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From: TSS ()
Subject: Re: Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES)
Date: April 4, 2007 at 2:07 pm PST

----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Wednesday, April 04, 2007 4:10 PM
Subject: Re: Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES)

FURTHER into this case study, Colorado Surveillance Program for Chronic
Wasting Disease Transmission to Humans
(TWO SUSPECT CASES) a look at case 1 and case 2 ;

CASE 1

A 52-year-old right-handed woman presented with a
1-year history of progressive memory loss, language impairment,
visuospatial disturbance, and myoclonus. She
related that she had been a histology technician in a laboratory
that processed tissue specimens from deer and elk
with CWD and had handled specimens without wearing
gloves. Both she and her family expressed significant
concerns about the possibility of transdermal transmission
of CWD. Her family history was negative for
dementia and other neurologic disorders. Brain magnetic
resonance imaging showed mild diffuse volume loss,
and electroencephalography demonstrated mild diffuse
slowing. Other laboratory studies were unremarkable. Cerebrospinal
fluid findings were unremarkable except for
a weakly immunostaining 14-3-3 protein band, an indeterminate
finding for the diagnosis of prion disease. Genetic
testing of the prion protein gene was normal, revealing
methionine homozygosity at codon 129. Brain
biopsy results were negative for the presence of proteaseresistant
prion protein but showed definite Alzheimer disease
with numerous neuritic plaques and tau-positive neurofibrillary
tangles (Figure). Further analysis of brain
tissue at the National Prion Disease Pathology Surveillance
Center was negative for prion disease by Western
blot analysis. Subsequent investigation by the state department
of health revealed the patient had worked in
an area of the laboratory that conducted necropsies on
domestic animals and had never been assigned to the
CWD testing laboratory. The Colorado Department of
Public Health and Environment could not confirm that
the technician had ever worked with deer and elk tissues.

CASE 2

This 25-year-old right-handed man had a 4-month history
of progressive gait disturbance, myoclonus, hallucinations,
slowed cognition, impaired attention, and
memory loss. He had hunted deer and elk in a CWD endemic
area of southern Wyoming and cooked and ate the
field-dressed meat. His family history was significant in
that his mother had died of a dementing disease at age
40 years, although there was neither a clinical diagnosis
nor an autopsy. Brain magnetic resonance imaging findings
were unremarkable, and electroencephalography
demonstrated 1-Hz high-amplitude periodic sharp wave
complexes. Other laboratory studies had negative results.
Testing for the 14-3-3 protein had positive results,
but the cerebrospinal fluid was otherwise unremarkable.
The diagnosis of Gerstmann-Stra¨ussler-Scheinker
syndrome, a familial prion disease, was confirmed with
a detailed autopsy examination and referral of the brain
to the National Prion Disease Pathology Surveillance Center.
Autopsy brain tissue showed the presence of proteaseresistant
prion protein by Western blot analysis. Genetic
evaluation revealed the P102L mutation in the prion protein
gene with methionine/valine heterozygosity at codon
129.

snip...end


I can't understand how they can keep claiming 'low, or no occupational
transmission of CJD' ??? when there have been many cases that should have
raised awareness, and in some cases they did, only to be swept under the rug
as the infamous sporadic CJD, or some other TSE other than the nvCJD of the
ukbsenvcjd only theory. it's a blown theory no one will accept too. lets
look at a few occupational cases. ...TSS

now, some things to ponder ;

Questions:

1. Do neuritic plaques and tau-positive neurofibrillary tangles indicate
definite AD? Aren't these also found in GSS? What about concurrent AD
and TSE?

2. Are the NPDPSC results conclusive? Do WB results depend on the part
of the brain sampled?

3. Doesn't it seem unlikely the woman would flat-out lie about working
with CWD tissues? (I'm working on this locally.)

4. What about cross-contamination? The lab gets large numbers of
scrapie-infected sheep and CWD-infected deer and elk. I assume the
necropsy area is contaminated with TSEs.


> Approved-By: tom
> Message-ID:
> Date: Mon, 19 Jul 1999 11:21:25 -0800
> Reply-To: Bovine Spongiform Encephalopathy
> Sender: Bovine Spongiform Encephalopathy
> From: tom
> Subject: iatrogenic scrapie from sheep dura mater?
> Someone kindly sent me the full text of a very curious 1993 Lancet
article.
> This is available from the Ovid service -- Lancet itself ironically does
not
> offer an electronic version this far back.
>
> An orthopaedic surgeon employed by the lyodura company [Braun Melsungen]
> extracted dura mater from sheep and human cadavers and came down with fast
> CJD 22 years later. The ratio of sheep to human dura mater he collected
> was150 sheep to 12 humans. Apparently the surgeon and the sheep were
German.
> Scrapie has long been present in Germany but reported levels are very low,
> about a flock a year has to be destroyed. I am not aware of any high
> sensitivity tests or random screening every being used in Germany to
assess
> the levels of preclinical animals.
>
> This raises the question, what did the lyodura company do with so much
dura
> mater from sheep? The market for specialty surgical products was
> overwelmingly in humans in 1968. The Lancet article only says it was for
> research -- but in what species? Perhaps dura mater gives an immune
> response across species after processing, ruling out its use in humans.
> But as far as I know, blood type or other genetic differences do not
matter
> within humans, ie, there is no tissue matching with dura mater.
>
> I always wondered how CJD could show up from a handful of human dura mater
> donations with sporadic CJD supposedly so rare -- on the other hand, there
> would be no surprise at all if a case of subclinical scrapie showed up in
> 150 sheep.
>
> This raises the question, have dura mater recipients or the surgeon
> subsequently been strain-typed? This might give a very different outcome
> than other forms of iatrogenic CJD or simply co-classify with pituitary
> growth hormone if route of infection (injected, oral, hereditary, etc.) is
> more important than strain source.
>
> Otherwise, iatrogenic scrapie (like cwdCJD) is somwhat unpredictable in
its
> gel pattern (though strains of scrapie in other primate species might be
> re-examined). The original scrapie strain is not be identifiable directly
> because material was not likely retained. Strain-typing was not available
> at the time of the article -- but Collinge was one of the authors.
>
> There is little doubt that scrapie could be transfered to humans by
> intracerebral injection (based on lack of species barrier in primates) and
> that processed pooled (human?) dura mater can carry sufficient infectivity
> to cause CJD. I am not aware of animal experiments that specifically used
> sheep dura mater as experimental dose source.
>
>
> tom
>
> -=-=-=-=-=-=-
>
>
> Transmission of Creutzfeldt-Jakob disease by handling of dura mater.
> The Lancet Volume 341(8837) January 9, 1993 pp
> 123-124
> Weber, Thomas; Tumani, Hayrettin; Holdorff, Bernd; Collinge, John; Palmer,
> Mark; Kretzschmar, Hans A.; Felgenhauer, Klaus
>
>
> Sir,- Creutzfeldt-Jakob disease (CJD) can be transmitted iatrogenically by
> human pituitary growth hormone, corneal transplants, and dura mater grafts
> (1). Possible accidental transmission has been reported in only four
> people-a neurosurgeon (2), a pathologist (3), and two laboratory
technicians
> (4,5) . We have encountered an unusually rapid case of CJD probably
acquired
> through handling of sheep and human dura mater.
> In May, 1992, a 55-year-old orthopaedic surgeon developed paraesthesia of
> the left arm. A few days later he had spatial disorientation, apraxia, and
> gait ataxia. In June he was admitted and a neurologist suspected CJD on
the
> basis of the clinical signs, typical electroencephalogram (EEG) pattern,
and
> history. An EEG in June revealed a typical pattern of periodic biphasic
and
> triphasic sharp wave complexes. We saw the patient in July, 1992. He was
> awake and oriented, with dyscalculia, dysgraphia, disturbed vision,
apraxia
> mainly of the left side, rigidity of wrists, spasticity of all muscles,
> myoclonus of the left arm, increased tendon reflexes, ataxia of limbs and
> trunk, and incoordination of left arm. Within 3 weeks he had impaired
> consciousness and attention, mildly impaired memory, and threatening
visual
> hallucinations with restless turning. He had periodic states with
movements
> of his head and eye-bulbs resembling tonic adversive seizures. During
sleep
> these motor disturbances stopped. 2 1/2 months later the patient died.
>
> This patient had worked with sheep and human dura mater from 1968 to 1972.
> He handled about 150 specimens of ovine origin and at least a dozen human
> preparations for research. Handling involved opening skulls with a band
saw,
> removing dura, and testing them either fresh (usually), preserved, or
> lyophilised for mechanical qualities. These specimens were sent to a
company
> that has sold dura mater preparations by which CJD was transmitted in six
> instances. No information was available from the company about a possible
> connection with this patient's disease and the earlier cases of
transmitted
> CJD. Brain biopsy was consistent with diagnosis of CJD. Cerebrospinal
fluid
> obtained in July showed neuron-specific enolase (NSE) at 82.0 ng/mL,
> compared with 16.7 ng/mL in serum of other cases (6). Proton magnetic
> resonance spectroscopy of parieto-occipital and temporal grey matter,
> parietal white matter, and thalamus revealed a 20-30% reduction of
> N-acetylaspartate, as described (7). DNA was genotyped with
allele-specific
> oligonucleotides (8) and was homozygous for methionine at the polymorphic
> codon 129. Subsequent direct DNA sequencing for the PrP gene open-reading
> frame demonstrated normal sequence on both alleles, excluding known or
novel
> pathogenic PrP mutations.
>
> It is tempting to speculate that prions were transmitted to this patient
> from sheep or human dura mater through small lacerations of his skin, but
> the patient and his wife did not remember any significant injury during
his
> four years of working with these samples. It cannot be excluded that this
> was a case of sporadic CJD although this assumption is unlikely in view of
> the clinical course which was similar to iatrogenic CJD transmitted by
> peripheral inoculation, such as with human pituitary growth hormone or
> gonadotropin or to kuru (1). Iatrogenic cases resulting from intracerebral
> inoculation with the transmissible agent, for instance following dura
mater
> grafts (2-5), present with a dementing picture, as is usual in sporadic
CJD,
> rather than with ataxia as in this case.
>
>
> 1. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones,
> homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27.
[Medline
> Link] [Context Link]
>
> 2. Schoene WC, Masters CL, Gibbs CJ Jr, et al. Transmissible spongiform
> encephalopathy (Creutzfeldt-Jakob Disease): atypical clinical and
> pathological findings. Arch Neurol 1981; 38: 473-77. [Medline Link]
[Context
> Link]
>
> 3. Gorman DG, Benson DF, Vogel DG, Vinters HV. Creutzfeldt-Jakob disease
in
> a pathologist. Neurology 1992; 42: 463. [Medline Link] [Context Link]
>
> 4. Miller DC. Creutzfeldt-Jakob disease in histopathology technicians. N
> Engl J Med 1988; 318: 853-54. [Medline Link] [Context Link]
>
> 5. Sitwell L, Lach B, Atack E, Atack D, Izukawa D. Creutzfeldt-Jakob
disease
> in histopathology technicians. N Engl J Med 1988; 318: 854. [Medline Link]
> [Context Link]
>
> 6. Wakayama Y, Shibuya S, Kawase J, Sagawa F, Hashizume Y. High
> neuron-specific enolase level of cerebrospinal fluid in the early stage of
> Creutzfeldt-Jakob disease. Klin Wochenschr 1987; 65: 798-801. [Medline
Link]
> [Context Link]
>
> 7. Bruhn H, Weber T, Thorwirth V, Frahm J. In-vivo monitoring of neuronal
> loss in Creutzfeldt-Jakob disease by proton magnetic resonance
spectroscopy.
> Lancet 1991; 337: 1610-11. [Medline Link] [Context Link]
>
> 8. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic
> Creutzfeldt-Jakob disease. Lancet 1991; 337: 1444-42. [Medlin


http://www.vegsource.com/talk/madcow/messages/1000330.html


Dormont*, and Jean-Philippe Deslys* et al, that 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;

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


NOT to forget all the farmers, and one wife of a farmer of a BSE herd, that
contracted CJD i.e. sporadic. ...tss


----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Thursday, March 29, 2007 11:21 AM
Subject: ANOTHER FARMER DIES OF MAD COW DISEASE i.e. sporadic CJD

Subject: ANOTHER FARMER DIES OF MAD COW DISEASE i.e. sporadic CJD
Date: March 29, 2007 at 8:00 am PST

'MAD COW' DEATH LINK


March 29, 2007 02:15am


A YORKE Peninsula man who had worked as a farmer has died from a rare
illness linked with mad cow disease.

The man, aged in his late 50s, died in the Royal Adelaide Hospital on
Sunday, after becoming ill in late January.
Communicable Diseases branch director Dr Ann Koehler said an autopsy had
confirmed the man died from Creutzfeldt-Jakob Disease, a variant of which is
called mad cow disease in other countries.

The death was the first in the state attributed to the disease for at least
two years.


http://www.news.com.au/adelaidenow/story/0,22606,21466465-2682,00.html


----- Original Message -----
From: XXXXXXXX XXXXXXXXXXXX(SEAC)
To: 'flounder9@verizon.net'
Sent: Friday, February 23, 2007 8:13 AM
Subject: RE: SEAC ANNUAL REPORT 2006


Dear Mr Singeltary

Thank you for your email about a possible relationship between BASE and
sCJD.

SEAC is continually informed about and considers the emerging science on
both animal and human TSEs. The committee regularly receives updates from
the UK's National CJD Surveillance Centre Unit on the epidemiology of vCJD
and sCJD and is also aware of emerging research on BASE. It is envisaged
that SEAC will conduct a detailed consideration of the available science and
the possible animal and human health implications of BASE at its meeting on
10th May 2007.

Yours sincerely

XXXXXXXXXXXXXX


----------------------------------------------------------------------------
----
From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
Sent: 02 February 2007 16:44
To: Bovine Spongiform Encephalopathy
Cc: SEACsecretariat (AHEG); cjdvoice@yahoogroups.com;
BLOODCJD@YAHOOGROUPS.COM; madcow@lists.iatp.org; Sustainable Agriculture
Network Discussion Group
Subject: SEAC ANNUAL REPORT 2006


Date: February 2, 2007 at 8:25 am PST
SEAC ANNUAL REPORT 2006 (ignoring the obvious)


http://www.seac.gov.uk/publicats/annualreport2006.pdf


i guess the sporadic CJD cases have all be resolved, and the route and cause
classified.
to this day, i cannot figure this out. if BSE farmers died from sporadic
CJD, and now another study seems to point
that BSE and BASE may be the same thing, and that BASE does not produce vCJD
like pathology, but pathology resembling that of sporadic CJD, then why does
SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC
could not even speak about it in there 2006 report. would it not seem
prudent to mention these findings in this 2006 SEAC report ;


Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.


snip...


http://www.seac.gov.uk/minutes/95.pdf


***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***


6:30 Close of Day One


http://www.healthtech.com/2007/tse/day1.asp


64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.


http://www.seac.gov.uk/minutes/95.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


I STILL think to ignore these findings below is not only rediculous, but
indeed very suspicious ;


CJD FARMERS WIFE 1989


http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf


http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf


cover-up of 4th farm worker ???


http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf


http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf


CONFIRMATION OF CJD IN FOURTH FARMER


http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf


now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be
worrying, especially as all four farmers with CJD would have had BSE
cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf


4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf


2. snip...


Over a 5 year period, which is the time period on which the advice
from Professor Smith and Dr. Gore was based, and assuming a
population of 120,000 dairy farm workers, and an annual incidence
of 1 per million cases of CJD in the general population, a
DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN
an individual in the general population to develop CJD. Using the
actual current annual incidence of CJD in the UK of 0.7 per
million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in
1993 and by Dr. Gore this month used the sub-population of dairy
farm workers who had had a case of BSE on their farms -
63,000, which is approximately half the number of dairy farm
workers - as a denominator. If the above sums are repeated using
this denominator population, taking an annual incidence in the general
population of 1 per million the observed rate in this sub-population
is 10 TIMES, and taking an annual incidence of 0.7 per million,
IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than
that in the general population...


http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf


CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in
a retrospective study (published 1991), having been originally
misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf


20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. A 19 year old died from sCJD in
France in 1985. There is no evidence of an iatrogenic
cause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

FOR SEAC TO continue to flounder with this ukbsenvcjd only theory, when we
know that sporadic CJD's were proven long ago to transmit via the medical
and surgical arena, will only continue to spread this agent around the globe
via a multitude of proven routes and sources.

IN my opinion, for SEAC to continue to go down this same road, year after
year, and continue to ignore sporadic CJD's, and continue to say that "BSE
in the UK sheep population is most likely to be zero, or very low if present
at all." "Consequently, any impact of the schemes on human health from
removing BSE from sheep is likely to be negligible." when in fact they do
not know. and in fact science is showing that in "transgenic mice BSE
passaged in sheep may be more virulent and infectious to a wider range of
species than bovine derived BSE." IN my opinion this continued neglect of
other TSEs in human and animals, the continued belief in this ukbsenvcjd
only theory, and the risk thereof, is reckless, dangerous, and in my mind,
criminal. SEAC has failed the public terribly in this regard. ...TSS

----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Friday, February 02, 2007 10:43 AM
Subject: SEAC ANNUAL REPORT 2006


Date: February 2, 2007 at 8:25 am PST
SEAC ANNUAL REPORT 2006 (ignoring the obvious)


http://www.seac.gov.uk/publicats/annualreport2006.pdf


i guess the sporadic CJD cases have all be resolved, and the route and cause
classified. to this day, i cannot figure this out. if BSE farmers died from
sporadic
CJD, and now another study seems to point that BSE and BASE may be the same
thing, and that BASE does not produce vCJD like pathology, but pathology
resembling that of sporadic CJD, then why does SEAC still seem to put
sporadic CJD on the back burners ??? IN fact, SEAC could not even speak
about it in there 2006 report. would it not seem prudent to mention these
findings in this 2006 SEAC report ;
........SNIP.........END............TSS

-------- Original Message --------
Subject: ANOTHER FARMER KILLED BY CJD !!!
Date: Wed, 23 Apr 2003 10:45:34 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
CC: CJDvoice , bloodcjd


FARMER KILLED BY CJD

10:30 - 22 April 2003

A father-of-two from Lincolnshire has died from suspected Variant
Creutzfeldt- Jakob Disease.

Farmer David Fussey (34), who owned Manor Farm, Middle Rasen, near
Market Rasen, was diagnosed with the condition in October 2002.

He died last Tuesday with his wife Jess (28) at his side. He left two
children Tom (four) and Laura (two).

Today his wife paid tribute to her husband, who spent the last seven
months being cared for at Blenheim House Nursing Home, in Hemswell
Cliff, near Market Rasen.

She said: "David was a strong man who was dedicated to his family.

"He was very proud to be a farmer and was keen to work with the land and
protect the environment.

"He was also at the heart of the community and represented the village
football and dart teams."

Doctors do not know how Mr Fussey contracted the disease.

Mrs Fussey said she first noticed a change in her husband's behaviour in
December 2001.

"It was Christmas and usually David would be out having a few drinks but
instead he was withdrawn and tired," she said.

"He had recently started a second job building barns to supplement our
main income as arable farmers, so I just thought he was worn out."

Mr Fussey's condition continued to worsen and by July 2002 Mrs Fussey
started to notice signs that something was seriously wrong.

She said her husband had started to have hallucinations.

"One day he said he had spoken to staff who had not worked with us for
years," she said.

"Another time he was looking for a tractor we had sold some time ago."

Mr Fussey was admitted to Lincoln County Hospital on September 23. In
October the doctors told Mrs Fussey the news she had feared.

She said: "I was deeply upset and thought this shouldn't be happening to
me and my family.

"But in a strange way it was comforting because I had the answer to why
my husband had deteriorated."

Mrs Fussey said she was not looking for anybody to blame.

"It is hard to know how to feel," she said. "I think what has happened
is cruel and unfair but the disease does not discriminate.

"Nobody knows exactly how the condition is caught so until someone tells
me the definitive answer I am not prepared to be bitter."

Variant CJD was first reported internationally in 1996 and since then
127 people in Britain have died from the disease. Currently there are
seven British people thought to be infected.

The UK Creutzfeldt-Jakob Disease Surveillance Unit at the University of
Edinburgh is researching the illness.

The unit's Professor James Ironside said: "Variant CJD is thought to be
contracted by the same agent that causes BSE in cattle.

"The most likely way humans would come into contact with the agent is
through the food chain. Fortunately the disease is very rare."

Mr Fussey's funeral will be held at 1pm on Friday at the St Peter and St
Paul Church, in North Street, Middle Rasen. Mr Fussey will be taken to
his funeral in a coffin on a trailer pulled by his favourite green John
Deere tractor.

http://www.thisislincolnshire.co.uk/displayNode.jsp?nodeId=57711&command=displayContent&sourceNode=57238&contentPK=5269534


La Medicina del Lavoro
Med Lav 2003; 94,4:353-363

Occupational risk factors for the sporadic form of
Creutzfeldt-Jakob disease

PL. Cocco, A. CAPERNA*, F. VINCI*

Dipardmento di Sanita Pubblica, Sezione di Medicina del Lavoro, Universita
di Cagliari
* Isdtuto di Medicina del Lavoro, Facolta di Medicina e Chirurgia A.
Gemelli, Universita Cattolica del Sacro Cuore, Roma


snip...


All the observed cases were livestock farmers,
four of whom (0.6 expected) occurred in farms
where BSE cases had been reported. No cases were
observed among veterinarians (0.03 expected), or
butchers. (0.15 expected). A matched case-control
study of 206 sCJD cases and controls did not iden-
tify any association with a priori suspected occupa-
tions (38). However, 21 cases (10%) e 14 controls
(7%) occurred in subjects with occupational contact
with animal products (p=0.17). The authors did
not explore this finding in detail. We conducted a
crude calculation based on the published data, and
we found instead an Odds Ratio of 1.6 (95% confi-
dence interval 1.18,2.15).

snip...

METHODS

The 24 US states death certificates database we
used consists of several million coded death certfi-
cates from 24 US states, covering the years 1984-
95. The 24 states are: Colorado, Georgia, Idaho
(from 1988), Indiana (from 1986), Kansas, Ken-
tucky, Missouri (in 1984-86), Maine, Nebraska (in


CJD AND OCCUPATION

357

1984-85), Nevada, New Hampshire, New Jersey
(from 1988), New Mexico (from 1986), North
Carolina (from 1987), Ohio (from 1985), Okla-
homa, Rhode Island, South Carolina, Tennessee
(in 1985-88), Utah (from 1985), Vermont, West
Virginia (from 1988), Washington (from 1989),
and Wisconsin. In addition to standardized coding
procedures, information on usual occupation and
kind of business or industry, reported in the death
certificate for each decedent, was included in the
database provided to the National Center for
Health Statistics (NCHS) (7). The information on
occupation and industry was coded according to
the 1980 US Bureau of the Census classification
(6). The underlying cause of death was coded ac-
cording to the International Classification of Dis-
eases - 9th revision. No further details, such as du-
ration of employment or concurrent diseases, are
available from this database.


snip...

DISCUSSION

In this death certificate based case-control study,
we observed a statistically significant association of
CJD with work as a butcher and with employment
in the office of a physician, occupation and indus-
try categories for which previous literature reports
suggested potential exposure to a TSE agent.
However, other occupations and industries for
which the same hypothesis was raised, such as
work on a livestock farm, were unassociated with
CJD. The positive associations with the occupation


CJD AND OCCUPATION

of butcher and employment in physician's offices
cannot be conclusively interpreted because of the
small numbers and lack of information on the type
and extent of exposure to potentially infectious
material. Also, other generally smaller studies of
CJD in Europe have not found specific associa-
tions with livestock farming or other specific occu-
pations, including health care workers (1,42).


snip...

An important limitation in our study is that it
was based on the one occupation and industry
combination on the death certificate of study sub-
jects, and no further details, such as duration of
employment, were available. ...snip...end


see full text ;


http://www.vegsource.com/talk/madcow/messages/91447.html

THE LAST PARAGRAPH SAYS IT ALL, just imagine what would happen
IF CJD was reportable Nationally and Internationally, can you just imagine
what
they would find. maybe thats why it is not reportable nationally and
internationally ;

JOURNAL OF NEUROLOGY

MARCH 26, 2003


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


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

IN the reply from Dr. Maddox to Terry S. Singeltary Sr., he states;

snip...

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)...

snip...

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

THERE seems to be some difference of opinion;

Mouse model sheds new light on human prion disease

snip...

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.

snip...

http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-
news_archive/public-news-archive_nov_dec_02/public- bse_and_sporadic_cjd.htm


ALSO, Dr. Maddox states;

make routine mortality surveillance a useful surrogate for ongoing CJD
surveillance...

THIS has proven not very useful in the U.K.;

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically
qualified and it is not surprising that coding errors occur in the
processing of large numbers of certificates. In 1982, 12,000 certificates
per week were processed at the office of population censuses and surveys bu
15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both
inter- and intra-observer coding errors has been described (Curb et al.,
1983) and the _inaccuracies_ of BOTH certification and coding discovered in
this study _support_ the introduction of a more accurate system of death
certificates and a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf


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.

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf


DR. Maddox states here;

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.

HOWEVER in a recent article in the UPI out of Washington;

CJD screening may miss thousands of cases

By Steve Mitchell UPI Medical Correspondent Published 7/21/2003 3:00 PM

snip...

In addition, the NPDPSC sees less than half of all the CJD cases each year,
so the CDC's investigational system not only is missing many of the
misdiagnosed CJD cases, it also is not conducting autopsies on most of the
detected cases.

snip...

http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r


ALSO in Philip Yams book 'The Pathological Protein';

''Answering critics like Terry Singeltary, who feels that the US undercounts
CJD, Schonberger _conceded_ that the current surveillance system has errors
but stated that most of the errors will be confined to the older
population''....

http://www.thepathologicalprotein.com/


THERE has been a _documented_ case of nv/v CJD in a 74 year old, so the
errors Schonberger speaks of (above) would be of significant importance, if
one believes in the nv/v CJD 'only' theory.

NOW we have _documented_ cases of very young CJD victims in the USA
continuing to appear in several different states.

HOW does Dr. Maddox explain this, and does he still believe that a National
CJD surveillance program with a CJD questionnaire to every victims family is
still not warranted?

WITH CWD and Scrapie running rampant in the USA, with BSE now _documented_
in North America, with the feeding of ruminant-to-ruminant animal protein
still happening in the USA in 2003 even though there has been a partial
voluntary ban on ruminant feeding since 8/4/97, with only 48,000 BSE/TSE
tests done on USA cattle in some 14 years of surveillance, when in any given
year there are 100 million cattle in the USA, with all this, i think
refusing to make CJD/TSEs reportable Nationally in the USA is not ONLY a
grave mistake, but in my opinion, should be looked at with great
suspicion...

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

http://www.vegsource.com/talk/madcow/messages/1072.html

> The clinical phenotype of patients with dura mater-associated CJD is
similar
> to that seen in the classical sporadic form of CJD: rapidly progressive
> dementia, myoclonus, and in the majority of patients a characteristic EEG.
> However, there are some differences: dura-associated cases tend to have
more
> prominent early cerebellar symptoms and a somewhat more prolonged clinical
> course. In sporadic CJD the median illness duration is 4 1/2 months and
this
> is doubled on average in dura mater cases. The age at onset is about 10-15
> years younger on average than we see with sporadic CJD.
>
> I think the youngest dura mater case documented was 16 or 17. The average
> incubation period from the time the patient received their graft to onset
of
> their illness is approximately 6 years, but ranges from 16 months to 16
> years. Although most of these cases have arisen through the use of dura
> mater for cranial surgery, there are some cases which have been known to
> have resulted from ear, nose and throat surgery or from spinal surgery.
Two
> cases from France, as mentioned, followed embolism procedures, in one case
> the patient had a nasopharyngeal tumor and dura mater was cut up into lots
> of little pieces and injected into the external carotid artery to embolise
> this, and in the other case the dura mater was inoculated into an artery
in
> the chest to embolise an area of infection.
>
> I would like to just go back and show you the countries which are known to
> have had dura mater cases of CJD. Most of the reports come from Japan, and
> we were rather surprised at the WHO consultation last year in Geneva, to
> hear reports from Dr Tateishi of a recent study conducted in Japan which
had
> shown the presence of 43 cases of dura mater CJD.
>
> I think you will agree looking at the slide that the other cases have been
> reported really quite widely throughout the world. Virtually all of these
> patients received one particular form of dura mater graft that was
> commercially manufactured by a single German company. The product was
called
> Lyodura, and most of the patients had received grafts that had been
> manufactured during a 4 year period between 1983 and 1987. Lyodura was
> pooled during this time, so there was a potential for cross contamination
> and the sterilization procedures used involved 10% hydrogen peroxide for
24
> hours and ionizing radiation. Subsequent animal experiments have shown
that
> this is not an adequate form of sterilization.
>
> An important question is whether any of the dura mater cases were
recipients
> of grafts that were treated with more thorough and adequate sterilisation.
> By this, I mean treatment with 1N sodium hydroxide, which is in the
standard
> step which was introduced in the treatment of Lyodura after 1987. There
are
> four cases out of this series of 83 which perhaps I'll talk about in a
> little bit more detail. Two cases were clearly not Lyodura. These were
> locally procured grafts, one from Italy and the other from the United
> Kingdom - these were used between 1969 and 1981.
>
> Furthermore, there was the a recently reported case from America which we
> heard about yesterday. Perhaps the case of most interest is one from the
> Japanese series. This was a lady in her mid-60s who received a graft in
1991
> and later developed clinically probable CJD, but this was not
histologically
> confirmed. The hospital records did not note whether or not her graft was
> Lyodura or the other form of dura used in that hospital at that time. It
was
> concluded in the report of the Japanese cases that it was unlikely that
this
> patient had received Lyodura produced before 1987.
>
> So the possibility exists that this patient had received a form of dura
> which was considered to be adequately sterilised. It is important to note
> two points, first, as this case did not undergo histological examination
the
> diagnosis of CJD is not 100% certain, and second, we can not be absolutely
> sure that in this or some of the of other 82 cases that the history of
> receiving a dural mater graft is coincidental. In none of these cases is
> there data which tells if the graft donor had CJD.
>
> Following the announcement of the first case here in the United States,
> doctors in the United Kingdom, Australia and New Zealand decided that they
> were going to use alternatives to cadaveric dura homografts, and here in
the
> States I believe there was an importation ban on Lyodura.
>
> So what alternatives are there to cadaveric-derived dura? There are
> several - I'll just run through these. One of the most popular is fascia
> lata, this is the fibrous covering of the lateral thigh muscle. The
removal
> of this can add about 30 minutes to the length of the operation and of
> course leaves a wound which, as with all wounds, can potentially become
> infected or have other complications.
>
> Other alternatives include pericranium (the covering of the skull bone),
> temporalis fascia (the membrane which surrounds the temporalis muscle at
the
> side of the head) and synthetic materials - a number of such materials
have
> been tried over the years including gold foils, cellophane, and dacron
> grafts. However, there has been some concern about the safety of synthetic
> materials and neurosurgeons have felt that these were rather inferior,
> although I think with the newer materials that's not so clearly the case.
I
> should note that there is no controlled trial that has ever been conducted
> to answer the question as to whether or not these substitutes are better
or
> worse than cadaveric-derived dura.
>
> I think there are two key questions that need to be addressed, first, are
> there situations where cadaveric dura is better than available
alternatives?
> If the answer is no then we need to question why we are using cadaveric
> dural grafts at all. If the answer is yes, then the next question is how
can
> dura be made as safe as possible? I'd like to show you some of the report
> from the WHO meeting over a year ago. I'll read it to you.
>
> "Because over 50 cases of CJD have resulted from cadaveric dura mater
> grafts, the group strongly recommended that dura mater no longer be used,
> especially in the case of neurosurgery, unless no alternative is
available.
> If dura mater is to be used, only material which is from non-pooled
sources
> originating from carefully screened donors subjected to validated
> inactivated treatment should be considered."
>
> Following this recommendation the Japanese authorities decided that they
> were no longer going to issue a license for the use of dura mater and the
> TSE Advisory Committee here in the States met again to discuss the issue
of
> dura mater. I just want to run through their recommendations, there were
> some differences from WHO's: although they also discouraged use of dura
> mater, the final decision on its usage was left up to the individual
> physicians, but certain additional safeguards were put into place.
>
> For instance, it was felt mandatory that for every donor a full brain
> autopsy should be performed and examined histologically and with
> immunocytochemistry, which is probably the most sensitive method that we
> have, other than transmission studies. It was further recommended that a
> sample of the dura and the brain should be kept for further testing as
> needed.
>
> Additionally, standard protocols for determining donors eligibility and
> tissue procurement were recommended, and dura should be collected before
the
> brain at autopsy - which obviously makes sense to avoid contamination of
the
> graft. Furthermore, decontamination with 1N sodium hydroxide for one hour
> should be used. This had previously been confirmed by Paul Brown and
> colleagues to be an effective decontamination procedure. There should be
no
> pooling of grafts, to prevent cross-contamination and there should be
> documentation to allow tracking from the donor to the recipient and from
the
> recipients to the donor. I think there can be little doubt that if these
> recommendations are adopted, then the safety of dura mater grafts will be
> dramatically improved.
>
> However, I would like to just play the devil's advocate here and to
mention
> a few cautions. We know from animal experiments that infectivity can
predate
> any pathological changes and this includes immunocytological changes as
> well. We also know that standard decontamination procedures using sodium
> hydroxide, as David Taylor mentioned yesterday, may not completely be
> effective. I think we have to remember that dura is a potentially
high-risk
> material, and that studies also performed by David Taylor have shown that
> dura mater can have 106 ID50 per gram. Perhaps through the use of current
> decontamination procedures we will produce grafts which are much safer
than
> those previously used, with but with low-level residual infectivity which
> may lead to disease with a potentially long incubation period. (For TSE
> agents it is known that dose administered is inversely proportional to
> incubation period)...
>
>
> http://www.life.umd.edu/jifsan/tse/brown.htm


Subject: Re: Transmission and adaptation of chronic wasting disease to
hamsters and transgenic mice: evidence for strains
Date: April 1, 2007 at 7:30 pm PST

>>>Nonetheless, the rodent-adapted CWD models we have developed may be
useful to experimentally analyze TSE species and strain differences. Despite
the low initial attack rate on the first passage of CWD into Sg hamsters,
CWD derived initially from elk and mule deer readily adapted to hamsters as
evidenced by the 100% infection rate on second and third passages. The
average incubation periods were similar for the second and third passages,
but considerably shorter than the first passage in the Sg hamsters,
suggesting that any species barrier to infection (formally, the shortening
of incubation period between the first and subsequent passages in a new
species) was overcome quickly.<<<

very disturbing considering the potential for iatrogenic CJD via silent
human CWD carriers. ...TSS


Chronic Wasting Disease and Potential Transmission to Humans
Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W.
Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*
*Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
†University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of
Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University,
Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES,
Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential
transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun
[date cited]. Available from:
http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm


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

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner
area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been
detected in other parts of the United States. Although detection in some
areas may be related to increased surveillance, introduction of CWD due to
translocation or natural migration of animals may account for some new foci
of infection. Increasing spread of CWD has raised concerns about the
potential for increasing human exposure to the CWD agent. The foodborne
transmission of bovine spongiform encephalopathy to humans indicates that
the species barrier may not completely protect humans from animal prion
diseases. Conversion of human prion protein by CWD-associated prions has
been demonstrated in an in vitro cell-free experiment, but limited
investigations have not identified strong evidence for CWD transmission to
humans. More epidemiologic and laboratory studies are needed to monitor the
possibility of such transmissions.


snip...

Conclusions

The lack of evidence of a link between CWD transmission and unusual cases of
CJD, despite several epidemiologic investigations, and the absence of an
increase in CJD incidence in Colorado and Wyoming suggest that the risk, if
any, of transmission of CWD to humans is low. Although the in vitro studies
indicating inefficient conversion of human prion protein by CWD-associated
prions raise the possibility of low-level transmission of CWD to humans, no
human cases of prion disease with strong evidence of a link with CWD have
been identified. However, the transmission of BSE to humans and the
resulting vCJD indicate that, provided sufficient exposure, the species
barrier may not completely protect humans from animal prion diseases.
Because CWD has occurred in a limited geographic area for decades, an
adequate number of people may not have been exposed to the CWD agent to
result in a clinically recognizable human disease. The level and frequency
of human exposure to the CWD agent may increase with the spread of CWD in
the United States. Because the number of studies seeking evidence for CWD
transmission to humans is limited, more epidemiologic and laboratory studies
should be conducted to monitor the possibility of such transmissions.
Studies involving transgenic mice expressing human and cervid prion protein
are in progress to further assess the potential for the CWD agent to cause
human disease. Epidemiologic studies have also been initiated to identify
human cases of prion disease among persons with an increased risk for
exposure to potentially CWD-infected deer or elk meat (47). If such cases
are identified, laboratory data showing similarities of the etiologic agent
to that of the CWD agent would strengthen the conclusion for a causal link.
Surveillance for human prion diseases, particularly in areas where CWD has
been detected, remains important to effectively monitor the possible
transmission of CWD to humans. Because of the long incubation period
associated with prion diseases, convincing negative results from
epidemiologic and experimental laboratory studies would likely require years
of follow-up. In the meantime, to minimize the risk for exposure to the CWD
agent, hunters should consult with their state wildlife agencies to identify
areas where CWD occurs and continue to follow advice provided by public
health and wildlife agencies. Hunters should avoid eating meat from deer and
elk that look sick or test positive for CWD. They should wear gloves when
field-dressing carcasses, bone-out the meat from the animal, and minimize
handling of brain and spinal cord tissues. As a precaution, hunters should
avoid eating deer and elk tissues known to harbor the CWD agent (e.g.,
brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD
has been identified.


http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"
To:
Cc: "Race, Richard (NIH)" ; ; "Belay,
Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was
attached to your email), we did not say CWD in humans will present like
variant CJD.

That assumption would be wrong. I encourage you to read the whole
article and call me if you have questions or need more clarification
(phone: 404-639-3091). Also, we do not claim that "no-one has ever been
infected with prion disease from eating venison." Our conclusion stating
that we found no strong evidence of CWD transmission to humans in the
article you quoted or in any other forum is limited to the patients we
investigated.

Ermias Belay, M.D.
Centers for Disease Control and Prevention


> > -----Original Message-----
> > From:
> > Sent: Sunday, September 29, 2002 10:15 AM
> > To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
> > Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG
> > HUNTERS


Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS


also,


A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most
serious because of rapid horizontal spread and higher prevalence than BSE in
UK, up to 15% in some populations. Also may be a risk to humans - evidence
that it is not dangerous to humans is thin.


http://www.tseandfoodsafety.org/activities/bse_conference_basel_april_02/2summary_of_conference.htm


JOURNAL OURNAL OF VIROLOGY IROLOGY, Nov. 2005, p. 13794–13796 Vol. 79, No.
21
0022-538X/05/$08.00 !0 doi:10.1128/JVI.79.21.13794–13796.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

NOTES

Interspecies Transmission of Chronic Wasting Disease Prions to
Squirrel Monkeys (Saimiri sciureus sciureus)

Richard F. Marsh, 1† Anthony nthony E. Kincaid, 2 Richard A. Bessen, 3 and
Jason C. Bartz Bartz4*
Department of Animal Health and Biomedical Sciences, University of
Wisconsin, Madison 53706 537061; Department of
Physical Therapy Therapy2 and Department of Medical Microbiology and
Immunology, 4 Creighton University, Omaha,
Nebraska 68178; and Department of Veterinary Molecular Biology, Montana
State University, Bozeman, Montana 59718 597183
Received 3 May 2005/Accepted 10 August 2005

Chronic wasting disease (CWD) is an emerging prion disease of deer and elk.
The risk of CWD transmission
to humans following exposure to CWD-infected tissues is unknown. To assess
the susceptibility of nonhuman
primates to CWD, two squirrel monkeys were inoculated with brain tissue from
a CWD-infected mule deer. The
CWD-inoculated squirrel monkeys developed a progressive neurodegenerative
disease and were euthanized at
31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel
monkeys contained the abnormal
isoform of the prion protein, PrP-res, and displayed spongiform
degeneration. This is the first reported
transmission of CWD to primates. ...snip...end


Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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


http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535

CJD QUESTIONNAIRE

http://www.vegsource.com/talk/madcow/messages/1000981.html

2007 USA, we now have documented not only BSE mad cow, but also BASE mad cow
, of which is more virulent to humans and BSE, of which is also tied to
sporadic CJD. There is much science now pointing to more than one strain of
CWD in deer and elk, of which, Despite the low initial attack rate on the
first passage of CWD into Sg hamsters, CWD derived initially from elk and
mule deer readily adapted to hamsters as evidenced by the 100% infection
rate on second and third passages, a very disturbing factor considering
iatrogenic CJD. this BSe that scrapie does not transmit to humans is another
false myth. there is more science pointing to just the opposite, that in
fact scrapie is just as capable of transmitting to humans as is BSE, and
with the very likelyhood that BSE has been in sheep/goat for some time, and
the fact of atypical scrapie, i.e. NOR98 of which was just documented in the
USA, to say there is no risk to humans from sheep or goat is simply a false
myth. AS well with blood issue and TSE, the fact that for years it could not
happen, would not happen, and now it has. They have floundered to long with
this. ...TSS


OTHER THINGS TO PONDER


Subject: CJD SURGICAL INCIDENT REPORTING FORM UPDATE NOVEMBER 2006
Date: November 18, 2006 at 9:05 am PST
CJD SURGICAL INCIDENT REPORTING FORM UPDATE NOVEMBER 2006

snip...


7. Which tissues were involved?

Please tick Please circle or underline where appropriate
Any notes/details?


Brain

CNS / Spinal cord


Posterior eye / anterior eye / cornea


Olfactory epithelium


Tonsil / appendix / spleen / thymus


Other lymphoid tissue


Peripheral nerve


Dental pulp / gingival tissue / Blood / bone marrow / CSF / Placenta / Urine


Other or not know (please give available details):

8. How many instruments were used, and what were they?

(Please continue overleaf or attach details if necessary)


9. What type of decontamination procedures are used for these instruments?


10. How many times have the instruments been used and decontaminated since
this procedure?


10. Can you trace the instruments?

(e.g. all / some / none / disposable / don’t know)


11. Where are the instruments now?*

(e.g. all are quarantined / some are quarantined / none are quarantined /
not applicable/ don’t know)


12. How many people might have been exposed to the instruments (or pool of
instruments)?


13. Other comments (Please continue overleaf or attach if necessary)


snip.....


http://www.hpa.org.uk/infections/topics_az/cjd/SI_formNov06.doc


Subject: CJD: update for dental staff
Date: November 12, 2006 at 3:25 pm PST
1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

CJD: update for dental staff.

Scully C,
Smith AJ,
Bagg J.
Eastman Dental Institute, University of London.

It is almost a decade since the recognition of the emergence of a new
infectious disease termed variant Creutzfeldt-Jakob disease (vCJD) caused by
prions (PrPTSE), abnormal variants of a normal human cell surface protein
(PrP).This disease has a number of similarities to other forms of
CJD--lethal disorders characterized by a prolonged incubation period, and
progressive mental deterioration. In relation to oral tissues, PrPTSE have
been found in neural, gingival, pulpal, lingual, lymphoreticular and
salivary gland tissue in animal models. In both sporadic and variant CJD,
PrPTSE is detectable in the trigeminal ganglion and, in vCJD, in
lymphoreticular tissues, but infectivity has not been tested in other human
oral tissues. CLINICAL RELEVANCE: PrPTSE is much more resistant to the
common methods of inactivation than conventional pathogens, and it adheres
avidly to steel whilst retaining its infectivity. Particular attention must
be paid to cleaning and sterilizing re-usable dental instruments. Single-use
devices, such as endodontic files and matrix bands, must never be re-used.
Advice on the reprocessing of dental instruments used on known CJD patients
must be obtained from local infection control teams. Research into effective
methods of prion inactivation appears promising, although further work on
the applicability to general dental practice is required.

PMID: 17087448 [PubMed - in process]


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17087448&query_hl=1&itool=pubmed_docsum


Sent: Thursday, December 28, 2006 10:23 AM
Subject: Ophthalmic Surgery in Prion Diseases

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0612&L=sanet-mg&D=0&P=24255

Evidence For CJD/TSE
Transmission Via Endoscopes
From Terry S. Singletary, Sr
flounder@wt.net


1-24-3


http://www.rense.com/general34/scopes.htm

http://www.gutjnl.com/cgi/content/abstract/50/6/888

http://www.microbes.info/forums/index.php?showtopic=378

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

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

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

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

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

PMID: 8006664 [PubMed - indexed for MEDLINE]


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

Possible occupational risks from TSEs


Although CJD has been documented in a neurosurgeon, two neuropathology
technicians, an

orthopaedic surgeon and a pathologist, it is reassuring to note that in none
of these individuals

was there a history of a definite infective event. The orthopaedic surgeon
had however worked

with human and ovine dura mater 20 years prior to his illness. Case-control
studies do not

suggest that individuals potentially exposed to the TSE agent in the health
care setting are at an

increased risk of developing CJD. However, the possibility that cases of CJD
have rarely

occurred in such circumstances cannot be confidently dismissed.


snip...


If CJD were transmitted in pooled blood products or saliva, clusters would
be detected. Most

clusters have usually been attributed to familial disease (Masters, 1979 &
Reingold 1996).

Surveillance systems have found cases of CJD among persons who have received
blood

transfusions but none have been linked to blood transmission.

It must be remembered, however, that surveillance systems may not detect
cases when unique

epidemiological or clinical features are present.

SECTION 8: CONCLUSIONS

A number of factors must be taken into consideration when assessing the risk
of transmission of

nvCJD during dental treatment. Dental patients are at risk of infection from
a number of sources,

the most significant being the consumption of infected animal products
during the 1980s. A

small minority of patients will also be at increased risk in their place of
work, such as

neuropathologists, neurosurgeons and laboratory technicians.

The most likely route of infection is via ineffectively sterilised
instruments. If a patient is

suspected of having or has been diagnosed with nv-CJD further precautions
can be taken. The

patient is likely to be showing clinical signs, which are likely to make
dental treatment difficult,

therefore only emergency treatment is going to be appropriate. Where
possible, a treatment

option that involves the least cross infection risk should be undertaken. .
To reduce this risk, all

instruments that have been used on a patient with nvCJD should be disposable
or discarded. This

includes oral surgery equipment, root planing hand instruments and
ultrasonic tips in addition to

needles and blades. In the case of accidental inoculation it is unlikely
that sufficient infective

material will be involved to transmit the disease. As discussed previously,
the peripheral route of

infection is ineffective compared with intracerebral inoculation, and blood
or saliva contains

little infectivity compared to central nervous tissue. All patients should
be treated using universal

precautions, which should be employed in all dental practices as a matter of
routine, providing

the maximum protection equally to clinical staff and patients. This includes
the use of gloves,

masks and eye protection at all times. After each patient all instruments
should be autoclaved and

disposable alternatives should be used where appropriate.

The number of patients likely to be incubating nv-CJD is impossible to
predict at present. Much

depends on the average incubation time, the longer the time, the higher the
figure is likely to be.

At present the average incubation time can not be calculated nor is it
possible to estimate the

dose required to infect a human. With the possibility of a nationwide
epidemic investigation

must be carried out to determine the risk from cross infection. Research has
yet to prove that

there is a risk, however, until all possibility of this can be discounted
caution must prevail.

The resistance of the TSE agent to standard medical sterilisation procedures
is noteworthy.

Experimental evidence demonstrates that the agent shows resistance to the
following: exposure

to boiling, freezing, ethanol, H2O2, permanganate, iodine, ethylene oxide
vapour, detergents,

organic solvents, formaldehyde, UV and gamma irradiation, and standard
autoclaving.

Since conventional methods of sterilisation and disinfection do not
decontaminate the CJD

infectious agent, specific measures must be used, however, many of these are
impractical in the

dental practice.

Although the TSE agent is known to be infectious it is not contagious in the
usual sense.

Individuals exposed to patients with CJD: their spouses, nurses and doctors,
do not appear to

have an increased risk of developing the disease. Furthermore, professionals
who might be

considered 'high risk' in relation to exposure to TSE agents: e.g.
pathologists, neurosurgeons,

butchers etc. also do not appear to be at an increased risk of developing
CJD. No proven instance

of CJD contracted occupationally has yet been identified. However, over 170
cases of iatrogenic

CJD contracted through inoculation of contaminated CNS tissue or corneal
transplantation serve

to remind those of us involved in the management of all CJD patients of the
importance of safety

procedures in relation to the TSE agents.

REFERENCES


snip...


http://www.bseinquiry.gov.uk/files/ws/s201a.pdf


Subject: MASTER DENTIST FALLS VICTIM TO CJD
Date: March 31, 2007 at 1:27 pm PST

'In the hands of God'
Thursday, March 29, 2007
By CRYSTAL HARMON
TIMES WRITER
Dr. Gregory J. Bever, 54, died Tuesday morning, at his home on Linwood
Beach, surrounded by his family. His death was the result of a rare
neurological malady called Creutzfeldt-Jakob disease. It struck suddenly and
progressed rapidly. ...snip...end

http://www.mlive.com/news/bctimes/index.ssf?/base/news-9/1175181333132150.xml&coll=4


Subject: SEAC Position statement vCJD and Endodontic dentistry
Date: May 8, 2006 at 6:45 am PST
CJD WATCH MESSAGE BOARD
TSS 2005
SEAC Position statement vCJD and Endodontic dentistry
Mon May 8, 2006 09:08
68.238.108.206


SEAC
Position Statement

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

Position statement vCJD and Endodontic dentistry
Issue

1. The Department of Health (DH) asked SEAC to advise on the findings and
implications of a preliminary risk assessment of potential vCJD transmission
via endodontic procedures (dental procedures involved in the maintenance of
dental pulp and the treatment of the pulp cavity) 1. This is particularly
pertinent because of the large number of endodontic procedures undertaken in
the UK.

Background ...snip...end


Page updated: 8th May 2006


http://www.seac.gov.uk/statements/statement0506.htm


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle

03-025IFA
03-025IFA-2


http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

THE SEVEN SCIENTIST REPORT ***


http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf

4th CASE VCJD VIA BLOOD TRANSFUSION, BSE, BASE, AND SPORADIC CJD

By Terry S Singeltary

Bacliff, Texas USA Jan 24, 07


http://bloodindex.org/view_news_zone.php?id=206

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

----- Original Message -----
From: Terry S. Singeltary Sr.
To: Terry S. Singeltary Sr. ; [log in to unmask]
Cc: [log in to unmask] ; [log in to unmask]
Sent: Thursday, November 30, 2006 1:47 PM
Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS
SUBMISSION PART II]


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0611&L=sanet-mg&T=0&P=16159

TSS

----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Tuesday, March 13, 2007 12:03 PM
Subject: Re: Colorado Surveillance Program for Chronic Wasting Disease
Transmission to Humans (TWO SUSPECT CASES)


> Subject: Re: Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES)
> Date: March 13, 2007 at 8:28 am PST
>
> In Reply to: Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES) posted by TSS on March 13, 2007
> at 8:15 am:
>
> Greetings,
>
> SOMETHING ELSE TO PONDER HERE, lets go back and look and the other
potential
> cases of CWD transmission to humans and where it was explained away as
> genetic too i.e. GSS ;
>
>
> In 2001, the case of a 25-year-old man who reportedly died of a prion
> disease after an illness lasting ˜22 months was investigated (Table 2).
> Although this man had hunted deer only rarely, his grandfather hunted deer
> and elk throughout much of the 1980s and 1990s and regularly shared the
> venison with the case-patient's family. The grandfather primarily hunted
in
> southeastern Wyoming, around the known CWD-endemic area. The
case-patient's
> illness began with a seizure and progressed to fatigue, poor
concentration,
> and depression. Memory loss, ataxia, speech abnormalities, combative
> behavior, and recurrent seizures also developed. Histopathologic,
> immunohistochemical, and Western blot testing of brain autopsy samples
> confirmed a prion disease diagnosis. Analysis of the prion protein gene
> indicated a P102L mutation coupled with valine at the polymorphic codon
129
> in the mutant allele, confirming a diagnosis of
> Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was
> unusually young even for a person with a GSS P102L mutation. It remains
> unknown whether the possible exposure of the case-patient to CWD-infected
> venison potentially contributed to the early onset of his prion disease.
>
>
> http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
>
> Table 2. Creutzfeldt-Jakob disease patients investigated for a possible
> causal link of their illness with chronic wasting disease of deer and elk,
> United Statesa
>
> LOOK AT 1 AND 3B BOTH GSS. ...TSS
>
>
> http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm#table2
>
>
> just another strange coincidence i suppose, or maybe not. ...TSS
>
> p.s. i apologize for the double posting again. ...terry
>
>
> ----- Original Message -----
> From: "Terry S. Singeltary Sr."
> To:
> Sent: Tuesday, March 13, 2007 10:34 AM
> Subject: Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES)
>
>
> Subject: Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES)
> Date: March 13, 2007 at 8:15 am PST
> Colorado Surveillance Program for Chronic Wasting Disease Transmission to
> Humans
>
> Lessons From 2 Highly Suspicious but Negative Cases
>
> C. Alan Anderson, MD; Patrick Bosque, MD; Christopher M. Filley, MD; David
> B. Arciniegas, MD; B. K. Kleinschmidt-DeMasters, MD; W. John Pape, BS;
> Kenneth L. Tyler, MD
>
>
> Arch Neurol. 2007;64:439-441.
>
> Objective To describe 2 patients with rapidly progressive dementia and
risk
> factors for exposure to chronic wasting disease (CWD) in whom extensive
> testing negated the possible transmission of CWD.
>
> Design/Methods We describe the evaluation of 2 young adults with initial
> exposure histories and clinical presentations that suggested the
possibility
> of CWD transmission to humans.
>
> Patients A 52-year-old woman with possible laboratory exposure to CWD and
a
> 25-year-old man who had consumed meat from a CWD endemic area.
>
> Interventions Clinical evaluation, neuropathological examination, and
> genetic testing.
>
> Results Neuropathological and genetic assessment in the 2 patients proved
> the diagnoses of early-onset Alzheimer disease and a rare genetic prion
> disease.
>
> Conclusion No convincing cases of CWD transmission to humans have been
> detected in our surveillance program.
>
>
> Author Affiliations: Departments of Neurology (Drs Anderson, Bosque,
Filley,
> Arciniegas, Kleinschmidt-DeMasters, and Tyler), Psychiatry (Drs Anderson,
> Filley, and Arciniegas), Pathology (Dr Kleinschmidt-DeMasters), Medicine
(Dr
> Tyler), Microbiology (Dr Tyler), and Immunology (Dr Tyler); University of
> Colorado School of Medicine, Denver; Denver Veterans Affairs Medical
Center,
> Denver (Drs Anderson, Filley, Arciniegas, and Tyler); Denver Health
Medical
> Center, Denver (Dr Bosque); and Colorado Department of Public Health and
> Environment, Denver (Mr Pape).
>
>
> http://archneur.ama-assn.org/cgi/content/abstract/64/3/439?ct
>
>
>
>
> > Results Neuropathological and genetic assessment in the 2 patients
proved
> the
>
> > diagnoses of early-onset Alzheimer disease and a rare genetic prion
> disease
>
>
> very interesting, and something to ponder here for sure ;
>
>
>
>
> 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.
>
> snip...
>
>
>
> http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
>
>
>
>
> and i think this would apply to CWD to humans as well.
>
>
>
>
> > rare genetic prion disease
>
>
>
>
> would be interesting to know the exact genetic TSE they are speaking of.
> GSS, FFI, Familial/Genetic CJD, and or the sporadic FFI that is not
genetic,
> and don't ask me why ??? does not make sense to me either. it's either
> genetic or not. like i have said many times, the diagnostic criteria
> differentiating the different human and animal TSE is missing something.
but
> if you have a strain of genetic/familial TSE i.e. FFI, and then you
classify
> a sub-type of that strain that use to be gentic to sporadic, then you have
> either gone back to sCJD, or the complete damn diagnostic criteria is
wrong.
> you just have well named the damn thing ;
>
>
>
>
>
Parchi-Capellari-Chin-Schwarz-Schecter-Butts-Hudkins-Burns-Powers-Gambetti-D
> ISEASE.
>
> TSS
>
>
>
>
> Subject: Alzheimer-type neuropathology in a 28-year old patient with
> iatrogenic CJD after dural grafting
> Date: March 9, 2007 at 9:15 am PST
>
> HUMAN-04
>
> Alzheimer-type neuropathology in a 28-year old patient with iatrogenic
>
> Creutzfeldt-Jakob disease after dural grafting
>
> M Preusser1, T Stroebel1, E Gelpi1, 2, M Eiler3, G Broessner4, E
> Schmutzhard4, H Budka1, 2
>
> 1 Institute of Neurology, Medical University Vienna, Austria; 2 Austrian
> Reference Centre for Human Prion Diseases
>
> (OERPE), General Hospital Vienna, Austria; 3 Department of Neurology, LKH
> Rankweil, Austria; 4 Department of
>
> Neurology, Medical University Innsbruck, Austria
>
> We report the autopsy case of a 28-year old male patient who had received
a
> cadaverous dura
>
> mater graft after a traumatic open skull fracture with tearing of dura at
> the age of 5 years. A
>
> clinical suspicion of Creutzfeldt-Jakob disease (CJD) was confirmed by a
> brain biopsy 5 months
>
> prior to death and by autopsy, thus warranting the diagnosis of iatrogenic
> CJD (iCJD) according
>
> to WHO criteria. Immunohistochemistry showed widespread cortical
depositions
> of diseaseassociated
>
> prion protein (PrPsc) in a synaptic pattern and western blot analysis
> identified PrPsc of
>
> type 2A according to Parchi et al. Surprisingly, we found Alzheimer-type
> senile plaques and
>
> cerebral amyloid angiopathy in widespread areas of the brain. Plaque-type
> and vascular amyloid
>
> was immunohistochemically identified as deposits of beta-A4 peptide. CERAD
> criteria for
>
> diagnosis of definite Alzheimer´s disease (AD) were met in the absence of
> neurofibrillar tangles
>
> or alpha-synuclein immunoreactive inclusions. There was no family history
of
> AD, CJD, or any
>
> other neurological disease, and genetic analysis showed no
disease-specific
> mutations of the
>
> prion protein, presenilin 1 and 2, or amyloid precursor protein genes.
This
> case represents 1. the
>
> iCJD case with the longest incubation time after dural grafting reported
so
> far, 2. the youngest
>
> documented patient with concomitant CJD and Alzheimer-type neuropathology
to
> date, 3. the
>
> first description of Alzheimer type-changes in iCJD, and 4. the second
case
> of iCJD in Austria.
>
> Despite the young patient age, the Alzheimer-type changes may be an
> incidental finding, possibly
>
> related to the childhood trauma.
>
>
> 249 of 411 pages...tss
>
>
>
http://www.tse-forum.de/tse_forum/deutsch/oeffentlich/bilder/Abstract_BookFINAL_nov2.pdf
>
>
>
>
> some other things to ponder ;
>
>
>
>
> Alzheimer's and Transmissible Spongiform Encephalopathies
>
> http://neurotalk.psychcentral.com/showthread.php?t=13175
>
>
>
>
> ------------------------------------------------------------
> Comments sent via JAMA Feedback Page
> ------------------------------------------------------------
> NAME: Terry S. Singeltary Sr.
> E-MAIL: flounder9@verizon.net
> IP ADDRESS: 68.238.99.53
> HOSTNAME: pool-68-238-99-53.dfw.dsl-w.verizon.net
> PREVIOUS PAGE: http://jama.ama-assn.org/misc/authors.dtl
> BROWSER: Mozilla/5.0 (Windows; U; Win98; en-US; rv:1.0.2) Gecko/20030208
> Netscape/7.02
> PROMOTIONAL USE: (not answered)
> ------------------------------------------------------------
> COMMENTS:
> I wish to submit the following ;
>
>
> 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 of that, I even believe that
> physical and or blunt trauma may play a role of onset
> of clinical symptoms in some cases, but 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.
>
>
> My name is Terry S. Singeltary Sr. and I am no
> scientist, no doctor and have no PhDs, but have been
> independently researching human and animal TSEs since
> the death of my Mother to the Heidenhain Variant of
> Creutzfeldt Jakob Disease on December 14, 1997
> 'confirmed'. ...TSS
>
>
>
> Terry S. Singeltary Sr.
> P.O. Box 42
> Bacliff, Texas USA 77518
>
>
> SOURCES
>
>
> Full Text
> Diagnosis and Reporting of Creutzfeldt-Jakob Disease
> Singeltary, Sr et al.
> JAMA.2001; 285: 733-734
>
>
> http://jama.ama-assn.org/
>
>
> Coexistence of multiple PrPSc types in individuals with
>
> Creutzfeldt-Jakob disease
>
>
> Magdalini Polymenidou, Katharina Stoeck, Markus
> Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi
>
>
> Summary
>
>
> Background The molecular typing of sporadic
> Creutzfeldt-Jakob disease (CJD) is based on the size
> and glycoform
>
> ratio of protease-resistant prion protein (PrPSc), and
> on PRNP haplotype. On digestion with proteinase K, type
> 1 and
>
> type 2 PrPSc display unglycosylated core fragments of
> 21 kDa and 19 kDa, resulting from cleavage around amino
>
> acids 82 and 97, respectively.
>
> Methods We generated anti-PrP monoclonal antibodies to
> epitopes immediately preceding the differential proteinase
>
> K cleavage sites. These antibodies, which were
> designated POM2 and POM12, recognise type 1, but not
> type 2, PrPSc.
>
> Findings We studied 114 brain samples from 70 patients
> with sporadic CJD and three patients with variant CJD.
>
> Every patient classified as CJD type 2, and all variant
> CJD patients, showed POM2/POM12 reactivity in the
>
> cerebellum and other PrPSc-rich brain areas, with a
> typical PrPSc type 1 migration pattern.
>
> Interpretation The regular coexistence of multiple
> PrPSc types in patients with CJD casts doubts on the
> validity of
>
> electrophoretic PrPSc mobilities as surrogates for
> prion strains, and questions the rational basis of
> current CJD
>
> classifications.
>
>
> snip...
>
>
> The above results set the existing CJD classifications
>
> into debate and introduce interesting questions about
>
> human CJD types. For example, do human prion types
>
> exist in a dynamic equilibrium in the brains of affected
>
> individuals? Do they coexist in most or even all CJD
>
> cases? Is the biochemically identified PrPSc type simply
>
> the dominant type, and not the only PrPSc species?
>
>
> Published online October 31, 2005
>
>
> http://neurology.thelancet.com
>
>
> Detection of Type 1 Prion Protein in Variant
>
> Creutzfeldt-Jakob Disease
>
> Helen M. Yull,* Diane L. Ritchie,*
>
> Jan P.M. Langeveld,? Fred G. van Zijderveld,?
>
> Moira E. Bruce,? James W. Ironside,* and
>
> Mark W. Head*
>
> From the National CJD Surveillance Unit,* School of
> Molecular
>
> and Clinical Medicine, University of Edinburgh, Edinburgh,
>
> United Kingdom; Central Institute for Animal Disease
> Control
>
> (CIDC)-Lelystad, ? Lelystad, The Netherlands; Institute
> for Animal
>
> Health, Neuropathogenesis Unit, ? Edinburgh, United Kingdom
>
> Molecular typing of the abnormal form of the prion
>
> protein (PrPSc) has come to be regarded as a powerful
>
> tool in the investigation of the prion diseases. All
> evidence
>
> thus far presented indicates a single PrPSc molecular
>
> type in variant Creutzfeldt-Jakob disease (termed
>
> type 2B), presumably resulting from infection with a
>
> single strain of the agent (bovine spongiform
> encephalopathy).
>
> Here we show for the first time that the PrPSc
>
> that accumulates in the brain in variant Creutzfeldt-
>
> Jakob disease also contains a minority type 1 component.
>
> This minority type 1 PrPSc was found in all 21
>
> cases of variant Creutzfeldt-Jakob disease tested,
> irrespective
>
> of brain region examined, and was also
>
> present in the variant Creutzfeldt-Jakob disease tonsil.
>
> The quantitative balance between PrPSc types was maintained
>
> when variant Creutzfeldt-Jakob disease was
>
> transmitted to wild-type mice and was also found in
>
> bovine spongiform encephalopathy cattle brain, indicating
>
> that the agent rather than the host specifies their
>
> relative representation. These results indicate that PrPSc
>
> molecular typing is based on quantitative rather than
>
> qualitative phenomena and point to a complex relationship
>
> between prion protein biochemistry, disease phenotype
>
> and agent strain. (Am J Pathol 2006, 168:151-157;
>
> DOI: 10.2353/ajpath.2006.050766)
>
>
> snip...
>
>
> Discussion
>
> In the apparent absence of a foreign nucleic acid genome
>
> associated with the agents responsible for transmissible
>
> spongiform encephalopathies or prion diseases,
>
> efforts to provide a molecular definition of agent strain
>
> have focused on biochemical differences in the abnormal,
>
> disease-associated form of the prion protein, termed
>
> PrPSc. Differences in PrPSc conformation and glycosylation
>
> have been proposed to underlie disease phenotype
>
> and form the biochemical basis of agent strain. This
>
> proposal has found support in the observation that the
>
> major phenotypic subtypes of sCJD appear to correlate
>
> with the presence of either type 1 or type 2 PrPSc in
>
> combination with the presence of either methionine or
>
> valine at codon 129 of the prion protein gene.2 Similarly,
>
> the PrPSc type associated with vCJD correlates with the
>
> presence of type 2 PrPSc and is distinct from that found in
>
> sCJD because of a characteristically high occupancy of
>
> both N-linked glycosylation sites (type 2B).6,11 The
>
> means by which such conformational difference is detected
>
> is somewhat indirect; relying on the action of proteases,
>
> primarily proteinase K, to degrade the normal
>
> Figure 6. Type 1 PrPSc is a stable minority component
> of PrPSc from the vCJD
>
> brain. Western blot analysis of PrP in a sample of
> cerebral cortex from a
> case
>
> of vCJD during digestion with proteinase K is shown.
> Time points assayed
>
> are indicated in minutes (T0, 5, 10, 30, 60, 120, 180).
> Duplicate blots were
>
> probed with 3F4, which detects both type 1 and type 2
> PrPSc, and with 12B2,
>
> which detects type 1. The insert shows a shorter
> exposure of the same time
>
> course study from a separate experiment also probed
> with 3F4. Both blots
>
> included samples of cerebral cortex from a case of
> sporadic CJD MM1 (Type
>
> 1) and molecular weight markers (Markers) indicate
> weights in kd.
>
> Figure 7. A minority type 1-like PrPSc is found in vCJD
> tonsil, vCJD
> transmitted
>
> to mice and in BSE. Western blot analysis of PrPSc in a
> concentrated
>
> sample of tonsil from a case of vCJD (Tonsil), in a
> concentrated brain
> sample
>
> of a wild-type mouse (C57BL) infected with vCJD and in
> a sample of cattle
>
> BSE brain (BSE) is shown. Tissue extracts were digested
> with proteinase K.
>
> Duplicate blots were probed with either 3F4 or 6H4,
> both of which detect
>
> type 1 and type 2 PrPSc, and with 12B2, which detects
> type 1. The blots
>
> included samples of cerebral cortex from a case of
> sporadic CJD MM1 (Type
>
> 1) and molecular weight markers (Markers) indicate
> weights in kd.
>
> Type 1 PrPSc in Variant Creutzfeldt-Jakob Disease 155
>
> AJP January 2006, Vol. 168, No. 1
>
> cellular form of PrP and produce a protease-resistant
>
> core fragment of PrPSc that differs in the extent of its
>
> N-terminal truncation according to the original
>
> conformation.
>
> A complication has recently arisen with the finding that
>
> both type 1 and type 2 can co-exist in the brains of
>
> patients with sCJD.2,5-8 More recently this same phenomenon
>
> has been demonstrated in patients with iatrogenically
>
> acquired and familial forms of human prion disease.
>
> 9,10 The existence of this phenomenon is now
>
> beyond doubt but its prevalence and its biological
> significance
>
> remain a matter of debate.
>
> Conventional Western blot analysis using antibodies
>
> that detect type 1 and type 2 PrPSc has severe quantitative
>
> limitations for the co-detection of type 1 and type 2
>
> PrPSc in individual samples, suggesting that the prevalence
>
> of co-occurrence of the two types might be underestimated.
>
> We have sought to circumvent this problem by
>
> using an antibody that is type 1-specific and applied this
>
> to the sole remaining human prion disease where the
>
> phenomenon of mixed PrPSc types has not yet been
>
> shown, namely vCJD.
>
> These results show that even in vCJD where susceptible
>
> individuals have been infected supposedly by a
>
> single strain of agent, both PrPSc types co-exist: a
> situation
>
> reminiscent of that seen when similarly discriminant
>
> antibodies were used to analyze experimental BSE in
>
> sheep.14,17 In sporadic and familial CJD, individual
>
> brains can show a wide range of relative amounts of the
>
> two types in samples from different regions, but where
>
> brains have been thoroughly investigated a predominant
>
> type is usually evident.2,6,10 This differs from this
> report
>
> on vCJD, where type 1 is present in all samples
> investigated
>
> but always as a minor component that never
>
> reaches a level at which it is detectable without a type
>
> 1-specific antibody. It would appear that the relative
> balance
>
> between type 1 and type 2 is controlled within
>
> certain limits in the vCJD brain. A minority type-1-like
>
> band is also detected by 12B2 in vCJD tonsil, in BSE
>
> brain and in the brains of mice experimentally infected
>
> with vCJD, suggesting that this balance of types is agent,
>
> rather than host or tissue, specific. Interestingly the
> "glycoform
>
> signature" of the type 2 PrPSc found in vCJD (type
>
> 2B) is also seen in the type 1 PrPSc components, suggesting
>
> that it could legitimately be termed type 1B.
>
> PrPSc isotype analysis has proven to be extremely
>
> useful in the differential diagnosis of CJD and is
> likely to
>
> continue to have a major role in the investigation of human
>
> prion diseases. However, it is clear, on the basis of
>
> these findings, that molecular typing has quantitative
> limitations
>
> and that any mechanistic explanation of prion
>
> replication and the molecular basis of agent strain
> variation
>
> must accommodate the co-existence of multiple
>
> prion protein conformers. Whether or not the different
>
> conformers we describe here correlate in a simple and
>
> direct way with agent strain remains to be determined. In
>
> principle two interpretations present themselves: either
>
> the two conformers can be produced by a single strain of
>
> agent or vCJD (and, therefore, presumably BSE) results
>
> from a mixture of strains, one of which generally
> predominates.
>
> Evidence for the isolation in mice of more than one
>
> strain from individual isolates of BSE has been presented
>
> previously.18,19
>
> One practical consequence of our findings is that the
>
> correct interpretation of transmission studies will depend
>
> on a full examination of the balance of molecular types
>
> present in the inoculum used to transmit disease, in
> addition
>
> to a thorough analysis of the molecular types that
>
> arise in the recipients. Another consequence relates to
>
> the diagnostic certainty of relying on PrPSc molecular
>
> type alone when considering the possibility of BSE
> infection
>
> or secondary transmission in humans who have a
>
> genotype other than methionine at codon 129 of the
>
> PRNP gene. In this context it is interesting to note
> that this
>
> minority type 1B component resembles the type 5 PrPSc
>
> described previously to characterize vCJD transmission
>
> into certain humanized PRNP129VV transgenic mouse
>
> models.12,20 This apparently abrupt change in molecular
>
> phenotype might represent a selection process imposed
>
> by this particular transgenic mouse model. Irrespective of
>
> whether this proves to be the case, the results shown
>
> here point to further complexities in the relationship
> between
>
> the physico-chemical properties of the prion protein,
>
> human disease phenotype, and prion agent strain.
>
> Acknowledgments
>
>
> snip...
>
>
> Type 1 PrPSc in Variant Creutzfeldt-Jakob Disease 157
>
> AJP January 2006, Vol. 168, No. 1 ...TSS
>
>
>
http://ajp.amjpathol.org/cgi/content/abstract/168/1/151maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1136646133963_237&FIRSTINDEX=0&volume=168&issue=1&journalcode=amjpathol
>
>
> snip...end...TSS
>




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