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The Pathological Protein. Ch. 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 F uture.
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The Pathological Protein Ch. 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?
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
