Topography of α-internexin-positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease

Richard A. Armstrong*, Nigel J. Cairns

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

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 μm 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 μm. 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. © 2006 EFNS.

Original languageEnglish
Pages (from-to)528-532
Number of pages5
JournalEuropean Journal of Neurology
Volume13
Issue number5
DOIs
Publication statusPublished - May 2006

Fingerprint

Intermediate Filaments
Cytomegalovirus Infections
Dentate Gyrus
Frontal Lobe
Temporal Lobe
Cerebral Cortex
Hippocampus

Keywords

  • α-internexin
  • intermediate filaments
  • neuronal inclusions
  • neuronal intermediate filament inclusion disease
  • topographic pattern

Cite this

@article{7355ede44aa54fb79a68d69d3aec28a7,
title = "Topography of α-internexin-positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease",
abstract = "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 μm 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 μm. 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. {\circledC} 2006 EFNS.",
keywords = "α-internexin, intermediate filaments, neuronal inclusions, neuronal intermediate filament inclusion disease, topographic pattern",
author = "Armstrong, {Richard A.} and Cairns, {Nigel J.}",
year = "2006",
month = "5",
doi = "10.1111/j.1468-1331.2006.01284.x",
language = "English",
volume = "13",
pages = "528--532",
journal = "European Journal of Neurology",
issn = "1351-5101",
publisher = "Wiley-Blackwell",
number = "5",

}

Topography of α-internexin-positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease. / Armstrong, Richard A.; Cairns, Nigel J.

In: European Journal of Neurology, Vol. 13, No. 5, 05.2006, p. 528-532.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Topography of α-internexin-positive neuronal aggregates in 10 patients with neuronal intermediate filament inclusion disease

AU - Armstrong, Richard A.

AU - Cairns, Nigel J.

PY - 2006/5

Y1 - 2006/5

N2 - 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 μm 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 μm. 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. © 2006 EFNS.

AB - 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 μm 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 μm. 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. © 2006 EFNS.

KW - α-internexin

KW - intermediate filaments

KW - neuronal inclusions

KW - neuronal intermediate filament inclusion disease

KW - topographic pattern

UR - http://www.scopus.com/inward/record.url?scp=33744744717&partnerID=8YFLogxK

UR - http://www3.interscience.wiley.com/journal/118554581/abstract

U2 - 10.1111/j.1468-1331.2006.01284.x

DO - 10.1111/j.1468-1331.2006.01284.x

M3 - Article

C2 - 16722980

VL - 13

SP - 528

EP - 532

JO - European Journal of Neurology

JF - European Journal of Neurology

SN - 1351-5101

IS - 5

ER -