From the jungles to Nobel to incarceration: Carleton Gajdusek and prion diseases

Carleton Gajdusek's life story is compelling and complex, filled with groundbreaking scientific achievements overshadowed by severe ethical transgressions. Born in 1923 in Yonkers, New York, Gajdusek's journey in medicine, biophysics, and anthropology led him to uncover the nature of a mysterious neurodegenerative disease, but his life later took a dark turn, resulting in his incarceration for child molestation.

Early career

Carleton Gajdusek received his MD degree from Harvard Medical School in 1946. He then undertook postdoctoral research at Caltech, followed by clinical training in pediatrics at Johns Hopkins and Columbia. After this, he was a visiting investigator at the Walter and Eliza Hall Institute of Medical Research in Melbourne, Australia, and he also worked at the Institut Pasteur in Tehran, Iran. In 1954, Gajdusek started working at the National Institutes of Health (NIH) in the United States, where he conducted much of his groundbreaking research.

Research in the jungle

In the late 1950s, Gajdusek found himself deep in the jungles of Papua New Guinea investigating an unusual disease called “kuru,” a fatal neurodegenerative disorder that was rampant among the Fore people. The Fore tribe was plagued by a mysterious ailment, starting with an uncontrollable tremble, progressing to severe motor impairment, and eventually resulting in an untimely death. The local term “kuru” literally meant “to shake.”

Gajdusek was puzzled. The disease had a strange pattern, predominantly affecting women and children but sparing adult males. Moreover, kuru was unknown outside the Fore tribe. Gajdusek hypothesized that there must be a unique cultural practice responsible for this selective transmission.

On delving deeper into the Fore's customs, Gajdusek unearthed a chilling practice--endocannibalism. The Fore people consumed the bodies of their deceased as a form of respect and mourning. Women and children were usually the ones involved in this ritual, explaining the strange disease pattern.

Gajdusek hypothesized that the kuru disease agent was being transmitted through this practice of endocannibalism. To test this, he took brain tissue samples from deceased kuru victims back to the United States. In a groundbreaking experiment, he injected the extracts into chimpanzees. After a long incubation period, the chimpanzees started showing symptoms resembling kuru.

Groundbreaking discovery

Around this time, another neurodegenerative disease, Creutzfeldt-Jakob disease, was gaining recognition in medicine. Creutzfeldt-Jakob disease shared uncanny similarities with kuru, including rapid progression and similar changes in brain tissue.

Putting the pieces together, Gajdusek hypothesized that kuru and Creutzfeldt-Jakob disease were transmitted by a new class of infectious agents. The fact that kuru was not associated with any inflammation or immune response in the brain, as is typically seen with bacterial or viral infections, suggested that a different type of agent was involved. Gajdusek's discovery was groundbreaking. It challenged the conventional wisdom that infectious diseases could only be caused by organisms like bacteria and viruses, opening a whole new chapter in the field of infectious disease.

In 1976, Carleton Gajdusek was awarded the Nobel Prize in Physiology or Medicine for his path-breaking work on "slow virus" diseases, now known as prion diseases.

Prion diseases

Although Gajdusek's discovery was pioneering, it was Stanley Prusiner's proposal of "prions" (proteinaceous infectious particles) in 1982 that illuminated the cause of these strange infectious diseases. Prusiner proposed that these proteins could cause disease in a novel way - not by carrying genetic material, like viruses or bacteria, but simply by their abnormal shape. Prusiner showed that prions, entirely devoid of nucleic acids like DNA or RNA, could trigger diseases, thus revolutionizing our understanding of certain neurodegenerative disorders. Stanley Prusiner’s theory initially met with skepticism but eventually gained wide acceptance and earned him the Nobel Prize in Physiology or Medicine in 1997.

A dark turn for Gajdusek

Gajdusek’s professional achievements were overshadowed by his personal life's dark side.

Gajdusek adopted numerous boys from his Pacific and Asian field trips, a fact celebrated by the media initially. The press often portrayed Carleton Gajdusek's adoption of children from the regions where he conducted research as an act of humanitarianism. His work took him to remote and often impoverished parts of the world, and the fact that he brought dozens of children from these areas to live with him in the United States was seen as a benevolent act, giving these children opportunities they would not have had otherwise.

However, in 1996, Gajdusek's life took a dramatic turn when one of his adopted children accused him of sexual abuse. This revelation shed a new and deeply troubling light on Gajdusek's actions when he pleaded guilty to child molestation charges in 1997. His legacy became inextricably linked to the controversy and criminal conviction that followed, tarnishing his reputation permanently.

After serving his sentence, Gajdusek moved to Europe, where he lived until his death in 2008. The tale of Carleton Gajdusek serves as a stark reminder of the dual nature of humanity, capable of both great scientific discovery and terrible misdeeds.

MedLink acknowledges the use of GPT-4 in drafting this blog entry.