Daniel Carleton Gajdusek

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Daniel Carleton Gajdusek
Born(1923-09-09)September 9, 1923
Yonkers, New York, U.S.
DiedDecember 12, 2008(2008-12-12) (aged 85)
Tromsø, Norway
Alma materUniversity of Rochester
Harvard Medical School
Known forEarly discovery of prion disease
AwardsE. Mead Johnson Award (1963)
Nobel Prize in Physiology or Medicine (1976)
Scientific career
FieldsMedicine

Daniel Carleton Gajdusek (/ˈɡaɪdəʃɛk/ GHY-də-shek;[1] September 9, 1923 – December 12, 2008) was an American physician and medical researcher who was the co-recipient (with Baruch S. Blumberg) of the Nobel Prize in Physiology or Medicine in 1976 for work on the transmissibility of kuru,[2] implying the existence of an infectious agent, which he named an 'unconventional virus'.[3] In 1996, Gajdusek was charged with child molestation and, after being convicted, spent 12 months in prison before entering a self-imposed exile in Europe, where he died a decade later. Despite Gajdusek openly admitting to molesting boys and his approval of incest[4], he still received support from peers advocating for clemency who felt his crimes were lessened by his scientific contributions.

His papers are held at the National Library of Medicine in Bethesda, Maryland[5] and at the American Philosophical Society in Philadelphia, Pennsylvania.[6]

Early life and education

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Gajdusek's father, Karol Gajdusek, was a Slovak butcher from Büdöskö, Kingdom of Hungary, now Smrdáky, Slovakia. His mother Ottilia Dobróczki, and maternal grandparents, ethnic Hungarians of the Calvinist faith, emigrated from Debrecen, Hungary. Gajdusek was born in Yonkers, New York, and graduated in 1943 from the University of Rochester, where he studied physics, biology, chemistry, and mathematics.

He obtained an M.D. from Harvard University in 1946 and performed postdoctoral research at Columbia University, the California Institute of Technology, and Harvard. In 1951, Gajdusek was drafted into the U.S. Army and assigned as a research virologist at the Walter Reed Army Medical Service Graduate School.[7] He was discharged from the military in 1954.

Career

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In 1954, he worked as a visiting investigator at the Walter and Eliza Hall Institute of Medical Research in Melbourne, Australia. There, he began the work that culminated in the Nobel Prize. From 1970 to 1996, Gajdusek was the chief of the Laboratory of Central Nervous System Studies at NINDS at the National Institutes of Health (NIH).[8] He was elected as a member of the American Philosophical Society in 1978.[9]

Kuru research

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Gajdusek's best-known work focused on kuru. This disease was rampant among the South Fore people of New Guinea in the 1950s and 1960s. He received the 1976 Nobel Prize in Physiology or Medicine for demonstrating, alongside Michael Alpers and Clarence Gibbs Jr, that Kuru was a transmittable disease.

Gajdusek was introduced to the problem of kuru by Vincent Zigas, a district medical officer in the Fore Tribe region of New Guinea. Gajdusek provided the first medical description of this unique neurological disorder, which was miscast in the popular press as the "laughing sickness" because some patients displayed risus sardonicus as a symptom. Gajdusek began investigating the disease in 1957 but could find no likely cause for the epidemic; no conventional infectious agent, no nutritional deficiency, no toxin (Gajdusek and Zigas 1957).

He is often incorrectly credited for establishing the direct link between cannabilism and Kuru disease. In reality, the first investigators to propose the hypothesis were married couple Shirley Lindenbaum and Robert Glasse.[10][11][12] In 1968, Shirley Lindenbaum and Robert Glasse, together with John D. Mathews, an epidemiologist based at Okapa, published an article in the Lancet that consolidated all the anthropological and epidemiological evidence, demonstrating definitively that cannibalism was responsible for the spread of kuru among the Fore.[13]

Gadjusek was initially dismissive of anthropological evidence gathered by Shirley Lindenbaum and Robert Glasse in 1963 that suggested not only that Kuru was transmissible, but that cannablism was the likely mode of transmission. The Glasses felt further evidence was required to support this theory and discussed it with Alpers who relayed the theory to Gadjusek. It was with Alpers' encouragement that the experiment involving the inoculation of affected brain tissue into chimpanzees was conducted and then established in 1965 the transmissibility of the disease.[13] This was done by drilling holes into chimps' heads and placing pureed brain matter into the cerebellum.[14][15] These animals then developed symptoms of kuru.

In the 2000s, Gajdusek was asked when the hypothesis of cannibalism as a vehicle to spread kuru was first envisaged. His response, “even completely drunk would come to the conclusion that a disease endemic among cannibals must be spread through eating corpses”. Contradictingly, during his Nobel Prize lecture he stated that kuru was a "slow virus" spread by “conjunctival, nasal, and skin contamination with highly infectious brain tissue” [16]

Kuru was shown to have remarkable similarity to scrapie, a disease of sheep and goats caused by an unconventional infectious agent. Subsequently, additional human agents belonging to the same group were discovered. They include sporadic, familial, and variant Creutzfeldt–Jakob disease. Gajdusek recognized that diseases like Kuru and Creutzfeldt–Jakob disease were caused by a new infectious agent that had not yet been identified.[2] Further research on the scrapie agent by Stanley Prusiner and others led to the identification of endogenous proteins called prions as the cause of these diseases.

"Unconventional viruses"

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In his 1977 paper "Unconventional Viruses and the Origin and Disappearance of Kuru", Gajdusek postulated that the cause of kuru, scrapie and Creutzfeldt–Jakob disease were caused by what he termed an "unconventional virus". In comparison to normal viruses, unconventional viruses had a long incubation period and did not cause an immune response in the host. Although Gajdusek noted that there were no demonstrable nucleic acids in unconventional viruses, he did not rule out the possibility that unconventional viruses contained RNA at a low level, despite their radiation resistance.[3]

These infectious agents were later discovered to be misfolded proteins, or prions.

Child molestation convictions

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In the course of his research trips in the South Pacific, Gajdusek had brought 56 mostly male children back to live with him in the United States and provided them with the opportunity to receive high school and college education. One of these boys, now a grown man, later accused Gajdusek of molesting him as a child.

Gajdusek was charged with child molestation in April 1996, based on incriminating entries in published journals, his personal diary, and statements from a victim. In journals published and distributed by the NIH, Gajdusek wrote about sex between men and boys in New Guinea, Micronesia, and other Polynesian islands, and about his own sexual experiences with boys during his research trips.[17]

Gajdusek pleaded guilty in 1997 and, under a plea bargain, was sentenced to 12 months in jail. After his release in 1998, he was permitted to serve his five-year unsupervised probation in Europe. He never returned to the United States and lived in Amsterdam, spending winters in Tromsø, Norway,[18] where the polar night around the winter solstice helped him to do more work.[1]

Gajdusek's treatment was denounced in October 1996 as anti-elitist and unduly harsh by controversial former Edinburgh University psychologist Chris Brand.[19]

The documentary The Genius and the Boys by Bosse Lindquist, first shown on BBC Four on June 1, 2009, notes that "seven men testified in confidentiality about Gajdusek having had sex with them when they were boys", that four said "the sex was untroubling", while for three of them "the sex was a shaming, abusive, and a violation". One of these boys, the son of a friend and now an adult, appears in the film. Furthermore, Gajdusek openly admits to molesting boys and his approval of incest.[4] The film tries to analyse Gajdusek's sexual behaviour, and also to understand his motivations for science, exploration, and life.

Death and legacy

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Gajdusek died December 12, 2008, in Tromsø, Norway, at the age of 85. He was working and visiting colleagues in Tromsø at the time of his death.[20]

Hanya Yanagihara's 2013 novel, The People in the Trees, is based on Gajdusek's life, research, and child molestation conviction.[21] The novel centers on a character named A. Norton Perina, inspired by Gajdusek, who researches the life-extending properties of sacred turtle meat in Micronesia.[22]

Works

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Books

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  • Acute Infectious Hemorrhagic Fevers and Mycotoxicosis in the Union of Soviet Socialist Republics (1953), Washington, DC: Walter Reed Army Medical Center.

Articles and monographs

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Daniel C. Gajdusek was a prolific science author come diarist, and published over 1,000 original papers, reviews and commentaries in scientific and medical journals.[23] This is an incomplete list of some of the more cited ones.

  • Gajdusek, Daniel Carleton. Unconventional viruses and the origin and disappearance of kuru. National Institutes of Health, 1977.
  • Gajdusek; Carleton, Daniel; Gibbs, Clarence J.; Alpers, Mâ (1966). "Experimental transmission of a Kuru-like syndrome to chimpanzees". Nature. 209 (5025): 794–796. Bibcode:1966Natur.209..794G. doi:10.1038/209794a0. PMID 5922150. S2CID 39242676.
  • Gajdusek, Daniel Carleton (1957). "Degenerative disease of the central nervous system in New Guinea: the endemic occurrence of "kuru" in the native population". New England Journal of Medicine. 257 (20): 974–978. doi:10.1056/nejm195711142572005. PMID 13483871.
  • Gajdusek, Daniel Carleton (1985). "Hypothesis: interference with axonal transport of neurofilament as a common pathogenetic mechanism in certain diseases of the central nervous system". The New England Journal of Medicine. 312 (11): 714–719. doi:10.1056/nejm198503143121110. PMID 2579335.
  • Gibbs, Clarence J.; Carleton Gajdusek, Daniel (1969). "Infection as the etiology of spongiform encephalopathy (Creutzfeldt–Jakob disease)". Science. 165 (3897): 1023–1025. Bibcode:1969Sci...165.1023G. doi:10.1126/science.165.3897.1023. PMID 5804726. S2CID 206567023.

References

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  1. ^ a b Holley, Joe (December 16, 2008) "D. Carleton Gajdusek; Controversial Scientist", The Washington Post, p. B5.
  2. ^ a b "Physiology or Medicine 1976 – Press Release". Nobelprize.org. October 14, 1976. Retrieved July 21, 2018.
  3. ^ a b Gajdusek, D. Carleton (1977). "Unconventional Viruses and the Origin and Disappearance of Kuru". Science. 197 (4307): 943–960. Bibcode:1977Sci...197..943C. doi:10.1126/science.142303. JSTOR 1744284. PMID 142303. S2CID 2046908.
  4. ^ a b Lindquist, Bosse (June 1, 2009). "The Genius and the Boys". Storyville. BBC Four. Retrieved May 12, 2012.
  5. ^ "D. Carleton Gajdusek Papers 1918–2000". National Library of Medicine.
  6. ^ "D. Carleton (Daniel Carleton) Gajdusek correspondence, 1934-1988". American Philosophical Society.
  7. ^ Maugh, Thomas (December 18, 2008). "D. Carleton Gajdusek dies at 85; Nobel Prize winner identified exotic disease, was unrepentant pedophile". Los Angeles Times. Archived from the original on January 27, 2013. Retrieved May 12, 2012.
  8. ^ Gillis, Justin (1996). "NIH SCIENTIST CHARGED WITH ABUSING TEEN". Washington Post. Retrieved November 26, 2022.
  9. ^ "APS Member History". search.amphilsoc.org. Retrieved May 19, 2021.
  10. ^ Glasse R., Lindenbaum S. Fieldwork in the South Fore: The process of ethnographic inquiry. In: Prusiner S.B., Collinge J., Powell J., Anderton B., editors. Prion Diseases of Humans and Animals. Ellis Horwood; New York, NY, USA: London, UK: Toronto, ON, Canada: Sydney, Australia: Singapore: 1993. pp. 77–91
  11. ^ Matthews J.D., Glasse R.M., Lindenbaum S. Kuru and cannibalism. Lancet. 1968;292:449–452. doi: 10.1016/S0140-6736(68)90482-0.
  12. ^ Glasse R. Cannibalism in the Kuru region of New Guinea. Trans. N. Y. Acad. Sci. 1967;29:748–754. doi: 10.1111/j.2164-0947.1967.tb02298.x.
  13. ^ a b Anderson, Warwick (April 27, 2018). "Epidemiology, social history, and the beginnings of medical anthropology in the highlands of New Guinea". Medicine Anthropology Theory. 5 (1). doi:10.17157/mat.5.1.591. ISSN 2405-691X.
  14. ^ Kelleher, Colm A. (2004). Brain Trust: The Hidden Connection Between Mad Cow and Misdiagnosed Alzheimer's Disease. Simon & Schuster. p. 53. ISBN 9781416507567.
  15. ^ "Fatal Infections". New England Anti-Vivisection. 2013. Retrieved July 23, 2013.
  16. ^ Liberski, Paweł P.; Gajos, Agata; Sikorska, Beata; Lindenbaum, Shirley (March 7, 2019). "Kuru, the First Human Prion Disease". Viruses. 11 (3): 232. doi:10.3390/v11030232. ISSN 1999-4915. PMC 6466359. PMID 30866511.
  17. ^ Molotsky, Irvin (February 19, 1997). "Nobel Scientist Pleads Guilty to Abusing Boy". The New York Times. Retrieved November 26, 2022.
  18. ^ Richmond C (February 24, 2009). "Carleton Gajdusek". The Guardian. Retrieved May 9, 2019.
  19. ^ Wojtas, Olga (March 27, 1998). "'Racist' Brand loses dismissal appeal". Times Higher Education. Retrieved May 12, 2012.
  20. ^ McNeil, Donald G. Jr. (December 15, 2008). "D. Carleton Gajdusek, Who Won Nobel for Work on Brain Disease, Is Dead at 85". The New York Times. Retrieved May 12, 2012.
  21. ^ Ciuraru, Carmela (September 27, 2013). "Bitter Fruit: 'The People in the Trees,' by Hanya Yanagihara". The New York Times. Retrieved March 12, 2015.
  22. ^ Yanagihara, Hanya (August 13, 2013). The People in the Trees. Doubleday. ISBN 978-0385536776.
  23. ^ "D. Carleton Gajdusek Papers" (PDF). Special Collections & Archives, UC San Diego. 2009.[permanent dead link]

Further reading

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Wikimedia Commons has media related to Daniel Carleton Gajdusek.