Kuru is a prion disease seen in a region of New Guinea.
Kuru means trembling from cold or fear in the native language.
Location: Eastern Highlands of New Guinea
Tribe: Fore
Mode of transmission: cannibalism of dead relatives
Clinical findings:
(1) The first sign is the onset of locomotor ataxia with a swaying gait.
(2) The patient develops a tremor of the extremities, trunk and head which may be aggravated by excitement or fatigue.
(3) variable involuntary athetoid movements
(4) deterioration in the ability to ambulate over several weeks
(5) dysarthria with slurred speech
(6) intelligence and awareness are preserved
(7) variable emotional lability (excessive laughter, depression, belligerence)
(8) becomes homebound then bedridden and incontinent
(9) develops a mask-like face, convergent strabismus and dysarthria prior to death
(10) eventually the person is unable to swallow and dies from starvation or dehydration
Mortality rate: 100% over 3-6 months
