Communication
Horse Prion Protein NMR Structure and Comparisons with Related Variants of the Mouse Prion Protein
Daniel R. Pérez†, a, Fred F. Damberger†, a and Kurt Wüthrich, a, ,
a Institute of Molecular Biology and Biophysics, ETH Zurich, Schafmattstrasse 20, CH-8093 Zurich, Switzerland
Received 2 March 2010; revised 30 April 2010; accepted 30 April 2010. Edited by M. F. Summers. Available online 10 May 2010.
Abstract
The NMR structure of the horse (Equus caballus) cellular prion protein at 25 °C exhibits the typical PrPC [cellular form of prion protein (PrP)] global architecture, but in contrast to most other mammalian PrPCs, it contains a well-structured loop connecting the ß2 strand with the a2 helix. Comparison with designed variants of the mouse prion protein resulted in the identification of a single amino acid exchange within the loop, D167S, which correlates with the high structural order of this loop in the solution structure at 25 °C and is unique to the PrP sequences of equine species. The ß2–a2 loop and the a3 helix form a protein surface epitope that has been proposed to be the recognition area for a hypothetical chaperone, “protein X,” which would promote conversion of PrPC into the disease-related scrapie form and thus mediate intermolecular interactions related to the transmission barrier for transmissible spongiform encephalopathies (TSEs) between different species. The present results are evaluated in light of recent indications from in vivo experiments that the local ß2–a2 loop structure affects the susceptibility of transgenic mice to TSEs and the fact that there are no reports on TSE in horses.
Keywords: cellular horse prion protein; transmissible spongiform encephalopathy; NMR structure determination; protein structure; ß2–a2 loop
Abbreviations: PrP, prion protein; PrPC, cellular form of PrP; PrPSc, scrapie form of PrP; ecPrP, horse PrP; mPrP, mouse PrP; TSE, transmissible spongiform encephalopathy; NOE, nuclear Overhauser enhancement; NOESY, NOE spectroscopy; ePrP, elk PrP; bvPrP, bank vole PrP
http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WK7-5023KTP-2&_user=4973225&_coverDate=05%2F10%2F2010&_rdoc=1&_fmt=high&_orig=search&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=4973225&md5=f13341b4309d957a836f844109ef4733
Equine Spongiform Encephalopathy
THERE was a case of a horse with suspect Bovine Spongiform Encephalopathy and or another scrapie-like TSE, that was fed cattle cake. IN fact there were a report of similar cases in other horses, but as with the hounds with TSE, those studies were all ignored and finally just set aside due to all the other species going down with BSE. ...TSS
IN CONFIDENCE
SUSPECT BSE IN A HORSE
CYO BSE 1 9
IN CONFIDENCE
SUSPECT BSE IN A HORSE
The Parliamentary Secretary (Mr Maclean) will wish to be aware that, in making his differential diagnosis, a veterinary surgeon in the Reading area has included the possibility of BSE in a horse under his care. Although it is unlikely to be BSE, because of the symptoms exhibited the veterinarian believes that he cannot exclude the possibility. The case was brought to the notice of one of the veterinary staff at the CVL by the owner's veterinary surgeon and liaison is being maintained.
The horse in question is a five-year old eventing gelding which was purchased by the present owner about four months ago. Approximately two months after purchase the animal became a little apprehensive, developed mild nervous symptoms and became over-sensitive to noise. The nervous symptoms have increased and the horse is now practically impossible to ride. Investigations by the owner's private veterinary surgeon are continuing but it is likely that the animal will have to be destroyed.
If the horse should die or be destroyed, a full post-mortem examination will be required for insurance purposes and will probably be carried out at a non-Ministry laboratory. However, Mr Bradley of the Pathology Department, CVL, has informed the private veterinary surgeon that he is willing to provide a second opinion on the brain histology if requested.
I will keep the Parliamentary Secretary informed of any further developments in the case.
I CRAWFORD
14 May 1990
Mr M P H Hill, PS/Parliamentary secretary (Mr Maclean) - by FAX
cc:
Private Offices
Mr K C Meldrum
Mrs E A J Attridge D J Evans Mr K C Taylor Mr R Lawson Mr R Bradley. CVL
(hand written notes i cannot read all (cut short) as follows...tss)
The Parliamentary Secretary (Mr Maclean was grateful for this. He said that we must keep very close to ...on it, and when the horse dies, or is put down we must be told immediately. He also feels it is very important that our veterinary staff be involved in the brain examination. .........(cannot read the rest .............TSS)
90/05.14/10.1
http://collections.europarchive.org/tna/20080102214337/http://www.bseinquiry.gov.uk/files/yb/1990/05/14010001.pdf
1. Mr G S Podmore
PS/Secretary of State
cc PS/Permanent Secretary Mr .T W Preston Mr .T I Davies Mr D R Williams Mr D Summers
POSSIBLE SPONGIFORM ENCEPHALOPATHY IN A HORSE
You will wish to be aware that I have just received a note from Mr D Summers, the DRVO, that a possible case of a Spongiform Encephalopathy (SE) has been reported in a horse in the Brecon area.
The local veterinary surgeon reported yesterday afternoon a suspicion that a horse had contracted SE as it was showing clinical- symptoms similar to those described in cattle suffering from BSE. By yesterday evening the horse was in a comatose state and on humane grounds was destroyed by the veterinary surgeon.
At his request a full post mortem and a laboratory investigation will be started at the Carmarthen Veterinary Investigation Centre this morning in an attempt to ascertain the exact course. This will probably take at least 2 weeks. Charges to the veterinary surgeon have been waived in this instance.
Points particularly mentioned by the veterinary surgeon about this case were that the premises had an apparently similar case in another of their horses, several months ago; and that the horses had been fed cattle cake. Clearly. in his mind, there is a suspicion of a link with BSE; It must be emphasised. however, that at present we cannot even be certain that an SE of any sort is involved. These events are not exactly confidential but clearly no one is anxious to go out of their way to publicise them.
Any further information will be passed on as soon as it is available.
26th June 1990
ALUN HUWS WOAD2A
Note by The BSE Inquiry Secretariat The attached document may now be found at the following locations 90/06.26/10-1
90/06.26/9.1
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/06/26009001.pdf
Mr A Huws Principal WOAD2A CP2
SUSPECT BSE IN A HORSE
You will wish to be aware that on Thursday afternoon 25 June the T/DVO Powys received a phone call from a veterinary Surgeon reporting his suspicion that a horse had contracted BSE after having been fed cattle cake. The clinical symptoms described were similar to those shown by cattle there being a similar case some months ago on the same premises.
The owner' s name and address is:
Irene Thomas J Thomas & Company Riding Stables Penybryn Llangorse Brecon
The horse is a 12 year old gelding used for pony trekking.
By yesterday evening the horse was in a comatose state and on humane grounds was destroyed by the veterinary Surgeon. At his request a full post mortem and laboratory investigation will be carried out at the Carmarthen Veterinary Investigation Centre this morning to ascertain the exact cause; I have been told this will take at least two weeks. Charges to the veterinary Surgeon have been waived in this instance.
I will inform you immediately I receive a diagnosis.
26 June 1990
D SUMMERS DRVO
cc
Mr D R Williams, RVO
Mr A R Hunter, SVIO
90/06.26/10.1
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/06/26010001.pdf
Greetings,
PLEASE NOTE, TWO WEEKS, TWO MONTHS, TWO YEARS, TWO DECADES PASSED, AND NO OTHER MENTION OF THE CASE OF BSE TO HORSE WAS EVER MENTIONED AGAIN, that I could find. The above links were the last ever mentioned of these suspect cases of BSE in Horses, and the pathology reports there from were never furnished. SO, in my opinion, since the silence was so deafening, the BSE case to HORSE must have been positive, or they would have proudly displayed the pathology report negating the BSE positive in the Horse. ...TSS
BSE-NON-CONFIRMATION OF DISEASE
3. A question posed by Mr Whaley (para 2) is that classical lesions of BSE may not occur in all cases. Supposing we had a strain variant that produced it's lesions in the cerebrum these would not be detected by our current method. I think this would be unlikely but not impossible - another reason why at least a proportion of complete brains (or blocks) should be retained during the epidemic so if the problem Mr Whaley indicates escalates, it can be investigated.
snip...
5. IF you had the information what benefit would there be ? what would you do with it ?
CONCLUSION
I do not recommend any action. The situation should be accepted. I do not think the VIS can do more at present. The situation should be kept under review particularly if there is an escalation in numbers in this category.
R BRADLEY
15 MAY 1990
90/5.15/3.2
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/05/15003001.pdf
BSE DIAGNOSED AT LASSWADE
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/12/03008001.pdf
IN CONFIDENCE
Scrapie-like spongiform encephalomyelopathy in a domestic cat: report of a confidential consultation with the Department of Pathology, School of Veterinary Science, University of Bristol, Langford, Bristol .....
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/05/08003001.pdf
TSE & HOUNDS
GAH WELLS (very important statement here...TSS)
HOUND STUDY
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://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
76 pages on hound study;
http://web.archive.org/web/20030327022236/http://www.bseinquiry.gov.uk/files/sc/seac16/tab04.pdf
2005
DEFRA Department for Environment, Food & Rural Affairs
Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk
GTN: FAX:
Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518
21 November 2001
Dear Mr Singeltary
TSE IN HOUNDS
Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.
As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.
Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.
Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to peer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less
critical. For more details see- http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf
As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.
Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK
You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.
I hope this is helpful
Yours sincerely 4
HUGH MCDONAGH BSE CORRESPONDENCE SECTION
======================================
http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf
MAD DOGS AND ENGLISHMAN
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/11/28001001.pdf
TSE in dogs have not been documented simply because OF THE ONLY STUDY, those brain tissue samples were screwed up too. see my investigation of this here, and to follow, later follow up, a letter from defra, AND SEE SUSPICIOUS BRAIN TISSUE SAF's. ...TSS
http://www.mad-cow.org/00/aug00_late_news.html#ggg
HOUND SURVEY
I am sorry, but I really could have been a co-signatory of Gerald's minute.
I do NOT think that we can justify devoting any resources to this study, especially as larger and more important projects such as the pathogenesis study will be quite demanding.
If there is a POLITICAL need to continue with the examination of hound brains then it should be passed entirely to the VI Service.
J W WILESMITH Epidemiology Unit 18 October 1991
Mr. R Bradley
cc: Mr. G A H Wells
http://collections.europarchive.org/tna/20081106102318/http://www.bseinquiry.gov.uk/files/yb/1991/10/18001001.pdf
3.3. Mr R J Higgins in conjunction with Mr G A Wells and Mr A C Scott would by the end of the year, indentify the three brains that were from the ''POSITIVE'' end of the lesion spectrum.
http://collections.europarchive.org/tna/20080103034308/http://www.bseinquiry.gov.uk/files/yb/1993/12/06001001.pdf
37. Putative TSE in hounds - work started 1990 -(see para 41)
Robert Higgins, a Veterinary Investigation Officer at Thirsk, had been working on a hound survey in 1990. Gerald Wells and I myself received histological sections from this survey along with the accompanying letter (YB90/11.28/1.1) dated November 1990. This letter details spongiform changes found in brains from hunt hounds failing to keep up with the rest of the pack, along with the results of SAF extractions from fresh brain material from these same animals. SAFs were not found in brains unless spongiform changes were also present. The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.
38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.
39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.
40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.
41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.
Histopathological support to various other published MAFF experiments
42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).
http://www.bseinquiry.gov.uk/witness/htm/stat067.htm
1. I have had no further submission of material or communication regarding this survey since January 1991.
http://collections.europarchive.org/tna/20080103024918/http://www.bseinquiry.gov.uk/files/yb/1991/10/17001001.pdf
HOUND SURVEY PATHOLOGICAL REPORT (see positive results)
http://web.archive.org/web/20030605233318/http://www.bseinquiry.gov.uk/files/yb/1990/11/28001001.pdf
28. Concluding, Dr Tyrrell said that there was a range of opinions in the Committee from those who thought further work a waste of time to those who wished to do limited further experiments using immunocytochemistry. The Committee did not suggest transmission studies and thought that the lack of clinical data was a major weakness. Hounds were initially studied on the recommendation of the Southwood Committee because they were perceived as a 'high risk' population exposed to large quantities of potentially infective bovine tissues. Since the, however, a range of other species had been identified with TSEs, and the study of hounds was therefore less critical.
Idiopathic Brain Stem Neuronal Chromatolysis (IBNC)
29. Mr Bradley described the results of transmission studies in mice from brains of two cows with IBNC (paper SEAC 19/8). At the previous meeting of SEAC, and at the review of R&D, it had been announced that there was no clinical observation of a scrapie-like disease in mice: this information had proved to be incorrect for a number of reasons. ...
see full text ;
95/6.21/5.8
http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf
see more here ;
http://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html
I think it is time to call a temporary halt to the submission of samples. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1991/06/28010001.pdf
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1991/10/04003001.pdf
HOUND SURVEY
I am sorry, but I really could have been a co-signatory of Gerald's minute.
I do not think that we can justify devoting any resources to this dtudy, especially as larger and more important projects such as the pathogenesis study will be quite demanding.
If there is a POLITICAL need to continue with the examination of hound brains then it should be passed entirely to the VI Service.
J W WILESMITH Epidemiology Unit 18, October 1991
Mr. R Bradley
cc: Mr G A H Wells
91/10.18/1.1
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1991/10/18001001.pdf
CONFIDENTIAL
Confidential cats/dogs and unsatisfactory posture MAFFs failure to assure key research
3. First, I am very uneasy about the relative lack of urgency and interest that MAFF appear to hold for getting the necessary research programme on BSE and related encephalopathies started, and getting it going fast. FOR EXAMPLE, MR BRADLEY of CVL said that there were difficulties in organizing transmission experiments from the brain of the cat which died of an encephalopathy in Bristol. There were arguments going on about who should pay for this work. Should it be MAFF, the Bristol Veterinary School or someone else? Dr. Tyrrell was clearly exasperated.
snip...
11. The Committee were even LESS FORTHCOMING on what their reaction might be if an encephalopathy is found in another species, perhaps in DOGS. Their first reaction was that, as with the cats, the first step could be to investigate whether this was really a new disease, or simply one that had not previously been recognized and to see whether it has any links to BSE, scrapie or other transmissible encephalopathies. Indeed, some members of the Committee seem to regard the whole question of another species as a hypothetical question to be addressed only when it happened. A rather UNSATISFACTORY POSTURE.
12. In advance of your meeting with Dr Tyrrell on Monday morning, I have not voiced my ANXIETIES about the support the Committee is receiving from MAFF to anyone OTHER THAN DR PICKLES. ...
http://www.bseinquiry.gov.uk/files/yb/1990/06/14006001.pdf
NEW URL
http://web.archive.org/web/20030605151602/http://www.bseinquiry.gov.uk/files/yb/1990/06/14006001.pdf
Nature 413, 760 (25 October 2001) doi:10.1038/35101729
Brain mix-up leaves BSE research in turmoil Declan Butler
In a report that could have plunged the British meat industry back into crisis, parliament was due to receive research results this week confirming that traces of bovine spongiform encephalopathy (BSE) had been found in sheep.But at the eleventh hour, the Department for Environment, Food and Rural Affairs (DEFRA) declared that the experiments conducted by the Institute for Animal Health (IAH) near Newbury, Berkshire, were suspect.
http://www.nature.com/nature/journal/v413/n6858/full/413760a0.html
Subject: BSE IN SHEEP? COVER-UP OR STUPIDITY OR BOTH? (Lord Hansard)
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Thu, 25 Oct 2001 10:22:40 -0700
Content-Type: text/plain
Parts/Attachments: text/plain (69 lines)
Reply
######## Bovine Spongiform Encephalopathy
Table of Contents for Monday 22 Oct 2001
Volume No. 627 Part No. 30
snip...
I understand from the Statement that it was decided that the announcement would be left and that there was not a cover-up. Many of us are very concerned that, by not making a Statement, a cover-up has in fact happened. The Statement itself goes on to say that there was little press interest following the announcement by Margaret Beckett. We are then told that the experiment may not be conclusive and that it does not always excite the media. Do the Government read the news? Certainly in the past few days the press reports have highlighted this problem, and it does indeed look as though there has been a cover-up.
We acknowledge the fact and are pleased that many of the 3,000 abattoir sheep brains have indicated no scrapie cases at all. Contingency plans published on 28th September estimate that the testing of the some 20 million lambs or so entering the food chain might cost £400 million annually and question whether that would be feasible. The date of 28th September brought to mind the fact that Elliot Morley made an announcement that day, saying that in all matters relating to BSE and animal health the Government's handling had been open and transparent. I question that. On the same day Mr Morley also announced that it was a possibility that all the sheep in the country might have to be culled. Does it not seem very strange that various announcements and suggestions have been made, and yet there has been no open statement until today?
I accept that it is too early for us to have firm conclusions from the experiments that are going on. However, does the noble Lord agree that Professor Bostock said almost a year ago that he was concerned that there might be a mix-up between the brains being tested? If there was concern, why did nothing happen until just recently? Either there was concern and the Government took action, or the Government were not aware of the need for action; in which case it begs the question as to how MAFF was running its department.
Another statement was made was that British baby food did not contain British lamb. I should be grateful if the Minister would offer clarification on that point. My understanding is that on one day it was said that baby food did not contain any British lamb and the next day the manufacturers said that it did. Can the noble Lord clarify for us whether it is safe for British babies to eat British lamb?
22 Oct 2001 : Column 832
snip...
http://www.publications.parliament.uk/pa/ld199697/ldhansrd/pdvn/lds01/text/11022-06.htm#11022-06_head0
also;
http://www.publications.parliament.uk/pa/ld199697/ldhansrd/pdvn/lds01/text/11022-07.htm
also;
http://www.publications.parliament.uk/pa/ld199697/ldhansrd/pdvn/lds01/text/11022-08.htm
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
Subject: No sign of BSE in sheep ???
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Thu, 18 Oct 2001 09:11:02 -0700
Content-Type: text/plain
Parts/Attachments: text/plain (78 lines)
Reply
######## Bovine Spongiform Encephalopathy
No sign of BSE in sheep - Defra
Tests to discover whether BSE is affecting currently sheep have so far shown no signs of the disease, according to Government scientists.
But further research to establish whether it was in the UK flock in the early 1990s may be flawed, according to the Department for the Environment, Food and Rural Affairs (Defra).
Samples taken from sheep at the time may have become contaminated and the release of the test results, scheduled for Friday, has now been postponed.
Scientists have previously expressed fears that BSE may have been passed between cows and sheep because it behaves like the sheep disease scrapie.
Lucian Hudson, director of the communications directorate at Defra, said: "Research has been under way for some time to see if it can be established whether BSE might have been present in the sheep flock in the early 1990s and masked as scrapie.
"Some work has also been undertaken by the Veterinary Laboratories Agency. They have been seeking to establish whether BSE might be in sheep now.
"The results so far on about 180 TSE (Transmissible Spongiform Encephalopathy) affected brains have not shown BSE.
"The work on material from the early 1990s has been conducted by the Institute for Animal Health (IAH) and it was anticipated that some results would be ready to present to the Spongiform Encephalopathy Advisory Committee (SEAC) on Friday.
"However Defra, who commissioned the work, also commissioned cross-checking to guard against the possibility of material being contaminated by cattle brains, not least because it had been collected for entirely different experiments.
"This cross-checking has indeed raised doubts about the viability of the original sample and the SEAC chairman decided that Friday's meeting should be postponed."
Story filed: 00:40 Thursday 18th October 2001
http://www.ananova.com/news/story/sm_426645.html
could someone please explain to me why these tests may have been flawed, and why after so many years of waiting for these results, how/why would something this important be postponed again, especially since we know BSE _can_ transmit to sheep in the lab?
to continue to postpone and put off this important research, is beyond me.
i know they tried explaining in this article the why/how, just after so many years of waiting, just smells to high heaven to me to still have no definate results, one way or the other. we will wait another decade or so i guess.
more tea and coffee anyone...
kind regards, Terry S. Singeltary Sr., Bacliff, Texas USA
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
Subject: Re: No sign of BSE in sheep ??? [tests terribly flawed]
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Thu, 18 Oct 2001 21:38:12 -0700
Content-Type: text/plain
Parts/Attachments: text/plain (419 lines)
Reply
######## Bovine Spongiform Encephalopathy
18 October 2001
FSA UPDATE ON THE RISK OF BSE IN SHEEP
The FSA has previously issued advice on the theoretical risk of BSE being in sheep.
The FSA is not advising against the consumption of lamb. However, the theoretical risk of BSE in sheep remains, although on-going studies of the current sheep flock indicate that no sheep have tested positive for BSE.
The FSA has previously publicised the potential problems in relation to an experiment on sheep brains from the early 1990s to determine whether sheep had BSE then. The Agency agrees with DEFRA that there are now significant doubts about the validity of the original sample that prevents it from being further considered in relation to the presence of BSE in sheep in the early 1990s.
The Agency will discuss its current position at an open Board meeting in London on Monday 22 October 2001.
Background information
The theoretical risk of BSE in sheep has long been known and has been publicly recognised by the Food Standards Agency and was raised in the Agency’s review of BSE controls. This theoretical risk was originally identified as a result of experiments in which it was shown that sheep deliberately fed BSE-infected cows’ brains were susceptible to infection. Despite the fact that sheep as well as cows are known to have eaten the same feed that caused the BSE epidemic in cows, evidence to date has not shown that any sheep have contracted BSE except experimentally.
http://www.foodstandards.gov.uk/press_releases/statements/st011018.htm
Letter to the Committee Chairman from Dr A G Dickinson (R 11)
I have not submitted any evidence to your Committee and this letter is intended to explain why. The enclosed copy of Hugh Pennington's Review of the Phillips Report [not printed] explains my involvement with TSE research, which has been longer than for anyone else world-wide.
I was one of the half-dozen who put a great deal of effort into arguing for a BSE Inquiry to be set up, as I had been close to the subject over several decades. The Phillips Report has, with one major exception, been widely regarded as excellent—unfortunately, its science section is unsatisfactory in many respects. This is not surprising because this subject is at the frontier of knowledge and Lord Phillips deliberately excluded from his team anyone with direct involvement in TSE research (he rightly criticises various BSE committees for doing this).
Some still disapprove of there having been a public inquiry, but they do not realise that the alternative was that we would now be half way through a High Court action lasting twice as long as Phillips took, with adversarial methods potentially doing untold co-lateral damage to both groups of victims (livestock farmers and vCJD families), but almost certainly failing to reveal the main aspects leading to the epidemic (I speak from the experience of having raised the warning, seven years in advance, that human growth hormone could be CJD-contaminated).
From the wording of your Committee's invitation for the submission of written evidence, the Phillips Report is being assumed to be a sound basis from which to asses the "scale and focus of MAFF's research into TSEs". Because I am trying to be as constructive as possible, I hope that you will not be offended by commenting that it seems to me that the nearly impossible is being attempted. Unless the breadth of this subject is thoroughly understood by those concerned, along with all the technical limitations involved in interpreting the research, "scope and focus" cannot be judged. With the aim of being helpful, I will include a few examples of important unresolved issues which could provide a constructive basis for your inquiry.
Since I started in this research in 1955, of the many committees intended to appraise or fund TSE work, there have been hardly any that have been of value. Many have had very wasteful or harmful consequences, because they comprised busy "experts" from other fields, who recommended the current scientific band-wagons (in 1955 for a scrapie vaccine or in 1988-89 ill-considered overemphasis on PrP) or the blatantly obvious (the need since 1960 for diagnostic tests). However, by making recommendations they succeeded in deflecting funds from other important aspects. During the BSE epidemic there has been extensive waste of the large, mainly MAFF-controlled, research funds. This is a view shared by many of those with proven TSE-research expertise, some of whom are still involved in the research and therefore too prudent or intimidated to publicise their opinions.
There were two types of reason why BSE funding has been very wastefully focused. One, from the late 1980s, was the plethora of research committees controlling the policy and funds, hardly any of whose members were familiar with the subject, but who were mesmerised by the hype surrounding the protein, PrP. In 1971 I discovered the crucial role of this protein in the pathogenesis of the disease and published this along with a range of predictions, most of which have now been confirmed. You may be assuming that the molecular nature of TSE agents has been "proved" to be, simply, a modified form of this protein (a so-called "rogue protein"), but the number of those who doubt this is steadily growing, if only for the reason that this hypothesis has never been able to explain the facts, and such anomalies progressively increase in number. This short-sighted view of molecular aspects deflected funding away from several important areas. One vital aspect from which work was deflected for a decade after 1988 was the investigation of substances (polyanions) which have the prospect of providing therapies for TSE infections—I drew the Inquiry's attention to this lapse in my Statement.
The second reason for considerable waste of funds was that staff at the Central Veterinary Lab, who were inexperienced with TSE research, controlled early decisions. An example of where this proved very costly was their misjudged decision to base routine BSE diagnosis on neuropathology, rather than on our quick, cheap 1986 biochemical assay, which would, importantly, also have been applicable to tissue from dead cattle that was unsuitable for neuropathology.
The foregoing relates to the past but is symptomatic of the present. Three current examples are given below under separate headings. The first example still remains as the most important issue needing active debate and good experiments, because it may become necessary to undo public misconceptions about whether or not the BSE strain of agent per se is more dangerous to humans than other TSE strains. The impression created since 1996 by governments and the media has certainly been that it is a more dangerous strain, but where is the hard evidence?
(1) The unresolved question is whether the TSE strain that causes BSE and vCJD is intrinsically more easily transmitted to other species, including humans, than other TSE strains. [This issue was presented to the BSE Inquiry in paragraphs 4-9 of my Statement.]
The misleading word that has been most popular with the media in recent years to describe BSE being transferred to another species is that it "jumped". I am certain that a more accurate term is that it was "pushed". In order to "push" one of these types of agents across to another species, the greater the amount of infective agent involved, the more likely it is to achieve infection of the other species. My assessment of the current situation with the transfer of BSE to humans, is that the whole picture could be explained solely in terms of the enormous scale of the BSE epidemic having massively exposed people to the otherwise very small risk of being infected.
It is only because of the precautionary principle that the provisional assumption has to be made that the BSE strain is very much more liable to infect humans than strains that have been present in sheep for hundreds of years. For 15 years we have urgently needed to know the relative risk of the BSE strain to humans—relative, that is, compared with other strains of TSEs. I am not aware of any properly designed experiments with this objective: enquiries whether some are, at last, in progress have been unproductive.
It is often claimed that no scrapie strain has infected humans, but this is an unjustified extrapolation. What has been well established is that if any scrapie strain has passed from sheep to humans, this must be such a rare event that it has not been detected as a component of the 1:50,000 rate of incidence of CJD in humans. It seems reasonable to conclude that the scale of exposure of humans to scrapie strains during the 20th century will have been vastly less than that to the BSE strain during the epidemic. The simple observation that humans and several other species have become infected with BSE proves nothing about whether it transmits more easily to other species when the same doses of different strains of infective agent are compared. The cases seen in other species can be explained either by the massive scale of exposure to BSE agent afforded by the epidemic, or by the BSE strain having "higher infectiousness" for other species, or both. Its known greater thermal stability than other strains may well be an important aspect of this. The Phillips Report, unfortunately, jumps to a premature conclusion on this whole question, but that is not their only lapse.
I am certainly not arguing that the BSE strain will prove to be no more dangerous to other species than most TSE strains—we must have hard facts. An extensive range of experiments is urgently needed, comparing the relative transmissibilities of various TSE strains with the BSE strain: these must cover a full range of doses of agent and species sources, and must examine several routes of potential infection. The work will need to be done in several species.
(2) The search for the BSE strain in the sheep population
Whether or not it should be a very high priority to search for the BSE strain in British sheep (or even world-wide) depends on the outcome of work under the previous heading. There have been hints of MAFF contingency plans to deal with British flocks on a draconian scale should the BSE strain be found, which heightens the urgency of answering the underlying question. But, at least, the Phillips Report kills off over a decade of MAFF propagation of the unsupported claim that the BSE strain originated on many occasions from scrapie strains being transferred to cattle in Meat and Bone Meal. (Unfortunately, the Report backs an origin for BSE that is implausible in the extreme.)
I was responsible for devising the type of strain-typing test needed for identifying different TSE strains and, with former colleagues at the Neuropathogenesis Unit (NPU), devised various means of separating component strains from mixtures. Nowhere else is there any such experience. It was a cause for amazement, therefore, when I heard that MAFF was funding attempts to search for particular strains in the UK sheep population by pooling the brains of sheep in batches, to economise this search. They will need considerable good luck with any such approach. I hope that they have run pilot trials with deliberate mixtures of a dozen known strains, along with the BSE strain, to test the proposition. At least, the NPU have declined to adopt any such method.
This brain-pool approach sounds like another MAFF-associated emulation of the botched CVL design for cattle "maternal transmission" experiments, which largely wasted several million pounds and many years. Is it fair comment to recall that in 1986-87, as director of the NPU, I only had £75,000 for all our TSE research experiments, after paying salaries and overheads for nearly 40 staff?
(3) The National Scrapie Eradication Plan for GB
The National Sheep Association know of my long-standing concern about the often unfounded speculations that have been damaging to their industry during the last decade.
Late last year, I was shown a copy of the glossy MAFF booklet dealing with the scheme aimed to eradicate scrapie from British sheep by breeding from rams carrying a particular version of the gene which codes for the PrP protein. As I had done the pre-molecular groundwork for this, by 20 years of selecting sheep genetically for some variants of this gene, I am in a position to understand the potential complications. Indeed, it was long realised that the notion of a version of the gene that would "resist" all known (and future) strains of TSEs, may not be realistic. This was underlined by the fact that in 10 years of searching for a strain of scrapie agent that could break such a barrier, I had been lucky enough to find one, with approximately this property. Furthermore, this finding was not a surprise because the work with scrapie in mice had taught me to avoid the notion of genetic "resistance" to TSEs: this complication is fundamental to understanding of the whole subject and is widely unrecognised, for example on occasion by leading members of SEAC. The nagging possibility of "carrier-infections" with TSE agents is one aspect of this complication.
Where the balance of judgement lies in the present context is dealt with in the response to the MAFF document appended to this letter, which is signed by four senior animal-disease scientists [not printed]. It came as a surprise that the booklet was issued as a "Consultation on proposals for Phase 1—a Ram Genotyping Scheme" when there appeared to have been several years of active support by MAFF for implementing this scheme. It seems to have the de facto status of an ongoing programme.
In closing, I must query the implication underlying the stated objective of your Committee. A subject like BSE is far bigger than any single department should attempt to handle. A very significant error was that MAFF was intent on keeping exclusive control, for example, by their early determination to exclude the Government Chief Scientist.
I consider it entirely inappropriate that any government department or group of departments (or their agencies) should control research on basic scientific issues, especially areas so near the frontier of knowledge. Such direct control should only involve practical and applied topics in well known areas. The research role of departments should focus almost entirely on having first-hand, comprehensive information about "who and where" there is success, but this must be staffed on criteria very different from present ones. Whitehall norms will need to be changed radically, where science is involved.
The basic research should be funded so as to ensure its objectivity and freedom from coercion—the Research Councils, as originally created, were well conceived to achieve this. Radical changes are needed to avoid the administrative traps into which MAFF fell headlong, when it allowed a disease outbreak to turn into a huge epidemic.
25 January 2001
http://www.parliament.the-stationery-office.co.uk/pa/cm200001/cmselect/cmagric/388/388m11.htm
Transmission studies of BSE to sheep;
http://www.bseinquiry.gov.uk/files/mb/m09/tab01.pdf
http://www.bseinquiry.gov.uk/files/mb/m09/tab11.pdf
TSS
Subject: Re: No sign of BSE in sheep ??? [tests terribly flawed]
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Fri, 19 Oct 2001 21:18:03 -0700
Content-Type: text/plain
Parts/Attachments: text/plain (735 lines)
Reply
######## Bovine Spongiform Encephalopathy
Labour 'buried report of BSE inquiry'
By Marie Woolf Chief Political Correspondent
20 October 2001
The Government was accused of spreading "the Jo Moore syndrome" after apparently trying to hide details of the disastrous scientific investigation into BSE in sheep by releasing them in a misleading statement late at night.
The Tories called for the resignation of Margaret Beckett, the Secretary of State for Rural Affairs, saying she tried to "bury" the news on a government website after newspapers had gone to press.
The statement was also criticised for failing to spell out clearly what was meant by "doubts over the validity" of a scientific sample. For five years, scientists at the Government's Institute for Animal Health in Edinburgh had been analysing cattle brains when they thought they were the brains of sheep.
Peter Ainsworth, shadow Secretary for Environment, Food and Rural Affairs, said the Government could no longer be trusted to keep the public properly informed about health issues because of its obsession with concealing bad news.
"You would have thought that after the Jo Moore e-mail, lessons about burying bad news might have been learnt. The despicable way in which the publication of the findings was handled confirms that the Jo Moore syndrome is endemic in Whitehall," Mr Ainsworth said.
"The Government should order an independent inquiry into how this shambles occurred, and the findings should be made public," he said.
Officials at the Department of the Environment, Food and Rural Affairs were told about the mistake on Wednesday morning. Mrs Beckett and Elliott Morley, the minister responsible for animal health, were told in the afternoon.
The department said the ministers took the decision to release the news in the evening aftercommittees of experts were informed. A spokesman said: "There was a phone call from the Laboratory of the Government Chemist on Wednesday morning. Ministers were alerted in the afternoon.
"[They] decided they would rather release it in the evening ... The Press Association was alerted at about 9pm. The view was that it was best to release it as soon as possible to avoid accusations of a cover-up."
The way the mistake was revealed will heighten public perception that the Government "manipulates" potentially damaging news events. It follows the e-mail from Stephen Byers' special adviser Jo Moore which said 11 September was a "good day" to "bury" bad news.
http://news.independent.co.uk/uk/politics/story.jsp?story=100484
How did top scientists mix up brains from cattle and sheep?
By Steve Connor Science Editor
20 October 2001
The mystery of how a world-class team of scientists could have spent nearly five years and £217,000 mistakenly testing cow brains instead of sheep brains for signs of BSE deepened yesterday.
The study was meant to assess whether bovine spongiform encephalopathy had infected sheep at the end of the 1980s when they were fed the same contaminated feed as cattle, but the results have now been declared uninterpretable because of the mix-up.
On a scale of laboratory blunders the error ranks about as high as they come, given that the fate of Britain's 40 million sheep might have rested on its outcome. Finding BSE in sheep could have led to the culling of the entire national flock.
How scientists could have confused brain material from cattle and sheep is now the subject of two inquiries, one by the Institute for Animal Health, the government-funded laboratory where the research was done, and another by the Department for the Environment, Food and Rural Affairs (Defra), which funded the study.
Both inquiries will attempt to explain how two tests early in the experiment appeared to confirm that the material was the mashed-up brains of sheep while a third, more definitive test, which was made public on Wednesday night, found only bovine brain and no evidence of sheep brain at all.
The roots of the story lie in an experiment conceived and run at the height of the BSE epidemic in the late 1980s and early 1990s. At that time, scientists wanted to know what effect different meat-rendering practices would have on the infectious agents behind both BSE and scrapie, a similar brain disease of sheep.
During 1990, scientists from the Government's Central Veterinary Laboratory at Weybridge, Surrey, collected the diseased brains of more than 800 cows with BSE to "pool" the material and test how it would survive different rendering practices. A second phase of the study took place over the next two years when 2,860 brains of sheep affected by scrapie were collected by 18 veterinary laboratories around the country and pooled for a similar rendering experiment.
Material left over from both these experiments was frozen and stored. Not until 1996, when the link was established between BSE and a variant of human Creutzfeldt-Jakob disease (vCJD), did scientists think the stored sheep material might be useful in answering another question: could BSE have got into sheep?
There has always been a theoretical possibility that BSE infected sheep. Laboratory experiments show the agent can be transmitted through feeding and some sheep were known to have eaten the same BSE-contaminated feed as cattle.
The big question is whether infection had actually happened in the late 1980s, before measures were fully enforced to ban the feeding of ruminant- derived feed to ruminants such as sheep and cattle. Realising that they had a store of sheep brains from the early 1990s, scientists suggested "testing" this material for BSE.
However, a possible complication was that the pooled sheep brains may have been cross-contaminated with infected cattle brains. Both sets of brains were removed by veterinary scientists in the same centres using the same instruments and surgical slabs.
With cross-contamination a possibility, the Institute for Animal Health and the Central Veterinary Laboratory did two sets of tests to see whether the material – which looks like porridge – was fundamentally ovine rather than bovine in origin.
Professor Chris Bostock, the institute's director, said one test involved looking for the presence of the amino acid arginine at "position 171" on the molecules of the prion protein present in the sample. The "arginine 171" signature is unique to sheep and the test proved positive, he said. The Central Veterinary Laboratory was also unable to detect bovine material. "This was psychologically good news as far as contamination was concerned," Professor Bostock said.
Convinced that the pooled material was largely if not entirely sheep brains, the complicated experiment took place within the institute's neuropathogenesis unit in Edinburgh, the research centre that did the work proving the link between BSE and vCJD. The research involved injecting the pooled brain material into different strains of laboratory mice, which incubate the disease in a precise pattern depending on whether it is scrapie or BSE. The object was to see if the sheep that had died with "scrapie" were in fact suffering from BSE.
Preliminary results of the experiment were sent to the Food Standards Agency, which issued a statement two months ago to coincide with a separate initiative by the sheep industry to boost the consumption of lamb. The agency warned there was still a "theoretical risk" of BSE infecting sheep and said early results from the experiment involving the pooled brains from the early 1990s "could be compatible with BSE having been in sheep at that time".
Professor Bostock, who was on a holiday when the agency made its statement, was not happy. He said at the time: "I personally think that it is completely unhelpful to start discussing results until they are complete and in the public domain in a way that everyone can see what we are talking about."
The issue of possible cross-contamination had still not been resolved so Defra organised independent DNA tests of the brain material, which began in September. The results were unequivocal: the material was entirely bovine with no trace of sheep tissue. "Extraordinary," declared Professor Peter Smith of the Government's spongiform encephalopathy advisory committee. Professor Bostock said he was "flabbergasted".
One possibility being investigated is that the pool of cattle brains collected in 1990 was confused with the pool of sheep brains collected two years later.
Professor Bostock said: "There has to be an explanation for this discrepancy. We'll have to wait to see what [it] is."
Meanwhile, we are still no nearer to knowing whether BSE has infected sheep.
http://news.independent.co.uk/uk/science/story.jsp?story=100445
BSE and goats Sunday 20 September 2009
There are only two confirmed cases of BSE in a sheep or a goat that were not infected experimentally. These were in a French goat that died in 2002 and a Scottish goat that died in 1990.
The Agency was informed in 2005 that the Scottish goat might have had BSE. BSE was finally confirmed in May 2009.
As regards the current UK goat herd: in August 2008, Defra reported a case of TSE in an English goat herd in which initial tests could not exclude BSE. Defra's Veterinary Laboratories Agency (VLA) is undertaking more tests but it will be some years before the results are available.
snip...end...TSS
http://www.food.gov.uk/safereating/animaldiseases/bse/what/bseandgoats/
The neuropathology of experimental bovine spongiform encephalopathy in the pig
IN CONFIDENCE
The result, albeit confined to one animal in the experimental challenge group is incontrovertible evidence of the transmissibilty of BSE to the pig by simultaneous intracerebral, intravenous and intraperitoneal inoculation routes. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/08/20003001.pdf
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/08/29003001.pdf
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
7 OF 10 LITTLE PIGGIES WENT ON TO DEVELOP BSE;
1: J Comp Pathol. 2000 Feb-Apr; 122(2-3): 131-43. Related Articles,
The neuropathology of experimental bovine spongiform encephalopathy in the pig.
Ryder SJ, Hawkins SA, Dawson M, Wells GA.
Veterinary Laboratories Agency Weybridge, Woodham Lane, New Haw, Addlestone, Surrey, KT15 3NB, UK.
In an experimental study of the transmissibility of BSE to the pig, seven of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral inoculation with a homogenate of bovine brain from natural BSE cases developed lesions typical of spongiform encephalopathy. The lesions consisted principally of severe neuropil vacuolation affecting most areas of the brain, but mainly the forebrain. In addition, some vacuolar change was identified in the rostral colliculi and hypothalamic areas of normal control pigs. PrP accumulations were detected immunocytochemically in the brains of BSE-infected animals. PrP accumulation was sparse in many areas and its density was not obviously related to the degree of vacuolation. The patterns of PrP immunolabelling in control pigs differed strikingly from those in the infected animals.
PMID: 10684682 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?holding=npg&cmd=Retrieve&db=PubMed&list_uids=10684682&dopt=Abstract
DEER BRAIN SURVEY
Subject: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY Date: Thu, 17 Oct 2002 17:04:51 -0700 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L
Greetings BSE-L,
is there any other CWD surveys/testing in the UK on their deer? what sort of testing has been done to date on UK/EU deer? any input would be helpful... thank you
DEER SPONGIFORM ENCEPHALOPATHY SURVEY
3. This will be a low key study with no publicity to avoid unnecessary media interest. It will be carried out in two stages ;
(I) A small scale examination of around 30 deer brains to establish the normal histology of the healthy brain; and
(II) A larger scale random examination of 300 or more adult deer brains drawn from both deer farms and parks to establish whether there is any evidence of a cervine spongiform encephalopathy. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1991/11/20004001.pdf
Ministry of Agriculture Fisheries and Food Veterinary Investigation Centre West House. Station Road. Thirsk Y07 IPZ Telephone: 0845·522065 Fax: 0845·525224
Your reference
Our reference RJH/ASB
Date 4 November 1992
DEER SPONGIFORM ENCEPHALOPATHY SURVEY
Dear Paul
I have now found time to review the 10 deer- brains collected from Mr Walker farm··via Winchester Via Winchester VIC. In answer to your specific question was there sufficient difference in preservation of brain tissue to warrant the extra effort involved in rapid brain removal on the farm, the answer is definitely "Yes." The original five brains (Winchester ref M487/11) showed varying degrees of autolytic vacuolation affecting both white and grey matter throughout the brain. vacuolation and separation of Purkinje cells and marked perivascular spaces. These artifacts made interpretation of subtle, specific pathological vacuolation more difficult. By contrast the second submission (Winchester reference N736/2) showed excellent preservation of white and grey matter. Any vacuolar Change present could be confidently interpreted as pathological albeit of unknown pathogenesis.
I can only reiterate the comments made by Gerald Wells and myself at the preliminary discussion at Weybridge in Autumn 1991. If the survey's purpose is an accurate histopathological interpretation of brain tissue. the material must be collected in a pristine state. This is particularly valid when looking for ar unrecognised and undefined spongiform encephalopathy in a new species. Deer brains are very large structures which take a lot of fixation and therefore must be handled sympathetically from the start. We have already seen the problems encountered in comparatively smaller hound brains where delayed fixation was a major limitation on interpretation of true pathological change.
The bottom line must be that if a pathologist's expertise is to be used, it is critical to collect artefact free brain material. If the politics or economics do not allow this, then I would suggest that an electron microscopy survey involving detection of scrapie associated fibrils would be much more appropriate.
Best wishes Yours sincerely
R J HIGGINS VIO 92/11.4/2.1
http://collections.europarchive.org/tna/20081106170644/http://www.bseinquiry.gov.uk/files/yb/1992/11/04002001.pdf
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1992/11/04002001.pdf
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ References: <3daf5023.4080804@wt.net>
Dear Terry,
An excellent piece of review as this literature is desparately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler ===============
Incubation periods for BSE are proportional to the life expectancy of the animal affected. The disease's incubation period is 18% of a cow's life expectancy and would be expected to about double when crossing to another species [---] that is, to 36% of 70 years in humans.
Steve Dealler, consultant in medical microbiology. Burnley General Hospital, Burnley BB10 2PQ deal@airtime.co.uk
=======================================
Marmoset Experiments
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1992/02/14002001.pdf
BSE - Research Study : Transmission of BSE to Primate
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1992/02/14003001.pdf
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEES
snip...
5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that ALL THESE AGENTS could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. ...
snip...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1990/09/26003001.pdf
IN CONFIDENCE
TRANSMISSION TO CHIMPANZEE'S
1. Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not. 2. We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of filed isolates subsequently strain typed in mice be inoculated by the appropriate routes (i/c, i/p and i/v).
3. I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. Proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.
4. In view of Dr. Gibbs' probable use of chimpanzees Mr. Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.
5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I HAVE A VIEW THAT ALL THESE AGENTS COULD BE TRANSMITTED provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to RETAIN that hypothesis.
A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding man's susceptibility. In the meantime no doubt the negativity would be used defensively. IT WOULD HOWEVER BE COUNTERPRODUCTIVE IF THE EXPERIMENT BECAME POSITIVE. We may learn more about public reactions following next Monday's meeting.
R. Bradley
23 September 1990
CVO (+ Mr Wells' comments)
Dr T W A Little
Dr B J Shreeve
90/9.23/1.1
http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
IN CONFIDENCE
CHIMPANZEES
http://www.bseinquiry.gov.uk/files/yb/1990/09/26003001.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
EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE
This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........
http://web.archive.org/web/20010305222246/www.bseinquiry.gov.uk/files/yb/1994/02/07002001.pdf
1: J Infect Dis 1980 Aug;142(2):205-8
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.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
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://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC.
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK
National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html
Epidemiology of Scrapie in the United States 1977
http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf
Like lambs to the slaughter
31 March 2001
by Debora MacKenzie Magazine issue 2284
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 ...
http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html
BSE: TIME TO TAKE H.B. PARRY SERIOUSLY
If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...
http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf
Sunday, April 18, 2010
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html
Wednesday, March 31, 2010
Atypical BSE in Cattle
http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html
http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html
Transmissible Spongiform Encephalopathy
http://transmissiblespongiformencephalopathy.blogspot.com/
PLEASE NOTE *
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
Subject: MAD COW SLURRY FED TO SCHOOL CHILDREN AND OTHERS !
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Sat, 27 Oct 2001 20:08:20 -0700
Content-Type: text/plain
Parts/Attachments: text/plain (114 lines)
Reply
######## Bovine Spongiform Encephalopathy
October 28 2001 BRITAIN
© Breeding trouble: companies that used slurry scraped from the bones of cattle, which has been linked to vCJD, the human form of mad cow disease, have now been named Photograph: Stone Leading food groups used 'mad cow' danger meats
Mark Macaskill
SOME of Britain's best-known food producers used mechanically recovered meat - the slaughterhouse slurry blamed for spreading the human form of mad cow disease - and supplied it to unwitting consumers.
The companies - including Bowyers, Batchelors and Dalepak - used the now-outlawed product in pies, burgers and sausages sold to thousands of schools, hospitals, corner shops and supermarkets.
People who ate such products are thought to be at greater risk of developing the fatal brain disease vCJD, the human form of BSE. This is because mechanically recovered meat (MRM) from cows is scraped from the bone and is likely to include tissue from the nervous system.
Publication of the company names will embarrass the Food Standards Agency (FSA), which has been criticised for its failure to track down the firms responsible. In August, scientists tasked by the government with investigating the spread of vCJD, complained that, despite repeated demands, the agency had still to come up with the names, six years after the request was first filed.
Despite the FSA's difficulties, The Sunday Times has been able to identify products that contained MRM by tracking forward from the companies that bought the machinery needed to produce it.
These include products from two firms - Bowyers and Dalepak - both now owned by Northern Foods, the food conglomerate chaired by Lord Haskins, the prime minister's adviser on rural affairs.
MRM was banned for use in food in 1995, when the links between BSE and vCJD began to emerge. At least 107 people have died of vCJD and a further six have the disease. The worst- case scenario in official forecasts is for 40,000 people in Britain to be infected.
Batchelors, the soup brand, confirmed last week that bovine MRM was used in "non-branded" soups and other products until 1992. Bovine MRM was also used by Bowyers, the sausage manufacturer, and Dalepak, which made the Ross range of burgers, steaks and grills, Northern Foods confirmed.
A company that produced and supplied MRM was Scotbeef, one of the UK's largest beef producers. Last week it admitted producing MRM from 1980 until 1984 and selling it on to smaller wholesalers.
MRM became popular in the mid-1970s, when machines that recovered residual flesh from carcasses became available. Bones were dropped into a chamber and "squeezed" by a hydraulic ram. The slurry was then forced through tiny holes that filtered out any large particles. The product was then used to "pad out" a range of foods.
Two MRM machines, the Protecon and the Hydro, were made in the Netherlands. A number of slaughterhouses and meat manufacturers bought them, including Scotbeef, Midland Meat Packers Ltd, one of Britain's largest abattoirs, and Chard Meat Company, which has since gone into liquidation.
Another company, Perimax, with plants in Scotland and England, was set up in 1976 specifically to produce MRM from pig, cattle and poultry carcasses.
Most MRM was produced from poultry and pigs, but a significant amount - estimated at 5% - came from cattle.
Last week Northern Foods admitted that Bowyers used bovine MRM in its economy sausages before it took the company over in 1984. It also revealed that Dalepak had used bovine MRM. "It has always been our policy not to use mechanically recovered meat. When we acquired Bowyers and Dalepak, the company moved swiftly to remove MRM, but this would have taken a few months to complete," said a spokesman for Northern Foods last week.
Midland Meat Packers, one of the largest producers of bovine MRM, and Newtech Ltd, based in Wisbech, Cambridgeshire, which distributed the Hydro machine, declined to comment.
An FSA spokesman said: "So far, our only contact with the meat industry has been through the representing bodies, but all manufacturers will be contacted when our research gets under way."
http://www.sunday-times.co.uk/news/
TSS
########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############
http://downercattle.blogspot.com/
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America
update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
page 114 ;
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
International Society for Infectious Diseases Web: http://www.isid.org/
please see full text ;
http://transmissiblespongiformencephalopathy.blogspot.com/
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
please see full text ;
Wednesday, March 31, 2010
Atypical BSE in Cattle
http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Monday, October 19, 2009
Atypical BSE, BSE, and other human and animal TSE in North America Update October 2009
http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas.
She left 6 Kids and a Husband.The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-
Physician Discharge Summary, Parkland Hospital, Dallas Texas
Admit Date: 12/29/2009
Discharge Date: 1/20/2010 Attending Provider: Greenberg, Benjamin Morris;
General Neurology Team:
General Neurology Team Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence.She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.
http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8
>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<
please see full text ;
Monday, March 29, 2010 Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas
http://creutzfeldt-jakob-disease.blogspot.com/2010/03/irma-linda-andablo-cjd-victim-she-died.html
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html
USA sporadic CJD cases rising ;
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection. He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
CJD USA RISING, with UNKNOWN PHENOTYPE ; 5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.
http://www.cjdsurveillance.com/pdf/case-table.pdf
Friday, February 05, 2010
New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review
http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
Archive Number 20100405.1091 Published Date 05-APR-2010 Subject PRO/AH/EDR> Prion disease update 1010 (04)
snip...
[Terry S. Singeltary Sr. has added the following comment:
"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.
The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"
http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101
Monday, April 5, 2010
Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010
http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518 USA
