Titre
Von Economo Neuron Pathology in Familial Dysautonomia: Quantitative Assessment and Possible Implications.
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Auteur(s)
Jacot-Descombes, S.
Auteure/Auteur
Keshav, N.
Auteure/Auteur
Brosch, CMS
Auteure/Auteur
Wicinski, B.
Auteure/Auteur
Warda, T.
Auteure/Auteur
Norcliffe-Kaufmann, L.
Auteure/Auteur
Kaufmann, H.
Auteure/Auteur
Varghese, M.
Auteure/Auteur
Hof, P.R.
Auteure/Auteur
Liens vers les unités
ISSN
1554-6578
Statut éditorial
Publié
Date de publication
2020-10-01
Volume
79
Numéro
10
Première page
1072
Dernière page/numéro d’article
1083
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Publication Status: ppublish
Résumé
Von Economo neurons (VENs) and fork cells are principally located in the anterior cingulate cortex (ACC) and the frontoinsular cortex (FI). Both of these regions integrate inputs from the autonomic nervous system (ANS) and are involved in decision-making and perception of the emotional states of self and others. Familial dysautonomia (FD) is an orphan disorder characterized by autonomic dysfunction and behavioral abnormalities including repetitive behavior and emotional rigidity, which are also seen in autism spectrum disorder. To understand a possible link between the ANS and the cortical regions implicated in emotion regulation we studied VENs and fork cells in an autonomic disorder. We determined the densities of VENs, fork cells, and pyramidal neurons and the ratio of VENs and fork cells to pyramidal neurons in ACC and FI in 4 FD patient and 6 matched control brains using a stereologic approach. We identified alterations in densities of VENs and pyramidal neurons and their distributions in the ACC and FI in FD brains. These data suggest that alterations in migration and numbers of VENs may be involved in FD pathophysiology thereby supporting the notion of a functional link between VENs, the ANS and the peripheral nervous system in general.
PID Serval
serval:BIB_FF8E3BC36FCE
PMID
Open Access
Oui
Date de création
2024-05-23T09:45:51.254Z
Date de création dans IRIS
2025-05-21T06:22:30Z