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From: TSS (
Subject: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT]
Date: March 27, 2003 at 7:15 am PST

In Reply to: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT] posted by TSS on February 22, 2003 at 7:38 am:

Subject: RE--Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Date: Wed, 26 Mar 2003 15:45:32 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

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

Neurology 2003;60:176-181
© 2003 American Academy of Neurology
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

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.

Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600
Clifton Road, Mailstop A-39, Atlanta, GA 30333.

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.

# Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States [FULL TEXT] - TSS 2/22/03 (0)

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

Terry S. Singeltary Neurology Online, 27 Jan 2003 [Full text]

Reply to Singletary

Ryan Maddox, MPH, et al. Neurology Online, 26 Mar 2003 [Full text]

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Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, and Lawrence B.

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

Neurology 2003; 60: 176-181 [Abstract] [Full text]
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RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States

Terry S. Singeltary Neurology Online, 27 Jan 2003 [Full text]

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States 26 March 2003
Next Post-Publication Peer Review Top Terry S. Singeltary,
retired (medically)

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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). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?

Reply to Singletary 26 March 2003
Previous Post-Publication Peer Review Top Ryan Maddox, MPH
CDC, Division of Viral and Rickettsial Diseases,
Ermias D. Belay, MD, Lawrence B. Schonberger, MD

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Mr. Singletary raises several issues related to current Creutzfeldt-
Jakob disease (CJD) surveillance activities. Although CJD is not a
notifiable disease in most states, its unique characteristics,
particularly its invariably fatal outcome within usually a year of
onset, make routine mortality surveillance a useful surrogate for
ongoing CJD surveillance.1 In addition, because CJD is least accurately
diagnosed early in the course of illness, notifiable-disease
surveillance could be less accurate than, if not duplicative of, current
mortality surveillance.1 However, in states where making CJD officially
notifiable would meaningfully facilitate the collection of data to
monitor for variant CJD (vCJD) or other emerging prion diseases, CDC
encourages the designation of CJD as a notifiable disease.1 Moreover,
CDC encourages physicians to report any diagnosed or suspected CJD cases
that may be of special public health importance (e.g., vCJD, iatrogenic
CJD, unusual CJD clusters). As noted in our article, strong evidence is
lacking for a causal link between chronic wasting disease (CWD) of deer
and elk and human disease,2 but only limited data seeking such evidence
exist. Overall, the previously published case-control studies that have
evaluated environmental sources of infection for sporadic CJD have not
consistently identified strong evidence for a common risk factor.3
However, the power of a case-control study to detect a rare cause of CJD
is limited, particularly given the relatively small number of subjects
generally involved and its long incubation period, which may last for
decades. Because only a very small proportion of the US population has
been exposed to CWD, a targeted surveillance and investigation of
unusual cases or case clusters of prion diseases among persons at
increased risk of exposure to CWD is a more efficient approach to
detecting the possible transmission of CWD to humans. In collaboration
with appropriate local and state health departments and the National
Prion Disease Pathology Surveillance Center, CDC is facilitating or
conducting such surveillance and case- investigations, including related
laboratory studies to characterize CJD and CWD prions. Mr. Singletary
also expresses concern over a recent publication by Asante and
colleagues indicating the possibility that some sporadic CJD cases may
be attributable to bovine spongiform encephalopathy (BSE).4 The authors
reported that transgenic mice expressing human prion protein homozygous
for methionine at codon 129, when inoculated with BSE prions, developed
a molecular phenotype consistent with a subtype of sporadic CJD.
Although the authors implied that BSE might cause a sporadic CJD-like
illness among persons homozygous for methionine, the results of their
research with mice do not necessarily directly apply to the transmission
of BSE to humans. If BSE causes a sporadic CJD-like illness in humans,
an increase in sporadic CJD cases would be expected to first occur in
the United Kingdom, where the vast majority of vCJD cases have been
reported. In the United Kingdom during 1997 through 2002, however, the
overall average annual mortality rate for sporadic CJD was not elevated;
it was about 1 case per million population per year. In addition, during
this most recent 6-year period following the first published description
of vCJD in 1996, there was no increasing trend in the reported annual
number of UK sporadic CJD deaths.3, 5 Furthermore, surveillance in the
UK has shown no increase in the proportion of sporadic CJD cases that
are homozygous for methionine (Will RG, National CJD Surveillance Unit,
United Kingdom, 2003; personal communication).


1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and
reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-4.

2. Belay
ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of
emerging forms of Creutzfeldt-Jakob disease in the United States.
Neurology 2003;60:176-181.

3. Belay ED. Transmissible spongiform
encephalopathies in humans. Annu Rev Microbiol 1999;53:283-314.

Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as
either variant CJD-like or sporadic CJD-like prion strains in transgenic
mice expressing human prion protein. EMBO J 2002;21:6358-66.

5. The
UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics.
Available at: Accessed February 18,

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Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Terry S. Singeltary,

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

Email Terry S. Singeltary, et al.:

Greetings again Neurology,

i must respectfully disagree with Ryan Maddox, MPH, et al. looks like
sporadic CJDs are increasing to me, but some will just claim better
surveillance i suppose. odd how the USA can claim better surveillance
when CJD is not reportable in most states and the elderly demented do
not get autopsied in most cases. most doctors and medical personnel i
have dealt with knew absolutely nothing about CJD, much less
human/animal TSEs. so how would one know? also, i must say, this statement;

>>>In addition, because CJD is least accurately diagnosed early in the
course of illness, notifiable-disease surveillance could be less
accurate than, if not duplicative of, current mortality surveillance.<<<

this is very disturbing to me, if most elderly demented (either fast or
slow Alzheimer's and other related demented disease's are not autopsied,
and they die from disease's brought on by dementia in the terminally
ill, and this is what is put on the death certificate, then how would
mortality surveillance pick up these false diagnosis of cause of death?
several studies have shown a substantial number of misdiagnosed cases of

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

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)

personal communication Dr. laura manuelidis YALE;

Subject: Re: Hello Dr. Manuelidis Date: Fri, 22 Dec 2000 17:47:09 -0500
From: laura manuelidis Reply-To: 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

BESIDES the increase in Switzerland, and despite the fact the U.K. did
increase from 1993 to 2002 from 37 to 63 cases of sporadic CJD, look at
France, Germany, Italy and their increase of sporadic CJDs;

The number of people dying from sporadic Creutzfeldt-Jakob disease (CJD)
has risen sharply in Switzerland. The finding is raising fears that 'mad
cow disease' could have spread to humans in another form1.

Nature 420, 450 (2002); doi:10.1038/420450a

Prion data suggest BSE link to sporadic CJD


Predicting the number of cases of Creutzfeldt–Jakob disease (CJD) in
people as a result of transmission of bovine spongiform encephalopathy
(BSE) has just got more difficult.

Whereas it was thought that BSE only caused a new form of the disease
called variant CJD (vCJD), a study in mice from a team led by John
Collinge at University College London suggests that it may also cause a
disease indistinguishable from the commonest form of classical, or
'sporadic', CJD (E. A. Asante et al. EMBO J. 21, 6358–6366; 2002). If
the group's mouse model is relevant to the human disease, the results
also suggest that the true extent of infection may be difficult to
assess because of the large number of asymptomatic carriers.

The latest work uses mice engineered to carry the human gene for a
cell-membrane protein called PrP. Prion diseases occur when PrP is
converted to the abnormal 'prion' form, PrPSc. Collinge has developed a
test, based on a standard western blot for analysing proteins, to study
PrPSc extracted from the brain. This previously showed disease caused by
BSE or vCJD to give a characteristic molecular signature that is
distinct from sporadic CJD (J. Collinge, K. C. L. Sidle, J. Meads, J.
Ironside and A. F. Hill Nature 383, 685–690; 1996).

In their latest experiments, Collinge and his team injected material
from the brains of cows with BSE or people with vCJD directly into the
brains of two strains of mice with a human PrP genotype known as 129MM.
Almost 40% of the British population has this genotype. In one mouse
strain, those that became infected showed the usual BSE pattern in the
western blot. But in the other, Collinge's team tested 11 mice infected
with BSE material using the western blot. Ten of them showed a pattern
consistent with sporadic CJD.

The number of cases of sporadic CJD have been rising in Britain since
the 1970s, and this had been attributed to better monitoring for the
condition. But in July, researchers led by Adriano Aguzzi of the
University Hospital Zurich reported a sudden increase in sporadic CJD
figures in Switzerland in 2001, and suggested that infection with BSE
might be to blame (see Nature 418, 266; 2002). Collinge's new data
provide worrying molecular evidence that BSE might be to blame for the
rise in sporadic CJD.

In previous experiments, Collinge had injected BSE and vCJD material
into mice with another human PrP genotype, known as 129VV. The new data
are thought to be more relevant to the transmission of BSE because all
of the known human victims of vCJD have the 129MM genotype. Another
worrying finding is that the 129MM mice seem to be more susceptible to
developing a subclinical infection, with no obvious symptoms.

If a large pool of the British population is carrying a subclinical BSE
infection, this would have serious consequences for the potential
transmission of the disease, for instance through contaminated surgical
instruments. And although laboratory mice are short-lived, infected
humans might go on to develop the disease later in life.

The UK Department of Health, which has been briefed by Collinge on his
findings, says that it will ask its Spongiform Encephalopathy Advisory
Committee to consider the results closely at its next meeting in
February. Collinge says that an urgent nationwide screening of tonsil
material is needed to get a better estimate of the level of infection in
the population.

Macmillan MagazinesNature © Macmillan Publishers Ltd 2002 Registered No.
785998 England.

Mouse model sheds new light on human prion disease

New findings from Professor John Collinge and his MRC Prion Unit team
have increased our understanding of human prion diseases, for example
Creutzfeldt-Jacob disease (CJD) which destroys the brain. Their work
also raises new research questions about susceptibility to exposure to
the infective agent that causes bovine spongiform encephalopathy (BSE),
a similar brain disease that affects cows. The research is published in
the 28 November 2002 edition of the European Molecular Biology
Organisation journal.

In order to study human susceptibility to BSE and the “species barrier”
between cattle and humans, Professor Collinge’s team has developed mice
which have the human form of prion protein – the infectious protein, or
prion, commonly believed to cause spongiform encephalopathies. The mice
show remarkably similar damage to that seen in people, indicating that
these mice are a good model of the human disease. This means we can see
how prions from BSE and human new variant CJD (vCJD) – which is believed
to be caused by BSE prion infection – act on the mice to mimic human
patterns of the disease.

The experiments confirmed earlier findings that the BSE and vCJD prions
are closely similar. However, the scientists were surprised to see that
while some of the mice developed a disease pattern closely similar to
human vCJD, others produced a pattern like a form of sporadic
(classical) CJD. “Sporadic CJD” simply refers to a form of CJD with no
known cause (such as genetic mutation or accidental exposure to prions).
It has always been possible that it may have multiple causes. These data
suggest that some cases of apparently sporadic CJD in the UK and
elsewhere where people have been exposed to BSE prions may in fact be
caused by BSE.

Professor John Collinge said “We are not saying that all or even most
cases of sporadic CJD are as a result of BSE exposure, but some more
recent cases may be – the incidence of sporadic CJD has shown an upward
trend in the UK over the last decade. While most of this apparent
increase may be because doctors are now more aware of CJD and better at
diagnosing it, serious consideration should be given to a proportion of
this rise being BSE-related. Switzerland, which has had a substantial
BSE epidemic, has noted a sharp recent increase in sporadic CJD.

While few of the mice inoculated with BSE or vCJD prions showed clinical
signs of disease, which might have suggested a good species barrier,
closer examination revealed that many of the mice were in fact infected.
This finding of a “sub-clinical” form of prion disease has been
recognised before by this research group and now also seems relevant to
BSE infection.

Further work in the paper argues that it is the genetic makeup of the
mice which determines whether BSE infection makes them produce the type
4 prion strain (as seen in variant CJD in humans) or the type 2 strain
(as seen in sporadic CJD in humans).

©2003 Medical Research Council

i also respectfully disagree with the assumption that;

>>>Because only a very small proportion of the US population has been
exposed to CWD, a targeted surveillance and investigation of unusual
cases or case clusters of prion diseases among persons at increased risk
of exposure to CWD is a more efficient approach to detecting the
possible transmission of CWD to humans<<<

CWD is not the only TSE in the USA. we have Scrapie in sheep/goats, TME
in mink and the fact USA BSE/TSE surveillance in USA cattle to date has
been lacking, to say the least (re-recent reports from GAO). the cattle
industry has flagrantly violated the voluntary ruminant-to-ruminant
partial feed ban of 8/4/97 up and through 2002. all these TSEs being fed
to different species of animals over the decades in the USA, sub
clinical TSEs, 2nd and 3rd passage of TSEs? we must not forget that;

IN CONFIDENCE Pathology Report:



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.


G A H WELLS 4 January 1991



The summary does tend to give a particular slant to the epidemiology of
BSE which is not totally sound. It is a possibility that the agent of
BSE may be in the cattle population in a number of countries already
apart from the USA and that clinical cases are occurring on rare
occasions. It is also important to off the possibility of the
relationship between BSE and certain low-temperature rendering systems.
For that reason a number of other countries apart from the USA and
France are at risk and, in particular, the Netherlands, Denmark, Germany
and Belgium. For these reasons it would be wise to move to an
international ban on the feeding of ruminant protein to ruminants.

Clearly the summary also needs to refer to the incidence of BSE in the
UK and not solely to Great Britain. No doubt this has been tidied up in
your comments on the summary conclusions. It is a pity that more of the
comments put forward by Dr. Kimberlin have not been included in the
summary since his views on page 13 are succinct and valuable...


Is there a Scrapie-like disease in cattle ?




re-mink rancher 'Wisconsin' dead stock feeder using >95% downer or dead
dairy and a few horses...

As of March 11, CVM had received inspection reports covering inspections
(both initial inspections and re-inspections) of 10,458 different firms.
The majority of these inspections (around 80%) were conducted by State
officials under contract to FDA and the remainder by FDA officials.

Various segments of the feed industry had different levels of compliance
with this feed ban regulation. The results to date are reported here
both by "segment of industry" and "in total".

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.

Food-Processing Security: Voluntary Efforts Are Under Way, but Federal
Agencies Cannot Fully Assess Their Implementation. GAO-03-342

i am not sure about 2003, they have ceased posting those warning
letters. it seems i now have to go through the F.O.I.A. we have been
feeding TSE infected ruminants to ruminants for decades. to only be
concerned with TSE to humans from only CWD in deer and elk in the USA,
would be foolish in my opinion. besides, it would not take too many CWD
infected humans to spread the agent via the many other vectors for
transmission (medical/surgical arena, pharmaceuticals, supplements,)
just to name a few potential routes.

with all due respect, with present data to date, further floundering on
making all CJD/TSEs reportable in the USA, and further refusal to deal
with sporadic CJDs in the USA and around the Globe without CJD
questionnaire asking questions pertaining to route and source of agent,
will only allow the agent to continue to spread, and should be regarded
with great suspicion!

thank you,

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

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