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From: TSS (wt-d6-183.wt.net)
Subject: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT]
Date: November 13, 2004 at 2:35 pm PST

In Reply to: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT] posted by TSS on September 10, 2003 at 1:43 pm:

Re: Reply to Singletary
Terry S. Singeltary Sr.,
CJD WATCH
P.O. Box 42,
none

Send Correspondence to journal:
Re: Re: Reply to Singletary

Email Terry S. Singeltary Sr., et al.

Singeltary Reply to Dr. Maddox, Belay, Schonberger et al 11/13/04;

Greetings Dr. Maddox, Belay, Schonberger et al;

IN your reply to me on 26 March 2003, Dr. Maddox, Belay, Schonberger et al write;

Mr. Singletary raises several issues related to current Creutzfeldt- Jakob disease (CJD) surveillance activities. Although CJD is not a notifiable disease in most states, its unique characteristics, particularly its invariably fatal outcome within usually a year of onset, make routine mortality surveillance a useful surrogate for ongoing CJD surveillance.[1]

I kindly wish to submit the following to dispute this;

Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will

snip...

IDENTIFICATION OF CASES

Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

full text;

http://www.bseinquiry.gov.uk/files/yb/1989/05/00005001.pdf

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

IN your reply to me on 26 March 2003, Dr. Maddox, Belay, Schonberger et al write;

[2] but only limited data seeking such evidence exist. Overall, the previously published case-control studies that have evaluated environmental sources of infection for sporadic CJD have not consistently identified strong evidence for a common risk factor.

I kindly wish to submit the following to dispute this;

11 November 2004

Genetic make-up may determine what type of CJD occurs when humans are infected with BSE

New research published by a team from the Medical Research Council (MRC) Prion Unit offers an explanation about why only people with a particular genetic make-up have so far developed vCJD. It also provides evidence that other types of BSE-derived prion infection with a different pattern of symptoms might occur in humans. The findings are published in the journal Science.

Variant CJD (vCJD) is the human disease thought to be caused by eating food contaminated with the infectious agent, known as a prion, responsible for the epidemic of BSE or “mad cow disease” in cattle. So far, everyone known to have developed vCJD has been of a particular genetic type – known as MM. Until now it has been a mystery why everyone that has developed vCJD is of the MM type and one possibility is that they are simply the first to develop the disease when infected with BSE, and that people with the other genetic types1 (known as VV and MV*) infected with BSE prions will also develop vCJD, but some years later.

In a series of experiments spanning more than ten years, the MRC team has been studying mice genetically modified so that they make human prion proteins – which are used to model human susceptibility to BSE. The team has now shown that mice with the human VV genetic type do become infected when given BSE or vCJD prions, but manifest a different form of the disease which looks quite different to vCJD and has a novel prion “strain” type.

Remarkably, when these novel prions were used to infect mice of the MM genetic type, the mice either developed a disease very like vCJD, or else a pattern of disease that looks like so-called sporadic CJD – the “classical” form of CJD. This form has been known about for many years, is seen all over the world and has not hitherto been associated with BSE. However, the new strain identified in the mice, being called ‘type 5’, has not been seen yet in people and we do not know what pattern of disease it would cause. It could look like one of the forms of classical or sporadic CJD or perhaps be yet another different “variant” form.

The work from the MRC team suggests that type 4 prions, the type associated with vCJD, can only propagate themselves in people that make the M form of the protein. It seems the V form of the protein just cannot adopt the particular molecular shape that characterises type 4.

The studies in mice also suggest that if these prions were to pass from person to person (for example by blood transfusion) then, depending on the genetic type of the person becoming infected, at least three different patterns of disease might result: type 2 (which is seen in sporadic CJD); type 4 (which causes vCJD) or type 5 (which may cause a new pattern of disease).

Professor John Collinge, Director of the MRC Prion Unit, which is based at University College London, said: “These mouse studies give us vital clues about the behaviour of prions and how they appear to modify and adapt depending on the genetic makeup of the individual they are infecting.

“We always have to be cautious about making direct comparison to the human condition, but our work strongly suggests that we can not assume only those with one genetic profile are vulnerable to BSE infection.

“At this stage it is not possible to say how this should alter estimates of those likely to become ill, but our findings do suggest we should be taking steps to draw up a more sophisticated system of categorizing the disease so that we don’t mistake BSE related infection for a version of sporadic CJD.”

For more information call the MRC press office on 020 7 637 6011

Notes to Editors

*The human prion protein comes in two common forms, known as M and V. Because everyone has two copies of this gene, there are three possible genetic types: MM, MV and VV.

Paper - Human Prion protein v129 prevent expression of vCJD phenotype – Science On line 11.11.04

Prions are rogue forms of one of the body’s own proteins – known as the prion protein – which are misshapen. There are several different rogue or misshapen forms that can infect humans, and these different types of prions are known as “strains”. This is analogous to different strains of other germs such ‘flu virus causing influenza or strains of salmonella causing different forms of food poisoning for example.

The strain of prion causing vCJD is known as type 4, types 1-3 cause the different forms of sporadic or classical CJD. Each strain causes a different pattern or type of disease. It is known that prion strains can change or “mutate” when they pass between different animals.

The Medical Research Council (MRC) is a national organisation funded by the UK tax-payer. Its business is medical research aimed at improving human health; everyone stands to benefit from the outputs. The research it supports and the scientists it trains meet the needs of the health services, the pharmaceutical and other health-related industries and the academic world. MRC has funded work which has led to some of the most significant discoveries and achievements in medicine in the UK. About half of the MRC’s expenditure of £430 million is invested in its 40 Institutes, Units and Centres. The remaining half goes in the form of grant support and training awards to individuals and teams in universities and medical schools.

©2004 Medical Research Council http://www.mrc.ac.uk/public-11_november_2004

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 1 Corresponding author e-mail: j.collinge@prion.ucl.ac.uk

Received August 1, 2002; revised September 24, 2002; accepted October 17, 2002

Abstract

Variant CreutzfeldtJakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure...

http://embojournal.npgjournals.com/cgi/content/full/21/23/6358

THE new findings of BASE in cattle in Italy of Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease

http://www.pnas.org/cgi/content/abstract/0305777101v1

Adaptation of the bovine spongiform encephalopathy agent to primates

and comparison with Creutzfeldt- Jakob disease: Implications for

human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate;

http://www.pnas.org/cgi/content/full/041490898v1

Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice

Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Jennifer Buckell, Sebastian Brandner, Jonathan D. F. Wadsworth and John Collinge

Correspondence John Collinge j.collinge@prion.ucl.ac.uk

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, London WC1N 3BG, UK Received 9 December 2003 Accepted 27 April 2004

Distinct prion strains can be distinguished by differences in incubation period, neuropathology and biochemical properties of disease-associated prion protein (PrPSc) in inoculated mice. Reliable comparisons of mouse prion strain properties can only be achieved after passage in genetically identical mice, as host prion protein sequence and genetic background are known to modulate prion disease phenotypes. While multiple prion strains have been identified in sheep scrapie and CreutzfeldtJakob disease, bovine spongiform encephalopathy (BSE) is thought to be caused by a single prion strain. Primary passage of BSE prions to different lines of inbred mice resulted in the propagation of two distinct PrPSc types, suggesting that two prion strains may have been isolated. To investigate this further, these isolates were subpassaged in a single line of inbred mice (SJL) and it was confirmed that two distinct prion strains had been identified. MRC1 was characterized by a short incubation time (110±3 days), a mono-glycosylated-dominant PrPSc type and a generalized diffuse pattern of PrP-immunoreactive deposits, while MRC2 displayed a much longer incubation time (155±1 days), a di-glycosylated-dominant PrPSc type and a distinct pattern of PrP- immunoreactive deposits and neuronal loss. These data indicate a crucial involvement of the host genome in modulating prion strain selection and propagation in mice. It is possible that multiple disease phenotypes may also be possible in BSE prion infection in humans and other animals.

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471

THE recent discoveries of previously unidentified strains of Scrapie such as 221C44 and the Nor9845;

FULL TEXT APPRX. 91 PAGES

UK Strategy for Research and Development on Human and Animal Health Aspects of Transmissible Spongiform Encephalopathies

2004-2007

http://www.mrc.ac.uk/pdf-uk_strategy_v5.2.pdf

IP/04/1324

Brussels, 28 October 2004

Commission submits French Research Findings on TSE in a goat to Expert

Panel

Following the findings by a research group in France that they suspect the presence of a TSE infection in a goats brain which tests cannot distinguish from BSE, the European Commission has submitted data received from the French authorities to the Community Reference Laboratory (CRL) for TSEs based in Weybridge, England, for an evaluation by an expert panel. TSEs are transmissible spongiform encephalopathies, namely BSE affecting cattle, and scrapie affecting goats and sheep. The expert panel will evaluate, over the next two weeks or so, the scientific evidence to see if it indicates the presence of BSE in the goat. This isolated incident does not present a risk to public health as the goat and its herd did not enter the food and feed chain.

snip...

http://europa.eu.int/rapid/pressReleasesAction.do?reference=IP/04/1324&format=HTML&aged=0&language=EN&guiLanguage=en

According to Nov. 2 Yomiuri Newspaper, researchers of the Prion Disease Research Center, the National Institute of Animal Health of Japan reported in the International Symposium of Prion Diseases held in Sendai from October 31 to November 2., 2004, that they detected prion in the adrenal gland and peripheral (sciatic and peroneal) nerves of the 11th BSE case of Japan (a 94-months old cow found dead on the farm on March 4 this year).

http://www.maff.go.jp/www/press/cont2/20041101press_7.htm (only in Japanese)

Sendai and the International Symposium of Prion Diseases held here from October 31 to November 2.,2004

Abstract

ORAL 8

Bovine spongiform encephalopathy (BSE) in Japan

Takashi Yokoyama, Kumiko M. Kimura, Morikazu Shinagawa Prion Disease Research Center, National Institute of Animal Health, Japan

Bovine spongiform encephalopathy (BSE) has become an important problem not only for animal industry, but also for public health. In Japan, BSE was first recognized in September 2001 by fallen stock surveillance. Since October 2001, BSE examination for all cattle slaughtered at abattoirs has started. In April 2004, all dead cattle examination (over 24 months) has been conducted at livestock hygiene service center. Samples positive in enzyme linked immunosorbent assay (ELISA) are further subjected to western blot (WB) and immunohistochemistry (IHC). Thirteen BSE cases have been reported by September 2004. Twelve cases were classified as typical BSE, and the remained one was an atypical BSE. Variant forms of BSE with atypical histopathological and/or biochemical phenotype were reported in Italy and France. Further study is required for BSE prion characteristics. To characterize BSE prion properties, brain homogenates of Japanese BSE cases were intracerebrally inoculated into wild-type mice. The first case (BSE/Chiba) was successfully transmitted to rodents. The mean incubation periods (409.0 days) in this experiment was preferably longer than that of previously reported. PrPSc distribution, prion titer, mice susceptibility and/or storage condition of sample might be influenced the result. Recently, we introduced transgenic mice that overexpress a bovine PrP gene to overcome the species barrier problem. These mice are expected to accelerate the transmission experiment of BSE prion. Transmission of atypical BSE case is undergoing by using these transgenic mice.

http://www.knt.co.jp/ec/2004/prion/E2.htm

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

From: Terry S. Singeltary Sr. [flounder@wt.net] Sent: Tuesday, July 29, 2003 1:03 PM To: fdadockets@oc.fda.gov Cc: ggraber@cvm.fda.gov; Linda.Grassie@fda.gov; BSE-L Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION TO DOCKET 2003N-0312]

snip...

Greetings FDA,

PLUS, if the USA continues to flagrantly ignore the _documented_ science to date about the known TSEs in the USA (let alone the undocumented TSEs in cattle), it is my opinion, every other Country that is dealing with BSE/TSE should boycott the USA and demand that the SSC reclassify the USA BSE GBR II risk assessment to BSE/TSE GBR III 'IMMEDIATELY'. for the SSC to _flounder_ any longer on this issue, should also be regarded with great suspicion as well. NOT to leave out the OIE and it's terribly flawed system of disease surveillance. the OIE should make a move on CWD in the USA, and make a risk assessment on this as a threat to human health...

snip...full text;

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc- 000001.txt

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of the United States of America (USA) Publication date: 20 August 2004

Adopted July 2004 (Question N° EFSA-Q-2003-083)

* 167 kB Report

* 105 kB Summary

Summary of the Scientific Report

The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties.

A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre- clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre- clinically or clinically) infected with the BSE-agent persistently increases. http://www.efsa.eu.int/science/efsa_scientific_reports/gbr_assessments/573_en.html

IN your reply to me on 26 March 2003, Dr. Maddox, Belay, Schonberger et al write;

If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication).

I kindly wish to submit the following to dispute this;

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/index/public-interest/public- bse_and_sporadic_cjd.htm

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

A simple look at sporadic CJD statistics in EU countries with BSE will reveal this;

CANADA 2 IN 94 COMPARED TO 30 IN 2002

FRANCE 35 IN 93 COMPARED TO 102 IN 2003

GERMANY 21 IN 93 COMPARED TO 112 IN 2003

ITALY 27 IN 93 TO COMPARED TO 75 IN 2003

UK 37 IN 93 COMPARED TO 74 IN 2003

USA (UNKNOWN...TSS)

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

EVEN SEAC admits a rise in sporadic CJD in UK;

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the open session of the 84th meeting held on 28th September 2004

snip...

In contrast, the number of deaths in the UK from sCJD per annum had increased but this may reflect improved case ascertainment. A similar increase in sCJD had been observed in other countries... snip..

HOWEVER, I do not agree with this _assumption_!

I believe there are multiple routes and sources for this agent and they are going ignored, while being called 'sporadic' and or 'spontaneous' or 'classic' CJD.

TO continue to ignore Professor Collinge/Asanta et al's advice;

“We always have to be cautious about making direct comparison to the human condition, but our work strongly suggests that we can not assume only those with one genetic profile are vulnerable to BSE infection.

“At this stage it is not possible to say how this should alter estimates of those likely to become ill, but our findings do suggest we should be taking steps to draw up a more sophisticated system of categorizing the disease so that we don’t mistake BSE related infection for a version of sporadic CJD.”

TO continue to ingore this and the other evidence that is and has been mounting about sporadic CJD not being as sporadic and or spontaneos as once thought, to continue this ignorance and blantantly let this agent continue to spread via the proven routes to date and continue to infect and kill, should warrant a TSE Inquiry in the USA by a Congressional Investigation followed by International Council of some kind. WE are not only infecting US citizens by this ignorance, but also the International community that visits our Country. With the recent findings of nv/v CJD transmitting via blood, we must not flounder any longer;

Summary of SEACs discussion on the second presumed case of blood transfusion-associated infection with vCJD

7. SEAC agreed that the western blot results and glycotype profile suggested it was unlikely that the infection was preclinical sporadic CJD (sCJD). The committee noted that a single study by Glatzel et al (2003) had reported PrPres in the spleen of sCJD clinical cases. However, the levels of PrPres present in sCJD cases were low and detected in patients with a lengthy clinical illness from sporadic CJD.

http://www.seac.gov.uk/statements/state070804.htm

vCJD: Blood Transfusion Incident

http://www.publications.parliament.uk/pa/ld199697/ldhansrd/pdvn/lds03/text/31217 -09.htm

CJD (all human TSEs) should be made reportable Nationally and Internationally immediately, with a follow up investigation and questionnaire of each victim (family) asking questions pertaining to route and source of agent. ALL ages must be included in this. Anything less will only allow the agent to continue to spread and kill...

Thank You,

with kindest regards, I am sincerely,

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

http://www.neurology.org/cgi/eletter-submit




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