Sunday, August 21, 2011

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

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

(CJD...see old video here from EU)

see new url here ;

Saturday, June 25, 2011

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

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

RE - "BSE-L in North America may have existed for decades" YA THINK ???

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


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

Sunday, June 26, 2011

Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque

Thursday, June 23, 2011

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

Sunday, August 21, 2011

Classical Bovine Spongiform Encephalopathy by Transmission of H-Type Prion in Homologous Prion Protein Context

Volume 17, Number 9–September 2011


Thursday, July 21, 2011

A Second Case of Gerstmann-Sträussler-Scheinker Disease Linked to the G131V Mutation in the Prion Protein Gene in a Dutch Patient Journal of Neuropathology & Experimental Neurology:

August 2011 - Volume 70 - Issue 8 - pp 698-702

Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

Wednesday, June 15, 2011

Galveston, Texas - Isle port moves through thousands of heifers headed to Russia, none from Texas, Alabama, or Washington, due to BSE risk factor

Saturday, July 23, 2011


Thursday, July 28, 2011

An Update on the Animal Disease Traceability Framework July 27, 2011

Risk.16: Clinical Disease in Cattle Experimentally Inoculated with All Types of BSE

Catherine Graham,1,† Michel Levy,2 Ed Pajor,2 Garth McGregor,1 Rheana Flitton1 and Stefanie Czub1

1Canadian Food Inspection Agency; Lethbridge, AB Canada; 2Faculty of Veterinary Medicine; University of Calgary; Calgary, AB Canada†Presenting author; Email:

Background. Classical, or C-type, bovine spongiform encephalopathy (BSE) has been extensively described in the literature. Recently, two novel forms of BSE, termed atypical BSE, have been reported in a number of countries. These new forms show differences in the biochemical characteristics of the prion protein and, where reported, tend to occur in aged animals but descriptions of clinical presentation are incomplete or absent.

Materials and Methods. Female Hereford/Angus cross calves were intracranially challenged at approximately five months of age with 1 ml of a 10% brain homogenate originating from Canadian field cases of BSE which had been previously classified as C-, L-, or H- type.

The animals were monitored during incubation period, and clinical disease is described using a standardized examination protocol. Incubation period, description and progression of clinical signs was recorded and videotaped for objective evaluation.

Results. All L- and H- type atypical BSE challenged animals began to display signs of clinical disease at approximately 11 months post inoculation, and disease progression was slow but constant until animals were euthanized. Clinical signs in all atypical BSE inoculated animals included hesitation at doors and gates, spontaneous muscle fasciculations and sensitivity to touch. Teeth grinding and excessive salivation are occasionally noted. Animals with L-type BSE are very anxious and show high levels of sensitivity to hand movement. One H-type animal shows periods of somnolence. Both H-type inoculated animals go down during handling and have difficulty rising and show sensitivity to movement around their head and neck area, but to a lesser degree than the L-type BSE inoculated animals. Interestingly, no locomotor abnormalities have been observed in either group.

C-type challenged animals remain normal at approximately 18 months post inoculation. Clinical disease in C-type inoculated animals from a previous transmission study was typically slow and intermittently displayed during the initial stages and after a period of two to four months was more consistent and progressive. Clinical signs in C-type BSE were as previously reported in the literature.

Discussion. The spectrum of clinical signs for all three types of BSE examined is similar. Incubation period is shorter for H- and L-type BSE as compared with C-type. Once clinical signs begin, progression is slow but relentless in atypical BSE, and more insidious in classical BSE. A summary of clinical signs presented in the three different types of BSE will be presented, and video of clinical disease will be displayed.

Risk.04: Demographic and Diagnostic Differences in Minorities with Sporadic Creutzfeldt-Jakob Disease

Brian S. Appleby,1,† Kristin K. Appleby2, 3 and Mitchell T. Wallin2

1Johns Hopkins University School of Medicine; Baltimore, MD USA; 2Veterans Affairs Medical Center; Washington, DC USA; 3Parkinson’s and Movement Disorders Center of Maryland; Elkridge, MD USA†Presenting author; Email:

Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of human prion disease. Prior epidemiologic studies of CJD in the U.S.A have reported a lower age adjusted incidence rate for blacks compared to whites. The goal of this study was to explore possible demographic and diagnostic features that could explain this finding.

Method. Previously collected data from Johns Hopkins and the Veterans Affairs Health Care System (VHS) between 1995-2007 were analyzed in this study following IRB approval. Only probable and definite cases of sCJD were included in the final analyses. Caucasian and Hispanic subjects were characterized as white and all other ethnicities were considered non-white in analyses. Chi-square analyses were used for categorical variables and Kaplan-Meier analyses were used for continuous variables.

Results. 116 subjects [n = 100 (86.2%) Caucasian, n = 6 (5.2%) black, n = 6 (5.2%) Hispanic, and n = 4 (3.4%) other race] were included in this study. Age at disease onset differed significantly between whites (mean = 65.2 ± 0.89 years) and non-whites (mean = 57.8 ± 2.94 years) (Log Rank = 5.32, p = 0.021). The correct clinical diagnosis was determined more rapidly in non-whites (46.7 ± 19.5 days) compared to whites (197.3 ± 35.6 days) (Log Rank = 7.08, p = 0.008). Although tissue diagnosis did not differ significantly between groups, non-whites were less likely to undergo autopsy (1/10, 10%) compared to whites (51/103, 49.5%) (Fisher’s exact test, 2-sided, p = 0.02).

Conclusions. In this study population, non-whites had an earlier age at disease onset compared to whites. Non-whites also received the correct clinical diagnosis more quickly compared to whites and were less likely to undergo autopsy. Given these results, it is unclear if non-whites truly have a younger age at onset compared to whites as this may be the result of ascertainment bias. The much more rapid clinical diagnosis of non-whites suggests that a diagnosis of CJD was considered earlier in non-whites who had an earlier age at disease onset. The decreased autopsy rate of non-whites is also concerning as this may falsely lower the estimated incidence of sCJD in non-whites in epidemiologic studies that rely heavily on neuropathologic data. Further studies examining the incidence and diagnostic process of sCJD in non-whites is needed. This study also demonstrates the importance of including patients that are diagnosed clinically and who do not undergo autopsies in CJD surveillance efforts.

Risk.06: Could National Mortality Registers Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from the 2000-2008 Mortality Data

Jean-Philippe Brandel,1, 5, 7,† Arlette Welaratne,1 Dominique Salomon,2, 7 Isabelle Capek,3 Véronique Vaillant,3 Albertine Aouba,4 Stéphane Haïk5, 7, 1 and Annick Alpérovitch2, 8

1APHP Groupe Hospitalier Pitié Salpêtrière; Paris, France; 2INSERM U708 Neuroepidemiology; Paris, France; 3Institut de Veille Sanitaire; Saint Maurice, France; 4INSERM CépiDc; Le Vésinet, France; 5INSERM UMR-S 975 Equipe maladie d’Alzheimer-maladies à prions; Paris, France; 6CNRS UMR 7225; Paris, France; 7Université Pierre et Marie Curie-Paris; Paris, France†Presenting author; Email:

Active surveillance of Creutzfeldt-Jakob disease, which has been implemented in European countries, requires important human resources and funding. As the epidemic of variant CJD due to the bovine spongiform encephalopathy agent is in decline, less intense surveillance systems, based on routine mortality or morbidity registers, could be considered. CJD data collected by the French national CJD surveillance centre were compared with CJD data registered in the national mortality statistics. From

2000 to 2008, the two sources reported fairly similar numbers of CJD deaths (1188 and 1221 for the surveillance centre and the mortality register respectively). However, analysis of individual data showed important between-sources disagreements. At least 13% of CJD reported by the mortality register were false positive diagnoses and 21.6% of the CJD cases diagnosed by the surveillance centre were not registered as CJD in the national mortality statistics. For 126 deaths registered as CJD in the mortality statistics that had not been notified to the surveillance center, available data did not allow CJD diagnosis to be confirmed or excluded. One out of 22 variant CJD cases was not reported as having any type of CJD in the mortality statistics. Without further investigation, the conclusion could have been that CJD surveillance could be based on routinely collected mortality data. Considering the uncertainties on the evolution of the vCJD epidemics and the emergence of novel prion diseases in animals consumed by humans with zoonotic potential, these results support the idea that an active surveillance should be maintained to provide reliable data on future cases that may arise in next decades.

Risk.10: CSF Proteins and Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Canada

Michael B. Coulthart,1,† Gerard H. Jansen,2 Elina Olsen,3 Deborah L. Godal,1 Tim Connolly,3 Bernard C. Choi,3 Zheng Wang3 and Neil R. Cashman4

1Public Health Agency of Canada; Winnipeg, MB Canada; 2University of Ottawa; Ottawa, ON Canada; 3Public Health Agency of Canada; Ottawa, ON Canada; 4University of British Columbia; Vancouver, BC Canada†Presenting author; Email:

Background. With its range of initial symptoms that may accompany other conditions, and a frequent need for timely diagnosis, sporadic Creutzfeldt-Jakob disease (sCJD) can present the clinician with significant challenges. Particularly widely employed for this purpose are assays for certain brain proteins in cerebrospinal fluid (CSF). Data are needed to better support systematic revision of diagnostic probabilities for sCJD on the basis of CSF protein assay results, in patient populations that also include diverse subacute encephalopathies eliciting a clinical suspicion of sCJD.

Methods. CSF 14-3-3, total Tau and S-100B proteins were studied prospectively in 948 Canadian patients suspected of having sCJD, including 121 with autopsy-confirmed sCJD and 827 with probable non-CJD diagnoses. Various metrics of diagnostic accuracy including sensitivity, specificity, predictive values and likelihood ratios were estimated.

Results. Estimated diagnostic accuracy for individual markers were mostly consistent with those of previously published studies at optimal cutoff thresholds for this study population (empirically defined for 14-3-3 immunoblot; 976 pg/mL for Tau; 2.5 ng/mL for S-100B). Sensitivity and specificity estimates respectively at these thresholds were 0.88 (95% CI, 0.81–0.93) and 0.71 (0.68–0.74) for 14-3-3; 0.90 (0.83–0.95) and 0.87 (0.85–0.90) for Tau; and 0.86 (0.78–0.91) and 0.86 (0.84–0.89) for S-100B; thus, the only outlier was 14-3-3 specificity (~0.7). Positive likelihood ratio (LR+) estimates were low to moderate: 3.0 (2.8–3.3) for 14-3-3; 7.1 (6.6–7.6) for Tau and 6.3 (5.8–6.8) for S-100B at optimal cutoff thresholds. Negative likelihood ratios were moderate: 0.17 (0.10–0.30) for 14-3-3; 0.12 (0.07–0.2) for Tau; and 0.17 (0.10–0.30) for S-100B. Interval LR estimates strengthened accuracy for patient subsets—for example, 31.4% of sCJD patients displayed extreme CSF Tau levels (>12 000 pg/mL), associated with an LR of 64.0 (23.3–175.9). Combining Tau and S-100B results, even at intermediate values, also enhanced accuracy; e.g., LR+ = 55.6 (20.1–153.7) with Tau > 5000 pg/mL and S-100B > 5.0 ng/mL.

Conclusions. CSF Tau and S-100B show comparable or better diagnostic accuracy compared to 14-3-3 in a heterogeneous patient population with low average pre-test probability of sCJD. Tau and S-100B may be optimal choices for many sCJD case investigations. Reporting of quantitative assay results as well as combining Tau and S-100B could enhance the clinical utility of surveillance case definitions for sCJD.

Risk.12: Transmission of Atypical Italian sCJD Case to Humanized Mice Reveals a Novel Infectious Strain

Roberta Galeno,1,† Marco Sbriccoli,1 Loredana Ingrosso,1 Silvia Graziano,1 Angelina Valanzano,1 Anna Poleggi,1 Angela De Pascalis,1 Anna Ladogana,1 Franco Cardone,1 Maria Puopolo,1 Gianluigi Zanusso2 and Maurizio Pocchiari1

1Istituto Superiore di Sanità; Rome, Italy; 2University of Verona; Verona, Italy†Presenting author; Email:

Sporadic Creutzfeldt-Jakob disease (sCJD) is a neurodegenerative prion disorder with uncertain etiology characterized by a typical combination of clinical symptoms, neuropathological lesions, and by the deposition of the pathological protein PrPTSE in the brain.

The vast majority of patients affected by sCJD can be categorized according to the genotype at the polymorphic position

129 of PrP (methionine or valine) and to the molecular mass of PrPTSE (type 1 or 2, corresponding to 21 or 19 kDa), yielding six possible combinations (MM1, MM2, VV1, VV2, MV1, and MV2) that associate with five clinico-pathological variants. Transmission studies of these sCJD subtypes into transgenic mice expressing the human prion protein allowed to identify four different infectious strains, which can partly explain the heterogeneity observed in sCJD patients.1

We recently described a novel molecular and pathological phenotype of sCJD (MV at position 129 of PrP), associated with an unprecedented electrophoretic pattern of PrPTSE characterized by the absence of the highly glycosylated isoform. In this work, we sought to characterize the prion strain associated with this atypical case by intracerebral inoculation into gene-targeted transgenic mice (HuTg) carrying the human PRNP gene with the three 129 genotype combinations. For comparison, three Italian sCJD cases heterozygous at position 129 of the prion protein, belonging to different subtypes (MV1, MV1/2, MV2), were transmitted to the same panel of transgenic mice. Survival times, attack rates, lesion profiles, and molecular analysis of the PrPTSE type recovered from mouse brains injected with the atypical case were compared with data from control animals. Mice inoculated with the atypical case displayed a restricted host tropism, with only a small number of VV animals that resulted PrPTSE-positive after an exceedingly long survival time. Interestingly, PrPTSE accumulated in brains from these mice lacks the diglycosylated band similar to that in sCJD inoculum, yet dissimilar to any other PrPTSE observed in HuTg mice by us and by other authors.1,2 Overall, these results strongly indicate that our atypical case associates with a new infectious strain of sCJD. Further investigations are needed to understand the possible connection with other human and animal prion diseases.


1. Bishop MT, Will RG, Manson JC. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010; 107:12005-10.

2. Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, et al. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 2006; 5:393-8.

Risk.21: Thirty-Year Review of Prion Disease Surveillance in the United States

Robert C. Holman,1,† Ryan A. Maddox,1 Arianne M. Folkema,1 Arialdi M. Minino,2 Teresa A. Hammett,1 Kenneth D. Kochanek,2 James J. Sejvar,1 Ermias D. Belay1 and Lawrence B. Schonberger,1

1CDC; Atlanta, GA USA; 2CDC; Hyattsville, MD USA†Presenting author; Email:

Background. With the emergence of bovine spongiform encephalopathy/variant Creutzfeldt-Jakob disease (vCJD) in the UK, the Centers for Disease Control and Prevention began utilizing national mortality data with additional surveillance mechanisms to monitor US occurrences of human prion disease.

Objectives. To review US prion disease surveillance data.

Methods. We analyzed national mortality data for prion disease deaths (a surrogate for CJD incidence) among US residents for the 30 year period, 1979–2008, augmenting and extending these data through 2010 with information from other surveillance mechanisms (e.g., national neuropathology surveillance). We calculated age-adjusted and age-specific death rates per million persons; race-specific rates used data available beginning 1981. We age-adjusted death rates to the standard projected US 2000 population. Prion 131

Results. A total of 7,615 deaths during 1979–2008 were identified for an average annual age-adjusted rate of 0.98 cases per million persons. The highest rate (1.15) was observed in 1997; the highest number of reported cases was in 2008 (348). By race, the rate (1.06) among whites, who constituted 95% of the cases, was significantly higher than among blacks (0.40), Asian/Pacific Islanders (0.63) and American Indians/Alaska Natives (0.42). The rate (4.0) among persons =55 years old was strikingly higher than the rate (0.14) among persons <55 years old. The youngest decedent was age 21 years. None of the deaths during the 30 year period were reported with hereditary factor VIII or IX deficiency, thalassemia, or sickle cell disease. Through 2010, the only identified vCJD decedents among US residents were the three in 2004-2006 who were previously reported as likely infected in the UK or Saudi Arabia.

Conclusion. The annual age-adjusted US CJD death rates remained relatively stable over several decades although the most recent, complete, annual data show the highest number of cases. The absence of CJD in persons <20 years of age despite an estimated magnitude of 100,000 transfusion recipients <5 years of age who remained at risk during the surveillance period plus many times more such youngsters who received blood products and the absence of CJD in persons with hemophilia (current population ~20,000), thalassemia (~1,000) or sickle cell disease (~100,000) suggests the risk, if any, of blood-related CJD transmissions is likely very low. Racial differences in CJD rates deserve further investigation.

Risk.27: Creutzfeldt-Jakob Disease Among Hispanics in the United States, 1997–2008

Ryan A. Maddox,1,† Robert C. Holman,1 Arianne M. Folkema,1 Arialdi M. Minino,2 Teresa A. Hammett,1 Lawrence B. Schonberger1 and Ermias D. Belay1

1National Center for Zoonotic and Emerging Infectious Diseases; Centers for Disease Control and Prevention; Atlanta, GA USA; 2National Center for Health Statistics; Centers for Disease Control and Prevention; Hyattsville, MD USA†Presenting author; Email:

Introduction. At 16% of the US population, Hispanics make up the largest ethnic or racial minority in the country, and this proportion is expected to increase in the coming decades. The occurrence of Creutzfeldt-Jakob disease (CJD) among Americans of Hispanic ethnicity has not been widely investigated.

Methods. Hispanic CJD decedents of any age were identified from the US national multiple cause-of-death data and other sources for 1997–2008. Relevant portions of medical records and results from neuropathologic and genetic testing for Hispanic CJD decedents <55 years of age were obtained and reviewed, as available.

Results. During 1997–2008, 160 CJD decedents were identified as being of Hispanic ethnicity, for an average annual age-adjusted incidence of 0.65 per million population, an incidence significantly lower than that for non-Hispanics (RR =0.6; 95% CI 0.5–0.7). While 27 states reported at least one Hispanic decedent during the time period, almost half (47.5%) of the decedents were residents of California or Texas, the states with the highest Hispanic populations. A majority (55.0%) of the decedents were females, but the average annual age-adjusted incidence was slightly higher for males, although the difference was not significant. The median age at death was 64 years (range 36-93 years). Thirty-three Hispanic CJD decedents (20.6%) were <55 years of age, compared to 12.1% of cases in that age group among non-Hispanic CJD decedents; however, the average annual age-specific incidence for CJD decedents <55 years of age was significantly lower among Hispanics compared to non-Hispanics (0.08 compared to 0.17, respectively; p < 0.0001). Of the 33 young Hispanic CJD decedents, 21 (63.6%) had medical records and/or neuropathology reports available for review. Ten of these 21 cases (47.6%) had neuropathologic confirmation, including three decedents with familial CJD and one decedent with sporadic fatal insomnia.

Conclusions. Between 1997 and 2008, the reported CJD incidence among Hispanics in the US was significantly lower than that for non-Hispanics. This lower incidence may be at least partly due to underreporting of Hispanic ethnicity relative to surveys and censuses, and further study is warranted. Analyses of brain tissue remain important, especially considering that approximately half of the young Hispanic decedents with information available lacked CJD confirmation.

Risk.26: Sex Effect in Prion Diseases

Corinne Loeillet,1,† Pierre-Yves Boelle,2 Catherine Lemaire-Vieille,1 Philippe Naquet,3 Pierre Chambon,4 Marie-France Cesbron-Delauw,1 Alain-Jacques Valleron,2 Jean Gagnon1 and Jean-Yves Cesbron1

1CNRS LAPM 5163–Université Joseph Fourier; Grenoble, France; 2INSERM U 707–2 Université Pierre et Marie Curie–Paris 6; Paris, France; 3Centre d’Immunologie de Marseille-Luminy, INSERM-CNRS; Marseille, France; 4Institut de Génétique et de Biologie Moléculaire et Cellulaire, Université Louis Pasteur de Strasbourg; Strasbourg, France†Presenting author; Email:

Despite large exposure to BSE in the UK, less than 180 patients had developed clinical vCJD by October 2009. This figure was closely anticipated in 2001, thanks to an epidemiological model whose main assumptions was that the risk of acquiring vCJD was exponentially decreasing during childhood, which was consistent with the age distribution of vCJD. Further investigation of the models showed that this decrease of risk during childhood could not be explained by the age variation of meat consumption, and was likely a consequence of an age dependent susceptibility to the disease. The more likely explanation for this strong age-susceptibility relationship during childhood is hormonal.

In this context, we investigated if there was a sex difference in human vCJD cases, and we used a mouse model to test a first hypothesis on the possible role of sexual hormones on the risk of prion diseases.

In the 167 vCJD cases reported in the UK as of January 2009, age at onset was significantly lower in women (two years) than in men after stratification on birth cohort. In C57/BL6N mice infected with ME-7 scrapie strain, incubation was shorter in females than in males. The incubation period increased in castrated male mice after intraperitoneal infection, but not after intracerebral inoculation. We also observed that androgen receptor deficient mice the incubation period of prion disease also increased after intraperitoneal inoculation. In contrast, in ovariectomised or estrogen receptor a defective female mice, no effect was observed on the incubation period of mouse prion disease.

These results show that androgens influence the prion diseases incubation period in a peripheral site.1


1. Loeuillet C, Boelle PY, Lemaire-Vieille C, Baldazza M, Naquet P, Chambon P, et al. Sex effect in mouse and human prion disease. J Infect Dis 2010; 202:648-54.

Risk.49: Creutzfeldt-Jakob Disease in Canada, 1998–2009

Zheng Wang,1,† Gerard Jansen,1, 2 Elina Olsen,1 Stacy Sabourin,1 Rolande D’Amour,1 Tim Connolly,1 Jennifer Kruse,1 Neil Cashman3 and Michael Coulthart1

1The Canadian Creutzfeldt-Jakob Disease Surveillance System; Public Health Agency of Canada; Ottawa, ON Canada; 2Department of Pathology; Ottawa Hospital; Ottawa, ON Canada; 3Brain Research Centre; University of British Columbia; Vancouver, BC Canada†Presenting author; Email:

Background. Creutzfeldt-Jakob Disease (CJD) is a fatal, transmissible neurodegenerative disease with sporadic, genetic and acquired forms. In 1998, Canada launched comprehensive national CJD surveillance to assess the characteristics of CJD in Canada and its risks to the health of Canadians. This study describes the broad characteristics of CJD in Canada from 1998–2009.

Methods. Case ascertainment was based on internationally accepted criteria. Demographic information and risk-factor data were collected by standardized questionnaire and medical chart review. Poisson regression, descriptive analysis, and case investigation were employed.

Results. A total of 453 CJD deaths in Canadian residents were registered from 1998–2009. Four hundred and fifteen (92%) were sporadic (sCJD), 33 (7%) were genetic and five (1%) were acquired. Average annual sCJD mortality was 1.1 per million population, increasing gradually from 0.9 in 1999 to 1.4 in 2009 (P = 0.27). All provinces saw average annual mortalities ranging from 1.0 to 1.5 (P = 0.85), except three territories where population is small (~25,000 to ~45,000), sCJD occurred equally in both genders at 1.1. sCJD was rare under 50 years of age with only 11 cases identified (2.7%). Mortality increased after 50 and peaked at 8 per million in the 70–74 age group. Median age at death was 69 and median duration of illness was 4 months. Genetic TSE accounted for 33 deaths: 19 were GSS (P102L: 5, D202N: 2, P105T: 2, Q217R:1, A117V: 1, unknown mutation: 8); 13 were familial CJD (E200K: 9, D178N: 2, V203I: 1, V189I:1); one was FFI (D178N). Median age for genetic TSE was 59 and median duration of illness was 27 months. For the five acquired cases of CJD, four were associated with dura mater procedures (3 Lyodura, 1 Tutoplast) and were identified from 1998–2003 in patients aged 14–59. Investigation indicated the infections possibly occurred from 1981–1992 with incubation times from 10–16 years. One biochemically and neuropathologically confirmed variant CJD death occurred in 2002 in a person under 40 years old, likely acquired overseas.

Discussion and Conclusion. Characteristics of CJD in Canada are consistent with those observed in other countries. The increase in sCJD mortality can be at least partly attributed to increased awareness of CJD among referring clinicians. The finding of four dura matter associated CJD cases and one imported vCJD case in Canada demonstrate risks to Canadians from acquired CJD exist. Continued surveillance for iatrogenic risks and novel forms of CJD is warranted.

Risk.50: Investigating Dental Treatment as a Possible Risk Factor for Variant Creutzfeldt-Jakob Disease (vCJD) in the UK

Dawn Everington,1 Andrew Smith,2 Pauline Watt,1 Fiona Ord,1 Anna Molesworth,1 Robert Will1,† and Hester Ward1

1National CJD Surveillance Unit; Edinburgh, UK; 2College of Medical, Veterinary and Life Sciences; University of Glasgow; Glasgow, UK;†Presenting author; Email:

Introduction. The potential for vCJD transmission in the healthcare setting has raised concerns over the risk posed by dental surgery. The aim of this study was to determine whether dental treatment was a possible risk factor for vCJD.

Methods. Dental treatment records were collected from general dental practitioners or, where this was not possible, from NHS Dental Practice Board payment schedules. We looked for links between vCJD cases and whether there was an excess of dental treatment in vCJD cases compared with general population controls.

Results. Data were available for 49% (79/162) of cases and 82% (503/610) of controls. Two pairs of cases had attended the same dental practice, multiple treatment data were traced for one pair. While theoretically possible that the same instruments could have been used on both cases, after considering the type and timing of interventions we propose that the probability of cross-infection is very small. The review of specific dental treatments showed that there was no evidence that vCJD cases experienced an excess of any type of dental treatment compared with controls.

Conclusions. This study provided no compelling evidence of a strong association between dental treatment and vCJD, however because of the limited availability of dental information, and the possibility of undetected asymptomatic infection, we cannot exclude dental treatment as a possible risk factor for vCJD. We support current health policy to ensure that high standards of cleaning and sterilization of re-usable dental instruments are maintained.


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

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


USA 2011


National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

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

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

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

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

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

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

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


Saturday, March 5, 2011


Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

Friday, August 12, 2011

Creutzfeldt-Jakob disease (CJD) biannual update (2011/2), Incidents Panel, National Anonymous Tonsil Archive

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011

see video here ;

Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.

my comments to PLosone here ;

Sunday, August 09, 2009

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

Tuesday, August 18, 2009

BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

Friday, February 11, 2011

Creutzfeldt-Jakob disease (CJD) biannual update (2010/1) Emerging infections/CJD

Friday, April 15, 2011




"which includes the ___elimination___ of Prion activities ($5,473,000),"

All Other Emerging and Zoonotic Infectious Diseases CDC‘s FY 2012 request of $52,658,000 for all other emerging and zoonotic infectious disease activities is a decrease of $13,607,000 below the FY 2010 level, which includes the elimination of Prion activities ($5,473,000), a reduction for other cross-cutting infectious disease activities, and administrative savings. These funds support a range of critical emerging and zoonotic infectious disease programs such Lyme Disease, Chronic Fatigue Syndrome, and Special Pathogens, as well as other activities described below.


BY Philip Yam

Yam Philip Yam News Editor Scientific American

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.


Laying Odds

Are prion diseases more prevalent than we thought?

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

Revisiting Sporadic CJD

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

Singeltary has similar inclinations. ...



Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam


Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

Newsdesk 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

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.

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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.

Terry S. Singeltary, Sr Bacliff, Tex

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

DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen) USA: Loch in der Mauer Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehördensind lax.Link auf diesen Artikel im Archiv:

"Löcher wie in einem Schweizer Käse" hat auch Terry Singeltary im Regelwerk der FDA ausgemacht. Der Texaner kam auf einem tragischen Umweg zu dem Thema: Nachdem seine Mutter 1997 binnen weniger Wochen an der Creutzfeldt-Jakob-Krankheit gestorben war, versuchte er, die Ursachen der Infektion aufzuspüren. Er klagte auf die Herausgabe von Regierungsdokumenten und arbeitete sich durch Fachliteratur; heute ist er überzeugt, dass seine Mutter durch die stetige Einnahme von angeblich kräftigenden Mitteln erkrankte, in denen - völlig legal - Anteile aus Rinderprodukten enthalten sind.

Von der Fachwelt wurde Singeltary lange als versponnener Außenseiter belächelt. Doch mittlerweile sorgen sich auch Experten, dass ausgerechnet diese verschreibungsfreien Wundercocktails zur Stärkung von Intelligenz, Immunsystem oder Libido von den Importbeschränkungen ausgenommen sind. Dabei enthalten die Pillen und Ampullen, die in Supermärkten verkauft werden, exotische Mixturen aus Rinderaugen; dazu Extrakte von Hypophyse oder Kälberföten, Prostata, Lymphknoten und gefriergetrocknetem Schweinemagen. In die USA hereingelassen werden auch Blut, Fett, Gelatine und Samen. Diese Stoffe tauchen noch immer in US-Produkten auf, inklusive Medizin und Kosmetika. Selbst in Impfstoffen waren möglicherweise gefährliche Rinderprodukte enthalten. Zwar fordert die FDA schon seit acht Jahren die US-Pharmaindustrie auf, keine Stoffe aus Ländern zu benutzen, in denen die Gefahr einer BSE-Infizierung besteht. Aber erst kürzlich verpflichteten sich fünf Unternehmen, darunter Branchenführer wie GlaxoSmithKline, Aventis und American Home Products, ihre Seren nur noch aus unverdächtigem Material herzustellen.

"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA regulations. ...

Suspect symptoms

What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

28 Mar 01

Like lambs to the slaughter 31 March 2001 by Debora MacKenzie Magazine issue 2284. Subscribe and get 4 free issues. FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.

2 January 2000

British Medical Journal

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

15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.


USA sporadic CJD cases rising ;

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

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

Thursday, July 08, 2010


Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral vCJD...


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