IS THERE A LINK BETWEEN BSE, CJD AND SCRAPIE?
by Frances Wolferstan, BA, Vet MB, MRCVS
The Lantern: Supporting the Policy and Philosophy of the British Housewives League
January 2004, pp. 10-12.

The officially held view is that Bovine Spongiform Encephalitis (BSE) is caused by infectious misfolded prion protein. These prions are thought to have originated in meat and bone meal (MBM), which was widely used in concentrate rations for cows at that time, as it had been for decades.

It was speculated that the original misfolded prions arose either in the offal of sheep carrying the disease Scrapie, or from naturally-occurring sporadic cases of BSE in cattle similar to the sporadic cases of Creutzfeldt-Jakob Disease (CJD) that occur in humans. It was argued that although similar material must have entered the food chain previously, a change in the processing methods in the production of MBM was now allowing the infectious agent to survive.

In the Seventies, a gradual switchover had taken place in the production of MBM from a batch rendering process to a continuous rendering process with reduced use of heat and solvents. This was thought to have been at the root of the problem.

As a result of these conclusions the use of MBM in ruminant rations was banned in July 1988. This ban was continuously tightened in the next few years to close every possible loophole.

There are several weaknesses in this accepted theory.

Firstly, prion is just a protein molecule. It contains none of the genetic material found in all other living organisms, which enables them to grow and multiply and, in the case of viruses and bacteria, spread the infection.

Secondly, prior to the outbreak of BSE, MBM was widely exported from Britain without apparently causing outbreaks of BSE in any of these countries.

Thirdly, at this time, other countries had also switched to a continuous rendering process without any similar problem arising.

Finally, despite the most rigorous controls on the use and production of MBM in Britain, animals born after the various bans continued to succumb to BSE in thousands.

Despite the expenditure of vast sums of money on research in the last fifteen years, the scientists have been unable to produce any really convincing explanation of these anomalies. So the theory remains just that, a theory.

As more and more animals born after the ban on the use of MBM in cattle feed succumbed to BSE, I became increasingly sceptical of the official theory. I eventually decided to carry out my own retrospective survey into the relationship between Farm Husbandry Practices and BSE.

The survey asked for information on BSE animals, their dams and their calves; herd fertility; genetics; the feeding pattern of the herd and the use of bought-in feedstuffs; the mineral status of the farm and its animals and the use of medicinal drugs on animals as well as the use of pesticides on crops and animals, and the use of other chemicals about the farm.

The survey drew my attention to the use of organophosphates, particularly pirimiphos methyl, in grainstores. It was common practice to dust or spray grainstores with the pesticide before bringing in the new harvest, but the pesticide was also dusted on the grain itself according to manufacturer's instructions. This was frequently done before dispatch of a load of grain to intervention store, miller or feed compounder for fear of rejection of the load if weevils, mites or any other pests were found in the grain.

This prompted me to make enquiries among feed merchants and to study the reports of the Working Party on Pesticide Residues to try to assess how much pesticide animals might be exposed to unknowingly in their food. This latter exercise showed that when grain was correctly treated with pesticide according to manufacturer's instructions, a moderately-yielding cow eating 1 tonne of a cereal product per year could ingest 200gm of Actellic Dust/year containing 4gm of the organophosphate, pirimiphos methyl.

This showed that farmers, who never knowingly used organophosphates (OP's) on their farm, could have been feeding them unwittingly to their cattle in considerable quantities in bought in compound feedstuffs.

The survey was necessarily small and the information often scrappy. I was asking farmers to dig deep into their records and memories. I am the first to admit that it is not up to the standards demanded by today's statisticians as proof', nevertheless it did produce some interesting pointers.

It indicated that [1]:

• those farms which were still run on mainly traditional lines were at much less risk of BSE occurring in their animals

• there was no evidence for a genetic link in the occurrence of BSE

• not only was copper deficiency linked to infertility (as is well known), but there also seemed to be a link between copper deficiency and the development of BSE

• there was a high infertility rate in BSE herds

• of all the chemicals used only OP's, in this limited survey, showed any apparent links with the development of BSE

• the link was not direct. OP's applied to an animal did not result in it developing BSE a few years later

• the link appeared to be between the exposure of a cow to OP's shortly before or during pregnancy. This resulted in the birth of a calf that went on to develop BSE some four to ten years later.

This last finding intrigued me and I decided to investigate further. I read extensively around the subject of slow neurodegeneration. Much to my surprise, various pieces of information slotted into place and I developed a hypothesis that seems to explain the phenomenon. My argument, except where indicated, is based on extensive laboratory work by many reputable scientists. It is as follows [2]:

• Nerve cells have an astonishing ability to repair themselves.

• If the initial damage is severe, the repair phase may be preceded by a degenerative phase in which damaged cell contents are cleared out of the distal/far end of the nerve cell membrane.

• This phase is initiated when Nerve Growth Factor (NGF) activates a molecule called Tyrosine Kinase A in the nerve cell membrane.

• Tyrosine Kinase A initiates a cascade of activities within the cell. Once the degenerative phase is complete its actions are modulated and new neurofibrils start to grow down the empty neuronal membrane.

• This pattern of slow neural degeneration and regeneration can be initiated by certain vitamin deficiencies and by many neurotoxins, including OP's.

• In all naturally-occurring cases of Transmissible Spongiform Encephalopathies (TSE's) e.g. CJD and Gerstmann-Staussler-Scheinker disease in man, and scrapie in sheep, there is evidence of genetic susceptibility and in particular of the production of aberrant prions.

• It is postulated here that prion, which has been shown to have antioxidant properties, is involved in the degenerative phase of nerve repair and that aberrant prion, in attempting to deal with activated oxygen, may be fractured and release a few fragments of prion protein known as PrP 106-126 (This describes a string of amino acids originating from positions 106-126 in an intact Prion molecule).

• Fibrils of PrP 106-126 have been shown in many experiments to activate astrocytes and microglia to produce nerve growth factor. Thus a continual reactivation of Tyrosine Kinase A would set up a vicious cycle, that could result in slow creeping degeneration of the nervous system.

• Such a cycle of activity could be initiated by almost any nerve toxin in those luckless individuals, who carry aberrant prions of either genetic or sporadic origin.

• I know of no evidence that the occurrence of BSE is related to genetic prion defects, but an experiment on the effect of very small doses of OP's on embryonic brain cells showed that they caused production of prions of molecular weights varying widely from the normal.

• It is postulated that OP's could be responsible both for the production of abnormal prions in cattle and act as the trigger that could set off the degenerative cycle in BSE.

This theory/hypothesis -- like the currently held MBM/infectious prion theory -- is still only a theory.

It could be tested by further work on the effects of very low doses of OP's on developing embryonic nerve cells and by subjecting prions produced in such experiments to oxidative stress.

The resulting "stressed prion" could then be analysed for the presence of PrP 106-126 and be used instead of macerated brain tissue from "infected" animals, in a repeat of some of the many laboratory experiments carried out on laboratory animals into the transmission of various spongiform encephalopathies.

Unfortunately, I am in no position to carry out this work. If any of your readers know of anyone who might be interested in doing it, I would be very pleased if they would contact me.

If my theory is correct, then BSE is not an infectious disease.

• It cannot be transmitted by any natural process to man, sheep or any other animal.

• There is no reason to destroy the national sheep flock as it cannot become "infected" by BSE.

• CJD is not acquired by eating meat from scrapie sheep or BSE cattle.

• Low level poisoning in the womb, can provide an explanation for the development of BSE in thousands of cattle even after the most stringent bans on the feeding of MBM.

The same theory could explain some cases of new variant CJD in humans. They could, like BSE, be initiated by OP's absorbed by the mother before or during the course of pregnancy. Such cases are likely to extremely rare. Human mothers, unlike high-yielding milk cows, do not consume large quantities of pesticide-treated grain products and they have far fewer pregnancies. The chances of them conceiving at a time when the OP level in their bodies is sufficient to damage the foetus, but insufficient to kill it, are very low.

Some cases of both CJD and Scrapie could be initiated when individuals carrying naturally-occurring aberrant prions are exposed to OP's. In humans these might be anti-headlice shampoos, flysprays and wood preservatives. In sheep, the most likely source would be OP dips, but almost any neurotoxin could initiate the condition in these vulnerable individuals.

References
[1] Wolferstan F., A preliminary report on a study of BSE in cattle in relation to farm husbandry: Its possible relevance to vCJD and infertility problems in humans. Journal of Nutritional and Environmental Medicine 2001; 11(3) 207-218.

[2] Wolferstan F., Slow neurodegeneration and transmissible spongiform encephalopathies/ prion diseases. Hypothesis: A cycle involving repeated tyrosine kinase A activation could drive the development of TSE's. Medical Hypotheses (2003), 60(1), 52-64.