Open AccessPathology and Immunocytochemistry of a Kuru Brain
Author(s) -
Hainfellner Johannes A.,
Liberski Pawel P.,
Guiroy Don C.,
Cervénaková Larisa,
Brown Paul,
Gajdusek D. Carleton,
Budka Herbert
Publication year - 1997
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.1997.tb01072.x
Subject(s) - kuru , thalamus , pathology , cortex (anatomy) , biology , cerebral cortex , cerebellar cortex , anatomy , basal ganglia , deep cerebellar nuclei , neuroscience , cerebellum , central nervous system , medicine , scrapie , disease , prion protein
We report here results of modern staining techniques including anti‐prion protein (PrP) immunocytochemistry to a set of archival brain specimens of a 16 year‐old male who died from kuru in 1967. Brain suspensions transmitted disease to chimpanzees and New World monkeys. The PrP gene is homozygous for valine at the polymorphic codon 129. Histology shows neuronal loss, spongiform change, and astrogliosis. Lesions are maximal in parasagittal and interhemispheric areas of frontal, central and parietal cortex, cingulate cortex, striatum, and thalamus, and are accentuated in middle and deep cerebral cortical layers. PrP accumulates as diffuse synaptic type deposits and mostly unicentric plaques. PrP deposition is maximal in parasagittal and interhemispheric areas of frontal, central and parietal cortex, cingulate cortex, basal ganglia, and cerebellar cortex. Plaques are prominent in the striatum, thalamus, and granular layer of cerebellar cortex. Meticulous examination reveals only rare “florid” plaques with surrounding vacuolation. We conclude that 1) pathology including immunomorphology of PrP deposition in this kuru brain is within the lesion spectrum of Creutzfeldt‐Jakob disease although plaques are unusually prominent and widespread; 2) kuru does not share the neuropathological hallmarks of the new Creutzfeldt‐Jakob disease variant recently reported in the UK and France; 3) topographic prominence of PrP deposition parallels that of spongiform change and/or astrogliosis.