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.
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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/
Saturday, February 27, 2010
Thursday, February 4, 2010
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009
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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.
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
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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:
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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
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
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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:
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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
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