Abstract:
:Abnormal neuronal intermediate filament (IF) inclusions immunopositive for the type IV IF alpha-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 microm in diameter. In seven cortical areas, there was a more complex pattern in which the clusters of inclusions were aggregated into larger super clusters. 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 microm. The topography of the lesions suggests that there is degeneration of the cortico-cortical projections in NIFID with the formation of alpha-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.
journal_name
Eur J Neuroljournal_title
European journal of neurologyauthors
Armstrong RA,Cairns NJdoi
10.1111/j.1468-1331.2006.01284.xsubject
Has Abstractpub_date
2006-05-01 00:00:00pages
528-32issue
5eissn
1351-5101issn
1468-1331pii
ENE1284journal_volume
13pub_type
杂志文章abstract:BACKGROUND:The burden of multiple sclerosis (MS) includes fatigue, depression and worsening of health-related quality of life (HRQOL). These changes have not been yet measured in neuromyelitis optica (NMO). Our aim was to assess the HRQOL, fatigue and depression in NMO. METHODS:We administered French validated self-qu...
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更新日期:1999-03-01 00:00:00
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