Globus pallidus internus activity increases during voluntary movement in children with dystonia

Estefania Hernandez-Martin; Maral Kasiri; Sumiko Abe; Jennifer MacLean; Joffre Olaya; Mark Liker; Jason Chu; Terence D Sanger

doi:10.1016/j.isci.2023.107066

Globus pallidus internus activity increases during voluntary movement in children with dystonia

iScience. 2023 Jun 7;26(7):107066. doi: 10.1016/j.isci.2023.107066. eCollection 2023 Jul 21.

Authors

Estefania Hernandez-Martin¹, Maral Kasiri², Sumiko Abe¹, Jennifer MacLean³, Joffre Olaya³, Mark Liker⁴, Jason Chu⁴, Terence D Sanger^{1

2

3}

Affiliations

¹ Department of Electrical Engineering and Computer Science, University of California, Irvine, Irvine, CA, USA.
² Department of Biomedical Engineering, University of California, Irvine, Irvine, CA, USA.
³ Department of Neurosurgery and Neurology, Children's Hospital of Orange County (CHOC), Orange, CA, USA.
⁴ Department of Neurosurgery, University of Southern California, Los Angeles, CA, USA.

Abstract

The rate model of basal ganglia function predicts that muscle activity in dystonia is due to disinhibition of thalamus resulting from decreased inhibitory input from pallidum. We seek to test this hypothesis in children with dyskinetic cerebral palsy undergoing evaluation for deep brain stimulation (DBS) to analyze movement-related activity in different brain regions. The results revealed prominent beta-band frequency peaks in the globus pallidus interna (GPi), ventral oralis anterior/posterior (VoaVop) subnuclei of the thalamus, and subthalamic nucleus (STN) during movement but not at rest. Connectivity analysis indicated stronger coupling between STN-VoaVop and STN-GPi compared to GPi-STN. These findings contradict the hypothesis of decreased thalamic inhibition in dystonia, suggesting that abnormal patterns of inhibition and disinhibition, rather than reduced GPi activity, contribute to the disorder. Additionally, the study implies that correcting abnormalities in GPi function may explain the effectiveness of DBS targeting the STN and GPi in treating dystonia.

Keywords: Neurology; Neuroscience; Pathophysiology.