From Cryonics magazine, September 1989:
BSE AND YOU
by Mike Darwin
It started when I was in Britain. To understand it, you have to
understand the British Press. To understand the British Press you have
only to think back to grade school about how and why dirty little scandals
and rumors propagate through a schoolyard full of children. (I should
point out that this kind of thing happens in the U.S.; it even happened to
Alcor with the Dora Kent case, but it just isn't the same as in the U.K.
There it has been been raised to a high art and major national pastime).
The scandal of the moment was the problem with the eggs. It seems one
Member of Parliament by the name of Edwina Curry (promptly rechristened
Eggwina by the British Press) alleged in public that British eggs were
contaminated with Salmonella and that all of Britain was in danger of death
by dysentery. The egg scandal grabbed national attention. Unfortunately,
it was more hype than help. However, concurrent with the egg scandal,
another little food-related problem began to be discussed. This one was
more serious. Potentially a lot more serious.
Mad Cow Disease
It seems cows all over the U.K. were going mad. Dreadfully, horribly
mad, as their brains were literally turned into a mass of spongy debris.
The cause of this catastrophe was not unknown: it was Bovine Spongiform
Encephalitis (BSE), which is like Creuzfeld-Jacob disease (CJD), and kuru
(the so-called cannibal disease), and the infection called scrapie in
sheep. We don't know much about these diseases, but what we know isn't
good. They are slow viruses, so named because the time from infection to
the the time of onset of the disease can be a decade or even two. Even
calling these diseases viruses may be wrong since every attempt to isolate
nucleic acid (RNA or DNA) molecules, the normal basis of heredity that
contains programming instructions for making cell (and virus) structures,
has failed.
Whatever it is, the infectious agents responsible for scrapie, kuru,
and now BSE are tough customers; they can withstand standard autoclaving,
freeze-drying, and filtration through 0.1 micron filters (normally 0.2
micron filters are used to sterilize pharmaceuticals such as IV fluids)!
Consider this; CJD has been transmitted to a chimpanzee from the brain of
a CJD victim which had been stored at room temperature in
formaldehyde/alcohol solution for seven months (D. C. Gajdusek, et al, N.
Engl. J. Med., 294, 553, (1976))!
The most serious problem about these diseases, however, is that they
may have wide interspecies transmissibility. And they may even be the same
disease with differences in presentation being related only to interspecies
variability. Scrapie's clinical presentation closely resembles that of
kuru and it has been suggested that they are in fact the same disease (W.
J. Hadlow, Lancet, 1959 II, 289, (1959). Regardless of whether CJD, kuru,
and scrapie are all really the same disease, it is clear that BSE and
scrapie are the same thing. And that it is especially worrisome because
scrapie has been transmitted to a wide variety of animals other than
sheep: goats, mice, rats, and, most worrisome, five species of monkeys (C.
J. Gibbs and D. C. Gajdusek, Nature 236, 73, (1972))! It passes to ewes
from lambs, possibly as a result of transplacental infection (E. E.
Manuelidis, Science, 190, 571, (1975)) and it can be transmitted to sheep
simply by having them graze in pastures previously occupied by infected
animals!
An Old Enemy
BSE, CJD, kuru and the other slow-virus neurological diseases
are invariably fatal, killing by wholesale destruction of the
cerebral cortex and related central nervous system structures. A
cryonicist entering suspension secondary to kuru or CJD would likely
need a lot of re-education on the reanimation end -- starting with
the basics -- like who he was! In the case of kuru and scrapie the
first symptoms seem to be cerebellar and cause motor abnormalities.
In kuru, the first clinical signs are usually a shivering-like tremor
that progresses to complete paralysis, dementia, and death in less
than one year. In CJD the dementia comes first and the disease is
often mistaken for Alzheimer's. In all of these diseases there is never
any remission or pause in progression of the disease, and they are always
fatal.
In short, these are not diseases you want to get, cryonicist or not.
So how did the cows of Britain get it and how can you avoid getting it?
The former is easy to answer, the latter is not.
The cows got it by being fed sheep. Yes, that's right, sheep. In
order to save money, some feed manufacturers began grinding up bone,
connective tissue, and other organ meats which consumers consider
undesirable and adding them to cattle feed (sheep's blood is also
"recycled" in this way), pet food, pig feed, and chicken feed! People who
raised concerns about spreading BSE to cows were told that their
speculations were "remote and theoretical." This was unconscionable since
over 20 years ago it had been established that mink encepalopathy was a
result of feeding scrapie-infected sheep meat to mink! So much for
armchair theorists. Right now according to the London Times (May 19,
1989), cows in more than 3000 herds have been afflicted with BSE and over
150 cows a week are being destroyed. Neuropathologist Dr. Helen Grant of
Charing Cross Hospital in London (interviewed in The Times) says that the
only logical approach in dealing with the disease is "to assume that every
beef animal is incubating the disease."
No one knows for sure whether BSE will be transmissible to humans, but
the indications are strongly that it will. It has been known for sometime
that people who eat sheep's brains or eyes have a 30- to 40-fold greater
incidence of CJD (D. C. Gajdusek,
Science 197, 943, (1977)). The histologi-
cal appearance of CJD, scrapie, and BSE are
close: in other words, they cause very
similar kinds of pathology in all three
hosts. BSE has definitely resulted from
feeding infected cattle scrapie-infected
sheep tissue, scrapie is known to have been
transmitted to a variety of primates by
feeding infected brain tissue, and the
resultant disease in these animals is
indistinguishable from experimentally pro-
duced CJD in these species.
Neurosurgery
As an aside, it is probably worth
mentioning that there is another apparently
significant risk of CJD: neurosurgery. A
disturbingly large number of people who
have had neurosurgical procedures go on to develop CJD (W. B. Mathews, J.
Neurol. Neurosurg. Psychiatry, 38, 210, (1975)) suggesting that the cause
of CJD in these cases was not the underlying neurological disease, but
rather contamination with CJD during the procedure! One particularly sad
and well documented case was the development of CJD in two young patients
who were recipients of implanted electrodes sterilized with
formalin/alcohol after they had been used on a patient with CJD.
What It Means
Where does all this leave British, and for that matter, American
cryonicists? Well as an ovo-lacto-vegetarian I can say "I told you so."
But I would have to sheepishly (?) add that no one knows for sure that CJD
cannot be transmitted by dairy products. I do not know what cattle feeding
practices are like in the United States, but a call to the Ralston Purina
service center yielded the information that Purina "does not now and has
never used meat or animal by products in their cattle feed." The
spokeswoman was also kind enough to read me a complete list of ingredients
in their cow chow (all safely vegetarian).
Of course, some of the beef that enters the U.S. food chain is from the
U.K., as are some processed products. But this probably constitutes only a
small fraction of beef consumed by Americans. In England the situation is
downright scary. Britishers (and Americans) unfortunately do end up eating
a lot more cow brains and eyes than they think. Those little white,
gelatinous flecks in your breakfast sausage are very likely what remains of
the brains of some cow. And brains aren't used just in sausages, they are
also used in hot dogs and some luncheon meats!
Avoiding meat products and perhaps dairy products from herds that have
been fed contaminated feed would be one way to eliminate the risk of
infection (if you're not, in fact, already infected). In Britain, this may
be a difficult thing to do (short of becoming a vegetarian).
There is some speculation that Alzheimer's disease may be caused by a
similar slow "virus" (although repeated attempts at achieving transmission
to animal hosts have failed) and that life-long vegetarians do not get the
disease! It is well established that vegetarians live approximately seven
years longer than meat eaters with comparable "other
risk" profiles (such as smoking and alcohol intake) and that they suffer
dramatically less autoimmune and degenerative diseases such as lupus,
autoimmune kidney disease, and arthritis. This alone should give meat
eaters pause for thought.
Perhaps the most disturbing thing about CJD, BSE, and scrapie is not
just that we know so little about them, but that we've known so little for
so long and despite the expenditure of a great deal and of time and money
using the best tools of modern molecular biology. If BSE turns out to be
the AIDS of England, then a disaster of unbelievable proportions is about
to unfold in the next decade or two.
Unfortunately, there is, given our current level of understanding, only
one way to find out: wait 10 or 20 years and see what happens.
In the meantime, the best recommendations we can give to avoid
BSE/CJD/Scrapie are:
1) Do not eat any brain or CNS system tissue from any animal in any
form, including sausages, luncheon meats, or "hot dogs."
2) Avoid skin, mucous membrane or wound contact with brain or CNS
material from any animal.
3) For an increased margin of safety do not eat meat at all.
4) If you are having a neurosurgical procedure, inquire as to how the
instrumentation will be sterilized. Normal formalin/alcohol
sterilization is ineffective against CJD and steam sterilization must
be at 120øC for 45 minutes at 20 psi.
5) Leave explicit instructions in your durable power of attorney for
medical care
that you do not want feeding if the disease
develops.
If you develop any symptoms of CNS
disease that you think may result from a
spongiform encephalopathy, contact Alcor
immediately for advice an help. And don't
worry, we'll still suspend you!
For readers wishing more detailed infor-
mation about the spongiform encephalopathies
the following works are recommended:
"Unconventional Viruses and the Origin and
Disappearance of Kuru," by D. Carelton
Gajdusek, Science, 197, 943-960, (1977).
(This is Gajdusek's Nobel Prize acceptance
speech)
"The Human Spongiform Encephalopathies," by Paul
E. Bendheim, Neurologic Clinics, Volume 2, p.
281-298, 1984.
My thanks to Steve Harris for his help
with references in preparing this article.
