Wednesday, February 1, 2012

Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Update on CJD and VCJD Transmission RG Will

Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Update on CJD and VCJD Transmission RG Will

Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety

Subject: Update on CJD and VCJD Transmission RG Will
Greetings sporadic CJD victims et al in the UK, and around the globe.
Sadly, once again, the UK Prion Gods and nvCJD victims groups in the UK and EU have alienated the sporadic CJD victims and their families, and nothing says it more than RG Will, and his report to the Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety. even though the report title states UPDATE ON CJD AND VCJD TRANSMISSION RG WILL, they play down to low key, with very little data at all on the recent science about sporadic CJD. you would think that with scientist around the globe saying that atypical BSE is now linked with some cases (if not all) of the sporadic CJD cases i.e. 85%+ of all human TSE, you would think that RG Will et al, and the victims group for nvCJD would all put their arms around these victims. BUT NO, instead, they alienate them as a happenstance of bad luck, and refuse to acknowledge update science, instead going by old and outdated science i.e. the UKBSEnvCJD only theory. I was hoping to finally see RG Will et al finally to speak about sporadic CJD and it’s links as a zoonosis disease, instead, we got nothing. IN this report to the industry pharmaceutical groups at the Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety by RG Will titled : Update on CJD and VCJD Transmission RG Will, sporadic CJD was mentioned only ONE TIME, with a title to a paper trying once again to alienate sporadic CJD as being tied to blood even though the Baxter study DID tie GSS to blood ;
Transmission of sporadic Creutzfeldt Jakob Disease by blood transfusion: risk factor or possible biases
NO where else was sporadic CJD mentioned in this report. NO WHERE !
Its very sad that not only scientist like RG Will, but nvCJD victims groups in the UK and EU still to this day in 2012, still they all refuse to wrap their arms around these victims to of sporadic CJD. maybe it’s the risk of loosing any compensation, or maybe afraid if they mingle with the 85%+, they would loose out on compensation, even though I know that some refused this blood money, they still refuse to wrap their arms around the sporadic CJD victms, and stand up for them in public.
it’s sad when you can be bought off, or manipulated $$$
some day will come, when people like RG Will and his UKBSEnvCJD only theory, will have to eat crow, and I hope I am still alive to see it. ...
Update on CJD and VCJD Transmission

RG Will

National CJD Research and Surveillance Unit
Edinburgh, UK
TSE Safety Forum
30th June 2011, Barcelona
Total number of vCJD cases in the UK 175
Number who were eligible to donate (ie >_ aged 17) 165
Number reported by relatives to have been blood donors 32
Number of cases where donor records have been traced 24*
Number of cases from whom components were actually issued 18
Number of recipients identified from 18 cases where recipient
and component information is available 67
*Donor records were traced on four cases where the relatives had reported the case not to be a donor.
One of these had donated while the other 3 were registered as donors but never donated
Recipient Transfusion
Number of donor
Interval from transfusion to onset of illness
1 1 38 4 years, 9 months
1 2 65 4 years, 6 months
2 1 2 15 years, 11 months
2 2 3 6 years, 3 months
3 1 4 5 years, 4 months
4 1 5 8 months1
5 (Case 1) 1 52 6 years, 6 months
6 (Case 3) 1 562 7 years, 10 months
7 1 2 13 years, 11 months
8 1 4 16 years, 9 months
9 (Case 4) 1 212 8 years, 4 months
9 (Case 4) 2 2 7 years, 8 months
10 1 2 5 years, 11 months
1timing of clinical illness excludes blood transfusion as the source of infection in one case.
2one donor developed vCJD.
Patients with inherited bleeding disorders registered in the
National Haemophilia Database on 1.1.2009 by diagnosis and
subgroups at risk of vCJD for public health purposes.
*11 million IUs (about 50%) of implicated batches remain unaccounted for [5]. As a result of this under-notification, it is estimated that the 787 patients represent approximately 50% of all patients wh had received implicated batchs.
Haemophilia (2011), 1-7
Zaman et al Haemophilia
‘604/787 patients were followed up for more than 13 years
following exposure to an implicated batch’
‘For these 604 patients , the estimated vCJD risk is’:
<1% for 595
> 50% for 164
100% for 51
‘94 (16%) received implicated batches from donations within 6
months of clinical onset in the donor’
‘151 (25%) received their first dose under 10 years of age.’
* administered intramuscularly
** prior to the considered start of the vCJD at risk period in 1980
Vox Sanguinis Variant Creutzfeldt-Jakob disease and exposure to fractionated plasma products HJT WARD 19/5/09
UK 172 (4) 3 (0) 175
France 25 (0) – 1
R of Ireland 4 (0) – 2
Italy 2 (0) – 0
USA 3† (0) – 2
Canada 2 (1) – 1
Saudi Arabia 1 (0) – 0
Japan 1* (0) – 0
Netherlands 3 (0) – 0
Portugal 2 (0) – 0
Spain 5 (0) – 0
Taiwan 1 (0) – 1
* the case from Japan had resided in the UK for 24 days in the period 1980-1996.
† the third US patient with vCJD was born and raised in Saudi Arabia and has lived permanently in the United States since
late 2005. According to the US case-report, the patient was most likely infected as a child when living in Saudi Arabia.
Psychiatry and Clinical Neurosciences 2010; 64; 652-658
vCJD case in Taiwan
• Male, aged 34 years
• Duration of illness 28 months
• Psychiatric onset, pain, ataxia, myoclonus, dementia
• Codon 129: MM
• Pulvinar sign +
• No tonsil biopsy or necropsy
• Diagnosis: probable vCJD
Second Canadian vCJD case
• Male, aged 24 years
• Typical history, MRI +, MM genotype
• Tonsil biopsy +
• WB type 2B PrP
• Never donated blood
• No history of iatrogenic exposure
Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Update on CJD and VCJD Transmission RG Will
Greetings again sporadic CJD victims and their Families et al,
Update on CJD and VCJD Transmission RG Will
SO, since RG Will either forgot, or refused to give the world his report on sporadic CJD, I thought I might. as follows ;
see where sporadic CJD cases went from 28 cases in 1990, to a high in 2008 of 88 cases of sporadic CJD, then 2009 dropped back a bit to 78 cases, and then in 2010 the sporadic CJD cases were back up to 84 cases. with a small rise in GSS and Familial cases of human TSE as well, through that time period ???

CJD Figures

These figures show the number of suspect cases referred to the NCJDRSU in Edinburgh, and the number of deaths of definite and probable cases in the UK, from 1 January 1990 up to 4th January 2012
see here ;
SO, we have atypical BSE and atypical Scrapie cases rising, we have sporadic CJD now linked to atypical BSE cases and atypical scrapie cases, and this report does not mention that $$$
BSe, and politics as usual $$$


2011 Monday, September 26, 2011


L-BSE BASE prion and atypical sporadic CJD
Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008

Vol. 37, No. 3-4, 2011 Original Paper Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.


Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD



CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.



USA 2011


National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

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 was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;


5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

THE steady rise of sporadic CJD cases in Canada AND USA, with many unusual cases of ''PENDING CLASSIFICATIONS" which have been pending now FOR 3 YEARS. HOW long can this cover-up continue $$$

The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.



Saturday, March 5, 2011



Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance?

A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey


Dement Geriatr Cogn Dis Extra. 2011 Jan-Dec; 1(1): 429–432. Published online 2011 December 24. doi: 10.1159/000332024 PMCID: PMC3265806

Thursday, January 26, 2012

The Risk of Prion Zoonoses

Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167

Thursday, January 26, 2012

Facilitated Cross-Species Transmission of Prions in Extraneural Tissue

Science 27 January 2012:


Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study

Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria

Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported.

Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious.

Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-Sträussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE).

Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months.

Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.

Saturday, September 5, 2009




Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products


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.

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


Monday, October 10, 2011

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


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.


see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;

P.9.21 Molecular characterization of BSE in Canada

Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.

Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis. Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE. Results and

Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types.

Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.



Saturday, November 19, 2011

Novel Prion Protein in BSE-affected Cattle, Switzerland


Saturday, December 3, 2011

Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number 12—December 2011


Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.


The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


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


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.


12 years independent research of available data


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 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.

Sunday, January 29, 2012
Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Dr. Detwiler
Dr. Detwiler published Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Forum\Presentations TSE\ Page 33 and 34 of 44 ;

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