SEAC Agenda 104th meeting on Friday 5th March 2010

SEAC SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE

Agenda 104th meeting on Friday 5th March 2010

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

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

snip...

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

snip...

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.

snip...

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.

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.

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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.

snip...

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.

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



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/

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

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SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

snip...

ITEM 3 – CURRENT ISSUES

9.

SEAC was informed about the following issues:

• Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.

http://www.seac.gov.uk/papers/101-1.pdf

3 10:10 Current issues

vCJD cluster in Spain Testing of a goat

snip...

In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.

* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.

http://www.seac.gov.uk/agenda/agen151008.htm

The CJD Foundation Newsletter

http://www.cjdfoundation.org/content/newsletters/september2008.pdf

snip...

SEPTEMBER 2008 VOLUME 1, ISSUE 3

The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.

Note 1: Not all new cases and deaths reported are confirmed by autopsy.

Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..

2008

MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS

January 35 24 203 6,424 February 24 9 212 6,848 March 19 19 164 7,492 April 36 14 231 8,427 May 26 20 191 8,839 June 19 17 144 9,646 July 28 14 171 7,791 August 27 16 150 5,323

TOTALS = 214 133 1,466 60,790

P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? help@cjdfoundation.org www.cjdfoundation.org

CJDF Questionnaire Update

With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at help@cjdfoundation.org or 1-800-659-1991. We greatly appreciate your help with this important project!

CJD QUESTIONNAIRE HISTORY

http://cjdquestionnaire.blogspot.com/

Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website

http://www.neuroprion.com/en/patients-events-07-6th-CJD-Conf.html

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

snip...

Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html

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

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

Communicated by: Terry S. Singeltary Sr.

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

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

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

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

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

sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/

JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535

THE PATHOLOGICAL PROTEIN

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

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

Terry S. Singeltary, Sr Bacliff, Tex

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

http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

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

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117

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

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406

Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html

http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html

http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html

USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/

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

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

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf

please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html

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

Progress Report from the National Prion Disease Pathology Surveillance Center

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

April 3, 2008

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

Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html

Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

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

SEAC 99th meeting on Friday 14th December 2007

snip...

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

snip...

ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.

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

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

snip...

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

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

10 people killed by new CJD-like disease

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

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

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

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

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

snip... see full text ;

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

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html

Communicated by: Terry S. Singeltary Sr.

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

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

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

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

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

2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html

http://seac992007.blogspot.com/

snip...

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


TSS