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All about prion diseases Creutzfeldt-Jakob disease types of Creutzfeldt-Jakob disease variant Creutzfeldt-Jakob disease (vCJD) causes of Creutzfeldt-Jakob disease symptoms of Creutzfeldt-Jakob disease diagnosis of Creutzfeldt-Jakob disease treatment for Creutzfeldt-Jakob disease prevention of Creutzfeldt-Jakob disease fatal familial insomnia (FFI) Kuru

What is Kuru?

Kuru is a degenerative nerve disease caused by a prion (infectious protein) transmitted to humans via contaminated human brain tissue. Kuru was first noted in New Guinea in the early 1900s. By the 1950s, anthropologists and Australian government officials reported that kuru ("trembling" in the language of the Fore) was rampant among the South Fore, a

single census division of approximately 8,000 individuals within the Okapa subdistrict. This particular group partook in ritual acts of mortuary cannibalism, which behaviour was later determined to be responsible for the epidemic transmission of the disease.

Kuru is an extremely rare prion disease. It is almost exclusively found among people from New Guinea, who practiced a form of cannibalism in which the brains of dead relatives were eaten as part of a funeral ritual. Kuru causes neurodegenerative changes similar to another prion disease, Creutzfeldt-Jakob disease, which occurs sporadically around the world and for which risk factors are unknown.

In addition, similar prion diseases appear in sheep as scrapie, mink as encephalopathy, and in cows as bovine spongiform encephalopathy (BSE) ("mad cow disease"). The main risk factor for the development of kuru is eating human brain tissue, which can contain the infectious particles.

Kuru may begin with difficulty walking and increasing incoordination, which eventually become severe. Tremors and muscle jerks are often seen in the later stages of the disease. Difficulty swallowing and inability to feed oneself lead to malnutrition or starvation.

The incubation period (the time it takes until symptoms develop) for this disease can be up to 30 years or longer. Death, however, usually occurs within a year after the onset of symptoms.

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.

More information on prion diseases (Creutzfeldt-Jakob disease, fatal familial insomnia, kuru)

What're prion diseases? - Prion diseases (transmissible spongiform encephalopathies) are a group of inherited and spontaneous neurodegenerative disorders affecting animals and humans.
What is Creutzfeldt-Jakob disease? - Creutzfeldt-Jakob disease is degenerative brain disorder. Creutzfeldt-Jakob disease (CJD) is a rapidly progressive disease causing damage to the brain.
What types of Creutzfeldt-Jakob disease are there? - There are three types of Creutzfeldt-Jakob Disease: sporadic (or classical), hereditary (or familial) and acquired (or iatrogenic).
What's variant Creutzfeldt-Jakob disease (vCJD)? - Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition. vCJD affects younger patients.
What causes Creutzfeldt-Jakob disease? - There are three main causes for classic CJD. Creutzfeldt-Jakob disease through infection is transmitted by prions.
What're the symptoms of Creutzfeldt-Jakob disease? - Symptoms of Creutzfeldt-Jakob disease include forgetfulness and nervousness, jerky, trembling hand movements, unsteady gait, myoclonus.
How is Creutzfeldt-Jakob disease diagnosed? - Creutzfeldt-Jakob disease is diagnosed by a clinical neurological exam and electroencephalography (EEG).
What's the treatment for Creutzfeldt-Jakob disease? - There is no cure for Creutzfeldt-Jakob disease, and no treatment which slows the progression of the disease.
How to prevent Creutzfeldt-Jakob disease? - Creutzfeldt-Jakob disease is prevented by handling their fluids and tissues with extreme caution and by using special sterilization methods to disinfect equipment.
What is fatal familial insomnia? - Fatal familial insomnia is caused by prions, similar to Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.
What is Kuru? - Kuru is a degenerative nerve disease caused by a prion (infectious protein) transmitted to humans via contaminated human brain tissue.
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