Von Economo Neuron Pathology in Familial Dysautonomia: Quantitative Assessment and Possible Implications.
Details
Serval ID
serval:BIB_FF8E3BC36FCE
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Von Economo Neuron Pathology in Familial Dysautonomia: Quantitative Assessment and Possible Implications.
Journal
Journal of neuropathology and experimental neurology
ISSN
1554-6578 (Electronic)
ISSN-L
0022-3069
Publication state
Published
Issued date
01/10/2020
Peer-reviewed
Oui
Volume
79
Number
10
Pages
1072-1083
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Publication Status: ppublish
Abstract
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.
Keywords
Adult, Aged, 80 and over, Dysautonomia, Familial/pathology, Female, Humans, Male, Middle Aged, Neocortex/pathology, Neurons/pathology, Anterior cingulate cortex, Autism spectrum disorder, Autonomic nervous system, Familial dysautonomia, Fork cells, Frontoinsular cortex, von Economo neurons
Pubmed
Web of science
Open Access
Yes
Create date
23/05/2024 10:45
Last modification date
24/05/2024 6:05