Daniel Carleton Gajdusek

Daniel Carleton Gajdusek (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, implying the existence of an infectious agent, which he named an 'unconventional virus'.

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.

This disease was rampant among the South Fore people of New Guinea in the 1950s and 1960s.

Gajdusek connected the spread of the disease to the practice of funerary cannibalism by the South Fore.

With elimination of cannibalism, kuru disappeared among the South Fore within a generation.

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.

He lived among the Fore, studied their language and culture, and performed autopsies on kuru victims.

Gajdusek concluded that kuru was transmitted by the ritualistic consumption of the brains of deceased relatives, which was practiced by the Fore.

He then proved this hypothesis by successfully transmitting the disease to primates and demonstrating that it had an unusually long incubation period of several years.

He did this by drilling holes into chimps' heads and placing pureed brain matter into the cerebellum.

These animals then developed symptoms of kuru.

This was the first demonstration of the infectious spread of a noninflammatory degenerative disease in humans.

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.

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.

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.

He was discharged from the military in 1954.

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).

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.

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

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.

He was elected as a member of the American Philosophical Society in 1978.

Gajdusek's best-known work focused on kuru.

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.

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

Gajdusek's father, Karol Gajdusek, was a Slovak butcher from Smrdáky, Slovakia.

His mother Ottilia Dobróczki, and maternal grandparents, ethnic Hungarians of the Calvinist faith, emigrated from Debrecen, Hungary.

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.

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.