Kuru is an acquired human
prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. The central clinical feature of
kuru is progressive
cerebellar ataxia and, in sharp contrast to most cases of
sporadic Creutzfeldt-Jakob disease (CJD),
dementia is a less prominent and usually late clinical feature. In this regard,
kuru is more similar to variant CJD, which also has similar
prodromal symptoms of sensory disturbance and
joint pains in the legs and psychiatric and behavioural changes. Since a significant part of the clinicopathological diversity seen in human
prion disease is likely to relate to the propagation of distinct human
prion strains, we have compared the transmission properties of
kuru prions with those isolated from patients with sporadic, iatrogenic and variant CJD in both transgenic and wild-type mice. These data have established that
kuru prions have
prion strain properties equivalent to those of classical (sporadic and iatrogenic) CJD
prions but distinct from variant CJD
prions. Here, we review these findings and discuss how peripheral routes of
infection and other factors may be critical modifiers of the
kuru phenotype.