Kuru

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ORPHA:454745OMIM:245300A81.8
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8Treatment centers

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Overview

Kuru is an extremely rare and fatal brain disease that belongs to a group of conditions called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. It is caused by an abnormal form of a protein called a prion, which damages the brain by creating tiny sponge-like holes in brain tissue. Kuru was found almost exclusively among the Fore people of the Eastern Highlands of Papua New Guinea, where it was spread through the practice of ritualistic cannibalism — specifically, the consumption of the brains of deceased relatives during funeral rites. The word 'kuru' comes from the Fore language and means 'to shake' or 'trembling,' which describes one of the disease's hallmark symptoms. The disease typically progresses through three stages. In the first stage, a person develops unsteady walking, tremors, and difficulty with balance. In the second stage, the person can no longer walk without support and develops more severe tremors and involuntary jerking movements. In the final stage, the person becomes bedridden, unable to speak, and loses the ability to swallow. Death usually occurs within 6 to 12 months after symptoms begin, often from pneumonia or infection. There is no cure or effective treatment for kuru. Since the practice of cannibalism was largely stopped in the late 1950s and early 1960s, new cases have become extraordinarily rare. However, because the incubation period can be extremely long — sometimes over 50 years — occasional cases were still reported decades after the practice ended. Management is entirely supportive, focusing on comfort and quality of life.

Key symptoms:

Unsteady walking and difficulty with balanceTremors and shaking, especially of the trunk and limbsInvoluntary jerking movementsDifficulty speaking and slurred speechDifficulty swallowingEmotional instability, including inappropriate laughter or cryingProgressive inability to walkLoss of coordinationMuscle weaknessJoint pain in the early stagesHeadachesBecoming bedridden in late stagesInability to control bodily functions (incontinence)Severe weight loss and malnutritionDementia in the final stages

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Kuru.

View clinical trials →

No actively recruiting trials found for Kuru at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Kuru community →

No specialists are currently listed for Kuru.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Kuru.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Kuru

No recent news articles for Kuru.

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How certain is the diagnosis, and what tests were used to confirm it?,What stage of the disease am I currently in, and what should I expect next?,Are there any clinical trials or experimental treatments available for prion diseases?,What supportive care options are available to help manage my symptoms?,Should my family members be tested for PRNP gene variants?,When should we consider palliative care or hospice services?,What can be done to help with swallowing difficulties and nutrition?

Common questions about Kuru

What is Kuru?

Kuru is an extremely rare and fatal brain disease that belongs to a group of conditions called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. It is caused by an abnormal form of a protein called a prion, which damages the brain by creating tiny sponge-like holes in brain tissue. Kuru was found almost exclusively among the Fore people of the Eastern Highlands of Papua New Guinea, where it was spread through the practice of ritualistic cannibalism — specifically, the consumption of the brains of deceased relatives during funeral rites. The word 'kuru' comes from

How is Kuru inherited?

Kuru follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Kuru typically begin?

Typical onset of Kuru is adult. Age of onset can vary across affected individuals.