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From: TSS ()
Subject: Full Report from HBSEF's Visit to Washington DC to Attend the CJD Foundation Family Conference July 9th -12th , 2005
Date: December 19, 2005 at 7:56 am PST

Full Report from HBSEF's Visit to Washington DC to Attend the
CJD Foundation Family Conference July 9th -12th , 2005

Report Prepared September 2005

Graham Steel left his hometown of Glasgow, Scotland on Friday the 8th of July to stay with friends in Manchester ahead of the following morning’s early connecting flight from Manchester Airport to Paris. Early on the 9th, he met up with Don Simms and then one of the Human BSE Foundation’s 3 Medical Advisors, Dr Nikolai Rainov. After a brief change over to Air France in Paris, we then flew across the Atlantic.

SATURDAY (USA TIME)

We arrived at the Washington Court Hotel, Washington DC around 6pm US time on the 9th, checked in very briefly and sat in the Lobby. Whilst the main event did not commence until the following morning, the three of us forgot that the informal reception was taking place one floor above us. Don then spotted Dr Pierluigi Gambetti. Graham swiftly went into action to track him down. Graham suddenly then remembered that the event was indeed underway when he entered a room full of faces of which he knew several, but most were new.

With so many people to connect with instantaneously, he summoned up Mr Simms and Dr Rainov. After an hour of socialising and discussing our trip across, the three of us got a chance to check into our rooms and get changed for Dinner. Around 40 of us attended an informal Dinner and then several of us retired to the Bar to enjoy some time out before the main event commenced the following morning.

PLEASE REFER TO THE UNDERNOTE FOR FULL DETAILS OF THE SPEAKERS AND A GLOSSARY OF TERMS *

SUNDAY

After an informal continental breakfast, proceedings then began.

The first Speaker was Linda Gregson, Master of Divinity and Executive Director and Bereavement Counsellor of CJD Foundation.

The Conference began with a Memorial Service with several nice pieces read and two pieces sung. The two soloists, both with a deep connection to CJD were Nadia Thomas, who is with the New York State Dept of Health CJD Surveillance Unit established in 2001 and Wanda Culp-Lias, the administrative assistant for the CJD Foundation.

The names of family members were read after each stanza of the following reading.

Prayer of Remembrance


At the rising of the sun and at its going down, we remember them.
At the blowing of the wind and in the chill of winter, we remember them.
At the opening of the buds and in the rebirth of spring, we remember them.
At the shining of the sun and in the warmth of summer, we will remember them.
At the rustling of the leaves and in the beauty of autumn, we remember them.
At the beginning of the year and at its end, we remember them.


When we are weary and in need of strength, we remember them.
When we have joy we crave to share, we remember them.
When we have decisions that are difficult to make, we remember them.
When we have achievements that are based on theirs, we remember them.
When we remember their courage as they faced pain and suffering, they will be our strength.
When we remember the joy and laughter they brought to life, they will be our strength.

As long as we live, they too will live; for they are now a part of us as we will remember them.

We then heard from President of CJD Foundation, Florence Kranitz

Florence gave a very warm welcome to all those present. Including speakers, around 120 people were present.

The first researcher to give a presentation was Dr Pierluigi Gambetti.

Dr Gambetti is the Director of the National Prion Disease Pathology Surveillance Centre, Case Western Reserve University, Ohio. Dr Gambetti is also Medical Director of CJDF.

The title of his presentation was:-

“Pathogenesis of Prion Disease”

His presentation began with recent information from Stanley Prusiner “Synthetic Mammalian Prion”.

The conclusion was “A transmissible prion disease has been generated from a synthetic, i.e. pure (no virus), Prion”.

He then discussed a recent publication by Dr Claudio Soto entitled “In Vitro Generation of Infectious Scrapie Prions”.

A very interesting slide detailed information from Byron Caughey, Rocky Mountain Laboratories (RML) entitled

“Uptake and Neurtitic Transport of Scrapie Prion Protein Coincident with Infection of Neuronal Cells”.

This experimental study showed the conversion of PrPc to PrPsc in the laboratory setting.

One fundamental part of Dr Gambetti’s presentation was in relation to possible Diagnostic Issues

He presented details of an ongoing study in relation to an extremely rare inherited form of Prion disease that he identified several years ago called Fatal Familial Insomnia (FFI). Based on a study of 9 asymptomatic patients involved in this study, using PET imaging scans, 1 asymptomatic patient was identified 13 months before the clinical onset of symptoms.

As early diagnosis is crucial in relation to any form of treatment issue, such breakthroughs in modern imaging techniques may provide some further hope with regards to methods of diagnosis of the onset of Prion Diseases (PrD).

Information was then shown in relation to recent studies on the transmissibility of Chronic Wasting Disease (CWD) to humans.

Studies in ‘humanised’ CWD challenged transgenic mice re-confirmed previous studies that CWD is not transmissible to humans.

Dr Gambetti then confirmed updated research from the National Prion Disease Pathology Surveillance Centre supported by the Centers for Disease Control & Prevention (CDC) and sponsored by the American Association of Neuropathologists (AANP).

Much progress continues to be made with regards to the number of expected cases of CJD to be referred for epidemiology studies at the Centre. Compared to say, 2000 when only 39% of possible cases were referred, 70% were examined in 2004.

2004 State Health Department Progress

Contacts have now been identified for 47 States in the US
CJD has been made or is in the process of being made reportable in 34 States
Reporting agreements have been established in 21 States
Letters have been provided in 14 States

Further progress was reported in relation to a network with new local providers across the US, State Heath Departments, Professional Organizations, CJD Foundation and follow up of 14-3-3 tested cases through Care Providers.

The next Speaker was Prof Robert G Will, Consultant Neurologist, National CJD Surveillance Unit

Edinburgh, UK

His presentation was entitled “vCJD, Recent Developments”

This presentation was not dissimilar from the one given by Dr Paul Brown at last year’s Conference on Prof. Will’s behalf. Please refer to

http://www.hbsef.org/americaconference04.htm

Prof. Robert G. Will has served as the Head of National CJD Surveillance of the United Kingdom and Coordinator of the European CJD Surveillance System. He discussed recent developments in vCJD.

The mean age of onset of vCJD is 26; of death is 28. There were 6 cases under 16 years of age at onset. 133 cases tested were Met/Met. He showed a chart for BSE from 1982 – 1995 and a similar chart for vCJD 1993 – 2003/4.

He showed a chart comparing diet and saw up to four fold increase in vCJD in those who had consumed ‘hamburgers’ as opposed to beef in general, sausage, meat pies, and MBM. Those with vCJD had birth years in the 1960s, 1970s, and 1980s. So far, there has not been a vCJD victim born after 1989. He reported that the number of clinical onsets of vCJD had increased in 2004 from 2003.

He noted this as potentially significant.

One of his slides was a recently taken (2005) photograph in the centre of Glasgow, Scotland, UK to demonstrate the ongoing flagrance of public health issues by unscrupulous individuals:-

Of blood recipients from donors who were CJD victims, there were 26 live recipients; all of them have been informed. Many of the transfusions were given close to the onset of the disease. They studied many medical procedures – neurosurgical, opthamological, endoscopic, abdominal, orthopaedic, transplant, sutures, thoracic, dental and tonsillectomy.

They also looked for vertical transmission and observed NO maternal transmission in familial or sporadic CJD. In terms of vCJD there were 9 women who were pregnant and close to disease onset or after disease onset. The oldest of the children is only 9 years old so it may be too soon to observe maternal transmission or the set of children (9) may be too small a set.

On why there have been “so few” cases of vCJD given the enormous numbers of cattle with BSE – Danny Matthews, a predominant UK BSE Expert from the VLA, showed that a cow can be infected with only 1 milligram of infectious (BSE) material, which is a miniscule dose in terms of weight. In the UK, it is estimated that 54 million doses of BSE infected meat/meat products were consumed by the general public. As to why under 200 cases of vCJD so far, speculation includes: need a huge dose, ? need genetic susceptibility, ? some other co-factor must be present such as inflammation of the bowel/environmental issues etc etc.?.

Prof. Will discussed vCJD in other Countries. France had just reported what is probably the 13th case (that makes 5 – 6 cases in France so far this year). In cases from France, Italy, Saudi Arabia, Netherlands, and Portugal there was no travel or residence in the UK. In the Japanese case there was only 1 month residence.

The last speaker of the first part of the morning session was Dr David Kocisko based at the Rocky Mountain Laboratories (RML)

Dr Kocisko opened his presentation with the subject of in vitro (in a laboratory dish or test tube; an artificial environment) testing of cell-culture PrP-res inhibitors.

He mentioned the main human resources in the TSE field at RML, being:-

Bruce Chesebro >20 years experience
Byron Caughey >15 years experience
Sue Priola > 12 years experience
Rick Race > 30 years experience

He confirmed their overall knowledge that TSE are slow, fatal and transmissible neurodegenerative diseases.

Studies have confirmed inclusion of humans, sheep, cattle, deer, elk, mink, cats, rodents, exotic ungulates and other mammals.

The function of normal PrP is unknown at the moment mentioned Dr Kocisko.

(PrPc has been implicated in the transport and compartmentalisation of copper in the synapse. It probably has multiple other functions that parallel other pathways; experimental animals without PrPc survive and do not exhibit the symptoms of any one disease

The normal cellular form of this protein, PrPc is highly conserved in mammals, and is widely expressed in embryogenesis. Techniques exist to delete or make ineffectual genes in mice. When a mice knockout of the PrPc (i.e. the gene for the protein was deleted in all cells) was made, the mice appeared normal. More recent data suggests however, that these mice had altered circadian rhythms and sleep patterns, which suggest a possible link to Fatal Familial Insomnia. The PrPc is a normal membrane protein in neurons. It is anchored to the membrane through a glycosyl-phosphatidyl inositol link, with the protein chain on the outside of the neuronal plasma membrane. The PrPc (without the PI link) is water soluble, protease sensitive, and consists of 42% alpha helix and 3% beta sheet. – Ed).

A Library of 2000 (FDA approved and natural products) potential compounds had been screened including 17 inhibitors of which 15 are new.

Dr Kocisko displayed several slides of data on the basis for consideration of these compounds. With regards to “treatment” of transgenic mice (ME7) inoculated with the 263K agent, Pentosan Polysulphate was the most powerful inhibitor.

To conclude, he stated that:-

Addition of sheep Scrapie to mouse strain testing may reduce unsuccessful in vivo (studies carried out in living organisms) tests
Many (if not most) inhibitors will not have anti-TSE activity in vivo
Cyclic tetrapyrroles are worthy of further anti-TSE testing
Screening for in vivo activity may be helpful in clinical trial decisions

After a short break for refreshments,

The morning session recommenced with a detailed presentation by Dr Robert Rowher

Again, this was not dissimilar to his presentation last year, see - http://www.hbsef.org/americaconference04.htm

Of note, further progress has been made by Dr Rowher and his colleagues in the field of eradicating the infectious PrPres agent from the blood supply.

He began by noting that the danger in TSEs in interspecies transmission is silent with incubation being many years for scrapie from louping ill vaccine (sheep), Kuru, BSE, and TME. He reviewed the first documented case of transmission via blood. In 1996 the blood donation was made. In 1999 the donor died. In 2003 the blood recipient died of vCJD. He noted that this incubation was fast, contrasting it with Dura mater cases where the incubation is 10 – 15 to over 15 years.

Therefore, given this transmission and relatively fast incubation, he said, there must be low-level systemic contamination and he noted in support the recent evidence of infectivity in muscle tissue. He also mentioned the 3 ½ years sub-clinical detection in the blood recipient who died of other causes but had PrPres in her spleen. Depending upon how one looks at the data, the transmission rate via blood is very high – he highlighted the number 2 out of 7 which is 25%.

Also, the prevalence of infectivity in the lymporeticular prion protein study (tonsil appendix study of 2000 in the UK) is noteworthy. Of 3800 cases, assuming 100% ascertainment (detected all the cases), one can assume ~ 400 infected blood donors. He compared 10 billion infectious doses in the brain versus 10 infectious doses in a millilitre of blood. However, an individual donates 500 millilitres which means 5000 doses. How soon is the person infectious? This time he said about 1/3 through the infection (versus ½ in his previous presentations).

Where is the infectivity? ½ of the infectivity is removed by Leukoreduction. He compared plasma which has 50% of the infectivity and white blood cells with 50% of the infectivity. Prions are lightly attached to white blood cells and can be washed off. Prions are not intrinsic to platelets or red blood cells.

How can blood infectivity be controlled? He mentioned 3 possibilities – (1) sourcing / deferrals; (2) screening; (3) inactivation; (4) removal. Dr Rowher started a company “PRDT” in partnership with the American Red Cross and a Pharma manufacturing company (Macopharma).

They are screening millions of compounds to find something that binds to PrPsc (about 8 million). He said that 1 part per 50,000 escapes removal (is still present after removal). They are working with the UK and the Irish Blood Services and their product will be available by the New Year for testing in humans.

The morning session continued with a detailed presentation by Dr Nikolai G Rainov

The opening title of this presentation was:-

“Therapeutic Strategies in Human TSE – The Role of Cerebroventricular Infusion of Pentosan Polysulphate”

He commenced this presentation to explain that antibodies directed against PrPc and PrPsc are more likely to work in pre-symptomatic cases in order to prevent neuroinvasion.

Antibodies do not penetrate the blood-brain barrier (BBB)

Pharmacological therapies for TSE: to date such as sulphated glycans, quinacrine and flupirtine have been applied in human trials.

Dr Rainov described The Rationale for Cerebroventricular Application of Drugs

Clinical and late preclinical phase of TSE with PrPsc formation in the CNS requires drugs that can cross into brain parenchyma (the key elements of an organ essential to its functioning)
The intact blood-brain barrier (BBB) severely limits penetration of drugs from blood into brain tissue
The CSF-brain barrier (CBB) is much more permeable because of its anatomical structure
Application of drugs in the CSF permits high local concentrations in the CNS
Continuous CSF circulation is a physiological means for dissemination of substances throughout the CNS

Dr Rainov proceeded to demonstrate

The procedure involved in Cerebroventricular drug delivery

Continuous infusion by an implanted programmable pump
Abdominal placement of pump
Telemetric programming of pump
Optional use of non-programmable pumps

His next slide contained details of a 2001 article published in the Associate Society of Microbiology (Michael Beekes et al) entitled

“Early Spread of Scrapie from the Gastrointestinal Tract to the Central Nervous System Involves Autonomic Fibres of the Splanchic and Vagus Nerves”

A number of slides were displayed in relation to the subject of Heart Rate Variability (HRV).

This is an extremely well established medical field

(Please refer to http://www.refer.nhs.uk/ViewRecord.asp?ID=1329 for its potential application in relation to CJD - Ed).

Dr Rainov then gave an overview of 17 cases of Prion Disease (PrD) where PPS has been administered.

A number of non-enhanced CT scans were displayed of some of these and other (no treatment) subjects.

He concluded his presentation by confirming it was now clear that a potential option was infusion of PPS.

Should this option be considered, this should be commenced as early as possible after probable disease diagnosis. There is some subjective (clinical) and objective (physiology) evidence that PPS may halt the progression of TSE and may improve brain stem function. PPS administration seems not able to reverse the clinical course of advanced disease and to achieve functional recovery of higher (cortical) CNS functions.

Systematic follow-up of patients with PPS administration (e.g. Neurophysiology, Neuropsychology, MRI/PET imaging) is required to test for neuronal damage and inflammation in the CNS.

Design and validation of an early screening test for TSE in humans is required

After Lunch, the next Speaker was Dr Gene Major who’s presentation was entitled

The Federal Government Interagency Work Group and Brain Tissue Storage at the NIH

Dr Major’s laboratory maintains extensive collaborations with clinical colleagues and participates in clinical trials as a basic science laboratory as a Clinical Laboratory Improvement Amendments (CLIA) laboratory, which also supports FDA regulatory studies. Dr Major reiterated the fact that the invaluable collection of (CJD) tissue samples will be preserved for current and ongoing research.

He is also the Acting Deputy Director of the NINDS. He spoke on the Interagency Working Group on Prions that was set up in 2004 – which we lobbied for last year, chartered by the White House Office of Science and Technology Policy. Includes Major and Dr. Caird Rexroad at executive level of working group. Their first report will be out soon. They have met 3 times over the past 12 months. Includes – Environmental Protection Agency, USDA, DoD, Health and Human Services, Dept of Homeland Security, Dept of Interior, Dept of State, Veteran Affairs, Office of Management and Budget, Office of Science and Technology

Policy, National Science Foundation, Smithsonian (e.g., zoo). Additional government agencies have asked to join.

Next, we heard from Dr James Sejvar, Neuroepidemiologist from the CDC, Atlanta, Georgia.

His presentation was not dissimilar to that of last years Conference – see http://www.hbsef.org/americaconference04.htm

Next up was a 1 ½ hour Panel Discussion involving:-

Dr Gambetti
Dr Will
Dr Rohwer
Dr Belay
Dr Kokisco
Dr Rainov
Dr Sejfar

After a short break, the closing session was:-

“Moving Round Tables: Researchers and Families”

Having spoken with many family members, like last year, many found this most helpful to get a chance to have a “one on one” with the researchers. As this session lasted for 1 ½ hours, plenty time was had for all to ask questions with the researchers revolving round the tables every 15 minutes.

After a very short “time-out”, we had a chance to get ‘dressed up’ for a formal Dinner at the Hotel.

FORMAL DINNER

Florence gave a very touching, heart warming and most passionate speech.

A special thanks was given to the Secretarial staff of CJDF who work tirelessly and with much devotion to their work.

This years Guest Speaker was Marjorie Mezvinsky, Former Member of Congress, Pennsylvania. Whilst it went down well, it was a tad lengthy.

After Dinner, we swooped back into informal mode and relaxed for what was left of the evening.

In the ‘wee small hours’, a handful of us went back into the main Conference room to carry out some technical work ahead of the following day’s presentations.

MONDAY

After Breakfast, the first Speaker was Dr Dan Morhaim, Maryland House of Representatives. Delegate, District 11. His Presentation was entitled Making CJD Reportable in your State.

After the CJDF Chairman (Mark Goldfarb) gave a presentation on the Foundation’s ‘Budget and Financials’ we heard from Deana Simpson, Founder of CJD Insight. Her Presentation was on Familial CJD.

The next Speaker was Nadia Thomas from N State DoH entitled NYS DoH Work Group Report

Nadia Thomas is with the New York State Dept of Health. TSE surveillance began in NY in 2001. Their “enhanced surveillance” allows them to retrieve medical records in a timely manner. They work with NYC (represented at the conference) and with funeral directors.

The schedule was altered slightly and it was then Graham Steel’s turn to give a presentation in his capacity as a Trustee of the Human BSE Foundation.

This section of the Conference was titled Support Organisations Around the World.

Graham Steel – Vice-Chair Human BSE Foundation

His presentation was namely in relation to four topics:-

CJD Support Organisations
Transmissible Spongiform Encephalopathy (TSE’s)
The Nature of TSE Disease Transmission
Potential Diagnostic Methods

He detailed the timeline from 1992 to the current day of the birth and emergence of the main nine Groups that he was aware of and has had contact with. There are groups established in the USA, the UK, France, Japan and Australia. (There is also an FFI organisation in Italy but contact was minimal mainly due to the language barrier).

He explained that groups from France and Japan were unable to attend this year, but would hopefully be represented next time. He discussed the challenges that face these groups but commented that:-

“Through the global unity that continues to grow,

I firmly believe that by forming such a Global Alliance, these groups combined voice

and strength for those who have lost, those current suffering from,

and those sadly still to come in the future is and will

always be really important”.

He discussed the background of TSE’s and the positive developments that have been made in the last few years in relation to better methods of early diagnostics.

He then moved on to display a number of slides aimed at looking into what is currently known about the different types of Prion Diseases (PrD) and how these conditions can affect different parts of the brain/brain stem.

He displayed one slide in relation to vCJD and what was currently known about the effect that the condition has on the brain.

He discussed Flair MFI Imaging, the Pulvinar and Thalamus areas of the brain. In relation to the latter, he referred to a very recent Peer Reviewed and Published Paper by Iwasaki et al. Neuropathologic characteristics of brainstem lesions in Classical Creutzfeldt-Jakob disease. See - http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15933870&itool=iconabstr&query_hl=5

Comment was passed on the continuing debate about “Final Proof of the Prion Hypothesis” and comments made by Dr Gambetti in a recent copy of Cell in this regard. Brief comment was made on the other existing unproven Hypotheses.

Graham then referred to a number of Articles contained within ‘The BSE Enquiry’

“Lessons (BSE) for other Countries”

was the theme of this part of his Presentation.

He also cited a quote from an article that was published by Irwin Mitchell Solicitors at the time of the publication of the Enquiry (September 2000) :-

“Insufficient research was undertaken into means of ameliorating the progress of vCJD. In particular, the Families are concerned that the work on Dextran Sulphate 500 and Pentosan Polysulphate undertaken at the ARC Neuropathogenesis Unit [NPU] during the 1970's was not immediately revisited once vCJD had been identified in 1996”.

He ended his presentation to remind the audience of DoH funded research carried out by Dr Chris Pomfrett and colleagues at the University of Manchester (June 2002 – 2004) in relation to HRV as a potential method of early diagnosis for vCJD (& BSE) and potentially, Classical CJD in light of the content of the above mentioned Iwasaki et al Paper.

Next, we heard from Suzanne Solvyns and Carol Wilson, Co-National coordinators of the CJD Support Network, Australia

Whilst neither have lost a loved one to CJD, like 2400 Australians (and many other thousands across the World), they are both at “low risk” and live with fear of developing CJD. They represent the “worried well”, those who know they are at risk of Iatrogenic CJD or health related CJD.

2400 Australians were treated with human pituitary hormones (hPH). This includes 1570 women and 60 men treated for fertility with human pituitary gondadotrophin (hPG) and 660 children with human growth hormone (hGH) for short statue.

This was done through the Australian Human Pituitary Hormone Programme (AHPHP), which ran in Australia from 1967 until 1985. This programme was suspended following CJD deaths of recipients of hGH in the USA.

Four recipients of hGH have died in Australia. A further five people have also died of CJD with histories of Dura Mater grafts. In 1992, public attention following the first two CJD deaths of hPG recipients resulted in the tracking of recipients. An Inquiry was commenced in 1993.

In 1994, the CJD Support Group Network Inc (CJDSGNI) was established with a funding grant of $10M for ‘Services’ for recipients of hPH.

Suzanne and Carol have been involved as Committee Members since then.

In 2004, due to changes in management, the Group was restructured and a non-profit company, CJD Support Group Network Pty. Ltd (CJDSGN) and a new website were established. The Organisation now offers support for all Australians whose lives are touched with CJD.

The next speaker was Ms. Margaret Leitch, National CJD Care Coordinator in the UK

Margaret joined the National CJD Surveillance Unit, Edinburgh, in 2000 as a Research Nurse looking at the risk factors associated with Classical and Variant CJD within the UK. In November 2004, she began working as the Acting National Care Coordinator for all forms of CJD within the UK. She has formally acted as National Coordinator since March 2005.

The CJD Surveillance Unit was set up in 1990 with the aim to identify all cases of CJD and later, Variant CJD in the UK, the co-ordinating centre for surveillance of CJD throughout the European Union and again later, to identify any risk to the human population from the BSE epidemic.

It consists of Neurologists, Neuropathologists, Research Nurses, Genetic Scientists, Biochemists, Epidemiologists, Statisticians, Admin Staff and of course the Care Team. The role of National Care Co-ordinator was established in 2000 following a report the year earlier by Dr Margaret Douglas into the experiences of families who had the diagnosis of CJD. See http://www.cjd.ed.ac.uk/carerep.html

These were largely very bad experiences and it was felt that an expert was needed to address the issues raised in the report. The role of National Care Co-ordinator was initially set up with the following remit

Co-ordination of care
Support of families and professional carers
Education and information giving


This role was further extended following the BSE Inquiry to include the management of a Care Package Fund. This was set up exclusively for those people affected by all forms of CJD.

What happens when a referral comes into the CJD Surveillance Unit?

Referrals generally come from a Neurologist but particularly in the case of familial or iatrogenic CJD, it can be other sources. In the case of Variant and Classical CJD, a Research Registrar will visit the family with a research nurse. This is to confirm probable diagnosis and to obtain research information for a case control study into the risk factors associated with CJD. A referral is then made to the care team if the patient has a probable or possible diagnosis of CJD. Contact is made by the National Care Co-ordinator with the family as soon as possible and a visit arranged.

A case conference is set up with all professionals involved in the care as soon as possible and a teaching session is arranged for staff. The Co-ordinator is available to visit on a regular basis possibly every 4 - 6 weeks. This is to offer support, counselling and information to families as well as to facilitate regular case conferences. Access to the care fund is also made available. The care fund is available to be used in conjunction with the funding normally provided by the local authorities, not to replace it. The Care Fund can be used for a whole variety of important things such as:-

extra nursing care
physiotherapy
equipment
adaptations
respite care
complementary therapies
transport
counselling
other health and social care needs.



The care package is there to ensure that any family affected by CJD gets a ‘Gold Standard’ Package of care.

Margaret’s hope is that by being there for families she makes a very positive difference to
their experience.

For further reading, see http://www.cjd.ed.ac.uk/care.html

The next person that we heard from was Don Simms with a presentation entitled

‘A Father’s Story’

For those that are not aware, Don is the father of Jonathan Simms, the first vCJD patient in the World to receive Pentosan Polysulphate (PPS) treatment for Prion disease.

At last years Conference, Don gave a scientific presentation with an overview of Prion related issues. This year, CJDF requested Don to give a presentation on ‘the personal side’ which he proceeded to do.

Don is an Ulster (Northern Ireland) man who has been married to Karen for 23 years. They have seven children (at the time of writing - Ed) ranging in age from 21 - 2 years.

Jonathan was diagnosed with vCJD in October 2001 and was the first person world wide to receive PPS directly into the brain in an attempt to arrest or slow down the condition.

Currently (September 2005), Jonathan is the longest surviving recorded victim to have lived with the disease. Jonathan has been cared for at home by his family since being diagnosed.

Don spoke of Jonathan’s birth, upbringing and his promising skills as a soccer player. Before Jonathan became ill he was an A-level student, with the possibility of having a promising soccer career. He was on the books for Glentoran FC, one of Belfast’s top semi-professional football teams.

Don and Karen were told that nothing could be done to save their eldest son's life. Not content with that advice, Don started his search for information on what could be done. Jonathan was briefly treated with a compound called Quinacrine, but the treatment was short lived due to adverse side effects.

After finding out about PPS via the Internet and Microbiologist Dr Steven Dealler, he found a Neurosurgeon to provide him with advice on how PPS could potentially be injected directly into Jonathan's brain. This form of drug delivery was nothing new and is a common form of brain surgery.

Whilst this was experimental treatment, this was not for experiments sake.

After a 9 month Court battle, and a ‘positive’ judgement, the all clear was given for Jonathan and another vCJD patient to get this treatment. Sadly, the other patient (a young girl who could not be named for legal reasons) whose condition was not dissimilar to Jonathan's died before she could be treated.

Jonathan's condition continued to deteriorate during this time.

After he first received PPS in January 2003, unchartered waters were truly entered into. Thankfully, the

"expected" side effects (seizures or even death) did not occur.

Small but positive important changes have been seen in his condition and not only is he the longest surviving vCJD patient, he is in a stable condition and a number of observations (objective and subjective) do appear to indicate that PPS has either halted or slowed down the progression of the disease.

Don and Karen would like to accept the advice of their son's GP who has said on record that Jonathan is no longer terminally ill.

One family member present at the Conference stated “More than any other presentation, Don showed the depth of the tragedy that befalls CJD families and the enormous struggle that they are faced with”.

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

Immediately after Lunch, the Conference’s second

Panel Discussion took place entitled “Options and Therapies for the Treatment of Prion Disease”

The Panel consisted of:-

Dr Gambetti
Dr Kokisco
Dr Andrew Monjan (Program Director, National Institute of Ageing – NIH)
Dr Michael Nunn, (Program Director, National Institute of Neurological Disorders and Stroke – NIH
Dr Belay
Dr Rainov

Immediately thereafter, Linda Gregson gave her presentation

“Lessons in Grief”

Linda was again present to discuss the realities of grief which everyone at the conference knows all too well. Linda presented an understanding of grief which helped the participants understand their own experience as a normal experience to this traumatic illness called CJD. Included in the presentation was an explanation of the various symptoms of grief. These symptoms included: inability o concentrate, inability to remember or to make decisions and fatigue. Her stories helped everyone to normalize their personal experiences and understand that while alone in their grief they were not alone in their journey through this dark valley.

The next piece of Linda’s presentation was an explanation of the typical time frame of grief. We live in a society that assumes grief should only take a few weeks or months but in reality takes years to journey through this experience.

Also shared was the experience every person in grief has. The STUG—Subsequent Temporary Upsurge of Grief—is real for all. This is the experience of having a smell, sound or visual of an unexpected item and then the tears flow uncontrollably. This commonly takes 5-7 minutes to put the

defense mechanism in place so that the tears as controlled again. As one participant noted, “Perhaps this should be called a SNUG—Subsequent Normal Upsurge of Grief. After all, we all still need to snuggle.

The final Presentations were given by

Tracie Kedzierski, CJDF Board Member and Questionnaire Chairman CJD Foundation: Questionnaire

Ruthie George, CJDF Treasurer CJD Foundation Projects – Involvement

Kathy Filosi Nelson, Writer/Producer/Field Director CJD Foundation Medical Educational DVD Project . In relation to the latter:-

“Our problem was born out of the increasing awareness of the lack of Prion disease knowledge and understanding on the part of many neurologists and neurosurgeons in the United States. 40% of families surveyed referred to problems with diagnosis and understanding of CJD. This gap in awareness creates cascading effects that encompass every possible quality of life issue facing CJD patients and their families in the brief span of time left when the disease becomes symptomatic. The solution – a Medical Educational Project”

Florence Kranitz, CJDF President

The main Conference concluded with a 1 ½ hour section entitled:-

“Advocacy Groups Prepare for Congressional Visits”

This given by Marjorie Margolies Mezvinsky.

In the evening, a crowd of around 30 or so headed out for Dinner. The feeling all round was that we had witnessed and taken part in an extremely productive Conference that would be hard to “better” next year. After walking back in the sweltering late evening heat to the Hotel, a handful of us had some informal time together before most departed home the following day after Scheduled Congressional Visits at Capitol Hill.

TUESDAY

After his first “long lie in” (9am !!) for a week, Graham had his first chance to fully unwind and see some sights that he had yet to see around Washington. Apart from Graham and Don, there was only one other family still present so we all enjoyed each others company during the evening.

WEDNESDAY

As our flight home was not until the evening, we visited a Mall at Pentagon City during the afternoon before getting a cab back to Dulles Airport to catch up with Dr Rainov.

We arrived in Paris around midday (GMT) on the Thursday and caught our connecting flight back to Manchester.

The three of us went our separate ways with Graham catching his final flight back to Glasgow with two minutes to spare.

END OF REPORT

UNDERNOTE *

SPEAKERS

Linda Gregson (Bereavement Counsellor)

Dr Pierluigi Gambetti (Director, National Prion Disease Surveillance Centre, Ohio)

Prof Robert Will (National CJD Surveillance Unit, Edinburgh)

Dr David Kocisko (Rocky Mountain Laboratories, Montana)

Dr Robert Rowher (Director of Molecular Neurovirology Unit VA Medical Center, Baltimore)

Dr Nikolai Rainov (Dept. of Neurological Science, University of Liverpool. Now Dept. Neurosurgery, Martin-Luther University Hospital Halle, Germany.)

Dr Gene Major (Acting Director Basic Neuroscience Program Chief, Laboratory of Molecular Medicine)

Lt James Sejvar MD (Centers for Disease Control and Prevention) (CDC)

Dr Ermias Belay (CDC)

Dan Morhain (MD Maryland House of Representatives, Delegate, District 11)

Mark Goldfarb (CJD Foundation Budget and Finances)

Deana Simpson (CJD Insight (Founder)) Familial CJD

Nadia Thomas (CJD Work Group Report, MS New York State DoH)

Don Simms (father of 1st vCJD PPS patient)

Graham Steel (Vice-Chair, Human BSE Foundation)

Suzanne Solvyns and Carol Wilson (Co-Chairpersons CJD Support Network Australia)

Margaret Leitch (UK Care Co-ordinator CJD Surveillance Unit, Edinburgh)

Dr Andrew Monjan (Program Director NIH [Aging])

Dr Michael Nunn (Program Director National Institute of Neurological Disorders and Strokes, NIH)

Tracie Kedzierski (CJD Foundation, Questionnaire)

Ruthie George (CJD Foundation Projects)

Kathy Nelson (CJD Foundation Medical Educational DVD Project)

Marjorie Margolies Mezvinsky (Former member of Congress – Preparing for Congressional Visits)

GLOSSARY OF TERMS

SEE http://www.cjd.ed.ac.uk/glos.htm

Contributions were sought and received from Hatte Blejer, Margaret Leitch and Linda Gregson.

Their assistance in the writing of the above was greatly appreciated. Thank you.

Greetings,

> Studies in ‘humanised’ CWD challenged transgenic mice re-confirmed

> previous studies that CWD is not transmissible to humans.

THIS by far, has NOT been confirmed yet, AS WITH THE MYTH,

that scrapie will not transmit to humans. there is more science to date

that shows both cwd and scrapie are just as capable as BSE to transmit

to humans. WE must never forget the sCJD's and there victims, and

finding the routes and sources of these sCJD's.

This seems to be the focus. ...TSS

HUMAN TSE USA 2005


Animal Prion Diseases Relevant to Humans (unknown types?)
Thu Oct 27, 2005 12:05
71.248.128.109


About Human Prion Diseases /
Animal Prion Diseases Relevant to Humans

Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.

Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)

Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)


http://www.cjdsurveillance.com/abouthpd-animal.html


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here;

p.s. please note the 47 PENDING CASES to Sept. 2005

p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ???

p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???

p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN???


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


CWD TO HUMANS = sCJD ???


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


ATYPICAL TSEs in USA CATTLE AND SHEEP ?


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

----- Original Message -----
From: "Terry S. Singeltary Sr."
To: "Jim Woodward" ; "'Shu Chen'"
Sent: Sunday, December 18, 2005 9:51 AM
Subject: Re: Human resistance to scrapie?


> Greetings Jim and Dr. Chen,
>
>
> as far as amplification and transmission, this is not rocket science, just
> junk science and or denial $ ;-)
> there are plenty of references in science 'sound science' to show that
> indeed the potential for scrapie transmission
> to man (who knows which strain of scrapie or all of them) is as real is as
> BSE or CWD. to continue with the myth
> that scrapie will not transmit to humans, under the pretence of 200 years of
> scrapie and no documented transmision
> to humans, is like saying that we have never had BSE/TSE in USA cattle herds
> until Dec. 2003. it's simply not true.
> the truth is they never looked until then, thus it was never documented. two
> different things, not here and not documented.
>
>
> but just look at the science just off the top of my head here, and then try
> referencing scrapie transmission studies to
> humans and or primates to dispute it. ......TSS
>
>
> Gerald Wells: Report of the Visit to USA, April-May 1989
>
> snip...
>
> The general opinion of those present was that BSE, as an
> overt disease phenomenon, _could exist in the USA, but if it did,
> it was very rare. The need for improved and specific surveillance
> methods to detect it as recognised...
>
> snip...
>
> It is clear that USDA have little information and _no_ regulatory
> responsibility for rendering plants in the US...
>
> snip...
>
> 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_...
>
> snip...
>
> http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
>
> To be published in the Proceedings of the
> Fourth International Scientific Congress in
> Fur Animal Production. Toronto, Canada,
> August 21-28, 1988
>
> Evidence That Transmissible Mink Encephalopathy
> Results from Feeding Infected Cattle
>
> R.F. Marsh* and G.R. Hartsough
>
> .Department of Veterinary Science, University of Wisconsin-Madison, Madison,
> Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin
> 53092
>
> ABSTRACT
> Epidemiologic investigation of a new incidence of
> transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
> suggests that the disease may have resulted from feeding infected
> cattle to mink. This observation is supported by the transmission of
> a TME-like disease to experimentally inoculated cattle, and by the
> recent report of a new bovine spongiform encephalopathy in
> England.
>
> INTRODUCTION
>
> Transmissible mink encephalopathy (TME) was first reported in 1965 by
> Hartsough
> and Burger who demonstrated that the disease was transmissible with a long
> incubation
> period, and that affected mink had a spongiform encephalopathy similar to
> that found in
> scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough,
> 1965).
> Because of the similarity between TME and scrapie, and the subsequent
> finding that the
> two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it
> was
> concluded that TME most likely resulted from feeding mink scrapie-infecied
> sheep.
> The experimental transmission of sheep scrapie to mink (Hanson et al., 1971)
> confirmed the close association of TME and scrapie, but at the same time
> provided
> evidence that they may be different. Epidemiologic studies on previous
> incidences of
> TME indicated that the incubation periods in field cases were between six
> months and
> one year in length (Harxsough and Burger, 1965). Experimentally, scrapie
> could not be
> transmitted to mink in less than one year.
> To investigate the possibility that TME may be caused by a (particular
> strain of
> scrapie which might be highly pathogenic for mink, 21 different strains of
> the scrapie
> agent, including their sheep or goat sources, were inoculated into a total
> of 61 mink.
> Only one mink developed a progressive neurologic disease after an incubation
> period of
> 22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was
> either caused
> by a strain of sheep scrapie not yet tested, or was due to exposure to a
> scrapie-like agent
> from an unidentified source.
>
> OBSERVATIONS AND RESULTS
>
> A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville,
> Wisconsin
> reported that many of his mink were "acting funny", and some had died. At
> this time, we
> visited the farm and found that approximately 10% of all adult mink were
> showing
> typical signs of TME: insidious onset characterized by subtle behavioral
> changes, loss of
> normal habits of cleanliness, deposition of droppings throughout the pen
> rather than in a
> single area, hyperexcitability, difficulty in chewing and swallowing, and
> tails arched over
> their _backs like squirrels. These signs were followed by progressive
> deterioration of
> neurologic function beginning with locomoior incoordination, long periods of
> somnolence
> in which the affected mink would stand motionless with its head in the
> corner of the
> cage, complete debilitation, and death. Over the next 8-10 weeks,
> approximately 40% of
> all the adult mink on the farm died from TME.
> Since previous incidences of TME were associated with common or shared
> feeding
> practices, we obtained a careful history of feed ingredients used over the
> past 12-18
> months. The rancher was a "dead stock" feeder using mostly (>95%) downer or
> dead dairy
> cattle and a few horses. Sheep had never been fed.
>
> Experimental Transmission. The clinical diagnosis of TME was confirmed by
> histopaihologic examination and by experimental transmission to mink after
> incubation
> periods of four months. To investigate the possible involvement of cattle in
> this disease
> cycle, two six-week old castrated Holstein bull calves were inoculated
> intracerebrally
> with a brain suspension from affected mink. Each developed a fatal
> spongiform
> encephalopathy after incubation periods of 18 and 19 months.
>
> DISCUSSION
> These findings suggest that TME may result from feeding mink infected cattle
> and
> we have alerted bovine practitioners that there may exist an as yet
> unrecognized
> scrapie-like disease of cattle in the United States (Marsh and Hartsough,
> 1986). A new
> bovine spongiform encephalopathy has recently been reported in England
> (Wells et al.,
> 1987), and investigators are presently studying its transmissibility and
> possible
> relationship to scrapie. Because this new bovine disease in England is
> characterized by
> behavioral changes, hyperexcitability, and agressiveness, it is very likely
> it would be
> confused with rabies in the United Stales and not be diagnosed. Presently,
> brains from
> cattle in the United States which are suspected of rabies infection are only
> tested with
> anti-rabies virus antibody and are not examined histopathologically for
> lesions of
> spongiform encephalopathy.
> We are presently pursuing additional studies to further examine the possible
> involvement of cattle in the epidemiology of TME. One of these is the
> backpassage of
> our experimental bovine encephalopathy to mink. Because (here are as yet no
> agent-
> specific proteins or nucleic acids identified for these transmissible
> neuropathogens, one
> means of distinguishing them is by animal passage and selection of the
> biotype which
> grows best in a particular host. This procedure has been used to separate
> hamster-
> adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The
> intracerebral
> backpassage of the experimental bovine agent resulted in incubations of only
> four months
> indicating no de-adaptation of the Stetsonville agent for mink after bovine
> passage.
> Mink fed infected bovine brain remain normal after six months. It will be
> essential to
> demonstrate oral transmission fiom bovine to mink it this proposed
> epidemiologic
> association is to be confirmed.
>
> ACKNOWLEDGEMENTS
> These studies were supported by the College of Agricultural and Life
> Sciences,
> University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the
> United
> States Department of Agriculture. The authors also wish to acknowledge the
> help and
> encouragement of Robert Hanson who died during the course of these
> investigations.
>
> REFERENCES
> Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II.
> Experimental and
> natural transmission. J. Infec. Dis. 115:393-399.
> Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and
> Gustatson,
> D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861.
> Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I.
> Epizoociologic and
> clinical observations. 3. Infec. Dis. 115:387-392.
> Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of
> the
> transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
> Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
> encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow
> transmissible
> diseases of the nervous system. Vol. 1, Academic Press, New York, pp
> 451-460.
> Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in
> cattle?
> Proceedings of the Seventh Annual Western Conference for Food Animal
> Veterinary
> Medicine. University of Arizona, pp 20.
> Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D.,
> Jeffrey, M.,
> Dawson, M. and Bradley, R. 1987. A novel progressive spongiform
> encephalopathy
> in cattle. Vet. Rec. 121:419-420.
>
> MARSH
>
> http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
>
>
>
>
> 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
>
>
> Like lambs to the slaughter
> 31 March 2001
> Debora MacKenzie
> Magazine issue 2284
> What if you can catch old-fashioned CJD by eating meat from a sheep infected
> with scrapie?
> FOUR years ago, Terry Singeltary watched his mother die horribly from a
> degenerative brain disease. Doctors told him it was Alzheimer's, but
> Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
> and he demanded an autopsy. It showed she had died of sporadic
> Creutzfeldt-Jakob disease.
>
> Most doctors believe that sCJD is caused by a prion protein deforming by
> chance into a killer. But Singeltary thinks otherwise. He is one of a number
> of campaigners who say that some sCJD, like the variant CJD related to BSE,
> is caused by eating meat from infected animals. Their suspicions have
> focused on sheep carrying scrapie, a BSE-like disease that is widespread in
> flocks across Europe and North America.
>
> Now scientists in France have stumbled across new evidence that adds weight
> to the campaigners' fears. To their complete surprise, the researchers found
> that one strain of scrapie causes the same brain damage in ...
>
> The complete article is 889 words long.
>
> full text;
>
> http://www.newscientist.com/article.ns?id=mg16922840.300
>
>
>
> Neurobiology
> Adaptation of the bovine spongiform encephalopathy agent to primates and
> comparison with Creutzfeldt- Jakob disease: Implications for human health
> Corinne Ida Lasmézas*,, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*,
> Domíníque Marcé*, François Lamoury*, Nicolas Kopp, Jean-Jacques Hauw§, James
> Ironside¶, Moira Bruce, Dominique Dormont*, and Jean-Philippe Deslys*
> * Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction
> des Sciences du Vivant/Département de Recherche Medicale, Centre de
> Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc,
> BP 6, 92 265 Fontenay-aux-Roses Cedex, France; Hôpital Neurologique Pierre
> Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; § Laboratoire de
> Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital,
> 75013 Paris, France; ¶ Creutzfeldt-Jakob Disease Surveillance Unit, Western
> General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and
> Institute for Animal Health, Neuropathogenesis Unit, West Mains Road,
> Edinburgh EH9 3JF, United Kingdom
>
> Edited by D. Carleton Gajdusek, Centre National de la Recherche
> Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
> (received for review October 16, 2000)
>
>
> Abstract
> Top
> Abstract
> Introduction
> Materials and Methods
> Results
> Discussion
> Conclusions
> References
> There is substantial scientific evidence to support the notion that bovine
> spongiform encephalopathy (BSE) has contaminated human beings, causing
> variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns
> about the possibility of an iatrogenic secondary transmission to humans,
> because the biological properties of the primate-adapted BSE agent are
> unknown. We show that (i) BSE can be transmitted from primate to primate by
> intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD
> to humans could be readily recognized pathologically, whether it occurs by
> the central or peripheral route. Strain typing in mice demonstrates that the
> BSE agent adapts to macaques in the same way as it does to humans and
> confirms that the BSE agent is responsible for vCJD not only in the United
> Kingdom but also in France. The agent responsible for French iatrogenic
> growth hormone-linked CJD taken as a control is very different from vCJD but
> is similar to that found in one case of sporadic CJD and one sheep scrapie
> isolate. These data will be key in identifying the origin of human cases of
> prion disease, including accidental vCJD transmission, and could provide
> bases for vCJD risk assessment.
>
>
> http://www.pnas.org/cgi/content/full/041490898v1TSS
>
>
> 1: Cent Eur J Public Health 2003 Mar;11(1):19-22
>
> Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases
> in Orava and Liptov regions (northern Slovak focus) 1983-2000.
>
> Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T.
>
> Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius
> University, Sklabinska 26, Martin, 037 53 Slovakia. MADAR@jfmed.uniba.sk
>
> While familial cases of Creutzfeldt-Jakob disease are extremely rare
> all over the world, 3 familial clusters were observed between
> 1983-2000 in a relatively small area situated in the North of
> Slovakia. Prevalence of CJD in this area exceeded the overall
> prevalence in Slovakia more than 8 times. The majority of CJD
> patients admitted consuming sheep brain. Most patients lived in
> small secluded villages with rather common familial intermarriage.
> CJD affected both sexes equally. All patients were prior to the
> disease mentally normal individuals. Shortly after the onset of CJD
> their mental status deteriorated remarkably with an average survival
> rate of 3.6 months.
>
> PMID: 12690798
>
> http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui
> ds=12690798&dopt=Abstract
>
> ------------------------------------------------------------------------
>
> 1: Eur J Epidemiol 1991 Sep;7(5):520-3
> > =pubmed_pubmed&from_uid=1761109>
>
>
> "Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.
>
> Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.
>
> Institute of Preventive and Clinical Medicine, Bratislava.
>
> Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in
> 1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a
> coincidence of genetic and environmental risks in clustering patients.
> Since Spongiform Encephalopathies might be transmitted orally, (Bovine
> Spongiform Encephalopathy), the possibility of zoonotic source of CJD
> cases in Orava was also considered. A deficient knowledge about the
> occurrence of scrapie in Slovakia stimulated an examination of sheep
> with signs of CNS disorders in two flocks of Valasky breed in Orava. In
> one flock, neurohistopathological examination revealed in sheep brains
> lesions characteristic for scrapie. Frozen brain tissue of these animals
> were used for the detection of scrapie associated fibrils. They were
> found in 2 animals from the same flock. This is the first laboratory
> confirmation of scrapie in Czecho-Slovakia. The possible epidemiological
> and economical implications are emphasized.
>
> http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui
> ds=1761109&dopt=Abstract
>
>
> STATEMENT OF DR HELEN GRANT MD FRCP
> ISSUED 13/05/1999
>
> BSE INQUIRY
>
> http://www.bseinquiry.gov.uk/files/ws/s410.pdf
> http://www.bseinquiry.gov.uk/files/ws/s410x.pdf
>
> http://www.bseinquiry.gov.uk/evidence/ws/ws8.htm
>
> CWD to CJD in humans (why not?), as easy as BSE/Scrapie;
>
> The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
> © European Molecular Biology Organization
>
> Evidence of a molecular barrier limiting
> susceptibility of humans, cattle and sheep to
> chronic wasting disease
>
> G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
> L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
> Smits2
> and B. Caughey1,7
>
> 1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
> 3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
> Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
> Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
> University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
> Institute for Animal Science and Health, Lelystad, The Netherlands
> 7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000;
> revised July 3, 2000; accepted July 5, 2000.
>
> Abstract
>
> Chronic wasting disease (CWD) is a transmissible
> spongiform encephalopathy (TSE) of deer and elk,
> and little is known about its transmissibility to other
> species. An important factor controlling
> interspecies TSE susceptibility is prion protein (PrP)
> homology between the source and recipient
> species/genotypes. Furthermore, the efficiency with which
> the protease-resistant PrP (PrP-res) of one
> species induces the in vitro conversion of the normal PrP
> (PrP-sen) of another species to the
> protease-resistant state correlates with the cross-species
> transmissibility of TSE agents. Here we
> show that the CWD-associated PrP-res (PrPCWD) of cervids
> readily induces the conversion of recombinant cervid PrP-sen
> molecules to the protease-resistant state in accordance
> with the known transmissibility of CWD between cervids. In contrast,
> PrPCWD-induced conversions of human and bovine PrP-sen were
> much less efficient, and conversion of ovine PrP-sen was
> intermediate. These results demonstrate a barrier at the
> molecular level that should limit the susceptibility of these non-cervid
> species to CWD.
>
> snip...
>
> Clearly, it is premature to draw firm conclusions about CWD
> passing naturally into humans, cattle and sheep, but the present
> results suggest that CWD transmissions to humans would be as
> limited by PrP incompatibility as transmissions of BSE or sheep
> scrapie to humans. Although there is no evidence that sheep
> scrapie has affected humans, it is likely that BSE has caused variant
> CJD in 74 people (definite and probable variant CJD cases to
> date according to the UK CJD Surveillance Unit). Given the
> presumably large number of people exposed to BSE infectivity,
> the susceptibility of humans may still be very low compared with
> cattle, which would be consistent with the relatively inefficient
> conversion of human PrP-sen by PrPBSE. Nonetheless, since
> humans have apparently been infected by BSE, it would seem prudent
> to take reasonable measures to limit exposure of humans
> (as well as sheep and cattle) to CWD infectivity as has been
> recommended for other animal TSEs.
>
> snip...
>
> http://www.emboj.org/current.shtml
>
> Scrapie to Humans USA?
>
>
> 1: Neuroepidemiology. 1985;4(4):240-9.
>
> Sheep consumption: a possible source of spongiform encephalopathy in humans.
>
> Davanipour Z, Alter M, Sobel E, Callahan M.
>
> A fatal spongiform encephalopathy of sheep and goats (scrapie) shares many
> characteristics with Creutzfeldt-Jakob disease (CJD), a similar dementing
> illness of humans. To investigate the possibility that CJD is acquired by
> ingestion of contaminated sheep products, we collected information on
> production, slaughtering practices, and marketing of sheep in Pennsylvania.
> The study revealed that sheep were usually marketed before central nervous
> system signs of scrapie are expected to appear; breeds known to be
> susceptible to the disease were the most common breeds raised in the area;
> sheep were imported from other states including those with a high frequency
> of scrapie; use of veterinary services on the sheep farms investigated and,
> hence, opportunities to detect the disease were limited; sheep producers in
> the area knew little about scrapie despite the fact that the disease has
> been reported in the area, and animal organs including sheep organs were
> sometimes included in processed food. Therefore, it was concluded that in
> Pennsylvania there are some 'weak links' through which scrapie-infected
> animals could contaminate human food, and that consumption of these foods
> could perhaps account for spongiform encephalopathy in humans. The weak
> links observed are probably not unique to Pennsylvania.
>
>
>
> http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list
> _uids=3915057&dopt=Abstract
>
>
> Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
> nonhuman primates.
>
> Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
>
> Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of
> sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that
> were exposed to the infectious agents only by their nonforced consumption of
> known infectious tissues. The asymptomatic incubation period in the one
> monkey exposed to the virus of kuru was 36 months; that in the two monkeys
> exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months,
> respectively; and that in the two monkeys exposed to the virus of scrapie
> was 25 and 32 months, respectively. Careful physical examination of the
> buccal cavities of all of the monkeys failed to reveal signs or oral
> lesions. One additional monkey similarly exposed to kuru has remained
> asymptomatic during the 39 months that it has been under observation.
>
> PMID: 6997404
> http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui
> ds=6997404&dopt=Abstract
>
>
>
>
> 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
>
> Issues raised by a subsequent comment letter seem dubious at best in the
> case of this surgeon:
>
>
> Ridley: Lancet, Volume 341(8845).Mar 6, 1993.pp 641-642.
> The Lancet Volume 341(8845) Mar 6, 1993 pp 641-642
>
> Occupational risk of Creutzfeldt-Jakob disease.
> [Letters to the Editor]
> Ridley, R.M.; Baker,H.F.
> Division of Psychiatry, Clinical Research Centre, Harrrow, Middlesex HA1
> 3UJ, UK.
>
>
> Sir,- Readers should be cautious about Dr Weber and colleagues' (Jan 9, p
> 123) suggestion that occupational transmission of Creutzfeldt-Jakob disease
> (CJD) may have taken place in a neurosurgeon, a pathologist, 2 histology
> technicians, and an orthopaedic surgeon. Large epidemiological surveys
> (1,2) have failed to find a link between occupation and CJD. This disease
> has been reported in several people working in occupations in which exposure
> to neural tissue could have happened (eg, butchers, farmers, and various
> health professionals (3) ) but the number of these cases is not in excess
> of that which would be expected by random association. In the absence of a
> clear excess of cases, as has occurred in the iatrogenic transmission of
> spongiform encephalopathy by exposure to human derived growth hormone (4),
> the occurrence of CJD in people from the medical and paramedical professions
> is no more remarkable than its occurence in people of any other profession.
> Brown et al (1) rep!
> orted six cases among clerics, but this does not necessarily implicate their
> occupation in their ultimate demise.
>
> The notion that CJD is always acquired (as opposed to idiopathic) and that
> the existence of any hypothetical risk factor must therefore be the cause of
> the disease led to the much cited claim that the high incidence of CJD among
> Libyan Jews was due to their consumption of sheep's eyeballs (5), despite a
> lack of evidence that their dietary habits differed from their ethnic
> neighbours in whom no increased incidence of this disease was recorded. The
> high frequency of CJD in the Libyan Jews is now known to be due to a codon
> 200 mutation in the PrP gene in affected families in that ethnic group (6).
>
> CJD is a peculiar disease that does not fit into any single pattern of
> distribution. The great majority of cases cannot be attributed to
> environmental exposure. Very particular precautions are required to prevent
> transmission from cases of human and animal spongiform encephalopathy since,
> when this does occur, a major outbreak of disease can arise. Under these
> circumstances it is especially important that the occurrence of CJD is
> viewed from an epidemiological rather than an anecdotal perspective.
>
> REFERENCES
>
> 1. Brown P, Cathala F, Raubertas RF, et al. The epidemiology of
> Creutzfeldt-Jakob: conclusion of a 15-year investigation in France and a
> review of the world literature. Neurology 1987; 37: 895-904. [Medline
> Link] [Context Link]
>
> 2. Harries-Jones R, Knight R, Will RG, et al. Creutzfeldt-Jakob disease in
> England and Wales, 1980-1984; a case control study of potential risk
> factors. J Neurol Neurosurg Psychiatry 1988; 51: 1113-19. [Medline Link]
> [Context Link]
>
> 3. Masters CL, Harris JO, Gajdusek C, et al. Creutzfeldt-Jakob disease:
> patterns of worldwide occurrence and the significance of familial and
> sporadic clustering. Ann Neurol 1978; 5: 177-88. [Medline Link] [Context
> Link]
>
> 4. Brown P, Gajdusek C, Gibbs CJ, Asher DM. Potential epidemic of
> Creutzfeldt-Jakob disease from human growth hormone therapy. N Engl J Med
> 1985; 313: 728-31. [Medline Link] [Context Link]
>
> 5. Kahana E, Alter M, Braham J, Sofer D. Creutzfeldt-Jakob disease: focus
> among Libyan Jews in Israel. Science 1974; 183: 90-91. [Medline Link]
> [Context Link]
>
> 6. Hsiao K, Meiner Z, Kahana E, et al. Mutation of the prion protein in
> Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 1991; 324: 1091-97.
>
>
> ----------------------------------------------------------------------------
> ----
>
>
>
>
> Approved-By: "Roland Heynkes @ T-Online"
> Message-ID: <006a01bed26b$d48e9400$31be9ec1@pentium>
> Date: Mon, 19 Jul 1999 23:28:00 +0200
> Reply-To: Bovine Spongiform Encephalopathy
> Sender: Bovine Spongiform Encephalopathy
> From: "Roland Heynkes @ T-Online"
> Subject: Re: iatrogenic scrapie from sheep dura mater?
> Dear Tom,
>
> >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 article does not say that he was an employee of Braun Melsungen, but
> sent et least some of the lyodoras to the company. Do you have additional
> information from one of the authors and do you know if the company got
> the ovine dura maters too?
>
> >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.
> >
> I don't know if the Groschup group in Tuebingen does use a sensitive
> scrapie test like the dutch test in order to perform a random screening
> program. When I asked last time a few years ago, they "only" tested animals
> with unclear neurological symptoms.
> Scrapie still occurs in Germany, but we have less than one recorded case
> per year.
>
> >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?
> >
> Are you sure that this research with the ovine dura mater has been done
> at Braun Melsungen?
>
> best regards
>
> Roland Heynkes
>
> Erkwiesenstr. 19
> D-52072 Aachen (Germany)
> Tel.: +49 (0)241/932070
> Fax: +49 (0)241/932071
> email: roland.heynkes@t-online.de
> http://home.t-online.de/home/Roland.Heynkes
>
>
> ----------------------------------------------------------------------------
> ----
>
>
>
>
> Approved-By: tom
> Message-ID:
> Date: Tue, 20 Jul 1999 11:48:17 -0800
> Reply-To: Bovine Spongiform Encephalopathy
> Sender: Bovine Spongiform Encephalopathy
> From: tom
> Subject: Re: iatrogenic scrapie from sheep dura mater?
> In-Reply-To: <006a01bed26b$d48e9400$31be9ec1@pentium>
>
> >
> >>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 article does not say that he was an employee of Braun Melsungen, but
> >sent et least some of the lyodoras to the company. Do you have additional
> >information from one of the authors and do you know if the company got
> >the ovine dura maters too?
>
> I really do not know any more at this time than what was in the article.
> The way I read it, he was not an internal employee but an independent
> orthopaedic surgeon who had a contract to supply dura mater to the company.
> The victim's wife may be able to supply details, however the family name is
> not given.
>
> >
> >>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.
> >>
> >I don't know if the Groschup group in Tuebingen does use a sensitive
> >scrapie test like the dutch test in order to perform a random screening
> >program. When I asked last time a few years ago, they "only" tested animals
> >with unclear neurological symptoms.
> >Scrapie still occurs in Germany, but we have less than one recorded case
> >per year.
>
> Sure. According to this, it was a bit of extraordinary bad luck that any of
> 150 sheep + 12 humans could have carried the disease, but we know not to
> equate recorded cases with incidence. Portugal had very few recorded cases
> per year of BSE too.
>
> In your opinion, how exactly has it been possible for scrapie to persist in
> Germany for all these decades at this vanishingly small level? Cases are
> probably not geographically or farm-exchange linked. Surely the
> authorities don't allow live imports from England.
>
> >
> >>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?
> >>
> >Are you sure that this research with the ovine dura mater has been done
> >at Braun Melsungen?
>
> I would guess that they did not do the research there but sold it to the
> marketplace. Perhaps you could give the German authors a call (Weber,
> Thomas; Tumani, Hayrettin; Holdorff, Bernd all in Hamburg I think) I will
> shop around on Internet catalogues, see which companies today sell sheep
> dura mater for research (my guess is no one).
>
> It is annoying that so many details relevent to interpretation were left out
> of the paper. Still, any forward tracing of the dura mater will soon hit a
> brick wall at the company, Braun Melsungen- B. Carl-Braun-Str. 1
> D-34212 Melsungen Germany Tel: ++49 5661 - 71-1739, Fax: ++49 5661 -
> 71-1632. If any humans received sheep dura mater, I doubt that this will be
> disclosed or that specific recipients will be identified to their doctors.
> It is probably better to trace backwards from affected recipients -- see if
> they strain-type out to be sheep.
>
> Japan has been particularly hard hit by dura mater CJD (curious in itself)
> and researchers there might be motivated to find out what happened. I am
> not aware of agricultural agencies that would impede research over there.
>
> tom
>
> P.S. The US would never think of pooling dura mater in the same container.
> However, even after the lyodura experience, apparently we thought it safe to
> use the same rinse water on 26 consecutive dura mater donations:
>
> Creutzfeldt-Jakob Disease (CJD) in a Recipient of a U.S.-Processed Dura
> Mater Graft: Cause or Coincidence? Belay E.D.1, Dobbins, J.G.1, Malecki,
> J.2, Buck, B.E.3, Bell, M.1, Cobb, J.2, Schonberger, L.B.1, 1Centers for
> Disease Control and Prevention (CDC), Atlanta, GA; 2Palm Beach County Health
> Department, West Palm Beach, FL; 3Department of Orthopedics and
> Rehabilitation, University of Miami School of Medicine, Miami, FL.
> http://www.life.umd.edu/jifsan/tse/belay.htm
>
> In 1997, CDC was notified about a 72-year-old man who developed CJD 54
> months after he received a dura mater graft during removal of a meningioma.
> CDC confirmed that CJD diagnosis was based on standard clinical criteria,
> including typical electroencephalographic changes. Investigation of patients
> who underwent craniotomy within 4 months before or after the case-patient's
> surgery revealed no evidence for nosocomial transmission of CJD. The dura
> donor was a previously healthy 34-year-old man with no known risk factors
> fore CJD who had died in a motor vehicle collision. The dura was processed
> in the United States with no direct contact with other dura. However,
> possible indirect contact through water used to rinse dural grafts from
> about 25 other donors simultaneously could not be ruled out; tracing of
> recipients of these grafts is in progress. If this case-patient remains the
> only recipient of a U.S.-processed dural graft with CJD, then this graft-CJD
> association is more likely to be coincidental than causal. This report
> underscores the potential importance of recent recommendations to minimize
> the risk of CJD transmission by such grafts, including neuropathologic
> screening of all donors and removal of opportunities for cross-contamination
> among grafts.
>
>
>
> IA#84-03 --- 6/27/87
> http://www.fda.gov/ora/fiars/ora_import_ia8403.html
>
> BACKGROUND
>
> A recent reported case of Creutzfeldt-Jakob Disease (CJD) in a 28 year-old
> patient who had received a human dura mater graft indicates that the graft
> may
> have been the source of this always fatal disease. The woman died 22 months
> after receiving the lyophilized, irradiated human cadaveric dura mater
> graft.
> The dura mater used in the graft was packaged in October 1982 under lot
> #2105
> by B. Braun Melsungen AG of West Germany, shipped to Tri Hawk International,
> Inc., Montreal, Quebec, Canada and sold to Saint Francis Hospital, Hartford,
> Connecticut, on April 4, 1985.
>
> This is the first known case of CJD transmission associated with a dura
> mater
> graft. Present methods of sterilizing the dura mater do not completely
> inactivate the CJD agent.
>
> The dura mater is manufactured by the West German firm under the trade name
> Lyodura. Although the material is primarily used in neurosurgery, it is
> also
> used in orthopedic, otologic, dental, urologic, gynecologic, and cardiac
> surgical procedures.
>
> We have been unable to determine the total number of packages of Lyodura
> that
> were imported into the United States because the Canadian distributor failed
> to maintain adequate records of distribution for all lots which may have
> been
> distributed by mail to hospitals in the United States and Canada.
>
> As stated in the FDA Safety Alert which issued April 28, 1987, we strongly
> recommend that users of dura mater choose only products from known sources
> which retrieve, process and handle the material according to guidelines such
> as those of the American Association of Tissue Banks.
>
> To report cases or for further information, please contact:
>
> Gordon C. Johnson, M.D.
> Center for Devices and Radiological Health
> Food and Drug Administration
> 8757 Georgia Ave,
> Silver Spring, Maryland 20910
>
>
> GUIDANCE
>
> Alert your local Customs office to be aware of this import alert and to
> monitor mail shipments for this product.
>
> Detain all shipments of Lyodura (dura mater) received from Tri Hawk
> International, Inc., Montreal, Quebec, Canada or from B. Braun Melsungen AG
> of
> West Germany. Charge: "The article is subject to refusal of admission
> pursuant to section 801(a)(1) in that it appears to be adulterated under
> section 501(h), because the methods and controls used for the storage and
> distribution of Lyodura (dura mater) are not in conformance with current
> good
> manufacturing practice requirements under section 520(f)(1)."
>
> -=-=-=-=-
>
> DR. MARTIN ZEIDLER:
> http://www.life.umd.edu/jifsan/tse/zeidler.htm
> 8 June 1998
>
> Thank you very much and I'd like to start this by thanking JIFSAN for kindly
> inviting me here today.
>
> I became involved in the whole issue of Creutzfeldt-Jakob disease and dura
> mater grafts about a year ago when I had the good fortune to be working with
> the World Health Organization. It was at this time that WHO had a
> consultation addressing the issue of safety of medicinal and other products
> in relation to human and animal TSEs. This meeting recommended that dura
> mater grafts could no longer be used, which caused some controversy and I
> was involved in the debate which ensued with various dura mater
> manufacturers....
>
> So, what is dura mater? Well, it's the outer covering of the meninges, this
> is the fibrous sheath which encircles the central nervous system. It really
> has two functions, first, to keep the spinal fluid in, and, second, to stop
> infection from getting into the central nervous system. Surgical procedures
> or trauma that broach the dura mater may result in a hole, that because of
> the fibrous, inelastic nature of dura, may not be possible shut by primary
> closure. If the defect is to be filled, perhaps the obvious tissue to do
> this is a dural graft.
>
> Since the 1950s, dura mater grafts have been utilised ; some of the first
> grafts came from the U.S. Navy's Medical School here in the United States.
> The popularity of dural grafts came to the fore in the 1970s and 1980s when
> they were commercially produced. It appeared that surgeons were actually
> very happy with these particular materials - they provided a good barrier to
> infection and stopped the leakage of CSF.
>
> It was in 1987 that the first case was reported of a person with CJD who
> had previously been known to have received a dura mater graft. The patient
> was a 28-year-old woman who had an operation 18 months previously to remove
> a cholesteatoma and she subsequently developed histologically confirmed CJD.
> To date I've managed to find a total of 83 case reports of dura mater
> related CJD cases, and I am grateful Dr Paul Brown for providing me with
> data. There are 3 additions to the number which Paul mentioned during his
> talk earlier, although these three cases are slightly questionable. Two of
> these were reported from France, and both had undergone embolisation
> procedures with dura mater rather than receiving a conventional graft. The
> third case is from Thailand and has not yet been pathologically confirmed.
>
> 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
>
> 3 corneal
> 2 sterotactic
> 4 neurosurgery
> 80 dura mater
> 106 growth hormone
> ...25 US (includes 5 New Zealand + 1 Brazilian case using US-prepared
> hormone)
> ...28 UK
> ...53 France
> 4 gonadotrophin Approved-By: "Roland Heynkes @ T-Online"
>
> Message-ID: <001601bed3c7$60a91940$0f2d9c3e@pentium>
> Date: Wed, 21 Jul 1999 11:33:00 +0200
> Reply-To: Bovine Spongiform Encephalopathy
> Sender: Bovine Spongiform Encephalopathy
> From: "Roland Heynkes @ T-Online"
> Subject: Re: iatrogenic scrapie from sheep dura mater?
> Dear Tom,
>
> >>>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 article does not say that he was an employee of Braun Melsungen, but
> >>sent et least some of the lyodoras to the company. Do you have additional
> >>information from one of the authors and do you know if the company got
> >>the ovine dura maters too?
> >
> >I really do not know any more at this time than what was in the article.
> >The way I read it, he was not an internal employee but an independent
> >orthopaedic surgeon who had a contract to supply dura mater to the company.
> >The victim's wife may be able to supply details, however the family name
> >is not given.
> >
> Perhaps the journal's editors were happy to demonstrate with this ambiguous
> letter the advantages of peer reviewing.
>
> >>>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.
> >
> >>I don't know if the Groschup group in Tuebingen does use a sensitive
> >>scrapie test like the dutch test in order to perform a random screening
> >>program. When I asked last time a few years ago, they "only" tested
> animals
> >>with unclear neurological symptoms.
> >>Scrapie still occurs in Germany, but we have less than one recorded case
> >>per year.
> >
> >Sure. According to this, it was a bit of extraordinary bad luck that any
> >of 150 sheep + 12 humans could have carried the disease, but we know not
> >to equate recorded cases with incidence. Portugal had very few recorded
> >cases per year of BSE too.
> >
> Of course the incidence must be higher than the number of recorded cases
> and the Lelystad-scrapie-test should be used in order to approach the real
> incidence of scrapie in Germany. But in Germany sheep unlike cows are not
> high productivity farm animals that become killed very young and especially
> when their productivity decreases. Therefore in my opinion scrapie infected
> sheep have a good chance to develop symptomes and if a vet observes such
> neurological symptomes, governmental vets including the central German lab
> for scrapie diagnosis in Tuebingen will check it histopathologically.
>
> >In your opinion, how exactly has it been possible for scrapie to persist
> >in Germany for all these decades at this vanishingly small level?
> >
> A good question that I can not answer. One reason may be the use of
> occationally contaminated animal meal. But of course a much higher
> incidence of sublethal infected sheep is also possible.
>
> >Cases are probably not geographically or farm-exchange linked.
> >Surely the authorities don't allow live imports from England.
> >
> Unfortunately importing british sheep has never been forbidden in
> Germany. In my opinion this is extreemly stupid and may be a further
> reason for scrapie in Germany.
>
>
> >>>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?
> >
> >>Are you sure that this research with the ovine dura mater has been done
> >>at Braun Melsungen?
> >
> >I would guess that they did not do the research there but sold it to
> >the marketplace. Perhaps you could give the German authors a call
> >(Weber, Thomas; Tumani, Hayrettin; Holdorff, Bernd all in Hamburg I
> >think)
> >
> Thomas Weber at least was at the Institut for Neurology, University of
> Goettingen, but we have hundreds of Thomas Weber in Germany and at least
> seven around Goettingen. But perhaps I can find an email-address on the
> Institute site.
>
> >It is annoying that so many details relevent to interpretation were
> >left out of the paper. Still, any forward tracing of the dura mater
> >will soon hit a brick wall at the company, Braun Melsungen- B.
> >Carl-Braun-Str. 1 D-34212 Melsungen Germany Tel: ++49 5661 - 71-1739,
> >Fax: ++49 5661 - 71-1632. If any humans received sheep dura mater,
> >I doubt that this will be disclosed or that specific recipients will
> >be identified to their doctors. It is probably better to trace
> >backwards from affected recipients -- see if they strain-type out
> >to be sheep.
> >
> This letter to the editor is indeed of such a low quality that it hardly
> could be worse if it were published on internet instead of a well known
> journal.
>
> >P.S. The US would never think of pooling dura mater in the same container.
> >However, even after the lyodura experience, apparently we thought it safe
> >to use the same rinse water on 26 consecutive dura mater donations:
> >
> unbelievable clever!
>
> best regards
>
> Roland Heynkes
>
> Erkwiesenstr. 19
> D-52072 Aachen (Germany)
> Tel.: +49 (0)241/932070
> Fax: +49 (0)241/932071
> email: roland.heynkes@t-online.de
> http://home.t-online.de/home/Roland.Heynkes
> ----------------------------------------------------------------------------
> ---->Cases are probably not geographically or farm-exchange linked.
> >Surely the authorities don't allow live imports from England.
> >
> Unfortunately importing british sheep has never been forbidden in
> Germany. In my opinion this is extreemly stupid and may be a further
> reason for scrapie in Germany. ...
>
>
>
> 0208h023: UK exports of sheep, goats and sheep/goat meats and meat products
> (1988 - 2001)
>
>
> http://www.vegsource.com/articles/sheep_exports.htm
>
>
>
> Infected and Source Flocks
>
> As of August 31, 2005, there were 115 scrapie infected and source flocks
> (figure 3). There were 3 new infected and source flocks reported in August
> (Figure 4) with a total of 148 flocks reported for FY 2005 (Figure 5). The
> total infected and source flocks that have been released in FY 2005 are 102
> (Figure 6), with 5 flocks released in August. The ratio of infected and
> source flocks released to newly infected and source flocks for FY 2005 =
> 0.69 :
> 1. In addition, as of August 31, 2005, 574 scrapie cases have been confirmed
> and reported by the National Veterinary Services Laboratories (NVSL), of
> which 122 were RSSS cases (Figure 7). This includes 55 newly confirmed cases
> in August 2005 (Figure 8). Fifteen cases of scrapie in goats have been
> reported since 1990 (Figure 9). The last goat case was reported in May 2005.
>
> snip...
>
> full text ;
>
> http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.htm
> l
>
>
>
>
> Published online before print October 20, 2005
>
> Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
> Medical Sciences
>
> A newly identified type of scrapie agent can naturally infect sheep with
> resistant PrP genotypes
>
> ( sheep prion | transgenic mice )
>
> Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *,
> Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||,
> Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
> *Virologie Immunologie Moléculaires and ||Génétique Biochimique et
> Cytogénétique, Institut National de la Recherche Agronomique, 78350
> Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la
> Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions
> Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité
> Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,
> 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut
> National de la Recherche Agronomique, 37380 Nouzilly, France; and
> ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway
>
>
> Edited by Stanley B. Prusiner, University of California, San Francisco, CA,
> and approved September 12, 2005 (received for review March 21, 2005)
>
> Scrapie in small ruminants belongs to transmissible spongiform
> encephalopathies (TSEs), or prion diseases, a family of fatal
> neurodegenerative disorders that affect humans and animals and can transmit
> within and between species by ingestion or inoculation. Conversion of the
> host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a
> misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission
> and pathogenesis. The intensified surveillance of scrapie in the European
> Union, together with the improvement of PrPSc detection techniques, has led
> to the discovery of a growing number of so-called atypical scrapie cases.
> These include clinical Nor98 cases first identified in Norwegian sheep on
> the basis of unusual pathological and PrPSc molecular features and "cases"
> that produced discordant responses in the rapid tests currently applied to
> the large-scale random screening of slaughtered or fallen animals.
> Worryingly, a substantial proportion of such cases involved sheep with PrP
> genotypes known until now to confer natural resistance to conventional
> scrapie. Here we report that both Nor98 and discordant cases, including
> three sheep homozygous for the resistant PrPARR allele (A136R154R171),
> efficiently transmitted the disease to transgenic mice expressing ovine PrP,
> and that they shared unique biological and biochemical features upon
> propagation in mice. These observations support the view that a truly
> infectious TSE agent, unrecognized until recently, infects sheep and goat
> flocks and may have important implications in terms of scrapie control and
> public health.
>
>
>
> ----------------------------------------------------------------------------
> ----
>
> Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,
> T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.
> contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;
> and H.L. wrote the paper.
>
> A.L.D. and V.B. contributed equally to this work.
>
> To whom correspondence should be addressed.
>
> Hubert Laude, E-mail: laude@jouy.inra.fr
>
> www.pnas.org/cgi/doi/10.1073/pnas.0502296102
>
>
> http://www.pnas.org/cgi/content/abstract/0502296102v1
>
>
> TSS
>
>
>
>
> ----- Original Message -----
> From: "Jim Woodward"
> To: "'Shu Chen'"
> Sent: Saturday, December 17, 2005 11:02 PM
> Subject: RE: Human resistance to scrapie?
>
>
> > Dr. Chen,
> >
> > Thanks for your reply. The human lifespan at the end of the eighteenth
> > century was in the mid-30s, and was only about ten years higher at the end
> > of the nineteenth century. That would not seem to be long enough for the
> > onset of scrapie-induced CJD. In fact, CJD was not described as a disease
> > until the 1920s. Is it appropriate to make such a blanket statement about
> > human resistance to scrapie and to dismiss the Marsh experiments?
> >
> > Kind regards,
> >
> > Jim
> >
> >
> >
> > > -----Original Message-----
> > > From: Shu Chen [mailto:shu.chen@case.edu]
> > > Sent: Saturday, December 17, 2005 8:49 AM
> > > To: Jim Woodward
> > > Subject: Re: Human resistance to scrapie?
> > >
> > > Jim,
> > > Thank you for your interest. The resistance of humans to
> > > scrapie is due to the fact that sheep naturally develop
> > > scrapie and consumption of sheep by humans in several
> > > hundreds of years since scrapie is known does not seem to be
> > > a cause for CJD. I have no specific reference on this, but
> > > you may refer to many prion reviews by Prusiner, some of
> > > which may have mentioned this.
> > > Best,
> > > Shu G. Chen, Ph.D.
> > > Institute of Pathology, Rm 406
> > > Case Western Reserve University
> > > 2085 Adelbert Road
> > > Cleveland, OH 44106-4907
> > > 216)368-8925; (216)368-2546-fax
> > > sxc59@cwru.edu
> > >
> > > ----- Original Message -----
> > > From: Jim Woodward
> > > Date: Friday, December 16, 2005 0:41 am
> > > Subject: Human resistance to scrapie?
> > > > Dear Dr. Chen,
> > > >
> > > > I recently read your very interesting paper, CHRONIC
> > > WASTING DISEASE
> > > > OF ELK AND DEER AND CREUTZFELDT-JAKOB DISEASE: COMPARATIVE
> > > ANALYSIS OF
> > > > THE SCRAPIE PRION PROTEIN. Near the end of the discussion section,
> > > > you assert that the transmission of CWD to squirrel monkey
> > > by Marsh,
> > > > et al. is not relevant to the question of human
> > > susceptibility to CWD
> > > > since squirrel monkeys are "very permissive to prions of different
> > > > species including sheep scrapie to which humans are resistant". In
> > > > contrast to the rest of your paper which is carefully
> > > referenced, your
> > > > statement that humans are resistant to scrapie is not
> > > referenced. Was
> > > > this an oversight? Are there data to support thisstatement?
> > > >
> > > > Regards,
> > > >
> > > > Jim Woodward
> > > > Wellington, Colorado


THE UKnvCJDBSE only theory is only spreading this agent more.

THE diagnostic criteria for human TSE must be readdressed;


http://neurology.thelancet.com Published online October 31, 2005


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?


http://neurology.thelancet.com Published online October 31, 2005

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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


http://jama.ama-assn.org/cgi/content/full/285/6/733

Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.

Full Text

Tue, 13 Feb 2001 JAMA Vol. 285 No. 6, February 14, 2001 Letters

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor:

In their Research Letter in JAMA. 2000;284:2322-2323, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

To the Editor:

At the time of my mother's death, various diagnoses were advanced such as "rapid progressive Alzheimer disease," psychosis, and dementia. Had I not persisted and personally sought and arranged a brain autopsy, her death certificate would have read cardiac failure and not CJD.

Through CJD Voice1 I have corresponded with hundreds of grief-stricken families who are so devastated by this horrific disease that brain autopsy is the furthest thing from their minds. In my experience, very few physicians suggest it to the family. After the death and when families reflect that they never were sure what killed their loved one it is too late to find the true cause of death. In the years since my mother died I think that the increasing awareness of the nature of CJD has only resulted in fewer pathologists being willing to perform an autopsy in a suspected case of CJD.

People with CJD may die with incorrect diagnoses of dementia, psychosis, Alzheimer disease, and myriad other neurological diseases. The true cause of death will only be known if brain autopsies are suggested to the families. Too often the physician's comment is, "Well, it could be CJD but that is so rare it isn't likely."

Until CJD is required to be reported to state health departments, as other diseases are, there will be no accurate count of CJD deaths in the United States and thus no way to know if the number of deaths is decreasing, stable, or increasing as it has recently in the United Kingdom.

Dorothy E. Kraemer Stillwater, Okla

In Reply:

Mr Singeltary and Ms Kraemer express an underlying concern that our recently reported mortality surveillance estimate of about 1 CJD case per million population per year in the United States since 1985 may greatly underestimate the true incidence of this disease. Based on evidence from epidemiologic investigations both within and outside the United States, we believe that these national estimates are reasonably accurate.

Even during the 1990s in the United Kingdom, where much attention and public health resources have been devoted to prion disease surveillance, the reported incidence of classic CJD is similar to that reported in the United States.

In addition, in 1996, active US surveillance for CJD and new variant (nv) CJD in 5 sites detected no evidence of the occurrence of nvCJD and showed that 86% of the CJD cases in these sites were identifiable through routinely collected mortality data.

Our report provides additional evidence against the occurrence of nvCJD in the United States based on national mortality data analyses and enhanced surveillance. It specifically mentions a new center for improved pathology surveillance. We hope that the described enhancements along with the observations of Singeltary and Kraemer will encourage medical care providers to suggest brain autopsies for more suspected CJD cases to facilitate the identification of potentially misdiagnosed CJD cases and to help monitor the possible occurrence of nvCJD.

Creutzfeldt-Jakob disease is not on the list of nationally notifiable diseases. In those states where surveillance personnel indicate that making this disease officially notifiable would meaningfully facilitate collection of data that are needed to monitor the incidence of CJD and nvCJD, including the obtaining of brain autopsy results, we encourage such a change. However, adding CJD to the notifiable diseases surveillance system may lead to potentially wasteful, duplicative reporting because the vast majority of the diagnosed cases would also be reported through the mortality surveillance system.

Furthermore, making CJD a notifiable disease may not necessarily help identify undiagnosed CJD cases. The unique characteristics of CJD make mortality data a useful surrogate for ongoing surveillance. Unlike many other neurologic diseases, CJD is invariably fatal and in most cases rapidly progressive and distinguishable clinically from other neurologic diseases.

Because CJD is least accurately diagnosed early in the course of the illness, notifiable disease surveillance of CJD could be less accurate than mortality surveillance of CJD. In addition, because death as a condition is more completely and consistently reported, mortality surveillance has the advantage of being ongoing and readily available.

The absence of CJD and nvCJD from the list of nationally notifiable diseases should not be interpreted to mean that they are not important to public health; this list does not include all such diseases. We encourage medical caregivers to report to or consult with appropriate public health authorities about any diagnosed case of a transmissible disease for which a special public health response may be needed, including nvCJD, and any patient in whom iatrogenic transmission of CJD may be suspected.

Robert V. Gibbons, MD, MPH Robert C. Holman, MS Ermias D. Belay, MD Lawrence B. Schonberger, MD, MPH Division of Viral and Rickettsial Diseases National Center for Infectious Diseases Centers for Disease Control and Prevention Atlanta, Ga


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

Singeltary, Sr et al. JAMA


Neurology 2003;60:176-181
© 2003 American Academy of Neurology


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


VIEWS & REVIEWS:
Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, and Lawrence B. Schonberger

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Neurology 2003; 60: 176-181 [Abstract] [Full text] [PDF]


Correspondence published:

Reply to Singletary
Ryan A. Maddox, MPH, Ermias D. Belay, MD, Lawrence B. Schonberger, MD (26 March 2003)

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary (26 March 2003)

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

Send Post-Publication Peer Review to journal:


Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States


Email Terry S. Singeltary:


flounder@wt.net


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


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


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

LANCET INFECTIOUS DISEASE JOURNAL


Volume 3, Number 8 01 August 2003


Newsdesk


Tracking spongiform encephalopathies in North America


Xavier Bosch

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

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

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

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


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

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

rapidly progressive dementia compatible with spontaneous

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

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

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

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

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

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

people, it did uncover a largely disappointing situation.


Singeltary was greatly demoralised at the few attempts to monitor the

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

reportable. Human and animal TSEs should be reportable nationwide and

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

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

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

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


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

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

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

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

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.


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

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

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

plausibility of CWD spreading to people has remained elusive.


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

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

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.


Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

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

ingredient in feed for any animal species, epidemiological control and

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

UK and Europe regarding BSE.


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

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

and only what they want you to have.


Norman Foster, director of the Cognitive Disorders Clinic at the

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

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

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

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

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

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

system that identifies and analyses a high proportion of suspected prion

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

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


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

reported the cases of three hunters two of whom were friends who died

from pathologically confirmed CJD, says that at present there are

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

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

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

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.


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

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

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

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

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

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

limited.

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


BRITISH MEDICAL JOURNAL


SOMETHING TO CHEW ON


BMJ


Terry S Singeltary,
NA
medically retired
Send response to journal:
Re: Re: vCJD in the USA * BSE in U.S.

In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease. Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know. My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD. The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?

CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997. So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.

No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;

Since 1990 the U.S. has raised 1,250,880,700 cattle;

Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;

There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;

Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;

Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.

I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.

Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.

It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........

The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.

Terry S. Singeltary Sr.
P.O. Box 42, Bacliff, Texas 77518 USA
flounder@wt.net

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


BMJ

2 January 2000

Terry S Singeltary
retired
Send response to journal:
Re: U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well...


In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."

A few more factors to consider, page 15;

"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."

"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."

"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."

Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow. Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.

To be continued...

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


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

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

TSS



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