The Journals of Dr. Carleton Gajdusek

 

The Bernard Becker Medical Library houses a very interesting collection of over forty type-written journals that were published by Dr. Carleton Gajdusek, the 1976 Physiology/Medicine Nobel laureate. Dr. Gajdusek used the journals to record his thoughts, research notes, and general experiences as a scientist who studied neurological diseases in developing countries. The journals include entries for each year between 1951 and 1988. Dr. Gajdusek’s conducted research in a number of foreign countries including Iran, Afghanistan, Turkey, Australia, and Papua New Guinea.

Daniel Carleton Gajdusek was born on September 9, 1923 in Yonkers, New York. After graduating summa cum laude from the University of Rochester with a bachelor’s degree in biophysics in 1943, he enrolled in Harvard Medical School. Completing his medical degree in 1946, he then worked through a series of residencies, internships, and fellowships. Following his medical training, Dr. Gajdusek moved to Australia to work as a visiting instructor in virus genetics and immunology at the Walter and Eliza Hall Institute of Medical Research. During this time, Dr. Gajdusek began his work in the Eastern Highlands of Papua New Guinea.

It was in Papua New Guinea where Dr. Gajdusek first heard of kuru, a degenerative disease of the nervous system that was reaching epidemic proportions within a number of Highland tribes. Dr. Gajdusek and his colleagues worked with nearby tribes, carrying out epidemiological studies and working to develop a rough sketch of the disease. They performed a number of autopsies on victims of the disease. Despite returning to the United States in 1957 to accept a position with the National Institutes of Health, Dr. Gajdusek continued his research on the transmission of kuru.

By the mid-1960s, Gajdusek had proven that kuru was transmittable using liquefied brain tissue from deceased sufferers of the disease. With this knowledge, Gajdusek was able to illustrate that the existence of “slow virus” infections in humans was possible. This meant that certain viruses could lie dormant in the body for decades before the disease manifested itself. The breakthrough also led to the discovery of a new type of “slow virus” called transmissible spongiform encephalopathies (TSEs), infections which could be transmitted between humans and animals. TSEs contain small infectious agents made of protein (later known as prions) which bind to pieces of plasma membrane and induce abnormal folding of normal prion proteins in the brain.

In 1976, Gajdusek’s two decades of work on kuru were acknowledged when he was awarded the Nobel Prize in Physiology or Medicine for his discovery into the mechanisms of infections and the pathogenesis of human diseases. For the remainder of his life, Dr. Gajdusek continued his work on slow viruses, devoting the majority of his time to TSEs and treatments. His work has led to number of medical breakthroughs and great strides were taken which helped to establish the genetic basis for Huntington ’s disease, the causes of hermaphroditism, and the causes of a number of rare illnesses. His work on kuru provided the basis for the eventual discovery of prions and a number of other TSEs or prion diseases. In addition to prion diseases known to infect humans, such as Creutzfeldt-Jakob disease (CJD) and Variant Creutzfeldt-Jakob disease (vCJD), some researchers now believe that prions may play a role in producing dementia in certain patients and possibly even triggering certain types of cancer.