Presentation
Kuru 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. It is characterized by truncal ataxia, preceded by headaches, joint pains and shaking of the limbs. Trembling is present in almost all patients with transmissible spongiform encephalopathy; Kuru is also known as "shiver".
The preclinical or asymptomatic phase, also called the incubation period, lasts between possibly 5 to 20 years following initial exposure. The clinical stage lasts an average of 12 months.
The symptoms of Kuru are broken down into three specific stages. The first, ambulant stage, exhibits unsteady stance and gait, decreased muscle control, tremors, deterioration of speech and dysarthria (slurred speech). In the second stage, sedentary stage, the patient is incapable of walking without support, suffers ataxia (loss of muscle coordination) and severe tremors. Furthermore, the victim is emotionally unstable, depressed, yet having uncontrolled sporadic laughter. Interestingly, the tendon reflexes are still normal at this point. In the final, terminal stage, the patient is incapable of sitting without support, suffers severe ataxia (no muscle coordination), is unable to speak, is incontinent (unable to restrain natural discharges/evacuations of urine or feces), has dysphagia (difficulty swallowing), is unresponsive to their surroundings, and acquires ulcerations (sores with pus and necrosis). An infected person usually dies within 3 months to 2 years after the first symptoms, often because of pneumonia or pressure sores infection.
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