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Von Economo Neuron Loss in Frontotemporal Dementia : A Meta-Analysis of Neuropathological Studies

Talmasov, Daniel ; Santillo, Alexander F. LU orcid and Hof, Patrick R. (2026) In Annals of Clinical and Translational Neurology
Abstract

Von Economo neurons (VENs) have been reported to be vulnerable to neurodegeneration in frontotemporal dementia (FTD), particularly the behavioral variant (bvFTD), but these findings have not been systematically assessed across independent brain banks. We conducted a meta-analysis of neuropathological studies measuring VEN density in the anterior cingulate cortex or frontoinsular cortex in FTD using random-effects models with cluster-robust variance estimation. Seven studies (135 FTD, 68 controls) from four international brain banks showed significantly reduced VEN density in FTD with a large effect size (g = −1.45, 95% CI [−1.69, −1.21], p < 0.001) and remarkable consistency (I2 = 0%). VEN loss was greater in FTD than... (More)

Von Economo neurons (VENs) have been reported to be vulnerable to neurodegeneration in frontotemporal dementia (FTD), particularly the behavioral variant (bvFTD), but these findings have not been systematically assessed across independent brain banks. We conducted a meta-analysis of neuropathological studies measuring VEN density in the anterior cingulate cortex or frontoinsular cortex in FTD using random-effects models with cluster-robust variance estimation. Seven studies (135 FTD, 68 controls) from four international brain banks showed significantly reduced VEN density in FTD with a large effect size (g = −1.45, 95% CI [−1.69, −1.21], p < 0.001) and remarkable consistency (I2 = 0%). VEN loss was greater in FTD than Alzheimer's disease and occurred across TDP-43 and tau pathological subtypes.

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author
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organization
publishing date
type
Contribution to journal
publication status
epub
subject
keywords
frontotemporal dementia, frontotemporal lobar degeneration, neurodegeneration, neuronal vulnerability, von economo neurons
in
Annals of Clinical and Translational Neurology
publisher
John Wiley & Sons Inc.
external identifiers
  • pmid:41789587
  • scopus:105032125527
ISSN
2328-9503
DOI
10.1002/acn3.70362
language
English
LU publication?
yes
id
4c410414-1f76-432e-b45a-fe7749d6c9d5
date added to LUP
2026-04-24 13:39:00
date last changed
2026-05-08 14:31:32
@article{4c410414-1f76-432e-b45a-fe7749d6c9d5,
  abstract     = {{<p>Von Economo neurons (VENs) have been reported to be vulnerable to neurodegeneration in frontotemporal dementia (FTD), particularly the behavioral variant (bvFTD), but these findings have not been systematically assessed across independent brain banks. We conducted a meta-analysis of neuropathological studies measuring VEN density in the anterior cingulate cortex or frontoinsular cortex in FTD using random-effects models with cluster-robust variance estimation. Seven studies (135 FTD, 68 controls) from four international brain banks showed significantly reduced VEN density in FTD with a large effect size (g = −1.45, 95% CI [−1.69, −1.21], p &lt; 0.001) and remarkable consistency (I<sup>2</sup> = 0%). VEN loss was greater in FTD than Alzheimer's disease and occurred across TDP-43 and tau pathological subtypes.</p>}},
  author       = {{Talmasov, Daniel and Santillo, Alexander F. and Hof, Patrick R.}},
  issn         = {{2328-9503}},
  keywords     = {{frontotemporal dementia; frontotemporal lobar degeneration; neurodegeneration; neuronal vulnerability; von economo neurons}},
  language     = {{eng}},
  publisher    = {{John Wiley & Sons Inc.}},
  series       = {{Annals of Clinical and Translational Neurology}},
  title        = {{Von Economo Neuron Loss in Frontotemporal Dementia : A Meta-Analysis of Neuropathological Studies}},
  url          = {{http://dx.doi.org/10.1002/acn3.70362}},
  doi          = {{10.1002/acn3.70362}},
  year         = {{2026}},
}