Laughing Death Syndrome

Kuru is a very rare disease. It is caused by an infectious protein (prion) found in contaminated human brain tissue. Kuru is found among people from New Guinea who practiced a form of cannibalism in which they ate the brains of dead people as part of a funeral ritual. This practice stopped in 1960, but cases of kuru were reported for many years afterward because the disease has a long incubation period. The incubation period is the time it takes for laughing death disease syndrome to appear after being exposed to the agent that causes disease.

The name kuru means “to shiver” or “trembling in fear.” The symptoms of the disease include muscle twitching and loss of coordination. Other laughing death disease syndrome include difficulty walking, involuntary movements, behavioral and mood changes, dementia, and difficulty eating. The latter can cause malnutrition. Kuru has no known cure. It’s usually fatal within one year of contraction.

The identification and study of kuru helped along scientific research in a number of ways. It was the first neurodegenerative disease resulting from an infectious agent. It led to the creation of a new class of diseases including Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. Today the study of kuru still impacts research on neurodegenerative diseases.

Kuru Laughing Death Disease Syndrome?

Syndrome of more common neurological disorders such as Parkinson’s disease or stroke may resemble kuru syndrome. These include:
  • difficulty walking
  • poor coordination
  • difficulty swallowing
  • slurred speech
  • moodiness and behavioral changes
  • dementia
  • muscle twitching and tremors
  • inability to grasp objects
  • random, compulsive laughing or crying

Kuru occurs in three stages. It’s usually preceded by headache and joint pain. Since these are common syndrome, they are often missed as clues that a more serious disease is underway. In the first stage, a person with kuru exhibits some loss of bodily control. They may have difficulty balancing and maintaining posture. In the second stage, or sedentary stage, the person is unable to walk. Body tremors and significant involuntary jerks and movements begin to occur. In the third stage, the person is usually bedridden and incontinent. They lose the ability to speak. They may also exhibit dementia or behavior changes, causing them to seem unconcerned about their health. Starvation and malnutrition usually set in at the third stage, due to the difficulty of eating and swallowing. These secondary symptoms can lead to death within a year. Most people end up dying from pneumonia.

What are the causes of Kuru?

Kuru belongs to a class of diseases called transmissible spongiform encephalopathies (TSEs), also called prion diseases. It primarily affects the cerebellum — the part of your brain responsible for coordination and balance.

Unlike most infections or infectious agents, kuru is not caused by a bacteria, virus, or fungus. Infectious, abnormal proteins known as prions cause kuru. Prions are not living organisms and do not reproduce. They are inanimate, misshapen proteins that multiply in the brain and form clumps, hindering normal brain processes.

Creutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia are other degenerative diseases caused by prions. These spongiform diseases, as well as kuru, create sponge-like holes in your brain and are fatal.

You can contract the disease by eating an infected brain or coming into contact with open wounds or sores of someone infected with it. Kuru developed primarily in the Fore people of New Guinea when they ate the brains of dead relatives during funeral rites. Women and children were mainly infected because they were the primary participants in these rites.

The New Guinea government has discouraged the practice of cannibalism. Cases still appear, given the disease’s long incubation period, but they are rare.

Transmission

In 1961, Australian Michael Alpers conducted extensive field studies among the Fore accompanied by anthropologist Shirley Lindenbaum. Their historical research suggested the epidemic may have originated around 1900 from a single individual who lived on the edge of Fore territory and who is thought to have spontaneously developed some form of Creutzfeldt–Jakob disease. Alpers and Lindenbaum’s research conclusively demonstrated that kuru spread easily and rapidly in the Fore people due to their endocannibalistic funeral practices, in which relatives consumed the bodies of the deceased to return the “life force” of the deceased to the hamlet, a Fore societal subunit. Corpses of family members were often buried for days, then exhumed once the corpses were infested with maggots, at which point the corpse would be dismembered and served with the maggots as a side dish.

The demographic distribution evident in the infection rates – kuru was eight to nine times more prevalent in women and children than in men at its peak – is because Fore men considered consuming human flesh to weaken them in times of conflict or battle, while the women and children were more likely to eat the bodies of the deceased, including the brain, where the prion particles were particularly concentrated. Also, the strong possibility exists that it was passed on to women and children more easily because they took on the task of cleaning relatives after death and may have had open sores and cuts on their hands.

Although ingestion of the prion particles can lead to the disease, a high degree of transmission occurred if the prion particles could reach the subcutaneous tissue. With elimination of cannibalism because of Australian colonial law enforcement and the local Christian missionaries’ efforts, Alpers’ research showed that kuru was already declining among the Fore by the mid‑1960s. However, the mean incubation period of the disease is 14 years, and 7 cases were reported with latencies of 40 years or more for those who were most genetically resilient, continuing to appear for several more decades. Sources disagree on whether the last sufferer died in 2005 or 2009.

How Laughing Death Syndrome Diagnosed?

Neurological exam

Your doctor will perform a neurological exam to diagnose kuru. This is a comprehensive medical exam including:

  • medical history
  • neurological function
  • blood tests, such as thyroid, folic acid level, and liver and kidney function tests (to rule out other causes for symptoms).

Electrodiagnostic tests

Tests such as electroencephalogram (EEG) are used to examine the electrical activity in your brain. Brain scans such as an MRI may be performed, but they may not be helpful in making a definitive diagnosis.

What are the treatments for kuru?

There is no known successful treatment for kuru. Prions that cause kuru can’t be easily destroyed. Brains contaminated with prions remain infectious even when preserved in formaldehyde for years.

What is the outlook for kuru?

People with kuru require assistance to stand and move and eventually lose the ability to swallow and eat because of symptoms. As there is no cure for it, people infected with it may lapse into a coma within six to 12 months after experiencing initial symptoms. The disease is fatal and it’s best to prevent it by avoiding exposure.

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