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Posted Aug 09 2009 11:45pm
NCJDSU Scientific Report 2008


Question 7. What is the cause of sporadic CJD (sCJD) ?

What are the risks of secondary transmission of sCJD?

Case control studies

Risk factor information has been collected for cases of sporadic CJD since before the Unit was established in 1990. Over the years various control groups have been recruited; the method chosen depending on the resources available and anticipated validity. These have been detailed in various previous NCJDSU Annual Reports.

Since 1990 there have been five papers published examining risk factors for sCJD that the Unit has either led or collaborated in:-

1) Wientjens et al, Neurology 1996. A meta analysis of three case control studies (178 CJD cases and 333 controls). The results showed an elevated risk of CJD for those with a family history of dementia, a history of poliomyelitis, those employed as health professionals and those exposed to cows and sheep. There was no association with consumption of animal organs, including brain.

2) van Duijn et al, Lancet 1998- this compared 405 CJD cases and 405 hospital controls recruited as part of the 1993-95 EU collaborative studies of CJD in Europe. The findings suggested that genetic factors other than CJD mutations may play an important part in CJD. Iatrogenic transmission seemed rare in the population studied. There was little evidence of association between the risk of CJD and animal exposure or consumption of processed bovine meat or milk products for the period studied.

3) Zerr I et al, J Clin Epid 2000- medical risk factors were examined using the 405 CJD cases and 405 hospital controls recruited as part of the 1993-95 EU collaborative studies of CJD in Europe. The study failed to identify any common medical risk factor for CJD.

4) Ward et al, Neurology 2002- Surgical risk factors from 326 sCJD cases recruited as part of the 1993-95 EU collaborative studies of CJD in Europe were compared with 326 community controls recruited by telephone in 2000. A history of surgery was associated with risk of sCJD and the results supported the hypothesis that sCJD may result from hitherto unrecognised surgical contamination events.


NCJDSU Scientific Report 2008

5) Ward et al, Annals of Neurology 2007- Medical risk factors for 431 sCJD cases resident in the UK and referred to NCJDSU between 1998-2006 were compared with 454 general population control subjects recruited 2002-2003 (see NatCen controls in Appendix 6). This study found some evidence for a link between increased risk of sCJD and surgery, however there was no convincing evidence of temporal-geographical links between cases undergoing neurosurgery or gynaecological surgery. It concluded that it was unlikely that a high proportion of UK sCJD cases were the result of surgical transmission, but the possibility of such transmission cannot be excluded.

As for vCJD (see Questions 2 and 5 above), further work examining data from general practitioner records of cases and controls needs to be carried out in order to accurately determine risk for sCJD associated with medical or surgical procedures. This is on-going, though the priority has been given to vCJD at present.

Blood transfusion

An analysis of the potential for blood transfusion to be a risk factor for the development of sporadic CJD has been undertaken collaboratively by the major EU countries (coordinator M Pocchiari). A prospective study is under consideration.

Genetic factors

Possible genetic factors related to sCJD are being investigated by the Unit, both internally and through external collaboration (see Question 8 below).

Geographical distribution

The investigation of the geographical distribution of sporadic CJD by genetic and molecular subtype is on-going in the Unit.


NCJDSU Scientific Report 2008

Question 8: What are the clinico-pathological, genetic & molecular features of sporadic CJD and how are they related?

While this is an interesting question in its own right, it has an extremely important bearing on the Unit’s core surveillance function, particularly in relation to vCJD. This is because the most important and potentially difficult differential diagnosis of vCJD is sCJD; many initial reports of suspect cases of vCJD in the UK and elsewhere, have been found to be atypical cases of sCJD. A full characterisation of the clinico- pathological, epidemiological and molecular phenotypes of sCJD is therefore clearly essential, which the Unit has continued to carry out within the UK.
Atypical clinical presentations, disease courses and pathological findings are found in only a small percentage of sCJD cases and so are difficult to characterise, even in the UK population of 50-60 million. This is of particular importance in relation to any attempt to recognise new clinical disease phenotypes either related to BSE or, potentially, to other animal diseases. Therefore, our international collaborations have contributed greatly to the analysis of clinical, epidemiological and pathological data. For example, within the NEUROCJD collaboration, a detailed study was undertaken of particular presentations of sporadic CJD, such as pure cerebellar ataxia and Heidenhain’s syndrome (currently being prepared for publication). Studies of atypical forms and cases with young age of onset are in progress (Murray et al, J Neurol Neurosurg Psychiatry 2008). The large number of cases of sCJD accumulated within the system allowed a detailed study of the factors that separately influence disease duration (Pocchiari et al, Brain 2004).
It is well established that the clinico-pathological features of sCJD vary with PRNP-129 genotype and PrP protein type. Both the Unit’s Molecular Genetic and Protein Laboratories contribute to the full clinico-pathological-molecular characterisation of sCJD cases and research is being undertaken into these correlations. Research into possible genetic factors that affect susceptibility and disease phenotype in sCJD has been described above in Question 3.
The differentiation of atypical sCJD from vCJD is potentially aided by prion protein molecular data. However, the relationship between PrP protein type and CJD strain is not as straightforward as it initially seemed and, in particular, the Unit has been active in research into the phenomenon of the co-occurrence of prion types in individual cases of


NCJDSU Scientific Report 2008

CJD. Work carried out by the Prion Protein Laboratory used a type 1 specific antibody (12B2) to show that type 1 PrPres is a minor component in brains of sporadic CJD cases previously classified as type 2 and also that a minority type 1 component is present in BSE brain, in all tested cases of vCJD and in vCJD transmitted to wild-type mice (Yull et al, Am J Pathol 2006). Whilst this work provoked interest and a study with similar conclusions was published by the Aguzzi laboratory (University Hospital, Zurich), the findings are somewhat controversial. To address this, the Unit has conducted a detailed study of the WHO CJD standard reference materials (available from the UK National Institute for Biological Standards and Control) to compare the PrPres mixtures in cases of sporadic CJD that are acknowledged to be genuine mixtures, to those of vCJD where antibodies such as 12B2 are needed to detect the type 1 component. A paper is in preparation for publication. The distribution of disease-related prion protein in extra-neural tissues (such as skeletal muscle and pituitary) in cases of sCJD is an area of developing interest (Peden et al, Am J Pathol 2006; J Gen Virol 2007), which the Prion Protein Laboratory plans to continue to pursue in the future.

The protein laboratory studies have also included work on other forms of CJD. The study of iatrogenic CJD shows the presence of types 1 and/or type 2 PrPres, similar to those found in sporadic CJD, but with a very different protein type and codon 129 genotype distribution. These data, and that on panencephalopathic CJD, have been correlated with clinico-pathological data and are currently being prepared for publication and provide valuable comparisons with the sporadic and variant forms of CJD.

The relationship between PrPres type and agent strain is being investigated by ongoing analysis of transmission to wild-type mice in collaboration with the Neuropathogenesis Division, Roslin Institute. Studies of the transmission characteristics of subtypes of sporadic CJD in a human transgenic model have also been completed (J Manson) and provide important information on the extent of strain variation in sCJD and the influence of codon 129 genotype and prion protein type. This later project is harmonised with the EU funded HUMTRANS project which aims at identifying strain variation in all forms of human prion disease, including ’atypical’ cases (J Manson).

Complementing animal transmission studies the Prion Protein Laboratory aims to model the transmission of human prions, and prion protein conversion, by comparing the


NCJDSU Scientific Report 2008

results of mouse transmission studies with the infection of cell cultures (including human stem cells) and the cell-free protein misfolding cyclic amplification (PMCA) method. The results of initial studies using PMCA indicate that amplification depends on both host and agent factors and that PrPres types are amplified with fidelity from sCJD brain (Jones & Head, unpublished observation). ...


Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

also see ;

Further characterisation of the prion protein molecular types detectable in the NIBSC CreutzfeldteJakob disease brain reference materials

1.4. Mixed PrP types The co-occurrence of types 1 and 2 in cases of sCJD is now a well recognised phenomenon [5,10,11,12] and several independent studies have each concluded that when an extensive brain sampling protocol is employed 20-50% of sCJD cases can be seen to contain both type 1 and type 2 PrPres [7,10,13,14,15].



Further characterisation of the prion protein molecular types detectable in the NIBSC CreutzfeldteJakob disease brain reference materials

Helen M. Yull, James W. Ironside, Mark W. Head* National CJD Surveillance Unit, School of Molecular & Clinical Medicine (Pathology), University of Edinburgh, Edinburgh, United Kingdom

Received 17 November 2008; revised 29 December 2008; accepted 23 January 2009


Sporadic and variant CreutzfeldteJakob disease brain reference materials available from the UK National Institute for Biological Standards and Control have been subjected to further characterisation by Western blot analysis, with particular reference to the co-occurrence of different abnormal disease-associated prion protein (PrPSc) types. The results confirm the presence of genuine type 1 and type 2 protease-resistant PrP (PrPres) in each of the three sporadic CreutzfeldteJakob disease reagents, and provide evidence supporting the lower level presence of type 1 PrPres in the variant CreutzfeldteJakob disease reagents. We conclude that these reagents provide a valuable resource for future research and development.


1.4. Mixed PrP types The co-occurrence of types 1 and 2 in cases of sCJD is now a well recognised phenomenon [5,10,11,12] and several independent studies have each concluded that when an extensive brain sampling protocol is employed 20-50% of sCJD cases can be seen to contain both type 1 and type 2 PrPres [7,10,13,14,15].


On a superficial level the presence of more than one PrPSc type in individual CJD brains may seem at variance with the molecular strain typing hypothesis, which proposes that individual prion strains are enciphered by unique and self-perpetuating conformations and glycosylation states. However, this is not necessarily the case. It has long been known that multiple strains may be derived in mice from individual scrapie isolates, and cross-species transmission can, on occasion lead to an abrupt change in apparent strain characteristics.

snip...see full text ;

2009 The International Association for Biologicals. Published by Elsevier Ltd. All rights reserved. Keywords: CreutzfeldteJakob disease; Standards; Prion protein; Molecular typing; Co-occurrence

Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

ArticleRelated ContentComments: 0.Formal Correction: This article has been formally corrected to address the following errors.

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Abstract Author Summary Introduction Materials and Methods Results Discussion Author Contributions References Emmanuelle Uro-Coste1#, Hervé Cassard2#, Stéphanie Simon3, Séverine Lugan2, Jean-Marc Bilheude4, Armand Perret-Liaudet5, James W. Ironside6, Stéphane Haik7,8, Christelle Basset-Leobon1, Caroline Lacroux2, Katell Peoch'9, Nathalie Streichenberger5, Jan Langeveld10, Mark W. Head6, Jacques Grassi3, Jean-Jacques Hauw8, Francois Schelcher2, Marie Bernadette Delisle1, Olivier Andréoletti2*

1 INSERM U858, Institut de Médecine Moléculaire de Rangueil and Service d'Anatomie Pathologique et Histologie-Cytologie, C.H.U. Rangueil, Toulouse, France, 2 UMR Institut National de la Recherche Agronomique (INRA)/Ecole Nationale Vétérinaire de Toulouse (ENVT) 1225, Interactions Hôtes Agents Pathogènes, ENVT, Toulouse, France, 3 Commissariat à l'Energie Atomique (CEA), Service de Pharmacologie et d'Immunologie, DRM, CEA/Saclay, Gif sur Yvette, France, 4 Bio-Rad, Research and Development Department, Marnes-la-Coquette, France, 5 Hôpital Neurologique, Services de Neurochimie et de Pathologie, Bron, France, 6 National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom, 7 INSERM, Equipe Avenir, Maladies à Prions chez l'Homme, Paris, France, 8 Neuropathology Laboratory, Salpêtrière Hospital, AP-HP, Paris, France, 9 Service de Biochimie et Biologie Moléculaire, Hôpital Lariboisière, Paris (Laboratoire associé au CNR “ATNC”) et EA 3621 Faculté de Pharmacie, Paris, France, 10 Central Institute for Animal Disease Control CIDC-Lelystad, Lelystad, The Netherlands

Abstract Top Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct PrPres types have been considered to represent potential distinct prion strains. However, since cases of CJD show co-occurrence of type 1 and type 2 PrPres in the brain, the basis of this classification system and its relationship to agent strain are under discussion. Different brain areas from 41 sCJD and 12 iatrogenic CJD (iCJD) cases were investigated, using Western blotting for PrPres and two other biochemical assays reflecting the behaviour of the disease-associated form of the prion protein (PrPSc) under variable PK digestion conditions. In 30% of cases, both type 1 and type 2 PrPres were identified. Despite this, the other two biochemical assays found that PrPSc from an individual patient demonstrated uniform biochemical properties. Moreover, in sCJD, four distinct biochemical PrPSc subgroups were identified that correlated with the current sCJD clinico-pathological classification. In iCJD, four similar biochemical clusters were observed, but these did not correlate to any particular PRNP 129 polymorphism or western blot PrPres pattern. The identification of four different PrPSc biochemical subgroups in sCJD and iCJD, irrespective of the PRNP polymorphism at codon 129 and the PrPres isoform provides an alternative biochemical definition of PrPSc diversity and new insight in the perception of Human TSE agents variability.


Discussion Top Coexistence of Different PrPres Types in the Same Subject In this study, detection, by WB, of the coexistence of two PrPres types in about 30% (13/41) of cases is consistent with already published data [12],[14]. This observation could suggest the existence in brain from a single patient of different abnormal PrP species. Although two main PK cleavage sites are associated with PrPres type 1 and type 2 (respectively amino acid 82 and 97), N-terminal sequencing revealed in all investigated cases the presence of a whole spectrum of overlapping cleavage sites. Moreover in a part of investigated cases this technique demonstrated the presence (i) of variable but consistent level of type 1 PrPres in patients classified type 2 using WB and (ii) in some patient classified type 1, of low amount of type 2 PrPres [10]. These observations could suggest that, rather than a pure type 1 or type 2 PrPres, PK digestion of a PrPSc specific conformer generate variable mixture of PrPres fragments (with presence of dominant or sub dominant type 1 or type 2 PrPres), which WB usually failed to reveal accurately because its intrinsic technical limits [14]. Antibodies either harbouring higher affinity to PrP (like Sha31) [18] or probing specifically type 1 PrPres (like 12B2) [20], now allow a better perception of such mixture. However, investigations carried out using artificial mixture of type 1 and type 2 brain homogenate, even using high affinity anti-PrP antibodies, clearly indicate the current limits of WB discriminative power [14]. Together, these data suggest that WB analysis of PrPres on its own could be misleading for adequate discrimination between PrPSc variants in CJD.

Both PrPSc PK resistance ELISA and strain typing ELISA are based on the characterization the N terminal part of the PrPSc PK digestion either by increasing PK amount or modifying detergent conditions. While WB profile could be compared to a snapshot picture of PrPres fragments generated by PK digestion process, these assays reflect the dynamics of the PK cleavage rather than its final result (different forms of PrPres). Consequently they could provide different but also more accurate perception of the PrPSc conformers.

Our findings from the PrPSc capture immunoassays clearly indicate that in a single patient, irrespective of brain area, sCJD associated PrPSc displays uniform biochemical properties, regardless of the regional variation of type 1 and type 2 isoforms determined by WB. Such findings support the idea of the presence of a specific TSE agent in each brain and the accumulation of a single associated PrPSc conformer.

sCJD Classification Because the limited size of our cohort of cases, an in depth comparison between the PrPSc signature (as established in this study) and the Parchi classification system is not possible.

However, despite this limitation, two major groups were identified in our panel according to the PrPSc properties. The first major group was constituted with patients harbouring a highly PK resistant PrPSc (MM1 and MV1 patients). The second group included patients harboring a PK labile PrPSc (VV2 and MV2 patients). Using both lesion profile and clinical parameters [2], two major forms of sCJD are commonly recognized. The first sCJD form, named “classical”, is characterized by a “rapid evolution” (usually around 4 months), and affects most of the MM1 and MV1 patients. The second sCJD form, named “atypical”, affects VV2 and MV2 with a longer symptomatic evolution (usually longer than 6 months) and a late dementia. Despite inter-individual variations, sCJD Groups 1 and 2, as we defined them on biochemical criteria were consistent with this classification.

Both VV1 and MM2 sCJD cases are extremely rare; they respectively represent 1% and 4% of the identified sCJD cases. According to the literature, these patients have clinical features and lesion profiles that are very different from other sCJD patients [2]. However, in our study as in previously published studies, WB did not identify any distinct biochemical difference from other type 1 and type 2 cases. In contrast, both the strain typing ELISA and PrPSc resistance assays clearly differentiated these cases from Group 1 and Group 2 cases. This finding, which is consistent with clinico/pathological observations carried out in patients, could indicate that there are indeed differences in PrPSc that distinguish these VV1 and MM2 cases from other sCJD groups.

Prion Strains and PrPSc Phenotype Although prion strains can only be identified definitively by bioassay, molecular in vitro tools to characterize PrPSc are more and more widely used for the rapid identification of particular agents, such as BSE in cattle, sheep, rodent and humans (vCJD) [20],[21]. This has come to be termed “molecular strain typing” and although widely employed, the exact relationship between PrPSc biochemistry and the biological properties of the agents responsible remain to be determined. In sCJD, the presence of four distinct PrPSc biochemical forms apparently correlated to clinico-pathological phenotypes as defined by Parchi et al. [2] could be an indication of the involvement of different TSE agents.

iCJD cases are a consequence of accidental human to human TSE transmission, most likely representing transmission of sCJD. The identification in iCJD cases of the four PrPSc signatures identified in sCJD is consistent with the existence of distinct prions associated with these biochemical forms.

Three examples of human-to-human transmission of variant CJD through blood transfusion have now been identified. While all blood donors were MM at codon 129 PRNP, the recipients had either a MM (n = 2) or a MV genotype (n = 1). Despite this genotype difference there appears to have been conservation of the disease phenotype and PrPres type in all “secondary” vCJD cases [22]–[25]. These observations could suggest that in case of inter-human transmission, difference in donor/recipient genotype could result in un-altered abnormal PrP signature.

Our identification of MM GH iCJD cases harbouring similar PrPSc signature as a VV1 sCJD case or of a VV dura mater iCJD case similar to MM2 sCJD might indicate preservation of a specific PrPSc biochemical signature after human to human transmission between individuals of different codon 129 genotypes.

Treatment with extracts of GH contaminated by CJD has lead to a high number of iCJD cases in France and the UK. The codon 129 genotypes of the affected individuals in the two countries differ, with the French cohort predominantly MM and MV and the British cohort MV and VV [26]. In the absence of any clear explanation for this finding, it was suggested that it might be due to contamination of different batches of GH with different prion strains from individuals of differing PRNP codon 129 genotypes. Our identification of different biochemical forms of PrPSc in GH French patients and in UK patients is consistent with this hypothesis. The variability observed within the French GH cases could signify involvement of different prion strains, consistent with multiple contaminated GH batches in the French epidemic.

Conclusion The identification in this study of different PrPSc species in CJD patients with the same PRNP polymorphism at codon 129 and WB PrPres profile offers a new perspective on our understanding of the relationship between PrP biochemistry, prion disease phenotype and agent strain. We highlight two novel approaches to analysing PrPSc in sCJD and iCJD and offer evidence that these analyses provide potentially-strain associated information, which appears to be lacking from the conventional WB assay.

Saturday, April 04, 2009 An unusually presenting case of sCJD—The VV1 subtype Volume 111, Issue 3, Pages 282-291 (April 2009)

An unusually presenting case of sCJD—The VV1 subtype

Kaloyan S. Taneva, Mimi Yilmab

Received 16 November 2007; received in revised form 4 September 2008; accepted 12 September 2008.

Abstract Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Typically CJD presents with a triad of rapidly progressive dementia, abnormal movements (e.g., myoclonus) and electroencephalographic (EEG) changes. Recently, CJD has been subdivided into subtypes based on host genetic polymorphisms and the characteristics of the pathological prion protein. Different subtypes likely have different clinical and laboratory presentations. We describe a case of sporadic CJD of the VV1 subtype. We describe our patient's clinical symptoms, time course, laboratory workup, structural and functional neuroimaging data, EEG data and CJD biomarkers. Our patient presented with clinical symptoms atypical for CJD. Because of that, her clinical symptoms were initially attributed to psychiatric reasons. After extensive clinical and laboratory investigation, we concluded that the patient probably had CJD. Postmortem neuropathological results confirmed this clinical hypothesis. We compare our patient's clinical, laboratory and neuroimaging data to the data on typical CJD as well as the data on the few CJD VV1 cases described in the literature. We discuss our case's relevance to the diagnosis of CJD.

Keywords: Creutzfeldt–Jakob disease, Dementia, Neuroimaging, Magnetic resonance imaging, Electroencephalography, Biomarkers, Prion diseases a Department of Psychiatry, Massachusetts General Hospital, 55 Fruit Street, Warren 1220/Blake 11, Boston, MA 02114, United States

b University of Connecticut Health Center, Farmington, CT, United States

Corresponding author. Tel.: +1 617 726 7511; fax: +1 617 724 9155.

PII: S0303-8467(08)00320-X


© 2008 Elsevier B.V. All rights reserved.

rare atypical strain of sporadic cjd ??? seems these rare strains are increasing ???

Wednesday, February 04, 2009

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

A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004

Thursday, July 10, 2008

A New Prionopathy update July 10, 2008



Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.


Sporadic creutzfeldt-jakob disease in two adolescents

see full text sporadic CJD the big lie;


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 ;

Sunday, August 10, 2008

A New Prionopathy OR more of the same old BSe and sporadic CJD

full text ;

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

Saturday, July, 18, 2009


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 ;

Saturday, June 13, 2009

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


Saturday, August 01, 2009 Cases of Early-Onset Sporadic Creutzfeld-Jakob Disease in Michigan


From: TSS
Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
Date: January 29, 2006 at 9:03 am PST
In Reply to: Tracking Spongiform Encephalopathies in North America (Lancet Infectious Disease Volume 3, Number 8 01 August 2003)
posted by TSS on August 14, 2003 at 6:56 pm:
Comments sent via JAMA Feedback Page
NAME: Terry S. Singeltary Sr.E-MAIL: flounder9@ verizon.

Comments sent via JAMA Feedback Page

NAME: Terry S. Singeltary Sr. E-MAIL:

COMMENTS: I wish to submit the following ;

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 the UKBSEnvCJD only theory in 2005. 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 routes and sources...snip...end...TSS

Monday, April 20, 2009

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

CJD TEXAS (cjd clusters)


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.

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


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

Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie. Emerg Infect Dis. 2009 Aug; [Epub ahead of print]

Transmissible mink encephalopathy - review of the etiology

Wednesday, July 1, 2009

Nor98 scrapie identified in the United States J Vet Diagn Invest 21:454-463 (2009)

Monday, June 01, 2009 Biochemical typing of pathological prion protein in aging cattle with BSE


O.K. confusious asks, IF all these new atypical BSEs i.e. new strains of mad cow disease is just an 'OLD COW PRION DISEASE', why then can not the 'old human prion disease' such as the sporadic CJD, be from an 'old cow prion disease', same as the nvCJD 'young people mad cow disease' (which also happens in 74 year old), but why cannot the 'old cow prion diseases', i.e. l-BSE, h-BSE, and ibncBSE, cause the 'old people prion disease', which looks like sporadic CJD. seems that is what some of the pathology is showing ???

OH, that probably makes too much sense, and that the only answer could be that it's all just a happenstance of bad luck and or a spontaneous event, that just happens out of the clear blue sky $$$


Tuesday, August 04, 2009

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

Thursday, July 23, 2009

UW Hospital warning 53 patients about possible exposure to rare brain disease

Wednesday, August 05, 2009 Rate of CWD infection increases in core area WISCONSIN

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