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Kuru - Causes, Symptoms, Diagnosis, and Treatment

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Kuru is a disease of the nervous system caused by an infectious protein seen in the contaminated tissue of the human brain among the people of New Guinea.

Medically reviewed by

Dr. Abhishek Juneja

Published At December 21, 2022
Reviewed AtJanuary 25, 2023

Introduction:

Kuru is an incurable neurodegenerative disease (a type of disorder in which the cells of the nervous system do not function or get damaged permanently) in nature. This disease was first discovered by Dr. D. Carlton. It was considered to be one of the most significant discoveries of the 20th century in the field of medicine. It occurred during the period 1950 to 1960 among the Fore tribal people of New Guinea. The disease got its name “kuru” from the word “Kuria” or “Guria” (the local language of the Fore people of New Guinea). The meaning of the word “kuru” is to shake or tremble. The changes observed in the brain and nervous system of the people affected with kuru are similar to Creutzfeldt-Jakob disease (a disorder of the brain which causes memory loss and leads to death).

What Is Kuru?

Kuru is a dangerous disorder of the brain that is caused as a result of an infectious protein called prion found in the human brain tissues. It affects the cerebellum (the part which is in charge of coordination and balance) of the brain. It is even known by the name laughing sickness, as the patient experiences uncontrollable episodes of laughing. This was discovered in tribal people of the New Guinea region who practiced cannibalism (the ritual of one human being eating the flesh of another human). This disease was transmitted as the tribal people of that region ate the brains of dead people as a funeral ritual. It is a commonly contractible disease and can spread through contact with open wounds of an infected person. Similar diseases are seen in cows as bovine spongiform encephalopathy (incurable deadly disease of the nervous system seen in cattle) and scrapie (deadly disease affecting the nervous system of goats and sheep) in goats.

What Are the Causes of Kuru?

It was found in tribal people of New Guinea, who acquired it by eating the brains of dead people, which was considered to be a part of their funeral ritual. The Fore (tribal) people ate the body parts of their relatives after death. According to them, this custom was practiced to incorporate the body of the dead person into the body of the living relatives. By doing so, the tribal people believed that it would help to free the spirit of the dead person. In this ritual, women and children consume the brain, whereas men eat the muscles. Since the brain was the organ that contained the infectious protein, women and children were more affected by this disorder than men. The infective protein called a prion is responsible for causing this disease. Prions are not living organisms. They are proteins that multiply inside the brain and form clumps disturbing the normal processes of the brain.

What Are the Symptoms of Kuru?

  • Loss of coordination (potential of an individual to use all the body parts to function efficiently).

  • Difficulty walking.

  • Trouble in holding things.

  • Shivering, which becomes uncontrollable.

  • Sudden episodes of laughing or crying.

  • The patient experiences difficulty swallowing.

  • Severe pain in the arms and legs.

  • Dementia (a condition in which a person’s memory and ability to think are affected, which in turn creates problems in day-to-day activities).

  • Sudden mood changes.

  • Limbs (arms and legs) become stiff.

  • Difficulty in feeding oneself leads to malnutrition and starvation.

  • Confused and unclear speech.

What Are the Stages of Kuru?

This disease has three stages:

  • First Stage (Ambulant): In this stage, the person experiences a loss of control of his body movements. The patient feels difficulty in standing and sitting.

  • Second Stage (Sedentary): In this stage, the person is almost unable to walk. Severe involuntary (action done without one’s own will) jerks occur throughout the body.

  • Third Stage (Terminal): The patient becomes completely bedridden. Most people lose their ability to speak. They show signs of memory loss, and their thinking ability gets affected. The difficulty in swallowing leads to poor eating habits. As a result, malnutrition develops. In the later stages, people may get infected with pneumonia and can go into a coma. In most cases, people die within one or two years.

How to Diagnose Kuru?

This test cannot be diagnosed by any particular test. However, certain examinations can help in finding out this disease. They are:

  • The doctor takes a detailed medical history of the patient to know about the symptoms.

  • Blood tests check the levels of thyroid hormone and folic acid levels.

  • Kidney function and liver tests (to eliminate the chance of other diseases which show the same symptoms).

  • Electroencephalogram (EEG): This is done to check the electrical activity inside the brain.

  • Magnetic Resonance Imaging (MRI): This can be done to check for any abnormalities in the brain. However, MRI does not provide any help in the definitive diagnosis of the disease.

What Are the Treatment Options for Kuru?

There are no known successful treatment options for kuru. The treatment focuses on relieving the symptoms. As it is a disease that was caused due to the consumption of the brains of dead people as a funeral ritual, the only way to prevent this disease was to stop this practice. Since this deadly disease started taking away the lives of people, the government and authorities took strict measures to stop the practice of cannibalism. The incubation period (the time span between the first infection and the appearance of symptoms) of this disease is between ten to fifty years. Due to this reason, cases have been reported even after cannibalism was stopped. However, according to the national institute of neurological disorders and stroke (NINDS), this disease has been wiped out completely.

Conclusion:

Although it is a deadly disease, it vanished as soon as the practice of cannibalism was put to an end. The discovery of kuru led Dr. D. Carlton Gajdusek (the person who first discovered this disease) to share the Nobel prize in medicine for “discoveries concerning new mechanisms for the origin and dissemination of infectious diseases.” The discovery of this disorder opened many new pathways in the field of human medicine and played an important role in finding out the ways of transmission of similar diseases from animals to human beings.

Frequently Asked Questions

1.

How Does Kuru Affect a Person?

Kuru disease mainly affects the cerebellum of the brain, which helps in the coordination of the body. The initial symptoms include unsteady gait, tremors, and slurred speech. Mood changes can also be seen, and dementia can either be minimal or absent. The symptoms are usually the same as Creutzfeldt-Jakob's disease.

2.

How Long Does Kuru Last?

Kuru disease is a rare and fatal condition of the brain. This disease was first found in the 1950s to 1960s in the highlands of New Guinea. It is an incurable disease in which the cells of the nervous system stop functioning or may get damaged permanently.

3.

What Are the Stages of Kuru?

Kuru is divided into three clinical stages - 
- Ambulant - This is the first stage in which the person loses control of body movements, and the patient may feel difficulty standing and sitting.
- Sedentary - In this stage, the person cannot walk and has unwanted jerks.
- Terminal - This is the third and last stage in which the patient is entirely bedridden; some people may not be able to speak and shows sign of memory loss, and their thinking may also get affected.

4.

Does Kuru Cause Pain?

Kuru disease is related to the brain and nervous system. This usually causes headaches and joint pain. As these symptoms are common, they can be easily missed as the patient has no idea a severe condition is underway. In addition to that, the patient may also experience hysterical laughter and personality-driven joys of cannibalism.

5.

Who Is Most Likely to Get Kuru?

Kuru is most commonly seen in women and children as they have more chances of getting brain infections. In addition, a person can get this infection by coming in contact with the open wound or sores of an infected person and by eating an infected person's brain. This cannibalism is most common in the Fore people of New Guinea, as they eat the brains of their dead relatives during funeral rituals.

6.

How to Diagnose Kuru?

There is no specific test present to diagnose Kuru. However, postmortem pathologic evaluation of central nervous tissues can help identify this condition. The presence of cerebrospinal fluid 14-3-3 protein, neuronal specific enolase (NSE), and S-100 may help diagnose this condition. Also, healthcare professionals may order blood tests to check thyroid hormone and folic acid levels.

7.

How Many People Got Kuru?

Kuru is an extremely rare condition. It is usually found in the Fore people of New Guinea as they consume the human brain. It was discovered in the 1960s, and the incubation period of this condition may last for 10 to 50 years. After five decades, this epidemic witnessed a significant decline in mortality rates, plummeting from 200 deaths per year in 1957 to no reported deaths since 2010. The most recent documented death related to this condition was reported in 2009.

8.

Can Anyone Survive Kuru?

There is no known cure for kuru, and usually, within one year of contraction, the patient dies. However, according to some researchers, it has been found that people who survive kuru have a genetic mutation called V127, and those without this mutation can get infected with this condition.

9.

What Is the Treatment for Creutzfeldt-Jakob Disease (CJD)?

Creutzfeldt-Jakob disease (CJD) is also known as mad cow disease. This condition usually has psychological symptoms such as anxiety and depression. These symptoms can be managed by taking medications such as sedatives and antidepressants, and patients with muscle jerks or tremors can be treated with medicines like Clonazepam and Sodium Valproate. Moreover, for pain, opiate-based painkillers can be taken.

10.

Why Does Kuru Cause Uncontrollable Laughter?

Kuru means trembling and is also known as laughing sickness, as this condition causes pathologic bursts of laughter, which is one of the common symptoms of this condition. However, laughter is not subjective and is the personality-driven reaction of the affected person to the joy of cannibalism and is usually an involuntary neurodegenerative reaction.

11.

Is Kuru a Genetic Condition?

Kuru usually transmits from any highly infectious individual with this condition and can get transmitted either through eating (cannibalism) or through direct contact with open sores or wounds. However, a genetic theory is based on family or population examinations that show that a single autosomal gene might control kuru.
Dr. Abhishek Juneja
Dr. Abhishek Juneja

Neurology

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