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From: TSS ()
Subject: Search for CJD answers begins, Health officials investigate women's health records LET THE SPIN BEGIN
Date: August 7, 2005 at 9:59 am PST

Search for CJD answers begins
Health officials investigate women's health records
By Sandy Miller
Times-News writer

TWIN FALLS -- Since January, five women -- four in Twin Falls County and one in Minidoka County -- have been diagnosed with Creutzfeldt-Jakob disease, an extremely rare and rapidly progressing fatal neurodegenerative disease. Four of the women have died.

The search for answers has begun. State epidemiologist Dr. Christine Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, are looking through the women's medical records and documenting family histories, looking for anything these women might have had in common.

"We'll do a specific history with families to find out if there's anything in common in these individuals' employment or family history of food preferences, even though we don't believe it was food borne," Becker said. "We'll look at where they've lived and what they did to see if they had anything in common."

Becker said they'll compare their findings with studies that have been done in other areas.

CJD is a rapidly progressing fatal neurodegenerative disease. It is carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

Health officials believe that all of the women had sporadic form of classic CJD, and not the variant form that people get when they eat meat from a cow with bovine spongiform encephalopathy -- commonly known as mad cow disease. All of the women were between the ages of 60 and 83. The average age of people who've died from classic CJD is 68. The average age of people who've died from the variant form of CJD is 28, according to the CDC.

However, the unusually high number of cases has drawn attention from state health officials as well as the CDC. Normally, the disease infects just one person per 1 million people worldwide a year. In Idaho, there are about three cases of CJD annually, and in recent years, the United States has reported fewer than 300 cases of CJD a year, according to the CDC.

State and local officials are in charge of the investigation. The CDC is only serving in an advisory role from its offices in Atlanta. CDC officials have no plans to travel to Idaho at this time, said Christine Pearson, public affairs specialist with the CDC.

"We leave it up to them," Pearson said. "We've been in contact with the state and we're monitoring the situation."

All states have their own lists of reportable diseases. Here in Idaho, CJD is a reportable disease, as is HIV, hepatitis, whooping cough, syphilis, E. coli and tuberculosis, said Trish Heath, the hospital's infection control practitioner. Heath said if a case of CJD were discovered at the hospital, she would call Becker directly.

Brain tissue from at least one of the women has been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland to be studied.

"The length of the study depends on the samples received," said Carrie Harris, manager of the National Prion Disease Pathology Surveillance Center. "It can take two months. We do a variety of tests that would not only confirm CJD, but would tell us whether it was acquired eating infected meat."

The local investigation also will take time.

"It's going to be a very slow process," Becker said. "We might end up with a lot of information, but no association."

Finding out the exact cause of the CJD cases will be a challenge.

"The causes of these illnesses may not be identified," Becker said. "In 85 percent of cases of CJD, a specific cause will never be identified."

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com.

http://www.magicvalley.com/articles/2005/08/07/news_localstate/news_local_state.2.txt

Carrie Harris, manager of the National Prion Disease Pathology Surveillance Center states;

>>>"It can take two months. We do a variety of tests that would not only confirm CJD, but would tell us whether it was acquired eating infected meat."<<<

r i g h t.

this is interesting. seems they now have test that will differentiate between cwd meat, scrapie meat (all 20+ strains), BSE meat (nvCJD),
and or the atypical TSE that was devised at Mission Texas from Scrapie transmission to cattle, that any human consumed? all of which transmits to primates, with no transmission studies ever on man. i have not seen any of this big news in the medical journals, maybe i missed it...

it will all be sporadic. you can rubber stamp it...

IN light of Asante/Collinge et al findings that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To:
"'flounder@wt.net'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

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

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

____________________________________

snip...

full text ;

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

AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease

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

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

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

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

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

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

From: TSS ()
Subject: MAD COW USA STRAIN MISSION TEXAS and the infamous USA sporadic CJDs, something to ponder
Date: July 29, 2005 at 11:16 am PST

NEW STRAIN OF TSE USA CATTLE OR JUST INCOMPETENCE IN TESTING???

DR. CLIFFORD: "Basically the IHC test, besides looking at location of the brain stem you're also doing a staining technique to identify abnormal prion proteins. In this case they had some staining, but the staining did not match up with what they would typically see in a BSE case. It didn't have the normal distribution it would see within the samples. So basically that's why the request for doing additional testing, and that's why we're sending it to Weybridge as well."

DR. CLIFFORD: "There was some staining present. But it did not match a normal pattern, and we're taking through that to do additional tests in additional parts of the brain stem to try to see if we can find a normal staining pattern as well as sending that sample to Weybridge to run against their IHC."

http://www.usda.gov/wps/portal/usdahome?contentidonly=true&contentid=2005/07/0280.xml

IN CONFIDENCE

PERCEPTION OF UNCONVENTENTIONAL SLOW VIRUS DISEASES OF ANIMALS IN THE USA

1985 The Stetsonville outbreak (farmer's name: Brecke). In addition to the downer cows and horses Brecke's mink recieved a cereal supplement. Hartsough's view was that this would contain bone meal and would be from a commercial source. If this were so and it was contaminated with a TME agent why were no other ranches affected?

Many mink ranches now feed a commerical pelleted diet. Brecke was equipped to process LARGE CARCASSES USING A CRUSHER/MIXER WHICH COULD ACCOMMODATE A WHOLE COW!

snip...

Dead mink go for rendering but are used only in poultry feed.

A commercial mink ranch was visited. This was Johny Werth's, Capitol Fur Farm comprising 1400 breeding females. The feed is bought in from a commercial supplier in the form of frozen packs of ''poultry'', ''fish'', ''dried egg'' or ''tripe''. A commercial mink cereal supplement is used and contains ''animal meat meal'' which was said to contain material mainly from poultry or fish origin but OCCASIONALLY FROM BEEF SOURCES. the partially thawed packs were tipped into an augur mixer which has a fully loaded capacity of 6000lb and this would be approximately 15000 mink per day.

In the fall at pelting time the skinned carcasses of the mink are placed in large barrels which are left in the open to freeze. When full, a renderer collects ''for use in poultry feeds''.

Sections from the brains of the two Brecke TME inoculated cattle were examined and Marsh provided all the blocks from the 2nd steer for study at CVL and comparison with BSE. In general the vacuolar changes were more severe than in most cases of BSE but very similar in distribution. Unfortunately material aken fro histopathology from those anials omitted representaion of most of the brain stem. ...........

Wilbur Clarke (reference the Mission, Texas scrapie transmission transmission to cattle study) is now the State Veterinarian for Montana based at Helena.

I was given confidential access to sections from the Clarke scrapie-cattle transmission experiment. Details of the experimental design were as supplied previously by Dr. Wrathall (copy of relevant information appended). Only 3 animals (2 inoculated with 2nd pass Suffolk scrapie and 1 inoculated with Angora goat passaged scrapie) showed clinical signs. Clinical signs were characterised by weakness, ''a stilted hindlimb gait'', disorientation, ataxia and, terminally, lateral recumbency. The two cattle from which I examined material were inocluated at 8 months of age and developed signs 36 months pi (goat scrapie inoculum) and 49 months pi (one of the Suffolk scrapie inoculated) respectively. This latter animal was killed at 58 months of age and so the clinical duration was only 1 month. The neuropathology was somewhat different from BSE or the Stetsonville TME in cattle. Vacuolar changes were minimal, to the extent that detection REQUIRED CAREFUL SEARCHING. Conversely astrocyte hypertrophy was a widespread and prominent feature. The material requires DETAILED NEUROPATHOLOGICAL ASSESSMENT BUT WHETHER OR NOT THIS WILL BE DONE REMAINS A QUESTION.

Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases

{the following was written but with a single line marked through it ''first passage (by this route)}...TSS

resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest
incubation period in the ferret.

snip...

Appendix 3

VISIT TO USA - DR A E WRATHALL - INFO OH BSE AND SCRAPIE

1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has
successfully transmitted ovine and caprine scrapie to cattle. The
experimental results have not been published but there are plans to do
this. This work was initiated in 1978. A summary of it is:-

Expt A
6 Her x Jer calves born in 1978 were inoculated as follows with
a 2nd Suffolk scrapie passage:-

i/c 1ml; i/m, 5ml; s/c 5ml; oral 30ml.
1/6 went down after 48 months with a scrapie/BSE-like disease.

Expt B
6 Her or Jer or HxJ calves were inoculated with angora Goat
virus 2/6 went down similarly after 36 months.

Expt C
Mice inoculated from brains of calves/cattle in expts A • B
were resistant, only 1/20 going down with scrapie and this was the
reason given for not publishing.

Diagnosis in A, B, C was by histopath. No reports on SAT were given.

2. Dr Warren Foote indicated success so far in eliminating scrapie in
offspring from experimentally- (and naturally) infected sheep by ET.
He had found difficulty in obtaining embryos from naturally infected
sheep (cf SPA).

3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr A Thiermann showed the
picture in the "Independent" with cattle being incinerated and thought
this was a fanatical incident to be avoided in the US at all costs.
BSE was not reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increase
since 1978. In 1953 when the National Control Scheme was started
there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitary
fetus.

6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of the
national sheep scrapie programme survey indicated

17/33 wished to drop it

6/33 wished to develop it

8/33 had few sheep and were neutral

Information obtained from Dr Wrathall's notes of a meeting of the U.S.
Animal Health Association at Little Rock, Arkansas Nov. 1988.

33

end...TSS

>> Differences in tissue distribution could require new regulations
>> regarding specific risk material (SRM) removal.

snip...end

full text 33 PAGES ;

http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

It was, however, performed in the USA in 1979, when it was shown that cattle inoculated with the scrapie agent endemic in the flock of Suffolk sheep at the United States Department of Agriculture in Mission, Texas, developed a TSE quite unlike BSE. 32 The findings of the initial transmission, though not of the clinical or neurohistological examination, were communicated in October 1988 to Dr Watson, Director of the CVL, following a visit by Dr Wrathall, one of the project leaders in the Pathology Department of the CVL, to the United States Department of Agriculture. 33 The results were not published at this point, since the attempted transmission to mice from the experimental cow brain had been inconclusive. The results of the clinical and histological differences between scrapie-affected sheep and cattle were published in 1995. Similar studies in which cattle were inoculated intracerebrally with scrapie inocula derived from a number of scrapie-affected sheep of different breeds and from different States, were carried out at the US National Animal Disease Centre. 34 The results, published in 1994, showed that this source of scrapie agent, though pathogenic for cattle, did not produce the same clinical signs of brain lesions characteristic of BSE.

http://www.bseinquiry.gov.uk/

1: J Infect Dis. 1994 Apr;169(4):814-20.

Intracerebral transmission of scrapie to cattle.

Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM, Robinson MM.

USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA 50010.

To determine if sheep scrapie agent(s) in the United States would induce a disease in cattle resembling bovine spongiform encephalopathy, 18 newborn calves were inoculated intracerebrally with a pooled suspension of brain from 9 sheep with scrapie. Half of the calves were euthanatized 1 year after inoculation. All calves kept longer than 1 year became severely lethargic and demonstrated clinical signs of motor neuron dysfunction that were manifest as progressive stiffness, posterior paresis, general weakness, and permanent recumbency. The incubation period was 14-18 months, and the clinical course was 1-5 months. The brain from each calf was examined for lesions and for protease-resistant prion protein. Lesions were subtle, but a disease-specific isoform of the prion protein was present in the brain of all calves. Neither signs nor lesions were characteristic of those for bovine spongiform encephalopathy.

MeSH Terms:
Animals
Brain/microbiology*
Brain/pathology
Cattle
Cattle Diseases/etiology*
Cattle Diseases/pathology
Encephalopathy, Bovine Spongiform/etiology*
Encephalopathy, Bovine Spongiform/pathology
Immunoblotting/veterinary
Immunohistochemistry
Male
Motor Neurons/physiology
Prions/analysis
Scrapie/pathology
Scrapie/transmission*
Sheep
Sleep Stages
Time Factors

Substances:
Prions

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8133096&dopt=Citation

Intracerebral transmission of scrapie to cattle FULL TEXT PDF;

SNIP...

Discussion

WE conclude that American sources of sheep scrapie are transmissible to cattle by direct intracerebral inoculation but the disease induced is NOT identical to BSE as seen in the United Kingdom. While there were similarities in clinical signs between this experimental disease and BSE, there was no evidence of aggressiveness, hyperexcitability, hyperesthesia (tactile or auditory), or hyperemetria of limbs as has been reported for BSE (9). Neither were there extensive neurologic lesions, which are primary for BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis and gliosis. Although some vacuolation of neuropil, chromotolysis in neurons, and gliosis were seen in the brains of some affected calves, these were industinguishable from those of controls. Vacuolated neurons in the red nucleus of both challenged and normal calves were considered normal for the bovines as previously described (50).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, and the amount of PrP-res positively related to the length of the incubation. ...

snip...

WE also conclude from these studies that scrapie in cattle MIGHT NOT BE RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGEST THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is necessary to make a definitive diagnosis. THUS, undiagnosed scrapie infection could contribute to the ''DOWNER-COW'' syndrome and could be responsible for some outbreaks of transmissible mink encephalopathy proposed by Burger and Hartsough (8) and Marsh and harsough (52). ...

snip...

Multiple sources of sheep affected with scrapie and two breeds of cattle from several sources were used inthe current study in an effort to avoid a single strain of either agent or host. Preliminary results from mouse inoculations indicate multiple strains of the agent were present in the pooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by using intracerebral, intramuscular, subcutaneous, and oral routes of inoculation of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1 affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48 months after inoculation. Signs were disorientation, incoordination, a stiff-legged stilted gait, progressive difficulty in rising, and finally in terminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattle similarly inoculated with brain tissue from a goat with scrapie exhibited similar signs 27 and 36 months after incoluation. Clinical courses were 43 an 44 days. Brain lesions of mild gliosis and vacuolation and mouse inoculation data were insufficient to confirm a diagnosis of scrapie. This work remained controversial until recent examination of the brains detected PrP-res in all 3 cattle with neurologic disease but in none of the unaffected cattle (62). Results of these studies are similar to ours and underscore the necessity of methods other than histopathology to diagnose scrapie infection in cattle. We believe that immunologic techniques for detecting PrP-res currently provide the most sensitive and reliable way to make a definitive diagnosis...

http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf

Visit to USA ... info on BSE and Scrapie

http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf

http://www.ngpc.state.ne.us/cgi-bin/ultimatebb.cgi?ubb=get_topic;f=12;t=000385

12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie
A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.

The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.

It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.

Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"

Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.

snip...

76/10.12/4.6

http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.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

THE infamous USA SPORADIC CJDs, something to ponder;

IF the USA TSE in some cattle does not look like UK BSE, why would the USA human TSE all look like UK nvCJD???

TSS

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