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From: TSS (
Subject: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT]
Date: February 22, 2003 at 7:38 am PST

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

From the Division of Viral and Rickettsial Diseases (Drs. Belay and
Schonberger and R.A. Maddox), National Center for Infectious Diseases,
Centers for Disease Control and Prevention, Atlanta, GA; and National
Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of
Neuropathology, Institute of Pathology, Case Western Reserve University,
Cleveland, OH. Received May 7, 2002. Accepted in final form August 28,
2002. Address correspondence and reprint requests to Dr. Ermias D.
Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.
176 Copyright © 2003 by AAN Enterprises, Inc.

Views & Reviews

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

Ermias D. Belay, MD; Ryan A. Maddox, MPH; Pierluigi Gambetti, MD; and
Lawrence B. Schonberger, MD

Abstract—Transmissible spongiform encephalopathies (TSEs) attracted
increased attention in the mid-1980s because of the emergence among UK
cattle of bovine spongiform encephalopathy (BSE), which has been shown
to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob
disease (vCJD). The BSE outbreak has been reported in 19 European
countries, Israel, and Japan, and human cases have so far been
identified in four European countries, and more recently in a Canadian
resident and a US resident who each lived in Britain during the BSE
outbreak. To monitor the occurrence of emerging forms of CJD, such as
vCJD, in the United States, the Centers for Disease Control and
Prevention has been conducting surveillance for human TSEs through
several mechanisms, including the establishment of the National Prion
Disease Pathology Surveillance Center. Physicians are encouraged to
maintain a high index of suspicion for vCJD and use the free services of
the pathology center to assess the neuropathology of clinically
diagnosed and suspected cases of CJD or other TSEs.

NEUROLOGY 2003;60:176–181

Transmissible spongiform encephalopathies (TSEs),
also known as prion diseases, are neurodegenerative
disorders that occur in humans and animals. They are
believed to be caused by the accumulation in neurons
of an abnormal isoform of a membrane glycoprotein
known as the prion protein. TSEs in humans generally
occur as a rare, sporadic disease with no recognizable
pattern of transmission or as a familial disease associated
with prion protein gene mutations.1 Iatrogenic
transmission of Creutzfeldt-Jakob disease (CJD) is relatively
uncommon, but may occur in outbreaks (e.g.,
human growth hormone–associated CJD) that can be
controlled with the implementation of appropriate preventive
measures.2-4 In the 1980s, the emergence in
Europe of a large outbreak of bovine spongiform encephalopathy
(BSE) in cattle and, more recently, an
outbreak of a new variant form of CJD (vCJD) in humans
linked with food-borne transmission of the BSE
agent has raised serious concerns about the geographic
spread and ultimate number of cases of these diseases.
In April 2002, a probable case of vCJD was identified
in a Florida resident who was born in Britain and
raised there throughout the 1980s, a period when the
BSE outbreak was rising and no control measures to
prevent human exposure were instituted. Increased
concern about the occurrence of TSEs led to increased
surveillance and implementation of BSE control measures
in the United States and other countries currently
free of endemic BSE and vCJD.

The presumed food-borne transmission of BSE to
humans has also raised concerns about the possible
zoonotic transmission of chronic wasting disease
(CWD) of deer and elk.5 CWD was first recognized in
the late 1960s among captive mule deer housed in
research facilities in Fort Collins, CO. Since then,
the disease has been shown to be endemic among
free-ranging deer and elk in northeastern Colorado
and southeastern Wyoming.6 From 2000 through
2002, CWD in free-ranging deer was detected in locations
outside of the known endemic areas in western
Colorado, Nebraska, New Mexico, South Dakota,
Wisconsin, and Saskatchewan, Canada. Beginning in
1996, outbreaks of CWD in privately-owned elk
farms were reported in different parts of the United
States and Canada, including Alberta, Colorado,
Kansas, Montana, Nebraska, Oklahoma, Saskatchewan,
and South Dakota.7

Bovine spongiform encephalopathy. BSE was
first identified as an emerging TSE in 1986 in the
United Kingdom where it caused a major outbreak
among cattle that is now on the decline. Statistical
models have indicated that the first BSE cases may
have occurred in the early 1980s.8 As of June 30,
2002, over 179,300 cases of BSE had been confirmed
in Great Britain; 1,032 of the cases occurred in 2001
and 665 during the first half of 2002.9 However, the
actual number of cattle infected with the BSE agent
has been estimated to be around 1 million.10 BSE is
believed to have resulted from the feeding of cattle
with contaminated protein-rich meat-and-bone meal
produced by rendering discarded animal carcasses.11
The leading hypotheses for the origin of BSE include
contamination of the meat-and-bone meal by an
agent causing scrapie in sheep or by a spontaneously
occurring, unrecognized BSE in cattle. Before the
late 1970s, rendering of carcasses to produce meatand-
bone meal had included a solvent extraction and
solvent recovery steps that subjected the rendered
material to prolonged heating in the presence of a
hydrocarbon solvent. Omission of these treatment
steps in the late 1970s and early 1980s in most rendering
plants in the United Kingdom is believed to
have contributed to the emergence of BSE by allowing
passage of the infective agent to the finished
product.11 As more cattle died of BSE, their carcasses
continued to be rendered and fed to other cattle, and
this practice amplified the BSE outbreak until the
use of ruminant protein in ruminant feed was
banned in the United Kingdom in 1988.11,12 Consistent
with the 2- to 8-year incubation period of the
disease in cattle, the BSE outbreak in the United
Kingdom declined beginning in 1993, about 5 years
after the feed ban, although cases continue to occur
at a much lower rate. The continued occurrence of
BSE, particularly among cattle born after the 1988
ban, was primarily attributed to cross-contamination
of cattle feed by feed intended for nonruminant species
that could have been contaminated by BSE.13

In 1989, the concern that BSE may cross species
and infect humans precipitated a ban in the United
Kingdom to exclude specified risk materials (e.g.,
brain, spinal cord, distal ileum) from cattle products
destined for human consumption.12 The UK preventive
measures were increasingly tightened, and a
policy was put in place in 1996 to prevent cattle aged
30 months, regardless of their health status, from
entering the human or animal food chains.13

Elsewhere in Europe, through 1999, the occurrence
of BSE among native cattle had been reported
in Belgium, France, Ireland, Liechtenstein, Luxembourg,
Netherlands, Portugal, and Switzerland.14

From 2000 through 2001, the reported incidence of
BSE rose in some of these European countries, and
initial indigenous BSE cases were detected in Austria,
the Czech Republic, Denmark, Finland, Germany,
Greece, Italy, Japan, Slovakia, Slovenia, and
Spain. The growing number of countries with endemic
BSE led to increased public concern about the
safety of European and Japanese cattle products. In
response to these BSE outbreaks, the European
Commission proposed a temporary prohibition of the
use of animal protein in all farmed animals, pending
re-evaluation of other control measures adopted by the
member states.15 These control measures, which varied
by country, included ruminant feed bans, removal of
specified risk materials, such as the brain and spinal
cord, from cattle products intended for human use, and
mandatory brain testing of cattle older than 30 months
that are destined for human consumption. Most recently,
during the first half of 2002, Israel and Poland
reported their first indigenous BSE cases. BSE in imported
cattle only, in the absence of indigenous cases,
was reported in Canada (1 case), Falkland Islands
(1 case), and Oman (2 cases).14

Since 1989, to prevent the introduction of BSE into
the United States, the US Department of Agriculture
(USDA) restricted the importation of live cattle and
certain cattle products from the United Kingdom
and other BSE-endemic countries. In 1997, this restriction
was expanded to prohibit importation of cattle and
certain cattle products from all European countries,
and most recently from Japan and Israel. In addition,
the USDA trained veterinarians and veterinary laboratory
workers on the clinical and pathologic manifestations
of BSE and instituted an ongoing BSE
surveillance program.16 As of September 2002, these
USDA surveillance efforts, including analysis of
brain specimens from 36,594 cattle, had detected no
evidence of the occurrence of BSE in the United
States. To provide protection against the spread of
BSE should it be introduced into the United States,
the Food and Drug Administration (FDA) published
a final rule in 1997 that prohibited the use of most
mammalian protein, particularly ruminant tissues,
in the manufacture of ruminant feed.17,18

Variant Creutzfeldt-Jakob disease. Variant
CJD was reported as a distinct disease entity in
April 1996 after the UK government’s expert advisory
committee announced its conclusion that the
BSE agent may have crossed the species barrier,
causing an outbreak of the disease in humans.19 The
possibility that BSE could cross the species barrier
to infect humans had been suspected about 6 years
earlier with the identification among domestic cats
in the United Kingdom of feline spongiform encephalopathy,
whose agent characteristics were similar to
the BSE agent. Transmission of the BSE agent presumably
via ingestion of contaminated feed has also
been reported in exotic ungulates and wild cats in
British zoos.11

As of October 4, 2002, a total of 138 vCJD cases
had been reported: 128 definite or probable cases in
the United Kingdom (includes one UK resident who
was hospitalized and died in Hong Kong), 6 in
France, and 1 each in Canada, Ireland, Italy, and the
United States.12,20-22 The Canadian, Irish and US
cases resided in the United Kingdom during a key
exposure period of the UK population to the BSE
agent. The continued occurrence of vCJD cases only
in persons who have lived in BSE-endemic areas,
particularly the United Kingdom where most BSE
cases were identified, and several animal and molecular
laboratory studies provide strong evidence that
vCJD is causally linked with BSE.23-27

January (2 of 2) 2003 NEUROLOGY 60 177

Statistical analysis of the numbers of definite and
probable vCJD cases reported through June 2000
indicated an increasing trend for the vCJD outbreak
in the United Kingdom.28 However, the persistency
of this increasing trend or the eventual magnitude of
the vCJD outbreak remains unknown. From statistical
models, UK researchers had predicted that the
total number of vCJD cases would likely range between
70 and 136,000, depending upon various assumptions
about the mean incubation period.29 Two
recently published studies using other statistical
models suggested that the UK vCJD outbreak might
not exceed several hundred clinical cases.30,31 One of
these studies pointed out the possibility that the incubation
period could be so long that even if millions
of persons were infected with the agent of vCJD, a
large majority of these people could die of other competing
causes before they developed the TSE illness.
31 Such infected persons could pose a potential,
albeit unknown, risk for secondary transmission of
the agent (e.g., transmissions by contaminated surgical

A most striking feature of patients with vCJD
compared with patients who have classic CJD is
their unusually young age at the time of illness onset.
18,32 On the basis of the initially reported 110
vCJD deaths in the United Kingdom, the median age
at death was 28 years; 60% died at 30 years of age,
and approximately 13% died as teenagers (R.G. Will,
personal communication, 2002). In addition to the
age distribution, the clinicopathologic profile of patients
with vCJD and immunoblot characteristics of
the vCJD agent differed from that seen in patients
with classic CJD (table 1). The UK patients with
vCJD usually presented with early and persistent
psychiatric symptoms, commonly with depression,
anxiety, and withdrawal.32 Some of the patients were
initially regarded as having a primary psychiatric
illness and were treated by a psychiatrist early in
the course of the disease. Evaluation of the clinical
manifestations of the first 100 patients with vCJD in
the United Kingdom indicated that the onset of
frank neurologic signs such as gait disturbance, slurring
of speech, and tremor was usually delayed by
several months after illness onset.32,33 The most
striking early neurologic sign in some of these patients
was persistent dysesthesia or paresthesia.
Other neurologic signs, including chorea, dystonia,
and myoclonus, frequently developed late during the
course of the illness (see the Appendix). None of the
vCJD cases reported to date had the EEG tracing of
periodic triphasic complexes often seen in patients
with sporadic CJD. In addition, a prominent, symmetric
pulvinar high signal on T2-weighted or proton
density–weighted MRI has been reported in about
78% of patients with vCJD.34 This prominent pulvinar
high signal has been designated the “pulvinar sign.”
The MRI high signals have been shown to correlate
with underlying neuropathologic findings of astrocytosis
and neuronal loss. The polymorphic codon 129 of
the prion protein gene in all patients with vCJD on
whom genetic studies have been performed was
shown to be homozygous for methionine.31

Neuropathologic evaluation of a brain autopsy
specimen is required for a confirmatory diagnosis of
vCJD. In addition to the presence of the typical spongiosis,
gliosis, and neuronal loss that are considered a
hallmark of CJD, the neuropathologic characteristics of
vCJD include the presence of numerous amyloid
plaques that are surrounded by a halo of spongiform
changes, resembling the “florid plaques” first described
in experimental transmission of Icelandic scrapie in
mice.35 In addition, immunohistochemical staining
demonstrates marked accumulation of the diseaseassociated
prion protein in diffuse or pericellular deposits
in the cerebrum and cerebellum. Detection of
the protease-resistant prion protein by immunohistochemical
and immunoblot analyses outside of the
brain in lymph nodes, spleen, and tonsils of patients
with vCJD has been reported.36,37 On immunoblot
analysis, the protease-resistant prion protein fragment
from patients with vCJD characteristically has
a glycoform ratio that has not been described in sporadic

Table Clinical and laboratory characteristics distinguishing variant
Creutzfeldt-Jakob disease (CJD) from the classic form of CJD

Characteristics Variant CJD Classic CJD

Median age at death, y 28 68

Clinical presentation Psychiatric or sensory symptoms; delayed
appearance of neurologic signs
Dementia associated with
neurologic signs

Median illness duration, mo 13 4

Periodic short waves on EEG tracings Absent In about 75% of patients
Symmetrical pulvinar high signal on MRI In over 75% of patients Not reported

Codon 129 genotype Methionine/methionine Polymorphic

Numerous “florid plaques” on neuropathology In all patients Absent

Immunohistochemical analysis of brain tissue Marked accumulation of
PrPres Variable

Increased glycoform ratio on immunoblot
analysis of PrPres
In all patients Not reported

Presence of infective agent in lymphoid tissues Readily detected Not
readily detected

PrPres  Protease-resistant prion protein.

178 NEUROLOGY 60 January (2 of 2) 2003

The reason for the predominant occurrence of
vCJD among patients under 30 years of age is unknown.
Differential consumption of potentially contaminated
meat products by the younger population
has been suggested as a possible contributory factor
to the age distribution of vCJD.39 However, vCJD
has been confirmed in a 74-year-old man who died in
1999, 7 months after illness onset.40 The older patient’s
clinical and pathologic phenotype and methionine
homozygosity at codon 129 were similar to that
of other patients with vCJD. He presented with psychiatric
symptoms and was initially admitted to a
psychiatric unit and treated for a psychotic illness.
The patient became increasingly forgetful and unsteady
and had recurrent falls. He had complaints of
various episodes of pain for which no cause could be
established. Histopathologic examination of the
brain autopsy tissue showed the presence of florid
plaques in large numbers in the cerebral and cerebellar
cortices. Although clearly an outlier, the identification
of vCJD in a patient at 74 years of age
indicates that some persons in older age groups can
potentially be susceptible to contracting vCJD.

Creutzfeldt-Jakob disease surveillance in the
United States.

The emergence of BSE and vCJD in
Europe created a concern that US residents might be
exposed to BSE-contaminated cattle products from
Europe or to possibly unrecognized BSE in the United
States. For example, concern was raised about the
possibility that US residents who traveled or resided
in the United Kingdom or other BSE-endemic countries
since 1980 could potentially have been exposed
to the BSE agent. In April 2002, the first case of
vCJD in a US resident was reported in a 22-year-old
Florida patient who was born and raised in Britain and
moved to the United States in 1992. As of October
2002, the patient, whose illness began in November
2001, was still alive. Her illness fulfills UK criteria for
probable vCJD.

In 1998, the American Medical Association Council
on Scientific Affairs called for increased CJD surveillance
to monitor the possible occurrence of vCJD in the
United States.41 Among other recommendations, the
council indicated that physicians should become knowledgeable
about BSE to be able to advise their patients
about the possible risk of exposure to the BSE agent
during travel abroad. To facilitate such advice to travelers,
CDC provides a traveler’s advisory about the possible
risk of BSE exposure during travel to Europe.42
There is also a concern about a possible, albeit theoretical,
risk of secondary person-to-person transmission of
the vCJD agent via blood and blood products. This risk
is considered theoretical because no convincing evidence
for transmission of either classic CJD or vCJD
via blood or blood products has been reported in the
human population. However, the world’s limited experience
with vCJD, the presence of the vCJD agent in
lymphoid tissues of infected patients, and transmission
of the BSE agent by blood transfusion in an experimental
sheep model during the incubation period contributed
to the concern about the possible transmission of
the vCJD agent through the blood supply.43 This concern
is further complicated by the long incubation period
of the disease. Because the incubation period is
measured in years or decades, should blood-borne
transmissions occur in humans, many recipients would
have been exposed before the first blood-borne infection
is detected. Additional concerns have also been raised
about the potential for person-to-person spread of the
vCJD agent by surgical instruments coming in contact
with infected tissues, such as lymphoid tissues of patients
incubating vCJD.

Because the most likely source of exposure to the
BSE agent for US residents is consumption of contaminated
food in the United Kingdom, the FDA in 1999
instituted a deferral policy to exclude from donating
blood any person who traveled to the United Kingdom
for a cumulative period of 6 months or more between
1980 and 1996. The Transmissible Spongiform Encephalopathy
Advisory Committee of the FDA recently
recommended further tightening the UK travel criteria
to a cumulative period of 3 months or more and added
additional deferral criteria, including the exclusion of
donors who visited or resided in other European countries
for a cumulative period of 5 years or more during
1980 to the present.44

In 1996, after vCJD was reported in the United
Kingdom, the CDC enhanced its CJD surveillance to
monitor the possible occurrence of the disease in the
United States. One enhancement, which focused on
the striking difference in age distribution of vCJD,
included periodic review of national CJD mortality
data to monitor any increase in the occurrence of
CJD among unusually young patients. In collaboration
with state and local health authorities, the CDC
initiated follow-up investigations of patients with
CJD younger than 55 years through reviews of their
clinical and neuropathologic records. In addition, in
collaboration with the American Association of Neuropathologists,
the CDC established the National
Prion Disease Pathology Surveillance Center at Case
Western Reserve University, Cleveland, OH.45,46
Through its contacts with US pathologists, this center
helps to monitor for the occurrence of vCJD and
other potentially emerging human TSEs in the
United States regardless of the age of the patient or
clinical diagnosis. It also makes available state-ofthe-
art free diagnostic services for physiciandiagnosed
or suspected cases of TSE in humans.
Physicians are encouraged to make all efforts to arrange
for a brain autopsy in all such cases and to
take advantage of the free diagnostic services provided
by the National Prion Disease Pathology Surveillance
Center to assess the neuropathology of the
patients. Given the many unknowns associated with
TSEs, such assessments would not only help to confirm
clinical diagnoses of these diseases, but also
help to monitor the occurrence of a new TSE, such as
vCJD or possibly a human form of CWD, in the
United States. More detailed information about the
activities of the Center can be obtained at its

January (2 of 2) 2003 NEUROLOGY 60 179

Is chronic wasting disease transmissible to humans
or cattle?

The occurrence of CWD in several
states that were not known to be endemic foci for the
disease has increased the concern about a widespread
outbreak of CWD in many areas of the country,
and its possible transmission to humans and
domestic animals such as cattle. A recently published
experimental study has demonstrated transmission
of the CWD agent to cattle by intracerebral
inoculation.48 An experiment to determine susceptibility
of cattle to CWD by oral challenge is currently
in progress. The efficiency by which CWD-associated
prions influence the conversion of prion protein from
different sources, including cervids, cattle, and humans,
has been evaluated by cell-free conversion experiments.
49,50 The cell-free prion protein conversion
reactions are believed to assess the molecular compatibility
of disease-associated prions from one species
with normal prion protein obtained from
different species. These cell-free conversion experiments
indicated that the efficiency of CWDassociated
prions in converting bovine prion protein
was an average of at least 5- to 12-fold weaker than
the homologous conversion of cervid prion protein
and bovine prion protein. Similar experiments indicated
that the efficiency of CWD-associated prions in
converting human prion protein was over 14-fold
weaker than the homologous conversion of cervid prion
protein and over fivefold weaker than the homologous
conversion induced by CJD-associated prions. Although
conversion studies showed some degree of incompatibility
of cervid prion protein with that of cattle
and humans, the authors indicated that it may be premature
to draw firm conclusions about CWD naturally
transmitting to humans or cattle. Lack of efficient cellfree
conversion of human prion protein by BSEassociated
prions has also been reported, despite the
fact that BSE has been shown to be transmitted to
humans.49 The authors of the cell-free conversion studies
also indicated that other factors are important in
determining in vivo transmission of TSE agents between
species, including dose and strain of the agent,
route of infection, stability of the agent inside and outside
of the host, and the efficiency of agent delivery to
the nervous system.

A recent epidemiologic and laboratory investigation
of three unusually young patients with CJD who regularly
consumed venison did not identify convincing evidence
for a causal link between CWD and the patients’
illness.5 Two of the patients, aged 28 and 30 years at
death, were hunters, and the third patient, aged 28
years, consumed venison harvested by family members.
None of the patients was reported to have consumed
deer meat obtained from the known CWDendemic
areas of Colorado and Wyoming. The patients’
disease phenotype and the prion protein gene polymorphism
at codon 129 were heterogeneous, possibly indicating
lack of exposure to a similar agent. This was
unlike patients with vCJD in whom the disease phenotype
and codon 129 polymorphism had some homogeneity,
owing to infection of the patients by the same
agent of BSE. In addition, brain tissues from over
1,000 deer and elk harvested in the areas where the
venison consumed by the three patients originated
from tested negative for CWD.

Although strong evidence for CWD transmission
to humans is lacking, it should be recognized that
limited studies designed to seek such evidence have
been conducted. Given the BSE experience in Europe
where an animal TSE previously believed to be nonpathogenic
to humans was later shown to be responsible
for an outbreak of vCJD, both epidemiologic
and laboratory studies and ongoing CJD surveillance
remain critical for continuing to assess the risk, if
any, of CWD transmission to humans.


The following are prominent clinical features that lead to a suspected
diagnosis of variant Creutzfeldt-Jakob disease (CJD):

1. Young age of the patient (commonly  55 years).
2. Early psychiatric symptoms or persistent painful sensory symptoms
such as dysesthesia or paresthesia.
3. Dementia and delayed appearance of ataxia and at least one of the
following three neurologic signs: myoclonus, chorea, or dystonia.
4. A normal or abnormal EEG but not the diagnostic EEG changes often
seen in classic CJD.
5. A prominent, symmetrical pulvinar high signal on T2-weighted or
proton density–weighted MRI.
6. Duration of illness of at least 6 months.
7. Routine investigations of the patient do not suggest an alternative
non-CJD diagnosis.
8. No history of receipt of cadaveric human pituitary growth hormone or
a dura mater graft.
9. No history of CJD in a first-degree relative or absence of prion protein
gene mutation in the patient.
The authors thank John O’Connor for his suggestions and editorial
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31. Huillard d’Aignaux JN, Cousens SN, Smith PG. Predictability of the
UK variant Creutzfeldt-Jakob disease epidemic. Science 2001;294:
32. Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant
Creutzfeldt-Jakob disease. Ann Neurol 2000;47:575–582.
33. Spencer MD, Knight RSG, Will RG. First hundred cases of variant
Creutzfeldt-Jakob disease: retrospective case note review of early
and neurological features. BMJ 2002;324:1479–1482.
34. Zeidler M, Sellar RJ, Collie DA, et al. The pulvinar sign on magnetic
resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000;
35. Ironside JW. Neuropathological findings in new variant CJD and
transmission of BSE. FEMS Immunol Med Microbiol 1998;21:
36. Hill AF, Zeidler M, Ironside J, Collinge J. Diagnosis of new variant
Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 1997;349:99–100.
37. Hill AF, Butterworth RJ, Joiner S, et al. Investigation of variant
Creutzfeldt-Jakob disease and other human prion diseases with tonsil
biopsy samples. Lancet 1999;353:183–189.
38. Parchi P, Capellari S, Chen SG, et al. Typing prion isoforms. Nature
39. Gore SM, Bingham S, Day NE. Age related dietary exposure to meat
products from British dietary surveys of teenagers and adults in the
1980s and 1990s. BMJ 1997;315:404–405.
40. Lorains JW, Henry C, Agbamu DA, et al. Variant Creutzfeldt-Jakob
disease in an elderly patient. Lancet 2001;357:1339–1340.
41. Tan L, Williams MA, Khan MK, Champion HC, Nielsen NH. Risk of
transmission of bovine spongiform encephalopathy to humans in the
United States: report of the council on scientific affairs. JAMA 1999;
42. Centers for Disease Control and Prevention. Update 2002: bovine
encephalopathy and variant Creutzfeldt-Jakob disease. Available at: Accessed October 29, 2002.
43. Houston F, Foster JD, Chong A, Hunter N, Bostock CJ. Transmission of
BSE by blood transfusion in sheep. Lancet 2000;356:999–1000.
44. Food and Drug Administration. Guidance for industry: revised preventive
measures to reduce the possible risk of transmission of Creutzfeldt-
Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) by
blood and blood products. Available at:
cjdvcjd.pdf. Accessed April 1, 2002.
45. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-
Jakob disease in the United States: 1979–1998. JAMA 2000;284:2322–
46. Holman RC, Khan AS, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States, 1979–1994: using national mortality data
to assess the possible occurrence of variant cases. Emerg Infect Dis
47. National Prion Disease Pathology Surveillance Center (homepage).
Available at: Accessed April 1, 2002.
48. Hamir AN, Cutlip RC, Miller JM, et al. Preliminary findings on the
experimental transmission of chronic wasting disease agent of mule
deer to cattle. J Vet Diagn Invest 2001;13:91–96.
49. Raymond GJ, Hope J, Kocisko DA, et al. Molecular assessment of the
potential transmissibilities of BSE and scrapie to humans. Nature
50. Raymond GJ, Bossers A, Raymond LD, et al. Evidence of a molecular
barrier limiting susceptibility of humans, cattle and sheep to chronic
wasting disease. EMBO 2000;19:4425–4430.
January (2 of 2) 2003 NEUROLOGY 60 181



i would like to respond to the above article. i will first post
my submission to the Neurology Journal about this article with
replies (i do not know if they will publish any of it me being
the ''lay person'' et al). probably will rule out any of it getting
published after me posting this to WWW, but i could not hold this
data with the recent news media reports and insinuations of no CWD
transmission to man. there are just too many hunters and family
and friends of hunters, with freezer's full of venison, that
will take these 'false assurances' to heart. so i would like to
post some facts (fact is, they still don't know). then i will post
some transmission studies, then some private comments from other
well known and respected TSE Scientist, some data i have accumulated
(some never posted), and some questions...

thank you...TSS


Subject: RE-Monitoring the occurrence of emerging forms of
Creutzfeldt-Jakob disease in the United States
Date: Mon, 27 Jan 2003 17:29:36 -0600
From: "Terry S. Singeltary Sr."
To: "Terry S. Singeltary Sr."

Thank you for your Post-Publication Peer Review

We value your contribution. The Neurology Post-Publication Peer Reviews
editor will review your submission, which if accepted, should be
viewable within a few days.

If you have a problem with this process or other comments that do not
pertain to the submission of electronic responses, then please use our
feedback form.

Here is what your Post-Publication Peer Review will look like online:

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States

Terry S. Singeltary,
retired (medically)

Send Post-Publication Peer Review to journal:
Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States

Email Terry S. Singeltary:


i lost my Mother to hvCJD (Heidenhain Variant CJD) 12/14/97, so i would
like to make several comments on the attempt to monitor the occurrence
of emerging forms of CJD that the CDC and the National Prion Disease
Pathology Surveillance Center are attempting;

1st, i find it very disturbing, that with the findings from Asante,
Collinge et al that BSE transmission to the 129-methionine genotype can
lead to an alternate phenotype which is indistinguishable from type 2
PrPSc, the commonest sporadic CJD, i find it very disturbing that the
CDC and NPDP still refuse to make CJD and all human TSEs reportable
nationally. CJD and all human TSEs _must_ be made reportable in every
State and Internationally ASAP.

2nd, i also find it very disturbing that the only CJD Questionnaire that
the CDC and Case Western is using (when they decide to issue one), i
find it very disturbing that this CJD Questionnaire asks absolutely not
one question as to route and source. Only how the disease was diagnosed.
how will the route and source of this agent ever be traced in the USA if
we do not ask the questions pertaining to route and source? i only hope
that the CDC does not continue to expect us to still believe that the
85%+ of all CJDs which is sporadic CJD, is all spontaneous, without

we have many TSEs in the USA in both animal and man, and they are
spreading. CWD in deer/elk is spreading rapidly and CWD does transmit to
mink, ferret, sheep, cattle, and to the primate by inoculation, humans
are primates, so i would take these findings very seriously. with the
known incubation periods in other TSEs, oral transmission studies of
this may take much longer. also, another cow has gone down with CWD by
inoculations, this brings to 4 cattle infected with CWD by inoculation.
also, considering the fact the late Richard Marsh already has shown some
TSE to exist in the USA bovine, all this should warrant immediate
actions pertaining to making CJD/human TSEs reportable in every state.
we must issue a CJD Questionnaire to every victim/family of CJD/TSEs to
be filled out and to ask _real_ questions as pertaining to route and
source of this agent. to prolong this, will only spread the agent and
further expose many humans needlessly. also with Asante and Collinge et
al findings, 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?

-------- Original Message -------- Subject: re-BSE prions propagate as
either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43
-0000 From: "Asante, Emmanuel A" To:

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am
a Senior Scientist in the MRC Prion Unit and the lead author on the
paper. I have attached a pdf copy of the paper for your attention. Thank
you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you
will find in the paper, we have managed to associate the alternate
phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in
respect of Heidenhain variant CJD or Vicky Rimmer's version. It will
take further studies, which are on-going, to establish if there are
sub-types to our initial finding which we are now reporting. The main
point of the paper is that, as well as leading to the expected new
variant CJD phenotype, BSE transmission to the 129-methionine genotype
can lead to an alternate phenotype which is indistinguishable from type
2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I
can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial
College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG
Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: (until 9/12/02) New e-mail:
(active from now) ____________________________________

Diagnosis and Reporting of Creutzfeldt-Jakob Disease T. S. Singeltary,
Sr; D. E. Kraemer; R. V. Gibbons, R. C. Holman, E. D. Belay, L. B.

#Docket No. 01-068-1 Risk Reduction Strategies for Potential BSE
Pathways Involving Downer Cattle and Dead Stock of Cattle and Other
Species - TSS 1/21/03 (2)

In Reply to: Docket No. 01-068-1 Risk Reduction Strategies for Potential
BSE Pathways Involving Downer Cattle and Dead Stock of Cattle and Other
Species [TSS SUBMISSION] January 21, 2003

Re: Docket No. 01-068-1 -- (200,000 USA DOWNERS ANNUALLY) TSS 1/21/03

Re: Docket No. 02N-0273 – Substances Prohibited From Use In Animal Food
Or Feed;


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


CJD Watch/NEWS message board


[Back to Article] [See other Post-Publication Peer Reviews]

Copyright © 2003 by AAN Enterprises, Inc.

Subject: Letter to Neurology
Date: Mon, 3 Feb 2003 12:03:03 -0500
To: "''"

Dear Terry Singletary:
We do not, as a rule, post letters from the lay public. However, we are
having your letter reviewed. We will let you know the disposition within one

Managing Editor
Neurology online []
1351 Mount Hope Ave. Suite 203
Rochester, NY 14620
tel. (585) 275-5858
fax (585) 271-2009


Subject: Re: Letter to Neurology
Date: Mon, 03 Feb 2003 12:33:04 -0600
From: "Terry S. Singeltary Sr."

Greetings Neurology and hello Dr. Griggs and Kathy,

> We do not, as a rule, post letters from the lay public.

i know some that would dispute this about me being a
''lay public'' ;-)

a private email from the late Dr. Gibbs, a true pioneer
in the research of human/animal TSEs and one that never
wavered on helping the families and victims of this
horrible disease, and one that helped me many times
in trying to seek out the truth;

Subject: Re: Hello Dr. Gibbs...........
Date: Wed, 29 Nov 2000 14:14:18 -0500
From: "Clarence J. Gibbs, Jr., Ph.D."
To: "Terry S. Singeltary Sr."
References: <>

Hi Terry: 326 E Stret N.E., Washington, D. C. 20002. Better shrimp
and oysters than cards!!!! Have a happy holiday and thanks for all
the information you bring to the screen.
Joe Gibbs

although i still cannot dispute the fact i have no PhDs:-(

but this should/does not alter the facts in my research.

however i am very grateful you are even considering
a publishing of my rebuttal to the article, honored
in fact. and i thank you for that. those 2 items i
proposed are very important to the finding of route
and source of TSE agent, and the finding of the
true extent of these TSEs and to the protection of
public health in the USA and i would hope Globally.

i should quit while i am ahead at this point, but i
find it very important, that since you are even considering
my short reply, you should at least have this other very
important data, if for nothing else, your own personal use.

i have pasted the CJD surveillance unit in the USA
(New CJD Foundation CJD Questionnaire at Case Western)
CJD Questionnaire and the CJD Questionnaire i proposed,
and also some critical data on _endoscopy_ equipment and
CJD/TSEs for you to evaluate/compare and/or for your private
use, and the Warning from the W.H.O. this week about Global
BSE/TSEs and the 'audio' interview with Dr. Ricketts below,
speaking about a new study about .1 gram of BSE as being



Name of Patient*:
(not required; if provided, must be with express consent of family member)

Date form filled out: / / (mm/dd/yy)

Person filling out form:

Relationship of person filling out form to patient:

Location where patient died: State: County: City:

Location where patient resided: State: County: City:

Sex of patient: male female unknown

Race of patient: white African-American -- Asian/Pacific Islander
American-Indian/Alaskan Native Other (please identify:

Patient's date of birth: (mm/dd/yy)

Age of patient at onset of symptoms:

Date of patient's initial symptoms: (mm/dd/yy)

Age of patient at time of death:

Patient's date of death: (mm/dd/yy)

Duration of illness: months

Was this case referred to the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University in Cleveland,
Ohio? yes no unknown

If yes, by whom was this case referred?
Pathologist -- Neuropathologist -- Neurologist
Other Physician (please identify which kind:

Who made initial diagnosis of CJD or other TSE?

Pathologist - Neuropathologist - Neurologist
Other Physician (please identify which kind: )

Please describe the clinical neurological presentation of the illness
(list the symptoms or signs):

at onset of the illness:

during the course of illness:

Was an EEG (electroencephalogram) performed? yes -- no -- unknown

If yes,

how long after onset was the EEG performed?

how many times was the EEG performed?

can you indicate the results?

- slow periodic sharp waves (PSW)

- unilateral periodic sharp waves (LSW)

- not reported

- other

Was the cerebrospinal fluid tested for the 14-3-3 protein? yes - no -

If yes, what was the result? positive - negative - unknown

Was a brain biopsy performed? - yes - no - unknown

If yes, what was the result?_____positive for____

______negative for CJD and other TSE's


Was an autopsy performed? yes - no - unknown

If yes, what was the result? _____positive for____

______negative for CJD and other TSE's


Was the neuropathology of this case consistent with new variant CJD?
yes - no - unknown

What was the final diagnosis of this case?

___CJD, probably sporadic

___Familial (hereditary) CJD

___Iatrogenic (by infection) CJD; please specify_______________

___Gerstmann-Strausster-Scheinker Syndrome (GSS)

___Fatal Familial Insomnia (FFI)



* I hereby give consent to the Creutzfeldt-Jakob Disease Foundation,
Inc. to use the above information, including name of patient if
supplied, in connection with activities to promote the research,
education and awareness of Creutzfeldt-Jakob Disease and related
transmissible spongiform encephalopathies.



NOW (below), compare to the CJD questionnaire _i_ propose
for _all_ CJD/TSE victims/family in the USA;


1. NAME______________________________________

A. What is the subjects SURNAME____________________________
B. What is the subjects status? ___________________________
(1=suspect/confirmed CJD, 2=hospital control (specify
diagnosis), 3=GP control).
C. If the subject is a (suspect) case, are they alive on the
day of interview?_____(yes or no or not applicable)
D. What is your (respondent's) name?_______________________
(first name, and surname)
What is the relationship to (subject)?________________
E. DATE OF INTERVIEW__________________________
LOCATION OF INTERVIEW_____________________
F. NAME OF INTERVIEWER________________________

A. SEX___________________
B. BIRTH DATE____________
C. BIRTH PLACE___________ (country, state, county, city)
E. MARITAL*DOMESTIC STATUS__________________
(If the subject is female and is/has been married)
record the subjects maiden name if different from current surname.
F. PRESENT HOME ADDRESS_________________________
(ALSO, If deceased, last home address, before subject
became ill?)
G. Is/was subject right or left handed?__________________
F. How many years of full-time education?________________

A. Has the Subject had dental treatment other than fillings:
e.g. extractions or root canal work?_________________
If yes, record a description of treatment; with dates;
Dentists name and address____________________________
B. Has the Subject ever had any operations, including eye
operations or stitching of wounds?___________________
(If yes, record the year, hospital and type of operation).
(record total number of operations)

For each type of operation record the number of such operations
undergone, the year of the first such operation and the year of the last
such operation. When no such operations were undergone record 0 for the
number of operations.

NEUROLOGIC (brain)_____________________________
TONSILS OUT?___________________________________
APPENDIX OUT?__________________________________
ever received an ORGAN TRANSPLANT, including corneal or bone
marrow transplant?_____________________________________
kidney, liver, and other_______________________
C. BLOOD TRANSFUSION__________________________
BLOOD DONOR____________________________________
D. Has Subject ever been admitted to a
E. Has Subject ever been to see psychiatrist (reason and

F. MEDICATIONS, has Subject taken any medications regularly, (if
yes, record the date, name of the medication, the reason for taking it,
and route of administration) prompt for prescription drugs, including
insulin and type.
Prompt for hormone therapy or nutritional supplements including
oral contraceptives and hormone replacement therapy:
Prompt for homeopathic/herbal therapy:
Prompt for eyedrops


G. Has Subject ever been tested for allergy using

H. Has Subject ever received a treatment involving a course of
(If yes, record year, name of therapy, frequency, reason)

I. Has Subject been VACCINATED?_______________________________
(If yes, give name of vaccine, and route.)

J. Has Subject ever undergone lumbar puncture or electrical
tests involving needles?________________________________________________

K. Has Subject ever undergone acupuncture?____________________

L. Has Subject ever used drugs by needle?_____________________

M. Has Subject ever been tattooed, ear or body piercing of


(indicating years of birth and death) Subjects grandparents,
Subjects parents and parents siblings, Subject and siblings Subjects

A. From the genealogy, record whether the Subject has been
married more than once? ___________________________________________________

B. Have any of the BLOOD relatives of the Subject included in
the Pedigree above died with dementia (or remain alive with

C. Have any of these individuals been diagnosed as having
Creutzfeldt-Jakob disease, and or any other T.S.E.?________________
(if so, give name, address, and apprx. date of illness)


(1=definite 2=probable 3=possible 4=unable to confirm 5=not a

E. Has Subject had social contact, through family, friends or
work, with someone else who developed CJD?_____________________________
(record the persons name and the apprx. date of illness.)

F. Confirmation of social contact with case of CJD?____________

G. FOR NON-U.K. cases only, Has Subject lived in or visited the
United Kingdom during the period 1980-1999?________________________
(if yes, record dat and duration of visits)

A. Has Subject ever been a vegetarian for a period of 1 year or
more? (if yes), during what period was Subject vegetarian, and did the
Subject eat any meat or fish at all during this time?______________

B. Does Subject have a history of any other dietary
restrictions or eccentricities? (record apprx. dates and details of

C. How many years did Subject eat school
(give dates)

D. Has the Subject ever eaten animal food or pet
(If yes, record the types of food and dates)

E. How did/does the Subject like their steak

(1=well done 2=medium 3=medium-rare 4=rare 5=did not eat steak)

F. How often does/did Subject cut or chop up raw red meat or
bones, in their work or in their home?_______________________________

G. (For each of the following food items) How often did Subject
eat (food item)?
BRAIN_________________(specify animal which organ came from)
SAUSAGE OR PATE', STEAK TARTARE (raw minced steak with raw egg)
carpaccio, CHEESE, COWS MILK (1=drinks milk/eats breakfast
cereal with milk, 2=only in tea/coffee, 3=NO)_______________________

A. Did the Subject every HUNT, DRESS, AND EAT,
(if so, list location, and year, and list any specific organs
that the Subject may have considered to be a delicacy).

B. Did the Subject share a home with:

C. Has the Subject worked or stayed for more than one week on a
farm? (1=lived or worked, 2=stayed, 3=NO) If YES, did Subject work or
help with;

(If yes), did Subject participate in:
Treating cattle for Warble fly?______________
Dipping sheep?_________________________
Crop Spraying?________________________
(If the Subject took part in any of these activities), record
dates, places and details of the activity including agents
used; ________________________________________________________

D. Has the Subject used any of the following;
HOOF AND HORN____________
DRIED BLOOD________________
(if yes, record the item used and dates)

E. Has Subject ever DISSECTED ANIMAL EYES, for example at school?

6. RESIDENTIAL HISTORY (begin with the most recent residence and work
From(dd/mm/yy) TO(dd/mm/yy) STREET TOWN COUNTY STATE
(include zip code).

(begin with most recent occupation and work backwards)
FROM(dd/mm/yy) TO(dd/mm/yy) NAME OF EMPLOYER TOWN

A. Has the Subject ever worked in farming, the meat industry,
the pharmaceutical industry, or in a hospital?

B. Has the SUBJECT, their PARTNERS or PARENTS ever worked in the
following areas;

animal laboratories______________________________________________
pharmaceutical laboratories______________________________________
other research laboratories______________________________________
animal farming___________________________________________________
veterinary medicine______________________________________________
meat industry____________________________________________________
(catering other occupation involving animal products, including

*** NOTE ***

please include venison/sheep/lamb and the bovine to any of the above

example=brain tanning deer/elk hide or any other topics
that pertain to transmission of TSEs


example=antler velvet nutritional supplements


_any_ nutritional supplements??? name/ingredients


example=elk/deer brains ie/scrambled, sandwich or otherwise


COSMETICS-ie facial creams, eye make-up etc.



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

Greetings again Neurology,

snip...(snipped out repeated data/urls i posted again below)

Subject: Re: gutjnl_el;21 Terry S. Singeltary Sr. (3 Jun 2002) "CJDs
(all human TSEs) and Endoscopy Equipment"
Date: Thu, 20 Jun 2002 16:19:51 -0700
From: "Terry S. Singeltary Sr."
To: Professor Michael Farthing
References: <001501c21099$5c8bc620$>

Dear Gut,


here is my short submission i speak of,
lengthy one to follow below that;

>> Date submitted: 3 Jun 2002
>> eLetter ID: gutjnl_el;21
>> Gut eLetter for Bramble and Ironside 50 (6): 888
>>Name: Terry S. Singeltary Sr.
>>Title/position: disabled {neck injury}
>>Place of work: CJD WATCH
>>IP address:
>>Browser: Mozilla/5.0 (Windows; U; Win98; en-US; rv:0.9.4)
>>Gecko/20011019 Netscape6/6.2
>>Parent ID: 50/6/888
>> Creutzfeldt-Jakob disease: implications for gastroenterology
>> M G Bramble and J W Ironside
>> Gut 2002; 50: 888-890 (Occasional viewpoint)
>>"CJDs (all human TSEs) and Endoscopy Equipment"

regarding your article;

Creutzfeldt-Jakob disease: implications for gastroenterology

i belong to several support groups for victims and relatives
of CJDs. several years ago i did a survey regarding
endoscopy equipment and how many victims of CJDs have
had any type of this procedure done. to my surprise, many
victims had some kind of endoscopy work done on them.
as this may not be a smoking gun, i think it should
warrant a 'red flag' of sorts, especially since data now
suggests a substantial TSE infectivity in the gut wall
of species infected with TSEs. If such transmissions
occur, the ramifications of spreading TSEs from
endoscopy equipment to the general public would be
horrible, and could potential amplify the transmission
of TSEs through other surgical procedures in that
persons life, due to long incubation and sub-clinical
infection. Science to date, has well established
transmission of sporadic CJDs with medical/surgical

Terry S. Singeltary Sr.

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
TSS 1/27/03 (0)

Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
Date: Tue, 9 Jan 2001 16:49:00 -0800
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy BSE-L

#Docket No. 01-068-1 Risk Reduction Strategies for Potential BSE
Pathways Involving Downer Cattle and Dead Stock of Cattle and Other
Species - TSS 1/21/03 (2)

In Reply to: Docket No. 01-068-1 Risk Reduction Strategies for Potential
BSE Pathways Involving Downer Cattle and Dead Stock of Cattle and Other
Species [TSS SUBMISSION] January 21, 2003

Re: Docket No. 01-068-1 -- (200,000 USA DOWNERS ANNUALLY) TSS 1/21/03

Re: Docket No. 02N-0273 – Substances Prohibited From Use In Animal Food
Or Feed;

# Re: [Docket No. 99-017-2] Blood and Tissue Collection at Slaughtering
Establishments [TSS SUBMISSION]

TSS Submission will be on the 'slides' of the Jan. 19, meeting...tss

My submission to federal gov. on BSE and
the 'lack of' surveillance;$All)?OpenView


CJD Watch/NEWS message board


Abnormal protein deposits were first recognised histologically
in TSEs as amyloid plaques, which were found in the brains
of some but not all models of the disease.

Subject: TSE Conference: DIAGNOSTIC METHODS FOR TSEs (2)

Diagnosis of TSEs by detecting abnormal forms of the host
glycoprotein PrP

Robert A. Somerville
Institute for Animal Health, Neuropathogenesis Unit,
Edinburgh, Scotland, UK

[Submitted to the Electronic Conference on Surveillance for
TSEs of Livestock, by Dr. N. Hunter, 13 May 1997]

PrP is a host-encoded glycoprotein, with Mr 25,000-34,000,
depending on the degree of glycosylation. An abnormal form
of this protein, designated PrPSc, accumulates in organs of
animals infected with a TSE, notably in CNS tissues, but
also (in some but not all cases) in lymphoid and other
peripheral organs. PrPSc is thought to be specifically
associated with TSE infection. There is controversy about its
role: whether it comprises the agent (the protein-only or
"prion" hypothesis), whether it is a component of the agent,
or is solely a pathological product of infection.

Abnormal protein deposits were first recognised histologically
in TSEs as amyloid plaques, which were found in the brains
of some but not all models of the disease. Scrapie
associated fibrils (SAF) were found by negative stain
electron microscopy in extracts from all TSE infected brains
examined. The fibrils are similar but not identical to other
amyloid fibrils. They have been shown to be comprised of the
protein PrP. Examination of brain extracts for SAF has been
demonstrated to be a relatively effective although
cumbersome method for diagnosing TSEs

Biochemical analysis has shown that the normal form of PrP
(PrPC) can be distinguished operationally from the abnormal
form (PrPSc) according to two criteria. PrPC is soluble in
detergents whereas PrPSc sediments. PrPC is susceptible
to protease digestion whereas PrPSc is partially resistant.
These differences in properties may arise because PrP
aggregates to produce the abnormal form.

Many antibody reagents have been developed which
recognise PrP. A major problem in using them diagnostically
is the need to discriminate between PrPC and PrPSc. It is
necessary to apply at least one and preferably both
operational criteria (i.e. the sedimentation and partial
protease resistance of PrPSc) to discriminate between the
two forms of the protein, before determining whether PrPSc is
present in the test sample. Immunoblotting (Western blotting)
of protein after resolution by SDS-PAGE has been preferred
to dot blot or ELISA methodologies since specificity of
detection by the antibody can be checked. However fewer
samples can be processed. Immunohistochemical staining of
tissue sections in our hands is of similar sensitivity to
immunoblotting. It has the advantage of also providing
anotomical detail of deposition, but processing of samples is
more time consuming. It also depends on appropriate
preservation of the tissue.

We have studied the deposition of PrPSc in experimental
models of TSEs. PrPSc can be detected in brain, spleen,
lymph nodes, pancreas and other organs. However the time
at which PrPSc can be detected after infection in each organ
varies according to various biological parameters, including
strain of infecting agent, genotype of the infected animal and
route of infection. In some models PrPSc could be detected
in peripheral organs early after infection (e.g. 30 days after
infection in a model with an incubation period of 170 days).
However in other models PrPSc could not be detected until
much later.

Interestingly in a murine model derived from BSE, PrPSc
could only be detected in the spleen very late in the
incubation period, if at all.

In ruminants PrPSc can be detected in brains of all clinically
affected animals although sometimes the amounts are very
small. PrPSc could also be detected in spleens of some
sheep infected with scrapie but not in others; nor could it be
detected in spleens from BSE infected cattle.

In conclusion, PrPSc detection, in particular post-mortem
testing of brain, may sometimes be a useful adjunct to other
methods of diagnosis, since tissue can readily be analysed
for its presence. Indeed immunoblotting for PrPSc may be
feasible on tissue samples where autolysis has occurred
preventing other methods of diagnosis being applied, since
the partial resistance of PrPSc to proteolysis prevents its
degradation (if some tissue is frozen on autopsy and not all
is fixed in formalin). However our failure to find PrPSc in
spleens from BSE infected cattle, which correlates with the
failure to detect infectivity in this and other peripheral organs,
suggests that diagnosis for all TSEs based on sampling of
peripheral organs may not be valid, since these organs may
not accumulate PrPSc in all experimental or natural models
of TSE.

********************************TEXT ENDS HERE*******

Transmission of prion diseases by blood transfusion

Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David
Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2

Journal of General Virology (2002), 83, 2897–2905. Printed in Great Britain
Published ahead of print (16 July 2000) in JGV Direct as DOI
Transmission of prion diseases by blood transfusion
Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David
Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2
1 Institute for Animal Health, Neuropathogenesis Unit, West Mains Road,
Edinburgh EH9 3JF,
2 Institute for Animal Health, Compton, Newbury, Berkshire RG20 7NN, UK
Author for correspondence: Nora Hunter.
Fax +44 131 668 3872. e-mail
Received 16 May 2002; Accepted 9 July 2002
This article is now available in the November 2002 print issue of JGV
(vol. 83, 2897–2905). The complete issue of the
journal may be seen in electronic form on JGV Online
0001-8580 © 2002 SGM
Attempts to detect infectivity in the blood of humans and animals
affected with transmissible spongiform encephalopathies (TSEs or prion
diseases) have often been inconclusive because of the limitations of
cross-species bioassays and the small volumes of blood that can be
injected by the intracerebral route. A model has been developed for the
experimental study of TSE transmission by blood transfusion using sheep
experimentally infected with bovine spongiform encephalopathy (BSE) or
natural scrapie as donors and susceptible scrapie-free sheep as
recipients. Donors and recipients of the same species greatly
increase the sensitivity of the bioassay and in sheep large volumes of
blood can be injected by the intravenous (i.v.) route. Transmission of
BSE to a single animal using this approach was reported recently. This
study confirms this result with a second transmission of BSE and four
new cases of transmission of natural scrapie. Positive transmissions
occurred with blood taken at pre-clinical and clinical stages of
infection. Initial studies indicate that following such infection by the
i.v. route, deposition of the abnormal prion protein isoform, PrPSc, in
peripheral tissues may be much more limited than is seen following oral
infection. These results confirm the risks of TSE infection via blood
products and suggest that the measures taken to restrict the
use of blood in the UK have been fully justified.

see full text;


G A H Wells


"As implied in the Inset 25 we must _NOT_ assume that transmission
of BSE to other species will invariably present pathology typical
of a scrapie-like disease."


Subject: In Confidence - Perceptions of unconventional slow virus
diseases of animals in the USA - REPORT OF A VISIT TO THE USA -
APRIL-MAY 1989 - G A H Wells
Date: Sat, 29 Jul 2000 18:38:04 -0700
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

######### Bovine Spongiform Encephalopathy #########

In Confidence

Perceptions of unconventional slow virus diseases of animals in the USA

G A H Wells







Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and
compared with natural cases resulted in a more rapidly progressive
clinical disease with repeated episodes of synocopy ending in coma. One
control animal became affected, it is believed through contamination of
inoculam (?saline). Further CWD transmissions were carried out by Dick
Marsh into ferret, mink and squirrel monkey. Transmission occurred in
all of these species with the shortest incubation period in the ferret.


part 2

Oral transmission and early lymphoid tropism of chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus )
Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3,
Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1

Department of Pathology, College of Veterinary Medicine and Biomedical
Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
Department of Veterinary Sciences, University of Wyoming, 1174 Snowy
Range Road, University of Wyoming, Laramie, WY 82070, USA 2
Colorado Division of Wildlife, Wildlife Research Center, 317 West
Prospect Road, Fort Collins, CO 80526-2097, USA3
Colorado State University Veterinary Diagnostic Laboratory, 300 West
Drake Road, Fort Collins, CO 80523-1671, USA4
Animal Disease Research Unit, Agricultural Research Service, US
Department of Agriculture, 337 Bustad Hall, Washington State University,
Pullman, WA 99164-7030, USA5

Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail


Mule deer fawns (Odocoileus hemionus) were inoculated orally with a
brain homogenate prepared from mule deer with naturally occurring
chronic wasting disease (CWD), a prion-induced transmissible spongiform
encephalopathy. Fawns were necropsied and examined for PrP res, the
abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days
post-inoculation (p.i.) using an immunohistochemistry assay modified to
enhance sensitivity. PrPres was detected in alimentary-tract-associated
lymphoid tissues (one or more of the following: retropharyngeal lymph
node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42
days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No
PrPres staining was detected in lymphoid tissue of three control fawns
receiving a control brain inoculum, nor was PrPres detectable in neural
tissue of any fawn. PrPres-specific staining was markedly enhanced by
sequential tissue treatment with formic acid, proteinase K and hydrated
autoclaving prior to immunohistochemical staining with monoclonal
antibody F89/160.1.5. These results indicate that CWD PrP res can be
detected in lymphoid tissues draining the alimentary tract within a few
weeks after oral exposure to infectious prions and may reflect the
initial pathway of CWD infection in deer. The rapid infection of deer
fawns following exposure by the most plausible natural route is
consistent with the efficient horizontal transmission of CWD in nature
and enables accelerated studies of transmission and pathogenesis in the
native species.


Chronic wasting disease (CWD) is a fatal prion disease affecting mule
deer (Odocoileus hemionus), white-tailed deer ( Odocoileus virginianus)
and Rocky Mountain elk (Cervus elaphus nelsoni). This transmissible
spongiform encephalopathy (TSE) has been reported in captive and
free-ranging deer and elk from north-eastern Colorado and south-eastern
Wyoming (Spraker et al. , 1997 ; Williams & Young, 1980 , 1982 , 1992 ).
Although the pathology of CWD is well- described (Williams & Young, 1993
), little is known about CWD transmission. Epidemiological evidence from
captive animals suggests that horizontal transmission may occur at a
level apparently unparalleled in other prion diseases (Miller et al.,
1998 ; Williams & Young, 1992 ). Other non- familial TSEs, such as kuru,
transmissible mink encephalopathy and bovine spongiform encephalopathy
(BSE) appear to be transmitted via ingestion of PrPres-infected tissue
(Cervenakova et al. , 1998 ; Marsh & Bessen, 1993 ; Wells et al.,
1998 ).

Few studies of early preclinical TSE infections have been performed in
natural hosts or using probable natural routes of exposure; however, the
results have been intriguing. BSE has been orally transmitted to cattle
with infectivity detectable in the ileum of calves at 26 weeks
post-inoculation (p.i.) (by mouse bioassay) (Wells et al., 1994 ). In
another study, scrapie agent infectivity was first detected in the
prescapular lymph nodes of goats at 24 weeks post- subcutaneous
inoculation (Hadlow et al., 1974 ). However, mice inoculated
intragastrically with scrapie had detectable infectivity in Peyer's
patches and cervical lymph nodes as early as 1 week p.i. (Kimberlin &
Walker, 1989 ). Thus, it appears that prions can cross the mucous
membranes of the digestive tract to initiate infection in lymphoid
tissue prior to invasion of the central nervous system and development
of clinical disease.

Oral exposure is the most plausible pathway by which the CWD prion may
be introduced to deer in nature. Consequently, we chose this means of
inoculation in an attempt to demonstrate the feasibility of CWD
transmission by this route and to study early lymphoid tissue tropism of
the PrPres in deer. Each deer was repeatedly exposed to a known
infectious CWD inoculum over a 5-day-period because recent results with
scrapie in hamsters indicate repeated oral exposure increases the
incidence of infection (Diringer et al., 1998 ). Because mice are
relatively resistant to CWD (M. Bruce, personal communication)
precluding bioassay, and because several studies have shown that PrPres
strongly correlates with disease (McKinley et al., 1983 ; Race et al. ,
1998 ), we employed an enhanced immunostaining method (formic acid,
proteinase K and hydrated autoclaving) to detect PrPres in situ. Formic
acid and hydrated autoclaving have been previously described for PrPres
epitope exposure prior to immunohistochemistry (IHC) (Miller et al.,
1994 ; van Keulen et al., 1995 ). Using these methods, we demonstrate
PrPres in regional lymph nodes as early as 6 weeks after oral exposure
of deer fawns to the CWD agent.



These results indicate that mule deer fawns develop detectable PrP res
after oral exposure to an inoculum containing CWD prions. In the
earliest post-exposure period, CWD PrPres was traced to the lymphoid
tissues draining the oral and intestinal mucosa (i.e. the
retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and
ileocaecal lymph nodes), which probably received the highest initial
exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum
and spleen in a 10-month-old naturally infected lamb by mouse bioassay.
Eight of nine sheep had infectivity in the retropharyngeal lymph node.
He concluded that the tissue distribution suggested primary infection
via the gastrointestinal tract. The tissue distribution of PrPres in the
early stages of infection in the fawns is strikingly similar to that
seen in naturally infected sheep with scrapie. These findings support
oral exposure as a natural route of CWD infection in deer and support
oral inoculation as a reasonable exposure route for experimental studies
of CWD.


Establishing the transmission of BSE to mink

44. Transmissible mink encephalopathy ("TME") is a rare disease of ranch
reared mink, first recognised in the USA. It had been assumed to be
scrapie in mink and, like BSE, outbreaks have epidemiological
features consistent with a foodborne infection, but it has never been
possible to demonstrate that scrapie infected sheep brain tissue is
pathogenic to mink by oral exposure. In an incident of TME in
Stetsonville, Wisconsin, USA in 1985 Dr Richard Marsh observed that
although the rancher fed 'dead stock', mainly in the form of cattle
carcasses, sheep tissues had never been fed. Studies in the USA of
this incident showed not only that cattle inoculated intracerebrally
with the mink brain developed a fatal spongiform encephalopathy, but
also that the cattle passaged agent remained pathogenic for
mink by either intracerebral inoculation or feeding. In the absence of
reports of a clinical disease homologous to BSE in domestic cattle,
these findings prompted the suggestion that a rare or occult
form of such a disease might exist in the USA. Comparison of the
biological properties of the BSE12
pathogen with those of the Stetsonville isolate was therefore of
considerable interest in relation to hypotheses concerning possible
origins of BSE and potential for subclinical infection in cattle.
45. Proposals to carry out studies with mink in the USA were developed
in collaboration with, the United States Department of Agriculture
("USDA") Agricultural Research Service ("ARS") and the
Department of Veterinary Science, University of Wisconsin, Madison,
Wisconsin, USA. On 30th October, 1990 I attended a CVL/NPU BSE R&D
meeting at the NPU in Edinburgh (YB90/10.30/1.1). I reported that brain
material from BSE affected cows and a control cow (not fed meat and
bonemeal) had been sent coded to Mr Mark Robinson (USDA) for
transmission studies in mink. The studies were conducted from February
1991 under the control and principal funding of USDA-ARS. The results,
discussed at the tenth CVL/NPU BSE R&D meeting on 27th April, 1993
(YB93/4.27/1.1) indicated that mink were indeed susceptible to BSE and,
in contrast to previous attempts to transmit scrapie to the species,
were susceptible by the oral route of inoculation. The collaboration
resulted in the publication of a paper: Robinson, M.M. et al (1994)
Experimental infection of mink with bovine spongiform encephalopathy.
Journal of General Virology 75, 2151-2155 (J/JVIR /75/2151).



We show that (i) BSE can be transmitted from primate to primate by
intravenous route in 25 months, and (ii) an iatrogenic transmission of
vCJD to humans could be readily recognized pathologically, whether it
occurs by the central or peripheral route. Strain typing in mice
demonstrates that the BSE agent adapts to macaques in the same way as it
does to humans and confirms that the BSE agent is responsible for vCJD
not only in the United Kingdom but also in France. The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate. These data will be key in
identifying the origin of human cases of prion disease, including
accidental vCJD transmission, and could provide bases for vCJD risk

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of
sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus)
that were exposed to the infectious agents only by their nonforced
consumption of known infectious tissues. The asymptomatic incubation
period in the one monkey exposed to the virus of kuru was 36 months;
that in the two monkeys exposed to the virus of Creutzfeldt-Jakob
disease was 23 and 27 months, respectively; and that in the two monkeys
exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the
monkeys failed to reveal signs or oral lesions. One additional monkey
similarly exposed to kuru has remained asymptomatic during the 39 months
that it has been under observation.

PMID: 6997404

and probably the most frightening study to date for me...TSS

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by
electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of
Neurological Disorders and Stroke, National Institutes of Health,
Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral
cortex of a middle aged woman with progressive dementia were previously
implicated in the accidental transmission of Creutzfeldt-Jakob disease
(CJD) to two younger patients. The diagnoses of CJD have been confirmed
for all three cases. More than two years after their last use in humans,
after three cleanings and repeated sterilisation in ethanol and
formaldehyde vapour, the electrodes were implanted in the cortex of a
chimpanzee. Eighteen months later the animal became ill with CJD. This
finding serves to re-emphasise the potential danger posed by reuse of
instruments contaminated with the agents of spongiform encephalopathies,
even after scrupulous attempts to clean them.

PMID: 8006664

some comments from other TSE Scientists...TSS;

Subject: Re: hello Dr. Manuelidis...TSS ''BSE as another phenotype of
sporadic CJD''
Date: Fri, 17 Jan 2003 17:38:00 -0500
From: laura manuelidis
Organization: Yale University Medical School
To: "Terry S. Singeltary Sr."
References: <>

Dear Terry,

Many thanks for the references, a rather late recognition of lots of
experimental data that showed PrP was not predictive of disease. Nobody
wanted to listen when I said years ago that vCJD should be able to
transmit to people regardless of host PrP sequence differences. I also
warned the CDC ~5 years ago that people infected by BSE strain might
well show classic rather than vCJD lesions, and that their narrow
sampling for vCJD by young age and PrP band pattern was based on
preconceptions of Prion theory.

Additionally, Dickinson showed many years ago that scrapie could be
subclinical in mice dying of old age, (and we showed transmissions of
CJD that lacked PrP but had many vacuoles in the 1980s, also now
conveniently ignored. Our Science paper further showed a typical
sporadic CJD strain could evolve into one that provoked vCJD plaques. So
really, there is not much new here except the authorship.

I also do not believe the glycosylation story since in 1987 we showed
deglycosylation of PrP in infected brain samples yielded no differences
from unglycosylated parallel samples in terms of incubation time, PrP
band patterns or lesion profiles.


"Terry S. Singeltary Sr." wrote:

> JUST read your (short abstract) of your latest
> article;
> Unique inflammatory RNA profiles of microglia in Creutzfeldt-Jakob
> then i got to wondering if you had read the new data
> from Collinge et al?
> if not, i have posted below, with URL to go to full text.
> i think this _should_ play a major factor to the medical/surgical
> arena, but not to my surprize, nobody seems worried about
> these new findings. well, new findings, but what i have
> thought all along. i hope you find interest in this.
> i would be please to know your thoughts on these findings,
> they are very disturbing to me, but no media or medical
> community has seemed to have picked up on it, and it's
> not because i have not sent them the data;-)
> warmest regards,
> terry
> Subject: re-BSE prions propagate as either variant CJD-like or
sporadic CJD
> Date: Thu, 28 Nov 2002 10:23:43 -0000
> From: "Asante, Emmanuel A"
> To: "''"
> Dear Terry,
> I have been asked by Professor Collinge to respond to your
> request. I am a Senior Scientist in the MRC Prion Unit and the lead
> author on the paper. I have attached a pdf copy of the paper for your
> attention. Thank you for your interest in the paper.
> In respect of your first question, the simple answer is, yes. As you
> will find in the paper, we have managed to associate the alternate
> phenotype to type 2 PrPSc, the commonest sporadic CJD.
> It is too early to be able to claim any further sub-classification in
> respect of Heidenhain variant CJD or Vicky Rimmer's version. It will
> take further studies, which are on-going, to establish if there are
> sub-types to our initial finding which we are now reporting. The main
> point of the paper is that, as well as leading to the expected new
> variant CJD phenotype, BSE transmission to the 129-methionine genotype
> can lead to an alternate phenotype which is indistinguishable from type
> 2 PrPSc.
> I hope reading the paper will enlighten you more on the subject. If I
> can be of any further assistance please to not hesitate to ask. Best
> Emmanuel Asante
> <>
> ____________________________________
> Dr. Emmanuel A Asante
> MRC Prion Unit & Neurogenetics Dept.
> Imperial College School of Medicine (St. Mary's)
> Norfolk Place, LONDON W2 1PG
> Tel: +44 (0)20 7594 3794
> Fax: +44 (0)20 7706 3272



Thank you for your stimulating message. John Collinge's paper, as
usual, is provocative but also confusing as his infected Tg mice show a
tremendous variability in incubation times from inoculum to inoculum
which is puzzling. Unfortunately, John Collinge continues to use
confusing terminology that is different from that proposed by us and
endorsed by most groups around the world. The subtype of sporadic CJD
that Collinge calls type 129 MM 2 is the one that we call 129 MM (MV)
type 1 while your son, Jeffrey had sCJD MM2 which Collinge calls our
SCJD MM2 129 MM type 3. I know it is very confusing, but the bottom
line in that we our diagnosis on Jeffrey is different from the CJD
subtype Collinge reports in his publication.

We are working on further characterization of our sCJD MM2 cortical.
I enclose a reprint of one of our recent publications on this subject.
Also, on behalf of the National Prion Disease Pathology Surveillance
Center, I thank your for your support and interest.

Best regards,
Pierluigi Gambetti, M.D.
how can three very well known and respected TSE Scientist be on
totally seperate pages in regards with sporadic CJDs? how is it
with all these _documented_ animal TSEs in the USA, the fact
they all transmit to primates, with the unknown of BSE/TSE in
USA cattle (except Dr. Marsh did prove some TSE was in USA cattle),
and sporadic CJD and Alzheimer's increasing in the USA, with
Asante/Collinge et al new/old findings, how is it all sporadic CJD
in the USA are just a happen-stance of bad luck or spontaneous
happening of some sort $$$

now, why i have said all along we must keep the lobbyist and
politicians (same thing), far away from scientific matters,
especially human/animal TSEs, with there ties to some many
Industries $

Subject: Re: TSE's blood test
Date: Tue, 27 Jun 2000 21:30:13 -0500
From: "Mary Jo Schmerr"

Dear Mr. Singeltary,

I am very sorry about the terrible death that your mother had to endure.
Stories like this keep me motivated to continue in my research.
We are collaborating with scientists working on the human side and the
results are very promising.
We could move much faster, I believe, if we had more resources and if I
received much stronger support from my administrators. It seems that
many artificial roadblocks are placed in front of me.
I appreciate your interest and support of the research that I do.
Hopefully, we will get to a test soon.

Best wishes,
Mary Jo Schmerr


Subject: Re: hello Dr. Schmerr
Date: Wed, 25 Dec 2002 16:08:03 -0600
From: "Mary Jo Schmerr"

Dear Mr. Singeltary,

Thanks for your comments.

Mary Jo

>>> "Terry S. Singeltary Sr." 12/17/02 11:16 AM >>>
re-if we had more resources and if I received much stronger support
from my administrators. It seems that many artificial roadblocks
are placed in front of me....

bet those roadblocks won't be so difficult now;-)

MILLION AWARDED "her bosses held back her research and
related presentations."
Date: Tue, 17 Dec 2002 10:29:11 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

######## Bovine Spongiform Encephalopathy

Jury awards chemist $1.3 million

A USDA researcher in Ames filed a lawsuit claiming her bosses had
retaliated against her.

Register Staff Writer
A federal jury Monday awarded $1.3 million to an Ames-based U.S.
Department of Agriculture researcher who accused her bosses of
retaliating against her for a gender discrimination complaint that led
to the lawsuit.

Mary Jo Schmerr, a research chemist at the USDA National Animal Disease
Center, had gained notoriety for developing a test for mad cow disease.
Her bosses questioned whether the test worked and wouldn't let her
travel to conferences to report her findings.

Schmerr contended she was treated badly when she complained about the
treatment of a female researcher from the Czech Republic, when she
questioned safety at the lab and when she tried, unsuccessfully, to get
permission to accept speaking invitations.

Judge Ronald Longstaff said the jury in U.S. District Court in Des
Moines unanimously approved $135,000 in back pay and $1.2 million in
damages for retaliation. Schmerr had claimed she lost earning power
because her bosses held back her research and related presentations.

Schmerr, who has a doctoral degree, had her work highlighted in a news
release issued on Oct. 21, 1999, by USDA's Agricultural Research

"Schmerr's accomplishment is an excellent example of how long-term
investment in research can benefit American agriculture," Agriculture
Research Service Administrator Floyd Horn said in the statement.

Schmerr, who began working for the service in 1975, alleged that her
bosses began to treat her badly by late 1999. She filed her gender
discrimination complaint at the end of 2000. The lawsuit followed when
the Equal Employment Opportunity Commission did not act on the

During the six-day trial, the USDA denied wrongdoing.

"The case was without merit," Assistant U.S. Attorney John Beamer said.
"We did things in compliance with the law and in the best interests of
the program involved."

He said government lawyers are reviewing the verdict and had not decided
whether to appeal.

Beamer and Longstaff couldn't say how the verdict ranked in size
compared with similar cases.

Longstaff, who dismissed Schmerr's gender discrimination allegations,
said he hadn't decided whether to reduce the award.

"It's a long ways from being over," Longstaff said.

Schmerr and her lawyer, Charles Gribble, could not be reached for
comment Monday evening. A man who answered the phone at Schmerr's home
said, "They are out celebrating."

''During the six-day trial, the USDA denied wrongdoing.''

famous last words...TSS




a few more comments please;

> As of September 2002, these USDA surveillance efforts, including

> analysis of brain specimens from 36,594 cattle

Dr. Gambetti et al seems overjoyed at the amount of TSE testing
in the USA in cattle? i would like to compare apples and
oranges here;

to Aug. 1, 2001, the USA had tested approx 12,500 cattle
(TOTAL EVER) in the 13 years of surveillance.

SINCE THEN, to date, there are some 35,000+ cattle tested
since the inception of the USA BSE/TSE surveillance program
of 14 years (TOTAL EVER).

NOW, compare to EU Countries that once said they too were BSE
free, but are now finding many cases of BSE. they are testing
millions and finding BSE. we have had the same rendering practices,
the same feeding practices (except not only undocumented cases
of TSEs in cattle went to render), but also many CWD roadkill
deer/elk and Scrapie infected sheep have also been rendered
into feed for many many years in the USA;




and the same kind of flagrant ignoring of the facts by our
Government as those Countries in the EU did in past, still
exist in the USA today. The EU woke up, the USA is still
floundering and refuses to rapid test to find TSE in USA cattle.
also, all one has to do is just compare the numbers of testing
on deer/elk in the USA compared to cattle.

but for now lets just compare testing of cattle in the USA, to
those Countries in the EU;

Dr. Gambetti et al states;

[[the FDA in 1999 instituted a deferral policy to exclude from donating
blood any person who traveled to the United Kingdom for a cumulative
period of 6 months or more between 1980 and 1996.]]

TSS Submission will be on the 'slides' of the Jan. 19, meeting...tss

Variant Creutzfeldt-Jakob Disease Guidance Topic of Feb. 20 TSE Cmte.
[Committee Meeting on February 20, 2003]

Here Dr. Gambetti et al states;

[[Since 1989, to prevent the introduction of BSE into
the United States, the US Department of Agriculture
(USDA) restricted the importation of live cattle and
certain cattle products from the United Kingdom
and other BSE-endemic countries. In 1997, this restriction
was expanded to prohibit importation of cattle and
certain cattle products from all European countries,
and most recently from Japan and Israel.]]

let me put this more in perspective for you just on the 'passenger
air traffic' imports of TSE/BSE products into the USA;

Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of 2002;
Date: January 27, 2003 at 1:46 pm PST


Greetings FDA and public,

if you go to the below site, and search all BSE known countries and
check out their air traffic illegal meat they have confiscated, and
check out the low number checked, compared to actual passenger traffic,
would not take too much for some nut to bring in FMD/TSEs into the USA
as a 'suitcase bomb'.

[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284
air passengers from Israel were sampled for items of agricultural
interest in fiscal year 2001. Seven of these passengers, or 2 percent,
carried a total of 11 kg of meat items that could potentially harbor the
pathogen that causes BSE. None of these passengers from whom meat items
were confiscated reported plans to visit or work on a ranch or farm
during their visit to the U.S.]]

if they were to have questioned the terrorist that bombed the Twin
Towers with jets, if they were to have questioned them at flight school
in the USA, i am sure that they would have said they did not intend to
visit the Twin Towers as a flying bomb either. what am i thinking, they
probably did ask this? stupid me.

[[In 1999 a small amount of non-species specific meat and offal was
imported and a small amount of fetal bovine serum (FBS) was also
imported. FBS is considered to have a relatively low risk of
transmitting BSE.]]

more of the USA infamous 'non-species coding system', wonder how many of
these species are capable of carrying a TSE?


A total of 524,401 passengers arrived on direct flights to the U.S. from
Israel in fiscal year 2000. This number does not include passengers who
arrived in the U.S. from Israel via indirect flights.

Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air
passengers from Israel were sampled for items of agricultural interest
in fiscal year 2001. Seven of these passengers, or 2 percent, carried a
total of 11 kg of meat items that could potentially harbor the pathogen
that causes BSE. None of these passengers from whom meat items were
confiscated reported plans to visit or work on a ranch or farm during
their visit to the U.S.

Source: U.S. Department of Transportation and APHIS-PPQ Agricultural
Quarantine Inspection data base.

What is the level of passenger traffic arriving in the United States
from Japan?

Approximately 6.84 million passengers on 29,826 direct flights from
Japan arrived at US airports in fiscal year 2000. An undetermined number
of passengers from Japan arrived in the US via indirect flights.

Under APHIS-PPQ's agriculture quarantine inspection monitoring, 801 air
passengers from Japan were sampled for items of agricultural interest in
fiscal year 2000. Of these 801 passengers, 10 carried meat (non-pork)
items that could potentially harbor the pathogen(s) that cause BSE; most
passengers carried an average of 1.7 kilograms of meat. None of these
passengers from whom meat items were confiscated reported plans to visit
or work on a ranch or farm during their visit to the US.

Source: US Department of Transportation, and APHIS-PPQ Agricultural
Quarantine Inspection data base

What is the level of passenger traffic arriving in the United States
from the affected country?

A total of 3.3 million passengers arrived in the US on direct flights
from Germany in 1998, although many of these passengers would not have
originated in Germany. As part of APHIS-PPQ's Agriculture Quarantine
Inspection Monitoring, 8,247 air passengers from Germany were inspected
for items of agricultural interest. Of these, 198, or 2.3%, were found
to be carrying a total of 304 kg of items that could potentially present
a risk for BSE. Thirty (30) of the passengers with items reported plans
to visit or work on a farm or ranch while in the US. Reported
destination states of these 30 passengers were CA, CO, DE, FL, LA, MT,
OH, VA, and WY.

Source: US Department of Transportation, and APHIS-PPQ Agricultural
Quarantine Inspection data base

search archives at bottom of page of each BSE Country;

more on non-species coding system and TSEs and potential
'suitcase bombs';

To: Bovine Spongiform Encephalopathy
POLAND/non-species coding system strikes again
References: <>
Content-Type: text/plain; charset=ISO-8859-1; format=flowed
Content-Transfer-Encoding: 8bit
X-Virus-Scanner: Found to be clean

Greetings again List Members,

let me kick a madcow around here a bit.

on the imports from Poland and the infamous USA
'non-species' coding system.

the USDA/APHIS states;

> During the past four years (1998 - 2001), US imports from
> Poland included non-species specific animal products
> used in animal feeds and non-species specific sausage and offal
> products (Table 3). Given US restrictions on ruminant product
> imports, these US imports should not have contained ruminant
> material.

NOW, if you read Polands GBR risk assessment and opinion
on BSE, especially _cross-contamination_, it states;


Poland - Summary of the GBR-Assessment, February 2001


The very high to extremely high external
challenge met a very unstable system and
could have led to contamination of
domestic cattle in Poland from 1987

This internal challenge again met the still
very unstable system and increased over

The continuing very high external
challenge supported this development.

Not OK
since 1997,
but no feed
Reasonably OK
Heat treatment
equivalent to
133°C / 20min / 3
bar standards, but
no evidence
provided on

Not OK.
No SRM-ban,
SRM are
rendered and
included in
cattle feed.

BSE surveillance:

Not sufficient before


Lines for ruminant
and non-ruminant
feed in feed-mills only
separated in time and
no analytical controls
carried out.
Likely present since 1987 and growing.

see full text and ANNEX 1 at;

so in my humble opinion, the statement by the USDA/APHIS
that ''these US imports _should_ not have contained ruminant
materials, is a joke. a sad joke indeed.


full text;

check out imports from BSE Countries and our 'sealed borders'
and that infamous very handy NSCS ''non species coding system''

you get the picture...tss

Dr. Gambetti also states;

> In 1997, this restriction was expanded to prohibit importation of

> cattle and certain cattle products from all European countries,

let us look at this closer;

# Re: BSE .1 GRAM LETHAL NEW STUDY SAYS via W.H.O. Dr Maura Ricketts
[BBC radio 4 FARM news] - TSS 2/02/03 (9)



PRE-1997 imports of MBM to USA from UK alone;

Re: exports from the U.K. of it's MBM to U.S.???
Tue, 8 Feb 2000 14:03:16 +0000
To: (Receipt Notification Requested) (Non Receipt
Notification Requested)


Meat and bonemeal is not specifically classified for overseas trade
purposes. The nearest equivalent is listed as "flours and meals of meat
or offals (including tankage), unfit for human consumption; greaves". UK
exports of this to the US are listed below:

Country Tonnes
1981 12
1984 10
1985 2
1989 20

Data for exports between 1975 and 1979 are not readily available. These
can be obtained (at a charge) from data retailers appointed by HM
Customs and Excise: BTSL (Tel: 01372 463121) or Abacus (01245 252222).

Best wishes
Simon Pearsall
Overseas trade statistics Stats (C&F)C

as discussed
Forwarded message:
Sent: Fri Feb 04 21:47:01 2000
Received: Fri Feb 04 21:45:15 2000

helpline ou=inf o=maff p=maff400 a=attmail c=gb
From: ou=smtp o=maff p=maff400 a=attmail c=gb

Subject: exports from the U.K. of it's MBM to U.S.???

where could I locate data, on the exportation of the U.K.'s meat and
bone meal, to the U.S., between the years 1975 to 1990?
Thank You


Comment- It is known that British Bone Meal has been exported
to the Netherlands and the USA. It is possible that it may
have been used to compound animal feed stuffs, which may have
been used for cattle. The use of ruminant protein has _NOT_
been banned in the USA. The Company have not addressed the issue
of ensuring that source cattle have not been fed ruminant protein,
and that feeding practices are recorded and certified...

other potential TSE tainted imports...TSS

Other US BSE risks: the imported products picture

exactly what i uncovered at the infamous USA BSE Emergency
conference call Jan. 9, 2001;

Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
Date: Tue, 9 Jan 2001 16:49:00 -0800
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

######### Bovine Spongiform Encephalopathy

Greetings List Members,

I was lucky enough to sit in on this BSE conference
call today and even managed to ask a question.
that is when the trouble started.

I submitted a version of my notes to
Sandra Blakeslee of the New York Times,
whom seemed very upset, and rightly

"They tell me it is a closed meeting and
they will release whatever information
they deem fit. Rather infuriating."

and i would have been doing just fine,
until i asked my question. i was surprised
my time to ask a question so quick.

(understand, these are taken from my notes for now.
the spelling of names and such could be off.)

[host Richard Barns]
and now a question from Terry S. Singeltary of
CJD Watch.

yes, thank you,
U.S. cattle, what kind of guarantee can you
give for serum or tissue donor herds?

[no answer, you could hear in the back ground,
mumbling and 'we can't. have him ask the question

[host Richard]
could you repeat the question?

U.S. cattle, what kind of guarantee can you
give for serum or tissue donor herds?

[not sure whom ask this]
what group are you with?

CJD Watch, my Mom died from hvCJD and we are
tracking CJD world-wide.

[not sure who is speaking]
could you please disconnect Mr. Singeltary


full text;

NOW, what about the infamous ruminant-to-ruminant
USA (voluntary) mad cow feed ban, that no one
new about and what about those feed ban warning
letters that the Bush administration has now seemed
to make inaccessible to the public. here are the last
two made available to the public;

Food and Drug Administration
Kansas City District
Southwest Region
11630 West 60 Street
P.O. Box 15905
Lenexa, Kansas 66265-4905
Telephone: (913) 752-2100

July 29, 2002
Ref. KAN 2002-09

Jerry Behimer, Owner
Bakery Trading Company/Ingredient Exchange
401 N. Lindbergh Blvd., Suite 315
St. Louis, MO 63141-7816

Dear Mr. Behimer:

An inspection of your animal feed premix-manufacturing operations,
located at 14521 2nd Ave., Ottumwa, Iowa, was conducted by an
Investigator from our office on June 18 & 19, 2002. During this
inspection, a significant deviation from the requirements set forth in
Title 21, Code of Federal Regulations, Part 589.2000 - Animal Proteins
Prohibited in Ruminant Feed was identified. The regulation is intended
to prevent the establishment and amplification of Bovine Spongiform
Encephalopathy (BSE). Under 21 C.F.R. 589.2000(g)(2), such a deviation
causes products being manufactured and/or distributed by your facility
to be deemed misbranded within the meaning of Section 403(a)(l) of the
Federal Food, Drug, and Cosmetic Act (the Act), and these products may
not be lawfully introduced, or delivered for introduction, into
interstate commerce.

Our investigation found a failure to label your Powdered Cooked Beef,
Product No. 5013, produced during the period of 2/13/02 to approximately
4/18/02, with the cautionary statement "Do Not Feed to Cattle or Other
Ruminants," as required by 21 C.F.R. 589.2000(d). The FDA suggests the
statement be distinguished by different type size or color, or other
means of highlighting the statement so that it is easily noticed by a

The above is not intended to be an all-inclusive list of deviations from
the regulations. As a manufacturer of materials intended for animal feed
use, you are responsible for assuring that your overall operation and
the products you manufacture and distribute are in compliance with the law.

You should take prompt action to correct this violation, and you should
establish a system whereby such violations do not recur. Failure to
promptly correct these violations may result in regulatory action
without further notice, such as seizure and/or injunction.

It is necessary for you to take action on this matter now. We request
you provide our office documentation of corrective action and final
disposition for Lot 030402, approximately 21 tons, which was on hand
during the inspection. Let this office know in writing within fifteen
(15) working days from the date you received this letter what steps you
are taking to correct the problem.

Your reply should be sent to Nadine Nanko Johnson, Compliance Officer,
at the above address.



Charles W. Sedgwick

District Director

Kansas City District

Food and Drug Administration
Dallas District
4040 North Central Expressway
Dallas, Texas 75204-3145

November 7, 2002

Re: 2003-DAL-WL- 05

Mr. Ronald A. Christensen
President & General Manager
Sunnymead Ranch, Inc.
Rt. 1, Box 49
Idalou, TX 79329

Dear Mr. Christensen:

An inspection of your feed mill located at Route 1, Idalou, Texas, was
conducted by Food and Drug Administration (FDA) Investigator Lisa Yoder
on September 9/11, 2002. The inspection found significant deviations
from the requirements set forth in Title 21, Code of Federal
Regulations, Part 589.2000 - Animal Proteins Prohibited in Ruminant Feed
(21 C.F.R. 589.2000). This regulation is intended to prevent the
establishment and amplification of Bovine Spongiform Encephalopathy
(BSE). The deviations cause the feed for sheep (a ruminant animal)
manufactured at your facility to be adulterated within the meaning of
Section 402(a)(4) of the Federal Food, Drug, and Cosmetic Act (the Act).

Animal feeds and feed ingredients containing any mammalian derived
protein (prohibited material), such as bovine meat and bone meal, are
considered potentially injurious to ruminant and public health.
Therefore, the use of such materials in ruminant feed causes the feed to
be adulterated under Section 402(a)(4) of the Act.

Our inspection revealed that your firm manufactures feed for sheep, that
may contain residues of prohibited material. Your sheep feed is mixed in
the same equipment that is used for mixing chicken feed containing
bovine meat and bone meal. Your sheep, which are given this feed, are
ultimately auctioned for sale as food for human consumption.

A list of Inspectional Observations (FDA Form 483) was issued to your
firm and discussed with you at the conclusion of the inspection. The
following violations were observed during the inspection:

Failure to use clean-out procedures or other means adequate to prevent
carryover of protein derived from mammalian tissue to animal protein or
feeds that may be used for ruminants, 21 C.F.R. 589.2000(e)(1)(iii)(A)
and (B). Specifically, raw ingredients, such as soybean meal, limestone,
dehydrated alfalfa meal, and meat and bone meal (bovine) pass through
shared equipment. Your firm does not conduct any form of clean-out
between delivery of meat and bone meal and other feed ingredients like
alfalfa meal, limestone, and soybean meal used in both chicken and sheep

Additionally, there is no clean-out between batches of chicken feed
which contain the meat and bone meal and the sheep feed which is not
intended to contain the meat and bone meal.

Failure to maintain written clean-out procedures to prevent carryover of
protein derived from mammalian tissues to animal protein or feeds that
may be used for ruminants, 21 C.F.R. 589.2000(e)(l)(iv). Specifically,
your firm does not have any written clean-out procedure to prevent
cross-contamination between the sheep feed and the chicken feed nor do
they practice/conduct any clean-out procedures to prevent the
cross-contamination between the chicken feed and sheep feed.

The above is not intended to be an all-inclusive list of deficiencies by
your firm. As a feed manufacturer and ruminant feeder of sheep intended
for slaughter as food, you are responsible for ensuring that your
operations are in full compliance with the law. We have attached a copy
of the BSE regulations to assist you in meeting complete compliance
under the law.

We are in receipt of your letter to this office dated September 13,
2002, in which you state your "intent to not manufacture any sheep feed
for the immediate future." It is unclear to us from your letter whether
you intend to permanently discontinue manufacturing feed for sheep and
whether you are now purchasing ruminant feed from some other source. We
cannot stress enough the seriousness of these deficiencies, and the
importance of your firm being in complete compliance with the BSE
regulations in regard to the mixing of ruminant feed, and feeding and
marketing of ruminant animals. Your firm must adhere to the regulations
to assure a safe ruminant feed and meat supply, or establish complete
and separate receiving, mixing, handling, and feeding conveyances and
equipment for ensuring such safety.

In your letter, you also indicated your unfamiliarity with the concept
of "residuals" of prohibited materials being carried over into ruminant
feeds, and you imply that discussions concerning residuals were not held
with your firm during FDA’s previous inspections. For your information,
FDA conducted inspections of your firm on July 27, 1999, and again on
December 4, 2001 for coverage of the BSE regulations. During both
inspections, investigators questioned your plant manager, John Brown,
about the activities of your firm relative to BSE, including specific
questions addressing whether your firm was a feeder of ruminant animals.
During both inspections, it was also not conveyed to our investigators
that your firm raises sheep (for slaughter as food) at this facility or
manufactures feed for sheep. Therefore, during each of those inspections
no discussion was held regarding "residuals" of prohibited material
being carried over to ruminant feeds. We have attached copies of the
following FDA guidance documents for your review: FDA Guidance for
Industry 68 - Small Entities Compliance Guide -

Protein Blenders, Feed Manufacturers, and Distributors; Guide 69 -
Feeders of Ruminant Animals With On-Farm Feed Mixing Operations; Guide
70 - Feeders of Ruminant Animals Without On-Farm Mixing Operations; and
Guide 76 - Questions and Answers BSE Feed Regulation.

You should take prompt action to correct these deviations, and establish
procedures whereby such violations do not recur. Failure to promptly
correct these deviations may result in regulatory action without further
notice. Such action could include, but is not limited to seizure and/or

You should notify this office in writing within fifteen (15) working
days of receipt of this letter of the specific actions taken to bring
your firm in to compliance with the law. Your response should include an
explanation of each step taken, including any plans to be taken to
correct the violations that would involve the continued use of the
common mixing equipment for both sheep and chicken feeds, and a
timeframe for completion of the planned corrections. As part of your
written response, you should provide information regarding the current
feeding practices for sheep being kept at your facility, and information
about the current or planned marketing of such animals for slaughter.

Your written response should be directed to James R. Lahar, Compliance
Officer, at the above address. If you have any questions, you may
contact Mr. Lahar by telephone at (214) 253-5219.



Reynaldo R. Rodriguez Jr. for Michael A. Chappell

Dallas District Director

Date: Sun, 12 Jan 2003 12:56:44 -0600
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: Re: USA ruminant-to-ruminant feed ban warning letters ???

Subject: Re: USA ruminant-to-ruminant feed ban warning letters ??? (All
the President's yes-men?)
Date: Wed, 29 Jan 2003 15:32:22 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy



CWD/illegal DEER BAITING (coward/BRAINDEAD hunters) and animal protein, they have missed the big picture (ruminant-to-ruminant feeding)


FULL TEXT OF GOA REPORT BELOW (takes a while to load)

2. Mad Cow Disease: Improvements in the Animal Feed Ban and Other
Regulatory Areas Would Strengthen U.S. Prevention Efforts. GAO-02-183,
January 25.

Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks 2002) &
Date: Tue, 10 Dec 2002 08:17:17 -0600
From: "Terry S. Singeltary Sr."

Date: Mon, 9 Dec 2002 21:21:10 -0600
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: "Terry S. Singeltary Sr."
Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly infected flocks
2002) & CWD IN USA

As of September 30, 2002, there were 45 scrapie infected and source
flocks (figure 3). There were 105 newly infected flocks, reported in
FY2002 (figure 4). In addition, 379 scrapie cases were confirmed and
reported by the National Veterinary Services Laboratories (NVSL) in FY
2002 (figure 5) and (figure 6). Five cases of scrapie in goats were
reported in FY 2002 (figure 7), the last of which was confirmed in
August 2002. New infected and source flocks numbers and the number of
these flocks released in FY 2002 are depicted in chart 4. One hundred
(100) flocks which is 67 percent of the scrapie infected and source
flocks present in FY 2002 were released or put on clean-up plans in FY2002.

Slaughter Surveillance

Slaughter Surveillance is currently in Phase II which is intended to
determine the prevalence of scrapie in the US culled sheep population.
Through September 2002 samples from 3,269 sheep were submitted to NVSL
for testing. Samples from a total of 6,795 sheep have been submitted
since the beginning of Phase II on April 1, 2002. Surveillance regions
are depicted in (figure 8).

Scrapie Testing

During FY 2002 11,751 animals have been tested for scrapie which
includes: 2,711 regular necropsy cases, 1,343 third eyelid biopsies for
the test validation project, 546 third eyelid biopsies for the
regulatory program, and approximately 7,151 animals for Phase I & II of
SOSS (chart 5). Laboratory testing has been taking 10 - 11 days on
average with a range of 3 - 34 days.

Ear Tag Orders

During FY 2002 9.9 million plastic and 6.0 million metal tags were
distributed by APHIS (chart 6).


Oct. 2002)

CWD USA surveillance
Subject: Re: CWD AMERICA ???
Date: Fri, 12 Jul 2002 19:10:18 +0200
Organization: O.I.E
To: "Terry S. Singeltary Sr."
References: <> <012901c229b2$ad43bb90$7f00000a@HPKB>

I agree with you Dr Terry. The OIE, namely the International Animal
Health Code Commission is working on making proposals to Member
Countries to change the OIE lists so to avoid some the problems
mentioned in you e-mail. This will take at least two years before
adoption by the International Committee. For BSE, countries asked the
OIE to post information on BSE on the OIE web site.

Personally, I am interested in Chronic Wasting Disease and I follow what
is distributed through ProMed. Delegates of OIE Member Countries can
propose diseases to be added to the list.

Kind regards.

Karim Ben Jebara

----- Original Message -----
From: "Terry S. Singeltary Sr."
Sent: Friday, July 12, 2002 8:43 PM
Subject: Re: CWD AMERICA ???

> hello Dr. Jebara,
> many thanks for your swift and kind reply.
> if i am not mistaken, it was the same email address.
> it was 3 or 4 weeks ago i wrote, as it is, i don't
> save 'sent' emails anymore, unless very important.
> my main concern (besides the fact that a potential TSE
> has been in the USA cattle for some time, but the APHIS
> do not test to find), is that the CWD could very well be
> transmitting to humans, and i just did not see to much
> posted about it on OIE site.
> > Coming back to your question, Chronic Wasting Disease is not an OIE
> > listed disease. Please see OIE disease lists at
> why is this TSE (CWD) not listed and followed as with BSE ?
> Article
> 1. Countries shall make available to other countries, through the
> OIE, whatever information is necessary to minimise the spread of
> important animal diseases and to assist in achieving better worldwide
> control of these diseases.
> The USA CWD is an important animal disease.
> why is it not followed?
> > The decision to add or delete a disease from the OIE lists, come
> > through proposals made by Member Countries and it has to be adopted by
> > the International Committee.
> i _urgently_ suggest a proposal to the OIE to follow this disease very
> closely, and to propose _more_ testing in the USA for TSEs in the USA
> cattle...
> kindest regards,
> terry
> > Dear Sir,
> >
> > This is the first time that I receive your e-mail. To whom have you
> > in the OIE or to which address?
> >
> > Coming back to your question, Chronic Wasting Disease is not an OIE
> > disease. Please see OIE disease lists at
> >
> >
> > Countries should report to the OIE any disease even is not listed
in the
> > OIE's lists in some conditions (example: an exceptional epidemiological
> > event). Please read Chapter 1.1.3 of the International animal health
code to
> > have more information on disease notification and epidemiological
> > information agreed by OIE Member Countries at :
> >
> >
> > The decision to add or delete a disease from the OIE lists, come
> > proposals made by Member Countries and it has to be adopted by the
> > International Committee.
> >
> > Hope that I answered to your question.
> >
> > Best regards.
> >
> > Dr Karim Ben Jebara
> > Head
> > Animal Health Information Department
> > OIE
> >
> >
> >
> > ----- Original Message -----
> > From: "Terry S. Singeltary Sr."
> > To:
> > Sent: Friday, July 12, 2002 6:18 PM
> > Subject: CWD AMERICA ???
> >
> >
> >
> >>AMERICA' ? with no reply ? i am still seeking an answer ?
> >>
> >>many thanks,
> >>and kind regards,
> >>terry

Dr. Gambetti states;

[In addition, the USDA trained veterinarians and veterinary laboratory
workers on the clinical and pathologic manifestations of BSE and
instituted an ongoing BSE surveillance program.16]

again, i respectfully disagree. a few comments from within, and i am
omitting names and dates. you can make your own mind up about the


Hi. I saw the comment regarding the "emergency number" for BSE. I saw
the issue of "complete quotes." The issue is complete surveillance. I am
not trying to badmouth anyone, but between you and me, confidentially,
there is no program to make Vets aware of what to look for. Sure its on
the web, sure there are a few seminars but by and large there is
nothing. Producers don't know what to look for- except what the hyped up
news tells them. There is no program to educate them. As late as Dec
2000 there was no endemity plan to buy out herds that the USDA would
want to slaughter, like the vermont sheep that are still in court. Why
would a producer want to pay a vet to diagnosis a disease that will
result in the demise of his livelyhood? It makes no sense.


very very few know anything abou the workings of BSE. So Dr. Detwilers
comments about calling in are full of it. The only ones that could call
in would be the vets at a diagnostic clinic who get a cow head that is a
rabies suspect. I think she has over glorified the surviellance situation.



[In May, 1990, a plan of drastic surveillance was set up with
the cooperation of more than 250 federal veterinarians formed to
diagnose the foreign animal] >From XXXX email translation...
I had to laugh...then cry a little bit. If anyone thinks there
are 250 federal vets who would even recognize or be able to
diagnose BSE in cattle...someone is very, very naive. I believe
there are probably two, yes two, who would be honest enough,
skilled enough, and brave enough to actually diagnose the
disease. The next piece of information is only for your not reveal this info to anyone!!!


Do you know exactly how many federal veterinarians are
actually focused on BSE issues in USDA...........
ONE, yes ONE,.... there is ONE, yes one assigned to CWD...
a nice person, but totally new to this topic.... One for
Scrapie, nationally, who is only politically astute...
who will admit they know nearly nothing about the science...
one more who is temporarily assigned and does not know much
about the whole thing and wants out... meaning they are
applying to get another job someplace else!!! They are seeking
one more for the Scrapie, but the requirement is that the person
ONLY FOCUS ON SCRAPIE and cannot be interested, seeking,
inquisitive about any other TSE... the person they want won't
compromise and has told the powers that they will only support
the truth and the the science... and will not play political
games... now you know THAT person is not gonna be a player!!!!
Also, the winds of twisted distortion are roaring...
the latest is that the Mink Spongiform Encephalopathy described
by Dr. Richard Marsh is from CWD... that is the answer for all
our last problems... this is the new credo that is being spread to
unfocus attention on cattle/BSE and Scrapie.... All of the above
should not be passed on unless you can find another source. Please
do not reveal this information... but store it someplace with
the date of transmission... the future will afford an opportunity
for truth....///

[sorry:-(the future is here...tss)



As early as 1992-3 there had been long studies conducted on small
pastures containing scrapie infected sheep at the sheep research station
associated with the Neuropathogenesis Unit in Edinburgh, Scotland.
Whether these are documented... I don't know. But personal recounts
both heard and recorded in a daily journal indicate that leaving the
pastures free and replacing the topsoil completely at least 2 feet of
thickness each year for SEVEN years.... and then when very clean (proven
scrapie free) sheep were placed on these small pastures.... the new
sheep also broke with scrapie and passed it to offspring. I am not sure
that TSE contaminated ground could ever be free of the agent!!
A very frightening revelation!!!



Budget: let me know what you find out and the breakdown. There may be
some stuff stuffed into it which is not legit... They may figure some
salaries and such... the real gist of the matter is the shocking
amount of $ that is actually used to "ferret" out the disease and the $
that are used to P.R. the whole affair and give appearance of being
concerned and involved... again it was said years ago and it should
be taken seriously.... BSE will NEVER be found in the US!

As for the BSE conference call... I think you did a great service to
freedom of information and making some people feign integrity... I find
it scary to see that most of the "experts" are employed by the federal
government or are supported on the "teat" of federal funds. A scary
picture! I hope there is a confidential panel organized by the new
government to really investigate this thing.


Talk about being shut the shades of Richard Marsh...he lost
some funding, but seemed to persevere in spite of the attacks. I saw
his names mentioned recently on something through the FDA. Contact:


What a mess! Seems like the time is ripe for everyone to cover their
ass and start screaming... not me, not me, not my job, etc. etc. We
gotta get some big scary folks more involved in this mess.


You need to watch your back........ but keep picking at them....... like
a buzzard to the bone... you just may get to the truth!!! (You probably
have more support than you know. Too many people are afraid to show you
or let anyone else know. I have heard a few things myself... you ask
the questions that everyone else is too afraid to ask.)



Well, you must have bent someone's ear the right way.
Seems there is someone at FDA who is asking some
rather interesting questions and putting some folks on
the hot seat........ I don't know who it is or what
was said, but there are some questions going around
about who in APHIS is talking to FDA....


The most frightening thing I have read all day is the report of
Gambetti's finding of a new strain of sporadic cjd in young
people......... Dear God, what in the name of all that is holy is
that!!! If the US has different strains of scrapie..... why????
than the UK... then would the same mechanisms that make different
strains of scrapie here make different strains of BSE... if the
patterns are different in sheep and mice for scrapie..... could not
the BSE be different in the cattle, in the mink, in the humans.......
I really think the slides or tissues and everything from these young
people with the new strain of sporadic cjd should be put up to be
analyzed by many, many experts in cjd........bse.....scrapie


Okay, you need to know. You don't need to pass it on as nothing will
come of it and there is not a damned thing anyone can do about it.
Don't even hint at it as it will be denied and laughed at..........
USDA is gonna do as little as possible until there is actually a human
case in the USA of the nvcjd........ if you want to move this thing
along and shake the earth.... then we gotta get the victims families to
make sure whoever is doing the autopsy is credible, trustworthy, and a
saint with the courage of Joan of Arc........ I am not kidding!!!! so,
unless we get a human death from EXACTLY the same form with EXACTLY the
same histopath lesions as seen in the UK nvcjd........ forget any
action........ it is ALL gonna be sporadic!!!

And, if there is a case....... there is gonna be every effort to link it
to international travel, international food, etc. etc. etc. etc. etc.
They will go so far as to find out if a sex partner had ever traveled to
the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous
twisted journey to the truth. They have all the cards, all the money,
and are willing to threaten and carry out those threats....and this may
be their biggest downfall.


Thanks as always for your help.
(Recently had a very startling revelation from a rather senior person in
government here.......... knocked me out of my chair........ you must
keep pushing. If I was a power person.... I would be demanding that
there be a least a million bovine tested as soon as possible and
agressively seeking this disease. The big players are coming out of the
woodwork as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb....who's "will"!
"Will be the burden to bare if there is any coverup!"

Take Care!


and what better way is there to keep sporadic CJD in the USA
all sporadic, with no route and source, easy;

[[[It also makes available state-of-the-art free diagnostic services for
physiciandiagnosedor suspected cases of TSE in humans. Physicians are
encouraged to make all efforts to arrange for a brain autopsy in all
such cases and to take advantage of the free diagnostic services
provided by the National Prion Disease Pathology Surveillance
Center to assess the neuropathology of the patients.]]]

physiciandiagnosedor??? real handy word and a cop-out.
if you were to ask the many sporadic CJD family members
in the USA or their attending physicians, many would have
these same type symptoms. i think using this symptom as
a definite tool for distinguishing between vCJD and sCJD
is totally wrong, as with only the young theory of vCJD.

why else do you think there is no CJD Questionnaire's that ask anything
pertaining to route and source, only how the agent was diagnosed?

why else does the USA refuse to rapid TSE test cattle, 1 MILLION

Diagnosis and Reporting of Creutzfeldt-Jakob Disease T. S. Singeltary,
Sr; D. E. Kraemer; R. V. Gibbons, R. C. Holman, E. D. Belay, L. B.

Occasional PrP plaques are seen in cases of Alzheimer's Disease


full text;



Subject: Re: Hello Dr. Manuelidis
Date: Fri, 22 Dec 2000 17:47:09 -0500
From: laura manuelidis
Organization: Yale Medical School
To: "Terry S. Singeltary Sr."
References: <> <>
<> <>
<> <>

Dear Terry,

One of our papers (in Alzheimer's Disease Related Disord. 3:100-109,
1989) in text cites 6 of 46 (13%) of clinical AD as CJD. There may be a
later paper from another lab showing the same higher than expected
incidence but I can't put my hands on it right now. We also have a lot
of papers from 1985 on stating that there are likely many silent
(non-clinical) CJD infections, i.e. much greater than the "tip of the
iceberg" of long standing end-stage cases with clinical symptoms. Hope
this helps.

best wishes for the new year
laura manuelidis

"Terry S. Singeltary Sr." wrote:
> Hello again Dr. Manuelidis,
> could you please help me locate the 2 studies that were
> done on CJD where it showed that up to 13% of the people
> diagnosed as having Alzheimer's actually had CJD.
> trying to find reference...
> thank you,
> Terry S. Singeltary Sr.

Subject: CJD or Alzheimer's, THE PA STUDY...full text
Date: May 7, 2001 at 10:24 am PST

Diagnosis of dementia:
Clinicopathologic correlations

Francois Boller, MD, PhD; Oscar L. Lopez, MD; and John Moossy, MD

Article abstract--Based on 54 demented patients consecutively autopsied
at the University of Pittsburgh, we studied the accuracy of clinicians
in predicting the pathologic diagnosis. Thirty-nine patients (72.2%) had
Alzheimer's disease, while 15 (27.7%) had other CNS diseases (four
multi-infarct dementia; three Creutzfeldt-Jakob disease; two thalamic
and subcortical gliosis; three Parkinson's disease; one progressive
supranuclear palsy; one Huntington's disease; and one unclassified). Two
neurologists independently reviewed the clinical records of each patient
without knowledge of the patient's identity or clinical or pathologic
diagnoses; each clinician reached a clinical diagnosis based on criteria
derived from those of the NINCDS/ADRDA. In 34 (63 %) cases both
clinicians were correct, in nine (17%) one was correct, and in 11 (20%)
neither was correct. These results show that in patients with a clinical
diagnosis of dementia, the etiology cannot be accurately predicted
during life.

NEUROLOGY 1989;39:76-79


full text;

Received April 7, 1988. Accepted for publication
in final form July 20, 1988.

Address correspondence and reprint requests to
Dr. Boller, Department of Neurology, 322 Scaife
Hall, University of Pittsburgh Medical School,
Pittsburgh, PA 15261.

January 1989 NEUROLOGY 39 79


Evaluation of Cerebral Biopsies for the Diagnosis of Dementia
Tue, 8 May 2001 21:09:43 -0700
"Terry S. Singeltary Sr."
Bovine Spongiform Encephalopathy

######### Bovine Spongiform Encephalopathy #########

Evaluation of Cerebral Biopsies for the
Diagnosis of Dementia

Christine M. Hulette, MD; Nancy L. Earl, Md; Barbara J. Crain, MD, Phd

· To identify those patients most likely to benefit from a cerebral
biopsy to diagnose dementia, we reviewed a series of 14 unselected
biopsies performed during a 9-year period (1980 through 1989) at Duke
University Medical Center, Durham, NC. Pathognomonic features allowed a
definitive diagnosis in seven specimens. Nondiagnostic abnormalities but
not diagnostic neuropathologic changes were seen in five additional
specimens, and two specimens were normal. Creutzfeldt-Jakob disease was
the most frequent diagnosis. One patient each was diagnosed as having
Alzheimer's disease, diffuse Lewy body disease, adult-onset Niemann-Pick
disease, and anaplastic astrocytoma. We conclude that a substantial
proportion of patients presenting clinically with atypical dementia are
likely to receive a definitive diagnosis from a cerebral biopsy.
However, in those with coexisting hemiparesis, chorea, athetosis, or
lower motor neuron signs, cerebral biopsies are less likely to be
(Arch Neurol. 1992;49:28-31)

snip...full text;

Cerebral Biopsies in Dementia-- Hulette et al 31

Accepted for publication July 11, 1991.
>From the Department of Pathology, Division of Neuropathology (Drs
Hulette and Crain), the Department of Medicine, Division of Neurology
(Dr Earl), and the Department of Neurobiology
(Dr. Crain), Duke University Medical Center, Durham, NC.

Arch Neurol--Vol 49, January 1992




TSE sub-clinical infection and the medical/surgical arena ?

MRC-43-00 Text only version of this sitePrint this page
Issued: Monday, 28 August 2000

A team of researchers led by Professor John Collinge at the Medical Research Council Prion Unit1 report today in the Proceedings of the National Academy of Sciences, on new evidence for the existence of a ‘sub-clinical’ form of BSE in mice which was unknown until now.

The scientists took a closer look at what is known as the ‘species barrier’ - the main protective factor which limits the ability of prions2 to jump from one species to infect another. They found the mice had a ‘sub-clinical’ form of disease where they carried high levels of infectivity but did not develop the clinical disease during their normal lifespan. The idea that individuals can carry a disease and show no clinical symptoms is not new. It is commonly seen in conventional infectious diseases.

Researchers tried to infect laboratory mice with hamster prions3 called Sc237 and found that the mice showed no apparent signs of disease. However, on closer inspection they found that the mice had high levels of mouse prions in their brains. This was surprising because it has always been assumed that hamster prions could not cause the disease in mice, even when injected directly into the brain.

In addition the researchers showed that this new sub-clinical infection could be easily passed on when injected into healthy mice and hamsters.

The height of the species barrier varies widely between different combinations of animals and also varies with the type or strain of prions. While some barriers are quite small (for instance BSE easily infects mice), other combinations of strain and species show a seemingly impenetrable barrier. Traditionally, the particular barrier studied here was assumed to be robust.

Professor John Collinge said: "These results have a number of important implications. They suggest that we should re-think how we measure species barriers in the laboratory, and that we should not assume that just because one species appears resistant to a strain of prions they have been exposed to, that they do not silently carry the infection. This research raises the possibility, which has been mentioned before, that apparently healthy cattle could harbour, but never show signs of, BSE.

"This is a timely and unexpected result, increasing what we know about prion disease. These new findings have important implications for those researching prion disease, those responsible for preventing infected material getting into the food chain and for those considering how best to safeguard health and reduce the risk that theoretically, prion disease could be contracted through medical and surgical procedures."




Professor Collinge is a consultant neurologist and Director of the newly formed MRC Prion Unit based at The Imperial College School of Medicine at St Mary’s Hospital. He is also a member of the UK Government’s Spongiform Encephalopathy Advisory Committee (SEAC). The MRC prion unit is was set up in 1999, and its work includes molecular genetic studies of human prion disease and transgenic modelling of human prion diseases.

Prions are unique infectious agents that cause fatal brain diseases such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie and BSE (mad cow disease) in animals. In some circumstances prions from one species of animals can infect another and it is clear that BSE has done this to cause the disease variant CJD in the UK and France. It remains unclear how large an epidemic of variant CJD will occur over the years ahead.

The strain of prion used here to infect the mice is the Sc237 strain (also known as 263K) which infects hamsters, and until now was assumed not to infect mice.

This research was funded by the Medical Research Council and Wellcome Trust.

The Medical Research Council (MRC) is a national organisation funded by the UK tax-payer. Its business is medical research aimed at improving human health; everyone stands to benefit from the outputs. The research it supports and the scientists it trains meet the needs of the health services, the pharmaceutical and other health-related industries and the academic world. MRC has funded work which has led to some of the most significant discoveries and achievements in medicine in the UK. About half of the MRC’s expenditure of £345 million is invested in over 50 of its Institutes and Units, where it employs its own research staff. The remaining half goes in the form of grant support and training awards to individuals and teams in universities and medical schools.

The Wellcome Trust is the world's largest medical research charity with a spend of some £600 million in the current financial year 1999/2000. The Wellcome Trust supports more than 5,000 researchers, at 400 locations, in 42 different countries to promote and foster research with the aim of improving human and animal health. As well as funding major initiatives in the public understanding of science, the Wellcome Trust is the country's leading supporter of research into the history of medicine.

©2002 Medical Research Council
Data Protection policy | Contact the MRC

and the agent continues to spread and kill through the
various known proven routes...wake up America!

sporadic CJD is not one strain of CJD that just happens,
it is potentially multiple strains of CJD, all of which
have a route and source, many of which are right here in
the U.S.A.


CJD Watch message board


Moms death from hvCJD


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

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