Cited by 69Fetal and Pediatric Neurological DisordersNeural and Behavioral Psychology StudiesNeuroendocrine regulation and behavior

Selective reduction of Von Economo neuron number in agenesis of the corpus callosum

Kaufman, Jason A., Paul, Lynn K., Manaye, Kebreten F., Granstedt, Andrea E., Hof, Patrick R., Hakeem, Atiya Y., Allman, John M.·2008·Acta Neuropathologica

Abstract

Von Economo neurons (VENs) are large spindle-shaped neurons localized to anterior cingulate cortex (ACC) and fronto-insular cortex (FI). VENs appear late in development in humans, are a recent phylogenetic specialization, and are selectively destroyed in frontotemporal dementia, a disease which profoundly disrupts social functioning and self-awareness. Agenesis of the corpus callosum (AgCC) is a congenital disorder that can have significant effects on social and emotional behaviors, including alexithymia, difficulty intuiting the emotional states of others, and deficits in self- and social-awareness that can impair humor, comprehension of non-literal or affective language, and social judgment. To test the hypothesis that VEN number is selectively reduced in AgCC, we used stereology to obtain unbiased estimates of total neuron number and VEN number in postmortem brain specimens of four normal adult controls, two adults with isolated callosal dysgenesis, and one adult whose corpus callosum and ACC were severely atrophied due to a non-fatal cerebral arterial infarction. The partial agenesis case had approximately half as many VENs as did the four normal controls, both in ACC and FI. In the complete agenesis case the VENs were almost entirely absent. The percentage of neurons in FI that are VENs was reduced in callosal agenesis, but was actually slightly above normal in the stroke patient. These results indicate that the VEN population is selectively reduced in AgCC, but that the…

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Crossref · 10.1007/s00401-008-0434-7

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  • Deficit in emotional awareness (alexithymia) is commonly seen in conditions associated with neuropathological degeneration of VENs and functional deficits of AIC, such as bvFTD, callosal agenesis, and autism.

    “Deficit in emotional awareness, termed as alexithymia (Taylor, 2000), is commonly seen in conditions associated with neuropathological degeneration of the VENs and functional deficits of the AIC, such as behavioral variant frontotemporal dementia (Seeley et al., 2006; Seeley, 2010; Kim et al., 2012), callosal agenesis (Kaufman et al., 2008), and autism (Santos et al., 2011; Butti et al., 2013).”

    From Anterior insular cortex and emotional awareness (2013) · cites this paper as Kaufman et al., 2008

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Authors
Kaufman, J. A., Paul, L. K., Manaye, K. F., Granstedt, A. E., Hof, P. R., Hakeem, A. Y., & Allman, J. M.
Affiliations
  • California Institute of Technology (US)
  • Howard University (US)
  • Princeton University (US)
  • Icahn School of Medicine at Mount Sinai (US)
Corresponding author
Allman, John M.
Venue
Acta Neuropathologica
Published
2008-09-25
Volume
116
Issue
5
Pages
479-489
ISSN
  • 0001-6322 (ISSN-L)
  • 1432-0533
OpenAlex
W2105143748
Cited by
69
References
67
Citations by year
2026: 2 · 2025: 1 · 2023: 2 · 2022: 2 · 2021: 4 · 2020: 4 · 2019: 4 · 2018: 2 · 2017: 6 · 2016: 4 · 2015: 1 · 2014: 8 · 2013: 6 · 2012: 9
FWCI
4.21
Type
journal article
Language
en
Open access
closed
Locations
  • Acta Neuropathologica (publishedVersion)
  • PubMed (publishedVersion)
  • CaltechAUTHORS (California Institute of Technology) (submittedVersion)
Topics
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Fetal and Pediatric Neurological Disorders
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Neural and Behavioral Psychology Studies
  • Social Sciences › Psychology › Social Psychology › Neuroendocrine regulation and behavior
Keywords
von Economo neurons, agenesis of the corpus callosum, callosal dysgenesis, neuron number, anterior cingulate cortex, alexithymia, stereology, postmortem brain
MeSH terms
Aged, Aged, 80 and over, Cell Count, Cerebral Cortex, Corpus Callosum, Female, Humans, Functional Laterality, Male, Middle Aged, Neurons, Postmortem Changes, Agenesis of Corpus Callosum

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