Neuropathological changes in ten cases of neuronal intermediate filament inclusion disease (NIFID): A study using α-internexin immunohistochemistry and principal components analysis (PCA)

Richard A. Armstrong*, E. Kerty, K. Skullerud, N. J. Cairns

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Ten cases of neuronal intermediate filament inclusion disease (NIFID) were studied quantitatively. The α-internexin positive neurofilament inclusions (NI) were most abundant in the motor cortex and CA sectors of the hippocampus. The densities of the NI and the swollen achromatic neurons (SN) were similar in laminae II/III and V/VI but glial cell density was greater in V/VI. The density of the NI was positively correlated with the SN and the glial cells. Principal components analysis (PCA) suggested that PC1 was associated with variation in neuronal loss in the frontal/temporal lobes and PC2 with neuronal loss in the frontal lobe and NI density in the parahippocampal gyrus. The data suggest: 1) frontal and temporal lobe degeneration in NIFID is associated with the widespread formation of NI and SN, 2) NI and SN affect cortical laminae II/III and V/VI, 3) the NI and SN affect closely related neuronal populations, and 4) variations in neuronal loss and in the density of NI were the most important sources of pathological heterogeneity. © Springer-Verlag 2005.

Original languageEnglish
Pages (from-to)1207-1215
Number of pages9
JournalJournal of Neural Transmission
Volume113
Issue number9
DOIs
Publication statusPublished - Sep 2006

Keywords

  • lesion density
  • neurofilament intermediate filament inclusion disease (NIFID)
  • neuronal inclusions (NI)
  • principal components analysis (PCA)
  • swollen achromatic neurons (SN)

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