452 1,477 782,876 1,616 10,007,443 18,693 Nicaragua 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 18,804 18 18,804 18 Philippines 0 0 0 0 4,556 24 0 0 0 0 23,587 21 0 0 0 0 14,969 31 23,496 35 66,608 112 Poland 367,410 375 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 367,410 375 St Lucia 0 0 0 0 4,870 12 0 0 0 0 0 0 0 0 0 0 0 0 0 0 4,870 12 Taiwan 23,582 23 0 0 5,764 23 10,966 44 10,613 43 0 0 0 0 0 0 0 0 0 0 50,925 133 Trinidad and Tobago 0 0 40,771 23 78,583 59 43,543 17 9,050 14 22,706 18 6,526 10 18,894 15 0 0 22,829 17 242,902 171 United Arab Emirates 0 0 0 0 0 0 11,879 12 0 0 0 0 0 0 0 0 0 0 4,890 21 16,769 33 Vietnam 0 0 117,969 197 67,562 129 214,579 224 118,772 205 92,435 143 75,905 72 0 0 0 0 0 0 687,222 969

Source: Foreign Trade Division, U.S. Census Bureau. Prepared by the Office of Trade and Industry Information (OTII), International Trade Administration, U.S. Department of Commerce.

http://hq-tpisweb.ita.doc.gov/portal/page/portal/rptsforms/p_hsyrly2?p_year=2006&p_hs=0206290090&p_flow=''exports''&p_endmth=''October''&p_table=ita.hs_mthytd_expdom_ts2006

TSS

Subject: USA December 2004 Exports BOVINE STATS BRAINS, HEARTS, KIDNEYS, SWEATBREADS, LIPS, OFFAL, TONGUES, AND MORE Date: Mon, 25 Apr 2005 16:21:28 -0500 From: “Terry S. Singeltary Sr.” Reply-To: Bovine Spongiform Encephalopathy mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:-L@LISTS.UNI-KARLSRUHE.DE> To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000288/!x-usc:mailto:BSE-L@LISTS.UNI-KARLSRUHE.DE ##################### Bovine Spongiform Encephalopathy #####################

0206290030: BRAINS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 37,727 33 363,222 344 Mexico 37,727 33 338,475 326 Romania 0 0 24,747 19

0206290010: HEARTS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 581,872 418 7,564,955 5,685 Angola 0 0 211,527 46 Cambodia (Kampuchea) 0 0 22,682 60 China 0 0 49,887 36 Colombia 0 0 22,657 28 Gabon 0 0 24,947 11 Hong Kong 0 0 24,494 45 Indonesia 400,639 261 4,420,683 2,747 Italy 0 0 24,494 20 Korea 0 0 124,089 71 Mexico 181,233 157 2,494,078 2,517 Poland 0 0 47,359 20 Russia 0 0 98,058 85

0206290020: KIDNEYS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram)

December 2004 2004, through December Quantity Value Quantity Value .World 303,293 175 3,009,780 1,684 Angola 0 0 60,075 15 Bahamas 0 0 11,431 8 Cayman Islands 0 0 4,450 10 China 0 0 48,988 26 Gabon 48,200 15 489,329 206 Hong Kong 0 0 48,988 26 Indonesia 0 0 47,174 17 Ivory Coast 188,414 96 1,694,772 959 Jamaica 10,546 5 78,933 37 Mexico 56,133 59 203,788 204 Moldova 0 0 295,091 166 Romania 0 0 26,761 10

0206290040: SWEATBREADS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 0 0 95,209 83 Bulgaria 0 0 25,243 22 Ghana 0 0 0 0 Mexico 0 0 69,859 58 Netherlands 0 0 107 4

0206290050: LIPS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 1,389,825 2,709 7,355,294 13,917 Bahamas 0 0 40,000 5 Mexico 1,386,800 2,706 7,293,673 13,895 Montserrat 0 0 18,596 15 Namibia 3,025 3 3,025 3

0206290090: OFFAL OF BOVINE ANIMALS, EDIBLE, NESOI, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 2,386,597 2,913 22,701,393 25,320 Antigua Barbuda 2,141 6 3,162 11 Argentina 590 4 1,044 7 Aruba 1,000 4 1,476 10 Bahamas 0 0 117,929 184 Bulgaria 0 0 315,543 301 Canada 337,392 255 3,544,821 3,347 Cayman Islands 0 0 5,350 21 China 0 0 22,185 37 Colombia 0 0 24,127 36 Cyprus 0 0 25,052 20 Denmark 0 0 46,416 25 Dominican Republic 0 0 24,086 16 Egypt 0 0 145 3 Gabon 96,208 92 316,411 271 Germany 0 0 2,545,197 554 Greece 0 0 190,564 146 Guatemala 0 0 117,362 197 Haiti 0 0 13,125 25

Haiti 0 0 13,125 25 Honduras 23,940 34 23,940 34 Hong Kong 0 0 48,343 130 Indonesia 7,470 9 640,472 249 Italy 0 0 47,849 38 Ivory Coast 192,410 184 1,133,273 1,012 Jamaica 80,703 54 124,514 86 Japan 25,094 53 432,608 2,659 Korea 0 0 23,596 25 Malaysia 97,997 48 457,516 203 Mexico 1,376,419 1,977 9,425,957 13,261 Netherlands 20,229 20 245,555 129 New Zealand 0 0 0 0 Panama 23,399 44 161,425 117 Philippines 0 0 22,184 39 Poland 0 0 805,355 477 Romania 48,988 33 1,294,879 1,191 Senegal 0 0 52,909 103 Singapore 0 0 728 3 Spain 0 0 202 4 St Christopher-Nevis 0 0 1,020 3 St Lucia 0 0 5,313 8 Switzerland 0 0 6,506 35 Taiwan 46,920 86 97,268 127 Trinidad and Tobago 0 0 38,102 84 Turks and Caicos Islands 5,697 9 6,082 13 United Kingdom 0 0 291,303 76 Uruguay 0 0 499 4

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020629.html

0206100000: OFFAL OF BOVINE ANIMALS, EDIBLE, FRESH OR CHILLED

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 149,260 308 1,312,166 2,933 Bahamas 2,174 3 6,710 6 Canada 15,265 14 115,659 129 Cayman Islands 0 0 1,312 5 China 48,988 44 48,988 44 Egypt 0 0 27,654 81 Gabon 0 0 97,462 80 Hong Kong 0 0 48,988 30 Indonesia 0 0 38,000 33 Ivory Coast 0 0 127,000 108 Kuwait 0 0 12,487 36 Mexico 82,833 247 667,881 2,070 Philippines 0 0 26,797 88 Taiwan 0 0 43,544 30 Turks and Caicos Islands 0 0 1,907 3 United Arab Emirates 0 0 47,777 190

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020610.html

0206210000: TONGUES OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 432,185 755 3,479,807 6,127 Bangladesh 23,623 38 47,380 77 Honduras 15,163 28 17,105 31 Hong Kong 0 0 47,151 86 Indonesia 0 0 83,245 193 Japan 0 0 25,697 57 Kazakhstan 0 0 145,001 175 Mexico 368,562 638 3,046,441 5,384 Nicaragua 6,537 13 6,537 13 Philippines 18,300 38 61,250 111

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020621.html

0206220000: LIVERS OF BOVINE ANIMALS, EDIBLE, FROZEN

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 5,712,394 4,115 55,278,021 38,020 Albania 0 0 74,863 45 Angola 24,677 22 888,634 430 Antigua Barbuda 2,682 3 5,111 5 Azerbaijan 97,471 45 633,575 324 Bahamas 1,836 3 10,561 8 Belarus 0 0 355,818 130 Belgium 488,886 295 2,998,386 1,265 Bulgaria 0 0 355,234 310 Canada 18,416 49 372,057 910 Dominican Republic 0 0 13,599 7 Ecuador 0 0 8,378 5 France 0 0 92,361 29 Georgia 0 0 243,876 170 Germany 609,187 323 2,104,312 908 Greece 0 0 149,205 87 Guatemala 0 0 19,962 11 Honduras 65,432 30 196,264 104 India 0 0 67,491 55 Indonesia 769,971 1,139 4,495,414 7,077 Iraq 0 0 48,984 21 Italy 0 0 9,002 12 Ivory Coast 0 0 24,701 8 Jamaica 170,782 137 170,782 137 Kazakhstan 0 0 868,130 762 Korea 0 0 23,593 17 Kuwait 0 0 1,560 7 Latvia 0 0 577,885 166 Liberia 0 0 24,950 16 Liechtenstein 0 0 24,369 11 Lithuania 0 0 996,660 552 Madagascar 0 0 24,494 10 Mali 0 0 49,211 40 Mexico 264,561 131 5,094,330 5,868 Moldova 249,107 125 2,743,394 1,314 Mozambique 0 0 24,093 17 Netherlands 0 0 49,520 26 Nicaragua 31,131 19 48,335 25 Philippines 24,504 14 97,948 156 Poland 1,553,201 953 23,262,526 13,113 Romania 0 0 199,072 121 Russia 0 0 544,267 225 Saudi Arabia 70,805 88 243,230 381 Senegal 0 0 285,311 302 Turkey 884,029 496 1,733,636 881 Ukraine 0 0 244,559 132 United Kingdom 362,446 235 4,709,748 1,792 Venezuela 0 0 45,360 19 Zaire 23,270 9 23,270 9

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020622.html

0201203550: MEAT OF BOVINE ANIMALS, NESOI, CUTS WITH BONE IN, PROCESSED, FRESH OR CHILLED

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 293,718 1,252 703,296 2,552 Anguilla 0 0 11,482 35 Aruba 0 0 4,775 24 Bahamas 19,416 50 247,589 684 Barbados 0 0 14,377 33 Bermuda 0 0 1,361 3 Cayman Islands 0 0 13,502 48 Colombia 244,578 738 244,578 738 France 6,242 368 6,700 400 Germany 1,564 48 1,564 48 Guatemala 21,918 49 21,918 49 Honduras 0 0 19,051 24 Hong Kong 0 0 14,618 76 Indonesia 0 0 1,008 3 Italy 0 0 750 6 Korea 0 0 6,023 35 Marshall Islands 0 0 37,817 121 Mexico 0 0 20,347 21 Netherlands Antilles 0 0 449 8 St Lucia 0 0 436 4 Suriname 0 0 240 4 Switzerland 0 0 2,402 81 Trinidad and Tobago 0 0 6,047 18 Turks and Caicos Islands 0 0 26,262 90

0201206000: MEAT OF BOVINE ANIMALS, CUTS WITH BONE IN, EXCEPT PROCESSED, FRESH ORCHILLED

U.S. Domestic Exports: December 2004 and 2004 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilogram) December 2004 2004, through December Quantity Value Quantity Value .World 86,480 824 698,955 4,793 Antigua Barbuda 0 0 410 6 Bahamas 10,521 27 60,334 200 Bahrain 0 0 3,721 11 Canada 39,462 342 472,538 3,481 Chile 0 0 9,694 29 Dominican Republic 0 0 28,327 37 French Polynesia 0 0 1,596 10 Greece 0 0 2,825 16 Honduras 0 0 9,887 20 Japan 0 0 0 0 Mexico 0 0 9,189 28 Netherlands Antilles 478 8 4,250 44 Philippines 0 0 393 3 Saudi Arabia 1,053 15 22,470 102 St Lucia 0 0 372 3 Svalbard, Jan Mayen Island 34,064 430 57,812 715 Trinidad and Tobago 0 0 6,258 59 Turks and Caicos Islands 902 3 8,879 29

http://ita.doc.gov/ td/ industry/ otea/ Trade-Detail/ Latest-Month/ Exports/ 02/ 020120.html

Greetings,

I find it very disturbing that the USA is exporting all these products all over the globe without proper testing for BSE/TSE. I wonder if the consumer in these countries know what they are getting?

WHO is to say that some of these nvCJD cases are not from imported USA product?

TSS

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0206.29.0030: BRAINS OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 57,279 56 192,198 225 Ivory Coast 0 0 24,971 8 Mexico 57,279 56 161,158 211 Sweden 0 0 6,069 6

0206.29.0040: SWEATBREADS OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 48,735 69 581,306 689 Bahamas 0 0 4,551 5 Hong Kong 0 0 48,988 15 Japan 0 0 18,629 51 Mexico 48,735 69 507,453 611 Switzerland 0 0 1,685 6

0206.29.0050: LIPS OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 620,626 1,393 16,539,594 23,262 Hong Kong 0 0 23,587 8 Mexico 620,626 1,393 16,513,038 23,245 Taiwan 0 0 2,969 10

0206.29.0090: OFFAL OF BOVINE ANIMALS, EDIBLE, NESOI, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 8,464,956 16,117 118,262,413 197,532 Argentina 1,497 9 1,497 9 Australia 6,103 6 72,627 71 Bahamas 0 0 25,367 55 Barbados 0 0 1,282 6 Belgium 0 0 718,837 142 Bulgaria 0 0 328,698 261 Burkina 0 0 23,496 21 Canada 304,064 276 8,137,388 6,048 China 734,212 1,750 7,554,286 16,429 Colombia 0 0 109,398 141 Costa Rica 0 0 53,911 37 Denmark 0 0 8,327 33 Dominican Republic 19,578 168 112,192 767 Egypt 0 0 167,000 96 Federal Rep. of Germany 104,016 21 2,266,317 583 Gabon 24,494 16 339,168 177 Greece 23,610 26 47,220 51 Guatemala 0 0 200,509 233 Guyana 0 0 11,555 12 Hong Kong 339,453 704 4,490,896 7,651 Indonesia 104,013 108 1,231,976 666 Israel 0 0 119,230 121 Ivory Coast 0 0 1,429,316 876 Jamaica 79,203 73 780,910 696 Japan 2,614,703 7,006 29,370,030 78,245 Jordan 0 0 72,709 390 Korea, South 1,084,495 2,217 19,825,887 37,280 Macedonia (Skopje) 0 0 143,699 51 Malaysia 0 0 24,776 10 Mexico 2,463,516 2,922 30,710,290 37,936 Netherlands 0 0 38,512 65 Nicaragua 0 0 9,411 11 Panama 0 0 480,391 472 Peru 0 0 47,135 29 Philippines 37,875 15 216,218 116 Poland 47,175 36 954,552 532 Romania 0 0 991,737 765 Russia 368,385 325 3,490,349 2,441 Singapore 0 0 5,307 15 St Lucia 2,442 3 10,896 14 Sweden 0 0 46,200 45 Taiwan 106,122 436 1,601,333 3,327 Turks and Caicos Islands 0 0 8,536 14 United Arab Emirates 0 0 27,439 130 United Kingdom 0 0 1,842,710 369 Uruguay 0 0 112,893 95

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http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-December/Exports/02/020629.html

0206.21.0000: TONGUES OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 1,377,073 7,372 27,349,941 105,661 Canada 0 0 5,159 7 China 66,968 208 675,449 1,382 Costa Rica 0 0 6,567 18 Hong Kong 121,237 431 2,176,415 3,917 Indonesia 24,957 13 39,957 17 Japan 920,049 5,943 17,255,240 83,562 Korea, South 89,412 404 2,435,561 8,129 Malaysia 0 0 23,596 10 Mexico 45,264 126 1,258,740 3,282 Poland 0 0 23,596 14 Russia 51,472 49 3,083,619 3,942 Taiwan 57,714 198 354,691 1,260 Vietnam 0 0 11,351 121

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Source: Foreign Trade Division , U.S. Census Bureau. Presented by: Office of Trade and Economic Analysis (OTEA), International Trade Administration, U.S. Department of Commerce.

http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-December/Exports/02/020621.html

0206.29.0010: HEARTS OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 1,180,635 1,038 17,267,397 12,630 Angola 0 0 47,849 31 China 0 0 97,868 41 Colombia 0 0 355,787 379 Costa Rica 0 0 4,816 4 El Salvador 4,545 4 4,545 4 Greece 0 0 15,000 6 Guatemala 0 0 19,051 18 Honduras 0 0 9,780 8 Hong Kong 45,347 110 454,574 862 Indonesia 597,243 459 8,098,035 4,681 Ivory Coast 0 0 27,216 8 Japan 0 0 19,835 20 Korea, South 49,890 50 213,036 213 Lithuania 0 0 55,194 31 Mexico 280,421 234 2,664,118 2,384 Netherlands 0 0 108,698 61 Peru 0 0 452,116 458 Russia 203,189 181 4,528,474 3,280 Saudi Arabia 0 0 3,293 6 Singapore 0 0 44,906 21 Switzerland 0 0 8,010 8 United Arab Emirates 0 0 135 3 United Kingdom 0 0 35,061 105

0206.29.0020: KIDNEYS OF BOVINE ANIMALS, EDIBLE, FROZEN

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U.S. Domestic Exports: December 2003 and 2003 Year-to-Date, not Seasonally Adjusted

(FAS Value, in Thousands of Dollars) (Units of Quantity: Kilograms) December 2003 2003, through December Quantity Value Quantity Value WORLD TOTAL 330,004 231 3,566,918 1,818 China 49,424 26 141,576 64 Gabon 0 0 49,437 28 Greece 0 0 966 6 Indonesia 0 0 23,610 15 Ivory Coast 49,891 25 1,699,427 704 Jamaica 115,626 67 875,874 436 Mexico 115,063 113 521,638 465 Russia 0 0 115,377 70 Saudi Arabia 0 0 1,660 3 South Africa 0 0 111,960 18 Thailand 0 0 25,393 10

http://www.ita.doc.gov/td/industry/otea/Trade-Detail/Latest-December/Exports/02/020629.html

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

Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.

USDA/OIG-A/50601-10-KC Page 27

observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.

snip...

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

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

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."

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125

Progress Report from the National Prion Disease Pathology Surveillance Center

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

April 3, 2008

The importance to public health in the U.S. of timely diagnosis and monitoring of human prion diseases is unquestionable. Here are some compelling reasons for this:

Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S.

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

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

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. This was done to ensure that samples inappropriate for the rapid screen test were still tested, and also to monitor and improve upon IHC testing protocols. ...

http://www.aphis.usda.gov/lpa/issues/bse_testing/test_results.html

full text ;

http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html

""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

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

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

full text ;

http://foiamadsheepmadrivervalley.blogspot.com/2008/09/re-foia-of-declaration-of-extraordinary.html

STANLEY PRUSINER NOBEL PEACE PRIZE WINNER ON THE PRION

US AG SEC AND LAYCRAFT

"nothing matters, except beef from Canada under 30 months bone in beef product, that's ALL THAT MATTERS!"

US SENATOR AND STAN THE MAN SLAM USDA "DAMNING TESTIMONY"

Senator Michael Machado from California

"USDA does not know what's going on". "USDA is protecting the industry". " SHOULD the state of California step in"

Stanley Prusiner

"nobody has ever ask us to comment"

"they don't want us to comment"

"they never ask"

i tried to see Venemon, after Canadian cow was discovered with BSE. went to see lyle. after talking with him.

absolute ignorance.

then thought i should see Venemon.

it was clear his entire policy was to get cattle boneless beef prods across the border.

nothing else mattered.

his aids confirmed this.

5 times i tried to see Venemon, never worked.

eventually met with carl rove the political.

he is the one that arranged meeting with Venemon.

just trying to give you a sense of the distance.

threat to health public safety.

was never contacted.

yes i believe that prions are bad to eat and you can die from them.END

Dr. Stan bashing Ann Veneman - 3 minutes - Damning testimony

http://maddeer.org/video/embedded/08snip.ram

File Name: USDA DON'T ASK DON'T TELL POLICY 02snip.rpm

DAMNING testimony of consumer consumption of Washington mad cow in California

http://www.maddeer.org/video/embedded/02snip.rm

IN A NUT SHELL ;

(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

full text ;

http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html

http://madcowtesting.blogspot.com/

Sunday, February 15, 2009

Scientists warn of first ever case of human mad cow disease from blood plasma

http://vcjdtransfusion.blogspot.com/2009/02/scientists-warn-of-first-ever-case-of.html

Sunday, February 15, 2009

Scientists warn of first ever case of human mad cow disease from blood plasma

http://vcjdtransfusion.blogspot.com/2009/02/scientists-warn-of-first-ever-case-of.html

Thursday, January 29, 2009

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research

http://creutzfeldt-jakob-disease.blogspot.com/2009/01/medical-procedures-and-risk-for.html

Monday, December 08, 2008

vCJD & dental treatment

http://creutzfeldt-jakob-disease.blogspot.com/2008/12/vcjd-dental-treatment.html

Tuesday, August 12, 2008

Biosafety 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

Thursday, November 27, 2008

Prion diseases are efficiently transmitted by blood transfusion in sheep

http://vcjdblood.blogspot.com/2008/11/prion-diseases-are-efficiently.html

November 25, 2008

Update On Feed Enforcement Activities To Limit The Spread Of BSE

http://madcowfeed.blogspot.com/2008/11/november-2008-update-on-feed.html

OIE amending the Annex to Decision 2007/453/EC establishing the BSE status of Member States or third countries or regions thereof according to their BSE risk

http://docket-aphis-2006-0041.blogspot.com/2009/01/oie-amending-annex-to-decision.html

Friday, October 24, 2008

CBER 2007 Annual Report Assessing the Potential Risk of variant Creutzfeldt-Jakob Disease from Blood Products

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cber-2007-annual-report-assessing.html

Saturday, August 02, 2008

WARNING OVER SECOND WAVE OF CJD CASES

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/warning-over-second-wave-of-cjd-cases.html

Thursday, April 17, 2008

Use of Materials Derived From Cattle in Human Food and Cosmetics [Docket No. 2004N-0081] RIN 0910-AF47 [Federal Register: April 17, 2008 (Volume 73, Number 75)] [Rules and Regulations] [Page 20785-20794] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr17ap08-7]

http://cjdmadcowbaseoct2007.blogspot.com/2008/04/use-of-materials-derived-from-cattle-in.html

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

Thursday, December 04, 2008 2:37 PM

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

personal communication with Professor Kong. ...TSS

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

http://bse-atypical.blogspot.com/2009/01/bovine-spongiform-encephalopathy-h-bse.html

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007

http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html

Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

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

Monday, February 09, 2009

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have CWD

http://chronic-wasting-disease.blogspot.com/2009/02/exotic-meats-usa-announces-urgent.html

Monday, December 1, 2008

When Atypical Scrapie cross species barriers

http://nor-98.blogspot.com/2008/12/when-atypical-scrapie-cross-species.html

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report

http://bse-atypical.blogspot.com/2009/01/research-project-detection-of-tse.html

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

http://seac992007.blogspot.com/

ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

2008-10-23T08:48:00.001-07:00

SEAC

ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 101st meeting in London on 15th October 2008, and discussed the following:

CURRENT ISSUES SEAC was informed about:

• A mother and son in Spain who had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. Both the mother and son lived in a region of Spain with a history of BSE and had frequently shared meals of cattle brain. As no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure.

• Results of tests on a single goat from a culled UK dairy herd with a large classical scrapie outbreak. On the basis of the results the presence of Bovine Spongiform Encephalopathy (BSE) cannot be excluded. Further testing by mouse bioassays, which may take at least two, if not more, years to complete, is required to make a definitive diagnosis.

UPDATE ON vCJD PREVALENCE STUDIES

SEAC was updated by the Health Protection Agency (HPA) about the progress of the National Anonymous Tonsil Archive (NATA), a proposed second retrospective survey of 30 000 stored appendix samples and a proposed post mortem tissue archive. These studies would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease).

Around 62 500 tonsil samples collected by NATA have been tested with no positive samples found. An application for the second retrospective survey of appendix samples is currently under consideration by a Research Ethics Committee. SEAC learned that the establishment of a post mortem tissue archive, which is dependent on the collection of samples from Coroners’ autopsies, does not have the support of Coroners needed to take it forward. SEAC is extremely disappointed about the lack of support from Coroners for the post mortem tissue archive. As SEAC has repeatedly stated, the archive is key to obtaining better estimates of the prevalence of subclinical vCJD. These estimates are vital to make meaningful assessments of the risks to public health from vCJD and of the effectiveness of current, and the need for further, very costly public health protection measures. SEAC acknowledged the strenuous efforts made by the HPA, the Department of Health (DH) and National Health Service Blood and Tissue to devise a system to collect samples that would have the least impact on the work of Coroners. SEAC remains strongly in favour of establishing the archive.

snip...

PROTEASE SENSITIVE PRIONOPATHY

SEAC discussed with Dr Pierluigi Gambetti (US National Prion Disease Pathology Surveillance Center) his recently published report5 on the identification in the United States of America of a new human prion disease. SEAC agreed that there is considerable work to be done to characterise fully this new disease, its cause and whether it is infectious or not. As preliminary unpublished data were also presented, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

RESULTS ON HUMAN SCLERA

SEAC considered preliminary results provided by the HPA and National CJD Surveillance Unit from tests on eye tissue (sclera) from a vCJD case. The results suggest the presence of infectivity and, in contrast with previous testing of samples from other vCJD cases, abnormal prion protein in this tissue. However, as the sclera is very difficult to remove from surrounding eye tissues, which are themselves known to carry vCJD infectivity, the findings may have arisen as a result of contamination at autopsy. Nevertheless, even if the data are reliable, they indicate that there may only be a relatively low level of infectivity present in sclera. As preliminary unpublished data were considered, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.

5 Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708. 5 © SEAC 2008

SEE FULL TEXT ;

http://www.seac.gov.uk/papers/101-summary.pdf

http://www.mad-cow.org/dec99_news.html#bbb

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

http://seac992007.blogspot.com/2008/10/seac-101st-meeting-on-wednesday-15th.html

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

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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

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:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000265/!x-usc:mailto: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/

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

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

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

2 January 2000

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

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

15 November 1999

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

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

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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 ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html

***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

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html

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

snip...

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. ...

snip...

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

TSS

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

2008-10-10T14:39:00.000-07:00

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

snip...

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

snip...

ITEM 3 – CURRENT ISSUES

9.

SEAC was informed about the following issues:

• Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.

http://www.seac.gov.uk/papers/101-1.pdf

3 10:10 Current issues

vCJD cluster in Spain Testing of a goat

snip...

In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.

* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.

http://www.seac.gov.uk/agenda/agen151008.htm

The CJD Foundation Newsletter

http://www.cjdfoundation.org/content/newsletters/september2008.pdf

snip...

SEPTEMBER 2008 VOLUME 1, ISSUE 3

The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.

Note 1: Not all new cases and deaths reported are confirmed by autopsy.

Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..

2008

MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS

January 35 24 203 6,424 February 24 9 212 6,848 March 19 19 164 7,492 April 36 14 231 8,427 May 26 20 191 8,839 June 19 17 144 9,646 July 28 14 171 7,791 August 27 16 150 5,323

TOTALS = 214 133 1,466 60,790

P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? help@cjdfoundation.org www.cjdfoundation.org

CJDF Questionnaire Update

With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at help@cjdfoundation.org or 1-800-659-1991. We greatly appreciate your help with this important project!

CJD QUESTIONNAIRE HISTORY

http://cjdquestionnaire.blogspot.com/

Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website

http://www.neuroprion.com/en/patients-events-07-6th-CJD-Conf.html

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

snip...

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. ...

snip...

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

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

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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

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/

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

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

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

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

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

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

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

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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 ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

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

Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html

***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

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html

SEAC 99th meeting on Friday 14th December 2007

snip...

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

snip...

http://www.seac.gov.uk/minutes/99.pdf

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip... see full text ;

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html

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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

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

http://seac992007.blogspot.com/

snip...

http://www.seac.gov.uk/minutes/99.pdf

TSS

SEAC Draft minutes of the 100th meeting held on 25th April 2008

2008-07-13T16:28:00.000-07:00

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

ITEM 2 – APPROVAL OF MINUTES FROM SEAC 99 (SEAC 100/1) 6. The minutes of SEAC 99 were agreed as a correct record with the following amendment: 4 © SEAC 2008 • Paragraph 7, second bullet point, change “…Institute of Animal Health (IAH).” to “…Roslin Institute (RI).” 7. The committee was updated about transmission studies using isolates from a case of Creutzfeldt-Jakob Disease (CJD)1 which had been discussed at SEAC 99. Two lines of transgenic mice expressing the human prion protein gene homozygous for methionine (MM) or valine (VV) at codon 129 and two lines of conventional mice had been inoculated with isolates from the case. Transmission has been more efficient to the humanised mice compared with the conventional mice. The molecular characteristics of the abnormal prion protein (PrPSc) altered on transmission suggesting the patient may have been infected with an unstable TSE strain. Further work was required to characterise the TSE strain. However, it would be difficult to confirm whether this was related to BSE infection unless additional similar cases arose which could be investigated. 8.

snip...

35. Members asked about the spread of chronic wasting disease (CWD) in the USA. Mr Burke replied that CWD was continuing to spread in the cervid population in the USA.

snip...

One sheep in Cyprus, two in the UK and four from France are under investigation for possible BSE infection. Two further BSE cases were identified with unusual transmission properties in a Defra research project (SE1849) and 5 Countries where cases of BSE in cattle were reported in 2007: UK, Austria, Canada, Czech Republic, France, Germany, Hungary, Ireland, Italy, Japan, Poland, Portugal, Slovenia, Slovakia, Spain and The Netherlands. 6 Countries where atypical scrapie in sheep has been reported since 2002: Belgium, Denmark, the Falkland Islands, Finland, France, Germany, Greece, Hungary, Ireland, Italy, The Netherlands, Norway, Portugal, Slovenia, Spain, Sweden, UK and USA. 13 © SEAC 2008 are also subject to further characterisation. BSE has been confirmed in one French goat slaughtered in 2002. A UK goat which was originally diagnosed as a case of scrapie in 1990 is under investigation for possible BSE infection. 37.

snip...

40. Members agreed that the statement should not focus entirely on the risk from BSE. A paragraph should be included to describe the risk that a new zoonotic TSE strain might emerge as a result of the relaxations to TSE controls. The possibility that the transmissibility to humans of such a new strain could be much greater than that of BSE should be acknowledged.

http://www.seac.gov.uk/minutes/final100.pdf

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

snip...

http://www.seac.gov.uk/minutes/99.pdf

>>There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. <<<

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

snip... see full text ;

http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

Thursday, July 10, 2008

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

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008

A New Prionopathy update July 10, 2008

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

Communicated by: Terry S. Singeltary Sr. <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:flounder9@verizon.net>

[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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

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

Rule out of variant CJD as the cause of death of a Virginia resident

Recently, the National Prion Disease Pathology Surveillance Center (NPDPSC) ruled out the presence of variant CJD (vCJD) as the cause of death of a young Virginia woman who died earlier this year. Although this suspected case received international media attention, NPDPSC determined that the cause of death was not due to vCJD.

As of June 2008, the total number of vCJD cases identified in residents of the United States is three; all of which were epidemiologically linked to likely exposures to cattle products contaminated with bovine spongiform encephalopathy (BSE, commonly known as "mad cow disease")while residing in the United Kingdom (2 cases) or Saudi Arabia (1 case).

The NPDPSC was established by the Centers for Disease Control and Prevention in collaboration with the American Association of Neuropathologists for the purpose of enhancing national surveillance of human prion diseases such as classic CJD and vCJD. US physicians are encouraged to utilize the diagnostic services of the NPDPSC to confirm all clinically suspected and diagnosed cases of CJD and vCJD. For additional information about the NPDPSC and how to submit diagnostic specimens, consult www.CJDSurveillance.com.

http://www.cdc.gov/ncidod/dvrd/vcjd/other/News_06122008.htm

DOES anyone know if a final diagnosis was ever made ???

IT would seem that IF it was important enough for the USDA et al to first announce this at a news conference about beef trade with Korea i.e. not nvCJD, they could at least announce whether or not it was some form of sporadic CJD or any other TSE. IF they cannot because of the infamous 'confidentiality' claus, then that should have applied to announcing the negative on the nvCJD announcement as well, ya think ???

please see full text ;

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html

Wednesday, July 9, 2008

[Docket No. FDA-2008-N-0369] Ruminant Feed Ban Support Project; ``Response to RFA-FDA-08-008''

http://madcowfeed.blogspot.com/2008/07/docket-no-fda-2008-n-0369-ruminant-feed.html

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***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

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

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

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

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

Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;

***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

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.

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

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

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

In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.

snip...

see full report here ;

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

Thursday, April 24, 2008

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E. OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No. 00-072-1]

http://foiamadsheepmadrivervalley.blogspot.com/2008/04/re-foia-of-declaration-of-extraordinary.html

Chronic Wasting Disease

8. Human susceptibility to CWD

Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle [2]. Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades [51]. Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,

8

*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al. [68] showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. [96], although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains [79]. To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days [41]. In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk [84]. However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indi rect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission [49]. Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans [72], and therefore may not indicate that human prion conversion would occur by CWD.

snip...

11. Disease control challenges posed by CWD

Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control [91]. Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease [50]. An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection [55]. Modelling studies have provided further

10

support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions [75]. Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.

12. Conclusion

CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.

Acknowledgements

I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.

snip...see full text 19 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v08092.pdf

http://chronic-wasting-disease.blogspot.com/

Wednesday, June 18, 2008 CHRONIC WASTING DISEASE FOUND IN 24 MORE DEER IN ALBERTA

http://chronic-wasting-disease.blogspot.com/2008/06/chronic-wasting-disease-found-in-24.html

Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008

1: Vet Res. 2008 Apr 3;39(4):41

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/

PLEASE NOTE THE PARTIAL AND VOLUNTARY MAD COW FEED BAN OF AUGUST 4, 1997 nothing more than ink on paper ... TSS

Wednesday, April 23, 2008

FDA Strengthens Safeguards for Consumers of Beef Issues Regulation on Animal Feeds with Added Safeguards Against BSE

http://madcowfeed.blogspot.com/

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

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

----- Original Message -----

From: "Terry S. Singeltary Sr." mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:flounder9@verizon.net To: "Bovine Spongiform Encephalopathy" mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:BSE-L@aegee.org Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:heggem.daniel@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:sibert.christopher@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:denne.jane@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:hazen.susan@epa.gov; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:mcrosby@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:erobinson@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:enegin@ucsusa.org; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:cjdvoice@yahoogroups.com; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:madcow@lists.iatp.org

Sent: Monday, April 28, 2008 9:48 PM

Subject: Interference at the EPA Science and Politics at the U.S. Environmental Protection Agency

Reports and Research

Interference at the EPA

Science and Politics at the U.S. Environmental Protection Agency

The U.S. Environmental Protection Agency (EPA) has the simple yet profound charge "to protect human health and the environment." EPA scientists apply their expertise to protect the public from air and water pollution, clean up hazardous waste, and study emerging threats such as global warming. Because each year brings new and potentially toxic chemicals into our homes and workplaces, because air pollution still threatens our public health, and because environmental challenges are becoming more complex and global, a strong and capable EPA is more important than ever.

Yet challenges from industry lobbyists and some political leaders to the agency's decisions have too often led to the suppression and distortion of the scientific findings underlying those decisions—to the detriment of both science and the health of our nation. While every regulatory agency must balance scientific findings with other considerations, policy makers need access to the highest-quality scientific information to make fully informed decisions.

Concern over this problem led the Union of Concerned Scientists (UCS) to investigate political interference in science at the EPA. The investigation combines dozens of interviews with current and former EPA staff, analysis of government documents, more than 1,600 responses to a survey sent to current EPA scientists, and written comments from EPA scientists.

The results of these investigations show an agency under siege from political pressures. On numerous issues—ranging from mercury pollution to groundwater contamination to climate change—political appointees have edited scientific documents, manipulated scientific assessments, and generally sought to undermine the science behind dozens of EPA regulations. ...

snip...please see full text ;

http://sciencebushwhacked.blogspot.com/

Wednesday, April 30, 2008

Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings

http://cjdmadcowbaseoct2007.blogspot.com/2008/04/consumption-of-beef-tongue-human-bse.html

SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS

http://cjdmadcowbaseoct2007.blogspot.com/2008/04/srm-mad-cow-recall-406-thousand-pounds.html

2006 was a banner year too for mad cow protein. those were just one of many ;

Specified Risk Materials

http://madcowspontaneousnot.blogspot.com/2008/02/specified-risk-materials-srm.html

Thu Dec 6, 2007 11:38

FDA IN CRISIS MODE, AMERICAN LIVES AT RISK

http://www.cidrap.umn.edu/cidrap/content/fs/food-disease/news/dec0407fda.html

FDA SCIENCE AND MISSION AT RISK

http://www.fda.gov/ohrms/dockets/ac/07/briefing/2007-4329b_02_01_FDA%20Report%20on%20Science%20and%20Technology.pdf

THE only fool is one who fools himself, and GW and his administration and their junk science will fool humans for just so long, then the incubation will catch up. none of this was about science, it was all about commodities and futures and the exporting of beef. nothing else mattered, literally, just ask old stanley prusiner the nobel prize winner for the PRION, what did old stan say ;

STANLEY PRUSINER NOBEL PEACE PRIZE WINNER ON THE PRION

US AG SEC AND LAYCRAFT

“nothing matters, except beef from Canada under 30 months bone in beef product, that’s ALL THAT MATTERS!”

US SENATOR AND STAN THE MAN SLAM USDA ”DAMNING TESTIMONY”

Senator Michael Machado from California

”USDA does not know what’s going on”. ”USDA is protecting the industry”. ” SHOULD the state of California step in”

Stanley Prusiner

”nobody has ever ask us to comment”

”they don’t want us to comment”

”they never ask”

i tried to see Venemon, after Canadian cow was discovered with BSE. went to see lyle. after talking with him…

absolute ignorance…

then thought i should see Venemon…

it was clear his entire policy was to get cattle boneless beef prods across the border…

nothing else mattered…

his aids confirmed this…

5 times i tried to see Venemon, never worked…

eventually met with carl rove the political…

he is the one that arranged meeting with Venemon…

just trying to give you a sense of the distance…

threat to health public safety…

was never contacted…

yes i believe that prions are bad to eat and you can die from them…END

Dr. Stan bashing Ann Veneman - 3 minutes - Damning testimony

http://maddeer.org/video/embedded/08snip.ram

File Name: USDA DON'T ASK DON'T TELL POLICY 02snip.rpm

DAMNING testimony of consumer consumption of Washington mad cow in California

http://www.maddeer.org/video/embedded/02snip.rm

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

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."

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125

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

Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.

USDA/OIG-A/50601-10-KC Page 27

observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.

snip...

http://www.usda.gov/oig/webdocs/50601-10-KC.pdf

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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

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:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto: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/

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

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

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

2 January 2000

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

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

15 November 1999

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

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

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.

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

APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8

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

9/13/2005

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

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

Saturday, March 22, 2008

10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures

http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html

re-Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease

http://betaamyloidcjd.blogspot.com/2008/04/re-association-between-deposition-of.html

full text ;

Friday, June 20, 2008

USDA TO KOREA AND THE WORLD, EAT THAT AND LIKE IT

http://usdavskorea.blogspot.com/2008/06/usda-to-korea-and-world-eat-that-and.html

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-2006-0041-0006 Public Submission Title Comment from Terry S Singletary Sr Views Add Comments How To Comment

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

http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&d=APHIS-2006-0041-0006

ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)

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 blood 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. ...

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

snip... 48 pages...

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8

PEACE

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

SEAC 2008 ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

2008-06-04T14:57:00.000-07:00

SEAC 2008

ONE HUNDREDTH MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 100th meeting in London on 25th April 2008, and discussed the following:

CURRENT ISSUES

SEAC was informed about:

• Two recently identified cases of variant Creutzfeldt-Jakob Disease (vCJD) in Spain. • Proposals for the future regulation of ‘high street’ dentistry outlined in a consultation issued recently by the Department of Health (DH)1. SEAC agreed to respond to the consultation, welcoming the proposals. • The detection of material of unknown animal origin in a batch of wheat feed distributed for use in livestock feed2.

ASSESSMENT OF THE PREVALENCE OF SUBCLINICAL vCJD SEAC was updated about the progress of the National Anonymous Tonsil Archive (NATA) and of discussions around a proposed post mortem tissue archive. These would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease). Approximately 55 000 NATA samples had been screened by the end of March 2008. Although none was positive for abnormal prion protein (PrPvCJD), some testing remains to be done on some samples. SEAC expressed disappointment that it is currently

1 DH (2008) The future regulation of health and adult social care in England: A consultation on the framework for the registration of health and adult social care providers.

http://www.dh.gov.uk/en/Consultations/Liveconsultations/DH_083625

2

http://www.food.gov.uk/news/newsarchive/2008/apr/feedcontam

2 © SEAC 2008

proving difficult to establish a post mortem tissue archive through collection of tissues from Coroners’ autopsies. In light of the current difficulties in establishing a post mortem tissue archive, DH asked for advice about how existing data from NATA might be combined with a completed survey of appendix samples3, which had found PrPvCJD in three out of about 11 000 samples. SEAC considered that the data from these studies are not, at the present time, discrepant. However, given the uncertainties about the tissue distribution of PrPvCJD during vCJD incubation, it would be hard to see how these data could be combined.

SEAC considered what further work might be done to obtain better estimates for the prevalence of subclinical vCJD including additional appendix studies and a post mortem tissue archive. SEAC strongly recommended that every avenue be pursued to establish such an archive.

The committee agreed to produce a statement.

UPDATE ON ANIMAL TSEs

SEAC was updated on transmissible spongiform encephalopathies (TSEs) in animals in the UK and elsewhere.

In the UK, the Bovine Spongiform Encephalopathy (BSE) epidemic in cattle peaked in 1992, with over 37 000 confirmed cases but has since declined with 67 cases confirmed in 2007. By the end of 2007, there had been 178 BSE cases confirmed in the UK in cattle born after the introduction of the reinforced feed ban in 1996. Two unusual (H-type) BSE cases have been found in the UK. Relatively low numbers of BSE cases have also been found in the other European Union (EU) countries and elsewhere.

No TSEs have been found in a relatively small EU survey of deer. There is no evidence for the presence of BSE from surveillance of UK sheep. Classical scrapie case numbers continue to decline in the UK with 31 cases confirmed in 2007. Small numbers of

3 Hilton et al. (2004) Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol. 203, 733-739.

3 © SEAC 2008

atypical scrapie cases continue to be found with a total of 194 cases confirmed in the UK since surveillance for atypical scrapie began in 2002. Classical and atypical scrapie continue to be found in EU and other countries.

CONSIDERATION OF OPTIONS FOR RELAXATION OF THE TOTAL FEED BAN

At SEAC 99, Rural Affairs Departments and the Food Standards Agency (FSA) asked SEAC to assess the possible consequences of various options for relaxing the total feed ban. These included the introduction of tolerance levels for certain types of processed animal protein (PAP) in feed, the inclusion of fish meal in young ruminant diets and the feeding of non-ruminant PAP to nonruminants of a different species. Following that meeting, a draft statement was prepared based on the discussions.

SEAC discussed the draft statement and agreed modifications. Once the statement is finalised, it will be published on the SEAC website.

PROPOSALS TO REDUCE TESTING OF CATTLE SLAUGHTERED FOR FOOD – IMPACT ON RISK TO HUMAN HEALTH

FSA asked SEAC to consider an analysis of the human health risk of a range of options for altering the BSE surveillance programme by increasing the minimum age at which healthy slaughtered and fallen stock cattle must be tested for BSE. A BSE risk model constructed by the Veterinary Laboratories Agency, that had been previously reviewed and accepted by SEAC, was used for the analysis.

SEAC noted that the increased risks calculated by the model arising as a result of raising the age at which cattle are tested for BSE are very small. These calculations are subject to uncertainties, particularly in relation to assumptions made about infectivity in tissues and the BSE epidemic. The committee asked for further information about how well outputs from the model fit actual surveillance data. SEAC suggested that future modelling could examine the effect of changing controls on effectiveness of one control. SEAC noted that BSE testing of cattle provides

4 © SEAC 2008

important data on the incidence of the disease and confers some public health protection.

HORIZON SCANNING

SEAC considered issues that might emerge on the TSE science horizon. It noted that significant progress continues to be made to understand TSEs better and to develop effective TSE control policies. SEAC considered areas of TSE science important for the future could include the:

• nature of the TSE carrier state • genetic factors that modulate susceptibility to TSEs • molecular basis of TSE strains and the relationship with host genetics • potential for animal TSEs to transmit to humans • development of ante mortem diagnostic tests • proportionality of TSE controls

http://www.seac.gov.uk/summaries/seac100_summary.pdf

The finalised minutes of the 99th meeting

http://www.seac.gov.uk/minutes/99.pdf

Thursday, January 31, 2008

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th meeting held on 14th December 2007

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION

40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base

13 © SEAC 2007

cases in Texas and Alabama, with both scrapie and chronic wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?”

41. A member considered that this question ............

http://www.seac.gov.uk/minutes/99.pdf

snip... please see full text, sources, and comments here ;

http://seac992007.blogspot.com/2008/01/spongiform-encephalopathy-advisory.html

FC5.3 Assessing the Risk of vCJD Transmission by Dentistry; Distribution of Infectivity in Oral Tissues of VM Mice after Simulated Oral Feeding of BSE-301V

Sutton, JM1; Kirby, E1; Dickinson, J1; Dennis, M1; Cornwall, M1; Vassey, MJ1; Smith, A2; Marsh, PD3; Walker, JT1; Raven, NDH1 1Health Protection Agency, Centre for Emergency Preparedness and Response,, TSE Research group, UK; 2University of Glasgow, Dental School, UK; 3Health Protection Agency, Centre for Emergency Preparedness and Response,, UK

Background: Ongoing concerns about the prevalence of variant Creutzfeldt Jakob Disease (vCJD) in the UK population has heightened concerns about the risks of iatrogenic transmission of the disease. Although there have been no cases to date of transmission by surgery there have been 4 cases involving blood transfusion. This study aims to assess the potential of transmission of the disease by dental procedures. Whilst the risks are undoubtably low the very large numbers of procedures carried out annually have the potential to amplify the risks considerably and there is very little data in this area to form the basis for accurate risk assessments. Aim(s)/Objective(s): To assess the relative levels of infectivity in oral tissues from a murine model following exposure to BSE-301V through the small intestine. Methods. The study uses a BSE-301V, VM mouse model as a clinically relevant model for assessing iatrogenic vCJD transmission between humans. Infectious mouse brain homogenate was prepared and inoculated into a loop of the duodenum, to prevent direct contamination of the oral tissues. Mice were sacrificed at 3-weekly intervals and at appearance of clinical symptoms. A range of oral tissues, including dental pulp, gingival margin, salivary gland, saliva, lingual tonsil and trigeminal ganglia, together with brain and spleen tissues were removed, processed as homogenates and reinoculated intracranially (ic.) into indicator mice. Results: The primary challenge proved to be a very efficient route of infection with a 100% attack rate and a mean incubation to clinical disease of 157 ± 17 days (compared to 120 days for the same titre inoculum ic.). Infectivity was observed in all oral and control tissues with varying time-courses and titres estimated from incubation period. Discussion: The results throw new light on the potential routes of dissemination and spread of infectivity from the small intestine to the oral cavity and its implications for possible iatrogenic transmission of vCJD via dental, endoscopic or other forms of surgery. Conclusion: The data generated from the study provides support for ongoing risk assessments to look at the potential for vCJD transmission via dental procedures alongside other elements of studies looking at effectiveness of decontamination and re-use of dental instruments.

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

Monday, December 31, 2007

Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

Subject: CJD: update for dental staff Date: November 12, 2006 at 3:25 pm PST

1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

CJD: update for dental staff.

Scully C, Smith AJ, Bagg J. Eastman Dental Institute, University of London.

It is almost a decade since the recognition of the emergence of a new infectious disease termed variant Creutzfeldt-Jakob disease (vCJD) caused by prions (PrPTSE), abnormal variants of a normal human cell surface protein (PrP).This disease has a number of similarities to other forms of CJD--lethal disorders characterized by a prolonged incubation period, and progressive mental deterioration. In relation to oral tissues, PrPTSE have been found in neural, gingival, pulpal, lingual, lymphoreticular and salivary gland tissue in animal models. In both sporadic and variant CJD, PrPTSE is detectable in the trigeminal ganglion and, in vCJD, in lymphoreticular tissues, but infectivity has not been tested in other human oral tissues. CLINICAL RELEVANCE: PrPTSE is much more resistant to the common methods of inactivation than conventional pathogens, and it adheres avidly to steel whilst retaining its infectivity. Particular attention must be paid to cleaning and sterilizing re-usable dental instruments. Single-use devices, such as endodontic files and matrix bands, must never be re-used. Advice on the reprocessing of dental instruments used on known CJD patients must be obtained from local infection control teams. Research into effective methods of prion inactivation appears promising, although further work on the applicability to general dental practice is required.

PMID: 17087448 [PubMed - in process]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17087448&query_hl=1&itool=pubmed_docsum

Subject: PrPSc in salivary glands of scrapie-affected sheep Date: February 15, 2007 at 9:33 am PST

J. Virol. doi:10.1128/JVI.02148-06 Copyright (c) 2007, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.

PrPSc in salivary glands of scrapie-affected sheep

Marta Vascellari*, Romolo Nonno, Franco Mutinelli, Michela Bigolaro, Michele Angelo Di Bari, Erica Melchiotti, Stefano Marcon, Claudia D'Agostino, Gabriele Vaccari, Michela Conte, Luigi De Grossi, Francesca Rosone, Francesco Giordani, and Umberto Agrimi Istituto Zooprofilattico Sperimentale delle Venezie, Histopathology Department, Viale dell'Università 10, 35020 Legnaro (PD), Italy; Istituto Superiore di Sanità, Department of Food Safety and Animal Health, Viale Regina Elena 299, 00161 Roma, Italy; Istituto Zooprofilattico Sperimentale delle Regioni Lazio e Toscana, Strada Terme, 01100 Viterbo, Italy

* To whom correspondence should be addressed. Email: mvascellari@izsvenezie.it .

Abstract

The salivary glands of scrapie-affected sheep and healthy controls were investigated for the presence of the pathological prion protein (PrPSc). PrPSc was detected in major (parotid and mandibular) and minor (buccal, labial and palatine) salivary glands of naturally and experimentally infected sheep. By western blot, PrPSc concentration in glands was estimated as 0.02-0.005% of brain. Immunohistochemistry revealed intracellular deposition of PrPSc in ductal and acinar epithelium and occasional labeling into the lumen of salivary ducts. The presence of PrPSc in salivary glands highlights the possible role of saliva in the horizontal transmission of scrapie.

http://jvi.asm.org/cgi/content/abstract/JVI.02148-06v1?papetoc

CJD TEXAS

http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html

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.

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

The Italian cases (11 and 15 years of age) originally named bovine amyloidotic spongiform encephalopathy (BASE) were characterized by an unglycosylated protein band with a lower molecular mass (thus named L cases) and the predominance of the monoglycosylated band. In addition, immunohistochemical detection of PrPres in these cases found greater deposits in the cerebral cortex and thalamus versus the brain stem. The French cases found a higher molecular mass associated with the unglycosylated protein band and were called H cases (see figure 1). *** The different "strains" are now called atypical BSE. ...

full text, skroll down to page 6 ;

http://www.usaha.org/committees/reports/2006/report-fe-2006.pdf

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE (the last two cases of mad cow disease in the USA were in Alabama, and Texas, both of which were atypical BSE).

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***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

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45

WHY were they planning to destroy all CJD tissue samples donated ???

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). ...snip...end...tss

http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm

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.

snip...

May 10, 2005

The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150 Dallas, 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 the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years.

I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded 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.) ...snip...end...tss

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

NIH says it will preserve CJD brains

Published: May 31, 2005 at 5:26 PM

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

© 2005 United Press International. All Rights Reserved. This material may not be reproduced, redistributed, or manipulated in any form.

http://www.upi.com/NewsTrack/Science/2005/05/31/nih_says_it_will_preserve_cjd_brains/6771/print_view/

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

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."

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125

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

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;

***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

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.

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

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

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

Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

Thursday, April 03, 2008 A prion disease of cervids: Chronic wasting disease 2008

1: Vet Res. 2008 Apr 3;39(4):41

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/

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]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,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.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

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

Journal of American Medical Association

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

http://jama.ama-assn.org/

2 January 2000 British Medical Journal

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

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

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

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

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 b y 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

www.cjdsupport.net
Newsletter issue 17 april 2008

the importance of gene types

We have known for many years that a common variation in a gene, known as the prion protein gene, is very important in determining the risk of developing prion diseases and how long it takes for the disease to develop when someone becomes infected. There are three genetic types in the UK population known as MM, VV and MV. So far, vCJD has only affected people with MM genetic type. Around 40% of healthy people in the UK are MM, about 50% are MV and around 10% are VV. It is likely that BSE prions will infect people of the VV and MV types also, but they may have much longer incubation periods (the time taken from being infected with prions until the brain disease becomes apparent) and may also develop a pattern of disease which may be different to vCJD. We suspect this again as result of research in laboratory mice wher e those that had the VV and MV genes had a different type of disease and different types or ‘strains’ of prions developed.

snip...

This unusual finding reminds us of the importance of keeping alert to the possibility that BSE prions will cause disease in individuals with different genetic types, who may develop a disease that may resemble sporadic CJD, or vCJD, or have a new pattern of disease.

Prion disease and gene types

Dr Simon Mead and Professor John Collinge, NHS National Prion Clinic

Reference: Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman Simon Mead; Susan Joiner; Melanie Desbruslais; Jonathan A. Beck; Michael O’ Donoghue; Peter Lantos; Jonathan D. F. Wadsworth; John Collinge Arch Neurol. 2007;64(12):1780-1784.

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

CJD QUESTIONNAIRE

http://cjdquestionnaire.blogspot.com/

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/

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

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