Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease.
While the neuropathology of kuru is well defined, there are few data concerning the distribution of disease-related prion protein in peripheral tissues. Here we report the investigation of brain and peripheral tissues from a kuru patient who died in 2003. Neuropathological findings were compared with those seen in classical (sporadic and iatrogenic) Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD). The neuropathological findings of the kuru patient showed all the stereotypical changes that define kuru, with the occurrence of prominent PrP plaques throughout the brain. Lymphoreticular tissue showed no evidence of prion colonization, suggesting that the peripheral pathogenesis of kuru is similar to that seen in classical CJD rather than vCJD. These findings now strongly suggest that the characteristic peripheral pathogenesis of vCJD is determined by prion strain type alone rather than route of infection.
Department of Neurodegenerative Disease, MRC Prion Unit, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Sebastian
S
Brandner
Jerome
J
Whitfield
Ken
K
Boone
Anderson
A
Puwa
Catherine
C
O'Malley
Jacqueline M
JM
Linehan
Susan
S
Joiner
Francesco
F
Scaravilli
Ian
I
Calder
Michael
M
P Alpers
Jonathan D F
JD
Wadsworth
John
J
Collinge
D018450:Disease Progression; D006801:Humans; D015151:Immunoblotting; D007150:Immunohistochemistry; D007729:Kuru / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009420:Nervous System / Q000473:pathology*; D011328:Prions / Q000235:genetics / Q000378:metabolism*
D011328:Prions