The Pathological ProteinCh. 1-3

Ch. 1 A Death in Devizes

• Opens with the death of a U.K man named Stephen Churchill• The shocker of the first page, is that Stephen Churchill is

only 19years old• He died 2 weeks after coming to the nursing home

A Boundless Future

• Background on Stephen Churchill• Smart, engaging, talented• Participation in Royal Air Force• At the age of 17, he was tested on his “Psychological, physical,

coordination, leadership”• The only reason he could not become a pilot was because his

legs were too long for the cockpit • Why is the author giving us this background on Stephen? Is it

working?

Troubling Sings

• All reasons they thought he began to downward spiral• Depression caused by his grandfather, failing school, and

totaling the car• He was let go from his school• Began to hallucinate and forget information, like what he ate• Given antidepressants that were to no avail• What would you do in this position? If it was your loved one?

You don’t die of depression

• Health was progressively getting worse• His parents decided to place him in a psychiatric hospital• Complete attitude change, he became submissive• Realized it was not psychiatric but neurological • Sent him to Royal United Hospital in Bath to be properly

tested• He didn’t have a fever, clear blood tests, and no signs of

infection

No answers

• The family decided to bring him for a second opinion• Final decision was to conduct a brain biopsy• Neurologist didn’t have answers• As he was becoming increasingly harder to take care of,

Stephen was put into a nursing home• His parents thought he had years to live, while he died 3

weeks after arriving• Autopsy results showed that he had CJD

Ch. 2One in a Million

• Dr. Hans Gerhard Creutzfeldt• Store of Bertha Elschker, an orphan• Her physical and mental changes

• By the time of her death she was in a coma• Alfons Maria Jakob- noticed her case file • “characterize the clinical picture of the disease”• The term Cruetzfeld-Jacob disease

The Unlucky Few

• “CJD wasn’t as rare as they thought”• One in a million = thousands of cases world wide• Symptoms to death • To own a cat or not?• What was the purpose of this statement?

• 10-15% of CJD patients contract by inheritance • What causes the one in a million cjd?

Diagnosing CJD

• Still no diagnosis after all of this time• Not all patients display the same symptoms• 14-3-3 Protein• Location of where it is found • Regulates signaling between cells

• Only way to diagnose CJD is to look under microscope• About half of CJD cases are autopsied• Bertha may not have had CJD at all

Stephen’s Case: CJD

• He was diagnosed with CJD• His symptoms were unusual with CJD patients• It lasted longer than other patients• Amyloid plaques on his cerebellum• Signs of Alzheimer's• His plaques were surrounded by holes making them

look like flowers• Normally scientist rely on enzymes to break down the

proteins• Why do you think that Stephens case was so unique?

Ch. 3The Cannibal’s Laughing Death

• Cannibalism is not unique to New Guinea• Background on the Fore tribe• Catherine and Ronald Berndt, along with Shirley Lindenbaum

and Robert Glasse were anthropologists who documented the Fore tribe• They did not eat all dead relatives, especially if they had

leprosy or dysentery• They thought that the body had regenerative power

• The difference between the life of a man and woman in the Fore tribe• Do you believe that the reasons for cannibalism are acurate?

Epidemic in the bush

• Variety of reasons people were interested in the Fore• John MacArthur- a patrol officer in North Fore• MacArthur told Zigas about kuru

• Apekono – Zigas guide through the Fore region• 1900 Fore people died of kuru• Mostly women and children

• Johns Hopkins Resident Paul Brown• Everyone new it was cannibalism

• What do we think about the last section on Paul Brown?

A Real-Life M*A*S*H* Doctor

• Treating kuru with drugs• Gajdusek is introduced as a character• Sir Frank Macfarlane Burnet• Joseph Smadel• Director of Walter Reed

• Paul Brown• Worked for Gadjusek

• All different opinions of Gadjusek• Which do you believe?

Burnet (left)

Smadel

A Lifelong Pursuit Begins

• Interested in studying “child growth and development and disease patterns in primitive cultures”• Initially tested measles vaccines in the Caroline Islands• Learned about kuru from Roy F.R. Scragg• Public health official

• Grant from Australian public health department to get adequate equipment

• Harrowing feats to reach different villages• Six day journey, climbing 7000 ft• Leeches, mosquitoes, elephant grass

• Even though he was tired, he still managed to interview and get samples• Compared kuru to Parkinson's• “ The key to unlocking the mystery of kuru lay in the brain,

and that meant opening up the skull to obtain samples for postmortem analyses.”• “since they were engaged in cannibalism themselves- they

had absolutely nothing against cutting up a body”• What do we think about this last quote?

Brain Clues

• Gajdusek sent 16 brains to Smadel at NIH• Igor Klatzo• Neuropathologist• He realized the resemblance of these brains to those of

patients with Cretuzfldt- Jacob disease. • Turns out the two had very different circumstances• William J. Hadlow• The brains of sheep

Igor Klatzo