Daniel Carleton Gajdusek, MD, Nobelist Who Showed Transmissibility of Chronic Neurodegenerative Diseases, Dies at 85

Neurology Today:

15 January 2009 - Volume 9 - Issue 2 - p 5–6

doi: 10.1097/01.NT.0000345030.62961.1c

Article

Ellis, Fay Jarosh

Daniel Carleton Gajdusek, MD, recipient of the Nobel Prize in Physiology or Medicine in 1976 for his work on kuru, Creutzfeldt-Jakob disease (CJD), and other chronic brain diseases, died Dec. 12, at the age of 85. Dr. Gajdusek was working in Tromso, Norway, at the time of his death.Dr. Gajdusek, a board-certified pediatrician, contributed enormously to numerous fields — from neurology to virology, pediatrics, endocrinology, genetics, epidemiology, and anthropology.

EARLY LIFE INFLUENCES

In Lex Prix Nobel en 1946, an autobiographical summary he wrote for the Nobel committee, he attributed his early passion for science in no small measure to his aunt, an entomologist who collected exotic specimens and artifacts from the Philippines and Southeast Asia. As a teenager, he spent hours working in the Boyce Thompson Laboratories in his hometown of Yonkers, NY, where he synthesized and characterized a large series of halogenated aryloxyacetic acids.He attended the University of Rochester between 1940 and 1943 and earned his medical degree at Harvard Medical School in 1946 at age 23. He conducted postdoctoral research at Columbia University and at the California Institute of Technology — with Nobel laureates Linus Pauling, PhD, and Max Delbrück, PhD — and at Harvard with Nobel laureate John Enders, PhD. He became board-certified as a clinical pediatrician, before being drafted to complete military service at the Walter Reed Army Medical Service Graduate School as a research virologist. From 1952 to 1953, he conducted research at the Institut Pasteur of Teheran on rabies, arborvirus infections, scurvy, and other epidemics in Iran, Afghanistan, and Turkey.In 1954, Dr, Gajdusek took off for Australia to work as an investigator at the Walter and Eliza Hall Institute of Medical Research in Melbourne, where he launched studies on child development and disease patterns with Australian aboriginal and New Guinean populations. There, he worked with another mentor and Nobel laureate, Macfarlane Burnet, MD.

WORK IN THE FIELD

“Starting in the early 1950s, he realized that isolated human populations, living in primitive tribes, could provide important insights on a variety of diseases,” Robert Klitzman, MD, associate professor of clinical psychiatry at Columbia University, wrote in an e-mail message to Neurology Today. Dr. Klitzman chronicled his graduate work with Dr. Gajdusek in New Guinea in The Trembling Mountain: A Personal Account of Kuru, Cannibals and Mad Cow Disease (Da Capo Press 2001).Vincent Zigas, MD, a district medical officer in the Fore Tribe region of New Guinea first introduced Dr. Gajdusek in 1956 to the illness that left its victims trembling and in madness before death. Dr. Gajdusek lived among the Fore, studied their language and culture — later donating a large collection of primitive art to the Salem Peabody Museum in Massachusetts — and performed autopsies on kuru victims. He found their brains were filled with spongiform holes, providing the first medical description of this unique neurological disorder.His subsequent research — and the basis for his Nobel prize — revealed that the disease could be transmitted to primates by injecting brain tissue from victims. Kuru was spread among the Fore by cannibalism.“Carleton helped show that these agents caused Creutzfeldt-Jakob disease in humans, scrapie in sheep, and bovine spongiform encephalopathy (or mad cow disease),” Dr, Klitzman said.Stanley Prusiner, MD, later identified the agents as prions — misshapen proteins that lacked any nucleic acid — for which he won the 1997 Nobel Prize in Physiology and Medicine.

CHANGING SCIENTIFIC DOCTRINE

Before Dr. Gajdusek's work, researchers thought viral infections had two characteristics — the latent period was short, measured in days, and the symptomatic illness lasted only a few days. Sometimes, however, non-progressive neurological dysfunction was permanent (as in polio survivors). Neurodegenerative diseases such as CJD did not fit the picture of a normal viral infection.Dr. Gajdusek made several important contributions. First, he showed that what seemed to be viral infections could have a latent period measured in years, with symptoms progressing for years. He did that by showing transmissibility to primates. In doing this, concepts of chronic disease and viral infections changed.In 1958, Dr. Gajdusek became head of laboratories for virological and neurological research at the NINDS and was inducted to the National Academy of Sciences in 1974 in the discipline of microbial biology. He was awarded the Nobel Prize in 1976, and remained at the NINDS until 1997.“Other Nobel laureates held him in awe,” Dr. Klitzman said. “When Harvard Medical School decided for its 1982 bicentennial to award honorary degrees for the first time, they gave three — one to him.”Raymond P. Roos, MD, Marjorie and Robert E. Straus Professor in Neurological Science at the University of Chicago Medical Center, first met Dr. Gajdusek more than 40 years ago when he interviewed for a position in his NIH ‘Slow Virus Lab.’ “It was a different kind of interview because Carleton did virtually all the talking — then and every time I was in his presence!” he said. “He was charismatic, passionate, and possessed with an infectious excitement. I subsequently spent two years in his NIH lab and then two and a half months on a Pacific expedition with him.“In many ways Carleton seemed undisciplined, but in other ways he attended to all the minute details that were key for learning new things, some of which were important for survival. Carleton documented those details in the best traditions of scholars, both in written articles and in his journals.“These journals are a gargantuan record of the days of his life, filled with everything — from the trip to Stockholm to fluctuations in his blood sugar. They chronicle a genius with a superhuman hunger for life, but with human flaws.”

PERSONAL CONTROVERSY

Indeed, those flaws sent him into exile in his later years. Over the years, Dr. Gajdusek brought more than 50 children from his trips to the South Pacific to live with him in the United States. He sent them college, and graduate school and, in some cases, medical school, Dr. Klitzman said. He legally adopted three children. But one of the boys, as an adult, accused Dr. Gajdusek of having sexually molested him as a child. Dr. Gajdusek was charged with child molestation in April 1996, and pleaded guilty in 1997. Under a plea bargain, he was sentenced to 19 months in jail, and after his release in 1998, he was permitted to serve his five-year probation in Europe.“He was a complex man, and leaves controversy behind him,” Dr. Klitzman said. “It is hard to reconcile the many aspects of his life, but he was an extraordinary scientist.”In later years, Dr. Gajdusek worked largely in the remote corners of Tramos, Norway.Dr. Roos last saw him in Amsterdam a few years ago. “Only recently,” he said, “I received some letters, two recently bound journals from the 1950s, as well as handwritten pages recounting daily events up through November 2008. A great deal of energy still filled his letters and journal entries (although his penmanship had clearly declined!. He wrote about the darkness of the Norwegian winter days, but I didn't realize quite how dark they would be.“Although many of Carleton's older colleagues have died, he maintained an extraordinarily large ‘interactome’ of friends and colleagues that seemed to remarkably increase, even though he was in his 80s. I always felt privileged to be part of this group. Carleton graciously helped and guided me in many ways, and I will miss him. He introduced me and many others to the excitement of science and medicine and to the importance of challenging established dogma.”

Source: journals.lww.com

Publication date: 2009 Jan 15

Cases: Boys adopted by Dr. Daniel Carleton Gajdusek