Virologist won Nobel for identifying exotic disease

Dr. D. Carleton Gajdusek, the brilliant yet deeply flawed pediatrician, 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 a family of mysterious agents called prions, died Dec. 12 at the hotel where he lived in Tromso, Norway. He was 85.

No cause of death has been released, but he had suffered for years from congestive heart failure, according to his biographer and former student, Dr. Robert Klitzman of Columbia University.

An energetic and intellectual researcher, Gajdusek often said he was more proud of his anthropological studies among the Fore and Anga people of Micronesia than he was of the research that brought him the ultimate prize.

But blinded by his hubris and self-admitted pedophilia, he spent the last decade of his life in exile in Europe after his arrest and imprisonment for molesting one of the more than 50 children he brought to the United States, adopted and educated.

Intrigued by his two years of studying rabies, plague, arbovirus infections and scurvy at the Institut Pasteur in Tehran, Gajdusek scoured the Hindu Kush, the jungles of South America and the mountains, 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 10 people in the 35,000-member tribe. Autopsies showed that victims' brains were riddled with gaping holes, making their once solid organs resemble sponges -- leading to the general name spongiform encephalopathies for diseases in the class.

The disease was not believed to result from an infection, because victims did not suffer a fever and there were no signs of inflammation -- both indications of classical infections. Most researchers thought kuru was either hereditary or the result of a dietary deficiency.

Gajdusek described the disease in a 1957 issue of the New England Journal of Medicine and took samples back to his lab at the National Institutes of Health in Bethesda, Md., for study. He concluded that the disease was spread during an ancient funerary ritual when women and children consumed the brains of the deceased.