The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD)

Richard Armstrong

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

Abstract

The occipital lobe is one of the cortical areas most affected by the pathology of variant Creutzfeldt-Jakob disease (vCJD). To understand the visual problems of vCJD patients, neuropathological changes were studied in striate (B17, V1) and extrastriate (B18, V2) regions of the occipital cortex in eleven cases of vCJD. No differences in the density of vacuoles or surviving neurons were observed in B17 and B18 but densities of glial cell nuclei and deposits of the protease resistant form of prion protein (PrPsc) were greater in B18. The density of PrPsc deposits in B17 was positively correlated with their density in B18. The density of the diffuse PrPsc deposits in B17 was negatively correlated with the density of the surviving neurons in B18. In B17 and B18, the vacuoles either exhibited density peaks in laminae II/III and V/VI or were more uniformly distributed across the laminae. Diffuse PrPsc deposits were most frequent in laminae II/III and florid PrPsc deposits more generally distributed. In B18, the surviving neurons were more consistently bimodally distributed and the glial cell nuclei most abundant in laminae V/VI compared with B17. Hence, both striate and extrastriate areas of the occipital cortex are affected by the pathology of vCJD, the pathological changes being most severe in B18. Neuronal degeneration in B18 may be associated with the development of diffuse PrPsc deposits in B17. These data suggest that the short cortico-cortical connections between B17 and B18 and the pathways to subcortical visual areas are compromised in vCJD. Pathological changes in striate and extrastriate regions of the occipital cortex may contribute to several of the visual problems identified in patients with vCJD including oculomotor and visuo-spatial function.

LanguageEnglish
Title of host publicationNeuropathology
Subtitle of host publicationnew research
EditorsEstevo Almeirao, Thiago Honrado
PublisherNova science
Pages147-168
Number of pages22
ISBN (Electronic)978-1-62257-584-8
ISBN (Print)978-1-62257-583-1, 1-62257-583-0
Publication statusPublished - Oct 2012

Publication series

NameNeuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments
PublisherNova Science Publishers

Fingerprint

Occipital Lobe
Creutzfeldt-Jakob Syndrome
Substantia Gelatinosa
Vacuoles
Cell Nucleus
Neurons
Neuroglia
Pathology
Neuropathology
Peptide Hydrolases

Cite this

Armstrong, R. (2012). The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD). In E. Almeirao, & T. Honrado (Eds.), Neuropathology: new research (pp. 147-168). (Neuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments). Nova science.
Armstrong, Richard. / The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD). Neuropathology: new research. editor / Estevo Almeirao ; Thiago Honrado. Nova science, 2012. pp. 147-168 (Neuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments).
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Armstrong, R 2012, The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD). in E Almeirao & T Honrado (eds), Neuropathology: new research. Neuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments, Nova science, pp. 147-168.

The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD). / Armstrong, Richard.

Neuropathology: new research. ed. / Estevo Almeirao; Thiago Honrado. Nova science, 2012. p. 147-168 (Neuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments).

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

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N2 - The occipital lobe is one of the cortical areas most affected by the pathology of variant Creutzfeldt-Jakob disease (vCJD). To understand the visual problems of vCJD patients, neuropathological changes were studied in striate (B17, V1) and extrastriate (B18, V2) regions of the occipital cortex in eleven cases of vCJD. No differences in the density of vacuoles or surviving neurons were observed in B17 and B18 but densities of glial cell nuclei and deposits of the protease resistant form of prion protein (PrPsc) were greater in B18. The density of PrPsc deposits in B17 was positively correlated with their density in B18. The density of the diffuse PrPsc deposits in B17 was negatively correlated with the density of the surviving neurons in B18. In B17 and B18, the vacuoles either exhibited density peaks in laminae II/III and V/VI or were more uniformly distributed across the laminae. Diffuse PrPsc deposits were most frequent in laminae II/III and florid PrPsc deposits more generally distributed. In B18, the surviving neurons were more consistently bimodally distributed and the glial cell nuclei most abundant in laminae V/VI compared with B17. Hence, both striate and extrastriate areas of the occipital cortex are affected by the pathology of vCJD, the pathological changes being most severe in B18. Neuronal degeneration in B18 may be associated with the development of diffuse PrPsc deposits in B17. These data suggest that the short cortico-cortical connections between B17 and B18 and the pathways to subcortical visual areas are compromised in vCJD. Pathological changes in striate and extrastriate regions of the occipital cortex may contribute to several of the visual problems identified in patients with vCJD including oculomotor and visuo-spatial function.

AB - The occipital lobe is one of the cortical areas most affected by the pathology of variant Creutzfeldt-Jakob disease (vCJD). To understand the visual problems of vCJD patients, neuropathological changes were studied in striate (B17, V1) and extrastriate (B18, V2) regions of the occipital cortex in eleven cases of vCJD. No differences in the density of vacuoles or surviving neurons were observed in B17 and B18 but densities of glial cell nuclei and deposits of the protease resistant form of prion protein (PrPsc) were greater in B18. The density of PrPsc deposits in B17 was positively correlated with their density in B18. The density of the diffuse PrPsc deposits in B17 was negatively correlated with the density of the surviving neurons in B18. In B17 and B18, the vacuoles either exhibited density peaks in laminae II/III and V/VI or were more uniformly distributed across the laminae. Diffuse PrPsc deposits were most frequent in laminae II/III and florid PrPsc deposits more generally distributed. In B18, the surviving neurons were more consistently bimodally distributed and the glial cell nuclei most abundant in laminae V/VI compared with B17. Hence, both striate and extrastriate areas of the occipital cortex are affected by the pathology of vCJD, the pathological changes being most severe in B18. Neuronal degeneration in B18 may be associated with the development of diffuse PrPsc deposits in B17. These data suggest that the short cortico-cortical connections between B17 and B18 and the pathways to subcortical visual areas are compromised in vCJD. Pathological changes in striate and extrastriate regions of the occipital cortex may contribute to several of the visual problems identified in patients with vCJD including oculomotor and visuo-spatial function.

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Armstrong R. The neuropathology of the occipital cortex and its significance in the visual problems of patients with variant Creutzfeldt-Jakob Disease (vCJD). In Almeirao E, Honrado T, editors, Neuropathology: new research. Nova science. 2012. p. 147-168. (Neuroscience Research Progress; Neurology - Laboratory and Clinical Research Developments).