Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID): an α-internexin immunohistochemical study

Richard A. Armstrong*, Nigel J. Cairns

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Neuronal intermediate filament inclusion disease (NIFID) is characterized by α-internexin positive neuronal cytoplasmic inclusions (NCI), swollen achromatic neurons (SN), neuronal loss, and gliosis. This study tested: 1) whether the spatial patterns of the lesions was topographically organized in areas of the frontal and temporal lobe and 2) whether a spatial relationship exists between the NCI and SN. The NCI were distributed in regular clusters and in a quarter of these areas, the clusters were 400-800 μm in diameter approximating to the size of the cells of origin of the cortico-cortical pathways. Variations in the density of the NCI were positively correlated with the SN. Hence, cortical degeneration in NIFID appears to be topographically organized and may affect the cortico-cortical projections, the clusters of NCI and SN developing within the same vertical columns of cells. © 2007 Springer-Verlag.

Original languageEnglish
Pages (from-to)451-456
Number of pages6
JournalJournal of Neural Transmission
Volume114
Issue number4
DOIs
Publication statusPublished - Apr 2007

Keywords

  • neuronal intermediate filament inclusion disease
  • neurofilament cytoplasmic inclusions
  • swollen achromatic neurons
  • glial cell nuclei
  • spatial pattern

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