Wednesday, February 26, 2014

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION 2004 THROUGH PRION 2013 CONFERENCE ABSTRACT BOOKS

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION 2004 THROUGH PRION 2013 CONFERENCE ABSTRACT BOOKS
 
 
Greetings,

 
I thought I would share with you all the abstracts of the prion2004 through prion2013 conferences. I hope you all find interested here, and please share or use as you wish. ...kind regards, terry

 
PRION2013

 


 

PRION DISEASE IN ANIMALS

 


 

PRION AND PRION-LIKE DISEASES IN HUMANS ... prion-like ???

 


 

PROTEIN STRUCTURE AND BIOLOGY

 


 

PRION2012

 

PO-100: Prion protein gene codon 129 polymorphism among confirmed sporadic prion disease cases by race and ethnicity, United States

 

Ryan Maddox,1 Pierluigi Gambetti,2 Robert Holman,1 Janis Blevins,2 Lawrence Schonberger,1 Ermias Belay1 1Centers for Disease Control and Prevention; Atlanta, GA USA; 2National Prion Disease Pathology Surveillance Center; Case Western Reserve Univ.; Cleveland, OH USA

 

Introduction. The human prion protein gene (PRNP) exhibits polymorphism for methionine or valine at codon 129, and this polymorphism may influence susceptibility to prion disease as well as disease phenotype. The proportion of individuals with methionine/methionine (MM), methionine/valine (MV), and valine/valine (VV) at codon 129 differs among racial groups.

 

Methods. Information for confirmed prion disease cases, including demographic data and PRNP analysis, was obtained by the United States National Prion Disease Pathology Surveillance Center. The distributions of the three combinations of methionine and valine at codon 129 (MM, MV, VV) were compared for different racial and ethnic groups.

 

Results. PRNP analysis results and race and/or ethnicity information were available for 1288 confirmed sporadic prion disease decedents. Of these, 1260 (97.8%) decedents were diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD) and *** 28 (2.8%) decedents were diagnosed with variably protease-sensitive prionopathy (VPSPr). Among the sCJD cases, 1154 (95.3%) were classified as white race, 34 (2.8%) as black race, and 23 (1.9%) as Asian. Fifty-three cases (4.2%) were classified as Hispanic ethnicity. MM was the most common polymorphism reported for all three racial groups. Among white sCJD decedents, 58.1% were MM at codon 129, 22.5% were MV, and 19.4% were VV. The distribution among black sCJD decedents was similar to whites, with 57.6% MM, 18.2% MV, and 24.2% VV, while a larger percentage of the Asian sCJD decedents (86.4%) were MM at codon 129. Among sCJD decedents of Hispanic ethnicity, 47.2% were MM at codon 129, 18.9% were MV, and 34.0% were VV. All VPSPr decedents with information available were white; the majority (64.3%) was VV at codon 129, followed by MV (25.0%) and MM (10.7%).

 

Conclusions. The MM polymorphism was overrepresented among white sCJD decedents compared with the general US white population. The high distribution of MM among Asian sCJD decedents was consistent with previous reports that the overwhelming majority of the Japanese population is methionine homozygous. The large percentage of VPSPr cases that were VV at codon 129 was expected, as this disease has been reported to preferentially affect this genotype, suggesting a different role of the codon 129 genotype in VPSPr and in sCJD.

 


 

Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.

 

*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.

 


 

Thursday, March 29, 2012

 

atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012

 

NIAA Annual Conference April 11-14, 2011San Antonio, Texas

 


 

Wednesday, November 13, 2013

 

Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein

 


 

MORE FROM PRION2012 SOME OF YOU MAY FIND INTERESTED IN...TSS

 


 


 


 


 


 


 


 


 


 


 

PRION2011

 

ORAL PRESENTATIONS

 


 

PRION BIOLOGY

 


 

PRIONS IN AFFECTED ENVIRONMENTS

 


 

PRION-LIKE PROPAGATION AND PROTEIN MISFOLDING

 


 

MANAGING PRION DISEASE RISKS

 


 

PRION2010

 


 

Third International CWD Symposium

 

July 22-24, 2009 – Park City, Utah

 


 

PRION2009 BOOK OF ABSTRACTS

 


 

PRION2008 BOOK OF ABSTRACTS

 


 

PRION2007 NEWSLETTER

 


 

PRION2007 BOOK OF ABSTRACTS

 


 

From: TSS

 

Subject: PRION2007 ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

 

Date: September 24, 2007 at 6:52 pm PST

 

 P02.35 Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

 

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

 

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

 

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

 

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

 

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

 

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

 

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

 

FC5.1.1 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.

 

 FC5.1.2 Interim Transmission Results in Cynomolgus Macaques Inoculated with BSE and vCJD Blood Specimens

 

Lasmezas, C1; Lescoutra, N2; Comoy, E2; Holznagel, E3; Loewer, J3; Motzkus, D4; Hunsmann, G4; Ingrosso, L5; Bierke, P6; Pocchiari, M5; Ironside, J7; Will, R7; Deslys, JP2 1Scripps Florida, Infectology, USA; 2CEA, France; 3PEI, Germany; 4DPZ, Germany; 5Istituto Superiore di Sanita, Italy; 6SMI, Sweden; 7CJD Surveillance Unit, UK

 

BSE and vCJD transmitted to cynomolgus macaques reproduce many features of human vCJD, including clinical symptoms, neuropathological hallmarks of vCJD, PrPres electrophoretical pattern and, most importantly, the wide distribution of infectivity in peripheral organs. The latter characteristic distinguishes vCJD from sCJD in both humans and cynomolgus macaques, and prompted us to use this non-human primate model for further investigations of vCJD and its risk for human health. The occurrence of four vCJD infections in humans transfused with blood from patients who later developed vCJD has raised concern about blood transfusion safety in countries with vCJD. In this collaborative European study, we investigated the infectivity of blood components and whole blood administered by intracerebral (ic) and intravenous (iv) routes. Buffy-coat and whole blood was inoculated by ic and iv route, respectively, from two vCJD patients and from two clinical vCJD-inoculated macaques. Transfusions were also performed from whole blood and blood leucodepleted according to hospital practice standards from two clinical BSE inoculated macaques. Blood infectivity during the preclinical phase is being examined in orally infected macaques. Whole blood was collected and transfused from one such animal two years after oral challenge, whereas buffy-coat and plasma from two animals at 2 and 4.5 years post-challenge, respectively, have been inoculated by the ic route. This is an ongoing study in which recipient animals continue to be observed at various times post-inoculation. So far, we have had one positive transmission in one animal transfused 65 months earlier with 40 ml of whole blood from a vCJD macaque (the characteristics of the disease in this animal will be shown in a separate poster by E. Comoy). This positive transmission reproduces transfusion transmission of vCJD in humans, with an incubation of 5.5 years compatible with incubation periods observed in humans.

 

FC5.3 Assessing the Risk of vCJD Transmission by Dentistry; Distribution of Infectivity in Oral Tissues of VM Mice after Simulated Oral Feeding of BSE-301V

 

Sutton, JM1; Kirby, E1; Dickinson, J1; Dennis, M1; Cornwall, M1; Vassey, MJ1; Smith, A2; Marsh, PD3; Walker, JT1; Raven, NDH1 1Health Protection Agency, Centre for Emergency Preparedness and Response,, TSE Research group, UK; 2University of Glasgow, Dental School, UK; 3Health Protection Agency, Centre for Emergency Preparedness and Response,, UK

 

Background: Ongoing concerns about the prevalence of variant Creutzfeldt Jakob Disease (vCJD) in the UK population has heightened concerns about the risks of iatrogenic transmission of the disease. Although there have been no cases to date of transmission by surgery there have been 4 cases involving blood transfusion. This study aims to assess the potential of transmission of the disease by dental procedures. Whilst the risks are undoubtably low the very large numbers of procedures carried out annually have the potential to amplify the risks considerably and there is very little data in this area to form the basis for accurate risk assessments. Aim(s)/Objective(s): To assess the relative levels of infectivity in oral tissues from a murine model following exposure to BSE-301V through the small intestine. Methods. The study uses a BSE-301V, VM mouse model as a clinically relevant model for assessing iatrogenic vCJD transmission between humans. Infectious mouse brain homogenate was prepared and inoculated into a loop of the duodenum, to prevent direct contamination of the oral tissues. Mice were sacrificed at 3-weekly intervals and at appearance of clinical symptoms. A range of oral tissues, including dental pulp, gingival margin, salivary gland, saliva, lingual tonsil and trigeminal ganglia, together with brain and spleen tissues were removed, processed as homogenates and reinoculated intracranially (ic.) into indicator mice. Results: The primary challenge proved to be a very efficient route of infection with a 100% attack rate and a mean incubation to clinical disease of 157 ± 17 days (compared to 120 days for the same titre inoculum ic.). Infectivity was observed in all oral and control tissues with varying time-courses and titres estimated from incubation period. Discussion: The results throw new light on the potential routes of dissemination and spread of infectivity from the small intestine to the oral cavity and its implications for possible iatrogenic transmission of vCJD via dental, endoscopic or other forms of surgery. Conclusion: The data generated from the study provides support for ongoing risk assessments to look at the potential for vCJD transmission via dental procedures alongside other elements of studies looking at effectiveness of decontamination and re-use of dental instruments.

 

P02.15 Beyond the PrPres Type1/ Type2 dichotomy in Creutzfeldt-Jakob Disease

 

Cassard, H1; Uro-Coste, E2; Simon, S3; Bilheude, JM4; Perret-Liaudet, A5; Ironside, J6; Haik, S7; Basset-Leobon, C2; Lacroux, C1; Peoch’, K8; Stressenberger, N9; Langeveld, J10; Head, M11; Hauw, JJ12; Schecher, F1; Delisle, MB13; Andreoletti, O1 1Ecole Natinale Vétérinaire de Toulouse, France; 22Service d’Anatomie Pathologique and INSERM U466 R, France; 3DRM, CEA/Saclay, France; 4 Bio-Rad, R&D, France; 5 Hôpital Neurologique Service de Neurochimie, France; 6School of Molecular & Clinical Medicine (Pathology),, National Creutzfeldt-Jakob Disease Surveillance Un, UK; 7Pitié Salpetriere Universitary Hospital, France; 8Hôpital Lariboisière, Service de Biochimie et Biologie Moléculaire,, France; 9Hôpital Neurologique -Service de Neurochimie, France; 10CIDC-Lelystad, Netherlands; 11University of Edinburgh, Western General Hospital, UK; 12Pitié Salpetriere Universitary Hospital,, Laboratoire de Neuropathologie, France; 13Rangueil Universitary Hospital, Service d’Anatomie Pathologique, France

 

Creutzfeldt-Jakob disease (CJD) cases are currently classified according to established diagnostic criteria and by the genotype at codon 129 of the PRNP gene and the Western blotting of the proteinase K digested abnormal prion protein that distinguishes a type 1 and a type 2 profile. These biochemically distinct PrPres types have been proposed to represent distinct prion strains. However, since the cooccurence of type 1 and type 2 PrPres in the same patient is common, the rationale of this classification and strain concept as applied to CJD are currently under discussion. Five different brain areas from of 40 sporadic CJD and 11 iatrogenic CJD (both dura matter-, and growth hormone-associated) cases, originating from UK and France, were systematically investigated, using Western blotting typing, and by two others biochemical assays that depend on the behaviour of PrPSc in variable PK digestion conditions. As described previously, co-occurrence of type 1 and type 2 PrPres was found in 30% of the CJD patients examined. However, our novel PK concentration dependent assays identified a single uniform PrP type in cases where both type 1 and type 2 were present. Moreover, in sCJD four distinct biochemical PrPSc signatures were identified by the PK concentration dependent assays and these correlated to the current genotype/clinico-pathological sCJD groups. In iCJD the four similar biochemical signatures were observed, but were not correlated to particular PRNP 129 polymorphism or Western Blot PrPres patterns. Moreover notable differences were observed between PrPSc biochemical properties of French and UK GH-CJD cases, which could reflect, as already suspected, differences in the causative agents. Identification, in sCJD and iCJD, of four different PrPSc phenotypes irrespective of patients PRNP polymorphism at codon 129 and Western blot profile provides new insights into human prion disease aetiology and could reflects an unsuspected diversity of TSE agents in human disease. Further investigations are currently underway using animal transmission to correlate agent strain with our new discriminant biochemical assays.

 

From: TSS

 

Subject: PRIONS IN PLASMA OF PATIENTS WITH SPORADIC CJD, ATYPICAL VCJD IN 73 YEAR OLD HUMAN BLOOD TRANSFUSION RECIPIENT AND MACAQUE, ENHANCED SURVEILLANCE

 

Date: September 30, 2007 at 1:17 pm PST

 

 P04.49

 

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

 

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

 

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

 

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

 


 

 P04.51

 

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

 

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

 

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

 


 

P04.36

 

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

 

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

 

Introduction:

 

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

 

Methods:

 

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

 

Results:

 

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

 


 

Prions in Plasma of Patients with Sporadic Creutzfeldt-Jakob Disease

 

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

 


 


 


 

P04.33

 

Diagnostic Utility of CSF Biomarkers and General CSF Findings in a U.S Sporadic CJD Cohort

 

Geschwind, MD; Haman, A; Torres-Chae, C; Raudabaugh, BJ; Devereux, G; Miller, BL University of California, San Francisco, USA

 

Background:

 

The diagnostic utility of CSF biomarkers, including 14-3-3 protein, neuron-specific enolase (NSE), AB42, total Tau (T-Tau), and phosphorylated Tau (PTau)/ T-Tau ratio are often are used for sporadic CJD (sCJD) diagnosis is controversial. We have previously reported the CSF 14-3-3 protein to have poor sensitivity for CJD diagnosis. In this study, now report the sensitivity and specificity of several CSF biomarkers and general characteristics in a U.S. cohort of sCJD and non-prion rapidly progressive dementia (RPD) controls (Non-CJD cohort) subjects.

 

Design/Methods:

 

Clinical diagnoses are made through review of medical records, clinical evaluation, and in many cases pathology. Data is stored in a secure clinical relational database, which was queried for various CSF findings, including cell count, protein, IgG index, oligoclonal bands (OCBs), 14-3-3, NSE, T-Tau in patients with probable or definite sporadic CJD and non-prion rapidly progressive dementias (RPD), most of whom were referred to our center with a potential diagnosis of CJD. For probable sCJD diagnosis, we used UCSF criteria that are modified from WHO to substitute MRI for 14-3-3. T-Tau and NSE were considered positive if > 1300 pg/ml and > 35 ng/ml, respectively. For this analysis, ambiguous 14-3-3 results were considered as negative.

 

Results:

 

14-3-3 protein (n=149) sensitivity was only 48% (47% for definite; 50% for probable sCJD). NSE (n=49) sensitivity was 63% (66% for definite; 59% for probable sCJD). T-Tau (n=28) was the most sensitive at 68% (69% for definite; 67% for probable sCJD). The specificity of these biomarkers among our CJD and RPD controls (n=72) was 66% for 14-3-3 (n=47), 81% for NSE (n=21), and 100% for T-Tau (n=7). The 14-3-3 had the lowest sensitivity and specificity. Mildly elevated CSF protein (<100 common="" dl="" high="" in="" is="" mg="" scjd="" wbc="">20, is uncommon in sCJD.

 

Conclusions/Relevance:

 

In a cohort from a major U.S. CJD referral center, the 14-3-3 is neither sensitive nor specific for sCJD. NSE and T-Tau also show poor sensitivity for sCJD. T-Tau may be more specific than 14-3-3 and NSE, but our “n” is small. WHO sCJD criteria should be revised; by eliminating 14-3-3 and including brain MRI into the criteria. We are currently analyzing the effects of disease duration and codon 129 polymorphism on these CSF biomarker results.

 


 

see full text 143 pages ;

 


 


 

PRION2006 NEWSLETTER

 


 

PRION2006 BOOK OF ABSTRACTS

 


 

PRION2005 PRESS RELEASE

 


 

PRION2005 BOOK OF ABSTRACTS

 


 

PRION2004 PRESS RELEASE

 


 

PRION2004 BOOK OF ABSTRACTS

 


 

 

PRION2014 ABSTRACT BOOK NOT PUBLISHED YET...I SUBMITTED THE FOLLOWING FOR WHATEVER IT WAS WORTH...TSS

 

 

From: Terry S. Singeltary Sr.

 

Sent: Saturday, December 21, 2013 9:59 AM

 


 

Cc: association@neuroprion.org ; giuseppe.legname@sissa.it

 

Subject: FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE

 

Greetings Prion2014 et al,

 

Members of the PRION 2014 Scientific Advisory Board:

 

Adriano Aguzzi -Institute of Neuropathology University Hospital of Zurich, Switzerland

 

Olivier Andreoletti - Ecole nationale vétérinaire de Toulouse, France

 

Jason C. Bartz, Creighton University, USA

 

Ilia Baskakov - University of Maryland School of Medicine, USA

 

Maria Laura Bolognesi - Università di Bologna, Italy

 

Paolo Carloni - German Research School for Simulation Sciences, Germany

 

Cristina Casalone - S.S. Laboratorio Neuropatologia, IZSTO, Italy

 

Neil Cashman - University of British Columbia (UBC), Canada

 

Joaquin Castilla - CICbioGUNE, Parque Tecnológico de Vizcaya, Spain

 

Roberto Chiesa - Istituto di Ricerche Farmacologiche "Mario Negri", Italy

 

Marc Diamond - Washington University School of Medicine, USA

 

Jean Philippe Deslys - Commissariat à l'Énergie Atomique, France

 

Pierluigi Gambetti - Case Western Reserve University, USA

 

Michael D. Geschwind - University of California, San Francisco, USA

 

Andrew Hill - Bio21 Molecular Science & Biotechnology Institute, Australia

 

Edward Hoover - Colorado State University, USA

 

Jan Langeveld - Central Veterinary Institute of Wageningen UR, Lelystad, The Netherlands

 

Giuseppe Legname - International School for Advanced Studies, Italy (Chair)

 

Giovanna Mallucci - MRC Toxicology Unit, Leicester, UK

 

Jean Manson - University of Edinburgh, UK

 

Vilma R. Martins - AC Camargo Cancer Center, São Paulo, Brazil

 

Glenn Millhauser - University of California, Santa Cruz, USA

 

Romolo Nonno - Istituto Superiore di Sanità, Italy

 

Piero Parchi - Università di Bologna, Italy

 

Janez Plavec - NMR Center at National Institute of Chemistry, Slovenia

 

Maurizio Pocchiari - Istituto Superiore di Sanità, Italy

 

Jesus Requena - Universidade de Santiago de Compostela, Spain

 

Detlev Riesner - Heinrich-Heine-Universität-Düsseldorf, Germany

 

Hermann M. Schatzl - University of Calgary, Canada

 

Fabrizio Tagliavini - Fondazione IRCCS Istituto Neurologico Carlo Besta, Italy

 

Motomasa Tanaka - RIKEN Brain Science Institute, Japan

 

Albert Taraboulos - The Hebrew University of Jerusalem, Israel

 

Glenn Telling - Colorado State University, USA

 

Juan Maria Torres - Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria, Spain

 

Ina Vorberg - German Center for Neurodegenerative Diseases (DZNE), Germany

 

David Westaway - Centre for Prions and Protein Folding Diseases, University of Alberta, Canada

 

Robert Will - National Creutzfeldt-Jakob disease Surveillance Unit, Western General Hospital, UK

 

Holger Wille - Department of Biochemistry, University of Alberta, Canada

 

Gianluigi Zanusso - Università di Verona, Italy

 

Chiara Zurzolo - Membrane traffic and Pathogenesis Unit, Institut Pasteur, France

 

 

I kindly Wish to submit the followoing ;

 

 

IF you really want to know, what they are feeding cows and other livestock for human and animal consumption, please see my latest... review of the OAI’s under the mad cow feed ban for 2013. please be aware, the mad cow feed ban of 1997, was nothing but ink on paper. the tonnage of banned mad cow feed that has gone into commerce is phenomenal, it’s in the 100s if not 1000s of tonnages. it’s flat out shocking...

 

 

Sunday, December 15, 2013

 

*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE

 


 

 

Good Luck with Prion2014, I look forward to reading the science that comes there from. ...if someone will kindly send me the Congressional Book of Abstracts. ...

 

with kindest regards, terry

 

 

layperson

 

MOM DOD 12/14/97 confirm ‘hvCJD’ just made a promise to mom, NEVER FORGET! and never let them forget. ...

 

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

 

 

From: Terry S. Singeltary Sr.

 

Sent: Monday, January 06, 2014 1:24 PM

 


 

Cc: association@neuroprion.org ; giuseppe.legname@sissa.it ; stanley@ind.ucsf.edu

 

Subject: PRICE OF CWD TSE PRION POKER GOES UP IN 2014

 

Greetings PRION2014, Professor Prusiner, et al,

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

Wednesday, January 01, 2014

 

Molecular Barriers to Zoonotic Transmission of Prions

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 


 


 

*** 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.

 


 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 

SNIP...

 

Subtype 1: (sCJDMM1 and sCJDMV1)

 

This subtype is observed in patients who are MM homozygous or MV heterozygous at codon 129 of the PrP gene (PRNP) and carry PrPSc Type 1. Clinical duration is short, 3‑4 months.32 The most common presentation in sCJDMM1 patients is cognitive impairment leading to frank dementia, gait or limb ataxia, myoclonic jerks and visual signs leading to cortical blindness (Heidenhain’s syndrome)...

 


 

Animals injected with iatrogenic Creutzfeldt–Jakob disease MM1 and genetic Creutzfeldt–Jakob disease MM1 linked to the E200K mutation showed the same phenotypic features as those infected with sporadic Creutzfeldt–Jakob disease MM1 prions...

 


 

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

 


 


 

SNIP...SEE FULL TEXT ;

 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 


 

Wednesday, January 01, 2014

 

APHIS-2006-0118-0100 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose

 


 

Friday, November 22, 2013

 

Wasting disease is threat to the entire UK deer population CWD TSE Prion disease Singeltary submission to Scottish Parliament

 


 

Thursday, October 10, 2013

 

*** CJD REPORT 1994 increased risk for consumption of veal and venison and lamb

 


 

Thursday, January 02, 2014

 

Tests Confirm CWD Case in Pennsylvania Release #001-14

 


 

Wednesday, September 04, 2013

 

*** cwd - cervid captive livestock escapes, loose and on the run in the wild

 


 

Sunday, September 01, 2013

 

hunting over gut piles and CWD TSE prion disease

 


 

Monday, January 16, 2012

 

9 GAME FARMS IN WISCONSIN TEST POSITIVE FOR CWD

 


 

Tuesday, June 11, 2013

 

*** CWD GONE WILD, More cervid escapees from more shooting pens on the loose in Pennsylvania

 


 

Tuesday, April 16, 2013

 

Cervid Industry Unites To Set Direction for CWD Reform and seem to ignore their ignorance and denial in their role in spreading Chronic Wasting Disease

 


 

 Tuesday, December 18, 2012

 

*** A Growing Threat How deer breeding could put public trust wildlife at risk

 


 

Thursday, February 09, 2012

 

50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE

 


 

Friday, August 31, 2012

 

COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK and CWD 2009-2012 a review

 


 

Tuesday, June 05, 2012

 

Captive Deer Breeding Legislation Overwhelmingly Defeated During 2012 Legislative Session

 


 

Saturday, February 04, 2012

 

Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised

 


 

Thursday, January 02, 2014

 

Tests Confirm CWD Case in Pennsylvania Release #001-14

 


 

Monday, January 06, 2014

 

North Dakota second deer taken from unit 3F2 during the 2013 deer gun season has tested positive for chronic wasting disease

 


 

Sunday, December 15, 2013

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 


 

Saturday, December 21, 2013

 

Complementary studies detecting classical bovine spongiform encephalopathy infectivity in jejunum, ileum and ileocaecal junction in incubating cattle

 


 

Friday, August 16, 2013

 

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates

 


 

Sunday, August 11, 2013

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010

 


 

Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

Saturday, November 16, 2013

 

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 

Infect Control Hosp Epidemiol.

 


 

Monday, December 02, 2013

 

*** A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures

 


 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

 


 

 

 

2003 Mad Cow Scaremongers

 

Mad Cow Scaremongers by Terry S. Singeltary Sr. a review of the TSE prion agent 2003-2011

 


 

 

 

Re: vCJD in the USA * BSE in U.S. 15 November 1999 Terry S Singeltary, NA

 

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.

 

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.

 


 

 

 

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

 

2 January 2000 Terry S Singeltary

 

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

 


 

 

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.

 


 

 

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

 


 

 

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.

 


 

 

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

 

Previous

 

Next

 

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.

 


 

 

Singeltary submission to PLOS ;

 

No competing interests declared.

 

see full text ;

 


 

 

Owens, Julie

 

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

 

Sent: Monday, July 24, 2006 1:09 PM

 

To: FSIS RegulationsComments

 

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98

 


 

FSIS, USDA, REPLY TO SINGELTARY

 


 

Sunday, December 15, 2013

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 


 

Saturday, December 21, 2013

 

Complementary studies detecting classical bovine spongiform encephalopathy infectivity in jejunum, ileum and ileocaecal junction in incubating cattle

 


 

Sunday, December 22, 2013

 

10 years after mad cow cover up started, and 16 years after Moms demise to hvCJD, were still feeding cows to cows

 


 

Friday, August 16, 2013

 

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates

 


 

 WHAT about the sporadic CJD TSE proteins ?

 

WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***

 


 

Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

Saturday, December 21, 2013

 

Parelaphostrongylus (Brainworm) Infection in Deer and Elk and the potential for CWD TSE prion consumption and spreading there from ?

 


 

 

layperson,

 

kindest regards, terry

 

Terry S. Singeltary Sr.

 

Bacliff, Texas USA 77518

 

 
 

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