Kuru

Kuru is a rare and fatal brain disorder that occurred at epidemic levels during the 1950s-60s among the Fore people in the highlands of New Guinea. The disease was the result of the practice of ritualistic cannibalism among the Fore, in which relatives prepared and consumed the tissues (including brain) of deceased family members. Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. Government discouragement of the practice of cannibalism led to a continuing decline in the disease, which has now mostly disappeared. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. Scientists believe that misshapen prion proteins have the ability to change their shape and cause other proteins of the same type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle (also known as mad cow disease), scrapie in sheep and goats, and chronic wasting disease in deer and elk.

Treatment

There were no treatments that could control or cure kuru, other than discouraging the practice of cannibalism. Currently, there are no cures or treatments for any of the other TSE diseases.

Prognosis

Similar to other the TSEs, kuru had a long incubation period; it was years or even decades before an infected person showed symptoms. Because kuru mainly affected the cerebellum, which is responsible for coordination, the usual first symptoms were an unsteady gait, tremors, and slurred speech. (Kuru is the Fore word for shiver.) Unlike most of the other TSEs, dementia was either minimal or absent. Mood changes were often present. Eventually, individuals became unable to stand or eat, and they died in a comatose state from 6 to 12 months after the first appearance of symptoms.

Research

The NINDS funds research to better understand the genetic, molecular, and cellular mechanisms that underlie the TSE diseases. Findings from this research will lead to ways to diagnose, treat, prevent, and ultimately cure these diseases.

Organizations

Creutzfeldt-Jakob Disease (CJD) Foundation Inc.
Non-profit, volunteer foundation that promotes research, education, and awareness of CJD and reaches out to people affected by CJD.

341 W. 38th Street, Suite 501
New York, NY 10018
help@cjdfoundation.org
http://www.cjdfoundation.org
Tel: New York
Fax: 330-668-2474

Centers for Disease Control and Prevention (CDC)

U.S. Department of Health and Human Services
1600 Clifton Road
Atlanta, GA 30333
inquiry@cdc.gov
http://www.cdc.gov
Tel: Atlanta

NIAID Office of Communications and Government Relations

National Institutes of Health, DHHS
5601 Fishers Lane, MSC 9806
Bethesda, MD 20892
http://www.niaid.nih.gov
Tel: Bethesda

CJD Aware!
Non-profit organization established for support, information sharing, and advocacy.

2527 South Carrollton Ave.
New Orleans, LA 70118-3013
cjdaware@iwon.com; info@cjdaware.com
http://www.cjdaware.com
Tel: New Orleans

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NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.

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