Kuru was first identified in the Fore tribe in the highlands of New Guinea in the southwestern Pacific.
People in this population had a ritual of eating the tissues of their dead, including the brains.
Some of those who ate the brains of people who died from kuru got the illness themselves.
Andrew Brookes/ Getty Images
In 1960, the New Guinea government discouraged the ritual, the practice stopped, and the disease declined.
Kuru caused about 200 deaths per year when it was first discovered.
Cases of kuru were reported for many years afterward because the disease has a long incubation period.
This number dropped sharply until there were few or no deaths each year.
The last known person with kuru died in 2009.
Estimates suggest there are around 2,700 recorded cases of people dying of kuru.The condition has since disappeared.
This article will review the history of kuru, its symptoms, and its prognosis.
It targets thecerebellum, the part of the brain that controls coordination.
People may live for many years or decades before showing signs.
Causes
Kuru is a prion disease.
It is also called transmissible spongiform encephalopathy (TSE).
Prion protein is called PrP and coded in the gene PRNP.
Its function is unknown, but it is present in tissues all over the body.
Other Prion Diseases
Kuru was the first prion disease discovered.
Since then, five types of prion diseases have been found in humans.
The other four areCreutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, andfatal familial insomnia.
Prions are contagious misfolded proteins.
This bending makes them:
Because misfolded proteins cause prion diseases, they’re sometimes called conformational diseases.
The best-known conformational disease isAlzheimer’s, but it may be implicated in many others.
It’s likely that one person spontaneously developed the genetic prion disease Creutzfeldt-Jakob.
you’re able to’t get kuru from eating animal brains.
But you’re free to acquire animal prion diseases from infected animals.
Variant Creutzfeldt-Jakob Disease
Variant Creutzfeldt-Jakob is ahuman disease resulting from BSE (mad cow disease).
But in the last few years, researchers have found that a nontoxic hypochlorous acid solution can disinfect prions.
This is great news for hospitals that have had just to throw away prion-contaminated surgical tools in the past.
Diagnosis
Kuru has never been diagnosed directly.
New tests may be able to detect cases of kuru if there is ever another outbreak.
Traditionally, healthcare providers usedneurological examsto detect changes in coordination and show deteriorating walking and other abilities.
In abiopsy, a sample of tissue is removed and analyzed microscopically in the lab.
Treatment
There are no treatments to control or cure kuru or any other prion disease.
The best treatment for kuru is preventiondiscouraging the practice of cannibalism The last case of kuru was in 2009.
Prognosis
Kuru has a long incubation period.
It can take years or even decades after infection for a person to develop symptoms.
As their cerebellum is destroyed, people with kuru lose their ability to stand or eat.
They die in a coma about six to 12 months after the first symptoms.
Summary
Kuru is an extremely rare disease.
It’s resulting from a contagious protein found in the brain tissue of infected humans.
The only way to contract the disease is by ingesting or having contact with contaminated brain tissue.
Researchers first found the disease in a New Guinea tribe that had a ritual of eating their dead.
The number of cases sharply decreased when the government discouraged this practice in 1960.
The last known kuru death occurred in 2009.
The disease causes the brain tissue in the cerebellum to erode.
It results in unsteady movement, tremors, slurred speech, and mood changes.
Kuru is a prion disease, also known as transmissible spongiform encephalopathy.
It is due to contagious misfolded proteins.
These proteins clump together, fold other prion proteins out of shape, and are very hard to destroy.
There are five known prion diseases in humans, with kuru being the first identified.
The best-known animal prion disease is BSE, or mad cow disease.
Humans contract variant Creutzfeldt-Jakob disease by eating contaminated meat from cows with BSE.
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