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Cited by 78Prion Diseases and Protein MisfoldingAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

Pathology and Immunocytochemistry of a Kuru Brain

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Hainfellner, Johannes A., Liberski, Pawel P., Guiroy, Don C., Cervénaková, Larisa, Brown, Paul, Gajdusek, D. Carleton, Budka, Herbert·1997·Brain Pathology
DOI 10.1111/j.1750-3639.1997.tb01072.xPMC PMC8098557
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Abstract

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.

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  • Immunohistochemical studies revealed that PrPTSE is present not only as kuru plaques but also in synaptic and perineuronal sites and in the spinal cord within substantia gelatinosa in kuru.

    “Immunohistochemical studies revealed that PrPTSE was present not only as kuru plaques but also in synaptic and perineuronal sites (64) and in the spinal cord within substantia gelatinosa.”

    From Kuru (2012) · cites this paper as [64]

    The Abstract (no page given) reports anti-PrP immunocytochemistry in a kuru brain and describes both diffuse synaptic-type deposits and plaques. Our copy does not report perineuronal deposits or PrP in the spinal cord’s substantia gelatinosa; searches for those sites returned no supporting passage. (LLM, conf=0.86)

    Experimental — accuracy not yet measured

  • Neuronal alterations in kuru are totally nonspecific in nature.

    “Neuronal alterations observed in many nuclei throughout the neuraxis, the anterior motor neurons of the spinal cord, brainstem, cerebellum, and cerebral cortex were totally nonspecific in nature (64);”

    From Kuru (2012) · cites this paper as [64]

    The Abstract (page unavailable) reports neuronal loss and its distribution but does not say that neuronal alterations were nonspecific. Searches for “totally nonspecific” and for neuronal alterations returned no supporting passage. Our indexed copy points to a separate PDF for the full text, so the decisive discussion may be missing. (LLM, conf=0.74)

    Experimental — accuracy not yet measured

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Authors
Hainfellner, J. A., Liberski, P. P., Guiroy, D. C., Cervénaková, L., Brown, P., Gajdusek, D. C., & Budka, H.
Affiliations
  • University of Vienna (AT)
  • Medical University of Lodz (PL)
  • National Institutes of Health (US)
Corresponding author
Budka, Herbert
ORCID
Venue
Brain Pathology
Publisher
Wiley
Published
1997-01
Volume
7
Issue
1
Pages
547-553
ISSN
  • 1015-6305 (ISSN-L)
  • 1750-3639
DOI
10.1111/j.1750-3639.1997.tb01072.x
PMC
PMC8098557
OpenAlex
W1977432546
Cited by
78
References
0
Citations by year
2024: 3 · 2022: 2 · 2021: 2 · 2020: 1 · 2019: 1 · 2017: 1 · 2014: 2 · 2013: 4 · 2012: 4
FWCI
2.26
Type
journal article
Language
en
Open access
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor, 4196 d)
Locations
  • Brain Pathology (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords
kuru, prion disease, prion protein, Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, spongiform change, astrocyte activation, neuronal loss, striatum, thalamus, cerebellar cortex
MeSH terms
Adolescent, Brain, Humans, Immunohistochemistry, Kuru, Male, Prions, Staining and Labeling

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