ArticleNeuropathology and applied neurobiology2019
Von Economo neurons are part of a larger neuronal population that are selectively vulnerable in C9orf72 frontotemporal dementia.
Article in Neuropathology and applied neurobiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
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Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 30 citations in OpenAlex.
- Von Economo Neuron Loss in Frontotemporal Dementia: A Meta-Analysis of Neuropathological Studies.Annals of clinical and translational neurology · 2026Pooled it
- An atlas of primate insular cortex reveals a signal-processing strategy in von Economo neurons.Nature cell biology · 2026Article
- Single-cell transcriptomic atlas of frontoinsular cortex reveals molecular correlates of selective neuronal vulnerability in FTD.bioRxiv : the preprint server for biology · 2026Article
- Vulnerability of anterior cingulate Von Economo neurons to FTLD-tauopathies in behavioral variant frontotemporal dementia.Cerebral cortex (New York, N.Y. : 1991) · 2026Article
- Autonomic dysfunction in neurodegenerative disease.Nature reviews. Neuroscience · 2025Review
- Cytoarchitectonic gradients of laminar degeneration in behavioural variant frontotemporal dementia.Brain : a journal of neurology · 2025Article
- FDG-PET anterior cingulate cortex hypometabolism as a marker of cerebral small vessel disease in Alzheimer's and vascular cognitive impairment and dementia.Cerebral circulation - cognition and behavior · 2025Article
- Selective cellular and regional vulnerability in frontotemporal lobar degeneration: a scoping review.Free neuropathology · 2025Review
- Frontotemporal lobar degeneration targets brain regions linked to expression of recently evolved genes.Brain : a journal of neurology · 2024Article
- Article
- The prefrontal cortex: from monkey to man.Brain : a journal of neurology · 2024Review
- FTLD targets brain regions expressing recently evolved genes.medRxiv : the preprint server for health sciences · 2023Article
- Frontotemporal Dementia, Where Do We Stand? A Narrative Review.International journal of molecular sciences · 2023Review
- The neurophysiological effect of NMDA-R antagonism of frontotemporal lobar degeneration is conditional on individual GABA concentration.Translational psychiatry · 2022Article
- Von Economo neurons: Cellular specialization of human limbic cortices?Journal of anatomy · 2022Review
- The severity of behavioural symptoms in FTD is linked to the loss of GABRQ-expressing VENs and pyramidal neurons.Neuropathology and applied neurobiology · 2022Article
- Transcriptomic analysis of frontotemporal lobar degeneration with TDP-43 pathology reveals cellular alterations across multiple brain regions.Acta neuropathologica · 2022Article
- The behavioral variant of Alzheimer's disease does not show a selective loss of Von Economo and phylogenetically related neurons in the anterior cingulate cortex.Alzheimer's research & therapy · 2022Article
- Spindle-Shaped Neurons in the Human Posteromedial (Precuneus) Cortex.Frontiers in synaptic neuroscience · 2021Article
- Von Economo Neurons - Primate-Specific or Commonplace in the Mammalian Brain?Frontiers in neural circuits · 2021Review
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
aimsThe behavioural variant of frontotemporal dementia with a C9orf72 expansion (C9-bvFTD) is characterised by early changes in social-emotional cognition that are linked to the loss of von Economo neurons (VENs). Together with a subset of neighbouring pyramidal neurons, VENs express the GABA receptor subunit theta (GABRQ). It is not known if the selective vulnerability of VENs in C9-bvFTD also includes this GABRQ-expressing population.
methodsWe quantified VENs and GABRQ immunopositive neurons in the anterior cingulate cortex (ACC) in C9-bvFTD (n = 16), controls (n = 12) and Alzheimer's disease (AD) (n = 7). Second, we assessed VENs and GABRQ-expressing populations in relation to the clinicopathological profiles.
resultsWe found the number of VENs and GABRQ-expressing neurons and their ratio over the total layer 5 neuronal population was lower in C9-bvFTD compared to control and AD. C9-bvFTD donors with underlying TDP43 type A pathology in the ACC showed the highest loss of GABRQ-expressing neurons. C9-bvFTD donors that did not present with motor neuron disease (MND) symptoms in the first half of their disease course showed a prominent loss of GABRQ-expressing neurons compared to controls. C9-bvFTD donors with no symptoms of psychosis showed a higher loss compared to controls. Across all donors, the number of VENs correlated strongly with the number of GABRQ-expressing neurons.
conclusionWe show that VENs, together with GABRQ-expressing neurons, are selectively vulnerable in C9-bvFTD but are both spared in AD. This suggests they are related and that this GABRQ-expressing population of VENs and pyramidal neurons, is a key modulator of social-emotional functioning.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.