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Topography of α ‐internexin‐positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease
Author(s) -
Armstrong R. A.,
Cairns N. J.
Publication year - 2006
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2006.01284.x
Subject(s) - dentate gyrus , cerebral cortex , protein filament , temporal lobe , cortex (anatomy) , neuroscience , intermediate filament , inclusion bodies , anatomy , hippocampus , pathology , medicine , biology , cell , cytoskeleton , genetics , epilepsy , biochemistry , escherichia coli , gene
Abnormal neuronal intermediate filament (IF) inclusions immunopositive for the type IV IF α ‐internexin have been identified as the pathological hallmark of neuronal intermediate filament inclusion disease (NIFID). We studied the topography of these inclusions in the frontal and temporal lobe in 68 areas from 10 cases of NIFID. In the cerebral cortex, CA sectors of the hippocampus, and dentate gyrus granule cell layer, the inclusions were distributed mainly in regularly distributed clusters, 50–800 μ m in diameter. In seven cortical areas, there was a more complex pattern in which the clusters of inclusions were aggregated into larger superclusters. In 11 cortical areas, the size of the clusters approximated to those of the cells of origin of the cortico‐cortical pathways but in the majority of the remaining areas, cluster size was smaller than 400 μ m. The topography of the lesions suggests that there is degeneration of the cortico‐cortical projections in NIFID with the formation of α ‐internexin‐positive aggregates within vertical columns of cells. Initially, only a subset of cells within a vertical column develops inclusions but as the disease progresses, the whole of the column becomes affected. The corticostriate projection appears to have little effect on the cortical topography of the inclusions.