PATHOLOGY AND IMMUNOCYTOCHEMISTRY OF A KURU BRAIN

We report here results of modern staining techniques including anti-pr ion protein (PrP) immunocytochemistry to a set of archival brain speci mens of a 16 year-old male who died from kuru in 1967. Brain suspensio ns transmitted disease to chimpanzees and New World monkeys. The PrP g ene is homozygous for valine at the polymorphic codon 129. Histology s hows neuronal loss, spongiform change, and astrogliosis. Lesions are m aximal 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 accumula tes as diffuse synaptic type deposits and mostly unicentric plaques. P rP 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, thalam us, and granular layer of cerebellar cortex. Meticulous examination re veals only rare ''florid'' plaques with surrounding vacuolation. We co nclude 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) ku ru does not share the neuropathological hallmarks of the new Creutzfel dt-Jakob disease variant recently reported in the UK and France; 3) to pographic prominence of PrP deposition parallels that of spongiform ch ange and/or astrogliosis.