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NINDS Kuru Information Page: NINDS
Article title: NINDS Kuru Information Page: NINDS
Main condition: Kuru
Conditions: Kuru, TSE
What is Kuru?
Kuru is a rare, degenerative, and fatal brain disorder that occurs primarily among the Fore natives in the highlands of New Guinea. The disease is thought to result from the practice of ritualistic cannibalism, in which relatives consume the tissues (including brain) of a deceased family member. Brain tissue from individuals with kuru is highly infectious, and can transmit the disease through either oral or peripheral routes of infection (e.g., cuts, sores). Cannibalism was prevalent before the 1960s, but government discouragement of the practice led to a continuing decline in the disease, which has now essentially disappeared. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. Other TSEs include Creutzfeldt-Jakob disease in humans, 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. “Prion” is the name given to an abnormal form of protein that appears to be able to cause more and more of its normal form to become malformed, eventually leading to deposits in cells of the central nervous system, and eventually killing these cells. Prion diseases are most easily transmitted by direct transfer of infected tissue to the CNS, although (like kuru) they can also be transmitted orally.
Is there any
treatment?
There are no treatments that can control or cure the
disease,
nor is there currently a diagnostic test for kuru or other TSEs.
The only way to confirm a diagnosis of a TSE is through a brain biopsy or
autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue
from the patient’s brain for examination.
Usually, this is done only to
rule out other, potentially treatable, diseases, like encephalitis or
meningitis that might mimic the symptoms of a TSE disease.
What is the prognosis?
Like
other TSEs, kuru has a long incubation period and it may take years or
even decades before an infected person shows symptoms. Because kuru mainly
affects the cerebellum, which is located at the base of the brain and is
responsible for coordination, the usual first symptoms are those of
incoordination, such as unsteady gait, tremors, and slurred speech. Unlike
most of the other TSEs, dementia is either minimal or absent; instead,
there is usually a marked change in mood, often taking the form of
indifference, or even euphoria. Eventually, patients become unable to
stand or eat, and they die in a helpless comatose state from 6 to 12
months after the onset of illness.
What research is being
done?
The NINDS funds research grants to institutions throughout
the country in an effort to learn how to better understand the genetic,
molecular, and cellular mechanisms that underlie kuru and other TSE
diseases.
Creutzfeldt-Jakob (CJD) Foundation Inc.
P.O. Box 611625
No. Miami, FL 33261-1625
crjakob@aol.com
http://cjdfoundation.org/
Tel:
954-704-0519 305-891-7579
Fax: 305-893-9050/954-436-7591 305-893-9050
Centers for Disease Control and Prevention (CDCP)
1600
Clifton Road, N.E.
Atlanta, GA 30333
inquiry@cdc.gov
http://www.cdc.gov/
Tel: 800-311-3435
National Institute of Allergy and Infectious Diseases
(NIAID)
National Institutes of Health
31 Center Drive, Rm. 7A50
MSC 2520
Bethesda, MD 20892-2520
(see website)
http://www.niaid.nih.gov/
Tel:
301-496-5717
Related NINDS Publications and Information
Creutzfeldt-Jakob
Disease (CJD) information sheet compiled by the National Institute of
Neurological Disorders and Stroke (NINDS).
Creutzfeldt-Jakob Disease (CJD) fact sheet
compiled by the National Institute of Neurological Disorders and Stroke
(NINDS).
This fact sheet is in the public domain. You may copy it.Provided
by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD
20892
» Next page: NINDS Landau-Kleffner Syndrome Information Page: NINDS
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