Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)

Richard A. Armstrong, Nigel J. Cairns

Research output: Contribution to journalArticle

Abstract

Neuronal cytoplasmic inclusions (NCI) immunoreactive for transactive response DNA-binding protein (TDP-43) are the pathological hallmark of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). We studied the spatial patterns of the TDP-43 immunoreactive NCI in the frontal and temporal cortex of 15 cases of FTLD-TDP. The NCI were distributed parallel to the tissue boundary predominantly in regular clusters 50-400 µm in diameter. In five cortical areas, the size of the clusters approximated to the cells of the cortico-cortical pathways. In most regions, cluster size was smaller than 400 µm. There were no significant differences in spatial patterns between familial and sporadic cases. Cluster size of the NCI was not correlated with disease duration, brain weight, Braak stage, or disease subtype. The spatial pattern of the NCI was similar to that of neuronal inclusions in other neurodegenerative diseases and may reflect a common pattern of degeneration involving the cortico-cortical projections.
LanguageEnglish
Pages653-659
Number of pages7
JournalNeurological Sciences
Volume32
Issue number4
DOIs
Publication statusPublished - Aug 2011

Fingerprint

TDP-43 Proteinopathies
Frontotemporal Lobar Degeneration
Inclusion Bodies
DNA-Binding Proteins
Brain Diseases
Frontal Lobe
Temporal Lobe
Neurodegenerative Diseases
Weights and Measures

Bibliographical note

The original publication is available at www.springerlink.com

Keywords

  • frontotemporal lobar degeneration with TDP-43 proteinopathy
  • FTLD-TDP
  • TAR DNA-binding protein of 43 kDa
  • TDP-43
  • neuronal cytoplasmic inclusions
  • NCI
  • spatial pattern

Cite this

@article{80d630ee7b2244ec853017f7495b9b47,
title = "Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)",
abstract = "Neuronal cytoplasmic inclusions (NCI) immunoreactive for transactive response DNA-binding protein (TDP-43) are the pathological hallmark of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). We studied the spatial patterns of the TDP-43 immunoreactive NCI in the frontal and temporal cortex of 15 cases of FTLD-TDP. The NCI were distributed parallel to the tissue boundary predominantly in regular clusters 50-400 µm in diameter. In five cortical areas, the size of the clusters approximated to the cells of the cortico-cortical pathways. In most regions, cluster size was smaller than 400 µm. There were no significant differences in spatial patterns between familial and sporadic cases. Cluster size of the NCI was not correlated with disease duration, brain weight, Braak stage, or disease subtype. The spatial pattern of the NCI was similar to that of neuronal inclusions in other neurodegenerative diseases and may reflect a common pattern of degeneration involving the cortico-cortical projections.",
keywords = "frontotemporal lobar degeneration with TDP-43 proteinopathy, FTLD-TDP, TAR DNA-binding protein of 43 kDa, TDP-43, neuronal cytoplasmic inclusions, NCI, spatial pattern",
author = "Armstrong, {Richard A.} and Cairns, {Nigel J.}",
note = "The original publication is available at www.springerlink.com",
year = "2011",
month = "8",
doi = "10.1007/s10072-011-0639-8",
language = "English",
volume = "32",
pages = "653--659",
journal = "Neurological Sciences",
issn = "1590-1874",
publisher = "Springer",
number = "4",

}

Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). / Armstrong, Richard A.; Cairns, Nigel J.

In: Neurological Sciences, Vol. 32, No. 4, 08.2011, p. 653-659.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)

AU - Armstrong, Richard A.

AU - Cairns, Nigel J.

N1 - The original publication is available at www.springerlink.com

PY - 2011/8

Y1 - 2011/8

N2 - Neuronal cytoplasmic inclusions (NCI) immunoreactive for transactive response DNA-binding protein (TDP-43) are the pathological hallmark of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). We studied the spatial patterns of the TDP-43 immunoreactive NCI in the frontal and temporal cortex of 15 cases of FTLD-TDP. The NCI were distributed parallel to the tissue boundary predominantly in regular clusters 50-400 µm in diameter. In five cortical areas, the size of the clusters approximated to the cells of the cortico-cortical pathways. In most regions, cluster size was smaller than 400 µm. There were no significant differences in spatial patterns between familial and sporadic cases. Cluster size of the NCI was not correlated with disease duration, brain weight, Braak stage, or disease subtype. The spatial pattern of the NCI was similar to that of neuronal inclusions in other neurodegenerative diseases and may reflect a common pattern of degeneration involving the cortico-cortical projections.

AB - Neuronal cytoplasmic inclusions (NCI) immunoreactive for transactive response DNA-binding protein (TDP-43) are the pathological hallmark of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). We studied the spatial patterns of the TDP-43 immunoreactive NCI in the frontal and temporal cortex of 15 cases of FTLD-TDP. The NCI were distributed parallel to the tissue boundary predominantly in regular clusters 50-400 µm in diameter. In five cortical areas, the size of the clusters approximated to the cells of the cortico-cortical pathways. In most regions, cluster size was smaller than 400 µm. There were no significant differences in spatial patterns between familial and sporadic cases. Cluster size of the NCI was not correlated with disease duration, brain weight, Braak stage, or disease subtype. The spatial pattern of the NCI was similar to that of neuronal inclusions in other neurodegenerative diseases and may reflect a common pattern of degeneration involving the cortico-cortical projections.

KW - frontotemporal lobar degeneration with TDP-43 proteinopathy

KW - FTLD-TDP

KW - TAR DNA-binding protein of 43 kDa

KW - TDP-43

KW - neuronal cytoplasmic inclusions

KW - NCI

KW - spatial pattern

UR - http://www.scopus.com/inward/record.url?scp=80054004766&partnerID=8YFLogxK

U2 - 10.1007/s10072-011-0639-8

DO - 10.1007/s10072-011-0639-8

M3 - Article

VL - 32

SP - 653

EP - 659

JO - Neurological Sciences

T2 - Neurological Sciences

JF - Neurological Sciences

SN - 1590-1874

IS - 4

ER -