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From: TSS ()
Subject: Medics are misreading CJD risk (maybe not???)
Date: March 13, 2005 at 8:36 am PST

-------- Original Message --------
Subject: Medics are misreading CJD risk (maybe not???)
Date: Sun, 13 Mar 2005 10:40:41 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@KALIV.UNI-KARLSRUHE.DE


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

Medics are misreading CJD risk

By Jennifer Cooke
March 14, 2005

A misinterpretation of infection-control guidelines has led to some
surgeons, dentists and hospitals refusing or deferring patients whose
previous medical regime has put them at small risk of a fatal brain disease.

The patients who need surgery or elective procedures to improve or
monitor their health feel they are mistakenly treated as being at "high
risk" rather than at "low risk" of contracting the lethal
Creutzfeldt-Jakob Disease, and that finance and insurance take priority
over their well-being because cleaning expensive surgical instruments to
the required standard to neutralise the CJD agent can destroy the
instruments.

The instruments, some of which cost tens of thousands of dollars, must
be treated either with longer and higher temperature surgical
autoclaving - after use on high-risk tissue such as the brain or spine -
or destroyed or quarantined for exclusive use on an individual patient.

The national co-ordinator of the CJD Support Group Network, Suzanne
Solvyns, said problems have occurred because some medical professionals
have mistakenly treated procedures on patients with low infectivity
tissue as the more dangerous high-infectivity tissue.

While problems have arisen sporadically over the past decade, a recent
spate of treatment deferrals involving former recipients of human
hormone drugs - human pituitary gonadotrophin (hPG) and human growth
hormone (hGH) - have caused stress to recipients as well as the
Department of Health and Ageing, which is concerned about them being
"disadvantaged" despite efforts to educate health-care workers.

A Sydney woman told the Herald she had been "treated like a leper" three
times in the past three years by different surgeons and hospitals
operating in the eastern suburbs - most recently by a gastroenterologist
who refused to treat her and referred her for an alternative procedure
and to an infectious diseases specialist.

Other cases include a woman from Canberra - already prepped for a gall
bladder removal at a hospital that had known of her hHG injections for
months - who was sent home after an infection-control nurse read her
medical history.

A Queensland man had his public dental surgery postponed - just as the
procedure was starting - when he belatedly told the dentist of his hGH
treatment.

The latest guidelines state that people regarded at "low" risk of
acquiring CJD include those recipients of cadaver-derived human
pituitary hormone drugs, but that it is only high-risk tissue such as
the brain and spine that requires the quarantining of equipment until
CJD is either confirmed - when the instruments must be destroyed - or
ruled out, which then allows the instruments to be re-used.

Mrs Solvyns said these patients were not in any high-risk group.

"Low infectivity tissue only needs normal sterilisation and that's
what's causing the stress to recipients. "It's almost like they won't
believe the guidelines - or the experts," she said.

A spokeswoman for the Department of Health and Ageing, Kay McNiece, said
yesterday: "The department was very disappointed to find that in some
cases doctors are misreading the infection-control guidelines which very
clearly spell out what precautions are needed, but also what precautions
aren't required."


http://www.smh.com.au/news/National/Medics-are-misreading-CJD-risk/2005/03/13/1110649058729.html?oneclick=true


> The national co-ordinator of the CJD Support Group Network, Suzanne
> Solvyns, said problems have occurred because some medical
> professionals have mistakenly treated procedures on patients with low
> infectivity tissue as the more dangerous high-infectivity tissue.
>

THESE same tissues they now claim to be low infectivity tissue once
were thought to be not infectious at all. now with new more sensitive
testing, we are finding infectivity where once it was thought no infectivity
were there. AS science progresses, more sensitive testing comes
forth, we may find that these same tissues are indeed a threat to
public health via a multitude of routes and sources, and IF accumulation
plays a part, who knows what the threshold of infectivity is. THE fear
these medical professionals have, might indeed be very real...

> Mrs Solvyns said these patients were not in any high-risk group.
>
> "Low infectivity tissue only needs normal sterilisation and that's
> what's causing the stress to recipients. "It's almost like they won't
> believe the guidelines - or the experts," she said.
>

MAYBE these guidelines for sporadic CJD _are_ wrong. they have been
wrong all along with respect to
other guidelines about human/animal TSEs. a few things to ponder ;


107
Vet Pathol 42:107–108 (2005)
Letters to the Editor

Editor:

Absence of evidence is not always evidence of absence.
In the article ‘‘Failure to detect prion protein (PrPres) by
immunohistochemistry in striated muscle tissues of animals
experimentally inoculated with agents of transmissible spongiform
encephalopathy,’’ recently published in Veterinary
Pathology (41:78–81, 2004), PrPres was not detected in striated
muscle of experimentally infected elk, cattle, sheep, and
raccoons by immunohistochemistry (IHC). Negative IHC,
however, does not exclude the presence of PrPSc. For example,
PrPres was detected in skeletal muscle in 8 of 32
humans with the prion disease, sporadic Creutzfeldt-Jakob
disease (CJD), using sodium phosphotungstic acid (NaPTA)
precipitation and western blot.1 The NaPTA precipitation,
described by Wadsworth et al.,3 concentrates the abnormal
isoform of the prion, PrPres, from a large tissue homogenate
volume before western blotting. This technique has increased
the sensitivity of the western blot up to three orders
of magnitude and could be included in assays to detect
PrPres. Extremely conspicuous deposits of PrPres in muscle
were detected by IHC in a recent case report of an individual
with inclusion body myositis and CJD.2 Here, PrPres was
detected in the muscle by immunoblotting, IHC, and paraf-
fin-embedded tissue blot. We would therefore caution that,
in addition to IHC, highly sensitive biochemical assays and
bioassays of muscle are needed to assess the presence or
absence of prions from muscle in experimental and natural
TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland

References

1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812–1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121–125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171–180,
2001

===============================

Creutzfeldt–Jakob Disease
and Inclusion Body
Myositis: Abundant
Disease-Associated Prion
Protein in Muscle

Gabor G. Kovacs, MD PhD,1,2
Elisabeth Lindeck-Pozza, MD,1 Leila Chimelli, MD, PhD,3
Abelardo Q. C. Arau´jo, MD, PhD,4
Alberto A. Gabbai, MD, PhD,5 Thomas Stro¨bel, PhD,1
Markus Glatzel, MD,6 Adriano Aguzzi, MD, PhD,6
and Herbert Budka, MD1

Pathological prion protein (PrPSc) is the hallmark of
prion diseases affecting primarily the central nervous system.
Using immunohistochemistry, paraffin-embedded
tissue blot, and Western blot, we demonstrated abundant
PrPSc in the muscle of a patient with sporadic
Creutzfeldt–Jakob disease and inclusion body myositis.
Extraneural PrPC-PrPSc conversion in Creutzfeldt–Jakob
disease appears to become prominent when PrPC is abundantly
available as substrate, as in inclusion body myositis
muscle.
Ann Neurol 2004;55:121–125
Presence of the normal cellular prion protein (PrPC) is
essential in the formation of a protease-resistant pathological
conformer termed PrPSc, a hallmark of prion
diseases or transmissible spongiform encephalopathies.
1,2 PrPC–PrPSc conversion may be initiated by external
PrPSc, as in iatrogenic or variant Creuzfeldt–Jakob
disease (vCJD). The basis of sporadic CJD (sCJD)
is currently undetermined, whereas genetic cases are
suggested to result from structural instability of a mutated
PrP gene (PRNP) product.2
PrPC is expressed, although in lower amounts, in
nonneural tissue including skeletal muscle. PrPSc was
deposition in sIBM muscle tissue of a patient with
concomitant CJD.

snip...

Results

Neuropathology and Paraffin-Embedded Tissue Blot
The propositus’ brain had nearly complete loss of
neurons in cerebral and cerebellar cortex with severe
astrogliosis and confluent spongiform change (Fig 1A,
C). Immunostaining for PrP showed diffuse synaptic
type deposits in the cerebral cortex and stained cerebellar
kuru type amyloid plaques (see Fig 1B, D).
Walls of intracerebral vessels and tissues of spleen and
lung lacked immunoreactivity. In cross-sections and
longitudinal sections of muscle, we noted lymphocytic
infiltration and rimmed vacuoles (see Fig 1E).
IHC demonstrated strong diffuse granular PrP deposits
within muscle cells surrounding vacuoles (see Fig
1F–H). This was not seen in formalin-fixed muscle of
other sIBM cases. PET blot showed focal PrPSc deposits
in muscle of the case, but not in controls (see
Fig 1I).
PRNP and Western Blot Analysis
No PRNP mutation was detected. The polymorphic
codon 129 was MV heterozygous.
Immunoblotted brain tissue of the propositus contained
high amounts of PrPSc in a pattern of type 2A
according to Parchi and colleagues,11 or type 3 according
to recent classification by Hill and colleagues14 (Fig
2A). Blotting for PrPSc in muscle gave a strong signal
after PTA treatment (data not shown) and was detectable
by conventional Western blotting (see Fig 2B),
showing a pattern similar to type 2B (or type 4).11,14
By performing serial dilutions of sCJD diseased
brain tissue, we found the relative PrPSc content in
muscle to be 30% of that found in another sCJD diseased
brain.

Discussion

In addition to neural tissue, PrPSc in humans has been
demonstrated only in lymphoreticular tissue of vCJD
and in vessels walls of sCJD and vCJD cases.15,16 Muscle
tissue in experimental prion infected mice contains
122 Annals of Neurology Vol 55 No 1 January 2004
PrPSc at a level approximately 5 to 10% of that in
brain, but PrPSc accumulation varies between muscle
groups.3 Using IHC, PET blot, and Western blot, we
demonstrated PrPSc in biceps muscle in a patient with
CJD and sIBM. Remarkably, very high amounts of
PrPSc (30% of that observed in a sCJD brain) were
present in the muscle. Apparently, sIBM in the muscle
has greatly amplified the amount of PrPSc that might
be present in CJD muscle.
On one hand, a protracted course of disease might
result in PrPSc reaching a higher level in a tissue such
as muscle, with normally little conversion to PrPSc,
which then becomes observable even by IHC. On the
other hand, the unique co-occurrence of sIBM, known
to be related with a high expression of PrPC in muscle,
and CJD offers an increased chance for extraneural
PrPC–PrPSc conversion as a consequence of a yet uncharacterized
pathogenic event. Whether other tissues
also show increased expression of PrPC in sIBM is unknown.
PrPSc was restricted to brain and muscle in our
case.
MV at PRNP codon 129 accompanied by type 2A
or type 3 PrPSc in the Western blot represents a subgroup
of sCJD with amyloid plaques and prolonged
duration of illness.11,14 However, this case is unusual
for several reasons: the protracted course of brain disease;
the initial progressive dysphasia; end-stage degree
of neuronal loss; and the prolonged muscle disease in
form of progressive tetraparesis. The role of PRNP
polymorphism at codon 129 in sIBM is debated.17,18
Presence of muscle disease in CJD subtypes merits further
systematic study. We show that distinct glycotypes
of PrPSc may exist in muscle and brain in accordance
with the different PrPC glycoform profile.8 Interestingly,
the glycotype of muscle in this case resembles
that observed in vCJD brain.
Knowledge of potentially infectious tissues has implications
for safety and public health. As PrPSc is
considered as surrogate for infectivity, its presence in
muscle might indicate a new iatrogenic transmission
risk.
Fig 1. (A) Severe spongiform change, neuronal loss, and
astrogliosis in frontal cortex of the propositus (20; H and E). (B)
Diffuse/
synaptic PrP immunoreactivity in the same area shown in panel A (20;
immunostaining with 3F4).
(C) Loss of granular cell layer and spongiform change of molecular layer
in the cerebellum of the
propositus (10; H and E). Note the kuru type plaques enlarged in the
lower right corner.
(D) Diffuse/synaptic and plaque (enlarged in lower right corner) type
PrP immunoreactivity in
the same area shown in panel C. (10; immunostaining with 3F4). (E)
Vacuoles in the muscle
fibers and variation in caliber size in the propositus (10; H and E).
(F) Coarse granular PrP
immunoreactivity in some muscle fibbers (10; immunostaining with 3F4).
(G, H) Patterns of PrP
immunoreactivity around vacuoles and within muscle fibers (both 20;
immunostaining with 3F4).
(I) Top panel shows longitudinal section of paraffin-embedded biceps
muscle tissue from the propositus
immunostained with 3F4 (20). Middle panel indicates longitudinal
section of paraffin-embedded tissue
(PET) blot of biceps muscle tissue immunostained with 3F4 from the
propositus (20). Bottom panel
shows longitudinal section of PET blot of muscle immunostained with 3F4
from a control case without muscle disease (20).

snip...end...TSS

===============================

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

snip...

Conclusions Using sensitive techniques, we identified extraneural
deposition of PrPSc in spleen and muscle samples from approximately
one third of patients who died with sporadic Creutzfeldt-Jakob disease.
Extraneural PrPSc appears to correlate with a long duration of disease.

http://content.nejm.org/cgi/content/short/349/19/1812?query=TOC

Prions in skeletal muscle (Prusiner et al)

http://www.pnas.org/cgi/doi/10.1073/pnas.052707499

EMBO reports AOP Published online: 11 April 2003 Widespread PrPSc
accumulation in muscles of hamsters orally infected with scrapie

http://www.emboreports.org/

Then i always like to go back to read this study, always scares the hell out
of me;

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


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

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

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

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

PMID: 8006664 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

> A Sydney woman told the Herald she had been "treated like a leper"
> three times in the past three years by different surgeons and
> hospitals operating in the eastern suburbs - most recently by a
> gastroenterologist who refused to treat her and referred her for an
> alternative procedure and to an infectious diseases specialist.
>

BEING treated as a ''leper'' is wrong, but being cautious with human
TSEs is paramount, and
to only be cautious with nvCJD and ignore all the rest of human TSEs is
suicidal not only for
the health care workers, but also for other patients those same tools
will be used on...

From: Terry S. Singeltary Sr.
(216-119-132-53.ipset12.wt.net)
Subject: Variant Creutzfeldt-Jakob disease: update LETTER GUT/ENDOSCOPY
Date: December 13, 2004 at 8:21 am PST

http://www.vegsource.com/talk/madcow/messages/93658.html

How much is too much? How much is not enough? What about accumulation?
What is the Threshold of exposure, to infectivity, to clinical disease?
How many strains/phenotype does sporadic CJD consist of and
What is the infectivity of each one of those phenotypes (cwdCJD),
or (aBSE or BaSE CJD)?


Until these questions are answered, you are gambling, nothing more, and the
fears these health care workers have and the risk to the public via
surgical tools are real and should be treated as such. ...


Terry S. Singeltary Sr.

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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