Kuru, or also known as Prion disease, is an extremely rare disease of the nervous system that causes rapid deterioration of mental function and loss of coordination. It primarily affects the cerebellum that is responsible for coordination and balance. It is caused by an infectious protein called Prion found in contaminated human brain tissue. Prions are misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They create sponge-like holes in the brain, leading to a class of diseases and symptoms.
THE BIZARRE HISTORY
The word kuru is derived from a term in the Fore Language of Papua New Guinea that means to ‘shake from fear’ and stems from trembling as a conspicuous symptom of the disease. It was first reported in Western medicine in 1957 by Gajdusek and Vincent Zigas.
Above is an image of the galley D.Carleton Gajdusek performed for his last chapter in the Fields Virology.
Kuru became endemic in all villages which it entered and became hyperendemic in the South Fore region. The transmission of this disease began with the practice of Ritualistic Endocannibalism (eating of relatives as part of a mourning ritual). In this ritual, meat, viscera (the internal organs in the main cavities of the body) and the brain were all eaten. Eating an infected brain led to Kuru disease. Women and children were mainly affected as they were the ones that were most involved in these practices. Even though the New Guinea government has discouraged these practices, the last case was recorded in 2005. This is due to its long incubation period. It can be as long as 30 years.
SYMPTOMS AND TREATMENT
Some symptoms:
Difficulty walking
Poor coordination
Difficulty swallowing
Slurred speech
Dementia
Muscle twitching and tremors
Random, compulsive crying or laughing
Behavioural changes
Inability to grasp objects
There are three stages of Kuru. The first stage involves headache and joint pain, which are common symptoms of other disorders. Some loss of bodily control may also be seen. The second stage involves the inability to walk, body tremors, aphasia (loss of ability to speak or communicate) and ataxia (uncoordinated movements). Starvation and malnutrition are set in the third stage. As there is no treatment for this disease, most people die within a year. There is no treatment as prions that cause Kuru cannot be destroyed easily. Brains contaminated with prions remain infectious even when preserved in formaldehyde (industrial disinfectant) for years.
Governments and societies preventing this disease in the mid-20th century by discouraging the social practice of cannibalism has contributed to the rarity of this disease.
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Written by: Svasti Tewari
Edited by: Sakshi Deshpande
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