Kuru

Definition:

A neurodegenerative disease cause by a "slow virus" and transmitted human-to-human.

Causes, incidence, and risk factors:

Kuru is a slow-virus disease, once prevalent in New Guinea and rarely seen now. Kuru causes neurodegenerative changes similar to another slow virus disease, Creutzfeldt-Jakob disease, whose distribution is worldwide. In addition, similar slow-virus diseases appear in sheep as scrapie, mink as mink encephalopathy, and in cows as bovine spongiform encephalopathy (BSE). Creutzfeldt-Jakob Disease has been found with increased incidence in North Africa where sheep brains and eyes are a common part of the diet.

Kuru may begin with a gait disturbance (cerebellar ataxia) and increasing incoordination. Incoordination leads to severe disability. Tremors and shivering are characteristic findings. Difficulty in swallowing and inability to feed oneself lead to malnutrition or starvation. Death occurs several years after the onset of symptoms.