Viewers of hospital serials like House become familiar with the names, symptoms and treatments for a variety of obscure diseases, from Wilson’s disease to Fulminating osteomyelitis to Ornithine Transcarbamylase Deficiency to Epstein Barr to Amyloidosis and Sarcoidosis.

But even to a hardened medical-series watcher like me, the most horrifying disease that I came across was in a news report. And the disease is Kuru.

Kuru is a prion disease. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Normal prion protein is found on the surface of many cells. Prion diseases occur when this protein becomes abnormal and clumps in the brain. It then causes brain damage. (https://www.hopkinsmedicine.org/)

This abnormal protein build-up in the brain can lead to memory problems, personality changes and trouble with movement. Symptoms include rapidly developing dementia, difficulty walking and changes in gait, jerking movements of the muscles, hallucinations, confusion etc. Death usually results within a year or two.

Experts still don’t know a lot about prion diseases. There are several of them, with the most common form of prion disease that affects humans being Creutzfeldt-Jakob disease (CJD). Others include Variably protease-sensitive prionopathy, Gerstmann-Sträussler-Scheinker disease etc.

These are sometimes spread to humans by infected meat products. In rare cases, the infection can spread through contaminated corneas or medical equipment.

But fortunately prion diseases are rare, with about 200 being reported in the US, because unfortunately these disorders are often fatal—there is just no cure.

Even among prion diseases, Kuru is particularly bizarre. It is caused by eating human brain tissue contaminated with infectious prions. But why would anyone eat human brain tissue? Well, it was a widely practiced funeral ritual among the Fore people of Papua New Guinea.

The Fore people live in the Okapa District of the Eastern Highlands Province of PNG. Research in the 1950s indicated that the Fore tribe had a population of about 11,000 people. Of this small number, almost 200 people a year were dying of an unknown illness. As they started investigating this, they ruled out contaminants from the environment, common infections and the possibility of genetic inheritance. They narrowed it down to something in their practices. And then a study of their funerary rituals gave a clue. The Fore people would cannibalize their dead. The anthropologist  Hertz uncovered the reason for this—it sprang from love and respect for the deceased. ‘By this rite the living incorporate into their own being the vitality and the special qualities residing in the flesh of the deceased; if this flesh were allowed to dissolve, the community would lose strength to which it is entitled…. at the same time, endocannibalism spares the deceased the horror of a slow vile decomposition’, he wrote.

Children and women were usually more affected by Kuru  than men, probably because they consumed the brain as compared to the men who preferred muscles.

‘Kuru’ in Fore means shivering. It is also called the laughing disease because the affected would exhibit sporadic uncontrolled laughter.

The practice was banned in the 1960s by the PNG government. But sadly, the deaths continued for quite a while after that, since Kuru has a long incubation period—30 to even 50 years. So someone infected in the late sixties might have shown symptoms only in the late nineties. The last diagnosed case was in 2005.

Thank God, it is behind us!

Sorry if this has been a morbid and depressing piece. Blame it on the news item which triggered it!

–Meena