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Spatial patterns of the pathological changes in the temporal lobe of patients with neuronal intermediate filament inclusion disease.

Abstract
Neuronal intermediate filament inclusion disease (NIFID) is a new neurodegenerative disease characterized histologically by the presence of neuronal cytoplasmic inclusions (NI) immunopositive for intermediate filament proteins, neuronal loss, swollen achromatic neurons (SN), and gliosis. We studied the spatial patterns of these pathological changes parallel to the pia mater in gyri of the temporal lobe in four cases of NIFID. Both the NI and SN occurred in clusters that were regularly distributed parallel to the pia mater, the cluster sizes of the SN being significantly greater than those of the NI. In a significant proportion of areas studied, there was a spatial correlation between the clusters of NI and those of the SN and with the density of the surviving neurons. In addition, the clusters of surviving neurons were negatively correlated (out of phase) with the clusters of glial cell nuclei. The pattern of clustering of these histological features suggests that there is degeneration of the cortico-cortical projections in NIFID leading to the formation of NI and SN within the same vertical columns of cells. The glial cell reaction may be a response to the loss of neurons rather than to the appearance of the NI or SN.
AuthorsNigel J Cairns, Richard A Armstrong
JournalNeuropathology : official journal of the Japanese Society of Neuropathology (Neuropathology) Vol. 25 Issue 4 Pg. 298-303 (Dec 2005) ISSN: 0919-6544 [Print] Australia
PMID16382778 (Publication Type: Journal Article)
Topics
  • Adult
  • Female
  • Humans
  • Image Processing, Computer-Assisted
  • Inclusion Bodies (pathology)
  • Intermediate Filaments (pathology)
  • Male
  • Middle Aged
  • Nerve Degeneration (pathology)
  • Neurodegenerative Diseases (pathology)
  • Neuroglia (pathology)
  • Neurons (pathology)
  • Temporal Lobe (pathology)

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