The articles that have appeared in recent scientific journals concerning BSE between September 1996 and December 1996

There are always more articles than you can read about TSEs, and I can only make an apology that some may be missed. If you feel that your article is missing and should be here, please forward it

Articles will probably not be still present on this page for greater than 6 months

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There are a lot

before September 1996

or the

between December 1996 and May 1997

and

after May 1997

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New diagnostic tests for prion diseases

Collinge J. Imperial College School of Medicine at St. Mary's Hosp. London W2 1PG UK.

New England Journal of Medicine 1996;335:963-5.

A short review of the tests. e.g. genetic associations with CJG (heterozygotes at position 129 of the prion protein), associations with presence of protein 130/131 in the CSF, histopathological methods using imaging. He then goes on to suggest how new techniques such as transgenic mice inoculations could perhaps be able to indicate which people were infected and saying whether the disease was derived from BSE.

Much more research than this is going on and by rights this should have been included in the article as a review.


The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies

. Hsich G, Kenney K, Gibbs CJ, Lee K, Harrington MG.Mailstop 139/74, Caltech, Pasadena CA91125

New England Journal of Medicine 1996;335:924-930

Brain protein 14-3-3 had been previously designated but its function not known. Proteins 130 and 131 were found by two dimentional electrophoresis in patients with CJD and matched 14-3-3. An immunoassay for the protein was set up for it and tested on patients with CJD and other encephalopathies. The test was found to have a sensitivity of 96%% and a specificity of 96% (but higher if recent strokes were excluded). It was also found to be positive in patients with viral encephalitis, but negative in patients (12 of which were found) had suspect but not confirmed CJD but no 130/131. The results in animals were similar to those found in humans with the CSF from six of nine cattle with experimentally induced TME or scrapie. One cow with features but no pathology yet of BSE also had a positive test. No control cattle had positive assays. Positive in 5 of 6 sheep with naturally acquired scrapie and was negative in one control. All infected 15 chimpanzees had positive tests wereas none of the 77 controls did.


Diagnosis of CJD by two-dimensional gel electrophoresis of cerebrospinal fluid.

Zerr I, Bodemer M, Otto M, Poser S, Windl O, Kretzschmar H, Gefeller O, and Weber T. Contact Dr. Weber at the Neurologische Klinik, Marienkrankenhaus, Alfredsr 9, D22087, Hamburg.

Lancet 1996;348:846-9.

A similar finding to the above groups and the NEJM but a less specific finding was found.


Premortem diagnosis of CJD by CSF analysis

Lee K, Harrington M Caltech, Pasadena

Lancet 1996;348:887

A later result than the NEJM paper showing a more specific result and comparing the results between this and the 1986 paper. They say that they are now investigating the point in the infection at which the test becomes positive. Earlier they had said that the marker may simply be an indication of neuronal damage and hence it may actually be a late marker of disease.


Trumping science in BSE

Editorial Lancet 1996;348:835

"We have already raised doubts about the UK government's hadling of allegations that a new variant of CJD might be linked with the epidemic of BSE..." This is an editorial explaining that the handling of the BSE crisis and the potential CJD one have been inadequate with underfunding of scientists and grasping at speiific results if they hold out hope for political factors (the editor does not put it like that). The editor says that the UK Government's policies have threatened the belief that we have in our UK scientists that they can respond adequately to a new infectious risk.

This is probably one of the most aggressive editorials I have seen from the Lancet.- Ed


Diagnosing CJD

Will R, Ziedler. CJD Surveillance Unit, Western General Hosp, Edinburgh EH4 2XU

BMJ Editorial. 1996;313:833-4

A short editorial on how diagnosis is currently carried out, using histopathology and immunostaining, how the new form of the disease is histopatholgically and clinically different from the previous types seen, and whether the psychiatric profesion ought to be more invovled. They are hopeful that the CSF test for protein 14-3-3 may be of some use but make it clear tha tmore research is needed before this is certain.


BSE - a review of causes and state of present knowledge

JB Owen, School of Agricultural and Forest Sciences, Bangor.

Presented to the Welsh Affairs meeting and to be published by the Inst of Wesh Affairs in a book.

Shows unease that the disease is disappearing and that we need not worry. This is a good and up to date review of the subject.


Scrapie in Norway

Ulvund MJ. Department of Sheep and Goat Research, Norwegian colllege of Vet Med. Box 264, 4301 Sandnes, Norway.

Presented at the 47th Annual Meeting of the European Assn for Animal Production, Lillehammer, Norway, 25-29 August. 1996

Scrapie was very rare, with the first case reported in 1981. There has been a pronounced increase in cases over the past 3 years, with 21 new flocks affected up to 29th August 1996 i.e. it is rising epidemically. The reason for this is unclear. Farms are emptied of sheep, the meat not used, the sheep slaughtered, and farms left empty for 2 years. Certain breeds are particularly affected and an affect has been seen due to the anxiety resulting from BSE in the UK. The rise is not felt to be purely due to BSE anxiety and the author things that something else has happened to cause the epidemic, so far unknown.


Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD

Collinge J, Sidle KCL, Meads J, Ironside J*, Hill AF Imperial College, London.*CJD Unit, Edinburgh.

Nature 1996;383:685-690

An attempt to compare the electrophoretic patterns of PrPsc from various forms of CJD and to compare these with that from BSE infection in cats, mice, and macaque monkeys. What was found was that the pattern depended on the glycosylation of the prion protein to some degree but that there was a further pattern that was still different between 4 separate human groups when the glycoside chains were removed. It was not noted as to the inefficiency of the enzyme used to remove the glycoside chains. The most important finding was that the cats, mice and macaque monkeys had the same pattern as humans with new variant CJD. It was explained how this could have taken place and how this might indicate that this was derived from BSE. The introduction is a good explanation of the arguments concerning the presence or absense of any nucleoside with the prion protein to make the infective agent. What is mentioned but not brought forward much is the fact that, if glycosylation is so important, then there may well be methods of both diagnosis and treatment for this sort of disease (published by Dealler. Medical Hypotheses 1994;42:69-75).


A suspicious signature

Aguzzi A, Weissmann C University of Zurich.

Nature 1996;383:666-7

This explains in layman's language what the previous paper had said. It puts forward the idea that it is the glycosylation that decides the strain of the agent (published by Dealler. Medical Hypotheses 1994;42:69-75). As such it might be possible to look for the glycosylation factors in the peripheral tissues of the animals and use that as a method of diagnosis. They put forward that we should now assume that variant CJD is derived from BSE until evidence appears aginst this.


CJD in a 52 year old woman with florid plaques

Kopp N, Streichenberger N, Deslys, JP, Laplanche JL, Chazot G. Hospital Neurologique, Pierre Wertheimer, 69003 Lyon, France.

Lancet 1996;348:1239

new variant CJD in a patient that had received a dura mater graft in 1984. Psychological changes, followed by ataxia and leading up to speech difficulties and death. The EEG was initially normal. Met-Met at 129. Biopsy showed kuru-type plaques in a similar manner to the nvCJD.


Fatal spongiform encephalopathy in a patient who had handled animal feed

Weis J, Kretschmar HA, Windl O, Podoll K, Schwartz M. Inst for Neurophathologie and Neurologische Klinik, TWTH, D-52074, Aachen, Germany.

Lancet 1996;348:1240

Patient handled the material almost daily for several years. 66 yr old man, similar pattern of disease and histopathology as CJD (not CJD-2). Met-Met at 129. Significance of this was unclear as the excess of CJD in farmers was unclear in UK and further research was needed.


Public health officials are confused over whetehr to eat beef

Cullen, M, Tocque K CDSC North West, PHL Fazakerly, Liverpool

British Medical Journal 1996;313:1146

A letter explaining a short survey of PH officials in which it was asked if they had decreased their intake of beef or beef products. In this over 50% of the officials had changed their eating habits for beef and all indicated that they did not trust the safety of beef products. The letter indicates that this was probably due to the lack of knowledge of the disease and that the fall in beef consumption would gradually turn to normal. The author said that this would depend on a clear message being put through both medical and non-medical channels, which was evidently not the case early on.


Study shows need for better communication between government and doctors

Simpson C, Dealler SF, Pattterson W. Friarage Hosp, Northallerton, North Yorkshire.

British Medical Journal 1996;313:1146

This was a study carried out in 1995, before the BSE crisis, and shows that the medical population had already decreased or stopped eating large amounts of beef. This was more likely to be true in female doctors and less likely in junior ones. The study was taken at a time when the Department of Health was the only source of data being provided and the major journals published little indicating the risks of what was taking place. What it shows was that the DofH was simply not believed by the medical profession and their repeated statements without scientific data would not be acceptable to them. It shows that the medical profession decreased their beef intake much more than the general population and that the vacuum of information from the DofH was easily filled by the media. The credibility of the DofH could not be assumed by them when subjects such as BSE were concerned.


Viewpoint: The BSE crisis

RW Lacey. University of Leeds, UK

British Food Journal. 1996;98:3-4

A short editorial explaining why misleading information was put out by the official channels (and possibly believed by them) concerning the risk to the population of BSE. It explains some factors on how odd statistics were derived by MAFF e.g. it is now known that one farm in Yorkshire colected 915 cases of BSE but only had 100 milking cattle itself. The way this was done was to import cattle from other farms with BSE and then claim the compensation from MAFF. In this way the apparent prevalence rate of BSE in farms remained relatively low in that area. He discusses the results from the vertical transmission study and suggests that the 13 cases in cattle not the offspring of infected mothers may well be due to endemic transmission and that this should not be ruled out at this time. He explains that it is unacceptable to assume that infection would be confined to the organs that were banned in the UK and that it is still unacceptable to predict the scale of CJD derived from BSE.


Selected Examples of Emerging and reemerging infectious diseases in animals

Three diseases have signficifance for both animals and man. Shadduck JA, Storts R, Adams LG College of Veterinary Medicine, Texas University.

American Society for Microbiology News (reviewed articles).December 1996;586-588

The author talks about this being a problem in the UK but describes just how awful it would be in economic terms if BSE was present in the USA ($50 billion). The authors describe just how knowledge has had to build on inadequate research and how scrapie had always been looked on as an insignificant disease.


Trumping Science in BSE

Maddocks A, Dealler S. Burnley General Hospital, Burnley, UK

Lancet , November 30 1996;348:1520.

Following the editorial of September 28th indicating that the scientists were not getting on to the subject of BSE quick enough, the authors indicate that it is not the scientists fault, as they had actually been discouraged from carrying out the necessary research. The scientists were perfectly good, but it was the directives from above that prevented adequate action.


Inactivation studies on BSE agent

Taylor D Neuropathogenesis Unit, Univ of Ediburgh site, Edinburgh, UK

. British Food Journal. 1996;98:36-39

This is quite an important article in that nobody else is actually finding out just how stable the BSE agent is in relation to other TSEs. One of the main problems with the research was that the level of infectivity present in his test tissue was apparently so low: 10E2.6 or 10E3.1IU per ml. Now, the mouse might be inoculated with only one hundredth of a ml and so the actual dose given would be 10E0.6 or 10E1.1. It is always attempted to inoculate the mouse with as much as possible but often some simply comes out again through the hole of inoculation. They say that you are lucky to really get in 20 microlitres even though 30 microlitres is inoculated. As a result of this, if the material is treated with whatever factor, it only has to remove around 1 log of infectivity to make the material lose its infectivity to the mice. As a result the effect of chlorine apparently removing infectivity cannot be looked on as being adequate. This is a rather poor review of some extremely difficult arduous work. I will include his findings in the BSE stats page.


The hidden costs and benefits of BSE

Loader R, Hobbs JE. Centre for Food Economics Research, Dept of Agriculture and Food Economics, Univ of Reading, UK.

British Food Journal 1996;98:26-35.

A discussion of the costs and the changes that will have taken place as a result of bovine regulations and slaughter policies. Few actual figures are produced, however, and things are probably changing still quite fast. Little politics is brought into the field dispite the heavy politics that has produced the changes. This sort of article is useful, however, in that it is a deep seated indication of how farmers think and how far ahead they would consider the hidden costs of their action (e.g. the costs of the BSE epidemic in cattle and people is not brought in to a great degree). However; a very interesting article giving insight into the business seen and the business unseen.


BSE: a reviw of the scientific evidence

Institute of Food Science and Technology: a statement.

British Food Journal. 1996;98:4-16.

A well written review with information derived largely from MAFF sources but accepting the results of research from Anderson and Collinge outside. No admission is made that much of the risk should have been realised earlier or that action sould have been taken earlier. Nothing is said to indicate whether the IFST felt it had been mislead by MAFF data earlier. Most of the article is a story of the epidemic but the assumptions contained are just those of MAFF (BSE derived from scrapie, did not think there was any VT, no reason to assume CJD would result, no infectivity in bovine tissues except brain spinal cord, ileum, and retina etc) and so the article is disappointing. By rights as an independent group the IFST would have been expected to look into the assumptions made by MAFF as to whether they were valid. A good review through MAFF's eyes. For an updated version see:
http://www.easynet.co.uk/if st/ The reply to this review can be found elsewhere on this site (see 20.12.96).


A week that shook the meat industry: the effects on the UK beef industry of the BSE crisis

Clephan Palmer. Head of Industry Stategy, Meat and Livestock commission, Milton Keynes, UK.

British Food Journal 1996;98:17-25

A short run up to the catastrophic drop in beef sales in March 1996 followed by the changes that have taken place in the market place and the industry since then. The article explains that beef had been decreasing in sales for many years and the export of veal calves from milking herds had been taking place in large numbers for some time. A short run through the 'Black Wednesday and black weeks' indicating the changes that took place in European, World wide and UK legislation. The re-emergence of the beef market after the crisis hadbeen seen with a reappearance of the price for beef but the cost of production was still high. Decreasing prices for cattle over 30 months due to compensation, decrease in the size of the number of abattoirs able to take the cattle etc. In the end it shows how the industry has kept itself going dispite the crisis but will decrease in size. This is the way that a farmer would have seen the crisis; in terms of sales, costs and market size. Nothing is put in concerning the prediction of the crisis that had been made for some time or the effect that the Europeans have had on their beef market. Little is put in to indicate that the producer of food's responsibility is to provide safe food and that the crash in the market was due to inaction by producers through NF or MAFF to take the risk away themselves. In the end the media produced a market crash that the producers had failed to prevent. What the article shows is that, if all the producers look at is short term return on capital, this sort of problem will recur with some other factor. A well written article from a market viewpoint.


New Variant CJD in France

Deslys JP, Lasmezas CI, Streichenberger N, Hill A, Collinge J, Dormont D, Kopp N. Service de Neurovirologie, CEA, DSV/DRM/SSA, BP 6, 92265 Fontenay-aux-Roses, France.

Lancet Jan 4 1997 p 30.

Showing that of the supposed nv-CJD cases one differed from the others in the glycoform of its prion protein (and hence was probably not associated with BSE) but the other did not.


The nature of scrapie

Narang, H. K. Bell International, Newcastle upon Tyne, UK

Laboratory News Jan 1997 pA8

Narang explains the structure of the particle, the nemavirus, that his data suggest is true for scrapie. The core of the nemavirus is the prion protein crystalloid, around this is a single stranded piece of DNA, and around that is a protein of unknown structure. Narang shows how this particle could explain all the experimental phenomena that are claimed for the prion. Narang has been treated badly by offial sources in the UK and this is good source to understand that his hypothesis is not so odd; he just trod on too many toes. One of the most important reports that Narang has made is concerned with the phenomenon of 'interference'. This is described as when one scrapie agent (incubation period 600 days) is inoculated into an animal and this is followed by scrapie of a much shorter incubation period. The scrapie that develops is always of the type of the first inoculum and the reason for this is both unclear and unsuitable for the prion hypothesis. No reference is given for this report but Narang has explained it at a number of events.


Diagnosis of new variant CJD by tonsil biopsy

Hill AF, Zeidler M, Ironside J, Collinge J. CJD surveillance Unit, Western Hospital, Edinburgh.

Lancet Jfan 11, 1997;349:99-100

This follows on from the work by Schreuder BEC at Lelystad in Holland in which scrapie was well demonstrated by tonsillar biopsy. Patients were diatnosed by both brain and tonsillar biopsies and the results compared. A stain of the tonsil folicular tissue with anti-PrP ELISA staining histopathology techniques showed the PrPsc to be present in reticulated patterns within the tonsil. It could also be shown that the electrophoretic pattern western blot of the PrPsc could perhaps be identified as nv-CJD by the demonstration of the glycoform.


Predicting the CJD epidemic in humans

Cousens S, Vynnycky D, Zeidler M, Will R, Smith PG, London School of Hygeine and Tropical Medicine

Nature Vol 385 16.1.96 p197

An investigation of the 14 cases of diagnosed nv CJD, their time of onset etc to give 3 predictive patterns for the case number in the future. They then look at the exposure patterns from 2 sorts: either assuming that all cases of BSE had been reported or assuming that back calculation under reporting could be assumed. Lowest possible likely was in hundreds and the highest was 80,000. They said that the numbers could not be adequately decided and this could not be done even after 4 years.

(this article was bounced by the Lancet and Nature demanded changes before publication)

It should be noticed that the numbers given do not include confidence intervals and when this is done (Dealler, 1995) this brings the potential numbers to over a million. The most useful thing stated was that we could not know the number of cases that would appear for several years and even then the precision would be poor.


Epidemic or false alarm

Skeggs DCG, Univ of Ortego, Dunedin, New Zealand

Nature vol 385 17.1.96 p200

Editorial: Still too early to tell the number of cases of nvCJD to expect. He explains that things should not be assumed to be going well and goes over the data from the previous article explaining why this was not surprising.


Prion test will identify victims of tainted beef

New Scientist, 18.1.97 p 10

This explains how the tonsil biopsy test may be adequate not just to diagnose if a patient is incubating CJD but also but perhaps to show if the disease is derived from BSE or not.


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