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.
Brandner
Sebastian
S
Department of Neurodegenerative Disease, MRC Prion Unit, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Whitfield
Jerome
J
Boone
Ken
K
Puwa
Anderson
A
O'Malley
Catherine
C
Linehan
Jacqueline M
JM
Joiner
Susan
S
Scaravilli
Francesco
F
Calder
Ian
I
P Alpers
Michael
M
Wadsworth
Jonathan D F
JD
Collinge
John
J
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