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From: TSS ()
Subject: Family puzzled until it was found Creutzfeldt-Jakob afflicted woman Houston Chronicle (USA HUMAN VERSION MAD COW SPORADIC CJD)
Date: March 2, 2005 at 8:52 am PST

-------- Original Message --------
Subject: Family puzzled until it was found Creutzfeldt-Jakob afflicted woman
Date: Wed, 2 Mar 2005 08:44:03 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


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

March 2, 2005, 12:58AM


Strange actions led to ominous diagnosis


Family puzzled until it was found Creutzfeldt-Jakob afflicted woman


By RICHARD STEWART
Copyright 2005 Houston Chronicle

ANGLETON - When Jones Creek housewife LaNeita Means started doing
strange things like putting a cake mix — still in the box — in her oven
and letting it catch on fire, her family thought she was going crazy.

ADVERTISEMENT

Her final diagnosis was much more sinister. She had Creutzfeldt-Jakob
disease, a rare, always fatal condition that causes spongelike holes in
the brain.

For more than a year and a half, her family watched helplessly as she
lost her mind, became blind, suffered terrible tremors and loss of
muscle control and finally curled up into a small ball, with her fingers
and toes curled inward.

"We couldn't do anything," said her daughter, Sherry Means, 26. "Nobody
could do anything. There's no treatment. I wouldn't wish this on anybody
in the world."

It's a bad disease that earned an even more evil reputation during the
last few years by being associated with Bovine Spongiform
Encephalopathy, better known as mad cow disease.

Although humans can get a variant type of Creutzfeldt-Jakob disease from
eating infected beef, experts think it's unlikely that is how Means
contracted the disease that took her life Jan. 14 at age 57.

"Nobody knows how she got it," Sherry Means said.

Indeed, researchers don't know why or how 85 percent of the sufferers of
the deadly condition contract it. Their cases are classified simply as
"sporadic." Most of the others are inherited and a few cases are
contracted from infected transplanted tissue or eating infected beef.

Only about one person in a million contracts Creutzfeldt-Jakob disease,
said Richard Taylor, who tracks disease reports for the Texas Department
of State Health Services.

An outbreak of mad cow disease in cattle in the United Kingdom in the
1980s and 1990s, and the subsequent detection in 2003 of one case in a
cow in Washington state, garnered a lot of headlines. But Taylor said
there has been only one known case in the United States in which a human
is believed to have gotten the disease from tainted meat.

That case was in 2002, and the victim was a 22-year-old woman in Florida
who grew up in the United Kingdom and probably contracted the disease there.

No cases in humans or cattle have been reported in Texas, Taylor said.

Creutzfeldt-Jakob, named for two German researchers who first diagnosed
it in the 1920s, is unusual in that it is spread not by bacteria or
viruses but by abnormal proteins called prions. Because the prions are
not actually alive, they can't be killed by antibiotics, heat or any
other known method of sterilization, Taylor said.

It isn't spread through close contact with infected individuals, said
Florence Kranitz, head of the Creutzfeldt-Jakob Foundation Inc. in
Akron, Ohio. The brain tissue of infected people does need to be handled
with great care, she said.

It can take years for the symptoms to show up, Kranitz said. Most of the
time, the sufferer is dead within a year of the first sign of symptoms.

LaNeita Means hung on longer than most. So did her family.

"I hate to say this, but I even thought of divorcing her," said her
husband, Doug Means.

She started acting so strange in the fall of 2003 that she no longer
seemed like the woman he had been married to for more than 35 years.

She became fearful and accused her family of hurting her and taking and
hiding things.

"I am so glad I didn't divorce her," he said softly. "Really, I thought
she had a brain tumor."

They went to many local doctors. Nobody could find anything wrong.
Always a big, active woman, she lost more than 90 pounds. Her eyesight
got worse and worse, she started developing Parkinson's-like shakes and
her muscles would stiffen.

When no doctors could find anything physically wrong with her, her
family had her placed in a hospital in Houston to be treated for mental
problems.

But the doctors there said she didn't respond to any of the normal
treatments for dementia.

Finally, a spinal tap indicated she had a prion disease — most likely
the classic form of Creutzfeldt-Jakob.

"I didn't know what that was," Sherry Means said. "The doctor told me it
was like mad cow disease. I was devastated.

"He said, 'I have no good news for you. There is no cure.' "

The diagnosis couldn't be certain. Only a brain biopsy can show the
spongelike holes that are the hallmark of the disease.

The Means family moved her to a nursing home in Lake Jackson to await
the final outcome.

There were some good times when LaNeita Means recognized her family. She
was often comforted by holding stuffed animals, particularly a small
stuffed dog she called "Little Puppy."

She also told her family that she was comforted by someone she called
"Emily." She said Emily was her guardian angel.

"When she talked about Emily, she would look at a particular spot in the
room and said Emily was there," Doug Means said.

Even when she couldn't see, her eyes would stare at a spot for long
periods of time, as if she was watching her angel, her husband said.

By then, she probably felt no pain, Kranitz said. Researchers think
those with the advanced disease are unaware of their condition.

Her brain was sent to the National Prion Disease Pathology Surveillance
Center in Cleveland, Ohio, one of several places in the country where
experts are conducting research into the disease.

Doctors there confirmed the diagnosis, but Sherry Means said a final
report won't be ready until April.

"We have some very good research going on," Kranitz said. "But no,
there's no real treatment now."

Meanwhile, her organization tries to inform and comfort families like
the Meanses.

Kranitz became involved in the foundation after her husband, Aaron
Kranitz, died of Creutzfeldt-Jakob disease.

Her group can be reached at www.cjdfoundation.org
or at P.O. Box 5312, Akron, Ohio 44334.

richard.stewart@chron.com

http://www.chron.com/cs/CDA/ssistory.mpl/metropolitan/3064119


Greetings,

> For more than a year and a half, her family watched helplessly as she
> lost her mind, became blind, suffered terrible tremors and loss of
> muscle control and finally curled up into a small ball, with her
> fingers and toes curled inward.
>


IT is very disturbing to me that the CJD Foundation (backed by CDC)
still refused to acknowledge the latest science about sporadic CJD.
here we have another CJD victim of LONG duration (use to be symptom
of only nvCJD). SCIENCE has been mounting that once and for
all disputes the stupid BSE/nvCJD only theory and the CJD Foundation
never ever mentions this in media statements. why is this?


Medical Sciences
Identification of a second bovine amyloidotic spongiform
encephalopathy: Molecular similarities with sporadic
Creutzfeldt-Jakob disease

Cristina Casalone *{dagger} , Gianluigi Zanusso {dagger} {ddagger} ,
Pierluigi Acutis *, Sergio Ferrari {ddagger} , Lorenzo Capucci § ,
Fabrizio Tagliavini ¶, Salvatore Monaco {ddagger} ||, and Maria Caramelli *

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto
Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via
Bologna, 148, 10195 Turin, Italy; {ddagger} Department of Neurological
and Visual Science, Section of Clinical Neurology, Policlinico G.B.
Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; § Istituto
Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via
Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico
"Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco,
CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
mammalian neurodegenerative disorders characterized by a
posttranslational conversion and brain accumulation of an insoluble,
protease-resistant isoform (PrPSc) of the host-encoded cellular prion
protein (PrPC). Human and animal TSE agents exist as different
phenotypes that can be biochemically differentiated on the basis of the
molecular mass of the protease-resistant PrPSc fragments and the degree
of glycosylation. Epidemiological, molecular, and transmission studies
strongly suggest that the single strain of agent responsible for bovine
spongiform encephalopathy (BSE) has infected humans, causing variant
Creutzfeldt-Jakob disease. The unprecedented biological properties of
the BSE agent, which circumvents the so-called "species barrier" between
cattle and humans and adapts to different mammalian species, has raised
considerable concern for human health. To date, it is unknown whether
more than one strain might be responsible for cattle TSE or whether the
BSE agent undergoes phenotypic variation after natural transmission.
Here we provide evidence of a second cattle TSE. The disorder was
pathologically characterized by the presence of PrP-immunopositive
amyloid plaques, as opposed to the lack of amyloid deposition in typical
BSE cases, and by a different pattern of regional distribution and
topology of brain PrPSc accumulation. In addition, Western blot analysis
showed a PrPSc type with predominance of the low molecular mass
glycoform and a protease-resistant fragment of lower molecular mass than
BSE-PrPSc. Strikingly, the molecular signature of this previously
undescribed bovine PrPSc was similar to that encountered in a distinct
subtype of sporadic Creutzfeldt-Jakob disease.

------------------------------------------------------------------------

{dagger} C.C. and G.Z. contributed equally to this work.

||To whom correspondence should be addressed.

E-mail: salvatore.monaco@mail.univr.it
.
www.pnas.org/cgi/doi/10.1073/pnas.0305777101

http://www.pnas.org/cgi/content/abstract/0305777101v1


Characterization of two distinct prion strains derived from bovine
spongiform encephalopathy transmissions to inbred mice

Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais, Susan
Joiner, Jennifer Buckell, Sebastian Brandner, Jonathan D. F. Wadsworth
and John Collinge

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College, London WC1N 3BG, UK

Correspondence
John Collinge
j.collinge@prion.ucl.ac.uk

Distinct prion strains can be distinguished by differences in incubation
period, neuropathology and biochemical properties of disease-associated
prion protein (PrPSc) in inoculated mice. Reliable comparisons of mouse
prion strain properties can only be achieved after passage in
genetically identical mice, as host prion protein sequence and genetic
background are known to modulate prion disease phenotypes. While
multiple prion strains have been identified in sheep scrapie and
Creutzfeldt–Jakob disease, bovine spongiform encephalopathy (BSE) is
thought to be caused by a single prion strain. Primary passage of BSE
prions to different lines of inbred mice resulted in the propagation of
two distinct PrPSc types, suggesting that two prion strains may have
been isolated. To investigate this further, these isolates were
subpassaged in a single line of inbred mice (SJL) and it was confirmed
that two distinct prion strains had been identified. MRC1 was
characterized by a short incubation time (110±3 days), a
mono-glycosylated-dominant PrPSc type and a generalized diffuse pattern
of PrP-immunoreactive deposits, while MRC2 displayed a much longer
incubation time (155±1 days), a di-glycosylated-dominant PrPSc type and
a distinct pattern of PrP-immunoreactive deposits and neuronal loss.
These data indicate a crucial involvement of the host genome in
modulating prion strain selection and propagation in mice. It is
possible that multiple disease phenotypes may also be possible in BSE
prion infection in humans and other animals.

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471


Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

Corinne Ida Lasmézas*,dagger
, Jean-Guy
Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François
Lamoury*, Nicolas KoppDagger , Jean-Jacques Hauw§, James Ironside¶,
Moira Bruce|| , Dominique Dormont*, and Jean-Philippe Deslys*

* Commissariat à l'Energie Atomique, Service de Neurovirologie,
Direction des Sciences du Vivant/Département de Recherche Medicale,
Centre de Recherches du Service de Santé des Armées 60-68, Avenue du
Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Dagger
Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon,
France; § Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83,
Boulevard de l'Hôpital, 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease
Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4
2XU, United Kingdom; and || Institute for Animal Health,
Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche
Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
(received for review October 16, 2000)


Abstract

Top
Abstract
Introduction

Materials and Methods

Results
Discussion
Conclusions
References

There is substantial scientific evidence to support the notion that
bovine spongiform encephalopathy (BSE) has contaminated human beings,
causing variant Creutzfeldt-Jakob disease (vCJD). This disease has
raised concerns about the possibility of an iatrogenic secondary
transmission to humans, because the biological properties of the
primate-adapted BSE agent are unknown. 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 assessment.

snip...

Conclusions
Top
Abstract
Introduction

Materials and Methods

Results
Discussion
Conclusions
References

From BSE and vCJD transmissions in nonhuman primates, a number of
conclusions can be drawn that are of major importance for human health:
(i) human-adapted BSE appears to be a variant of the BSE agent that is
more virulent for humans than cattle BSE and is efficiently transmitted
by the peripheral route; (ii) the detection of vCJD in unusually young
patients is probably not because of a lack of diagnosis of cases in
older patients, thus raising the question of the source of human
contamination with BSE early in life; and (iii) iatrogenic transmissions
from patients with vCJD would be readily recognized by using the same
diagnostic criteria as those applied to vCJD [clinical and pathological
criteria (27 )
comprising neuronal loss and gliosis in the thalamus correlated with
high MRI signal (28
, 29
)], whether such
contaminations had occurred by the central or i.v. route. Primary and
iatrogenic cases of vCJD could be distinguished on the basis of the
patient's clinical history.

The risk assessment of biological products of human origin, notably
those derived from blood, has been deeply modified by the appearance of
vCJD. We confirm that the BSE agent has contaminated humans not only in
the U.K. and the Republic of Ireland but also in France, and we show
that its pathogenic properties for primates are being enhanced by a
primary passage in humans. Considering the flow of potentially
contaminated bovine-derived products between 1980 and 1996, it is
obvious that further vCJD cases may occur outside the U.K. Thus, and in
the light of the present study, it is necessary to sustain worldwide CJD
surveillance regardless of national BSE incidence and to take all
precautionary measures to avoid iatrogenic transmissions from vCJD.

snip...

full text;

http://www.pnas.org/cgi/content/full/041490898v1


Ref: MRC/62/04

Under strict embargo until 19.00 British Time Thursday 11 November 2004

GENETIC MAKE-UP MAY DETERMINE WHAT TYPE OF CJD OCCURS WHEN HUMANS ARE

INFECTED WITH BSE

New research published today (19.00 hours Thursday 11th November) by a

team from the Medical Research Council (MRC) Prion Unit offers an

explanation about why only people with a particular genetic make-up have

so far developed vCJD. It also provides evidence that other types of

BSE-derived prion infection with a different pattern of symptoms might

occur in humans. The findings are published in the journal Science.

Variant CJD (vCJD) is the human disease thought to be caused by eating

food contaminated with the infectious agent, known as a prion,

responsible for the epidemic of BSE or mad cow disease in cattle. So

far, everyone known to have developed vCJD has been of a particular

genetic type  known as MM. Until now it has been a mystery why everyone

that has developed vCJD is of the MM type and one possibility is that

they are simply the first to develop the disease when infected with BSE,

and that people with the other genetic types1 (known as VV and MV)

infected with BSE prions will also develop vCJD, but some years later.

In a series of experiments spanning more than ten years, the MRC team

has been studying mice genetically modified so that they make human

prion proteins  which are used to model human susceptibility to BSE.

The team has now shown that mice with the human VV genetic type do

become infected when given BSE or vCJD prions, but manifest a different

form of the disease which looks quite different to vCJD and has a novel

prion strain type.

Remarkably, when these novel prions were used to infect mice of the MM

genetic type, the mice either developed a disease very like vCJD, or

else a pattern of disease that looks like so-called sporadic CJD  the

classical form of CJD. This form has been known about for many years,

is seen all over the world and has not hitherto been associated with

BSE. However, the new strain identified in the mice, being called type

5, has not been seen yet in people and we do not know what pattern of

disease it would cause. It could look like one of the forms of classical

or sporadic CJD or perhaps be yet another different variant form.

The work from the MRC team suggests that type 4 prions, the type

associated with vCJD, can only propagate themselves in people that make

the M form of the protein. It seems the V form of the protein just

cannot adopt the particular molecular shape that characterises type 4.

The studies in mice also suggest that if these prions were to pass from

person to person (for example by blood transfusion) then, depending on

the genetic type of the person becoming infected, at least three

different patterns of disease might result: type 2 (which is seen in

sporadic CJD); type 4 (which causes vCJD) or type 5 (which may cause a

new pattern of disease).

Professor John Collinge, Director of the MRC Prion Unit, which is based

at University College London, said: These mouse studies give us vital

clues about the behaviour of prions and how they appear to modify and

adapt depending on the genetic makeup of the individual they are infecting.

We always have to be cautious about making direct comparison to the

human condition, but our work strongly suggests that we can not assume

only those with one genetic profile are vulnerable to BSE infection.

At this stage it is not possible to say how this should alter estimates

of those likely to become ill, but our findings do suggest we should be

taking steps to draw up a more sophisticated system of categorizing the

disease so that we dont mistake BSE related infection for a version of

sporadic CJD.

ENDS

For more information call the MRC press office on 020 7 637 6011

Notes to Editors

1The human prion protein comes in two common forms, known as M and V.

Because everyone has two copies of this gene, there are three possible

genetic types: MM, MV and VV.

Paper - Human Prion protein v129 prevent expression of vCJD phenotype 

Science On line 11.11.04

Prions are rogue forms of one of the bodys own proteins  known as the

prion protein  which are misshapen. There are several different rogue

or misshapen forms that can infect humans, and these different types of

prions are known as strains. This is analogous to different strains of

other germs such flu virus causing influenza or strains of salmonella

causing different forms of food poisoning for example.

The strain of prion causing vCJD is known as type 4, types 1-3 cause the

different forms of sporadic or classical CJD. Each strain causes a

different pattern or type of disease. It is known that prion strains can

change or mutate when they pass between different animals.

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 MRCs expenditure of £430 million is invested in its 40

Institutes, Units and Centres. The remaining half goes in the form of

grant support and training awards to individuals and teams in

universities and medical schools. Web site at: http://www.mrc.ac.uk

.

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:

flounder@wt.net

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?

http://www.neurology.org/cgi/eletters/60/2/176#535

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

I SAY AGAIN, until the CJD Foundation acknowledges the
latest science, instead of living in the past, we will
never have answers. until our loved ones are autopsied
somewhere else, we HAVE to take there word on all the
sporadic CJD diagnosis. they criteria for diagnosing
sporadic CJD vs sporadic CJD has changed with the moon
and tide ever since this nightmare happened and it's
all changed to keep sporadic just that, sporadic, without
route and source....


Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW
BSE SAFEGUARDS (comment submission)

https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument

Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL
IMPORTS FROM CANADA


https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenDocument&AutoFramed


Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html

PART 2


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html

PDF]Freas, William TSS SUBMISSION

File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

Asante/Collinge et al, 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;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm


[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...


www.aphis.usda.gov/lpa/pubs/pubs/PL107-9_Appen.pdf

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
2002; [TSS SUBMISSION ON POTENTIAL FOR BSE/TSE & FMD 'SUITCASE BOMBS'] -
TSS 1/27/03 (0)

Docket Management

Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305
Comment Number: EC-254 [TSS SUBMISSION]

http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm


Dockets Entered On October 2, 2003 Table of Contents, Docket #,
Title, 1978N-0301,

OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr.
Vol #: 1, ...

www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm


Daily Dockets Entered on 02/05/03

DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2.
... Vol#: 1.

03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...


www.fda.gov/ohrms/dockets/dailys/03/Feb03/020503/020503.htm


Docket Management

Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater

Comment Number: EC -1

Accepted - Volume 1


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be11.html


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfe.html


http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004bdfc.html


Daily Dockets - 04/10/03

... 00D-1662 Use of Xenotransplantation Products in Humans.
EMC 98 Terry S. Singeltary Sr. Vol#: 3. 01F ...
www.fda.gov/ohrms/dockets/dailys/03/Apr03/041003/041003.htm - 05-20-2003
- Cached


2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed


EMC 1
Terry S. Singeltary Sr.
Vol #:
1

http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm


2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed


EMC 7
Terry S. Singeltary Sr.
Vol #:
1

2003D-0186
Guidance for Industry: Use of Material From Deer and Elk In Animal Feed


EMC 7
Terry S. Singeltary Sr.
Vol #:
1


http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm

01N-0423 Substances Prohibited from use in animal food/Feed Ruminant

APE 5 National Renderers Association, Inc. Vol#: 2

APE 6 Animal Protein Producers Industry Vol#: 2

APE 7 Darling International Inc. Vol#: 2

EMC 1 Terry S. Singeltary Sr. Vol#: 3

http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm

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:


flounder@wt.net

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?


http://www.neurology.org/cgi/eletters/60/2/176#535

LANCET INFECTIOUS DISEASE JOURNAL


Volume 3, Number 8 01 August 2003


Newsdesk


Tracking spongiform encephalopathies in North America


Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost

my mom to hvCJD (Heidenhain variant CJD) and have been searching for

answers ever since. What I have found is that we have not been told the

truth. CWD in deer and elk is a small portion of a much bigger problem.


49-year-old Singeltary is one of a number of people who have remained

largely unsatisfied after being told that a close relative died from a

rapidly progressive dementia compatible with spontaneous

Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

encephalopathy (BSE), Americans might get a similar disorder from

chronic wasting disease (CWD)the relative of mad cow disease seen among

deer and elk in the USA. Although his feverish search did not lead him

to the smoking gun linking CWD to a similar disease in North American

people, it did uncover a largely disappointing situation.


Singeltary was greatly demoralised at the few attempts to monitor the

occurrence of CJD and CWD in the USA. Only a few states have made CJD

reportable. Human and animal TSEs should be reportable nationwide and

internationally, he complained in a letter to the Journal of the

American Medical Association (JAMA 2003; 285: 733). I hope that the CDC

does not continue to expect us to still believe that the 85% plus of all

CJD cases which are sporadic are all spontaneous, without route or source.


Until recently, CWD was thought to be confined to the wild in a small

region in Colorado. But since early 2002, it has been reported in other

areas, including Wisconsin, South Dakota, and the Canadian province of

Saskatchewan. Indeed, the occurrence of CWD in states that were not

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.


To date, experimental studies have proven that the CWD agent can be

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

lymphoid tissue before invasion of the central nervous system. Yet the

plausibility of CWD spreading to people has remained elusive.


Part of the problem seems to stem from the US surveillance system. CJD

is only reported in those areas known to be endemic foci of CWD.

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.


Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

prohibiting material from CWD-positive animals from being used as an

ingredient in feed for any animal species, epidemiological control and

research in the USA has been quite different from the situation in the

UK and Europe regarding BSE.


Getting data on TSEs in the USA from the government is like pulling

teeth, Singeltary argues. You get it when they want you to have it,

and only what they want you to have.


Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunterstwo of whom were friendswho died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.


CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very

limited.


http://infection.thelancet.com/journal/journal.isa


he complained in a letter to the Journal of the American Medical

Association (JAMA 2003; 285: 733). I hope that the CDC does not

continue to expect us to still believe that the 85% plus of all CJD

cases which are sporadic are all spontaneous, without route or source.<<<

actually, that quote was from a more recent article in the Journal of

Neurology (see below), not the JAMA article...

Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama

BRITISH MEDICAL JOURNAL

SOMETHING TO CHEW ON

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

THE PATHOLOGICAL PROTEIN

BY Philip Yam

Yam Philip Yam News Editor Scientific American www.sciam.com
http://www.thepathologicalprotein.com/

IN light of Asante/Collinge et al findings 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;

-------- 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:
"'flounder@wt.net'"

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:

e.asante@ic.ac.uk (until 9/12/02)

New e-mail: e.asante@prion.ucl.ac.uk (active from now)

____________________________________

snip...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally...TSS

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, TEXAS USA 77518


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TSS

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