A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD)

Richard A. Armstrong

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The objective of this study was to determine the degree of white matter pathology in the cerebral cortex in cases of variant Creutzfeldt-Jakob disease (vCJD) and to study the relationships between the white matter and grey matter pathologies. Hence, the pathological changes in cortical white matter were studied in individual gyri of the frontal, parietal, occipital, and temporal cortex in eleven cases of vCJD. Vacuolation (‘spongiform change’), deposition of the disease form of prion protein (PrPsc) in the form of discrete PrP deposits, and gliosis were observed in the white matter of virtually all cortical regions studied. Mean density of the vacuoles in the white matter was greater in the parietal lobe compared with the frontal, occipital, and temporal lobes but there were fewer glial cells in the occipital lobe compared with the other cortical regions. In the white matter of the frontal cortex, vacuole density was negatively correlated with the density of both glial cell nuclei and the PrP deposits. In addition, the densities of glial cells and PrP deposits were positively correlated in the frontal and parietal cortex. In the white matter of the frontal cortex and inferior temporal gyrus, there was a negative correlation between the densities of the vacuoles and the number of surviving neurons in laminae V/VI of the adjacent grey matter. In addition, in the frontal cortex, vacuole density in the white matter was negatively correlated with the density of the diffuse PrP deposits in laminae II/III and V/VI of the adjacent grey matter. The densities of PrP deposits in the white matter of the frontal cortex were positively correlated with the density of the diffuse PrP deposits in laminae II/III and V/V1 and with the number of surviving neurons in laminae V/V1. The data suggest that in the white matter in vCJD, gliosis is associated with the development of PrP deposits while the appearance of the vacuolation is a later development. In addition, neuronal loss and PrP deposition in the lower cortical laminae of the grey matter may be a consequence of axonal degeneration within the white matter.
Original languageEnglish
Title of host publicationHandbook on White Matter: Structure, Function and Changes
EditorsTimothy B. Westland, Robert N. Calton
PublisherNova science
Pages133-146
Number of pages14
ISBN (Print)9781607410348
Publication statusPublished - 4 Jan 2009

Publication series

NameNeuroanatomy Research at the Leading Edge
PublisherNova Science

Fingerprint

Creutzfeldt-Jakob Syndrome
Frontal Lobe
Vacuoles
Occipital Lobe
Parietal Lobe
Temporal Lobe
Substantia Gelatinosa
Neuroglia
Gliosis
White Matter
Pathology
Neurons
Prion Diseases
Cell Nucleus
Cerebral Cortex

Bibliographical note

Also availble as an eBook

Keywords

  • white matter pathology
  • cerebral cortex
  • variant Creutzfeldt-Jakob disease
  • grey matter
  • pathology
  • pathological changes
  • temporal cortex
  • Vacuolation deposition
  • prion protein
  • discrete PrP deposits
  • gliosis

Cite this

Armstrong, R. A. (2009). A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD). In T. B. Westland, & R. N. Calton (Eds.), Handbook on White Matter: Structure, Function and Changes (pp. 133-146). (Neuroanatomy Research at the Leading Edge). Nova science.
Armstrong, Richard A. / A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD). Handbook on White Matter: Structure, Function and Changes. editor / Timothy B. Westland ; Robert N. Calton. Nova science, 2009. pp. 133-146 (Neuroanatomy Research at the Leading Edge).
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Armstrong, RA 2009, A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD). in TB Westland & RN Calton (eds), Handbook on White Matter: Structure, Function and Changes. Neuroanatomy Research at the Leading Edge, Nova science, pp. 133-146.

A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD). / Armstrong, Richard A.

Handbook on White Matter: Structure, Function and Changes. ed. / Timothy B. Westland; Robert N. Calton. Nova science, 2009. p. 133-146 (Neuroanatomy Research at the Leading Edge).

Research output: Chapter in Book/Report/Conference proceedingChapter

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Armstrong RA. A quantitative study of the pathological changes in the cortical white matter in variant Creutzfeldt-Jakob disease (vCJD). In Westland TB, Calton RN, editors, Handbook on White Matter: Structure, Function and Changes. Nova science. 2009. p. 133-146. (Neuroanatomy Research at the Leading Edge).