VARIANT CJD (vCJD) or nvCJD

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Saturday, June 22, 2013

Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes

Characterization of variant Creutzfeldt-Jakob disease prions in prion protein-humanized mice carrying distinct codon 129 genotypes

 

 

 

Atsuko Takeuchi1, Atsushi Kobayashi1, James W. Ironside2, Shirou Mohri3 and Tetsuyuki Kitamoto1* + Author Affiliations

 

 

 

1 Tohoku University Graduate School of Medicine, Japan;

 

2 University of Edinburgh Western General Hospital, United Kingdom;

 

3 National Institute of Animal Health, Japan ↵*

 

Corresponding author; email: kitamoto@med.tohoku.ac.jp

 

 

 

Capsule

 

 

 

Background: Secondary variant Creutzfeldt-Jakob disease (vCJD) infection may occur in all human PRNP genotypes, but its clinicopathological and biochemical phenotype is uncertain.

 

 

 

Results: The biochemical characteristics and transmission properties of the newly generated vCJD prions are not affected by the host PRNP genotypes.

 

 

 

Conclusion: Secondary vCJD infection can be adequately diagnosed by biochemical analysis and experimental transmission.

 

 

 

Significance: Effective means to identify secondary vCJD infection are presented.

 

 

 

 

Abstract

 

 

 

To date, all clinical variant Creutzfeldt-Jakob disease (vCJD) patients are homozygous for methionine at polymorphic codon 129 (129M/M) of the prion protein (PrP) gene. However, the appearance of asymptomatic secondary vCJD infection in individuals with a PRNP codon 129 genotype other than M/M, and transmission studies using animal models have raised the concern that all humans might be susceptible to vCJD prions, especially via secondary infection. To reevaluate this possibility and to analyze in detail the transmission properties of vCJD prions to transgenic animals carrying distinct codon 129 genotype, we performed intracerebral inoculation of vCJD prions to humanized knock-in mice carrying all possible codon 129 genotypes (129M/M, 129M/V, or 129V/V). All humanized knock-in mouse lines were susceptible to vCJD infection, although the attack rate gradually decreased from 129M/M to 129M/V and to 129V/V. The amount of PrP deposition including florid/amyloid plaques in the brain also gradually decreased from 129M/M to 129M/V and to 129V/V. The biochemical properties of protease-resistant abnormal PrP in the brain and transmissibility of these humanized mouse-passaged vCJD prions upon subpassage into knock-in mice expressing bovine PrP were not affected by the codon 129 genotype. These results indicate that individuals with the 129V/V genotype may be more susceptible to secondary vCJD infection than expected, and may lack the neuropathological characteristics observed in vCJD patients with the 129M/M genotype. Besides the molecular typing of protease resistant PrP in the brain, transmission studies using knock-in mice carrying bovine PrP may aid the differential diagnosis of secondary vCJD infection, especially in individuals with the 129V/V genotype.

 

 

 

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DISCUSSION

 

 

 

Here we report a detailed comparison of the transmission properties of vCJD prions among humanized knock-in mice carrying distinct PRNP codon 129 genotypes. All three humanized knock-in mouse lines were susceptible to vCJD infection, although the attack rate gradually decreased from 129M/M to 129M/V to 129V/V. The amount of PrP deposition including florid/amyloid plaques in the brain also gradually decreased from 129M/M to 129M/V to 129V/V. The biochemical properties of PrPres in the brain and the transmissibility of these humanized mouse-passaged vCJD prions upon subpassage into Ki-Bov/Bov were not affected by the codon 129 genotype. These results indicate that individuals with the 129V/V genotype may be more susceptible to secondary vCJD infection than expected and may lack some neuropathological characteristics observed in vCJD patients with the 129M/M genotype.

 

 

 

 

 

These results have potential public health implications concerning the future occurrence of secondary vCJD transmission to individuals carrying the 129M/V or 129V/V genotype. We had expected that Ki-Hu129V/V were highly resistant to vCJD infection because these mice showed negative results when intraperitoneally challenged with vCJD prions (17, 29). In addition, Bishop et al. reported a very low attack rate (1/16 (6.25%)) in humanized knock-in mice with the 129V/V genotype intracerebrally inoculated with vCJD prions (15). However, Ki-Hu129V/V showed moderate susceptibility to intracerebral transmission of vCJD in the present study (the sum total attack rate from two independent experiments using different inocula: 5/12 (41.7%)). This susceptibility is comparable to the reported attack rate in transgenic mice expressing human PrP with 129V (the sum total attack rate from six independent experiments using different inocula: 25/56 (44.6%)) (11, 13). Since the expression level of PrP directly affects the susceptibility to prion infection regardless of the codon 129 genotype, the susceptibility reported in the transgenic mice carrying human PrP with 129V to vCJD prions had been considered to be due to their high PrP expression level (15). However, the present study clearly shows that this is not solely due to the overexpression of PrP. The route of infection in the present study is not that expected for the human-to-human transmission of vCJD, e.g., blood transfusion contaminated with a lower dose of vCJD prions, suggesting that the possible secondary infection might be restricted. Meanwhile, intravenous transmission of BSE is as efficient as the intracerebral inoculation (29). We reported previously that knock-in mice expressing human PrP with heterozygosity for glutamine/lysine at another polymorphic codon 219 (219E/K) are susceptible to vCJD prions (28). Indeed, two vCJD patients with the 219E/K genotype were reported subsequently (30). Therefore, our transmission study using humanized knock-in mice could properly predict that individuals with the 219E/K genotype, which is rarely observed in the European population (31, 32), have a potential risk for vCJD infection. Taken together, the present intracerebral transmission data raises the concern that individuals with the 129V/V genotype are more susceptible to secondary vCJD infection than had been expected.

 

 

 

 

The reason for the fluctuating transmissibility of vCJD prions between the two inocula (vCJD96/02 and 05/02; attack rates in Ki-Hu129V/V were 0/4 (0%) and 5/8 (62.5%), respectively) is unclear. Similar fluctuation in transmissibility among vCJD inocula has been observed in transmission study using transgenic mice (from 0% to 80%) (13). These fluctuations might be due to differences in the prion titers in the inoculum. Western blot analysis of PrPres in the brain of the patients showed that vCJD05/02 had greater amounts of PrPres (about 5 times) than vCJD96/02, perhaps reflecting the clinical course of vCJD05/02 (43 months), which was much longer than that of vCJD96/02 (18 months). The low vCJD attack rate in humanized knock-in mice with the 129V/V genotype reported by another group (15) might be also explained by the prion titer in the inoculum, as their inoculum was diluted to 10-2, whereas our inoculum was a 10-1 dilution. Although typical vCJD cases were selected for this study, further extensive analysis with additional cases having various PrPres concentrations should be carried out in the future.

 

 

 

 

We confirmed that the characteristic neuropathological features of vCJD can be modified through transmission to the 129M/V or 129V/V genotype as reported previously (11, 13-15). Particularly, the amount of PrP deposition and the number of florid/amyloid plaques in the brain, one of the most important clinicopathological hallmarks of vCJD, markedly reduced in Ki-Hu129V/V in the present study. Four out of five (80%) diseased Ki-Hu129V/V lacked florid/amyloid plaques, despite extensive examination of the brain. Similarly, florid/amyloid plaques have never been observed in other mouse models of vCJD carrying the 129V/V (or 129M/V) genotype (11, 14, 15). Although the neuropathological features of humanized knock-in mice with vCJD may not be fully recapitulated in human brain tissue with vCJD, the present study, together with data from other groups, raises the concern that vCJD with the 129V/V genotype cannot be neuropathologically distinguished from sCJD patients with the 129V/V genotype (e.g., sCJD-VV2). In contrast to the neuropathological phenotype, the biochemical properties of PrPres were not altered through transmission to the 129M/V or 129V/V genotype as reported in another knock-in mouse model (15). These results support the view that the molecular typing of PrPres will remain a useful diagnostic feature of secondary vCJD infection irrespective of the codon 129 genotype (15).

 

 

 

 

The present study shows that transmission studies using Ki-Bov/Bov are also a useful means to detect vCJD prions, even in secondary infection. Not only Ki-Hu129M/M[vCJD05/02] but also Ki-Hu129M/V[vCJD05/02] and Ki-Hu129V/V[vCJD05/02] showed positive transmissibility to Ki-Bov/Bov, i.e., the traceback phenomenon, whereas all sCJD or dCJD prions examined failed to be transmitted. These results suggest that BSE prions retain their host preference after repeated passages through human PrP regardless of the codon 129 genotype. Intracerebral transmission generally shows higher sensitivity compared with intraperitoneal transmission, but requires a very long incubation period of over 2 years to obtain results. In contrast, the FDC assay after intraperitoneal inoculation requires only 75 days to assess the transmissibility. In addition, the positive rate of the FDC assay was as high as that of intracerebral transmission in the present study. Therefore, the FDC assay after intraperitoneal inoculation of patient materials to Ki-Bov/Bov may help in the differential diagnosis of vCJD when atypical cases emerge in CJD surveillance (33, 34).

 

 

 

 

In conclusion, the present study underpins the importance of systematic assessment of all human prion disease patients based on the clinicopathological phenotype and molecular typing of PrPres to monitor secondary vCJD infection (13-15). Traceback studies using Ki-Bov/Bov may facilitate the differential diagnosis of secondary vCJD infection, especially in individuals with the PRNP 129V/V genotype.

 

 

 

 

 

 

Amyloid Microbiology Neurobiology Prions Protein misfolding Knock-in mouse codon 129 genotype folicular dendritic cell vCJD Received March 19, 2013. Accepted June 21, 2013. Copyright © 2013, The American Society for Biochemistry and Molecular Biology

 

 

 

 

 


 

 

 

 

 


 

 

 

 

 

 

 

***Although the neuropathological features of humanized knock-in mice with vCJD may not be fully recapitulated in human brain tissue with vCJD, the present study, together with data from other groups, raises the concern that vCJD with the 129V/V genotype cannot be neuropathologically distinguished from sCJD patients with the 129V/V genotype (e.g., sCJD-VV2)....disturbing...how many have been misdiagnosed as sporadic CJD, AND what about iatrogenic potential therefrom ??? ...tss

 

 

 

 

 

 

 

Saturday, September 5, 2009

 

TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS

 

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Saturday, September 5, 2009

 

TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS

 

 snip...see full text ;

 

 

 

 

 

Monday, May 6, 2013

 

Warning of mad cow disease threat to blood transfusions

 


 

 

 

 

 

Tuesday, April 30, 2013

 

Mad cow infected blood 'to kill 1,000’

 


 

 

 

 

 

Sunday, February 10, 2013

 

Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection report/CJD

 


 

 

 

 

 

Tuesday, May 28, 2013

 

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

 


 

 

 

 

 

Thursday, June 6, 2013

 

BSE TSE PRION USDA FDA MAD COW FEED COMPLIANCE REPORT and NAI, OAI, and VAI ratings as at June 5, 2013

 


 

 

 

 

 

Thursday, February 14, 2013

 

The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and TSE prion disease

 


 

 

 

 

 

Sunday, June 9, 2013

 

TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast

 


 

 

 

 

 

Thursday, June 13, 2013

 

*** Experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle

 


 

 

 

 

 

Monday, June 17, 2013

 

Evaluation of a test for its suitability in the diagnosis of variant Creutzfeldt–Jakob disease

 


 

 

 

 

 

Saturday, January 22, 2011

 

Alzheimer's, Prion, and Neurological disease, and the misdiagnosis there of, a review 2011

 


 

 

 

 

 

 

Wednesday, June 19, 2013

 

Spreading of tau pathology in Alzheimer's disease by cell-to-cell transmission

 


 

 

 

 

 

 

Monday, October 10, 2011

 

EFSA Journal 2011 The European Response to BSE: A Success Story

 

snip...

 

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011).

 

*** This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded.

 

Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

 

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Thursday, August 12, 2010

 

Seven main threats for the future linked to prions

 

First threat

 

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

 

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

 

Second threat

 

 

 

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Rural and Regional Affairs and Transport References Committee

 

The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010

 

2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

 

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. *** Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

 


 

 

 

 

 

Wednesday, March 31, 2010

 

Atypical BSE in Cattle

 

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE.

 

When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

 

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

 


 

 

 

 

 

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

 


 

 

 

 

 

Saturday, June 15, 2013

 

Canada Fraser Health Statement on Creutzfeldt-Jakob Disease outbreak

 


 

 

 

 

 

Tuesday, March 5, 2013

 

Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

 

FDA believes current regulation protects the public from BSE but reopens comment period due to new studies

 


 

 

 

 

 

Saturday, December 15, 2012

 

Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012

 


 

 

 

 

 

Wednesday, March 20, 2013

 

GAO-13-244, Mar 18, 2013 Dietary Supplements FDA May Have Opportunities to Expand Its Use of Reported Health Problems to Oversee Product

 

From: Terry S. Singeltary Sr.

 

Sent: Tuesday, March 19, 2013 2:46 PM

 

To: gomezj@gao.gov

 

Cc: siggerudk@gao.gov ; youngc1@gao.gov ; oighotline@gao.gov

 


 

 

 

 

 

 

Thursday, February 14, 2013

 

The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and TSE prion disease

 


 


 

 

 

 

 

 

Thursday, May 30, 2013

 

World Organization for Animal Health (OIE) has upgraded the United States' risk classification for mad cow disease to "negligible" from "controlled", and risk further exposing the globe to the TSE prion mad cow type disease

 

U.S. gets top mad-cow rating from international group and risk further exposing the globe to the TSE prion mad cow type disease

 


 


 

 

 

 

 

 

 Thursday, June 20, 2013

 

atypical, BSE, CWD, Scrapie, Captive Farmed shooting pens (livestock), Wild Cervids, Rectal Mucosa Biopsy 2012 USAHA Proceedings, and CJD TSE prion Update

 


 

 

 

 

 

 

Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'

 

 

 

DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 McCullough Bldg. Galveston, Texas 77555-0785

 

FAX COVER SHEET

 

DATE: 4-23-98

 

TO: Mr. Terry Singeltary @ -------

 

FROM: Gerald Campbell

 

FAX: (409) 772-5315 PHONE: (409) 772-2881

 

Number of Pages (including cover sheet):

 

Message:

 

*CONFIDENTIALITY NOTICE*

 

This document accompanying this transmission contains confidential information belonging to the sender that is legally privileged. This information is intended only for the use of the individual or entry names above. If you are not the intended recipient, you are hereby notified that any disclosure, copying distribution, or the taking of any action in reliances on the contents of this telefaxed information is strictly prohibited. If you received this telefax in error, please notify us by telephone immediately to arrange for return of the original documents. -------------------------- Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name: POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Admitting Race: C

 

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

 

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

 

Autopsy NO.: AU-97-00435

 

AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown Residence: Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97 15:00 Pathologist/Resident: Pencil/Fernandez Service: Private Restriction: Brain only

 

FINAL AUTOPSY DIAGNOSIS

 

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

 

***TYPE: Anatomic(A) or Clinical(C) Diagnosis. IMPORTANCE: 1-immediate cause of death (COD); 2.ureterlying COD; 3-contributory COD: 4.concomitant, significant; 5-incidental ***

 

Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed Date; Time: 01/30/98 - 0832

 

Page: 1 Continued .... --------------

 

UTMB University of Texas Medical Branch Galveston, Texas 77555-

 

 

 


 

 

 

 

 

 

Published March 26, 2003

 

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

 

Terry S. Singeltary, retired (medically)

 

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?

 

Published March 26, 2003

 


 

 

 

 

 

 

Letters

 

JAMA. 2001;285(6):733-734. doi: 10.1001/jama.285.6.733

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

Terry S. Singeltary, Sr Bacliff, Tex

 

Since this article does not have an abstract, we have provided the first 150 words of the full text.

 

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

 

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

 


 

 

 

 

 

 

 

 

THE PATHOLOGICAL PROTEIN

 

BY Philip Yam

 

Yam Philip Yam News Editor Scientific American www.sciam.com

 

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.

 

CHAPTER 14

 

Laying Odds

 

Are prion diseases more prevalent than we thought?

 

Researchers and government officials badly underestimated the threat that mad cow disease posed when it first appeared in Britain. They didn't think bovine spongiform encephalopathy was a zoonosis-an animal disease that can sicken people. The 1996 news that BSE could infect humans with a new form of Creutzfeldt-Jakob disease stunned the world. It also got some biomedical researchers wondering whether sporadic CJD may really be a manifestation of a zoonotic sickness. Might it be caused by the ingestion of prions, as variant CJD is?

 

Revisiting Sporadic CJD

 

It's not hard to get Terry Singeltary going. "I have my conspiracy theories," admitted the 49-year-old Texan.1 Singeltary is probably the nation's most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union's Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow. org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people's health.

 

Singeltary has similar inclinations. ...

 

snip...

 

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.

 


 


 


 


 


 

 

 

14th ICID International Scientific Exchange Brochure -

 

Final Abstract Number: ISE.114

 

Session: International Scientific Exchange

 

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

 

T. Singeltary

 

Bacliff, TX, USA

 

Background:

 

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

 

Methods:

 

12 years independent research of available data

 

Results:

 

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

 

Conclusion:

 

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

 

 

 


 

 

 

 

 

CJD Singeltary submission to PLOS ;

 

No competing interests declared.

 

 

 

see full text ;

 

 

 


 

 

 

 

 


 

 


 

 

 

 

 

 

The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

 

Tracking spongiform encephalopathies in North America

 

Original

 

Xavier Bosch

 

“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.” 49-year—old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt—Jakob ...

 

 

 


 

 

 


 

 

 

 

 

 

SEE FULL TEXT ;

 

 

 

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

 

Subject: Tracking spongiform encephalopathies in North America LANCET INFECTIOUS DISEASE Volume 3, Number 8 01 August 2003

 

Date: Tue, 29 Jul 2003 17:35:30 –0500

 

From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy

 

To: BSE-L@uni-karlsruhe.de

 

Volume 3, Number 8 01 August 2003

 

Newsdesk

 

Tracking spongiform encephalopathies in North America

 

Xavier Bosch

 

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.

 

49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.

 

Singeltary was greatly demoralised at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.

 

Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.

 

To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.

 

Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk.

 

Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.

 

Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.

 

Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modelled on that established in the UK, he points out.

 

Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.

 

CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.

 

 

 

 


 

 

 

 

 

Greetings,

 

 

 

 

> > > he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source. < < <

 

 

 

 

actually, that quote was from a more recent article in the Journal of Neurology (see below), not the JAMA article.

 

 

 

 

 

 

Full Text

 

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

 

snip...end...tss

 

Re: vCJD in the USA * BSE in U.S.

 

15 November 1999 Terry S Singeltary, NA

 

In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease.

 

Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know.

 

My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following;

 

vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD.

 

The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?

 

CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.

 

So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.

 

No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;

 

Since 1990 the U.S. has raised 1,250,880,700 cattle;

 

Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;

 

There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;

 

Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;

 

Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.

 

I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.

 

Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.

 

It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........

 

The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.

 

Terry S. Singeltary Sr.

 

P.O. Box 42, Bacliff, Texas 77518 USA

 

flounder@wt.net

 

Competing interests:None declared

 

 

 

 


 

 

 

 

 

 

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

 

2 January 2000

 

Terry S Singeltary

 

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...

 

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

 

Competing interests:None declared

 

 

 

 


 

 

 

 

 

 

 

 

Sunday, August 21, 2011

 

The British disease, or a disease gone global, The TSE Prion Disease

 

(see video here)

 

 


 

 

 

 

 

 

 

 

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

 

(see video at bottom)

 

 


 

 

 

 

 

 

 

 

Sunday, September 6, 2009

 

MAD COW USA 1997

 

(SEE SECRET VIDEO)

 

 


 

 

 

 

 

 

Creutzfeldt-Jakob Disease Public Health Crisis

 

 

 


 

 

 


 

 

 


 

 

 

 


 

 

 

 

 

 

 

 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 


 

 

 

 

 

 

 

 

just made a promise to mom...back then, there was very little information to the public on the human and animal TSE prion disease. ... layperson...tss