Spatial patterns of the pathological changes in the cerebellar cortex in sporadic Creutzfeldt-Jakob disease (sCJD)

Richard A. Armstrong, Nigel J. Cairns

Research output: Contribution to journalArticle

Abstract

The spatial patterns of the vacuolation ("spongiform change"), surviving cells, and prion protein (PrP) deposition were studied in the various cell laminae of the cerebellar cortex in 11 cases of sporadic Creutzfeldt-Jakob disease (sCJD). Clustering of the histological features, with the clusters regularly distributed along the folia, was evident in all cell laminae. In the molecular layer, clusters of vacuoles coincided with the surviving Purkinje cells. In the granule cell layer, however, the spatial relationship between the vacuoles and surviving cells was more complex and varied between cases. PrP deposition was not spatially correlated with either the vacuoles or the surviving cells in any of the cerebellar laminae in the majority of cases. In some cases, there were spatial relationships between th histological features in the molecular and granule cell layers. The data suggest that degeneration of the cerebellar cortex in sCJD may occur in a topographic pattern consistent with the spread of prion pathology along anatomical pathways. The development of the vacuolation may be an early stage of the pathology in the cerebellum preceding the appearance of the PrP deposits. In addition, there is evidence that the pathological changes may spread across the different laminae of the cerebellar cortex.
Original languageEnglish
Pages (from-to)183-189
Number of pages7
JournalFolia Neuropathologica
Volume41
Issue number4
Publication statusPublished - 2003

Keywords

  • sporadic Creutzfeldt-Jakob disease
  • cerebellum
  • vacuolation
  • prion protein deposition
  • Purkinje cells
  • spatial pattern

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