Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease

Richard A. Armstrong

Research output: Chapter in Book/Report/Conference proceedingChapter (peer-reviewed)

Abstract

The objective of the present study was to compare quantitatively the neuropathology of two subtypes of Creutzfeldt-Jakob disease (CJD), viz., sporadic CJD (sCJD) and variant CJD (vCJD). The vacuolation (‘spongiform change’), surviving neurons, glial cell nuclei, and deposits of the disease form of prion protein (PrPsc) were quantified in histological sections of the cerebral cortex, hippocampus, and cerebellum in 11 cases of sCJD and 15 cases of vCJD. Three aspects of the quantitative pathology of each histological feature were studied: overall abundance (density or coverage), spatial distribution parallel to the tissue boundary, and laminar distribution across gyri of the cerebral cortex. Overall vacuole density was greater in sCJD than in vCJD in some regions while overall neuronal densities were greater in vCJD. In cerebral cortex, vacuoles and PrPsc deposits were distributed in clusters which exhibited a regular distribution parallel to the pia mater, this type of spatial pattern being more frequent in sCJD than in vCJD. In some cortical gyri there were differences in laminar distribution between subtypes, viz. the vacuolation was more generally distributed across cortical laminae in sCJD, neuronal loss was often greater in upper laminae in vCJD but in lower laminae in sCJD, and PrPsc deposits were more frequently distributed in upper laminae in vCJD but in lower laminae in sCJD. A significant gliosis affected lower cortical laminae in both sCJD and vCJD. Hence, there were differences in degeneration of cerebral cortex, hippocampus, and cerebellum in sCJD and vCJD, which may reflect variations in disease aetiology and propagation of PrPsc through the brain.
LanguageEnglish
Title of host publicationHorizons in neuroscience research
EditorsAndrea Costa, Eugenio Villalba
Place of PublicationHauppauge, NY (US)
PublisherNova science
Pages59-78
Number of pages19
Volume18
ISBN (Electronic)978-1-63482-382-1
ISBN (Print)978-1-63482-368-5
Publication statusPublished - 2015

Publication series

NameHorizons in neuroscience research
PublisherNova
Volume18

Fingerprint

Creutzfeldt-Jakob Syndrome
Cerebral Cortex
Vacuoles
Cerebellum
Hippocampus
Pia Mater
Prion Diseases
Gliosis
Neuropathology
Acquired CJD
Cell Nucleus
Neuroglia
Pathology
Neurons
Brain

Keywords

  • Sporadic Creutzfeldt-Jakob disease
  • Variant Creutzfeldt-Jacob disease
  • Spatial patterns
  • Laminar distribution
  • prion protein (PrP) deposition
  • cortical degeneration

Cite this

Armstrong, R. A. (2015). Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease. In A. Costa, & E. Villalba (Eds.), Horizons in neuroscience research (Vol. 18, pp. 59-78). (Horizons in neuroscience research; Vol. 18). Hauppauge, NY (US): Nova science.
Armstrong, Richard A. / Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease. Horizons in neuroscience research. editor / Andrea Costa ; Eugenio Villalba. Vol. 18 Hauppauge, NY (US) : Nova science, 2015. pp. 59-78 (Horizons in neuroscience research).
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Armstrong, RA 2015, Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease. in A Costa & E Villalba (eds), Horizons in neuroscience research. vol. 18, Horizons in neuroscience research, vol. 18, Nova science, Hauppauge, NY (US), pp. 59-78.

Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease. / Armstrong, Richard A.

Horizons in neuroscience research. ed. / Andrea Costa; Eugenio Villalba. Vol. 18 Hauppauge, NY (US) : Nova science, 2015. p. 59-78 (Horizons in neuroscience research; Vol. 18).

Research output: Chapter in Book/Report/Conference proceedingChapter (peer-reviewed)

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T1 - Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease

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N2 - The objective of the present study was to compare quantitatively the neuropathology of two subtypes of Creutzfeldt-Jakob disease (CJD), viz., sporadic CJD (sCJD) and variant CJD (vCJD). The vacuolation (‘spongiform change’), surviving neurons, glial cell nuclei, and deposits of the disease form of prion protein (PrPsc) were quantified in histological sections of the cerebral cortex, hippocampus, and cerebellum in 11 cases of sCJD and 15 cases of vCJD. Three aspects of the quantitative pathology of each histological feature were studied: overall abundance (density or coverage), spatial distribution parallel to the tissue boundary, and laminar distribution across gyri of the cerebral cortex. Overall vacuole density was greater in sCJD than in vCJD in some regions while overall neuronal densities were greater in vCJD. In cerebral cortex, vacuoles and PrPsc deposits were distributed in clusters which exhibited a regular distribution parallel to the pia mater, this type of spatial pattern being more frequent in sCJD than in vCJD. In some cortical gyri there were differences in laminar distribution between subtypes, viz. the vacuolation was more generally distributed across cortical laminae in sCJD, neuronal loss was often greater in upper laminae in vCJD but in lower laminae in sCJD, and PrPsc deposits were more frequently distributed in upper laminae in vCJD but in lower laminae in sCJD. A significant gliosis affected lower cortical laminae in both sCJD and vCJD. Hence, there were differences in degeneration of cerebral cortex, hippocampus, and cerebellum in sCJD and vCJD, which may reflect variations in disease aetiology and propagation of PrPsc through the brain.

AB - The objective of the present study was to compare quantitatively the neuropathology of two subtypes of Creutzfeldt-Jakob disease (CJD), viz., sporadic CJD (sCJD) and variant CJD (vCJD). The vacuolation (‘spongiform change’), surviving neurons, glial cell nuclei, and deposits of the disease form of prion protein (PrPsc) were quantified in histological sections of the cerebral cortex, hippocampus, and cerebellum in 11 cases of sCJD and 15 cases of vCJD. Three aspects of the quantitative pathology of each histological feature were studied: overall abundance (density or coverage), spatial distribution parallel to the tissue boundary, and laminar distribution across gyri of the cerebral cortex. Overall vacuole density was greater in sCJD than in vCJD in some regions while overall neuronal densities were greater in vCJD. In cerebral cortex, vacuoles and PrPsc deposits were distributed in clusters which exhibited a regular distribution parallel to the pia mater, this type of spatial pattern being more frequent in sCJD than in vCJD. In some cortical gyri there were differences in laminar distribution between subtypes, viz. the vacuolation was more generally distributed across cortical laminae in sCJD, neuronal loss was often greater in upper laminae in vCJD but in lower laminae in sCJD, and PrPsc deposits were more frequently distributed in upper laminae in vCJD but in lower laminae in sCJD. A significant gliosis affected lower cortical laminae in both sCJD and vCJD. Hence, there were differences in degeneration of cerebral cortex, hippocampus, and cerebellum in sCJD and vCJD, which may reflect variations in disease aetiology and propagation of PrPsc through the brain.

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KW - cortical degeneration

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M3 - Chapter (peer-reviewed)

SN - 978-1-63482-368-5

VL - 18

T3 - Horizons in neuroscience research

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Armstrong RA. Comparative quantitative neuropathology of the sporadic and variant subtypes of Creutzfeldt-Jakob disease. In Costa A, Villalba E, editors, Horizons in neuroscience research. Vol. 18. Hauppauge, NY (US): Nova science. 2015. p. 59-78. (Horizons in neuroscience research).