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