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Kuru (disease)

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Not to be confused with Koro (medicine).

Fore child with advanced kuru.

Kuru is a very rare, incurable neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru is caused by the transmission of abnormally folded prion proteins, which leads to symptoms such as tremors, loss of coordination, and neurodegeneration.

The term kuru derives from the Fore word kuria or guria ("to shake"),[1] due to the body tremors that are a classic symptom of the disease and kúru itself means "trembling".[2] It is also known as the "laughing sickness" due to the pathologic bursts of laughter which are a symptom of the disease. It is now widely accepted that kuru was transmitted among members of the Fore tribe of Papua New Guinea via funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead.[3] Women and children usually consumed the brain, the organ in which infectious prions were most concentrated, thus allowing for transmission of kuru. The disease was therefore more prevalent among women and children.

The epidemic likely started when a villager developed sporadic Creutzfeldt–Jakob disease and died. When the women ate the brain, they caught the disease, and then the people that ate them caught it, and on it went.[4]

While the Fore people stopped eating human meat in the early 1960s, when it was first speculated to be transmitted via endocannibalism, the disease lingered due to kuru's long incubation period of anywhere from 10 to over 50 years.[5] The epidemic declined sharply after discarding cannibalism, from 200 deaths per year in 1957 to 1 or no deaths annually in 2005, with sources disagreeing on whether the last known kuru victim died in 2005 or 2009.[6][7][8][9]

>>1686751

working

>>1686768

There is an ill defined prodromal period (kuru laik i-kamp nau –i.e., “kuru is about to begin”) characterized by the presence of headache and limb pains, frequently in the joints; knees and ankles came first, followed by elbows and wrists; sometimes, interphalangeal joints were first affected; abdominal pains and loss of weight. This period lasted approximately a few months. Fever and other signs of infectious disease are never seen but the patient’s general feeling was reported as reminiscent of that accompanying acute respiratory infection. Some patients even said that they expected a cough to come and when it did not, they started to fear incoming kuru.

>>1686782

>>1686767

back brace

The prodromal period is followed by the “ambulant stage”, the end of which is defined when the patient is unable to walk without a stick. The patients were psychologically supported by a community; one of the very important sign of this is to search for a sorcerer who, as already mentioned, they believed caused kuru. This period is characterized by the onset of subtle signs of gait unsteadiness that are usually only self-diagnosed, but which over a period of a month or so progress to severe astasia and ataxia. Incoordination of the muscles in trunk and lower limbs followed. As patients were well aware that kuru heralded death in about a year, they became withdrawn and quiet. A fine ‘shivering’ tremor, starting in the trunk, amplified by cold and associated with a “goose flesh”, is often followed by titubation and other types of abnormal movements. Attempts to maintain balance result in clawing of the toes and curling of the feet. Plantar reflex is always flexor while clonus, in particular ankle clonus but also patellar clonus, are hallmarks of the clinical picture, however, clonus may be only present for a limited period of time. The ankle clonus was in most cases the most enhanced, but patellar clonus and clonus of fingers and toes were also readily elicited.