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Kuru

Last updated Oct. 23, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Kuru is a fatal, irreversible, and degenerative neurological disorder that is caused by an abnormal protein called prions, which is found in the human brain.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Prion Disease (Kuru)
  • Laughing death

What is Kuru? (Definition/Background Information)

  • Kuru is a fatal, irreversible, and degenerative neurological disorder that is caused by an abnormal protein called prions, which is found in the human brain
  • Kuru was commonly found among the people of an island called Papua New Guinea who practiced cannibalism, a practice where the brain of dead people were eaten by their relatives and family members as part of certain funeral ritual
  • The abnormal prion proteins alter the internal structures of the brain cells, causing abnormal functioning of the brain and nervous system
  • The common signs and symptoms associated with Kuru include coordination problems with difficulty in walking, tremors, emotional liability, headache, muscle jerks,  difficulty in speech and swallowing, and many other features
  • A definitive diagnosis of Kuru can only be made either through a brain biopsy or post-mortem exam of brain
  • Kuru is incurable and presently, there is no known treatment for the condition. The prognosis is poor and death usually occurs within a year of onset of the first symptoms

Who gets Kuru? (Age and Sex Distribution)

  • Any individual who eats human flesh (especially brain) or practices cannibalism is susceptible to Kuru
  • The condition was mainly found among the people of the 4 linguistic groups of Eastern highlands in Papua New Guinea
  • Kuru affects both males and females; however, women are more commonly affected than men. It can also affect children

What are the Risk Factors for Kuru? (Predisposing Factors)

Following are the risk factors associated with Kuru:

  • Eating human brain tissue containing infectious particles is the major risk factor for Kuru. This practice was mostly seen, only amongst the tribes in New Guinea, in the early part of the 20th century. Since cannibalism has been abolished, this condition is rare
  • Women are at a higher risk for this disease because of the prevalent rituals (which were practiced)

What are the Causes of Kuru? (Etiology)

  • Kuru is an incurable, fatal and degenerative neurological disorder that is caused by an abnormal protein called prions, found in human brains. Endocannibalism or eating of human flesh (from the same tribe or community) is the main reason and cause of Kuru
  • Kuru belongs to a class of infectious disease called transmissible spongiform encephalopathies (TSE’s), which are also known as Prion diseases
  • In this condition, abnormal protein molecules clump together and accumulate in the brain tissue. These abnormal proteins alter the functioning of the brain cells, thus causing the clinical manifestations of Kuru

What are the Signs and Symptoms of Kuru?

The signs and symptoms of Kuru can be divided into 3 stages, namely the ambulant stage, sedentary stage, and terminal stage. The signs and symptoms vary among each stage and progressively get worse from the first stage to the final stage.

The signs and symptoms during the ambulant stage (1st stage) may include:

  • Arm and leg pain
  • Severe coordination problems
  • Tremors and muscle jerks (myoclonus), shivering
  • Difficulty in walking (cerebellar ataxia)
  • Unsteadiness in one’s stance, voice, hands, and eyes
  • Difficulty in swallowing
  • Slurred speech
  • Headache
  • Malnutrition

The signs and symptoms during the sedentary stage (2nd stage) may include:

  • The affected individual will not be able to walk without support
  • More severe tremors
  • Ataxia (loss of coordination of the muscles), shock-like muscle jerks
  • Emotional instability, outbursts of laughter without any reason
  • Depression
  • Mental slowness
  • The signs and symptoms during the terminal Stage (3rd stage) may include:
  • The individual will not be able to sit, stand, or move without any support
  • Very severe ataxia
  • Tremors
  • Dysarthria (slurring of speech)
  • Urinary and fecal incontinence
  • Difficulty in swallowing (dysphagia)
  • Deep ulcerations

How is Kuru Diagnosed?

The diagnosis of Kuru may involve:

  • A complete evaluation of the medical history and a thorough physical examination
  • Neurological examination is an important component of the physical exam
  • No laboratory studies are helpful in definitely diagnosing Kuru
  • Normally, a definitive diagnosis can only be made, either through a brain biopsy or during the post-mortem exam of the brain (during an autopsy) 

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Kuru?

The possible complications associated with Kuru include:

  • Individuals become vegetative and bedridden
  • Wound infection/ bed sores
  • Pneumonia (infection of the lung)
  • Malnutrition

How is Kuru Treated?

Currently, there are no known treatment measures available for Kuru. 

How can Kuru be Prevented?

Cannibalism (eating human flesh) as a symbol of respect to the deceased person is the only reason for Kuru. Abolishing this practice is the only way to prevent the condition.

What is the Prognosis of Kuru? (Outcomes/Resolutions)

  • The prognosis for Kuru is poor since 100% of the individuals diagnosed with Kuru end up succumbing to the disease. And hence, the condition may be termed as being uniformly fatal
  • The incubation period (the period after infection to the onset of the first symptom) may take up to 30 years. But then, death mostly occurs within 1 year from the period around which the symptoms first started appearing

Additional and Relevant Useful Information for Kuru:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/health-topics/neurological-institute/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Sept. 14, 2015
Last updated: Oct. 23, 2018