Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Kuru was first identified in the Fore tribe in the highlands of New Guinea in the southwestern Pacific. Kuru was first discovered in the early 1950s but likely started about 50 years prior, at the turn of the 20th century.
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. In 1960, the New Guinea government discouraged the ritual, the practice stopped, and the disease declined.
Kuru caused about 200 deaths per year when 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.
Andrew Brookes/ Getty Images
Kuru Symptoms
Kuru causes the brain tissue to erode to the point at which it develops microscopic holes, making the tissue look like a sponge. It targets thecerebellum, the part of the brain that controls coordination.
“Kuru” is the Fore word for “shiver,” and one of the main symptoms is movement issues. These include:
One of the frightening characteristics of prion disease is the amount of time between exposure and symptoms. People may live for many years or decades before showing signs.
Kuru is aprion 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 DiseasesKuru 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.
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. If misfolded prion protein comes in contact with normally folded prion protein, it can bend them out of shape.
This bending makes them:
Because misfolded proteins cause prion diseases, they’re sometimes called conformational diseases. The theory is that at the root of some of these diseases are misfolded proteins that don’t work right or build up and become toxic. The best-known conformational disease isAlzheimer’s, but it may be implicated in many others.
Kuru occurred at epidemic levels in the 1950s among the Fore people in the highlands of New Guinea, a population with a tradition of eating the brains of their dead.
You can’t get kuru from eating animal brains. But you can acquire animal prion diseases from infected animals. For example, the mad cow outbreak happened when people ate meat contaminated with nervous system tissue from cows with bovine spongiform encephalopathy (BSE), a prion disease.
By 2019, more than 200 people worldwide had acquired a prion disease from eating beef tainted with BSE, and they all have died.
Variant Creutzfeldt-Jakob Disease
Prions are especially dangerous because the normal methods used to kill viruses, bacteria, and other pathogens like heat, cold, soaps, and acids don’t work on prions.
For a long time, experts thought that very strong disinfectants or sustained heat for several hours at extremely high temperatures (900 degrees F and above) was the only way to destroy prions.
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.
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.
Other advanced diagnostic technologies that weren’t around in the 1950s and 1960s may also have been able to detect kuru.Brain magnetic resonance imaging (MRI)can show features suggestive of prion disease, so it could help diagnose a case of kuru should one occur.
Tests for prion disease also typically include an analysis of the fluid in the spinal cord.Cerebrospinal fluidtests include:
Other than these tests, a brain biopsy is the only way to detect a prion disease, but it is generally discouraged. In abiopsy, a sample of tissue is removed and analyzed microscopically in the lab.
There are no treatments to control or cure kuru or any other prion disease. The best treatment for kuru is prevention—discouraging the practice of cannibalism The last case of kuru was in 2009.
Kuru has a long incubation period. It can take years or even decades after infection for a person to develop symptoms. The average incubation period is 10 to 13 years, but incubation periods of 50 years or even longer have been seen.
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
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.
12 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institute of Neurological Disorders and Stroke.Kuru.Whitfield JT, Pako WH, Collinge J, Alpers MP.Cultural factors that affected the spatial and temporal epidemiology of kuru.R Soc Open Sci. 2017;4(1):160789. doi.org10.1098/rsos.160789MedlinePlus.Kuru.Centers for Disease Control and Prevention.Prion diseases.Peng Y, Alexov E.Protein conformational disease: visit the facts at a glance. In eLS, John Wiley & Sons, Ltd (Ed.). 2017.Food and Drug Administration.All about BSE (mad cow disease).Virginia Department of Wildlife Resources.What are Prions? | Virginia DWRHughson AG, Race B, Kraus A, et al.Inactivation of prions and amyloid seeds with hypochlorous acid.PLoS Pathog.2016;12(9):e1005914. doi:10.1371/journal.ppat.1005914School of Medicine, Case Western Reserve University.Brain magnetic resonance imaging (MRI).School of Medicine, Case Western Reserve University.Cerebrospinal fluid diagnostic tests.National Institutes of Health.Therapeutic approaches for prion diseases.Collinge J, Whitfield J, McKintosh E, et al.Kuru in the 21st century–an acquired human prion disease with very long incubation periods.Lancet. 2006;367(9528):2068-2074. doi:10.1016/S0140-6736(06)68930-7 (keep although older as is this a rare disease)
12 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institute of Neurological Disorders and Stroke.Kuru.Whitfield JT, Pako WH, Collinge J, Alpers MP.Cultural factors that affected the spatial and temporal epidemiology of kuru.R Soc Open Sci. 2017;4(1):160789. doi.org10.1098/rsos.160789MedlinePlus.Kuru.Centers for Disease Control and Prevention.Prion diseases.Peng Y, Alexov E.Protein conformational disease: visit the facts at a glance. In eLS, John Wiley & Sons, Ltd (Ed.). 2017.Food and Drug Administration.All about BSE (mad cow disease).Virginia Department of Wildlife Resources.What are Prions? | Virginia DWRHughson AG, Race B, Kraus A, et al.Inactivation of prions and amyloid seeds with hypochlorous acid.PLoS Pathog.2016;12(9):e1005914. doi:10.1371/journal.ppat.1005914School of Medicine, Case Western Reserve University.Brain magnetic resonance imaging (MRI).School of Medicine, Case Western Reserve University.Cerebrospinal fluid diagnostic tests.National Institutes of Health.Therapeutic approaches for prion diseases.Collinge J, Whitfield J, McKintosh E, et al.Kuru in the 21st century–an acquired human prion disease with very long incubation periods.Lancet. 2006;367(9528):2068-2074. doi:10.1016/S0140-6736(06)68930-7 (keep although older as is this a rare disease)
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Institute of Neurological Disorders and Stroke.Kuru.Whitfield JT, Pako WH, Collinge J, Alpers MP.Cultural factors that affected the spatial and temporal epidemiology of kuru.R Soc Open Sci. 2017;4(1):160789. doi.org10.1098/rsos.160789MedlinePlus.Kuru.Centers for Disease Control and Prevention.Prion diseases.Peng Y, Alexov E.Protein conformational disease: visit the facts at a glance. In eLS, John Wiley & Sons, Ltd (Ed.). 2017.Food and Drug Administration.All about BSE (mad cow disease).Virginia Department of Wildlife Resources.What are Prions? | Virginia DWRHughson AG, Race B, Kraus A, et al.Inactivation of prions and amyloid seeds with hypochlorous acid.PLoS Pathog.2016;12(9):e1005914. doi:10.1371/journal.ppat.1005914School of Medicine, Case Western Reserve University.Brain magnetic resonance imaging (MRI).School of Medicine, Case Western Reserve University.Cerebrospinal fluid diagnostic tests.National Institutes of Health.Therapeutic approaches for prion diseases.Collinge J, Whitfield J, McKintosh E, et al.Kuru in the 21st century–an acquired human prion disease with very long incubation periods.Lancet. 2006;367(9528):2068-2074. doi:10.1016/S0140-6736(06)68930-7 (keep although older as is this a rare disease)
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Virginia Department of Wildlife Resources.What are Prions? | Virginia DWR
Hughson AG, Race B, Kraus A, et al.Inactivation of prions and amyloid seeds with hypochlorous acid.PLoS Pathog.2016;12(9):e1005914. doi:10.1371/journal.ppat.1005914
School of Medicine, Case Western Reserve University.Brain magnetic resonance imaging (MRI).
School of Medicine, Case Western Reserve University.Cerebrospinal fluid diagnostic tests.
National Institutes of Health.Therapeutic approaches for prion diseases.
Collinge J, Whitfield J, McKintosh E, et al.Kuru in the 21st century–an acquired human prion disease with very long incubation periods.Lancet. 2006;367(9528):2068-2074. doi:10.1016/S0140-6736(06)68930-7 (keep although older as is this a rare disease)
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