OBSERVATIONS ON KURU. III : PATHOLOGICAL FEATURES IN FIVE CASES
Abstract
SUMMARY Five brains and two spinal cords together with various pieces of viscera from kuru patients have been examined. There was widespread atrophy of neurons in the central nervous system. The affected cells were shrunken and often vacuolated. The structures most prominently involved included the cerebellar cortex (particularly the vermis and flocculo‐nodular lobe), the dentate nucleus, thalamus, corpus striatum, globus pallidus and parts of the cerebral cortex (particularly the anterior central gyrus). In the two spinal cords, degeneration of the spino‐cerebellar and lateral cortico‐spinal tracts was found. Most of the cranial nerve nuclei were spared, and the substantia nigra was minimally involved. No evidence of an infective cause was discovered. Other organs, apart from the central nervous system, showed no significant changes.
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Fowler et al described severe demyelination in the lateral corticospinal tract and degeneration of dorsal and ventral spinocerebellar tracts in kuru.
“Fowler et al (67) described severe demyelination in the lateral corticospinal tract and degeneration of dorsal and ventral spinocerebellar tracts.”
From Kuru (2012) · cites this paper as [67]
Swollen neurons are occasionally described in kuru.
“Swollen neurons were occasionally described (67, 68).”
From Kuru (2012) · cites this paper as [67]
The most striking neuropathologic feature of kuru is the presence of numerous amyloid plaques, known as kuru plaques.
“The most striking neuropathologic feature of kuru is the presence of numerous amyloid plaques described as “spherical bodies with a rim of radiating filaments” and found in 6 of 12 cases studied by Klatzo et al (62) and in “about three quarters” of the 13 cases of Beck and Daniel (68); they then became known as “kuru plaques” (67, 69–71).”
From Kuru (2012) · cites this paper as [67]
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Kuru, the First Human Prion Disease
Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019) · Viruses · doi:10.3390/v11030232 · Cited by 69 · see citing works
Liberski, P. P., Sikorska, B., Lindenbaum, S., Goldfarb, L. G., McLean, C., Hainfellner, J. A., & Brown, P. (2012) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3182444efd · Cited by 50 · see citing works
“Fowler et al (67) described severe demyelination in the lateral corticospinal tract and degeneration of dorsal and ventral spinocerebellar tracts.”
BackgroundThe neuropathology of kuru and variant Creutzfeldt–Jakob disease
McLean, C. A. (2008) · Philosophical Transactions of the Royal Society B: Biological Sciences · doi:10.1098/rstb.2008.0086 · Cited by 17 · see citing works
- Authors
- FOWLER, M., & ROBERTSON, E. G.
- Affiliations
- The Royal Melbourne Hospital (AU)
- ORCID
- ROBERTSON, E. GRAEME
- Venue
- Australasian Annals of Medicine
- Publisher
- Wiley
- Published
- 1959-02
- Volume
- 8
- Issue
- 1
- Pages
- 16-26
- ISSN
- 0571-9283 (ISSN-L)
- OpenAlex
- W2403462274
- Cited by
- 41
- References
- 0
- Citations by year
- 2024: 1 · 2019: 2 · 2018: 1 · 2017: 1 · 2013: 2 · 2012: 1
- FWCI
- 1.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Australasian Annals of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Keywords
- kuru, Creutzfeldt-Jakob disease, neurodegeneration, neuronal atrophy, cerebellar cortex, dentate nucleus, thalamus, striatum, globus pallidus, corticospinal tract degeneration, neuropathology, myelopathy
- MeSH terms
- Brain, Brain Diseases, Central Nervous System Diseases, Humans, Kuru
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