X-Message-Number: 6017
Date: 01 Apr 96 04:54:43 EST
From: "Steven B. Harris" <>
Subject: SCI.CRYONICS Mad Cows & Englishmen


Thought I'd post this for your interest.  As usual, the
cryonics community has been years ahead of the game in
warning its people about this, just as they were with AIDS.  
In fact, the parallels between the last few years of
suggestions that the U.K. beef supply was contaminated with
an infectious slow agent, and the suggestions in 1983 
and 1984 that the blood supply was contaminated similarly,
are striking.  In both cases, economic considerations 
won out over common sense in the first years, with people
refusing to believe they were at risk until other people
started to die.  Common sense is not that common.  The
prion agent responsible for these encephalopathies crosses
between an incredible range of hosts, including from
mammals to birds.  The idea that humans would be 
somehow immune (especially since we have transmissable
prion diseases of our own) is really stupid.  And you
read this opinion in the pages of CRYONICS 
magazine years ago, so this is not just 20-20 
hindsight.  But that seems to have been the official 
hope in Britain.  Perhaps they should read cryonics 
literature more there.

COPYRIGHT  28 March - 3 April, 1996  THE EUROPEAN

Culling 'could have stopped the rot'
Professor Peter Behan examines the effects of official inaction

is one of a group of rare neurological illnesses, the best known
of which is scrapie.
     This disorder occurs predominantly in sheep and goats and
has been well-known in England and Europe since the 18th century.
The name comes from the tendency of the animal to scratch and rub
against poles, trees and fences. The sheep become excited and
nervous, developing inco-ordination and deteriorating
progressively until death. The disease can be transferred to
another animal by inoculating brain from an infected sheep, but
how the sheep becomes infected in the first place is not known
for certain. Vertical transmission, ie from mother to lamb,
appears the most likely. Blood is infected and afterbirths on the
grass can transmit the illness to other sheep. Scrapie has been
transmitted, experimentally to goats, mice, gerbils, mink,
monkeys, chimpanzees, hamsters, guinea pigs, cats, puma, cheetah,
tigers, gemsbok, ostriches and pigs.
     Using the microscope, investigators soon found that the
typical changes in the brains of sheep with scrapie were
identical to those which occurred in humans with Creutzfeldt-
Jakob disease (CJD) and also in another rare fatal disease, Kuru,
which is found in the Fore peoples of New Guinea.
     Other even more rare forms of spongiform encephalopathy have
been described. In one form a genetic mutation has occurred in
which there is an inability to sleep, the disease progressing
inexhaustibly to death. These human illnesses generally occur in
middle to adult life, and there is an overall incidence of one
per million of the population. Before the age of 30 the incidence
is one in every 400 million, which highlights the implications of
the recent cases in young people in Britain.
     In the 1960s the average number of cases of CJD in England
and Wales was approximately five per year; in 1992 it had risen
to 60. The ages were much lower than would be expected.
     The agent that transmits these diseases was originally
considered an atypical type of virus, but it is now known, from
the work of Professor Prusiner in California, that the agent that
transmits the disease is a protein, a non-living piece of
protein, that has the unique ability to cause conformational
change in other proteins that it comes in contact with.
     Prion proteins exist in two forms: one capable of producing
disease and the other harmless. The disease producing disease and
the other harmless. The disease-producing prion is a variant of a
normal protein and may differ from its counterpart only in
possessing one different amino acid. This small change is capable
of converting the prion protein into a disease-producing agent.
These prions are in all tissues, concentrated in brain and spinal
cord but also found in bone, kidneys, liver and lungs. Bones go
to make gelatine, and ingredient of multiple foods and sweets.
     When a large number of cows in Britain became ill with BSE
in 1986, it soon became clear that they had been fed with
scrapie-infected sheep. By 1994 800 cases of BSE per week were
being reported. Cats receiving similar food developed a similar
disease, as did tigers in Bristol zoo. Indeed, many animals,
including sheep, cattle, pigs, monkeys, kudu, eland, ostriches,
puma and cheetah, developed the illness after eating such meat.
The question that arose, therefore, was whether or not these
prions in the tissue of the infected cows are capable of
transferring the disease not only to animals but to humans as
     As stated, CJD is a rare disease in man but in Britain
recently there has been an increase in cases. These have a
slightly different clinical presentation and lack the typical
features of CJD in humans. In this form, a longer period ensues
before death occurs. So far there is no method of diagnosis other
than brain biopsy, although a test on the cerebro-spinal fluid
may soon be available. The fact that children are now affected is
particularly worrying in that affected cows may be implicated.
     These spongiform encephalopathies can be transferred from
man to monkey to experimental animal and from experimental
animals to monkey and to most other animal species. Indeed,
surgical instruments that have been sterilised have been shown to
be capable of transmitting the illness when used on other
patients, as can transmission by small amounts of tissue such as
occurs with corneal transplant. The prions are not killed by
steam or boiling and can remain infective for many years.
     Recently, it has been known that children lacking growth
hormone and receiving growth hormone prepared from pooled
pituitary glands taken from cadavers at autopsies have developed
CJD. This is because some of the pituitary glands were infected
with CJD material removed from undiagnosed cadavers. So far there
have been 17 cases in Britain and already we are seeing women who
have received fertility drugs prepared in a similar fashion, also
developing CJD. CJD is, of course, fatal after a lingering,
horrible illness of months to a year. There is no cure.
     The anxiety that has been generated in Britain centres
around the fact that we know that tissue from diseased animals
have entered the food chain, and that millions of individuals
will have consumed it. Only with time that we will know the exact
number of individuals who have become infected, and when one
considers that the incubation period, on average five to 15
years, can extend up to 30 or more, the position becomes a
     Frying or roasting the meat as in cooking has little effect
on its infectivity, and the fact that so small amounts when
introduced to another species or to man can produce the disease
makes the implications for possible transfer enormous.
     For some extraordinary reason the warnings of a potential
plague were not treated with the seriousness that they demanded
originally. In my opinion, every infected herd should have been
destroyed and the fields from which they came should not have
been allowed to be used for five to ten years. While we know from
scrapie that infectivity is almost certainly in the majority of
cases passed from mother to lamb, there is some other evidence
that it may occur horizontally but always be transmitted
vertically. [Harris note-- this may be a missprint.  Occasional
horizontal transmission is suggested by scrapie recurring in sheep 
placed in fields where infected sheep have previously been].
     For this reason a procedure of culling the affected herd and
leaving the ground undisturbed offers the best hope of curtailing
the illness. Such a policy in Ireland has reduced the problem
enormously. Drastic measures along these lines will need to be
implemented in Britain-- and implemented soon--for confidence to
be restored. Already within the past few days further suspected
cases occurring in humans have been reported.

     Author: Peter Behan, professor of clinical neurology at Glasgow
University, Scotland, since 1971, has studied CJD for 25 years.

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