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Kuru
Prion disease - kuru
Kuru is a disease of the nervous system.
Causes
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 symptoms to appear after being exposed to the agent that causes disease.
Kuru causes brain and nervous system changes similar to Creutzfeldt-Jakob disease . Similar diseases appear in cows as bovine spongiform encephalopathy (BSE), also called mad cow disease.
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease of movement and mental function.
The main risk factor for kuru is eating human brain tissue, which can contain the infectious particles.
Symptoms
Symptoms of kuru include:
- Arm and leg pain
- Coordination problems that become severe
- Difficulty walking
- Headache
- Swallowing difficulty
- Tremors and muscle jerks
Difficulty swallowing and being unable to feed oneself can lead to malnutrition or starvation.
Malnutrition
Malnutrition is the condition that occurs when your body does not get enough nutrients.
The average incubation period is 10 to 13 years, but incubation periods of 50 years or even longer have been reported.
Exams and Tests
A neurologic exam may show changes in coordination and walking ability.
Treatment
There is no known treatment for kuru.
Outlook (Prognosis)
Death usually occurs within 1 year after the first sign of symptoms.
When to Contact a Medical Professional
See your health care provider if you have any walking, swallowing, or coordination problems. Kuru is extremely rare. Your provider will rule out other nervous system diseases.
References
Bosque PJ, Tyler KL. Prions and prion diseases of the central nervous system (transmissible neurodegenerative diseases). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases, Updated Edition . 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 181.
Geschwind MD. Prion diseases. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice . 7th ed. Philadelphia, PA: Elsevier; 2016:chap 94.
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Central nervous system - illustration
The central nervous system is comprised of the brain and spinal cord. The peripheral nervous system includes all peripheral nerves.
Central nervous system
illustration
Review Date: 11/14/2016
Reviewed By: Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School; Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
