Kuru (disease)

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Kuru (disease)

Kuru (pronunciation: koo-roo) is a rare and fatal neurodegenerative disorder that was predominantly identified among the Fore people of Papua New Guinea. Kuru is considered a type of transmissible spongiform encephalopathies (TSEs), also known as prion diseases.

Etymology

The term "Kuru" derives from the Fore word kuria/guria (to shake), a reference to the body tremors that are a classic symptom of the disease. It is also known colloquially as the "laughing sickness" due to the pathologic bursts of laughter people would display when afflicted with the disease.

Symptoms

Kuru is characterized by progressive cerebellar ataxia, or loss of coordination and control over muscle movements. The initial stage, known as the Ambulatory stage, includes unsteady stance and gait, decreased muscle control, tremors, and dysarthria (impaired speech). The second stage, the Sedentary stage, is marked by the inability to walk without support, severe tremors, and emotional instability. The terminal stage of the disease, which may last up to a year, is characterized by the inability to sit without support, severe intellectual impairment, and incontinence.

Transmission

Kuru was transmitted among the Fore people through a practice of mortuary Cannibalism, in which relatives consumed the bodies of the deceased to return the "life force" of the deceased to the hamlet. A genetic mutation protected some individuals from kuru, leading to its eventual eradication.

Treatment and Prognosis

There is no known cure or treatment for kuru. The disease is invariably fatal. The incubation period of the disease is variable; it can be as short as a few years or last several decades. Death usually occurs within a year of the onset of symptoms.

See Also

External links

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