Neuropathologic Findings in Exotic Neurologic Disorders Among Natives of the Highlands of New Guinea

Neumann, Meta A.; Gajdusek, D. Carleton; Zigas, Vincent

doi:10.1097/00005072-196407000-00007

Cited by 41 publications

(10 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Despite the unusually long incubation period in this case, the neuropathological findings faithfully reproduced the central features described previously in kuru (Fowler & Robertson 1959; Klatzo et al . 1959; Neumann et al . 1964; Beck & Daniel 1965; Kakulas et al .…”

Section: Discussionmentioning

confidence: 99%

Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease

Brandner

Whitfield

Boone

et al. 2008

Phil. Trans. R. Soc. B

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease

Brandner

Whitfield

Boone

et al. 2008

Phil. Trans. R. Soc. B

View full text Add to dashboard Cite

show abstract

“…The triad of spongiform change, neuronal loss and gliosis was seen in all kuru cases in a distribution similar to that originally described, with prominent involvement of all cortical areas with the exception of the occipital cortex, hippocampus and insular gyri, and prominent changes also seen in the putamen, caudate and cerebellar cortex (figure 1; Fowler & Robertson 1958; Klatzo et al . 1959; Neumann et al . 1964; Kakulas et al .…”

mentioning

confidence: 99%

The neuropathology of kuru and variant Creutzfeldt–Jakob disease

McLean

2008

Phil. Trans. R. Soc. B

View full text Add to dashboard Cite

A comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. Archival kuru and recent variant Creutzfeldt–Jakob disease (vCJD) cases reveal distinct lesional differences, particularly with respect to prion protein, suggesting that the strain of agent is important in determining the phenotype. Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. The presence of valine does not therefore appear to determine peripheral selection of PrPCJD. vCJD remains restricted to date to MM homozygosity on codon 129. It remains to be determined whether this genotype is dictating a shorter incubation period.

show abstract

“…The neuropathology of kuru resembled that of sCJD (Neumann et al 1964 many cases was the presence of amyloid plaques with a solid core and radiating fibrils in the cerebellar cortex, subsequently termed "kuru plaques" (Fig. 7).…”

Section: Acquired Human Prion Diseases Kurumentioning

confidence: 96%