期刊
JOURNAL OF NEURAL TRANSMISSION
卷 114, 期 4, 页码 451-456出版社
SPRINGER WIEN
DOI: 10.1007/s00702-006-0595-2
关键词
neuronal intermediate filament inclusion disease (NIFID); neurofilament cytoplasmic inclusions (NI); swollen achromatic neurons (SN); glial cell nuclei; spatial pattern
Neuronal intermediate filament inclusion disease (NIFID) is characterized by alpha-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 mu m 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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