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Mad-Cow Disease in Cattle
and Human Beings
Bovine spongiform encephalopathy provides a case study
in how to manage risks while still learning the facts
Paul Brown
I n December 2003, the U.S. Depart-
ment of Agriculture discovered a case
of bovine spongiform encephalopathy
(BSE), often called mad-cow disease, in
a dairy cow from Washington state. The
news was more than a little disturbing
to the American cattle industry. The
mad-cow scare had previously devas-
tated the cattle business in the few
countries where BSE had been report-
ed, especially Great Britain and Cana-
da. The Canadian cattle industry has yet
to recover from the discovery of BSE in
a single cow on an Alberta farm in May
of last year. A farmer now earns as little
as 79 Canadian cents for a 400-kilogram
cow that would have earned the farmer
$500 before the scare—less than the
price of a fast-food burger.
The economic fallout is, of course, a
consequence of the discovery in 1996
that mad-cow disease could cross the
Paul Brown recently retired as a senior investiga-
tor in the Laboratory of Central Nervous System
Studies at the National Institutes of Health. He is
a board-certified internist and—by virtue of hav-
ing devoted more than 40 years of his career to the
study of transmissible spongiform encephalopa-
thy—an apprentice neuroscientist and the author
of more than 350 publications. His most recent
research focuses on iatrogenic Creutzfeldt-Jakob
disease, the potential for disease transmission
through the administration of blood and technolo-
gies to inactivate prions in contaminated meats.
He has served as chairman of the TSE advisory
committee to the Food and Drug Administration
and presently serves as a consultant to the
European Community CJD surveillance program.
His qualifications to write about spongiform
encephalopathies include a long history of han-
dling infected tissues, an increasing degree of for-
getfulness, diminishing visual acuity and an occa-
sional nocturnal muscle twitch. Address: 7815
Exeter Road, Bethesda, MD 20814. Internet:
paulwbrown@comcast.net
334 American Scientist, Volume 92 © 2004 Sigma Xi, The Scientific Research Society. Reproduction
with permission only. Contact perms@amsci.org.
species barrier to inflict human beings
with variant Creutzfeldt-Jakob disease
(vCJD). This disease is characterized by
a progression of psychiatric and neu-
rological symptoms that culminate in
death, usually a year or two after the
onset of the first indications of illness.
As of May 2000, a total of 155 cases of
vCJD had been identified: 144 in Great
Britain (where the outbreak began), 6
in France, 1 in Ireland, and 1 in Italy.
Additional single victims in Hong
Kong, Canada and the U.S. were in-
fected in the U.K., where they had been
residing during the years of peak risk,
in the late 1980s or early 1990s. The ex-
traordinary commercial and public-
health consequences of BSE, as well as
the near-global distribution of products
derived from cattle, have generated a
considerable amount of attention from
industry, government and the general
public. As a result, there is a daunting
volume of information—not all of it re-
liable—surrounding the nature of
mad-cow disease. I will here attempt
to distill the essence of what we know
about the disease, especially with re-
spect to its consequences for human
and animal welfare in North America.
The Nature of the Disease
Both BSE and vCJD belong to a family
of diseases known as the transmissible
spongiform encephalopathies (TSEs).
The oldest known TSE is scrapie,
which was first described in sheep in
the early 18th century. Natural TSE in-
fections have so far been restricted to
sheep and goats (scrapie), and to deer
and elk (chronic wasting disease).
However, many mammalian species
are susceptible to experimental infec-
tions by TSE agents, including pri-
mates, various ungulates, felines and
laboratory rodents. Although the dis-
ease has different names in different
species, each illness is an expression of
the same basic pathological process,
and they all share many clinical and bi-
ological similarities.
The disease agents that cause TSE
were recognized as being rather special
from the start. Although TSEs behave
in many ways like a viral illness, they
show some peculiar differences—for ex-
ample, a long latency period between
infection and illness and a correspond-
ingly long duration of illness. The
agents also seem to have an astonishing
resistance to inactivation and for a very
long time could not be linked to any vis-
ible structure. For many years the TSE
agents were therefore called “slow” or
“unconventional” viruses; however, all
known biological viruses contain nucle-
ic acids, and 50 years of exhaustive
searches for a disease-specific nucleic
acid have proved fruitless.
While the search was on for suspi-
cious nucleic acids, another line of re-
search was uncovering the crucial role
of a protein—called a prion—that ap-
pears to be inseparable from infectivity.
Prions are host-encoded proteins,
rather than foreign proteins, and more
than 30 different mutations in the gene
on human chromosome 20 that codes
for the prion are associated with inher-
ited forms of TSE. In the infected host,
the normal protein (which usually re-
sides on the surfaces of cells, including
neurons) is converted into an insoluble
form that is resistant to digestion by
proteinases. The chain of amino acids
that make up the insoluble form is
folded differently from the normal pro-
tein, and in some ways is similar to the
 Kevin P. Casey/Corbis

Figure 1. Mad-cow disease—or bovine spongiform encephalopathy (BSE)—was discovered on a farm in Washington State in December 2003. Al-
though only a single infected cow was found, some countries have refused to import beef from the United States unless the American cattle in-
dustry increases its surveillance of BSE. The disease has disrupted the cattle business in the United Kingdom since the 1996 discovery that BSE
could be transmitted to human beings. The cow pictured here resides on the Washington farm where BSE was discovered. The yellow tags on
the animal’s ears are part of an elaborate system to monitor the trade of individual cows. This system identified the infected cow as an import
from Canada; it also revealed that meat from the cow had entered the human food chain in certain Western states.

amyloid proteins associated with
Alzheimer’s disease. Although scien-
tists have begun to think of TSE as one
of a group of “misfolded-protein dis-
eases,” it is distinct from other amy-
loid-based diseases in that it alone is
transmissible.
Many scientists believe that the mis-
folded protein is the primary cause of
the disease—that is, the prion itself is the
transmissible agent of TSE. Healthy lab-
oratory animals inoculated with tissues
from infected animals develop the dis-
ease, and the misfolded protein that ac-
cumulates in their brains is readily de-
tectable by various immunological
methods. But there are still some funda-
mental questions that have yet to be an-
swered. How, for example, does infec-
tion trigger the accumulation of the
abnormal prion protein that clutters the
diseased brain? In other words, how
does the protein replicate? And how can
it confer the information required to pro-
duce different strains of the infectious
agent, both within and between species?
One theory suggests that the misfolded
protein acts as a seed molecule, a kind of
template, that imposes the abnormal
conformation on the normal protein.
This notion has generated considerable
interest in the scientific community, and
a number of laboratories are working on
the problem. Whatever the final judg-
ment on prions as the sole cause of TSE,
it is clear that the protein plays a crucial
role in the infectious process and is a
valuable marker of infectivity.
Whether it is a lone protein or not, the
itinerary of the infectious agent within
the body depends on how the infection
is initiated. When the agent is experi-
mentally introduced into rodents by in-
oculation, the abnormal protein repli-
cates in the spleen and the lymph nodes
and then travels along the splanchnic
nerves (which supply sympathetic in-
nervation in the abdomen) to the spinal
cord and then to the brain. If the agent is
ingested, it can bypass the spleen and
proceed directly from the gut to the
brain stem by way of the vagus nerve.
Experiments suggest that the optic and
olfactory tracts are also potential portals
of entry.
The role of circulating blood in nat-
urally occurring TSE remains uncer-
tain. Blood has been shown to be in-
fectious in experimental models of
TSE and naturally occurring scrapie
infections, and a highly probable case
of transmission by means of a
“packed” red-cell transfusion from a
patient with vCJD was recently re-
ported in Great Britain. During the
1990s, many countries, including the
U.S., imposed restrictions on blood
donations from persons who lived for
three months or more in the United

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 1960s 1720 1980s–1990s
deer and elk
?
sheep and
goats
other cattle
chronic wasting disease scrapie
BSE

1947 1986 1980s–1990s

mink

lions and tigers primates

cattle
transmissible mink bovine spongiform ungulates
encephalopathy encephalopathy (BSE)
various transmissible spongiform
encephalopathies

1994
1980s–1990s
1920

humans
19
73

variant Creutzfeldt-Jakob disease domestic cats

–2
00

humans feline spongiform encephalopathy

2003
3

sporadic Creutzfeldt-Jakob
disease

naturally
occurring
disease

humans
artificially
iatrogenic Creutzfeldt-Jakob occurring
disease disease

Figure 2. Transmissible spongiform encephalopathies (TSEs) have been found in a wide variety of mammals. Despite the various names given
to TSE in different species, these diseases are considered to be slightly different manifestations of a single pathological process. TSE appears to
be indigenous (blue) to sheep, deer and human beings. The oldest known TSE is scrapie, which was first described in sheep in 18th-century
Great Britain. Scrapie appeared in the U.S. in 1947, when it may have also entered wild populations of mink and, in the 1960s, deer and elk. In
human beings, the naturally occurring form of Creutzfeldt-Jakob disease (sporadic CJD) has been known since 1920. Bovine spongiform en-
cephalopathy (BSE) first emerged in Great Britain in 1986 after cattle ate contaminated dietary supplements believed to be made from the car-
casses of scrapie-infected sheep. Animal feed made from the remains of infected cattle then spread (red) the disease to other cattle, various zoo
animals and domestic cats. Human beings with the disease (known as variant CJD) are believed to have acquired it by eating processed-beef
products. Recently, a secondary transmission occurred in association with a blood transfusion.

Kingdom between 1980 and 1996, and
in March 2004, Great Britain instituted
a ban on blood donations from any of
its residents who had received a blood
transfusion since January 1980.
BSE Origins
The origin of BSE may never be known
with certainty, but any satisfactory ex-
planation must answer two fundamen-
tal questions. Why did BSE begin in
the mid 1980s? And why did it begin
in Great Britain?
It is generally believed that cattle were
infected by eating contaminated dietary
supplements made from the carcasses of
infected animals. The timing of the mad-
cow outbreak seems to coincide with
two changes made in the system of ren-
dering livestock carcasses into animal
feed during the late 1970s. First, the
process of liquefying separate batches of
carcasses into greaves (a water-soluble
proteinaceous slurry) and tallow (a wa-
ter-insoluble fat) was largely replaced by
a process in which carcasses moved con-
tinuously through the rendering equip-

336 American Scientist, Volume 92 © 2004 Sigma Xi, The Scientific Research Society. Reproduction
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ment, which could have resulted in un-
even or incomplete exposure to heat.
Second, a terminal extraction of greaves
with hydrocarbon solvents under steam
to extract a small amount of residual tal-
low was eliminated.
Although experiments suggest that
neither change by itself had a great ef-
fect, their combination may well have conversion
contributed to survival of the infectious
agent. If the level of infectivity was near
the threshold of transmission, even
small changes in the agent’s survival
rate could have made the difference.
Supporting evidence comes from the
fact that the prohibition of meat-and-
bone-meal dietary supplements in 1987
was followed 5 years later by a down- normal prion abnormal prion
turn in BSE cases. This is what would be
expected if the supplements were the Figure 3. A misfolded protein molecule (right) is critically involved in transmissible spongi-
source of the infection, since 5 years is form encephalopathy, and many scientists believe it to be the infectious agent of the disease. In
the average incubation period between a healthy individual, the normal prion molecule (left) typically resides on the surfaces of cells,
including neurons in the brain. In an infected person or animal, the normal protein is con-
BSE infection and manifest illness.
verted into the misfolded prion, which accumulates in plaques that clutter the diseased brain.
The use of dietary supplements made
One theory suggests that the misfolded prion acts as a template that imposes the abnormal
from cattle and sheep was widespread conformation on the normal prion. The structure of the normal protein has been confirmed by
in the 1980s. Livestock species (sheep, nuclear magnetic resonance, whereas the structure of the misfolded protein is predicted from
pigs and chickens), several kinds of zoo molecular modeling techniques.
animals, laboratory animals and pets
also received feed that was supplement- dering processes at about the same scrapie was prevalent and could easily
ed with meat-and-bone meal. Fortu- time. Moreover, the distribution of early have entered several rendering plants
nately, not all of these animals are sus- BSE cases in Britain (and other countries throughout Britain, whereas the spon-
ceptible to TSE. However, TSE does that unwittingly imported BSE from taneous BSE cases would have had to
appear to have been transmitted by feed Britain) is consistent with multiple initi- arise almost simultaneously throughout
to ungulates (including bison and some ation sites rather than a single-point the country.
zoo exotics, such as gemsbok, eland and source of infection. This favors sheep as To be fair, it should be mentioned that
kudu), felines (including lions, tigers, the source of the infection because the sporadic-BSE hypothesis more easi-
cheetahs and pet cats) and primates
(lemurs and rhesus monkeys). Pigs are
40,000 40
not at risk for oral BSE infection, and bovine variant
cases of bovine spongiform encephalopathy

chickens and dogs appear to be resis-

cases of variant Creutzfeldt-Jakob disease

spongiform Creutzfeldt-Jakob
tant to infection by any route. 35,000 35
encephalopathy disease
It is still not certain whether the in-
fected feed was made from the remains 30,000 30
ban on mecanically
of sheep or cattle. A species-crossing in- recovered meat
fection from sheep with scrapie is the 25,000 25
leading hypothesis. Accurate figures for
the international prevalence of scrapie 20,000 20
ban on meat-
are not available (farmers are generally and-bone meal
reluctant to report it), but the disease is 15,000 15
certainly widespread in Britain, which
has a relatively large ratio of sheep to 10,000 10
cattle. Thus a pervasive potential source
of infection had already existed in 5,000 5
Britain, whereas BSE was unknown un-
til the epidemic began in 1986. More- 0 0
over, the argument that an unrecog- 1987 1989 1991 1993 1995 1997 1999 2001 2003
nized spontaneous case of BSE served year of onset
as the founder of the BSE outbreak has a Figure 4. Incidence of bovine spongiform encephalopathy (BSE) anticipated the rate of variant
formidable obstacle. Sporadic cases of Creutzfeldt-Jakob disease (vCJD). The delay between the peak incidences of the two diseases
BSE could not have been occurring only corresponds to the latency period between infection and manifestation of the illness. The ban
in Britain, and yet no coincident epi- on using “meat-and-bone meal” for cattle feed in 1988 was responsible for the downturn of
demics occurred in other countries BSE, whereas the 1995 ban on “mechanically recovered meat” for human food products in the
(such as the U.S.) that changed their ren- U.K. greatly reduced the occurrence of vCJD.

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 slaughterhouse
cutting
carcass plant

inedible
offals
glands carcass
fat meat
remains

blood

hides long bones

rendering
plant
hooves
press

offals

bone residue
tallow

meat-and-bone mechanically
meal recovered meat

Figure 5. Two cattle-derived products—meat-and-bone meal and mechanically recovered meat (red)—were the likely vehicles for the spread of
BSE to animals and human beings in the 1980s and ‘90s. Meat-and-bone meal (partially derived from infected viscera and nervous tissue) was
incorporated into feed for cattle, zoo animals and domestic pets, whereas mechanically recovered meat (containing infected nervous tissue) was
incorporated into packaged meat products—such as sausages, beef patties and luncheon meats—intended for human consumption. Once a cow
enters a slaughterhouse, more than 99 percent of the animal is used for food or other products.

ly explains two curious features of the
disease. Like most infectious pathogens,
the scrapie agent has many distinguish-
able strains, but BSE seems to be caused
by a single strain. Although there is
precedence for strain selection when a
pathogen crosses from one species to
another, a bovine origin for BSE elimi-
nates the need for this kind of selection
process. The other distinguishing fea-
ture of BSE is that it is transmissible to
human beings, whereas all evidence to
date suggests that scrapie is not. Why
should the passage of scrapie through
a bovine species change its pathogenici-
ty for human beings? As a matter of
fact, an analogous phenomenon has
been experimentally documented: BSE
can infect mice directly but is unable to
infect hamsters unless first passed
through mice. However, because this
kind of interspecies behavior is excep-
tional, most scientists believe that the
balance of evidence points to scrapie as
the source of BSE.
If scrapie were the infectious source
of the disease in cattle, it raises the pos-
sibility that the BSE agent could com-
plete the circle by going back to sheep in
infected feed. This would be especially
disturbing because in the process of
“back-crossing” to sheep, the agent
might carry its newly acquired ability
to infect humans. It is presently impos-
sible to detect such back-crossing be-
cause the clinical, pathological and mol-
ecular biological manifestations of the
disease in experimentally infected
sheep are indistinguishable from native
versions of scrapie.
Variant CJD
Variant CJD is the fourth member of the
CJD family—sporadic and inherited
forms of CJD were first described in the
1920s, and iatrogenic CJD (caused by
medical procedures) was recognized in
the 1970s. The first clue that something
new was happening was the appear-
ance of a distinctive form of spongi-
form encephalopathy in adolescents

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with permission only. Contact perms@amsci.org.
and young adults in a country still re- 60
covering from a vast epidemic of
spongiform encephalopathy in cattle. A variant
Creutzfeldt-Jacob
connection between BSE and the hu- 50
disease
man cases was strongly suggested, and
has since been experimentally con- sporadic
firmed by the molecular similarity of 40 Creutzfeldt-Jacob

number of cases
disease
their misfolded proteins, which are dis-
tinct from those of all other TSEs. The
30
clinical and pathological features of
vCJD also differ from those of sporadic
CJD. Instead of the usual onset of mem- 20
ory loss and incoordination that charac-
terize sporadic CJD, patients with vCJD
present with psychiatric problems and 10
complain of sensory symptoms such as
pain, numbness or “pins and needles.”
Their illness lasts longer (on average, 0

4
9
4

9
40 9

9
4

4
4

4
50 9

4
4

9
9

–2
–2

–3

–5
about 14 months, instead of 4 months

–8

–9
–8
–5
–3

–6
–6

–7
–4

–4
–1

–7
–1
20

25

30

70

85

90
80
55
35

65
60

75
45
10

15
for the sporadic disease), and at autop-
sy their brains contain myriad “daisy age at onset of illness
plaques”—deposits of misfolded amy- Figure 6. Age at onset of illness for variant CJD and sporadic CJD helps to distinguish the two
loid protein surrounded by “petals” of forms of the disease. It is not known why variant CJD targeted such a relatively young seg-
spongy tissue that are not seen in the ment of the population. The clinical and pathological features of vCJD also differ from those
sporadic disease. of sporadic CJD.
Human infections most probably re-
sulted from the ingestion of beef prod- ed in the remains of carcasses from nervous system tissues were entering
ucts that were contaminated with cen- which as much meat as possible had the human food chain through this
tral nervous system tissue; however, been removed. Spinal cords were usu- “unadvertised” paste, and that this was
this hypothesis still lacks the kind of ally removed, but cord fragments and the most likely vehicle of infection.
laboratory evidence that clinched the spinal ganglia were certainly present. That said, two very curious phenom-
identification of BSE as the source of These truncated carcasses were then ena remain unexplained. The most puz-
infection. Furthermore, epidemiologi- subjected to a process of compression zling is the relatively young age at
cal studies have not uncovered any to yield a paste of “mechanically recov- which the disease appears: The great
convincing disease clusters or pointed ered meat.” This paste was often added majority of vCJD cases have been in
to any regional peculiarities that might to a variety of packaged meat prod- people under the age of 30, which is in
link contact with BSE-infected cattle to ucts—hot dogs, sausages, beef patties, stark contrast to sporadic CJD, which
human cases of the disease. Investiga- luncheon meats and beef stews—in typically affects the 50-to-70-year-old
tions of supposed high-risk groups, proportions as high as 30 percent, but age group. Two explanations have been
such as farmers, slaughterhouse work- usually in the range of 5 to 10 percent. It proposed. The first calls into play the
ers and butchers, have not found any is now abundantly clear that central normal aging process of the immune
cases of vCJD.
Because physical contact with infect-
ed cattle could not be implicated, the
next logical possibility was exposure to
cattle products. This has proved to be
difficult to assess because in one way or
another virtually the entire British pop-
ulation is exposed to a large number of
bovine-derived products. Consumption
of meat and dairy products and expo-
sure to products containing either tal-
low or gelatin (or their derivatives) is
nearly universal. No correlation has
been established between vCJD and ex-
posure to any particular product.
The most plausible hypothesis points
to slaughterhouse practices and meat
preparation—about which the scientific Figure 7. Amyloid plaque in the brain of a patient with variant Creutzfeldt-Jakob disease
community was completely naive—as (vCJD; left) differs from that seen in a patient with sporadic Creutzfeldt-Jakob disease (right).
the key to the transmission of the dis- The centers are similar, but the zone of “spongy” tissue surrounding the vCJD core gives the
ease. Before the appearance of BSE, ver- plaque a florid, or daisy-like, appearance. The unique structure of the vCJD plaque is central
tebral columns were routinely includ- to the diagnosis of the disease. (Images courtesy of the author.)

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no
data 1–19 100–700 50,000–150,000
0 20–99 2,000–7,000 1,000,000
number of live cattle exported from Great Britain, 1988–1993
Figure 8. Export of live cattle and meat-and-bone meal from Great Britain distributed BSE to
several countries around the globe. At least 24 countries have reported cattle with BSE. (Based
on data provided by Maura Ricketts, World Health Organization.)
system. As children mature, some ele- more likely than adults to eat compara-
ments of the immune system undergo tively inexpensive products (including
atrophy, inducing lymphatic tissue ele- school cafeteria offerings) that contain
ments scattered throughout the small mechanically recovered meat. However,
intestine (called Peyer’s patches). These information about the distribution and
tissues are known to be involved at a consumption of commercial foods,
very early stage of oral BSE infections, which might provide the best clues, is
and their atrophy in adulthood could unreliable, and the dietary histories of
provide a degree of comparative resis- patients with vCJD obtained from their
tance. The second possibility concerns relatives are similarly suspect.
dietary differences between age groups. The other puzzle concerns the rela-
Children and adolescents are probably tively small number of cases. Presum-
Figure 9. Slaughterhouse practices were changed in the wake of mad-cow disease. Tissues that
might contain the BSE agent, such as the cranium and the vertebral column, are prohibited for
use in animal feed and food products for human beings. On-site inspections of rendering
plants, feed mills and slaughterhouses have also been increased. (Photograph courtesy of
Linda Detwiler, University of Maryland.)
ably, the potentially contaminated beef
products were distributed throughout
the British population of 60 million, yet
fewer than 150 cases have been identi-
fied over a period of nearly 10 years of
active surveillance, and only a handful
of cases have appeared in countries
where BSE was exported. Although ge-
netic susceptibility may play a role, the
most reasonable explanation is that in-
fectivity was very unevenly distributed
among the meat products and only
rarely achieved a transmissible level.
Transmission would have been made
even more difficult by the comparative
inefficiency of oral infections.
BSE in North America
Were it not for the international trade
in cattle and cattle feed, BSE would
probably have been confined to Great
Britain. But cattle and feed continued
to be exported from Britain to countries
all over the globe for several years—
throughout the late 1980s and early
‘90s—after the cause of BSE had been
identified. From the mid-1990s on-
wards, cases of the disease began to ap-
pear in Europe and in countries as far
afield as Oman and Japan.
Ironically, the discovery of BSE in the
United States at the end of 2003 recapit-
ulated what had occurred with scrapie
nearly 60 years before, when infected
sheep were imported from Canada,
which had earlier imported infected
sheep from Great Britain. This time, at
least one BSE-infected cow (and proba-
bly more) was imported to Canada
from Great Britain during the high-risk
1980s. At some point these cows were
probably rendered into feed that infect-
ed some “next generation” cows. The
BSE cow that was discovered in Wash-
ington state in December 2003 had been
imported to the U.S. in September 2001
from the same province of Canada (Al-
berta) in which the Canadian BSE cow
had earlier been identified. Both cows
were born in 1997, just before the rumi-
nant-to-ruminant feed ban was imple-
mented, and it is now a matter of record
that some leftover feed continued to be
used to supplement the diets of new-
born calves.
When the two cows were slaugh-
tered in 2003, neither was suspected of
having BSE, so their carcasses were sent
to rendering plants for use in feed sup-
plements. The cows were only discov-
ered to have BSE when their brains
were later tested as part of a routine
surveillance program of “downer”

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 1.0
probability of finding an infected cow
0.8
0.6
50% probability
~150,000 cows per year
0.4
0.2
1 2
number of cows tested per year (hundreds of thousands)
Figure 10. Probability of detecting an infected cow increases with the number of cows that are
tested. Here the curve shows the chances that at least one infected cow will be found during a
five-year period when the incidence of BSE is one per million. Since the United States appears
to be free of indigenous disease, a testing program could reveal whether or not BSE occurs
spontaneously.
(nonambulatory or disabled) cows, and
beef products from the cow in Wash-
ington state had already entered the
human food supply.
Regulatory agencies in Canada and
the U.S., primed by the mad-cow scare
in Great Britain and continental Europe,
reacted quickly in response to the dis-
covery of the infected cows. All living
cows that could be traced from both
farms, as well as products made from
their tissues, were destroyed. A host of
new regulations were also either imple-
mented or proposed, of which the most
important were these: prohibition of tis-
sues known to be infectious in BSE cows
(cranium, vertebral column, distal
ileum, lymphoreticular tissues) for use
by either animals or human beings;
elimination of mechanically recovered
meat unless it could be shown by sensi-
tive immunologic tests to be free of ner-
vous tissue; initiation of a much-in-
creased BSE immunological testing
program for downer cows (which are
henceforth not to be used for food or
other products for either animals or hu-
man beings); and a “beefed-up” pro-
gram of onsite inspections of rendering
plants, feed mills and slaughterhouses.
Along with a strictly enforced ruminant-
to-ruminant feed ban, these measures
should prevent the future spread of BSE
to other animals and human beings—
even if the disease is eventually found to
occur as a rare spontaneous event.
www.americanscientist.org
95% probability
~600,000 cows per year
3 4 5 6 7 8 9 10
Given the absence of indigenously in-
fected cattle and the expanded BSE test-
ing program, the U.S. has both the moti-
vation and resources to answer, once and
for all, the vexing question of whether or
not BSE occurs spontaneously. If it occurs
at the same rate as sporadic CJD in hu-
man beings (one case per million per
year), several years of testing hundreds
of thousands of cattle will be required to
obtain a statistically significant result. Be-
cause other equally motivated countries
are disqualified by the occurrence of cat-
tle with foodborne BSE, only in the U.S.
do we still have this unique, one-time
only opportunity to confirm or refute the
thesis that the BSE outbreak could have
resulted from a spontaneous occurrence.
When BSE and its human counter-
part, vCJD, pass into history—which
they undoubtedly will—some may be-
lieve that a quarter century of scientific
knowledge was gained at the expense
of veterinary and public health. This
would be neither fair nor accurate.
Great Britain and indeed the world
owe an immense debt of gratitude to
the few key investigators who correctly
identified the basic issues with extra-
ordinary rapidity. The problem was in
translating the evolving scientific
knowledge into government policy, be-
cause until public concern forces the is-
sue, governments are almost always
loathe to take a proactive stance in the
face of purely hypothetical risks. This
© 2004 Sigma Xi, The Scientific Research Society. Reproduction
with permission only. Contact perms@amsci.org.
is due in great measure to the fact that
regulations designed to protect public
health from imagined dangers invari-
ably cause real damage to the commer-
cial sectors involved, and unless the
data are undeniable, these interests will
understandably argue against restric-
tive measures. It is thus not at all clear
that any so-called “lessons” will inform
future situations, because risk-benefit
analyses that are based on incomplete
knowledge depend on both scientific
and political considerations and will
continue to frustrate scientists and pol-
icy makers alike.
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For relevant Web links, consult this
issue of American Scientist Online:
http://www.americanscientist.org/
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2004 July–August 341