D Carleton Gajdusek:DR DANIEL Carleton Gajdusek, the brilliant yet tragically flawed US paediatrician, virologist and anthropologist who won the 1976 Nobel Prize in medicine for his identification and description of kuru, the exotic disease of a remote tribe in New Guinea that was caused by agents called prions, has died. He was 85.
An energetic and intellectual researcher, Gajdusek often claimed that he was more proud of his anthropological studies among the Fore and Anga people of Micronesia than the research that brought him the ultimate prize. But he spent the last decade of his life living in exile in Europe following his arrest and imprisonment for molesting one of the more than 50 children he brought to the US, adopted and educated.
Intrigued by his two years studying rabies, plague, arbovirus infections and scurvy at the Institut Pasteur in Tehran, Iran, Gajdusek scoured the Hindu Kush, the jungles of South America, the coasts and mountains of New Britain and the swamps and high valleys of Papua New Guinea and Malaysia searching for a rare disease he could make his own.
He found it when he met Dr Vincent Zigas, an Estonian medical officer who served the Fore people in eastern Papua New Guinea. Zigas introduced him to the stone-age people, who were suffering from a mysterious malady that they called kuru, from the Fore word meaning "to shake".
The disease, which caused trembling, sporadic fits of laughter and madness before inevitably leading to death, affected one out of every 10 people in the 35,000-member tribe. Autopsies after death showed that victims' brains were riddled with holes, making their once solid organs resemble sponges - leading to the general name spongiform encephalopathies for diseases in the class.
Gajdusek described the disease in a 1957 issue of the New England Journal of Medicine and took samples to his lab at the National Institutes of Health in Bethesda, Maryland, for study.
He concluded that the disease was spread during an ancient funerary ritual when women and children consumed the brains of the deceased.
Since that practice was formally banned in 1959, no new cases of the disease have appeared except in those who were exposed before the ban took effect.
Gajdusek injected samples of diseased human brain tissue into some chimpanzees. At first, that yielded no results. But after periods ranging from one and a half to three years, the animals began to develop symptoms similar to those exhibited by the Fore.
In a 1966 paper in the journal Nature describing the transmission experiments, he dubbed the infectious agent a "slow, unconventional virus".
Further studies by Gajdusek and others showed that a similar agent was responsible for a disease in sheep called scrapie, another disease in humans called Creutzfeldt-Jacob disease (CJD) and the variant form of Creutzfeldt-Jacob disease, commonly known as mad cow disease.
Neurologist Stanley Prusiner of the University of California, San Francisco, eventually identified the infectious agent as an unexpected rogue form of protein called a prion - a feat that won him a Nobel Prize in 1997.
Prions are mis-folded forms of protein that, through mechanisms not yet understood, induce other proteins to assume similar shapes, disrupting cellular metabolism and killing cells in the brain.
Gajdusek later helped other researchers find genetically isolated populations that helped shed light on the causes of hermaphroditism, Huntington's disease and other rare illnesses.
Daniel Carleton Gajdusek was born in 1923 in Yonkers, NY, the son of a Slovak father and a Hungarian mother.
As a child, he demonstrated an interest and skill in science, stimulated by his aunt, Irene Dobroscky, who worked at the Boyce Thompson Institute for Plant Research in New York.
She tutored him in the biology of plants and the insects that infested them and introduced him to other researchers at the institute. As a teenager, he spent time working in the laboratory of chemist John Arthur, synthesising a variety of compounds that Arthur thought might have insecticidal properties.
One of those chemicals, 2,4-dichlorophenoxyacetic acid, became a commercial weed killer, and the institute based its patent claims on young Gajdusek's laboratory notebook.
He was an undergraduate at the University of Rochester, then entered Harvard Medical School, where he received his degree in 1946, specialising in paediatrics.
In 1951, he was drafted into the US army and sent to the Walter Reed Army Medical Service Graduate School as a research virologist, spending time at the Tehran institute.
Afterwards, he went to Australia to perform postdoctoral work at the Walter and Eliza Hall Institute of Medical Research. It was on the way home from this interlude that he came across the Fore people, which changed the course of his life forever.
Some critics have charged that Gajdusek brought children from the tribes to the United States and adopted them to satisfy his paedophilia. But anthropologist Ceridwen Spark of Monash University in Australia, who studied Gajdusek, argues that it was his need for an extended family that led him to help the boys.
In 1996, one of his boys, by then an adult in college, went to police claiming that his adopted father had abused him as a teenager. The FBI then recorded a call between the youth and Gajdusek in which the latter admitted that he was a paedophile, that he had touched the boy sexually and that he had had sex with some of the other boys.
He named one other boy in the conversation, and the boy confirmed the story. But Gajdusek's friends helped the boy return to New Guinea and police could prosecute only in relation to the initial offence. On the advice of his lawyer, Gajdusek negotiated a plea bargain and in April 1997 was sentenced to 12-18 months in jail. He was released the following April and immediately left for France, spending the rest of his life working and writing in Europe.
Daniel Carleton Gajdusek: born September 9th, 1923; died December 12th, 2008