Kuru: Difference between revisions
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c. Terminal stage | c. Terminal stage | ||
During the progression of disease mobility decreases significantly. An infected person usually dies within 3 months to 2 years after the first symptoms, often because of pneumonia or pressure sores infection. | During the progression of disease mobility decreases significantly. An infected person usually dies within 3 months to 2 years after the first symptoms, often because of [[pneumonia]] or pressure [[sores infection]]. | ||
==Diagnosis== | ==Diagnosis== | ||
Revision as of 05:00, 7 August 2012
| Kuru | |
| ICD-10 | A81.8 |
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| ICD-9 | 046.0 |
| DiseasesDB | 31861 |
| MedlinePlus | 001379 |
| MeSH | D007729 |
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WikiDoc Resources for Kuru |
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Ongoing Trials on Kuru at Clinical Trials.gov Clinical Trials on Kuru at Google
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US National Guidelines Clearinghouse on Kuru
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Directions to Hospitals Treating Kuru Risk calculators and risk factors for Kuru
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Continuing Medical Education (CME) |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Laughing sickness
Overview
Kuru is an incurable degenerative neurological disorder (brain disease) that is a type of transmissible spongiform encephalopathy found in humans. It causes physiological as well as neurological effects that ultimately lead to death. It was endemic among the Fore tribe of Papua New Guinea and was confined to the Fore population and those nearby populations with whom they intermarried.
Historical Perspective
The word kuru is taken from the Fore word "kuria/guria", 'to shake'. It is also known as the laughing sickness due to the pathologic bursts of laughter people would display when afflicted with the disease.
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. [1] 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.
The disease was researched by Daniel Carleton Gajdusek who was awarded (along with Baruch S. Blumberg) the Nobel Prize in Physiology or Medicine in 1976 for his showing that kuru was transmissible to chimpanzees. This was the first time that this group of encephalopathies had been demonstrated to be infectious and therefore a major step forwards in their investigation. As kuru is the only epidemic of human prion disease in known human history, it has provided important insights into the variant CJD.
Pathophysiology
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.
Epidemiology and Demographics
It was endemic in the Fore tribe of New Guinea.
Gender
It was 8-9 times more prevalent in women and children than men.
Natural History, Complications and Prognosis
Kuru is a latent disease which manifests after an incubation period of 5 - 20years. This phase in asymptomatic phase of incubation period. The disease progress in three stages.
a. Ambulent stage
b. Sedentary stage
c. Terminal stage
During the progression of disease mobility decreases significantly. An infected person usually dies within 3 months to 2 years after the first symptoms, often because of pneumonia or pressure sores infection.
Diagnosis
Symptoms
- Headache
- Joint pains
- Shaking of limbs
Physical Examiation
Neurologic
- Ataxia
- Tremors
- Unsteady gait
- Decreased muscle control
- Deterioration of speech
- Dysarthria (slurred speech)
- Incapable of walking without support
- Emotionally unstable
- Depression
Other
- Uncontrolled sporadic laughter
- Ulcerations (sores with pus and necrosis)
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.
References
bg:Куру (болест) cs:Kuru de:Kuru (Krankheit) eo:Kuruo ko:쿠루병 hr:Kuru it:Kuru (malattia) he:מחלת קורו lt:Kuru (liga) nl:Kuru (ziekte) no:Kuru simple:Kuru (disease) sr:Куру fi:Kuru (tauti) sv:Kuru (sjukdom)