Monitoring the occurrence of emerging forms of CJD

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics

2009-09-09T08:36:00.000-07:00

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics.

by: Ignazio Cali, Rudolph Castellani, Amer Alshekhlee, Yvonne Cohen, Janis Blevins, Jue Yuan, Jan P. Langeveld, Piero Parchi, Jiri G. Safar, Wen-Quan Q. Zou, Pierluigi Gambetti Brain : a journal of neurology (4 September 2009)

ABSTRACT

Five phenotypically distinct subtypes have been identified in sporadic Creutzfeldt-Jakob disease (sCJD), based on the methionine/valine polymorphic genotype of codon 129 of the prion protein (PrP) gene and the presence of either one of the two protease K-resistant scrapie prion protein (PrP(Sc)) types identified as 1 and 2. The infrequent co-existence of both PrP(Sc) types in the same case has been known for a long time. Recently, it has been reported, using type-specific antibodies, that the PrP(Sc) type 1 is present in all cases of sCJD carrying PrP(Sc) type 2. The consistent co-occurrence of both PrP(Sc) types complicates the diagnosis and the current classification of sCJD, and has implications for the pathogenesis of naturally occurring prion diseases. In the present study, we investigated the prevalence of PrP(Sc) types 1 and 2 co-occurrence, along with its effects on the disease phenotype and PrP(Sc) strain characteristics, comparatively analysing 34 cases of sCJD, all methionine homozygous at codon 129 of the PrP gene (sCJDMM). To minimize overestimating the prevalence of the sCJDMM cases carrying PrP(Sc) types 1 and 2 (sCJDMM1-2), we used proteinase K concentrations designed to hydrolyse all fragments resulting from an incomplete digestion, while preserving the protease-resistant PrP(Sc) core. Furthermore, we used several antibodies to maximize the detection of both PrP(Sc) types. Our data show that sCJDMM cases associated exclusively with either PrP(Sc) type 1 (sCJDMM1) or PrP(Sc) type 2 (sCJDMM2) do exist; we estimate that they account for approximately 56% and 5% of all the sCJDMM cases, respectively; while in 39% of the cases, both PrP(Sc) types 1 and 2 are present together (sCJDMM1-2) either mixed in the same anatomical region or separate in different regions. Clinically, sCJDMM1-2 had an average disease duration intermediate between the other two sCJDMM subtypes. The histopathology was also intermediate, except for the cerebellum where it resembled that of sCJDMM1. These features, along with the PrP immunostaining pattern, offer a diagnostic clue. We also observed a correlation between the disease duration and the prevalence of PrP(Sc) type 2 and sCJDMM2 phenotypes. The use of different antibodies and of the conformational stability immunoassay indicated that the co-existence of types 1 and 2 in the same anatomical region may confer special conformational characteristics to PrP(Sc) types 1 and 2. All of these findings indicate that sCJDMM1-2 should be considered as a separate entity at this time.

http://www.citeulike.org/user/applebyb/article/5739998

> sCJDMM1-2 should be considered as a separate entity at this time.

Thank you very much !

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html

Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

snip...

COMMENT

snip...see full text ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html

Sunday, September 6, 2009

MAD COW USA 1997 VIDEO

SEE ANOTHER VIDEO THAT SHOWED IN CANADA, BUT NOT USA, ABOUT ANOTHER USA TSE COVER-UP

MORE BRAINS NOT TESTED PROPERLY, key brain parts missing. ...

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html

SEE THIS DAMNING VIDEO AT BOTTOM OF ;

Monday, July 27, 2009

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html

TSS

CJD FOUNDATION MIAMI FOUNDER CELE SARDO

2009-06-15T10:15:00.000-07:00

CJD FOUNDATION MIAMI FOUNDER CELE SARDO

Jun 15, 2009

Greetings CJD Family,

I want to let the old timers, and everyone know, that Cele Sardo, founder of the original CJD Foundation out of Florida, who pioneered support for CJD victims of all types here in the USA first, before the CJD Foundation INC at CWRU took it over, before CJD voice, or CJD watch. Cele passed away this morning about 4 AM. she was my friend, and as twisted as i may seem sometimes, she has always understood my anger. Joe told me that the family will bury Cele in the Kendall area of Miami later this week where Mr. Sardo, Joe's father is buried.Cele lost her husband Joe (Joseph's Father), to sporadic CJD around 1989. Mr. Sardo was 56 when he died from CJD. Cele and and her friend Mayra Lichter did a great job with the CJD Foundation when they had it, and Cele knew too well that the CJD surveillance was lacking here in the USA. Cele Sardo and Mayra Lichter Founded the CJD Foundation originally back in Miami, Florida around 1993, and what an organization of support is was. The Sardo Family has done a _great_ deal for research for CJD. Our thanks go out to them, along with our prayers for the family at this difficult time...

Two Women Against Mad Cow Disease November 1, 2001 Good Housekeeping by Lisa Belkin

http://www.organicconsumers.org/madcow/women110101.cfm

Proper sterilization may ward off deadly CJD transmission Healthcare Purchasing News , May, 2001

http://findarticles.com/p/articles/mi_m0BPC/is_5_25/ai_75644703/

CELE WAS A SOLDIER IN THE BATTLE TO FIND SUPPORT, TO FIND THE CAUSE, AND TO FIND A CURE FOR ALL FORMS OF CJD, OF ALL AGES. ...

with saddest regards,

i will miss my friend. ...terry

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

2009-06-13T19:58:00.000-07:00

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

Saturday, July, 18, 2009

Greetings,

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. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.

The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.

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. 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. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? 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. ...

please see history, and the ever evolving TSE science to date ;

2003

Neurology 2003;60:176-181 © 2003 American Academy of Neurology

-------------------------------------------------------------------------------- Views & Reviews

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.

Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.

Transmissible spongiform encephalopathies (TSEs) attracted increased attention in the mid-1980s because of the emergence among UK cattle of bovine spongiform encephalopathy (BSE), which has been shown to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD). The BSE outbreak has been reported in 19 European countries, Israel, and Japan, and human cases have so far been identified in four European countries, and more recently in a Canadian resident and a US resident who each lived in Britain during the BSE outbreak. To monitor the occurrence of emerging forms of CJD, such as vCJD, in the United States, the Centers for Disease Control and Prevention has been conducting surveillance for human TSEs through several mechanisms, including the establishment of the National Prion Disease Pathology Surveillance Center. Physicians are encouraged to maintain a high index of suspicion for vCJD and use the free services of the pathology center to assess the neuropathology of clinically diagnosed and suspected cases of CJD or other TSEs.

http://www.neurology.org/cgi/content/abstract/60/2/176

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

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

Reply to Singletary 26 March 2003

Ryan A. Maddox, MPH

Mr. Singletary raises several issues related to current Creutzfeldt- Jakob disease (CJD) surveillance activities. Although CJD is not a notifiable disease in most states, its unique characteristics, particularly its invariably fatal outcome within usually a year of onset, make routine mortality surveillance a useful surrogate for ongoing CJD surveillance.[1] In addition, because CJD is least accurately diagnosed early in the course of illness, notifiable-disease surveillance could be less accurate than, if not duplicative of, current mortality surveillance.[1] However, in states where making CJD officially notifiable would meaningfully facilitate the collection of data to monitor for variant CJD (vCJD) or other emerging prion diseases, CDC encourages the designation of CJD as a notifiable disease.[1] Moreover, CDC encourages physicians to report any diagnosed or suspected CJD cases that may be of special public health importance (e.g., vCJD, iatrogenic CJD, unusual CJD clusters).

As noted in our article, strong evidence is lacking for a causal link between chronic wasting disease (CWD) of deer and elk and human disease,[2] but only limited data seeking such evidence exist. Overall, the previously published case-control studies that have evaluated environmental sources of infection for sporadic CJD have not consistently identified strong evidence for a common risk factor.[3] However, the power of a case-control study to detect a rare cause of CJD is limited, particularly given the relatively small number of subjects generally involved and its long incubation period, which may last for decades. Because only a very small proportion of the US population has been exposed to CWD, a targeted surveillance and investigation of unusual cases or case clusters of prion diseases among persons at increased risk of exposure to CWD is a more efficient approach to detecting the possible transmission of CWD to humans. In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

Mr. Singletary also expresses concern over a recent publication by Asante and colleagues indicating the possibility that some sporadic CJD cases may be attributable to bovine spongiform encephalopathy (BSE).[4] The authors reported that transgenic mice expressing human prion protein homozygous for methionine at codon 129, when inoculated with BSE prions, developed a molecular phenotype consistent with a subtype of sporadic CJD. Although the authors implied that BSE might cause a sporadic CJD-like illness among persons homozygous for methionine, the results of their research with mice do not necessarily directly apply to the transmission of BSE to humans. If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication).

References

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.

2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003;60:176-181.

3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283-314.

4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002;21:6358-6366.

5. The UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics. Available at: http://www.cjd.ed.ac.uk/figures.htm. Accessed February 18, 2003.

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

Posted On December 20, 2003

Mad Cow Scaremongers

Terry Singletary -- A retired machinist and high school dropout, Terry Singletary suffered the tragic loss of his mother to “sporadic” Creutzfeldt-Jakob disease (CJD) in 1997. Desperate to find an explanation for his mother’s death, he has devoted himself to the sad and fruitless task of connecting her death to her diet. Various reports confirm that Mrs. Singletary’s life was claimed by the most common sub-type of CJD (one that accounts for 70 percent of “sporadic” cases). Sporadic CJD, unlike its newer “variant,” is not linked to meat.

As the self-appointed international coordinator of CJD Watch, an organization he co-founded with social worker Deborah Oney, Singletary is cited in media reports as an apparent expert on tracking mad cow disease. This despite his lack of formal education and the absence for support from any credible academic, medical or scientific authority. His sensationalist allegations about the safety of U.S. beef have found their way into hundreds of newspapers and broadcasts. Singletary moderates a mad-cow discussion forum run by a vegetarian activist group; his contributions account for more than half the traffic on the “BSE-L” mailing list, which is generally read by real scientists. Animal rights activists and other food-scare artists frequently refer to him as “Dr. Terry Singletary,” apparently an honorary degree as he has yet to finish high school.

Like many activists, Singletary ignores overwhelming epidemiological and laboratory evidence that rules out a connection between sporadic CJD and beef. Relying entirely on shallow circumstantial evidence and frequent repetition of claims which have been publicly refuted as false, he also blindly insists upon a mad-cow with Alzheimer’s, Parkinson’s, and Lou Gehrig’s disease. His specific allegations have been clearly refuted by Centers for Disease Countrol and Prevention scientists in the journal Neurology.

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

http://www.consumerfreedom.com/article_detail.cfm/article/138

Book

The Pathological Protein

Publisher Springer New York DOI 10.1007/b97488 Copyright 2003 ISBN 978-0-387-95508-7 (Print) 978-0-387-21755-0 (Online) DOI 10.1007/0-387-21755-X_14 Pages 223-237 Subject Collection Humanities, Social Sciences and Law SpringerLink

Laying Odds

snip...

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.

snip...

http://www.springerlink.com/content/r2k2622661473336/

http://books.google.com/books?id=ePbrQNFrHtoC&pg=PA223&lpg=PA223&dq=SINGELTARY+pathological+protein+it

2006

FOOD AND DRUG ADMINISTRATION

DEPARTMENT OF HEALTH AND HUMAN SERVICES

This transcripts has not been edited or corrected, but appears as received from the commercial transcribing service. Accordingly, the food and Drug Administration makes no representation as to its accuracy.

Meeting of:

TRANSMISSIBLE

SPONGIFORM ENCEPHALOPATHIES

ADVISORY COMMITTEE

September 18, 2006

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

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/2009/02/atypical-bse-north-america-update.html

2009

----- Original Message -----

From: Freas, William To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:flounder9@verizon.net Cc: mailto:flounder9@verizon.net

Sent: Monday, June 08, 2009 5:32 PM Subject: Written comments regarding TSEAC

Dear Mr. Singeltary,

Thank you for your written statement regarding the June 12, 2009 TSEAC meeting. Your statement will be copied, given to the committee members, made part of the official meeting record and made available to the public.

Thanks again.

Bill Freas Acting TSEAC Exec. Sec.

From: "Terry S. Singeltary Sr." <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:flounder9@verizon.net> To: <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:william.freas@fda.hhs.gov> Cc: "Dave Cavenaugh" <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:cott-dc@earthlink.net>; "DR. LAURA MANUELIDIS" <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:laura.manuelidis@yale.edu>; <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:rrohwer@umaryland.edu>; ncashman@prionetcanada.ca> Subject: Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission) Date: Sunday, May 10, 2009 12:55 PM TO: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:william.freas@fda.hhs.gov <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:william.freas@fda.hhs.gov>

May 8, 2009

2009 Meeting Materials, Transmissible Spongiform Encephalopathies Advisory Committee

Updated: 6/4/2009

http://www.fda.gov/AdvisoryCommittees/CommitteesMeetingMaterials/BloodVaccinesandOtherBiologics/TransmissibleSpongiformEncephalopathiesAdvisoryCommittee/ucm129559.htm

Transmissible Spongiform Encephalopathies Advisory Committee 21st Meeting Final Issue Summary June 12, 2009

Holiday Inn 2 Montgomery Village Avenue Gaithersburg, MD 20879

Topic I:

Modified FDA Risk Assessment for Potential Exposure to the Infectious Agent of Variant Creutzfeldt-Jakob Disease (vCJD) in US-licensed Plasma-Derived Factor VIII (pdFVIII)

ISSUE:

Plasma-derived Factor VIII (pdFVIII) products are used by blood clotting disorder patients with von Willebrand disease and some patients with hemophilia A. The announcement in February 2009 by health authorities in the United Kingdom that a vCJD infection had been recognized in a person with hemophilia treated with a UK manufactured "vCJD-implicated" pdFVIII 11 years earlier has prompted FDA to review the potential vCJD risk for US users of US-licensed pdFVIII products and current risk management strategies for such products.

Results from an updated FDA risk assessment model continue to indicate that the estimated risk of the potential for US-licensed pdFVIII products to transmit the agent of vCJD, the human form of "Mad Cow Disease," is highly uncertain but is most likely to be extremely small.

FDA seeks the advice of the Committee on whether additional risk reducing measures are needed (e.g. modifications to current donor deferral policies) to maintain the safety of plasma-derived biologic products and whether FDA should change its communications concerning the risks of vCJD associated with plasma derivatives. ...

snip...

see full text ;

http://www.fda.gov/downloads/AdvisoryCommittees/Calendar/UCM164322.pdf

http://www.fda.gov/downloads/AdvisoryCommittees/Calendar/UCM164326.pdf

http://www.fda.gov/downloads/AdvisoryCommittees/Calendar/UCM164322.pdf

this might be interesting to see what is said here too ;

SNIP...

TSEAC AGENDA, Friday, June 12, 2009 (page 2)

12:30 p.m. Lunch

1:30 p.m. Topic II: Informational Presentations

A. BSE Surveillance and USDA-Regulated Food Controls in the U.S., Janet A. Hughes, DVM, PhD, APHIS, USDA (20')

B. BSE Surveillance and Food/Feed Controls in Europe, Koen van Dyck, DVM, European Commission (20')

C. BSE Surveillance, Animal Feeds and Food Controls in Canada, Dr. Noel Murray, Canadian Food Safety Inspection Agency (20')

D. FDA Enhanced Animal "Feed Ban": Current Status, Burt Pritchett, DVM, CVM, FDA (20')

E. FDA-Regulated Food Controls in the U.S., Amber McCoig, DVM, CFSAN, FDA (20')

F. FDA Proposed BSE "Medical Products" Rule: Current Status, Theresa Finn, Ph. D., OVRR, FDA (20')

SNIP...

http://www.fda.gov/downloads/AdvisoryCommittees/Calendar/UCM164321.pdf

http://www.fda.gov/AdvisoryCommittees/CommitteesMeetingMaterials/BloodVaccinesandOtherBiologics/TransmissibleSpongiformEncephalopathiesAdvisoryCommittee/ucm129559.htm

Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html

Friday, June 12, 2009

vCJD-related abnormal prion protein in a person with haemophilia – an update

http://vcjdtransfusion.blogspot.com/2009/06/vcjd-related-abnormal-prion-protein-in.html

http://vcjdtransfusion.blogspot.com/

Sunday, April 12, 2009

BSE MAD COW TESTING USA 2009 FIGURES

Month Number of Tests

Feb 2009 -- 1,891

Jan 2009 -- 4,620

http://www.aphis.usda.gov/newsroom/hot_issues/bse/surveillance/ongoing_surv_results.shtml

http://madcowtesting.blogspot.com/2009/04/bse-mad-cow-testing-usa-2009-figures.html

Saturday, June 13, 2009

BSE FEED VIOLATIONS USA UPDATE From 01/01/2009 To 06/10/2009

http://madcowfeed.blogspot.com/2009/06/bse-feed-violations-usa-update-from.html

SPORADIC CJD CASES RISING IN U.S.A 2009 UPDATE

Monday, April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

National Prion Disease Pathology Surveillance Center Cases Examined1

(December 31, 2008)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 42 32 28 4 0 0

1997 115 68 59 9 0 0

1998 93 53 45 7 1 0

1999 115 69 61 8 0 0

2000 151 103 89 14 0 0

2001 210 118 108 9 0 0

2002 258 147 123 22 2 0

2003 273 176 135 41 0 0

2004 335 184 162 21 0 13

2005 346 193 154 38 1 0

2006 380 192 159 32 0 14

2007 370 212 185 26 0 0

2008 383 228 182 23 0 0

TOTAL 30715 17756 1490 254 4 2

1 Listed based on the year of death or, if not available, on year of referral; 2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted; 3 Disease acquired in the United Kingdom; 4 Disease acquired in Saudi Arabia; 5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded.

Rev 2/13/09 National

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

http://www.cjdsurveillance.com/resources-casereport.html

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

*5 Includes 20 cases in which the diagnosis is pending, and 17 inconclusive cases; *6 Includes 25 cases with type determination pending in which the diagnosis of vCJD has been excluded.

Greetings,

it would be interesting to know what year these atypical cases occurred, as opposed to lumping them in with the totals only.

are they accumulating ?

did they occur in one year, two years, same state, same city ?

location would be very interesting ?

age group ?

sex ?

how was it determined that nvCJD was ruled out ?

from 1997, the year i started dealing with this nightmare, there were 28 cases (per this report), up until 2007 where the total was 185 cases (per this report), and to date 2008 is at 182. a staggering increase in my opinion, for something that just happens spontaneously as some would have us believe. i don't believe it, not in 85%+ of all sporadic CJD cases. actually, i do not believe yet that anyone has proven that any of the sporadic CJD cases have been proven to be a spontaneous misfolding of a protein. there are many potential routes and sources for the sporadic CJD's. ...TSS

please see full text here ;

http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.html

don't forget, in people 55 and older, it's 1 in 9,000. the officials seem to forget about that when speaking with the media.

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://cjdusa.blogspot.com/

OH, and i might add, confusious is confused again.

IN the states where CJD is only reportable in a certain age group i.e. say only reportable in 55 and younger, how would there ever be an accurate count ???

Connecticut Reportable For under 55 within 12 hours

http://www.ct.gov/dph/lib/dph/infectious_diseases/ctepinews/vol29no1.pdf

In dealing with the news media and public about CJD, it is important to avoid confusion between vCJD and the near half-dozen cases of sporadic CJD that occur in NC each year. Confirmation by brain tissue examination of anyone with suspected CJD/vCJD who is less than 55 years of age is even more important.3

3. “Questions and Answers Regarding Bovine Spongiform Encephalopathy (BSE) and Variant Creutzfeldt-Jakob Disease (vCJD),” Centers for Disease Control and Prevention, 4 January 2007,

www.cdc.gov/ncidod/dvrd/vcjd/qa.htm#surveillance.

http://www.epi.state.nc.us/epi/gcdc/manual/diseasenotes/CREUTZFELDT-JAKOB%20DISEASE_DN.pdf

SO, if CJD is not reportable in some states over age 55, and the rate of CJD in 55 and older is 1 in 9,000, then what is the true count if all states reported it ??? human and animal TSEs and 'friendly fire' i.e. iatrogenic, know no age groups. so why put one on it, and continue to believe in this UKBSEnvCJD only theory $$$

ALL human TSE of ALL ages must be made reportable in every state and Internationally. ...tss

Mad Cow: Linked to thousands of CJD cases?

By STEVE MITCHELL, United Press InternationalPublished: Dec. 30, 2003 at 3:31 PM

http://www.upi.com/Science_News/2003/12/30/Mad-Cow-Linked-to-thousands-of-CJD-cases/UPI-47861072816318/

Rare BSE mutation raises concerns over risks to public health

SIR - Atypical forms (known as H- and L-type) of bovine spongiform encephalopathy (BSE) have recently appeared in several European countries as well as in Japan, Canada and the United States. This raises the unwelcome possibility that variant Creutzfeldt-Jakob disease (vCJD) could increase in the human population. Of the atypical BSE cases tested so far, a mutation in the prion protein gene (PRNP) has been detected in just one, a cow in Alabama with BSE; her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008). This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000138/!x-usc:mailto:maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA

NATUREVol 45726 February 2009

http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html

see full text ;

Monday, May 11, 2009

Rare BSE mutation raises concerns over risks to public health

http://bse-atypical.blogspot.com/2009/05/rare-bse-mutation-raises-concerns-over.html

Monday, June 01, 2009

Biochemical typing of pathological prion protein in aging cattle with BSE

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html

Sunday, June 07, 2009

L-TYPE-BSE, H-TYPE-BSE, C-TYPE-BSE, IBNC-TYPE-BSE, TME, CWD, SCRAPIE, CJD, NORTH AMERICA

http://bse-atypical.blogspot.com/2009/06/l-type-bse-h-type-bse-c-type-bse-ibnc.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

Sunday, May 17, 2009

WHO WILL WATCH THE CHILDREN ? SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

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

Saturday, April 04, 2009

An unusually presenting case of sCJD—The VV1 subtype Volume 111, Issue 3, Pages 282-291 (April 2009)

http://creutzfeldt-jakob-disease.blogspot.com/2009/04/unusually-presenting-case-of-scjdthe.html

IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???

lets look at the full circle, to date ;

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

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

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 Manuscript 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

Elsevier Editorial System(tm) for The Lancet Infectious Diseases Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary

Abstract:

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. 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 2007. snip...

see full text 31 pages ;

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

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

Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html

Friday, May 29, 2009

Characterization of a U.S. Sheep Scrapie Isolate with Short Incubation Time

http://scrapie-usa.blogspot.com/2009/05/characterization-of-us-sheep-scrapie.html

Friday, May 29, 2009

Seven Deer Test Positive for Chronic Wasting Disease During 2009 Spring Collections in Hampshire County, West Virginia

http://chronic-wasting-disease.blogspot.com/2009/05/seven-deer-test-positive-for-chronic.html

Tuesday, August 26, 2008

Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3

http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html

Sunday, June 7, 2009

ALZHEIMER'S DISEASE IS TRANSMISSIBLE

http://betaamyloidcjd.blogspot.com/2009/06/alzheimers-disease-is-transmissible.html

Saturday, March 22, 2008

10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures

http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html

http://betaamyloidcjd.blogspot.com/

see also BMJ year 2000 ;

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... Terry S Singeltary (2 January 2000)

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

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

Terry S Singeltary retired Send response to journal: Re: U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well...

In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."

A few more factors to consider, page 15;

"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."

"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."

"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."

Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow. Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.

To be continued...

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

ALL Human and Animal Transmissible Spongiform Encephalopathy, of all phenotypes, of ALL ages, in EVERY State and INTERNATIONALLY, should be made MANDATORY reportable ASAP. to be continued...

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

The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD) Case Report

2009-04-27T12:42:00.000-07:00

Case Report The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD)

Akiyo Shinde, 1 Takenobu Kunieda, 1 Yoshimi Kinoshita, 1 Reika Wate, 1 Satoshi Nakano, 1 Hidefumi Ito, 1 Masahito Yamada, 2 Tetsuyuki Kitamoto, 3 Yosikazu Nakamura, 4 Sadayuki Matsumoto 5 and Hirofumi Kusaka 1 1 Department of Neurology, Kansai Medical University, Moriguchi, 2 Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, 3 Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, Sendai, 4 Department of Public Health – Inquiry, Jichi Medical University, Shimotsuke, and 5 Department of Neurology, Kitano Hospital, Osaka, Japan Correspondence to Akiyo Shinde, md, Department of Neurology, Kansai Medical University, Fumizono-cho 10-15, Moriguchi 570-8506, Japan. Email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:shindea@takii.kmu.ac.jp This case was presented in a preliminary form in the annual meeting of Japanese Neuropathological Association in Tokyo, 2008.

Copyright © 2009 Japanese Society of Neuropathology KEYWORDS latent period • periodic synchronous discharge • pulvinar sign • spongiform encephalopathy • variant Creutzfeldt–Jakob disease

ABSTRACT

Eleven years after a brief visit to some European countries, a 48-year-old Japanese man developed writing difficulty, irritability and general fatigue. Then he complained of dysesthetic pains in his legs, for which benzodiazepines were prescribed. However, at the time pulvinar sign was retrospectively confirmed on brain MRI. Eighteen months after the onset, his gait became ataxic with rapid deterioration of mental status over the following several months. Thirty-one months after the onset, he became akinetic and mute with periodic synchronous discharges on EEG, and died at the age of 51. The total clinical course was approximately 43 months. Pathological examination revealed the characteristic alterations of spongiform encephalopathy, severe in the thalamus, moderate but widely spread in the cerebral cortices, and moderate in the cerebellum. Abundant amyloid plaques were easily identified in the cerebral cortex and the cerebellum on HE staining. Immunohistochemistry for abnormal prion protein (PrPsc) confirmed amyloid plaques in several forms, such as florid, uni- and multi-centric plaques as well as perineuronal and periaxonal deposits in the basal ganglia and synaptic patterns in the thalami. A Western blotting study identified type 2B protease-resistant PrP. This is the first Japanese patient who was definitely diagnosed as variant Creutzfeldt-Jakob disease (vCJD). The pathological findings were similar to those of previous reports of vCJD in the UK. However, the changes were much more severe both in degree and distribution, probably due to a longer duration of the illness than those in the UK.

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

Received 5 November 2008; revised and accepted 7 January 2009.

DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1440-1789.2009.01006.x About DOI

http://www3.interscience.wiley.com/journal/122241574/abstract?CRETRY=1&SRETRY=0

http://www.ncbi.nlm.nih.gov/pubmed/19389077?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

----- Original Message -----
From: "TERRY SINGELTARY" <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:flounder9@VERIZON.NET>
To: <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG>
Sent: Tuesday, March 10, 2009 9:03 PM
Subject: [BSE-L] JAPAN-Local governments to carry on BSE testing despite subsidy cuts

-------------------- mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000276/!x-usc:mailto:BSE-L@LISTS.AEGEE.ORG --------------------

11/03/2009 00:54:58

Japan-BSE Testing.

JAPAN-Local governments to carry on BSE testing despite subsidy cuts

Every local government across the country with meat inspection facilities will continue to test all beef cows for mad cow disease during the next fiscal year, a Mainichi survey has found.

The finding comes despite the central government's abolition of about 200 million yen in annual subsidies to local governments for bovine spongiform encephalopathy (BSE) tests. Japan is the only country where all beef cows are tested for the disease.

In August 2005, the Health, Labor and Welfare Ministry deemed that there is no need for BSE tests on cows 20 months old or younger, on the grounds that no cow born before January 2002 has been found infected with BSE and that there is little chance of finding BSE in such young cows even if they have been infected.

Nevertheless, the ministry had extended subsidies to local governments conducting BSE tests on all beef cows until July last year.

Officials in charge at all 77 prefectural and municipal governments that have beef inspection facilities said they will continue BSE tests on all beef cows in fiscal 2009.

Among the reasons given was the need to "maintain the brand image of their locally produced beef" and "prevent confusion in the marketing process."

However, 30 government bodies said that there was no discussion on whether to continue testing. The survey also suggested that governments tend to abide by the policy of their peers and requests from local residents.

"It would take a lot of nerve to stop it while other prefectures are continuing it," said an official at the Akita Prefectural Government.

"We'd like to stop it but we can't gain support from local residents," a Miyagi Prefectural Government representative said. An official at the Yokohama Municipal Government said that the national government needs to take the initiative in convincing the public of the safety of beef.

The Toyohashi Municipal Government in Aichi Prefecture called on the national government to organize a nationwide BSE testing system. "Since beef is marketed in widespread areas, there is no point in conducting inspections on them unless they are coordinated.."

The government has also applied with the World Organization for Animal Health to raise its evaluation of Japan's BSE countermeasures from the lowest level of "a country whose BSE risk is unknown" to the middle level of "a country having a controlled BSE risk.."

Japan filed the application after it was decided to abolish a practice called "pithing" at all meat treatment centers across the country by the end of this fiscal year. In pithing, a wire is inserted into the cow's head to destroy the brains and spinal marrow and to prevent them from thrashing around. The practice is feared to raise the risk of BSE infections.

The government expects its application to be approved at a general meeting of the organization to be held in May this year.

http://www.farminguk.com/news/Japan-BSE-Testing.12958.asp

I applaud Japans effort to continue to try and eradicate BSE (TSE) i.e. mad cow from their herds. A far cry as to what the USDA has done here in the USA. they did just the opposite. the truth hurts sometimes when reality sets in $$$

WITHOUT a doubt, IF the USA, Canada, and Mexico can have a terribly flawed favorable rating, even though they are BSE GBR risk factor III, and even at that it was on flawed data, with all this, why not Japan being as controlled as the USA and North America ??? it's all about money is it not $$$ that's what Prusiner et al told the hearing committee in California ;

DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN

''they don't wanna know, the dont' care''

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

----- Original Message -----

From: TERRY SINGELTARY
To: mailto:BSE-L@LISTS.AEGEE.ORG
Cc:
Sent: Friday, March 06, 2009 4:27 PM
Subject: Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom

Journal of Veterinary Medical Science

Vol. 71 (2009) , No. 2 February pp.133-138

Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom

Katsuaki SUGIURA1), Toyoko KUSAMA1), Tomotaro YOSHIDA1), Naoki SHINODA1) and Takashi ONODERA2)

1) Food and Agricultural Materials Inspection Center 2) Department of Molecular Immunology, University of Tokyo

(Received 10-Mar-2008) (Accepted 3-Sep-2008)

ABSTRACT. All cattle imported from the United Kingdom to Japan since 1980 and slaughtered before 2002 were traced (n=33), and the number of cattle that were possibly infected with BSE and entered the animal feed chain was calculated. Because there was no effective system to avoid recycling of the BSE agent via animal feed until the early 1990s, of the 33 cattle imported from the UK into Japan, most probably 7 or 8 were infected and entered the animal feed chain, 2 of which entered the animal feed chain in each of 1992 and 1993. In terms of infectivity, 400-550 cattle oral ID50 of the BSE agent entered the feed chain in each of these years. The amount of infectivity that entered the feed chain in 1989, 1991 and 1995 was smaller but still substantial, suggesting that the BSE agent might have entered the Japanese feed chain in any of these years.

KEY WORDS: bovine spongiform encephalopathy (BSE), import risk analysis, Japan, live cattle, simulation

snip...

http://www.jstage.jst.go.jp/article/jvms/71/2/133/_pdf

REFERENCES

http://www.jstage.jst.go.jp/article/jvms/71/2/71_133/_cit

Greetings BSE-L members !

Because there was no effective system to avoid recycling of the BSE agent via animal feed until the early 1990s, of the 33 cattle imported from the UK into Japan, most probably 7 or 8 were infected and entered the animal feed chain, 2 of which entered the animal feed chain in each of 1992 and 1993. In terms of infectivity, 400-550 cattle oral ID50 of the BSE agent entered the feed chain in each of these years. The amount of infectivity that entered the feed chain in 1989, 1991 and 1995 was smaller but still substantial, suggesting that the BSE agent might have entered the Japanese feed chain in any of these years.<<< usa =" 496" canada =" 198" href="http://www.inspection.gc.ca/english/sci/ahra/bseris/bserise.pdf">http://www.inspection.gc.ca/english/sci/ahra/bseris/bserise.pdf

HERE is another look at all the imports for both the USA and Canada of UK live cattle and greaves exports ;

UK Exports of Live Cattle by Value 1986-96

USA 697 LIVE CATTLE

CANADA 299 LIVE CATTLE

http://www.bseinquiry.gov.uk/files/mb/m11f/tab11.pdf

UK EXPORTS OF MBM TO WORLD

http://www.bseinquiry.gov.uk/files/mb/m11g/tab05.pdf

OTHERS

SNIP...

*** SEE FULL TEXT ;

Risk of Introduction of BSE into Japan by the Historical Importation of Live Cattle from the United Kingdom

http://bseusa.blogspot.com/2009/03/risk-of-introduction-of-bse-into-japan.html

SEE FULL TEXT HERE ;

Tuesday, March 10, 2009

JAPAN-Local governments to carry on BSE testing despite subsidy cuts

http://madcowtesting.blogspot.com/2009/03/japan-local-governments-to-carry-on-bse.html

USA

Monday, April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.html

TSS

Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN

2009-04-21T08:32:00.000-07:00

CESM lamenta la muerte del anatomopatólogo Ruiz Villaespesa, relacionada con el ejercicio de su profesión

La Confederación Estatal de Sindicatos Médicos (CESM) lamenta profundamente la muerte del doctor Antonio Ruiz Villaespesa, fallecido según todos los indicios por la enfermedad de Creutzfeldt-Jakob, y pide que desde todas las instancias concernidas se extremen las medidas de protección adecuadas para que casos así nunca vuelvan a producirse.

Hay que recordar que este jefe del servicio de Anatomía Patológica del Hospital Universitario Príncipe de Asturias (Alcalá de Henares), era además neuropatólogo y llevaba acumuladas más de 500 necropsias relativas a esta subespecialidad, de lo que se deduce que pudo entrar en más de una ocasión en contacto con polvo óseo procedente de cráneos humanos afectados por la Encefalopatía Espongiforme Transmisible Humana (Creutzfeldt-Jakob).

Es sabido, por otra parte, que esta enfermedad no sólo se transmite por comer carne o entrar en contacto directo con los tejidos de de animales contaminados. Hay otra modalidad de transmisión, la iatrogénica o CJD, que se produce a partir de procesos quirúrgicos, como trasplante de córnea de donantes infectados o la trepanación craneal para realizar necropsias. En estos casos, a los que estuvo expuesto en más de una ocasión el compañero fallecido, se produce un polvo óseo a través del cual pueden difundirse priones (partículas más pequeñas que los virus) que una vez inhalados son capaces de desencadenar el síndrome referido.

Dado que estamos ante un tipo de enfermedad profesional así reconocido, CESM no duda de que el doctor Ruiz Villaespesa contaba con todos los recursos necesarios para protegerse de la transmisión, aunque como organización que tiene entre sus fines velar por las condiciones en que se lleva a cabo el trabajo de los médicos, sí considera conveniente hacer una llamada de atención para que existan todas las garantías al respecto.

En cuanto al compañero fallecido sólo nos resta extender nuestro pesar a su familia y rendirle tributo a él personalmente por haber muerto a causa de su entrega a la ciencia. Sin duda él sabía el riesgo a que se exponía, y lo afrontó en beneficio de su profesión y de los pacientes a los que se debía. Que descanse en paz.

Noticias relacionadas:

En España habrá unos 20 afectados por el mal de las "vacas locas", pero Villaescusa no está entre ellos, según Badiola/30-03-2009

Fallece el jefe de Anatomía Patológica del Hospital de Alcalá de Henares por posible Creutzfeldt-Jakob /30-03-2009

http://www.cesm.org/nueva/index.asp?pag=detallenoticia.asp&formid=505932&categoria=34

Confederación Estatal de Sindicatos Médicos (CESM) lamenta profundamente la muerte del doctor Antonio Ruiz Villaespesa, fallecido a causa de la enfermedad de Creutzfeldt-Jakob, y pide que desde todas las instancias concernidas se extremen las medidas de protección adecuadas para que casos así nunca vuelvan a producirse. Hay que recordar que este jefe del servicio de Anatomía Patológica del Hospital Universitario Príncipe de Asturias (Alcalá de Henares), era además neuropatólogo y llevaba acumuladas más de 500 autopsias clínicas, de lo que se deduce que pudo entrar en más de una ocasión en contacto con polvo óseo procedente de cráneos humanos afectados por la Encefalopatía Espongiforme Transmisible Humana (Creutzfeldt-Jakob). Es sabido, por otra parte, que esta enfermedad no sólo se transmite por comer carne o entrar en contacto directo con los tejidos de de animales contaminados. Hay otra modalidad de transmisión, la iatrogénica o CJD, que se produce a partir de procesos quirúrgicos, como trasplante de córnea de donantes infectados o la trepanación craneal para realizar necropsias. En estos casos, a los que estuvo expuesto en más de una ocasión el compañero fallecido, se produce un polvo óseo a través del cual pueden difundirse priones (partículas más pequeñas que los virus) que una vez inhalados son capaces de desencadenar el síndrome referido. Dado que estamos ante un tipo de enfermedad profesional así reconocido, CESM no duda de que el doctor Ruiz Villaespesa contaba con todos los recursos necesarios para protegerse de la transmisión, aunque como organización que tiene entre sus fines velar por las condiciones en que se lleva a cabo el trabajo de los médicos, sí considera conveniente hacer una llamada de atención para que existan todas las garantías al respecto. En cuanto al compañero fallecido sólo nos resta extender nuestro pesar a su familia y rendirle tributo a él personalmente por haber muerto a causa de su entrega a la ciencia. Sin duda él sabía el riesgo a que se exponía, y lo afrontó en beneficio de su profesión y de los pacientes a los que se debía. Que descanse en paz. Noticias relacionadas: Fallece el jefe de Anatomía Patológica del Hospital de Alcalá de Henares por posible Creutzfeldt-Jakob /30-03-2009 lunes, 30 de marzo de 2009. Publicado por: CESM

http://www.simeg.org/noticiasanteriores/noticias2009/documents/CESM30-03-09.pdf

In English

State confederation of Medical Unions (CESM) is sorry deeply death of doctor Antonio Ruiz Villaespesa, deceased because of the disease of Creutzfeldt-Jakob, and asks that from all the concerned instances the suitable measures of protection are carried far so that cases thus never return a to take place. It is necessary to remember that this head of the service of Pathological Anatomy of the University Hospital Prince of Asturias (Alcala de Henares), he was in addition neuropatólogo and it took accumulated more than 500 clinical autopsies, than it is deduced that it could enter more than an occasion in contact with bony dust coming from human skulls affected by Human Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob). It is known, on the other hand, that this disease not only is transmitted to eat meat or to enter direct bonding with weaves of of contaminated animal. There is another modality of transmission, iatrogenic or the CJD, that takes place from surgical processes, as transplant of cornea of infected donors or cranial trepanation to realise post-mortem examinations. In these cases, to which it was exposed in more than an occasion the passed away companion, takes place a bony dust through what they can spread priones (particles smaller than the virus) that once inhaled is able to trigger the referred syndrome. Since we are before a type of professional disease thus recognized, CESM nondoubt that doctor Ruiz Villaespesa counted on all the resources necessary to protect themselves of the transmission, although like organization whom it has between his aims velar by the conditions in which the work of the doctors is carried out, yes considers advisable to make a call of attention so that all the exist guarantees on the matter. As far as the passed away companion to only it remains us to extend our grief to his family and to render tribute to him to him personally by to have died because of his delivery to science. Without a doubt he knew the risk to that he was exposed, confronted and it to the benefit of its profession and of the patients to whom it had. That it rests peacefully. The related news: The head of Pathological Anatomy of the Hospital of Alcala de Henares by possible Creutzfeldt-Jakob /30-03-2009 passes away Monday, 30 of March of 2009. Published by: CESM

En España habrá unos 20 afectados por el mal de las "vacas locas", pero Villaespesa no está entre ellos, según Badiola

Cree que este experto fue "víctima de su propia profesión", aunque de ser así el contagio se habría producido hace más de 15 años

MADRID, 30 Mar. (EUROPA PRESS) - El director del Centro Nacional de Referencia de las Encefalopatías Espongiformes Transmisibles, Juan José Badiola, afirmó hoy que en España está previsto que se diagnostiquen en los próximos años entre 15 y 20 casos por la variante en humanos de la encefalopatía espongiforme de Creutzfeldt-Jakob (EETH), conocido como el mal de las ´vacas locas´, aunque no cree que entre ellos esté el jefe del Servicio de Anatomía Patológica del Hospital Universitario Príncipe de Asturias, Antonio Ruiz Villaespesa, que falleció este viernes por causas que todavía se desconocen.

Badiola quiso distinguir así entre la variante humana de esta enfermedad, relacionada con la ingesta de carne con tejido nervioso infectado y de la que ya se han detectado cinco casos en España desde 2005 y más de 160 en Reino Unido, y otros tipos como el esporádico o iatrogénico, en cuyo caso la muerte de este experto estaría causada por la exposición a tejidos de pacientes humanos infectados en el transcurso de su trabajo.

Según el diagnóstico principal y falta de los resultados que determinen el origen de la enfermedad, el Gobierno de la Comunidad de Madrid afirma que la muerte de Ruiz Villaespesa estaría asociada a cualquiera de estas dos últimas variantes.

"Parece que este experto murió víctima de su propia profesión", explicó Badiola, quien matizó que de ser así el contagio se habría producido hace más de 15 años, ya que es éste el tiempo que tarda en incubarse la enfermedad hasta que aparece los primeros síntomas.

Además, de confirmarse esta hipótesis, Badiola aseguró que "hace 20 años no existían los mismos protocolos de seguridad que ahora" por lo que considera normal que se pudiera haber producido algún contagio, "sobre todo en un hombre que realizó tantas autopsias en su vida como él".

Desde el año 2001 se han registrado en España 702 casos de EETH, de los cuales 87 se han notificado en la Comunidad de Madrid, y sólo cinco son la variante humana de esta enfermedad, conocida desde hace más de un siglo pero cuyos estudios se intensificaron tras la aparición de una nueva variante en los años 90 en Inglaterra.

Sin embargo, y dado el periodo de incubación de la variante humana de la enfermedad, Badiola aseguró que se seguirán detectando algunos casos más, aunque todos ellos se tratarán de contagios precedentes a la entrada en vigor de la red de vigilancia establecido en toda Europa a raíz de los primeros casos en Reino Unido.

lunes, 30 de marzo de 2009.

Publicado por: CESM

http://www.cesm.org/nueva/index.asp?pag=detallenoticia.asp&formid=505936&categoria=34

Fallece el jefe de Anatomía Patológica del Hospital de Alcalá de Henares por posible Creutzfeldt-Jakob

El jefe del Servicio de Anatomía Patológica del Hospital General de Alcalá de Henares, Antonio Ruiz Villaespesa, falleció el sábado en dicho centro sanitario diagnosticado de posible enfermedad de Creutzfeldt-Jakob, según informó el sábado el Gobierno regional madrileño y recogen EUROPA PRESS y demás medios. Según el diagnóstico principal, se trata de un caso calificado de probable Creutzfeldt-Jakob, bien esporádico o iatrogénico. Dada la profesión del fallecido, habrá que establecer si la enfermedad ha sido causada por la exposición a tejidos de pacientes humanos infectados en el transcurso de su trabajo. En estos momentos se están realizando las pruebas necesarias para proceder al diagnóstico definitivo del tipo de EETH, lo que se confirmará con un estudio posmorten o necropsia.

Este doctor, de prestigio nacional e internacional en los campos de la anamopatología y la neuropatología, dedicó gran parte de su vida profesional al estudio de la Encefalopatía Espongiforme Transmisible Humana (EETH).

Su actividad profesional se desarrolló desde los años 70, primero en el hospital de La Paz, y posteriormente, en el Príncipe de Asturias, habiendo dedicado toda su vida a la sanidad pública y al diagnóstico de enfermedades.

Más información: EL PAIS, EL MUNDO

lunes, 30 de marzo de 2009.

Publicado por: CESM

http://www.cesm.org/nueva/index.asp?pag=detallenoticia.asp&formid=505930&categoria=34

Se estudian las causas del contagio Fallece el jefe de Anatomía Patológica del Hospital de Alcalá de Henares por posible Creutzfeldt-Jakob

MADRID, 29 Mar (EUROPA PRESS) El jefe del Servicio de Anatomía Patológica del Hospital General de Alcalá de Henares, Antonio Ruiz Villaescusa, falleció el sábado en dicho centro sanitario diagnosticado de posible enfermedad de Creutzfeldt-Jakob, según informó ayer el Gobierno regional madrileño.

Este doctor, de prestigio nacional e internacional en los campos de la anamopatología y la neuropatología, dedicó gran parte de su vida profesional al estudio de la Encefalopatía Espongiforme Transmisible Humana (EETH).

Su actividad profesional se desarrolló desde los años 70, primero en el hospital de La Paz, y posteriormente, en el Príncipe de Asturias, habiendo dedicado toda su vida a la sanidad pública y al diagnóstico de enfermedades.

Según el diagnóstico principal, se trata de un caso calificado de probable Creutzfeldt-Jakob, bien esporádico o iatrogénico. Dada la profesión del fallecido, habrá que establecer si la enfermedad ha sido causada por la exposición a tejidos de pacientes humanos infectados en el transcurso de su trabajo.

En estos momentos se están realizando las pruebas necesarias para proceder al diagnóstico definitivo del tipo de EETH, lo que se confirmará con un estudio posmorten o necropsia.

En principio, el caso no está relacionado con la ingesta de carne contaminada por priones (una partícula transmisible), que se asocia con la variante humana de Creutzfeldt-Jakob (vECJ), ya que se ha demostrado que hay "considerables diferencias" en la manifestación clínica y la duración de la enfermedad desde su diagnóstico hasta la defunción del paciente.

Desde el año 2001 se han registrado en España 702 casos de EETH, de los cuales 87 se han notificado en la Comunidad de Madrid.

La Encefalopatía Espongiforme Transmisible Humana (Creutzfeldt-Jakob) es una enfermedad conocida desde hace más de un siglo, pero los estudios sobre la misma se intensificaron tras la aparición de una nueva variante en los años 90 en Inglaterra.

El Jefe de Servicio de Anatomía Patológica de la Fundación Alcorcón, el doctor Rábano, se encargará de realizar la autopsia, cuyos resultados finales se conocerán aproximadamente en un mes.

La Consejería de Sanidad de la Comunidad de Madrid ya ha puesto en conocimiento de la Dirección General de Salud Pública del Ministerio de Sanidad dicho fallecimiento.

http://www.europapress.es/madrid/noticia-fallece-jefe-anatomia-patologica-hospital-alcala-henares-posible-creutzfeldt-jakob-20090329140143.html

stats for Spain as follows as of March 10, 2009. nvCJD holds at 5 documented to date, and 3 probable cases of sporadic CJD to date. does not mean that a case of nvCJD could not transmit, and or has not transmitted via 'friendly fire' i.e. the medical and or surgical and or occupational arena, and or what that pathology might look like via 2nd, 3rd, 4th passaged. will it continue to look like nvCJD or will it look like sporadic CJD and or any of the subtypes there of ? this case in question as ProMED said, has a great deal of information lacking to date. hopefully, a short report will be forth coming from the Instituto de Salud Carlos III. until then, i thought some of you might be interested in the recent stats. page 12 of 23 has a good chart. on the sporadic cjd cases, in terms of 3 to date, that was for 2009 'probable'. the reference to the nvCJD was a total of 5.....terry

(Situación a 10 de marzo de 2009) ;

REGISTRO NACIONAL DE ENCEFALOPATÍAS ESPONGIFORMES TRANSMISIBLES HUMANAS C.N.E y Servicios de Vigilancia Epidemiológica de CCAA (Situación a 10 de marzo de 2009)

http://www.isciii.es/htdocs/pdf/DatosRegistroCreutzfeldJacob2.pdf

ALSO, please note ;

SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)

SEAC

Agenda

102nd Meeting on Wednesday 4 March 2009

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

10.05 Approval of draft minutes from SEAC 101

snip...

ITEM 3 - CURRENT ISSUES 8. SEAC was informed about the following issues: . A mother and son in Spain had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. As both the mother and son lived in a region of Spain with a history of BSE, had frequently shared meals of cattle brain, and as no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure. Furthermore, the similar times of onset of disease of the cases did not suggest transmission had occurred from one to the other.

snip...

Thursday, February 26, 2009

SEAC 102nd Meeting on Wednesday 4 March 2009 (SEE DH risk assessment on sourcing and pooling plasma)

see full text ;

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

http://seac992007.blogspot.com/2009/02/seac-102nd-meeting-on-wednesday-4-march.html

Tuesday, August 12, 2008

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html

kind regards, terry

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

Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain

2009-03-31T11:15:00.000-07:00

Research Article

Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain

Jean-Philippe Brandel, MD 1 2 3 *, Craig A. Heath, MD 4, Mark W. Head, PhD 5, Etienne Levavasseur, PhD 2, Richard Knight, MD 5, Jean-Louis Laplanche, PharmD, PhD 6, Jan PM. Langeveld, PhD 7, James W. Ironside, MD 5, Jean-Jacques Hauw, MD 2 8, Jan Mackenzie 5, Annick Alpérovitch, MD 1, Robert G. Will, MD 5, Stéphane Haïk, MD, PhD 2 3 8 1Institut National de la Santé et de la Recherche Médicale, Equipe Avenir Human Prion Diseases, Paris, F-75013, France 2Institut National de la Santé et de la Recherche Médicale, U 708, Paris, F-75013, France 3Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Paris, F-75013, France 4Department of Neurology, Ninewells Hospital, Dundee, DD1 9S7, UK 5National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK 6Assistance Publique-Hôpitaux de Paris, Hôpital Lariboisière, Service de Biochimie et de Biologie Moléculaire, Paris, F-75010, France 7Central Veterinary Institute of Wageningen UR (CVI), NL-8200 AB, Lelystad, The Netherlands 8Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Laboratoire Raymond Escourolle, Paris, F-75013, France

email: Jean-Philippe Brandel (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000061/!x-usc:mailto:jean-philippe.brandel@psl.aphp.fr)

*Correspondence to Jean-Philippe Brandel, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13 France

Potential conflict of interest: Nothing to report.

Funded by: Department of Health and the Scottish Government; Grant Number: R40500 la Région Ile de France; Grant Number: F-07-691/R Neuroprion Network of Excellence; Grant Number: FOOD-CT-2004-506579 DG SANCO; Grant Number: 2003201

Abstract

Objective

Variant Creutzfeldt-Jakob disease (vCJD) was first reported in the United Kingdom in 1996. Since then, the majority of cases have been observed in the United Kingdom where there was a major epidemic of bovine spongiform encephalopathy. France was the second country affected. To address the hypothesis of the involvement of a common strain of agent, we have compared clinical, neuropathological, and biochemical data on vCJD patients from both countries.

Methods

In France and the United Kingdom, epidemiological and clinical data were obtained from analysis of medical records and direct interview of the family of the patients using the same standardized questionnaire in both countries. When brain material was available, we performed with similar methods a comparative study of brain lesions and PrPres glycoform ratios in both vCJD populations.

Results

Clinical data, genetic background, neuropathological finding, and biochemical findings in the 185 patients observed in France (n = 23) and the United Kingdom (n = 162) were similar except for age at death. Currently, blood transfusion is a risk factor identified only in the United Kingdom.

Interpretation

The close similarity between the cases of vCJD in France and the United Kingdom supports the hypothesis that a common strain of infectious agent is involved in both countries. The 5-year delay in the peak that we observed in France compared with the United Kingdom fits well with the increase in the importation of beef products to France from the United Kingdom between 1985 and 1995. Ann Neurol 2009;65:249-256

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

Received: 20 May 2008; Revised: 9 September 2008; Accepted: 17 October 2008 Digital Object Identifier (DOI)

10.1002/ana.21583 About DOI

http://www3.interscience.wiley.com/journal/122261459/abstract?CRETRY=1&SRETRY=0

one strain, two very close geographical locations, so what about the other TSE strains atypical BSEs, which are more virulent than the typical c-BSE, what about the other TSE in other species ???

do they transmit to man ??? or not $$$

how long will this UKBSEnvCJD only charade continue ???

Saturday, January 24, 2009

Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

2008 Annual Report

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

Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

see full text ;

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

Wednesday, February 04, 2009

Creutzfeldt-Jacob disease presenting as severe depression: a case report

http://creutzfeldt-jakob-disease.blogspot.com/2009/02/creutzfeldt-jacob-disease-presenting-as.html

Thursday, March 19, 2009

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

Wednesday, March 18, 2009

Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse Bioassay

http://chronic-wasting-disease.blogspot.com/2009/03/detection-of-cwd-prions-in-urine-and.html

PLEASE NOTE, THEY ARE NOT RECALLING ALL THIS CWD POSITIVE ELK MEAT FROM COMMERCE FOR THE ELKS WELL BEING. think again ???

RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

___________________________________

PRODUCT

a) Elk Meat, Elk Tenderloin, Frozen in plastic vacuum packaging. Each package is approximately 2 lbs., and each case is approximately 16 lbs.; Item number 755125, Recall # F-129-9;

b) Elk Meat, Elk Trim, Frozen; Item number 755155, Recall # F-130-9;

c) Elk Meat, French Rack, Chilled. Item number 755132, Recall # F-131-9;

d) Elk Meat, Nude Denver Leg. Item number 755122, Recall # F-132-9;

e) Elk Meat, New York Strip Steak, Chilled. Item number 755128, Recall # F-133-9;

f) Elk Meat, Flank Steak Frozen. Item number 755131, Recall # F-134-9;

CODE

Elk Meats with production dates of December 29, 30, and 31

RECALLING FIRM/MANUFACTURER

Recalling Firm: Sierra Meats, Reno, NV, by telephone on January 29, 2009 and press release on February 9, 2009.

Manufacturer: Noah's Ark Holding, LLC, Dawson, MN. Firm initiated recall is ongoing.

REASON

Elk products contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD).

VOLUME OF PRODUCT IN COMMERCE

Unknown

DISTRIBUTION

NV, CA, TX, CO, NY, UT, FL, OK

___________________________________

http://www.fda.gov/bbs/topics/ENFORCE/2009/ENF01099.html

Monday, February 09, 2009

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

snip...

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST

our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.

http://www.jbc.org/

snip...

Clearly, it is premature to draw firm conclusions about CWD passing naturally into humans, cattle and sheep, but the present results suggest that CWD transmissions to humans would be as limited by PrP incompatibility as transmissions of BSE or sheep scrapie to humans. Although there is no evidence that sheep scrapie has affected humans, it is likely that BSE has caused variant CJD in 74 people (definite and probable variant CJD cases to date according to the UK CJD Surveillance Unit). Given the presumably large number of people exposed to BSE infectivity, the susceptibility of humans may still be very low compared with cattle, which would be consistent with the relatively inefficient conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently been infected by BSE, it would seem prudent to take reasonable measures to limit exposure of humans (as well as sheep and cattle) to CWD infectivity as has been recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml

snip

http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

From: TSS (216-119-163-189.ipset45.wt.net) Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam, In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

Ermias Belay, M.D. Centers for Disease Control and Prevention

-----Original Message----- From: Sent: Sunday, September 29, 2002 10:15 AM To: [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask] Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

snip...

full text ;

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

Wednesday, March 18, 2009 Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html

When Atypical Scrapie cross species barriers

Authors

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

Content

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

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

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

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

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

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

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

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

SCRAPIE USA

INFECTED AND SOURCE FLOCKS

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

POSITIVE SCRAPIE CASES

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

CAPRINE SCRAPIE PREVALENCE STUDY (CSPS)

snip...

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

ANIMALS SAMPLED FOR SCRAPIE TESTING

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

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

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

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

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

CHAPTER 3 Animal Disease Eradication Programs and Control and Certification Programs

snip...

In FY 2007, two field cases, one validation study case, and two RSSS cases were consistent with a variant of the disease known as Nor98 scrapie.1 These five cases originated from flocks in California, Minnesota, Colorado, Wyoming, and Indiana, respectively.

snip...

http://www.aphis.usda.gov/publications/animal_health/content/printable_version/AHR_Web_PDF_07/D_Chapter_3.pdf

NOR-98 Scrapie FY 2008 to date 1

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

Monday, September 1, 2008

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

September 1, 2008

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

P03.141

Aspects of the Cerebellar Neuropathology in Nor98

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

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

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

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

Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

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

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

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

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

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

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

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

CONFIDENTIAL PAPER No: SEAC 78/9 Amendment 2 2 In March 2002, a SEAC Sub-Group considered the risks associated with certain genotypes entering the food chain if BSE were ever isolated from sheep. In contrast to the SSC opinion, SEAC concluded that: • In line with previous SEAC advice, only animals carrying the ARR allele should enter the food chain • On a precautionary basis, the 12 month cut off previously advised by SEAC remained appropriate for ARR heterozygotes. However, in view of existing SRM regulations there was no justification for any age cut off in ARR homozygotes • In line with SEAC advice in 2001, only milk from ARR homozygous sheep could be considered as highly unlikely to contain the infectious agent. Further experimental work was required before potential risks from small ruminant milk from goats and semi-resistant or susceptible sheep could be excluded. There is therefore a disparity of opinion between the SSC and SEAC on this issue. Whilst recognising the uncertainties relating to the science in this area, it is important that contingency planning is based on the most up to date scientific developments and assessments of risk that are available. SEAC will be presented with an update on the ongoing BSE in sheep studies, funded by Defra (Annex 31). This covering paper also provides a history of previous SEAC advice on this issue. BACKGROUND...snip...end

http://www.seac.gov.uk/papers/78-9-closed.pdf

12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie The Problem

Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it is fatal. It is enzootic in the United Kingdom but not in all countries.

The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.

It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasized by the finding that some strains of scrapie produce lesions identical to the once which characterize the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the scrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=6997404&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVAbstractPlus

Wednesday, January 28, 2009

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

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

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

IS THERE A SCRAPIE-LIKE DISEASE IN CATTLE ?

In April of 1985, a mink rancher in Wisconsin reported a debilitating neurologic disease in his herd which we diagnosed as TME by histopathologic findings confirmed by experimental transmission to mink and squirrel monkeys. The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle and a few horses. She had never been fed.

We believe that these findings may indicate the presence of a previously unrecognized scrapie-like disease in cattle and wish to alert dairy practitioners to this possibility.

snip...

PROCEEDINGS OF THE SEVENTH ANNUAL WESTERN CONFERENCE FOR FOOD ANIMAL VETERINARY MEDICINE, University of Arizona, March 17-19, 1986

http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf

http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf

IS THERE A SCRAPIE-LIKE DISEASE IN CATTLE ?

YOU BET THERE IS, AND HAS BEEN, AND WE BEEN FEEDING THE MOST HIGH RISK I.E. DEAD STOCK DOWNER COWS TO OUR CHILDREN FOR DECADES, who will follow these children for human TSE from mad cow disease here in the USA in the years, decades to come, and how many will they expose from the 'pass it forward' friendly fire modes ???

http://downercattle.blogspot.com/2008/12/evaluation-of-fsis-management-controls.html

http://downercattle.blogspot.com/

Saturday, March 14, 2009 Agriculture Secretary Tom Vilsack Announces Final Rule for Handling of Non-Ambulatory Cattle

Release No. 0060.09 Contact: Amanda Eamich (202) 720-9113

http://downercattle.blogspot.com/2009/03/agriculture-secretary-tom-vilsack.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 Manuscript 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://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf

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

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

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

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/

TSS

CJD SURVEILLANCE UK FIFTEENTH ANNUAL REPORT 2006

2007-11-30T14:24:00.000-08:00

CJD SURVEILLANCE UK FIFTEENTH ANNUAL REPORT 2006

SUMMARY

The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK wasinitiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became aWHO Collaborative Centre for Reference and Research on the surveillance and epidemiologyof human transmissible spongiform encephalopathies (TSEs). In September 2001 theNational Care Team was formed, which currently comprises a care coordinator and a secretary. Itis based within the NCJDSU and was formed in response to concerns regarding the care of CJDpatients.

The information provided in this fifteenth report continues to provide evidence of a good level ofcase ascertainment. There has been a lower number of referrals since 2003 but analysis suggeststhat much, if not all, of the decline is due to changes in the number of referrals who turn out notto be CJD cases. The number of sporadic cases remains relatively stable (the data for 2006 maystill be incomplete). Detailed clinical and epidemiological information has been obtained for thegreat majority of patients. Although the post mortem rate for patients with suspected CJD hasdeclined, in line with general autopsy rates in the UK, it remains high (around 60%). The numberof brain specimens examined in the neuropathology laboratory for sporadic CJD declined from 52in 2003 to 32 in 2004 but has remained stable (at 32) in both 2005 and 2006.

In 1990-2006 mortality rates from sporadic CJD in England, Wales, Scotland and NorthernIreland were, respectively, 0.89, 0.95, 0.95 and 0.57/million/year. The differences between theserates are not statistically significant (p>0.6). The mortality rates from sporadic CJD in the UK arecomparable to those observed in most other European countries and elsewhere in the world,including countries that are free of BSE. The highest and lowest mortality rates from sporadicCJD were observed in the South West (SMR=132) and Northern Ireland (SMR=77). Thevariation in the observed mortality rates between the different regions within the UK is notstatistically significant (p>0.1).

Up to 31 December 2006, there were 158 deaths from definite or probable variant CJD (vCJD) inthe UK. Of these, 112 were confirmed by neuropathology. A further 7 probable cases were aliveon 31st December 2006. The clinical, neuropathological and epidemiological features of thesecases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factorsfor the development of vCJD include age, residence in the UK and methionine homozygosity atcodon 129 of the prion protein gene - all 145 clinically affected cases of vCJD with availablegenetic analysis have been methionine homozygotes. The incidence of vCJD is higher in thenorth of the UK than in the south. Analysis of the incidence of vCJD onsets and deaths fromJanuary 1994 to December 2006 indicates that a peak has been passed. While this is anencouraging finding, the incidence of vCJD may increase again, particularly if different geneticsubgroups with longer incubation periods exist. The identification of disease-related PrP in thespleen of a clinically unaffected blood recipient of PRNP-129 MV genotype is not inconsistent

Fifteenth Annual Report 2006 4

with such an hypothesis. This case, along with the report of the prevalence of abnormal PrP inthe large study of appendix and tonsil tissues, suggests the possibility of a greater number ofpreclinical or subclinical cases in the population than might be indicated by the present numbers ofconfirmed clinical cases.

The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire.All geographically associated cases of vCJD are considered for investigation according to aprotocol which involves the NCJDSU, colleagues at the HPA, HPS and local public healthphysicians.

The activities of the NCJDSU are strengthened by collaboration with other surveillance projects,including the Transfusion Medicine Epidemiology Review and the study of ProgressiveIntellectual and Neurological Deterioration in Children. The collaboration of our colleagues inthese projects is greatly appreciated; the effectiveness of this collaboration allowed theidentification in 2003 of a case of vCJD associated with blood transfusion and the identification in2004 of PrPres in the spleen of a recipient of blood donated by someone incubating vCJD. In2006 a further two cases of vCJD associated with blood transfusion were identified.The success of the National CJD Surveillance Project continues to depend on the extraordinarylevel of co-operation from the neuroscience community and other medical and paramedical staffthroughout the UK. We are particularly grateful to the relatives of patients for their help with thisstudy.

snip...

please see full text 42 pages at link below. ...tss

http://www.cjd.ed.ac.uk/report15.pdf

PRION DISEASE UPDATE 2007 (07)******************************A ProMED-mail post<http://www.promedmail.org>ProMED-mail is a program of theInternational Society for Infectious Diseases<http://www.isid.org>

[Note: With continuing decline of the number of cases of variantCreutzfeldt-Jacob disease (abbreviated previously as vCJD or CJD (newvar.) in ProMED-mail) in the human population, it has been decided tobroaden the scope of the occasional ProMED-mail reports to includeother prion-related diseases. These updates supersede the previousupdate thread. ...

SNIP...

******

[2] USA: National Prion Disease Pathology Surveillance CenterDate: June 2007Source: National Prion Disease Pathology Surveillance Center (USA) [edited]<http://www.cjdsurveillance.com/pdf/case-table.pdf>

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

1996 / 42 / 32 / 26 / 4 / 0 / 01997 / 115 / 68 / 57 / 9 / 0 / 01998 / 93 / 53 / 45 / 7 / 1 / 01999 / 114 / 69 / 61 / 8 / 0 / 02000 / 151 / 103 / 89 / 14 / 0 / 02001 / 208 / 116 / 106 / 9 / 0 / 02002 / 255 / 143 / 118 / 23 / 2 / 02003 / 272 / 174 / 132 / 41 / 0 / 02004 / 334 / 183 / 157 / 21 / 0 / 1*2005 / 352 / 195 / 152 / 37 / 1 / 02006 / 372 / 186 / 143 / 30 / 0 / 1**2007 / 120 / 68 / 35 / 7 / 0 / 0TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2

*Acquired in UK** Acquired in Saudi Arabia*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36type pending (2 from 2005, 8 from 2006, 26 from 2007).

Notes:

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

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

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

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

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

[In submitting these data, Terry S. Singeltary Sr. draws attention tothe steady increase in the "type unknown" category, which, accordingto 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]

[see also:

snip...

************************************************************Become a ProMED-mail Premium Subscriber at<http://www.isid.org/ProMEDMail_Premium.shtml>************************************************************Visit ProMED-mail's web site at <http://www.promedmail.org>.

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

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotypeof 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

He estimates that it may be up to 14 or 15 persons which display selectivelySPRPSC 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

PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increasein ''TYPE UNKNOWN''. ...TSS

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17inconclusive and 9 pending (1 from 2006, 8from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1from 2001, 1 from 2003, 4 from 2004, 3from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,

*** 26 from 2007)

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

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

doi:10.1016/S1473-3099(03)00715-1Copyright © 2003 Published by Elsevier Ltd.Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost mymom to hvCJD (Heidenhain variant CJD)and have been searching for answers ever since. What I have found is that wehave not been told the truth. CWDin deer and elk is a small portion of a much bigger problem.”............................

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf

see history of cjd questionnaire

http://cjdquestionnaire.blogspot.com/

Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and theUKBSEnvCJD only theoryDate: January 29, 2006 at 9:03 am PST

Comments sent via JAMA Feedback Page------------------------------------------------------------NAME: Terry S. Singeltary Sr.E-MAIL: flounder9@verizon.netIP ADDRESS: xxxxxxxxHOSTNAME: xxxxxxxxPREVIOUS PAGE: http://jama.ama-assn.org/misc/authors.dtlBROWSER: Mozilla/5.0 (Windows; U; Win98; en-US; rv:1.0.2) Gecko/20030208Netscape/7.02PROMOTIONAL USE: (not answered)

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJDonly theory

TSEs have been rampant in the USA for decades in many species, and they allhave been rendered and fed back to animals for human/animal consumption.propose that the current diagnostic criteria for human TSEs only enhancesand helps the spreading of human TSE from the continued belief of theUKBSEnvCJD only theory in 2007. With all the science to date refuting it, tocontinue to validate this myth, will only spread this TSE agent through amultitude of potential routes and sources i.e. consumption, surgical, blood,medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey,Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more,that the world of TSE Transmissible Spongiform Encephalopathy is far from anexact science, but there is enough provenscience to date that this myth should be put to rest once and for all, andthat we move forward with a new classification for human and animal TSE thatwould properly identify the infected species, the source species, and thenthe route.

This would further have to be broken down to strain of species and then theroute of transmission would further have to be broken down. Accumulation andTransmission are key to the threshold from sub-clinical to clinical disease,and key to all this, is to stop the amplification and transmission of thisagent, the spreading of, no matter what strain. In my opinion, to continuewith this myth that the U.K. strain of BSE (one strain TSE in cows), and thenv/v CJD (one strain TSE humans) and that all the rest of human TSE are justone single strain i.e. sporadic CJD (when to date there are 6 differentphenotypes of sCJD, and growing per Gambetti et al), and that no otheranimal TSE transmits to humans, to continue with this masquerade will onlycontinue to spread, expose, and kill, who knows how many more in the yearsand decades to come. ONE was enough for me, My Mom, hvCJD i.e. HeidenhainVariant CJD, DOD 12/14/97 confirmed, which is nothing more than another mansname added to CJD, like CJD itself, Jakob and Creutzfeldt, orGerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE,named after another human.

WE are only kidding ourselves with the current diagnostic criteria for humanand animal TSE, especially differentiating between the nvCJD vs the sporadicCJDstrains and then the GSS strains and also the FFI fatal familial insomniastrains or the ones that mimics one or the other of those TSE? Tissueinfectivity and strain typing of the many variants of the human and animalTSEs are paramount in all variants of all TSE. There must be a properclassification that will differentiate between all these human TSE in orderto do this. With the CDI and other more sensitive testing coming about, Ionly hope that my proposal will some day be taken seriously. ...

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

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease inthe United States

http://cjdusa.blogspot.com/

i am reminded of a few things deep throat (high ranking official at usda)told me years ago;

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

The most frightening thing I have read all day is thereport of Gambetti's finding of a new strain ofsporadic cjd in young people.........Dear God,

https://www.blogger.com/comment.g?blogID=7842737484277562285&postID=5759550357128128100

BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS

http://madcowtesting.blogspot.com/

MADCOW USDA the untold story

http://madcowusda.blogspot.com/

MADCOW USDA the untold story continued

https://www.blogger.com/comment.g?blogID=6472149427883113751&postID=4829467681293855400

USA NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES

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

Government Accountability Project

https://www.blogger.com/comment.g?blogID=3995372399492420922&postID=295754279213239559

Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/

TME hyper/drowsy, INTER-SPECIES TRANSMISSION CWD and strainproperties

https://www.blogger.com/comment.g?blogID=37955408&postID=116577315153980667

USA NVCJD BLOOD RECALLS ONLY ;

http://www.google.com/search?hl=en&q=CJD+BLOOD+RECALLS+TSS&btnG=Search

vCJD case study highlights blood transfusion risk

http://vcjdblood.blogspot.com/

CREUTZFELDT JAKOB DISEASE MAD COW BASE, CWD, SCRAPIE UPDATE OCT 2007

http://cjdmadcowbaseoct2007.blogspot.com/

TSEAC MEETINGS

http://tseac.blogspot.com/

ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

Date: Mon, 24 Sep 2007 21:31:55 -0500

I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,blood, and some of the other abstracts from the PRION2007. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744

*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OFPOKER INDEED GOES UP. ...TSS

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling ittoday), please note that both the ALABAMA COW, AND THE TEXAS COW, both were''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 200711:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779

From: "Terry S. Singeltary Sr."Subject: CWD UPDATE 88 AUGUST 31, 2007

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&P=450

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

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

2007-10-25T12:07:00.000-07:00

Dear Dr. Singeltary,

According to our Information for Authors and Correspondence policy, we can only consider Correspondence written about articles published within the last six weeks. Please consider Neurology again in the future.

Sincerely,

Morgan Serry

Neurology Editorial Office

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

VIEWS & REVIEWS: Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, and Lawrence B. Schonberger

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Neurology 2003; 60: 176-181 [Abstract] [Full text] [PDF]

Correspondence ID: neurology_el;17008

Article ID: 60/2/176 Article Date: 28 January 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 25 October 2007

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

I URGE you to consider my response once again. I think I have been proven correct now.

3 MONTHS LATER IN A BOOK INTERVIEW, VENDICATION OF SORTS

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/

IN REALITY, sporadic CJD is 1 in 9,000 in 50 years of age and above, and that's with a inadequate or what I call passive surveillance system. see below ;

Dr. William Shulaw, a veterinarian with The Ohio State University extension service, is involved in a nationwide program to eradicate scrapie, the form of BSE found in sheep.

Shulaw said the chances of a person getting sporadic Creutzfeldt- Jakob disease is about one in a million. But that's the total population, infants, children, adults and the elderly. Chances increase to one in 9,000 when the group is restricted to those age 50 and older.

http://www.pjstar.com/stories/082207/REG_BE523NH6.049.php

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

FURTHER into my journal of neurology article, some years later, again, sadly, I was proven correct ;

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html

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

and today we indeed find ;

PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increase in ''TYPE UNKNOWN'' CJD IN THE USA ;

1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1 from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006, *** 26 from 2007)

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

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

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of theUKBSEnvCJD only theory in 2007. With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential and proven routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, 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.

This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of the TSE agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE (one strain TSE in cows), and the nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. HeidenhainVariant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human.

With the H-BASE atypical BSE strain, and the Nor-98 atypical Scrapie strain, BOTH now documented in the USA, and BOTH more closely related pathologically to the sporadic CJD, rather than the BSE strain, I once again urge you to make all human TSE strains in the USA reportable in every state, and Internationally, with a written mandatory CJD/TSE questionnaire asking real questions pertaining to route and source of the TSE agent. This is paramount for iCJD i.e. friendly fire, and secondary transmissions via the medical, dental, and surgical arena alone.

WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? 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. ...

flounder9@verizon.net

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

CJD NEWS

http://disc.server.com/Indices/236650.html

CJD VOICE (voice for _all_ victims of human TSE)

http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm

SOURCE

Subject: PRION2007 ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007 Date: September 24, 2007 at 6:52 pm PST

P02.35 Molecular Features of the Protease-resistant Prion Protein (PrPres) in H- type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE- infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H- type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C- terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) *** reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

FC5.5.1 BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc C-terminal Truncated Fragments

Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux, MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4; Monaco, S3 1University of Verona, of Neurological and Visual Sciences, Italy; 2CEA, IMETI/SEPIA, France; 3University of Verona, Neurological and Visual Sciences, Italy; 4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps Research Insitute, USA

The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human prion disease, remains still unknown. The marked disease phenotype heterogeneity observed in sCJD is thought to be influenced by the type of proteinase K- resistant prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V) codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE) and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type, distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all genotypes,;

(ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V- 2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD subtype M/V-2 shared molecular and pathological features with an atypical form of BSE, named BASE, thus suggesting a potential link between the two conditions. This connection was further confirmed after 2D-PAGE analysis, which showed an identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally inoculated with brain homogenates from BASE. Samples were separated by using a twodimensional electrophoresis (2D-PAGE) followed by immunoblotting. We here show that the PrPSc pattern obtained in infected primates is identical to BASE and sCJD MV-2 subtype. *** These data strongly support the link, or at least a common ancestry, between a sCJD subtype and BASE. This work was supported by Neuroprion (FOOD-CT-2004-506579)

*******************************************************

USA MAD COW CASES IN ALABAMA AND TEXAS

***PLEASE NOTE***

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were ''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007 11:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet- mg&T=0&P=19779

*******************************************************

FC5.5.2 Transmission of Italian BSE and BASE Isolates in Cattle Results into a Typical BSE Phenotype and a Muscle Wasting Disease

Zanusso, G1; Lombardi, G2; Casalone, C3; D’Angelo, A4; Gelmetti, D2; Torcoli, G2; Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3; Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2 1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER, Italy; 3IZSPLVA, Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta, Italy

The clinical phenotype of bovine spongiform encephalopathy has been extensively reported in early accounts of the disorder. Following the introduction of statutory active surveillance, almost all BSE cases have been diagnosed on a pathological/molecular basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance system has uncovered atypical BSE cases, which are characterized by distinct conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose clinicopathological phenotypes remain unknown. We recently reported two Italian atypical cases with a PrPSc type similar to BSE-L, pathologically characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A major limitation of transmission studies to mice is the lack of reliable information on clinical phenotype of BASE in its natural host. In the present study, we experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates by i.c. route. BASE infected cattle showed survival times significantly shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with previous observations in TgBov mice. Clinically, BSE-infected cattle developed a disease phenotype highly comparable with that described in field BSE cases and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle developed an amyotrophic disorder accompanied by mental dullness. The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle *** and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease. Oral Abstracts 14

P04.49

Case Report of Variant Creutzfeldt-Jakob Disease in a Macaque after Blood Transfusion

Lescoutra-Etchegaray, N1; Ruchoux, MM1; Correia, E1; Jolit, A1; Freire, S1; Lasmezas, CI2; Deslys, JP1; Comoy, E1 1CEA/DSV/IMETI/SEPIA, France; 2Scripps Florida, USA

A fourth human case of probable transmission of vCJD through transfusion has now been reported but a number of features affecting transfusion-related infection remain imprecise, including infectious dose, length of incubation period and critical infectious window of blood donors.

We report here the first case of experimental transmission of vCJD in primates by blood transfusion. Experimental infection of Cynomolgus macaque has been demonstrated to be a sensitive model for the investigation of human prion diseases, inducing similar distribution of infectivity in peripheral lymphoid tissues and equivalent brain pathology. In our study, transfusion was performed with 40 ml of whole blood drawn from a vCJD-infected macaque at the terminal stage of the disease. Clinical symptoms of vCJD appeared in the recipient animal after five years of incubation. The total amount of infectivity in the transfused blood was approximately 106 fold lower than in the brain (titration still in progress). In several animals infected intravenously with brain homogenate, the presence of PrPres in serial lymph nodes biopsies and in other organs at autopsy was examined and results will be presented.

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

P04.51

Atypical Presentation of Variant Creutzfeldt-Jakob Disease in a 73 Year Old Blood Transfusion Recipient

Wroe, S1; Pal, S1; Webb, T1; Alner, K2; Hewitt, P3; Brander, S4; Wadsworth, JD5; Collinge, J1 1National Hospital for Neurology and Neurosurgery, National Prion Clinic, UK; 2National Hospital for Neurology and Neurosurgery, Department of Neuropsychology, UK; 3Health Protection Agency, UK; 4National Hospital for Neurology and Neurosurgery, Department of Neuropathology, UK; 5Institute of Neurology, UCL, UK

We report atypical presentation of variant Creutzfeldt-Jakob Disease (vCJD) identified ante-mortem in a 73 year-old recipient of blood products. This patient was transfused following orthopaedic surgery in December 1997. Tracing of blood products identified a single unit of non-leucodepleted red cells from an individual who developed neuropathologically confirmed vCJD eleven months after donation. Nine years post transfusion, this individual was referred to the National Prion Clinic for specialist investigation. Six years post transfusion the recipient complained of fluctuating fatigue and impaired concentration. At this time neurological examination and MRI brain (T1/T2 weighted/DWI) were normal. Progressive symptoms emerged six months later with imbalance and deteriorating cognition. Examination two months after onset of neurological symptoms demonstrated cognitive deficits, dyspraxia or visuospatial dysfunction and normal motor, sensory and gait examination. Six weeks later cognitive impairment was identified alongside tremulousness, impaired manual dexterity and limb ataxia. Serological investigations were normal. MRI (T1/T2 weighted/FLAIR/DWI) demonstrated prominent signal change throughout the dorsal thalamus, consistent with vCJD. PRNP genotyping revealed no mutations and homozygosity for methionine at codon 129. The prolonged incubation period of vCJD and possibility of asymptomatic carrier states pose major public health concerns. This case highlights the significant risk encountered by recipients of contaminated blood products and the necessity for their specialist monitoring.

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

P04.36

Enhanced Surveillance of Persons Identified as at Increased Risk of CJD Due to Blood Transfusion or Healthcare Procedures

Brookes, D1; Chow, Y1; Ward, HJT2; Will, RG2; Hewitt, P3; Gill, ON1 1HPA, CJD, UK; 2National CJD Surveillance Unit, UK; 3Colindale, NHS Blood and Tissue, UK

Introduction:

Reports of four iatrogenic transmissions of variant-CJD (vCJD) infection in the UK (all due to transfusion of blood from donors who later developed vCJD), evidence from iatrogenic transmissions of sporadic CJD and experimental work on CJD infectivity in tissues and on healthcare instruments have given rise to concern about the risks of iatrogenic transmission of CJD. This risk warrants a) certain public health precautions, and b) follow-up of individuals with identified risks in order to gain evidence about their risks and ensure appropriate management of these risks. Evidence of transmission via iatrogenic routes is important to inform public health measures and so prevent ongoing transmission of CJD.

Methods:

The Health Protection Agency and Health Protection Scotland holds details of persons identified as ‘at-risk’ of vCJD due to blood transfusion and of persons identified as ‘at-risk’ of CJD (of any type) from other healthcare procedures. The GPs/clinicians of all persons identified as ‘at-risk’ for public health purposes are provided with: information; risk assessment updates; advice on public health precautions and advice on referral to specialist care. Procedures are being established to obtain enhanced surveillance data on these individuals, including: clinical status updates, date and cause of death, surplus tissue and blood specimens, and postmortem investigations.

Results:

Persons ‘at-risk’ of CJD have experienced a range of exposures. Estimated risks are uncertain and overlapping. Some individuals - recipients of vCJD implicated blood components - are considered to be at a clearly higher risk of infection: active follow-up is currently conducted for these individuals. In time, the enhanced surveillance of persons at increased risk of CJD will provide estimates of transmission risks and of the impact of iatrogenic exposures on mortality. Conclusion: Knowledge about iatrogenic transmission of CJD is being gained by the follow-up of individuals who have been identified as ‘at-risk’ of CJD in the UK. This enhanced surveillance may need to be sustained for many years.

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

Prions in Plasma of Patients with Sporadic Creutzfeldt-Jakob Disease Safar, J.G1,3, Geschwindm M.D2,3, Letessier, F1, Kuo, A1, Pomeroy, K1, Deering, C1, Serban, A1, Groth, D1, Miller, B.L2,3, DeArmond, S.J1,4, Prusiner, S.B1,3,5 1Institute for Neurodegenerative Diseases, 2Memory and Aging Center, Departments of 3Neurology, 4Pathology, and 5Biochemistry and Biophysics, University of California, San Francisco, California 94143

http://www.prion2007.com/pdf/Prion%20Friday.pdf

http://www.prion2007.com/pdf/Prion%20Thursday.pdf

http://www.prion2007.com/pdf/Prion%20Wednesday.pdf

October 3, 2007

October 2007 Update on Feed Enforcement Activities to Limit the Spread of BSE

To help prevent the establishment and amplification of Bovine Spongiform Encephalophathy (BSE) through feed in the United States, the Food and Drug Administration (FDA) implemented a final rule that prohibits the use of most mammalian protein in feeds for ruminant animals. This rule, Title 21 Part 589.2000 of the Code of Federal Regulations, here called the Ruminant Feed Ban, became effective on August 4, 1997.

The following is an update on FDA enforcement activities regarding the ruminant feed ban. FDA's Center for Veterinary Medicine (CVM) has assembled data from the inspections that have been conducted AND whose final inspection report has been recorded in the FDA's inspection database as of September 29, 2007. As of September 29, 2007, FDA had received over 57,000 inspection reports. The majority of these inspections (around 69%) were conducted by State feed safety officials, with the remainder conducted by FDA officials.

Inspections conducted by FDA or State investigators are classified to reflect the compliance status at the time of the inspection based upon the objectionable conditions documented. These inspection conclusions are reported as Official Action Indicated (OAI), Voluntary Action Indicated (VAI), or No Action Indicated (NAI).

An OAI inspection classification occurs when significant objectionable conditions or practices were found and regulatory sanctions are warranted in order to address the establishment's lack of compliance with the regulation. An example of an OAI inspection classification would be findings of manufacturing procedures insufficient to ensure that ruminant feed is not contaminated with prohibited material. Inspections classified with OAI violations will be promptly re-inspected following the regulatory sanctions to determine whether adequate corrective actions have been implemented.

A VAI inspection classification occurs when objectionable conditions or practices were found that do not meet the threshold of regulatory significance, but do warrant advisory actions to inform the establishment of findings that should be voluntarily corrected. Inspections classified with VAI violations are more technical violations of the Ruminant Feed Ban. These include provisions such as minor recordkeeping lapses and conditions involving non-ruminant feeds.

An NAI inspection classification occurs when no objectionable conditions or practices were found during the inspection or the significance of the documented objectionable conditions found does not justify further actions. The results to date are reported here both by “segment of industry” and “in total”. NOTE – A single firm can operate as more than one firm type. As a result, the categories of the different industry segments are not mutually exclusive.

RENDERERS

These firms are the first to handle and process (i.e., render) animal proteins and to send these processed materials to feed mills and/or protein blenders for use as a feed ingredient.

Number of active firms whose initial inspection has been reported to FDA – 266

Number of active firms handling materials prohibited from use in ruminant feed – 155 (58% of those active firms inspected)

Of the 155 active firms handling prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

6 firms (3.9%) were classified as VAI

LICENSED FEED MILLS

FDA licenses these feed mills to produce medicated feed products. The license is required to manufacture and distribute feed using certain potent drug products, usually those requiring some pre-slaughter withdrawal time. This licensing has nothing to do with handling prohibited materials under the feed ban regulation. A medicated feed license from FDA is not required to handle materials prohibited under the Ruminant Feed Ban.

Number of active firms whose initial inspection has been reported to FDA – 1,071

Number of active firms handling materials prohibited from use in ruminant feed – 466 (44% of those active firms inspected)

Of the 466 active firms handling prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

8 firms (1.7%) were classified as VAI

FEED MILLS NOT LICENSED BY FDA

These feed mills are not licensed by the FDA to produce medicated feeds.

Number of active firms whose initial inspection has been reported to FDA – 5,163

Number of active firms handling materials prohibited from use in ruminant feed – 2,481 (48% of those active firms inspected)

Of the 2481 active firms handling prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

46 firms (1.9%) were classified as VAI

PROTEIN BLENDERS

These firms blend rendered animal protein for the purpose of producing quality feed ingredients that will be used by feed mills.

Number of active firms whose initial inspection has been reported to FDA – 392

Number of active firms handling materials prohibited from use in ruminant feed – 191 (49% of those active firms inspected)

Of the 191 active firms handling prohibited materials, their most recent inspection revealed that:

0 firm (0%) was classified as OAI

5 firms (2.6%) were classified as VAI

RENDERERS, FEED MILLS, AND PROTEIN BLENDERS MANUFACTURING WITH PROHIBITED MATERIAL

This category includes only those firms that actually use prohibited material to manufacture, process, or blend animal feed or feed ingredients.

Total number of active renderers, feed mills, and protein blenders whose initial inspection has been reported to FDA – 6,577

Number of active renderers, feed mills, and protein blenders processing with prohibited materials – 493 (7.5%)

Of the 493 active renderers, feed mills, and protein blenders processing with prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

24 firms (4.9%) were classified as VAI

OTHER FIRMS INSPECTED

Examples of such firms include ruminant feeders, on-farm mixers, pet food manufacturers, animal feed salvagers, distributors, retailers, and animal feed transporters.

Number of active firms whose initial inspection has been reported to FDA – 18,358

Number of active firms handling materials prohibited from use in ruminant feed – 5,911 (32% of those active firms inspected)

Of the 5911 active firms handling prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

177 firms (3.0%) were classified as VAI

TOTAL FIRMS

Note that a single firm can be reported under more than one firm category; therefore, the summation of the individual OAI/VAI firm categories will be more than the actual total number of OAI/VAI firms, as presented below.

Number of active firms whose initial inspection has been reported to FDA – 20,807

Number of active firms handling materials prohibited from use in ruminant feed – 6,602 (32% of those active firms inspected)

Of the 6602 active firms handling prohibited materials, their most recent inspection revealed that:

0 firms (0%) were classified as OAI

190 firms (2.9%) were classified as VAI

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

Issued by: FDA, Center for Veterinary Medicine, Communications Staff, HFV-12 7519 Standish Place, Rockville, MD 20855 Telephone: (240) 276-9300 FAX: (240) 276-9115 Internet Web Site: http://www.fda.gov/cvm

http://www.fda.gov/cvm/BSE1007.htm

What Do We Feed to Food-Production Animals? A Review of Animal Feed Ingredients and Their Potential Impacts on Human Health

Amy R. Sapkota,1,2 Lisa Y. Lefferts,1,3 Shawn McKenzie,1 and Polly Walker1 1Johns Hopkins Center for a Livable Future, Bloomberg School of Public Health, Baltimore, Maryland, USA; 2Maryland Institute for Applied Environmental Health, College of Health and Human Performance, University of Maryland, College Park, Maryland, USA; 3Lisa Y. Lefferts Consulting, Nellysford, Virginia, USA

snip...

Table 1. Animal feed ingredients that are legally used in U.S. animal feeds

Animal

Rendered animal protein from Meat meal, meat meal tankage, meat and bone meal, poultry meal, animal the slaughter of food by-product meal, dried animal blood, blood meal, feather meal, egg-shell production animals and other meal, hydrolyzed whole poultry, hydrolyzed hair, bone marrow, and animal animals digest from dead, dying, diseased, or disabled animals including deer and elk Animal waste Dried ruminant waste, dried swine waste, dried poultry litter, and undried processed animal waste products

snip...

Sapkota et al. 668 VOLUME 115 NUMBER 5 May 2007 • Environmental Health Perspectives

http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1867957&blobtype=pdf

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

___________________________________

PRODUCT Bulk cattle feed made with recalled Darling’s 85% Blood Meal, Flash Dried, Recall # V-024-2007 CODE Cattle feed delivered between 01/12/2007 and 01/26/2007 RECALLING FIRM/MANUFACTURER Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007. Firm initiated recall is ongoing. REASON Blood meal used to make cattle feed was recalled because it was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI

___________________________________

PRODUCT Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot-Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI – 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J – PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK- MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A-BYPASS ML W/SMARTA, Recall # V-025-2007 CODE The firm does not utilize a code - only shipping documentation with commodity and weights identified. RECALLING FIRM/MANUFACTURER Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete. REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

http://www.fda.gov/bbs/topics/enforce/2007/ENF00996.html

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

PMID: 6997404

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;

Risk of oral infection with bovine spongiform encephalopathy agent in primates

Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to- human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.

snip...

BSE bovine brain inoculum

100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg

Primate (oral route)* 1/2 (50%)

Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%)

RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)

PrPres biochemical detection

The comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal.

Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inocula

Published online January 27, 2005

http://www.thelancet.com/journal/journal.isa

It is clear that the designing scientists must also have shared Mr Bradley’s surprise at the results because all the dose levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s145d.pdf

6. It also appears to me that Mr Bradley’s answer (that it would take less than say 100
grams) was probably given with the benefit of hindsight; particularly if one
considers that later in the same answer Mr Bradley expresses his surprise that it
could take as little of 1 gram of brain to cause BSE by the oral route within the
same species. This information did not become available until the "attack rate"
experiment had been completed in 1995/96. This was a titration experiment
designed to ascertain the infective dose. A range of dosages was used to ensure
that the actual result was within both a lower and an upper limit within the study
and the designing scientists would not have expected all the dose levels to trigger
infection. The dose ranges chosen by the most informed scientists at that time
ranged from 1 gram to three times one hundred grams. It is clear that the designing
scientists must have also shared Mr Bradley’s surprise at the results because all the
dose levels right down to 1 gram triggered infection.

http://www.bseinquiry.gov.uk/files/ws/s147f.pdf

2) Infectious dose:

To cattle: 1 gram of infected brain material (by oral ingestion)

http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml

Subject: Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route Date: September 29, 2007 at 12:50 pm PST

P04.27

Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route

Holznagel, E1; Yutzy, B1; Deslys, J-P2; Lasmézas, C2; Pocchiari, M3; Ingrosso, L3; Bierke, P4; Schulz-Schaeffer, W5; Motzkus, D6; Hunsmann, G6; Löwer, J1 1Paul-Ehrlich-Institut, Germany; 2Commissariat à l´Energie Atomique, France; 3Instituto Superiore di Sanità, Italy; 4Swedish Institute for Infectious Disease control, Sweden; 5Georg August University, Germany; 6German Primate Center, Germany

Background:

In 2001, a study was initiated in primates to assess the risk for humans to contract BSE through contaminated food. For this purpose, BSE brain was titrated in cynomolgus monkeys.

Aims:

The primary objective is the determination of the minimal infectious dose (MID50) for oral exposure to BSE in a simian model, and, by in doing this, to assess the risk for humans. Secondly, we aimed at examining the course of the disease to identify possible biomarkers.

Methods:

Groups with six monkeys each were orally dosed with lowering amounts of BSE brain: 16g, 5g, 0.5g, 0.05g, and 0.005g. In a second titration study, animals were intracerebrally (i.c.) dosed (50, 5, 0.5, 0.05, and 0.005 mg).

Results:

In an ongoing study, a considerable number of high-dosed macaques already developed simian vCJD upon oral or intracerebral exposure or are at the onset of the clinical phase. However, there are differences in the clinical course between orally and intracerebrally infected animals that may influence the detection of biomarkers.

Conclusions:

Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate. The difference in the incubation period between 5 g oral and 5 mg i.c. is only 1 year (5 years versus 4 years). However, there are rapid progressors among orally dosed monkeys that develop simian v CJD as fast as intracerebrally inoculated animals.

The work referenced was performed in partial fulfilment of the study “BSE in primates“ supported by the EU (QLK1-2002-01096).

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

Subject: Aspects of the Cerebellar Neuropathology in Nor98 Date: September 26, 2007 at 4:06 pm PST

P03.141

Aspects of the Cerebellar Neuropathology in Nor98

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

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

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

From: "Terry S. Singeltary Sr." Sent: Tuesday, August 21, 2007 9:50 AM Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringing total to 3 cases to date

Infected and Source Flocks

As of June 30, 2007, there were .....

snip...

One field case and one validation case were consistent with Nor-98 scrapie.

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

IN the February 2007 Scrapie report it only mentions ;

''One case was consistent with Nor98 scrapie.''

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/

Subject: NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 (RISES TO 5 DOCUMENTED CASES IN USA) Date: October 9, 2007 at 7:15 am PST

Greetings, seems the NOR-98 atypical scrapie cases are the rise in the USA. ...tss

INFECTED AND SOURCE FLOCKS

AS of August 31, 2007, there were 33 scrapie infected and source flocks with open statuses (Figure 3). Five new source flocks and one new infected flock were reported n August (Figure 4) with a total of 64 reported for FY 2007 (Figure 5).

snip...

IN FY 2007 TWO FIELD CASES, ONE VALIDATION CASE, AND TWO RSSS CASES WERE CONSISTENT WITH NOR-98 SCRAPIE. ...

(BRINGS A TOTAL OF 5 NOR-98 CASES DOCUMENTED IN 2007 IN USA. ...TSS)

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

(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS USDA, APHIS, VS ET AL. ...TSS)

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553

An evaluation of scrapie surveillance in the United States

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427

FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451

Subject: FSIS NOTICE SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) ONGOING SURVEILLANCE PROGRAM From: "Terry S. Singeltary Sr." Reply-To: Sustainable Agriculture Network Discussion Group Date: Fri, 2 Feb 2007 17:32:58 -0600

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&P=720

ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

Date: Mon, 24 Sep 2007 21:31:55 -0500

I suggest that you all read the data out about h-BASE and sporadic CJD, GSS, blood, and some of the other abstracts from the PRION2007. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744

*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OF POKER INDEED GOES UP. ...TSS

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were ''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007 11:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779

see full text 143 pages ;

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

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non- Ambulatory Disabled Cattle 03-025IFA 03-025IFA-2 9/13/2005

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

THE SEVEN SCIENTIST REPORT ***

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf

PAUL BROWN M.D.

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf

9 December 2005 Division of Dockets Management (RFA-305)

SEROLOGICALS CORPORATION James J. Kramer, Ph.D. Vice President, Corporate Operations

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf

Embassy of Japan

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02N-0273-EC240.htm

Dockets Entered on December 22, 2005 2005D-0330, Guidance for Industry and FDA Review Staff on Collection of Platelets by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...

http://www.fda.gov/ohrms/dockets/dailys/05/Dec05/122205/122205.htm

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

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03- 025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12. http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-631.pdf

Transmissible Spongiform Encephalopathy UPDATE USA OCTOBER 2007

http://www.phxnews.com/fullstory.php?article=53128

CREUTZFELDT JAKOB DISEASE MAD COW BASE, CWD, SCRAPIE UPDATE OCT 2007

http://cjdmadcowbaseoct2007.blogspot.com/

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

flounder9@verizon.net

BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS

http://madcowtesting.blogspot.com/

MADCOW USDA the untold story

http://madcowusda.blogspot.com/

MADCOW USDA the untold story continued

https://www.blogger.com/comment.g?blogID=6472149427883113751&postID=4829467681293855400

USA NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES

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

Government Accountability Project

https://www.blogger.com/comment.g?blogID=3995372399492420922&postID=295754279213239559

Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/

TME hyper/drowsy, INTER-SPECIES TRANSMISSION CWD and strainproperties

https://www.blogger.com/comment.g?blogID=37955408&postID=116577315153980667

USA NVCJD BLOOD RECALLS ONLY ;

http://www.google.com/search?hl=en&q=CJD+BLOOD+RECALLS+TSS&btnG=Search

vCJD case study highlights blood transfusion risk

http://vcjdblood.blogspot.com/

CREUTZFELDT JAKOB DISEASE MAD COW BASE, CWD, SCRAPIE UPDATE OCT 2007

http://cjdmadcowbaseoct2007.blogspot.com/

TSEAC MEETINGS

http://tseac.blogspot.com/

MRSA

http://staphmrsa.blogspot.com/

ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007Date: Mon, 24 Sep 2007 21:31:55 -0500I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,blood, and some of the other abstracts from the PRION2007. ...

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744

*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OFPOKER INDEED GOES UP. ...TSSUSA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling ittoday), please note that both the ALABAMA COW, AND THE TEXAS COW, both were''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 200711:52 PM. ...TSS

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779

From: "Terry S. Singeltary Sr."Subject: CWD UPDATE 88 AUGUST 31, 2007

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&P=450

PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increasein ''TYPE UNKNOWN''. ...TSS1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17inconclusive and 9 pending (1 from 2006, 8from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1from 2001, 1 from 2003, 4 from 2004, 3from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,*** 26 from 2007)

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

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

doi:10.1016/S1473-3099(03)00715-1Copyright © 2003 Published by Elsevier Ltd.Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463“

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.”............................

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf

see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408

if you will notice on the forms page on the cjd foundation site, there is not cjd questionnaire ??? strange...

http://www.cjdsurveillance.com/forms.html

vCJD questionnaire

http://www.bseinquiry.gov.uk/report/volume8/pdf/1stquestionnaire.pdf

USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/

why is a cjd questionnaire not listed on the cjd foundation 'forms' site with the rest of their forms ??? i thought they had one now going out to all families of victims of a human TSE??? this is very important that a _written_ cjd questionnaire, asking extensive questions pertaining to any potential route and source of the TSE agent be submitted to every family of a victim of a human TSE. this is key to finding cause. so why is this still so difficult? are the families of CJD victims getting these written cjd questionnaires??? i hope so.... 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

FDA FAILED US

http://fdafailedus.blogspot.com/

SCIENCE BUSHWHACKED

http://sciencebushwhacked.blogspot.com/

Sunday, July 20, 2008 Red Cross told to fix blood collection or face charges 15 years after warnings issued, few changes made to ensure safety

http://vcjdblood.blogspot.com/2008/07/red-cross-told-to-fix-blood-collection.html

Sent: Monday May 28, 2007 Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle

03-025IFA03-025IFA-2 Terry S. Singeltary

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

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

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

BRITISH MEDICAL JOURNAL

BMJ

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

BMJ

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

CJD NEWS

http://disc.server.com/Indices/236650.html

CJD VOICE (voice for _all_ victims of human TSE)

http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 Posted by Terry S. Singeltary Sr. at 12:07 PM 1 comments: Terry S. Singeltary Sr. said... i am reminded of a few things deep throat (high ranking official at usda) told me years ago;

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The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

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http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&T=0&P=10326

http://brain.hastypastry.net/forums/archive/index.php/t-5581.html

see full text ;

http://disc.server.com/discussion.cgi?disc=7498;article=3473;title=CJD%20Voice%20Discussion%20Group

TSS