Von economo neuron pathology in familial dysautonomia: Quantitative assessment and possible implications

6Citations
Citations of this article
32Readers
Mendeley users who have this article in their library.

This article is free to access.

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.

Cite

CITATION STYLE

APA

Jacot-Descombes, S., Keshav, N., Brosch, C. M. S., Wicinski, B., Warda, T., Norcliffe-Kaufmann, L., … Hof, P. R. (2020). Von economo neuron pathology in familial dysautonomia: Quantitative assessment and possible implications. Journal of Neuropathology and Experimental Neurology, 79(10), 1072–1083. https://doi.org/10.1093/JNEN/NLAA095

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Save time finding and organizing research with Mendeley

Sign up for free