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The volcanic island archipelago of New Guinea has

stirred the imaginations of westerners since its discovery

by the Europeans in the 16th century. The islands are

advantageously positioned when considering trading with

Japan and subsequent of its

colonization by the British –

Australia. The tropical

environment of the islands

combined with their sharp

mountain ranges encouraged the

development of a plethora of

animal life as well as diverse

human cultures which were able to evolve in relative

seclusion from one another. These peoples while existing in

relative freedom from the corruption inherent to formalized

society, suffered mortality rates much greater than those of

their European counterparts. Absence of formalized medical

study led to complications and deaths following even the

simplest procedures. This state of events would not be

allowed to continue however, and by the mid 1950’s the

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Australian, Danish, and American doctors were sent into the

remote areas of New Guinea to educate and cure the people

living there. It was during this time that one of them,

Vincent Zigas an Estonian born medical officer recruited by

the Australian government, who was assigned to the Fore

people inhabiting the Okapa district of Papua, encountered

what appeared to be a new disease.

Kuru, or shaking death, as the natives called it,

caused the sufferers to experience progressive muscular

tremors. Unlike other diseases of the central nervous system

Kuru killed rapidly once the symptoms developed. The

clinical stage was short in duration, and the sufferers died

invariably within months following the manifestation of

symptoms; rather than years of the as in conventional CNS

diseases. The Fore accredited the disease to sorcery and

sought retribution against suspected sorcerers – making the

already bad situation even worse by triggering revenge

killings. Zigas, however; recognized Kuru as a disease of

the central nervous system given the symptoms’ similarity to

those showcased by victims of Parkinson’s disease and early

A Fore woman caring for achild

afflicted with Kuru

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stages of Encephalitis Lethargica. His more troubling

finding was that this disease targeted women, children, and

the elderly who made up 98% of all the cases (Collinge et.

al); If the Fore were to survive as a people, Zigas would

need to find the cause of the

disease which was killing hundreds

of people each year. This troubling

situation promised to wipe the

group of nearly 35,000 Fore out

within a decade or two. He would

ship blood and brain specimens to

Melbourne’s Walter Eliza Hall Institute hoping that the

cause of the sickness could be found by conventional means

to no avail.

At a loss for what could be causing the sickness, Zigas

recruited the help of another specialist, the graduate of

Harvard Medical School, Dr. Carlton Gajdusek. The two men

took to their research with vigor and explored a number of

theories ranging from bacterial and viral pathogenesis to

the possibility that Kuru was simply a genetic defect passed

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through the familial lines. In the course of their research

they frequently found themselves buying the bodies of the

dead from their families, trading tobacco and tools for

specimens which they hoped would yield a bacterial or viral

agent; inadvertently protecting the sellers from the disease

which moved by unconventional means. (Time)

However, these theories were soon dismissed as no

bacterial or viral agent was noted in the cells of the brain

which the men studied. The genetic factor was also dismissed

as the type of mutation would have either wiped the people

out completely, or would have been found to afflict men in

the prime of their age. The researcher’s first concrete clue

came from the autopsied brains themselves. Kuru sufferers’

brains were riddled with holes, causing the organs to take

on the appearance of a sponge. Something was disintegrating

the brain tissue the question however remained as to what

the agent was. The men noted no fever, nor inflammation,

which discounted the viral theory. No bacterium or multi-

cellular organic infection was present, therefore the

Dr. Hans Gerhard Creutzfeldt.

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disease must have been caused by some sort of undocumented

at that time infectious factor.

The breakthroughs which helped the two men get to the

bottom of the disease came by pure happenstance. Perhaps

believing in superiority of the American medical

establishment; Gajdusek sent the brains of over a dozen Kuru

victims to the National Institute of

Health in Bethesda, Maryland where they

were studied by a neurologist by the

name of Dr. Igor Klatzo who found

amyloid protein plaques in the brains

of the children killed by Kuru

initially served to confuse rather than to illuminate the

situation at hand. Those types of protein deposits were

usually only found in the brains of individuals who died

from Alzheimer’s – a condition reserved for the aging, not

the young members of society. This finding appeared at first

to be yet another dead end, until Dr. Klatzo remembered a

similarity between the brains of the Kuru victims and those

documented nearly half a century earlier in 1913 by a German

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doctor by the name of Hans Gerhard Creutzfeldt. The case was

that of a young woman who was showing signs of what was

thought at the time to be dementia.

Bertha Elschker was an orphan who in 1912 was

hospitalized for muscle tremors and spasmodic involuntary

muscle action (Yam 13) her condition quickly deteriorated

and within a few months the once energetic and studious girl

was dead. When Dr. Creutzfeldt autopsied the girl’s brain he

found vacuoles present in the tissue similar to the holes

found in the brains of Kuru sufferers along with a total

absence of inflammation or swelling suggesting a strange new

agent. Bertha’s however wouldn’t be the last case of the

disease documented, as in the 1920’s another German

neurologist documented five cases of a disease which

resembled that discovered by Dr. Creutzfeldt. The medical

community noted the similarity as well and named the disease

Creutzfeldt - Jakob disease, or CJD for short.

The issue however

remained a mystery until

another serendipitous

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twist of fate brought William Hadlow, an American

veterinarian whose work focused on Scrapie, a central

nervous system disease found in sheep, across a 1959 museum

exhibit of Gajdusek’s Kuru research. Similar to Kuru and

CJD, Scrapie is a degenerative CNS disease; similar to Kuru

and CJD the fatality rate for Scrapie is 100%.

Significantly, much like CJD and Kuru – the brains of the

sheep diagnosed and subsequently killed by Scrapie are

riddled with holes which give them the appearance of

sponges, with no inflammation or anti bodies present in the

tissue. Most importantly, however; Scrapie has been proven

to be passable from one organism to another by inoculation

with stricken nerve tissue in 1939 when two French

veterinarians Jean Cuille and Paul-Louis Chelle transmitted

a mixture made from the spinal cord of a Scrapie positive

sheep into the eye of a healthy one. While the incubation

time was much longer than expected the sheep who received

the shot started showing signs of Scrapie after fifteen

months. To further test their theory the two men made

subdermal and direct-brain injection of the same mixture.

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Sure enough the inoculated sheep also developed the disease

with the incubation time extending to two years for sub-

dermal inoculation, and contracting to twelve months in case

of the injection into the brain.

Armed with the newfound knowledge Dr. Gajdusek begun to

closely study the cultural practices of the Fore people who

were known for their mortuary cannibalism; he discovered

that most of the preparation and consumption of the dead was

left to women and children as well as the elderly. This

opened a number of avenues for infection with the still

unknown agent as the children frequently played with the

brains of those deceased while their mothers, aunts, and

grandmothers prepared the carcasses for consumption. The

adult men did not partake in the custom and had been by the

virtue of doing so spared from the sickness. It didn’t take

the Australian government long to outlaw the mortuary

cannibalism once the news surfaced leading to a drastic drop

off in the Kuru incidence among the Fore people. The disease

itself hasn’t disappeared entirely, however; as recent

studies conducted in the area have yielded new cases

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following much longer incubation periods, in some cases more

than 30 years. This can be explained by levels of immunity

to the pathogen, or by “underground” cannibalism persisting

in some portions of the population of the Fore. Importantly,

however; the disease is no longer a major threat to the

peoples’ existence.

Gajdusek’s next breakthrough came when, after departing

with tissue samples, and armed with his newfound knowledge

on the earlier experiments carried out by the French team he

began experimenting on Chimpanzees having secured the

permission to do so in 1961 from Patuxent Wildlife Research

Center in Maryland. In the meantime Gajdusek returned to New

Guinea in order to continue studying Kuru in the Fore, or

perhaps simply because of the freedom he could afford there.

While he was out in the field in 1963 an associate of

Gajdusek’s, Clarence Gibbs injected three chimpanzees with a

mixture made from a Kuru ridden brain. Two years later, the

inoculated Chimpanzees begun to show signs of motor

dysfunction. Shortly thereafter the animals’ state

deteriorated sufficiently that they were euthanized and

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their brains were autopsied. The telltale vacuoles present

in the human Kuru victims were of course present. This

established the agents’ infectibility and the only remaining

piece of that particular puzzle which remained was to

transfer CJD in a similar fashion. The team was successful

in doing so and by 1968 a number of primates inoculated by

various routes including oral exposure begun showing signs

of CJD establishing its’ relationship with Kuru. (Ferreiro

54)

However, a piece of the puzzle still eluded Gajdusek

and his team against their best efforts, namely the agent

which caused the diseases. The lack of telltale swelling,

fungal growth, and inflammation ruled out the possibility of

bacteria, viruses, and parasitic infection. What could be

causing such severe diseases of people and animals, and more

worrisomely – diseases which could cross species with such

relative ease given only oral inoculation. The answer to

this question would take decades as scientists continued

experimentation with the Scrapie infected tissues of adult

sheep. Various individuals exposed the diseased tissues to

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extreme heat and cold, ultra violet radiation and in at

least one case study by Dr. Tikvah Alper dating from 1966 –

the bombardment by electrons. Not a single one of the

abovementioned approaches yielded the desired result –

namely the destruction of the Scrapie agent, and insinuated

by extension that those responsible for Kuru and CJD were

similarly resilient to the efforts of men. It seemed that no

matter what sort of punishment the tissue was exposed to,

the infectiousness persisted. These tests strengthened the

argument that the agent responsible is not a virus

considering the above approaches would have annihilated any

virus known to man.

The explanation for the agent’s endurance remained

elusive until a 1967 theory published by a mathematician by

the name of J.S. Griffith which suggested that there is a

possibility that the infectious agent responsible for Kuru,

Scrapie, and CJD among other “transmissible spongiform

encephalopathies” or TSE’s as the group of diseases became

known, could be a humble protein. His theory was met with

staunch resistance if in part to the fact that proteins lack

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nucleic acids, a component viewed as critical for the

transmission of genetic data between entities (Sommerville &

Bolton). While his theory was not immediately accepted by

the scientific establishment at large, it did provide a

launching point for other researchers in the field of

medicine by discounting the virus and germ theories which

appeared incompatible with the realities of the TSE group,

due to their resistance to destruction and filtering,

combined with a lack of telltale signs of viral infection

present in affected tissues.

The issue was delegated to the filing cabinet however;

perhaps due to what was perceived as the low probability of

the incidence of TSE’s among humans which have been at that

time observed at a rate of about one per one million per

annum in populations other than the Fore tribe which had

become famous for popularizing the issue in common

scientific research. However the world was about to find

itself to have been sitting on a time bomb, in 1971 the

clock finally finished ticking when a slew of CJD related

deaths began striking the recipients of transplants from

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individuals diagnosed with varying aging diseases, notably

Alzheimer’s. The first case was of a young American woman

who received a cornea transplant and within two years of the

operation thought to be successful, died of CJD due to an

apparent infection transferred by the organ. Things were

just beginning to get worse and by the mid 1980’s adults who

had, as children, received cadaver derived Human Growth

Hormone treatments aimed at curing dwarfism; begun to die

from a CNS disease whose course left their brains turned to

sponges much as CJD does (Walton 104). Between 1963 and

1985 approximately 7,700 U.S. children and 27,000 worldwide

were given the treatment with the hormones yielded this way,

promising a flood of deaths in the coming decade given the

long, sometimes multi-decade incubation period of the

pathogen the name and nature of which was not yet

established. However, now that the disease found its way

into the general population the race to finding it was back

on and the files found their way out of the dusty cabinets.

Scientists launched full force into the issue from vantages

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both trying to prove the existence of the modified rogue

proteins, and those aiming to dispel it.

Finally in 1978 Dr. Stanley B. Prusiner joined the

research following a 1972 encounter with a patient of his

who died of CJD. Prusiner and his team experimented with

various concentration mixtures of the Scrapie agent, having

finally focused on the

possibility that the disease is

not caused by a typical virus or

a bacterium but rather by protein

abnormalities. Prusiner and his

team continued purifying the mix while testing for the

presence of nucleic acids, the building blocks of DNA and

RNA, having continually failed to

find any in the mix they concluded

that a protein indeed is the agent

responsible for the development of

TSE’s. In 1982 they announced the

discovery to the scientific

community and 15 years later in

Left: normal proteinRight: prion aberration

viruslike particles with dense centers (as at arrows) collect in membrane bound arrays in an infected cell. (Manuelidis)

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1997 Dr. Prusiner was awarded the Nobel Award for his

discovery. Prions themselves remain a hotly contested field

of study in present day as different sides of the scientific

establishment; notably a Yale neuropathologist Dr. Laura

Manuelidis who claims that the disease is caused by a virus

and that the proteins are simply its “tracks” as the havoc

is wreaked within the brain of those affected (Manuelidis).

Regardless of the controversy over the prions (short

for protinaceous infective particle) discovered by Dr.

Prusiner there have been far-reaching resolutions passed in

order to protect people from the potential infections caused

by these pathogens. Specifically since they are able to

cross between species while remaining infective albeit with

prolonged incubation periods there has been a call issued by

the World Health Organization for a ban and immediate

cessation of use of any animal feed administered to cattle,

goat, sheep which may contain cadaver-derived materials.

Furthermore WHO released a recommendation that all countries

establish adequate testing and surveillance of animals at

risk for prion diseases and that any such animal found at

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risk for a prion disease be excluded from the processing for

human and animal consumption considering that certain prion

diseases such as the Bovine Spongiform Encephalopathy appear

to require only casual contact in order to be communicated

between hosts. This creates a humongous risk considering the

long incubation periods and the asymptomatic stages which

can take years, and in some cases – decades. The third

recommendation by the W.H.O. was that the tissues at high

risk for contamination with BSE be excluded entirely from

the diets of humans and pets. These include eyes, brains,

spinal cords, and intestines. However, as the supervision in

this area has been lax there is a strong case made for the

persistence of such practices and the inclusion of the high

risk tissues in “variety meats” and the possibility of them

being included in hamburgers and hot dogs. Furthermore

considering the survival of prions in Scrapie ridden tissues

even after exposure to extreme heat, cold, and at least in

one case – Felmaldachyde, all of which failed to stop the

communicability of Scrapie there is an ever present risk of

contamination of the machinery and tools used for

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slaughtering and separation of carcasses of asymptomatic

animals – which in turns may compromise the safety of meat

coming from healthy animals. The list of risks taken by the

U.S.A. meat industry doesn’t stop here as since the

introduction, in 1994, of the Advanced Meat Recovery systems

the processing plants have become able to trim nearly 100%

of the meat from the animals, something which they have been

previously unable to do due to the difficulties associated

with removing muscle from the bone. While initially the meat

yielded through this process was not allowed to be labeled

as beef due to what Dr. Greger refers to as the “texture

ranging from ground meat to that of tomato sauce” it took

the USDA less than one year to alter its labeling rules

therefore allowing for these products to be labeled as

“Beef” and included in jerky, sausages, and ground beef as a

volumizing substance. The risk associated with the above

process is of contamination of the meat destined by human

consumption with spinal cord and brain tissues, which as we

now know carry a higher risk for harboring prions, and as

per the W.H.O. recommendations – to be excluded from human

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consumption. To make the matters even more interesting it

also appears that the U.S.A. beef industry has taken to

feeding calves a milk replacer made out of protein and fat

derived from other cattle. This practice has been already

connected with BSE outbreaks in Germany, Japan, and Denmark.

However the practice continues because the farmers contend

that the substitute is much cheaper than whey protein.

Furthermore given the extremely long incubation periods of

these pathogens there is another added risk of contracting

BSE and vCJD via veal, long as the animal is slaughtered

before showing any serious symptoms, which as shown by

earlier research by Gajdusek can take years.

The laundry list of problems in the U.S. beef industry

continues however while the farmers pretend that BSE hasn’t

invaded the stock meant for human consumption even though

there have been 3 recorded cases between 1993 and 2008

(CDC). Since the 1990’s, vCJD a variant of the earlier

documented CJD was discovered by British researchers at the

University of London, and connected with the consumption of

contaminated tissues of cows affected with BSE. The

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aforementioned Yale researcher, Dr. Manuelidis has been

quoted that there is a strong case to be made for thousands

of Americans dying every year from the BSE triggered CJD

while being told that the disease is a simple unfortunate

draw by the hand of fate. Concurrent with Dr. Manuelidis’

statement there has been a development of CJD clusters in

the United States the incidence of CJD in which exceeds the

expected 1:1000,000 by as much as a factor of eight. These

clusters range from New Jersey through Florida, with two

originating in Allentown and Lehigh valley Pennsylvania, an

alarming development considering that the prevalence rates

in these clusters range from 1.3 times the expected

occurrence to an astounding 8.7 times the expected

occurrence in the Tampa, FL cluster of CJD.

Finally, in the nod to the Alzheimer’s connection noted

in one of the Fore child’s brain autopsied at the National

Institute of Health. Depending on the stage at which the

sufferer is killed by CJD there is a very good case to be

made for misdiagnosis of the disease. A 1989 sampling of

diagnoses performed by Dr. Maunuelidis and her husband

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uncovered that of the 46 cases performed 6 were proven to be

CJD when the autopsy was carried out. When one considers

that Alzheimer’s makes up 70 percent of all cases of

Dementia in the United States and is slated to increase

drastically over the next fifteen years there is a point for

concern over the possibility of those 13% misdiagnosed cases

having actually been vCJD. Additionally it appears that

Alzheimer’s has an increased prevalence rate amongst

minority males who can generally be expected to consume more

low quality and “variety” meat than their more affluent

counterparts. (Marcus)

In closing there are the two variants of CJD

themselves. The sporadic which appears to be connected to

its’ sufferers genetically and passed along the familial

lines, and the variant, which manifests itself within the

individuals who consumed meat contaminated by BSE. We

assume, for the time being that the two diseases are removed

cousins. However, it is also equally possible that the

sporadic CJD is actually an outgrowth of variant CJD having

been given sufficient resistance by an individual’s system.

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Furthermore there is additional risk of passing CJD to one’s

offspring sexually. This would explain the variants and

clusters having tendency of concentrating around families.

While the American consumers continue in their level of

relative ignorance to the food which is augmented with

genetic therapies, some of which may include utilizing

hormones extracted from already dead animals, and remain

indifferent to the inhumane and potentially dangerous

practice of feeding animals…well animals, they also remain

at a much higher risk for the TSE diseases.

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Works Cited

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Disease Control and Prevention. Web. 13 Dec. 2010.

<http://www.cdc.gov/ncidod/dvrd/bse/>.

"Do Reports of SCJD Clusters Matter?" Organic Consumers

Association. Web. 20 Dec. 2010.

<http://www.organicconsumers.org/madcow/morgan11304.cfm

>.

Ferreiro, Carmen. Mad Cow Disease (Bovine Spongiform Encephalopathy).

Philadelphia, PA: Chelsea House, 2005. Print.

Greger, Michael. "U.S. Continues to Violate World Health

Organization Guidelines for BSE." (2004). Web. 15 Dec.

2010.

<http://www.organicconsumers.org/madcow/Greger.pdf>.

Manuelidis, Laura. "Alzheimer's and CJD." Official Mad Cow

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cow.org/Alzheimer_cjd.html>.

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Manuelidis, Laura. "Potentially Pathogenic Virus Found in

Mad Cow Cell." Yale Office of Public Affairs & Communications.

Yale University, 30 Jan. 2007. Web. 11 Dec. 2010.

Marcus, Mary B. "Report: Minorities More Likely to Suffer

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Sept. 2010. Web. 14 Dec. 2010.

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Sommerville, Robert A., and David C. Bolton. "NOVA Online |

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Service. Web. 11 Dec. 2010.

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Walton, Priscilla L. Our Cannibals, Ourselves. Urbana: University

of Illinois, 2004. Print.

Yam, Philip. The Pathological Protein: Mad Cow, Chronic Wasting, and

Other Deadly Prion Diseases. New York: Copernicus, 2003.

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