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

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Summary

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.

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Authors and Affiliations

  1. Vision Sciences, Aston University, Birmingham, UK

    R. A. Armstrong

  2. Departments of Neurology and Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA

    N. J. Cairns

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  1. R. A. Armstrong
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  2. N. J. Cairns
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Armstrong, R., Cairns, N. Spatial patterns of the pathological changes in neuronal intermediate filament inclusion disease (NIFID): an α-internexin immunohistochemical study. J Neural Transm 114, 451–456 (2007). https://doi.org/10.1007/s00702-006-0595-2

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  • DOI: https://doi.org/10.1007/s00702-006-0595-2

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