From: TSS ()
Subject: Re: Local Man Diagnosed with Chronic Wasting Disease ?
Date: July 14, 2006 at 2:17 pm PST
In Reply to: Local Man Diagnosed with Chronic Wasting Disease ? posted by TSS on July 13, 2006 at 11:38 am:
##################### Bovine Spongiform Encephalopathy #####################
nice to see someone else post here. i have heard scientist state that to
actually cure a victim of TSE, once clinical, the damage is so severe to the
brain, that stem cell might be the only way to repair the brain that has
been damaged, this side of a brain transplant. also, something about prion
and stem cell regeneration ;
The curative properties of stem cells may rely on prions, a new study
suggests, the type of protein made infamous by mad cow disease.
Prions are a special class of protein that can change the shape and function
of other proteins around them. While these are found throughout any mammal’s
body, the understanding of their biological role is limited. What is known
is that prions that become misshapen, through some unknown process, can
result in BSE (bovine spongiform encephalopathy) – mad cow disease – and its
equivalents in other animals.
Researchers at the Whitehead Institute in Cambridge, Massachusetts, US, have
now found that adult stem cells in bone marrow gradually lose their ability
to regenerate without their normal complement of membrane-bound prions. Stem
cells are primitive cells which have the potential to divide endlessly, and
the ability to differentiate into any cell type in the body – offering hope
for future therapies.
Andrew Steele, Cheng Cheng Zhang and colleagues used radiation to deplete
the bone marrow of mice genetically engineered to not produce the prion
proteins. The animals’ marrow regenerated quickly at first, but eventually
slowed to a stop. The marrow also lost its regenerative abilities when
transplanted into normal mice.
“For years we’ve wondered why evolution has preserved this protein, what
positive role it could possibly be playing,” says Susan Lindquist, one of
the team. “With these findings we have our first answer.”
The question of how prions sustain stem cell activity remains unanswered,
but the finding is a first step to understanding the destructive streak of
misshapen prion proteins, Steele says. Similar tests on neural and lung stem
cells are underway.
Journal reference: Proceedings of the National Academy of Sciences (DOI:
Replacement of brain cells that have been damaged and removed
Neuronal cell allografting
The progression of research into neural transplantation of tissue and cells
has gone ahead rapidly over the last ten years. Initially this was
following the implantation of cells into the brain as a treatment for
Parkinsons disease but quickly spread out into a much wider propensity. It
is now clear that graft cells can be found and that they have the propensity
for replacement of neural tissue inside the brain. Human treatment is now
starting to follow major animal model research and has been well reviewed.
Cells to transplant into patients with vCJD would need to be able to
replace the apoptotic neurones resulting from the pathological process. For
this to be investigated it should be understood what cell sources are
availailable currently and how these may become of use. It should be noted
that the damaged cells in different parts of the infected brain will be of
separate cell strains and hence any implanted cells would need to be able to
alter their phenotypical nature to permit them to replace the cell that has
been lost. Also, instead of replacing a specific part of the brain, in
prion disease individual cells are lost in widespread sections, and would be
separated by apparently normal and active tissue. Neuronal graft cells must
also be able to make dendritic and axonal connections with other parts of
the brain in a useful manner to permit physiological usefulness. At the
moment the findings that, although scientifically we can created pluripotent
stem cells from adult tissue, it is not clear why the brain does not
organise this to replace apoptotic tissue itself and how we could alter
factors to permit this. The brain, being an immunologically privileged site
and unlikely to reject allografts (57) means that potential banks of
neurological cell types can potentially be made and used in many patients.
Surprising as it may seem this may actually be possible using the cell types
that are being organised and each should be discussed.
Embryo-derived stem cells. In mouse models these can be treated with
retinoic acid to restrict their differentiation into a neural fate. They
then can be implanted into areas of neural damage and the outcome of the
cells followed. The spectrum of cell types that appeared was apparently
wide but a tendency to produce astrocytes and oligodendrocytes rather than
neural cells has been disappointing. Neural stem cells from an adult rat
subventricular zone or cerebral cortex can be transplanted into a contused
spinal cord but again the outcome is mainly of undifferentiated cells or
Foetal central nervous system cells and tissue. This contains not just
cells that are in the process of development and differentiation but
matrices of tissue that are organised to permit neuronal and axonal
development. This type of tissue has been tested on spinal cord injury in
animals and to replace motorneurone pools lost in spinal damage. The
implantation of foetal tissue in Huntingdon’s disease and Parkinson’s
disease in humans has shown varying results but, although no certain blinded
trial has taken place major improvements have been seen.
Modified cultured stem cells. Genetically modified, post-culture
modified, and chemically modified cells are being investigated. The aim of
this is to prevent the graft cells differentiating into the cell type that
is not required and permitting it to produce enzymes (or not PrPc) to avoid
the disease problem that existed originally. The control of the phenotype
of the cell type has been a major problem and, because of its importance in
spinal column injury treatment, this direction of research has been
Bone marrow stromal cells. These will develop to a wide variety of cell
types and, because they would be available from the patient, expanded and
potentially modified in culture, and delivered into injured the brain
through intravenous or intraarterial inoculation. They appear to migrate to
damaged brain tissue in mice and to limit post-arterial occlusion
The poor survival rate of implanted cells is currently uncertain in nature
reports of 10% to 50%. The oxidative stress that takes place and the
apoptotic or potential destruction by local microglia must be considered.
The only current report of cell culture into mice late in the incubation
period of scrapie has shown. In this cells from neuronal foetal tissue of
mice that were not carrying the PrPc gene or immortalised murine stem cells
were inoculated directly into the brain of the scrapie-infected mice. When
compared with the inoculation of control of growth medium inoculation it
could the seen that fewer hippocampal cells were lost by the mice and that
the inoculated cells migrated to sites where cells were being lost(59).
To some degree there are problems involving other treatments that may take
place at the same time as cell inoculation. For instance,
microglia-derived growth factors may be important in causing the migration
of cells to damaged tissue and the anti-microglial treatment required to
prevent pathological progression may be a problem (e.g. pentosan
polysulphate, is a powerful inhibitor of cytokines).
i am only guessing here, but i think that is what this person would be
speaking of, a hypithetical, i dont recall a 'stem cell' transplant on any
human to date for a TSE. i suppose even 'stem cell' could be a potential
carrier of a TSE also, if contracted from an infected donor. it would be
nice to know more about this case, but this is how it has been all along, a
'trickle down affect' with human and animal TSE information.
there was a recent cjd conference, there yearly get-together. from what i
understand, it was mostly all nvCJD this, nvCJD that, not much on the USA
problem with human TSE. they need to get off the nvCJD bandwagon, and start
worrying about human TSE in the USA i.e. of the sporadic strains i.e.
For Immediate Release Contact:
July 14, 2006
CREUTZFELDT-JAKOB DISEASE FOUNDATION HOLDS 4th ANNUAL CONFERENCE
(City,State)Resident (Name) Attends Conference and Meets with Congressman
Akron, Ohio - The Creutzfeldt-Jakob Disease (CJD) Foundation held its 4th
annual family conference at the Washington Court Hotel in Washington, D.C,
July 7-10, 2006. The foundation brings together families who have been
affected by this rare, always fatal, neurological disease. Attendees heard
from leading experts and researchers including Dr. Pierluigi Gambetti from
the National Prion Disease Pathology Surveillance Center at Case Western in
Cleveland, Ohio; Dr. Bernardino Ghetti of Indiana University and
President-elect of the International Society of Neuropathology; Dr. Robert
Will prominent clinician in the United Kingdom; Dr. David Kocisko, Rocky
Mountain Laboratories; Dr.Neil Cashman of Vancover, British Columbia; Dr.
Richard Knight, Director of the National CJD Surveillance Unit, UK; Dr.
James Sejvar of the Centers for Disease Control and Prevention, Atlanta, GA;
the CJD Support Networks from Japan and Australia and other experts from the
CDC and NIH, as well as, members of the Foundation’s Board of Directors.
(INSERT NAME OF ATTENDEE and town) attended the conference and on Monday,
July 10 joined 150 representing families from the United States, UK, Japan
and Australia in Capitol Hill visits with members of Congress to bring
awareness to CJD, discuss research and surveillance of the brain disease and
food safety. (Attendee) met with (Congressman or Senator) or members of
Creutzfeldt - Jakob disease is a prion disease which is a group of rare,
invariably fatal brain disorders which occur both in humans and certain
animals. They first came to public attention in the mid 1980s in the form
of the BSE epidemic in the United Kingdom BSE (bovine spongiform
encephalopathy) also known as “mad cow disease” is a prion disease in
cattle. Tissue from infected animals may have contaminated cattle feed,
leading to the silent spread of the BSE epidemic. In humans the best known
of the prion diseases is CJD which reportedly affects 250-300 people a year
in the United States. Statistics indicate that 1 in 9000 over the age of 55
will be affected. Most of the cases are “sporadic” CJD (sCJD), occurring for
no, as yet, known reason while some are the familial form.
In response to the recent discovery of 3 cows testing positive for BSE in
the United States, Florence Kranitz, President of the CJD Foundation said,
“By eating beef that has not been rigorously tested, the American population
faces an ominous risk. It is important to keep the food supply safe to
prevent individuals from contacting the deadly human form of “mad cow
disease”, called variant Creutzfeldt - Jakob disease (vCJD). Only through
strict enforcement of a rigid and transparent animal surveillance system can
we realistically expect to protect the U.S. population from this 100% fatal,
ugly, brain eating disease.” Kranitz added the CJD Foundation feels it is
extremely important to continue the process of educating families,
healthcare providers, researchers and political leaders on the devastation
of the disease, as well as, the progress being made towards identifying a
cure for CJD.”
The CJD Foundation proudly unveiled its Medical Education DVD, “Confronting
CJD and Other Prion Disorders.” The purpose of the DVD is to educate
medical professionals about the prion disease and the care of patients and
families affected by CJD. It will be presented during Grand Rounds at many
hospitals/teaching institutions across the country.
The Creutzfeldt-Jakob Disease Foundation (CJD) is a not for profit
organization providing support and advocacy work on behalf of patients and
families affected by this rare, always fatal brain wasting disease. Anyone
needing information regarding CJD is encouraged to contact the Foundation at
1-800-659-1991 or through the website at www.cjdfoundation.org.
HOWEVER, do remember, there are strains of TSE in the USA now in the very
young, and there ae strains of TSE of 'UNKNOWN' strain;
(please note steady increase in sporadic cjd from 1997 to 2004 with steady
increase in type 'UNKNOWN' CJD? also, seems like they could come up with a
better, more readable chart. ...TSS)
HUMAN and ANIMAL TSE Classifications i.e. mad cow
disease and the UKBSEnvCJD only theory
TSEs have been rampant in the USA for decades in many
species, and they all have been rendered and fed back
to animals for human/animal consumption. I propose that
the current diagnostic criteria for human TSEs only
enhances and helps the spreading of human TSE from the
continued belief of the UKBSEnvCJD only theory in 2005.
With all the science to date refuting it, to continue
to validate this myth, will only spread this TSE agent
through a multitude of potential routes and sources
i.e. consumption, surgical, blood, medical, cosmetics
etc. I propose as with Aguzzi, Asante, Collinge,
Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis,
Marsh, et al and many more, that the world of TSE
Tranmissible Spongiform Encephalopathy is far from an
exact science, but there is enough proven science to
date that this myth should be put to rest once and for
all, and that we move forward with a new classification
for human and animal TSE that would properly identify
the infected species, the source species, and then the
route. This would further have to be broken down to
strain of species and then the route of transmission
would further have to be broken down. Accumulation and
Transmission are key to the threshold from subclinical
to clinical disease, and key to all
this, is to stop the amplification and transmission of
this agent, the spreading of, no matter what strain.
BUT, to continue with this myth that the U.K. strain of
BSE one strain in cows, and the nv/v CJD, one strain in
humans, and that all the rest of human TSE is one
single strain i.e. sporadic CJD (when to date there are
6 different phenotypes of sCJD), and that no other
animal TSE transmits to humans, to continue with this
masquerade will only continue to spread, expose, and
kill, who knows how many more in the years and decades
to come. ONE was enough for me, My Mom, hvCJD, DOD
12/14/97 confirmed, which is nothing more than another
mans name added to CJD, like CJD itself, Jakob and
Creutzfeldt, or Gerstmann-Straussler-Scheinker
syndrome, just another CJD or human TSE, named after
another human. WE are only kidding ourselves with the
current diagnostic criteria for human and animal TSE,
especially differentiating between the nvCJD vs the
sporadic CJD strains and then the GSS strains and also
the FFI fatal familial insomnia strains or the ones
that mimics one or the other of those TSE? Tissue
infectivity and strain typing of the many variants of
the human and animal TSEs are paramount in all variants
of all TSE. There must be a proper classification that
will differentiate between all these human TSE in order
to do this. With the CDI and other more sensitive
testing coming about, I only hope that my proposal will
some day be taken seriously. ...
PLUS, CDC has finally recognized the BASE and we do have 2 mad cows that
were of the atypical strain, the last Texas mad cow and the last documented
mad cow in USA in Alabama ;
However, based on analysis of molecular features of prion
diseases in cattle, this situation is similar to that in humans
(5), in which different subtypes of sporadic Creutzfeldt-
Jakob disease agents are found.
Project Number: 3625-32000-073-07
Project Type: Specific C/A
Start Date: Sep 15, 2004
End Date: Sep 14, 2007
The objective of this cooperative research project with Dr. Maria
Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is
to conduct comparative studies with the U.S. bovine spongiform
encephalopathy (BSE) isolate and the atypical BSE isolates identified in
Italy. The studies will cover the following areas: 1. Evaluation of
present diagnostics tools used in the U.S. for the detection of atypical
BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other
typical BSE isolates with atypical BSE cases. 3. Studies on
transmissibility and tissue distribution of atypical BSE isolates in
cattle and other species.
This project will be done as a Specific Cooperative Agreement with the
Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale
del Piemonte, in Turin, Italy. It is essential for the U.S. BSE
surveillance program to analyze the effectiveness of the U.S diagnostic
tools for detection of atypical cases of BSE. Molecular comparisons of
the U.S. BSE isolate with atypical BSE isolates will provide further
characterization of the U.S. BSE isolate. Transmission studies are
already underway using brain homogenates from atypical BSE cases into
mice, cattle and sheep. It will be critical to see whether the atypical
BSE isolates behave similarly to typical BSE isolates in terms of
transmissibility and disease pathogenesis. If transmission occurs,
tissue distribution comparisons will be made between cattle infected
with the atypical BSE isolate and the U.S. BSE isolate. Differences in
tissue distribution could require new regulations regarding specific
risk material (SRM) removal.
> Differences in tissue distribution could require new regulations
> regarding specific risk material (SRM) removal.
yep, that's what i been talking about.
least i know they know now, and are concerned.
too bad they did not finish decades ago what they started
at Mission, TEXAS ;
WHEN in fact, the findings from Marsh and the findings at
MISSION, TEXAS support even further evidence that there
are furthers strains of TSE in the USA besides that one
accidently documented BSE case in Washington on
Dec. 23, 2003;
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 [head of England's main veterinary lab]
2. Meeting with USDA, BSE Task Force
This group comprises Alex Thierman (USDA-APHIS, International Programs)
(Chairman), Roger Breeze (USDA-ARS Director, Plum Island), Bill Hadlow
(Neuropathologist - retired, formerly of NIH Rocky Mountain Laboratory,
Hamilton, Montana), John Gorhan and Mark Robinson (USDA-ARS, Pullman,
Washington) and Dick Marsh (Dept. Vet. Science, Univ Wisconsin - Madison).
The objectives of the group are to assess the implications of the
occurrence of BSE for US cattle particularly the risk of BSE occurrence
in the US in relation to endemic scrapie agent.
The purpose of my invitation to this meeting was to discuss aspects of
research which are of common interest and to identify a tentative USDA
research programme including any potential collaborative projects. The
discussions were informal and there was no agenda.
The general opinion
of those present was that BSE, as an overt disease phenomenon, could
exist in the USA but if it did it was very rare. The need for improved
and specific surveillance methods to detect it was recognised. Clinical
similarities between BSE and rabies suggested one means of sampling the
cattle population which might increase the probability of detection of
BSE. It was clear that the bovine rabies negative rate would vary
greatly between States but initially this should be determined and as it
would inevitably be high relative to positive cases, some differential
diagnosis carried out.
The work of Wilbur Clarke at Mission, Texas was discussed briefly. the
results of this study in which 10 calves were inoculated with scrapie
has not been published and perhaps would not be published. USDA are
sensitive regarding publicity of the results of the study which remain
far from conclusive. Apparently only 3 of the inoculated animals
developed neurological signs. The neuropathology of the affected cattle
has not been examined in any depth but Hadlow has the material. Marsh
indicated the requirement to obtain the fresh brain material from this
study in order to perform PrP extractions.
Because of the successful transmission of the Brecke (Stetsonville)
isolate of TME to cattle and the subsequent passage history in mink it
was generally considered important that comparisons be made with BSE
isolates in mink. Is BSE like scrapie in mink? Is BSE like the Brecke
isolate of TME?
Very little was said about CWD but some present considered that its
occurrence may indicate a sylvatic origin of agent. It was also agreed
that the role of possible subclinical infection in the
epidemiology of transmissible spongiform encephalopathies could well
be important but was unknown.
Marsh remarked on the possibility that
BSE was due to an extremely thermostable strain of agent. His
experience in the past with one particular Wisconsin isolate of TME
(Hayward strain) suggested that i/c biopsy needles could not be
effectively "scrapie sterilised", even employing an experimental
autoclave system capable of 60 psi and 300"C+ for 5 hours. This
experience led him to the policy that in scrapie or TME transmission
studies re-use of instruments or glassware that had contained agent was
an unacceptable protocol.
I was given confidential access to sections from the Clarke scrapie-
cattle transmission experiment. Details of the experimental design were
as supplied previously by Dr Wrathall (copy of relevant information
appended). Only 3 animals (2 inoculated with 2nd pass Suffolk Scrapie
and 1 inoculated with Angora goat passaged scrapie) show clinical signs.
Clinical signs were characterised by weakness, "a stilted hindlimb
gait", disorientation, ataxia and, terminally,
lateral recumbency. The two cattle from which I examined material were
inoculated at 8 months of age and developed signs 36 months pi (goat
scrapie inoculum) and 49 months pi (one of the Suffolk scrapie
This latter animal was killed at 58 months
of age and so the clinical duration was only 1 month. The neuropathology
was somewhat different from BSE or the Stetsonville TME in cattle.
Vacuolar changes were minimal, to the extent that detection required
careful searching. Conversely astrocyte hypertrophy was a widespread and
prominent feature. The material requires detailed neuropathological
assessment but whether or not this will be done remains in question.
VISIT TO USA - DR A E WRATHALL - INFO ON BSE AND SCRAPIE
1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has
successfully transmitted ovine and caprine scrapie to cattle. The
experimental results have not been published but there are plans to do
this. This work was initiated in 1978. A summary of it is:-
Expt A 6 Her x Jer calves born in 1978 were inoculated as follows with a
2nd Suffolk scrapie passage:-
i/c 1ml i/m, 5ml; s/c 5ml; oral 30ml.
1/6 went down after 48 months with a scrapie/BSE-like disease.
Expt B 6 Her or Jer or HxJ calves were inoculated with angora Goat virus
2/6 went down similarly after 36 months.
Expt C Mice inoculated from brains of calves/cattle in expts A & B were
resistant, only 1/20 going down with scrapie and this was the reason
given for not publishing.
Diagnosis in A, B, C was by histopath. No reports on SAF were given.
Dr Warren Foote indicated success so far in eliminating scrapie in
offspring from experimentally (and naturally) infected sheep by ET. He
had found difficulty in obtaining emhryos from naturally infected sheep
3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr A Thiermann showed the picture
in the "Independent" with cattle being incinerated and thought this was
a fanatical incident to be avoided in the US at all costs. BSE was not
reported in USA.
4. Scrapie incidents (ie affected flocks) have shown a dramatic increase
since 1978. In 1953 when the National Control Scheme was started there
were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.
5. Scrapie agent was reported to have been isolated from a solitary fetus.
6. A western blotting diagnostic technique (? on PrP) shows some promise.
7. Results of a questionnaire sent to 33 states on the subject of
the national sheep scrapie programme survey indicated;
17/33 wished to drop it
6/33 wished to develop it
8/33 had few sheep and were neutral
Information obtained from Dr Wrathall's notes of a meeting of the U.S.
Animal Health Association at Little Rock, Arkansas Nov. 1988.
CONFIDENTIAL TRANSMISSION (Day 4)
6.1 BSE to pigs
full text ;
3.57 The experiment which might have determined whether BSE and scrapie
were caused by the same agent (ie, the feeding of natural scrapie to
cattle) was never undertaken in the UK. It was, however, performed in
the USA in 1979, when it was shown that cattle inoculated with the
scrapie agent endemic in the flock of Suffolk sheep at the United States
Department of Agriculture in Mission, Texas, developed a TSE quite
unlike BSE. 32
findings of the initial transmission, though not of the clinical or
neurohistological examination, were communicated in October 1988 to Dr
Watson, Director of the CVL, following a visit by Dr Wrathall, one of
the project leaders in the Pathology Department of the CVL, to the
United States Department of Agriculture. 33
results were not published at this point, since the attempted
transmission to mice from the experimental cow brain had been
inconclusive. The results of the clinical and histological differences
between scrapie-affected sheep and cattle were published in 1995.
Similar studies in which cattle were inoculated intracerebrally with
scrapie inocula derived from a number of scrapie-affected sheep of
different breeds and from different States, were carried out at the US
National Animal Disease Centre. 34
results, published in 1994, showed that this source of scrapie agent,
though pathogenic for cattle, did not produce the same clinical signs of
brain lesions characteristic of BSE.
Visit to USA ... info on BSE and Scrapie
----- Original Message -----
From: "June Reed"
Sent: Friday, July 14, 2006 3:34 PM
Subject: Re: Local Man Diagnosed with Chronic Wasting Disease ?
> ##################### Bovine Spongiform Encephalopathy
> Good afternoon, everyone,
> Terry's post below (NOT his authorship ) included this:
> "If Kowalske does suffer from the disease it would make him the first
> human to contract the condition. The only cure for C.W.D. is a stem cell
> transplant, which costs $35 thousand dollars. "
> I may have been out of the loop for awhile, but that one had me
> my head...eh, what??
> Pleading ignorance, could someone enlighten me when they began stem-cell
> transplants for TSEs?
> And please, I have been away and my tone is sincere.
> Kind Regards and TIA,
> June Reed Reisinger
> ----- Original Message -----
> From: "Terry S. Singeltary Sr."
> Sent: Thursday, July 13, 2006 3:14 PM
> Subject: [BSE-L] Local Man Diagnosed with Chronic Wasting Disease ?
> ##################### Bovine Spongiform Encephalopathy
> Subject: Local Man Diagnosed with Chronic Wasting Disease ?
> Date: July 13, 2006 at 11:38 am PST
> Local Man Diagnosed with Chronic Wasting Disease
> Posted: 7/12/2006
> » View Picture » Play Video
> Hide Video
> A man in Traverse City may be the first person to ever contract a deadly
> disease that normally plagues deer. Chronic Wasting Disease is commonly
> known as C.W.D. Now doctors have diagnosed a man in Traverse City with the
> disease which eats away at nerves.
> Scott Kowalske's physician says they can not be 100% sure he has C.W.D.
> until a brain biopsy is performed. If Kowalske does suffer from the
> it would make him the first human to contract the condition. The only cure
> for C.W.D. is a stem cell transplant, which costs $35 thousand dollars.
> 9&10's Leah Beno and Photojournalist Dustin Bacon have the story.
> DONT know what this is all about, and to state categorically that this
> has human CWD, i think is a bit premature, not that i doubt cwd will
> transmit to humans. word i get is the man ate a lot of venison and it is
> doctor putting this out that the man has human CWD. when the cdc AND CWRU
> gets ahold of this, it will turn out to be sCJD. ...TSS
> #################### https://lists.aegee.org/bse-l.html
> #################### https://lists.aegee.org/bse-l.html
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