What is kuru?



Kuru, also known as mad human disease and laughing disease is a rare and fatal neurodegenerative disease that affects humans. It occurred as an epidemic in the 1950s-60s among the Fore people of Papua New Guinea. It is similar to bovine spongiform encephalopathy, more commonly known as mad cow disease. Kuru is transmitted by an infectious agent called a prion, a small protein that lacks DNA and is more resistant to being broken down. Because the prions do not break down as easy, they multiply and concentrate in nerve cells, thus damaging them. As the nerves are damaged, brain degeneration occurs, a process which can take up to two years to kill its victim, but usually occurs within one year.
Kuru infected brain showing the characteristic holes.
Kuru infected brain showing the characteristic holes.

Aside from being classified as a prion disease, kuru is also classified as a transmissible spongiform encephalopathy (TSE). This is so because as the disease continues to affect its victim, the victim’s brain becomes filled with holes. Other TSEs that fall into this category are Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.



What causes kuru?



Kuru is caused by the cannibalistic practices of the Fore people of Papua New Guinea. As part of their mourning rituals, the Fore people eat the brains of the deceased. The infectious agent, or prion, is found in contaminated brain tissue.

What are symptoms of kuru?



Symptoms of kuru include trembling or shivering, the meaning of kuru in the Fore language. Other symptoms include joint pain, varying emotions of sadness to happiness with sudden outbursts of laughter, and headaches which are later followed by loss of coordination, difficulty walking, difficulty swallowing, tremors, and dementia. However, sometimes the symptoms can take up to 30 years or longer to develop.

Who gets kuru?



Kuru patients of the Fore people in Papua New Guinea.
Kuru patients of the Fore people in Papua New Guinea.
The Fore people of Papua New Guinea are the most susceptible to kuru. Also, the disease mainly afflicts women and children because they live apart from men and are the only ones who carry out the mourning rituals.

How is kuru diagnosed?



Kuru can be diagnosed by a neurological exam that shows changes in coordination and walking ability.

How is kuru treated?



Currently, there is no treatment for kuru. There are also no methods to control it, other than the discouragement of the practice of cannibalism. Today, because the Fore people have stopped their cannibalistic mourning rituals, the spread of kuru has stopped. However, a few cases still develop each year due to kuru’s long incubation period.

Impact


At the time kuru was prevalent, it was considered an epidemic. Because kuru was restricted to the highlands of New Guinea, it did not have an impact on people in the rest of the world, other than the scientists studying it. As time progressed and the government interfered, the mourning rituals of the Fore people were abandoned in order to stop the epidemic. Today, kuru is not a problem because only a few cases develop each year.
external image kuru.gif


Current Research



Current research that is being done involves the genetic, molecular, and cellular mechanisms of the category of TSE diseases. The goal of this research is to find ways to diagnose, treat, prevent, and cure these diseases.

Video


A video of a Kuru patient of the Fore people.
http://hotmedicalnews.com/human_kuru_video.php