SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009
snip...
ITEM 4 – UPDATE ON CJD EPIDEMIOLOGY
11. Professor Richard Knight (National CJD Surveillance Unit) provided the Committee with the latest figures for the number of clinical vCJD and sporadic CJD (sCJD) cases. To date there had been 170 definite or probable clinical cases of vCJD in the UK - 167 from probable dietary infection with BSE and three from probable vCJD infection via transfusion of blood from donors who later developed vCJD. Of the 150 cases tested all were codon 129MM. Four cases are still alive. The number of deaths from vCJD peaked at 28 in 2000 and had since declined with two known deaths so far in 2009. The median age of death is 30 years of age.
12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case.
13. Professor Knight explained that one MV genotype case had been classified as possible vCJD as clinical features were consistent with the disease. However, it had not been possible to undertake neuropathological examination post mortem so the diagnosis could not be confirmed. The clinical profile of this MV case was consistent with that observed for MM cases.
14. Professor Knight summarised data on sCJD cases stating that from May 1990 to September 2009, 1080 cases of sCJD had been identified in the UK with a mean age at death of 67 years and genotype distribution of 63% MM, 19% MV and 18% VV at codon 129 of the prion protein gene.
15. Professor Knight also provided a brief report on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). The initial eleven cases described by Gambetti2 exhibited a mean age of onset of 62 years and mean disease duration of 20 months. Eight out of ten had a family history of dementia and were codon 129VV. Cases had minimal spongiform change and minimal immunohistochemical stained PrP deposits with distinct patterns in the cortex and cerebellum. Western Blot (WB) also shows a minimal amount of PrPres present. Further studies by Gambetti have now identified codon 129MV and MM cases which have a
longer disease duration and exhibit some PK resistance. The cases did not have clinical profiles typical for sCJD. A UK case and a Dutch case have also been identified, with characteristics not inconsistent with the Gambetti studies.
16. Professor Knight added that due to the unique clinical presentation of the disease it was likely that at least some cases of disease would not be identified for referral, making it hard to obtain complete data on this disease. However, it was likely that a case would be identified as a prion disease at autopsy and the WB currently used would be able to identify the unique profile which categorises this disease. A retrospective review of the NCJDSU brain bank is underway to look for more cases.
17. A Member asked whether the recent review of neuropathology archives in the UK would have identified PSPr. Professor Knight responded that it would be dependent on the type of WB used at the time which is currently not known. The use of appropriate WB methodology would be an issue in accurately identifying the relevant characteristics.
18. One Member was not convinced by the characterisation of this disease, adding that clinical cases classified as Alzheimer’s Disease have shown similar laddering profiles in WB, protease resistant fragments and the presence of abnormal PrP. The disease has, to date, not been shown to be transmissible which means it should not yet be categorised a prion disease under the current terminology.
19. Summing up, the Chair noted that it was clear that more information was required to fully characterise and fill knowledge gaps regarding this disease. It was important that its unique pathology be more widely recognised to enable future diagnosis and enable tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise the disease further.
http://www.seac.gov.uk/minutes/draftminutes103.pdf
>>>12. Professor Knight explained that elsewhere in the world 47 clinical vCJD cases have been reported with 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan. Infection was presumed to have occurred in the UK in respect of two Irish and two USA cases, one French case, one Japanese case and one Canadian case.<<<
>>>Two of the three U.S. cases, two of the four cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom. One of the 25 French cases may also have been infected in the United Kingdom.<<<
>>>There has never been a case of vCJD that did not have a history of exposure within a country where the cattle disease, BSE, was occurring.<<<
>>>vCJD Cases Reported in the US Three cases of vCJD have been reported from the United States. By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. There is strong evidence that suggests that two of the three cases were exposed to the BSE agent in the United Kingdom and that the third was exposed while living in Saudi Arabia.<<<
http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm
Heaven forbid any human mad cow disease coming from the U.S.A. ???
hmmm, i don't recall any madcows in Saudi Arabia ???
Eurosurveillance, Volume 11, Issue 49, 07 December 2006 Articles Editorial team1
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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
Showing posts with label USA. Show all posts
Showing posts with label USA. Show all posts
Thursday, February 4, 2010
Saturday, December 12, 2009
103RD MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
103RD MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
The Spongiform Encephalopathy Advisory Committee held its 103rd Meeting in London on 24th November 2009, and discussed the following:
CURRENT ISSUES
SEAC was informed about:
• Confirmation of BSE in a Scottish goat originally diagnosed with scrapie in 1990; the goat culled in 1990 was born prior to the introduction of the ruminant feed ban and could have been exposed to contaminated feed.
• The first case of atypical scrapie discovered in a sheep from New Zealand.
• A recommendation to Health Ministers by the Advisory Committee on the Safety of Blood Tissues and Organs that prion filtration should be introduced for blood used to treat patients who were not exposed to BSE through their diet.
• Recently published research1 on the discovery in Papua New Guinea of a novel protective prion protein variant.
CJD UPDATE
SEAC was updated on the number of clinical cases of variant Creutzfeldt-Jakob Disease (vCJD) and sporadic CJD (sCJD). To date there have been 170 definite or probable UK cases of clinical vCJD. 167 of these are associated with probable dietary exposure to BSE and three arose after transfusions of blood from donors who later developed vCJD. The mean age of death was 30 years. There have been no cases of vCJD in individuals born after 1989.
1 A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure by S Mead, J Whitfield, M Poulter, P Shah, J Uphill, T Campbell, H Al-Dujaily, H Hummerich, J Beck, C A Mein, C Verzilli, J Whittaker, M P Alpers & J Collinge. New Eng J Med., 2009, 361, 2056-65.
2 © SEAC 2009
Elsewhere in the world, 47 vCJD cases have been reported: 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan.
A brief report was provided on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). Eleven cases have been reported with a mean age of onset of 62 years and disease duration of 20 months. The committee thought it was important that the unique pathology of this disease be more widely recognised to enable future diagnosis and tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise this disease further.
EFFECT OF AGE ON THE PATHOGENESIS OF TSEs Most clinical cases of variant CJD have occurred in young adults, the median age at onset of disease being 26 years and the median age at death 28 years (the comparable ages for sporadic CJD are both 67). The reasons behind this apparent age-related susceptibility are uncertain. A recent paper2 reports the findings of a study in mice, which suggest that the age related decline in the functioning of follicular dendritic cells might impair TSE pathogenesis. The Committee agreed that a competent immune system was required for efficient replication of TSEs in the host. It was noted that older people are more immuno-compromised than the young, but there is very little data on the effect of ageing on the human immune system. The Committee concluded that there are insufficient data to suggest that this might provide an explanation for the young age of vCJD patients.
UP-DATE ON vCJD PREVALENCE STUDIES
The committee received an update on progress with the vCJD prevalence studies being conducted by the Health Protection Agency. This covered the National Anonymous Tonsil Archive (NATA), the post mortem archive and a new study of appendices. To date, NATA has tested approximately 80,000 pairs of tonsils and none were positive. A pilot for a study of spleens obtained
2 The effects of host age on follicular dendritic cell status dramatically impair scrapie agent neuroinvasion in aged mice. K L Brown, G J Wathne, J Sales, M E Bruce, and N A Mabbott, the Journal of Immunology 2009, doi:10.4049/jimmunol.0802695. 3 © SEAC 2009
from post mortems, that will assess a number of potential methodologies, will start in early 2010, and report on the efficacy of these methodologies in June 2010. The new study of 30,000 appendices, to be tested by immunohistochemistry, will also start in early 2010. The Committee noted progress, and commented that in order to have sufficient power, the spleen study would have to test approximately 50,000 samples.
CATEGORY 3 ANIMAL PRODUCTS IN FERTILISERS
Building on previous work in 2005, Defra has commissioned a full risk assessment, which SEAC was invited to consider, to evaluate the amount of potential infectivity available in the soil of nonpasture land following the application of Category 3-derived3 fertiliser.
The committee considered that the methodology used in the risk assessment was scientifically valid, but felt that too many worst case assumptions had been made in addressing the paucity of experimental data, that certain temporal aspects could have been addressed differently and that the possibility of regulatory failure had not been sufficiently considered.
FUTURE OPERATION OF SEAC
The Committee discussed the future organisation of SEAC business and the desirability of streamlining the decision making process, so that Ministers could obtain advice more quickly. In particular, SEAC considered a proposal that in future much of its business could be dealt with by correspondence, rather than in face-to-face meetings.
In discussion the committee recognised that some SEAC business could be carried out more efficiently by e-mail correspondence. The process would need to be structured, perhaps at fixed times of the year and the discussion would be summarised and recorded on the SEAC Website so that the decision-making process was transparent and available for public scrutiny. However, it was important that the committee still aimed to meet in person twice or three times per year.
3 Category 3 is low risk material, most of which is fit for human consumption, but not intended for human consumption.
3 © SEAC 2009
http://www.seac.gov.uk/pdf/seac103_summary.pdf
RE--Protease-Sensitive Prionopathy (PSPr) SEE ;
Protease-Sensitive Prionopathy (PSPr)
http://www.mhlw.go.jp/shingi/2009/02/dl/s0210-8d_0030.pdf
Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php
Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
see sporadic CJD, the big lie, POLICY IN CONFIDENCE; CONFIDENTIAL, coming full circle below ;
http://neuropathologyblog.blogspot.com/2008/07/new-prion-disease.html
http://www.wellsphere.com:86/cjd-article/a-novel-human-disease-with-abnormal-prion-protein-sensitive-to-protease-prionopathy/641206
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
>>>In the papers, the government alleges the meatpacking plant slaughtered and processed downer cows for nearly four years — from January 2004 to September 2007 — at the average rate of one every six weeks...<<<
http://downercattle.blogspot.com/2009/09/suit-meatpacker-used-downer-cows-for-4.html
do you actually believe all these schools recalled this meat because of a few cattle being abused,
see list ;
FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program
March 24, 2008
School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008
http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf
I have tried to get these papers through the court, but no luck. they want me to pay to retrieve the papers, and i am not going to pay for something I know happened. about like the last two FOIA on suspect mad cow feed going into commerce in the USA in 2009. I knew it had, but wanted them to say it. and they finally did via the FOIA.
Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.
http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Friday, September 4, 2009
FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009
http://madcowfeed.blogspot.com/2009/09/foia-request-on-feed-recall-product.html
Saturday, August 29, 2009
FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009
http://madcowfeed.blogspot.com/2009/08/foia-request-feed-recall-2009-product.html
----- Original Message ----- From: "Terry S. Singeltary Sr."To: Sent: Thursday, November 05, 2009 9:25 PM Subject: [BSE-L] re-FOIA REQUEST ON FEED RECALL PRODUCT contaminated with prohibited material Recall # V-258-2009 and Recall # V-256-2009
http://madcowfeed.blogspot.com/2009/11/re-foia-request-on-feed-recall-product.html
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
http://downercattle.blogspot.com/
please see full text here ;
Tuesday, November 17, 2009
SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2
http://downercattle.blogspot.com/2009/11/seac-effect-of-age-on-pathogenesis-of.html
Wednesday, November 18, 2009
R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission
http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html
Monday, October 19, 2009
Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009
http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html
Sunday, September 6, 2009
MAD COW USA 1997 SECRET VIDEO
http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom
http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html
DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN see video
http://maddeer.org/video/embedded/prusinerclip.html
CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008
PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS
BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START
http://madcowfeed.blogspot.com/2008/06/texas-firm-recalls-cattle-heads-that.html
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Saturday, December 05, 2009
Molecular Model of Prion Transmission to Humans
http://creutzfeldt-jakob-disease.blogspot.com/2009/12/molecular-model-of-prion-transmission.html
Monday, November 23, 2009
BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.
http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html
Atypical BSE North America Update February 2009
http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html
Sunday, December 28, 2008
MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy
http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html
Monday, May 4, 2009
Back to the Past With New TSE Testing Agricultural Research/May-June 2009
http://madcowtesting.blogspot.com/2009/05/back-to-past-with-new-tse-testing.html
Sunday, May 10, 2009
Identification and characterization of bovine spongiform encephalopathy cases diagnosed and NOT diagnosed in the United States
http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.html
Tuesday, July 14, 2009
U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST
WHERE did we go wrong $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
http://seac992007.blogspot.com/
TSS
The Spongiform Encephalopathy Advisory Committee held its 103rd Meeting in London on 24th November 2009, and discussed the following:
CURRENT ISSUES
SEAC was informed about:
• Confirmation of BSE in a Scottish goat originally diagnosed with scrapie in 1990; the goat culled in 1990 was born prior to the introduction of the ruminant feed ban and could have been exposed to contaminated feed.
• The first case of atypical scrapie discovered in a sheep from New Zealand.
• A recommendation to Health Ministers by the Advisory Committee on the Safety of Blood Tissues and Organs that prion filtration should be introduced for blood used to treat patients who were not exposed to BSE through their diet.
• Recently published research1 on the discovery in Papua New Guinea of a novel protective prion protein variant.
CJD UPDATE
SEAC was updated on the number of clinical cases of variant Creutzfeldt-Jakob Disease (vCJD) and sporadic CJD (sCJD). To date there have been 170 definite or probable UK cases of clinical vCJD. 167 of these are associated with probable dietary exposure to BSE and three arose after transfusions of blood from donors who later developed vCJD. The mean age of death was 30 years. There have been no cases of vCJD in individuals born after 1989.
1 A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure by S Mead, J Whitfield, M Poulter, P Shah, J Uphill, T Campbell, H Al-Dujaily, H Hummerich, J Beck, C A Mein, C Verzilli, J Whittaker, M P Alpers & J Collinge. New Eng J Med., 2009, 361, 2056-65.
2 © SEAC 2009
Elsewhere in the world, 47 vCJD cases have been reported: 25 in France, five in Spain, four in the Republic of Ireland, three in both the USA and the Netherlands, two in Portugal and Italy and single cases in Canada, Saudi Arabia and Japan.
A brief report was provided on the novel human disease known as Protease-Sensitive Prionopathy (PSPr). Eleven cases have been reported with a mean age of onset of 62 years and disease duration of 20 months. The committee thought it was important that the unique pathology of this disease be more widely recognised to enable future diagnosis and tissue collection during autopsy procedures. SEAC will keep a watching brief on emerging data which may characterise this disease further.
EFFECT OF AGE ON THE PATHOGENESIS OF TSEs Most clinical cases of variant CJD have occurred in young adults, the median age at onset of disease being 26 years and the median age at death 28 years (the comparable ages for sporadic CJD are both 67). The reasons behind this apparent age-related susceptibility are uncertain. A recent paper2 reports the findings of a study in mice, which suggest that the age related decline in the functioning of follicular dendritic cells might impair TSE pathogenesis. The Committee agreed that a competent immune system was required for efficient replication of TSEs in the host. It was noted that older people are more immuno-compromised than the young, but there is very little data on the effect of ageing on the human immune system. The Committee concluded that there are insufficient data to suggest that this might provide an explanation for the young age of vCJD patients.
UP-DATE ON vCJD PREVALENCE STUDIES
The committee received an update on progress with the vCJD prevalence studies being conducted by the Health Protection Agency. This covered the National Anonymous Tonsil Archive (NATA), the post mortem archive and a new study of appendices. To date, NATA has tested approximately 80,000 pairs of tonsils and none were positive. A pilot for a study of spleens obtained
2 The effects of host age on follicular dendritic cell status dramatically impair scrapie agent neuroinvasion in aged mice. K L Brown, G J Wathne, J Sales, M E Bruce, and N A Mabbott, the Journal of Immunology 2009, doi:10.4049/jimmunol.0802695. 3 © SEAC 2009
from post mortems, that will assess a number of potential methodologies, will start in early 2010, and report on the efficacy of these methodologies in June 2010. The new study of 30,000 appendices, to be tested by immunohistochemistry, will also start in early 2010. The Committee noted progress, and commented that in order to have sufficient power, the spleen study would have to test approximately 50,000 samples.
CATEGORY 3 ANIMAL PRODUCTS IN FERTILISERS
Building on previous work in 2005, Defra has commissioned a full risk assessment, which SEAC was invited to consider, to evaluate the amount of potential infectivity available in the soil of nonpasture land following the application of Category 3-derived3 fertiliser.
The committee considered that the methodology used in the risk assessment was scientifically valid, but felt that too many worst case assumptions had been made in addressing the paucity of experimental data, that certain temporal aspects could have been addressed differently and that the possibility of regulatory failure had not been sufficiently considered.
FUTURE OPERATION OF SEAC
The Committee discussed the future organisation of SEAC business and the desirability of streamlining the decision making process, so that Ministers could obtain advice more quickly. In particular, SEAC considered a proposal that in future much of its business could be dealt with by correspondence, rather than in face-to-face meetings.
In discussion the committee recognised that some SEAC business could be carried out more efficiently by e-mail correspondence. The process would need to be structured, perhaps at fixed times of the year and the discussion would be summarised and recorded on the SEAC Website so that the decision-making process was transparent and available for public scrutiny. However, it was important that the committee still aimed to meet in person twice or three times per year.
3 Category 3 is low risk material, most of which is fit for human consumption, but not intended for human consumption.
3 © SEAC 2009
http://www.seac.gov.uk/pdf/seac103_summary.pdf
RE--Protease-Sensitive Prionopathy (PSPr) SEE ;
Protease-Sensitive Prionopathy (PSPr)
http://www.mhlw.go.jp/shingi/2009/02/dl/s0210-8d_0030.pdf
Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php
Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
see sporadic CJD, the big lie, POLICY IN CONFIDENCE; CONFIDENTIAL, coming full circle below ;
http://neuropathologyblog.blogspot.com/2008/07/new-prion-disease.html
http://www.wellsphere.com:86/cjd-article/a-novel-human-disease-with-abnormal-prion-protein-sensitive-to-protease-prionopathy/641206
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
>>>In the papers, the government alleges the meatpacking plant slaughtered and processed downer cows for nearly four years — from January 2004 to September 2007 — at the average rate of one every six weeks...<<<
http://downercattle.blogspot.com/2009/09/suit-meatpacker-used-downer-cows-for-4.html
do you actually believe all these schools recalled this meat because of a few cattle being abused,
see list ;
FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program
March 24, 2008
School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008
http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf
I have tried to get these papers through the court, but no luck. they want me to pay to retrieve the papers, and i am not going to pay for something I know happened. about like the last two FOIA on suspect mad cow feed going into commerce in the USA in 2009. I knew it had, but wanted them to say it. and they finally did via the FOIA.
Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.
http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Friday, September 4, 2009
FOIA REQUEST ON FEED RECALL PRODUCT 429,128 lbs. feed for ruminant animals may have been contaminated with prohibited material Recall # V-258-2009
http://madcowfeed.blogspot.com/2009/09/foia-request-on-feed-recall-product.html
Saturday, August 29, 2009
FOIA REQUEST FEED RECALL 2009 Product may have contained prohibited materials Bulk Whole Barley, Recall # V-256-2009
http://madcowfeed.blogspot.com/2009/08/foia-request-feed-recall-2009-product.html
----- Original Message ----- From: "Terry S. Singeltary Sr."
http://madcowfeed.blogspot.com/2009/11/re-foia-request-on-feed-recall-product.html
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
http://downercattle.blogspot.com/
please see full text here ;
Tuesday, November 17, 2009
SEAC EFFECT OF AGE ON THE PATHOGENESIS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES SEAC 103/2
http://downercattle.blogspot.com/2009/11/seac-effect-of-age-on-pathogenesis-of.html
Wednesday, November 18, 2009
R-CALF: 40 Groups Disagree With USDA's Latest BSE Court Submission
http://bse-atypical.blogspot.com/2009/11/r-calf-40-groups-disagree-with-usdas.html
Monday, October 19, 2009
Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009
http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html
Sunday, September 6, 2009
MAD COW USA 1997 SECRET VIDEO
http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom
http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html
DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN see video
http://maddeer.org/video/embedded/prusinerclip.html
CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008
PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS
BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START
http://madcowfeed.blogspot.com/2008/06/texas-firm-recalls-cattle-heads-that.html
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Saturday, December 05, 2009
Molecular Model of Prion Transmission to Humans
http://creutzfeldt-jakob-disease.blogspot.com/2009/12/molecular-model-of-prion-transmission.html
Monday, November 23, 2009
BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E.
http://docket-aphis-2006-0041.blogspot.com/2009/11/bse-gbr-risk-assessments-update.html
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html
Atypical BSE North America Update February 2009
http://bse-atypical.blogspot.com/2009/02/atypical-bse-north-america-update.html
Sunday, December 28, 2008
MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy
http://bse-atypical.blogspot.com/2008/12/mad-cow-disease-usa-december-28-2008-8.html
Monday, May 4, 2009
Back to the Past With New TSE Testing Agricultural Research/May-June 2009
http://madcowtesting.blogspot.com/2009/05/back-to-past-with-new-tse-testing.html
Sunday, May 10, 2009
Identification and characterization of bovine spongiform encephalopathy cases diagnosed and NOT diagnosed in the United States
http://bse-atypical.blogspot.com/2009/05/identification-and-characterization-of.html
Tuesday, July 14, 2009
U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST
WHERE did we go wrong $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
http://seac992007.blogspot.com/
TSS
Friday, October 10, 2008
SEAC 101st meeting on Wednesday 15th October 2008 AGENDA
SEAC 101st meeting on Wednesday 15th October 2008 AGENDA
snip...
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008
snip...
ITEM 3 – CURRENT ISSUES
9.
SEAC was informed about the following issues:
• Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.
http://www.seac.gov.uk/papers/101-1.pdf
3 10:10 Current issues
vCJD cluster in Spain Testing of a goat
snip...
In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.
* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.
http://www.seac.gov.uk/agenda/agen151008.htm
The CJD Foundation Newsletter
http://www.cjdfoundation.org/content/newsletters/september2008.pdf
snip...
SEPTEMBER 2008 VOLUME 1, ISSUE 3
The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.
Note 1: Not all new cases and deaths reported are confirmed by autopsy.
Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..
2008
MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS
January 35 24 203 6,424 February 24 9 212 6,848 March 19 19 164 7,492 April 36 14 231 8,427 May 26 20 191 8,839 June 19 17 144 9,646 July 28 14 171 7,791 August 27 16 150 5,323
TOTALS = 214 133 1,466 60,790
P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? help@cjdfoundation.org www.cjdfoundation.org
CJDF Questionnaire Update
With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at help@cjdfoundation.org or 1-800-659-1991. We greatly appreciate your help with this important project!
CJD QUESTIONNAIRE HISTORY
http://cjdquestionnaire.blogspot.com/
Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website
http://www.neuroprion.com/en/patients-events-07-6th-CJD-Conf.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
snip...
Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
snip...
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html
A New Prionopathy OR more of the same old BSe and sporadic CJD
http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
sporadic Fatal Familial Insomnia
http://sporadicffi.blogspot.com/
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Creutzfeldt Jakob Disease
http://creutzfeldt-jakob-disease.blogspot.com/
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html
USA PRION UNIT BLOG
http://prionunitusaupdate2008.blogspot.com/
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.
see full text ;
http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html
CJD TEXAS (cjd clusters)
http://cjdtexas.blogspot.com/
USA WRITTEN CJD QUESTIONNAIRE ???
http://cjdquestionnaire.blogspot.com/
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Tuesday, August 19, 2008
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html
Review on the epidemiology and dynamics of BSE epidemics
Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article
snip...
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
snip...
Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf
please see full text ;
http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html
SEAC 99th meeting on Friday 14th December 2007
snip...
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.
A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.
There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.
snip...
http://www.seac.gov.uk/minutes/99.pdf
There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.
10 people killed by new CJD-like disease
Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]
Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist
10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.
No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.
Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
snip... see full text ;
http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php
Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
http://seac992007.blogspot.com/
snip...
http://www.seac.gov.uk/minutes/99.pdf
TSS
snip...
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008
snip...
ITEM 3 – CURRENT ISSUES
9.
SEAC was informed about the following issues:
• Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.
http://www.seac.gov.uk/papers/101-1.pdf
3 10:10 Current issues
vCJD cluster in Spain Testing of a goat
snip...
In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.
* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.
http://www.seac.gov.uk/agenda/agen151008.htm
The CJD Foundation Newsletter
http://www.cjdfoundation.org/content/newsletters/september2008.pdf
snip...
SEPTEMBER 2008 VOLUME 1, ISSUE 3
The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.
Note 1: Not all new cases and deaths reported are confirmed by autopsy.
Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..
2008
MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS
January 35 24 203 6,424 February 24 9 212 6,848 March 19 19 164 7,492 April 36 14 231 8,427 May 26 20 191 8,839 June 19 17 144 9,646 July 28 14 171 7,791 August 27 16 150 5,323
TOTALS = 214 133 1,466 60,790
P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? help@cjdfoundation.org www.cjdfoundation.org
CJDF Questionnaire Update
With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at help@cjdfoundation.org or 1-800-659-1991. We greatly appreciate your help with this important project!
CJD QUESTIONNAIRE HISTORY
http://cjdquestionnaire.blogspot.com/
Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website
http://www.neuroprion.com/en/patients-events-07-6th-CJD-Conf.html
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
snip...
Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
snip...
http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html
A New Prionopathy OR more of the same old BSe and sporadic CJD
http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
sporadic Fatal Familial Insomnia
http://sporadicffi.blogspot.com/
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
http://www.thepathologicalprotein.com/
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Creutzfeldt Jakob Disease
http://creutzfeldt-jakob-disease.blogspot.com/
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html
http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html
http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html
USA PRION UNIT BLOG
http://prionunitusaupdate2008.blogspot.com/
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.
see full text ;
http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html
CJD TEXAS (cjd clusters)
http://cjdtexas.blogspot.com/
USA WRITTEN CJD QUESTIONNAIRE ???
http://cjdquestionnaire.blogspot.com/
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
Tuesday, August 19, 2008
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html
Review on the epidemiology and dynamics of BSE epidemics
Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article
snip...
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
snip...
Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf
please see full text ;
http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html
SEAC 99th meeting on Friday 14th December 2007
snip...
SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: “With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?” 41.
A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.
There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.
snip...
http://www.seac.gov.uk/minutes/99.pdf
There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.
10 people killed by new CJD-like disease
Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]
Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:claire.bowles@rbi.co.uk 44-207-611-1210 New Scientist
10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.
No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.
Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.
snip... see full text ;
http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php
Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
2008
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
http://www.cjdfoundation.org/fact.html
http://seac992007.blogspot.com/
snip...
http://www.seac.gov.uk/minutes/99.pdf
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