Characteristics of Globus Pallidus Internus Local Field Potentials in Hyperkinetic Disease

Abstract

Background: Dystonia and Huntington's disease (HD) are both hyperkinetic movement disorders but exhibit distinct clinical characteristics. Aberrant output from the globus pallidus internus (GPi) is involved in the pathophysiology of both HD and dystonia, and deep brain stimulation (DBS) of the GPi shows good clinical efficacy in both disorders. The electrode externalized period provides an opportunity to record local field potentials (LFPs) from the GPi to examine if activity patterns differ between hyperkinetic disorders and are associated with specific clinical characteristics.Methods: LFPs were recorded from 7 chorea-dominant HD and nine cervical dystonia patients. Differences in oscillatory activities were compared by power spectrum and Lempel-Ziv complexity (LZC). The discrepancy band power ratio was used to control for the influence of absolute power differences between groups. We further identified discrepant frequency bands and frequency band ratios for each subject and examined the correlations with clinical scores.Results: Dystonia patients exhibited greater low frequency power (6–14 Hz) while HD patients demonstrated greater high-beta and low-gamma power (26–43 Hz) (p < 0.0298, corrected). United Huntington Disease Rating Scale chorea sub-score was positively correlated with 26–43 Hz frequency band power and negatively correlated with the 6–14 Hz/26–43 Hz band power ratio.Conclusion: Dystonia and HD are characterized by distinct oscillatory activity patterns, which may relate to distinct clinical characteristics. Specifically, chorea may be related to elevated high-beta and low-gamma band power, while dystonia may be related to elevated low frequency band power. These LFPs may be useful biomarkers for adaptive DBS to treat hyperkinetic diseases.

Cortico-subthalamic Coherence in a Patient With Dystonia Induced by Chorea-Acanthocytosis: A Case Report

“…The left STN did show increased high beta power compared to the right STN but that was largely above the frequency range where beta power is increased in PD patients. High-beta power increase in the GPi was recently linked to hyperkinetic symptoms in Huntington’s disease (Zhu et al, 2018) so beta activity could also be related to the patient’s choreatic mouth movements. In order to determine whether and how high beta power increase is related to disease symptoms, observations in more patients are necessary.…”

Theta/Alpha Band Suppression and Clinical Outcomes During Globus Pallidus Internus Deep Brain Stimulation in Huntington's Disease

“…[1][2][3] However, unlike other movement disorders, there is limited knowledge about the profile of frequency bands that predominate in the GPi in HD and about the effect of DBS on these frequency bands and on related clinical outcomes. 4 In previous studies of HD, local field potentials (LFPs) of the GPi recorded during OFF stimulation (DBS-OFF) showed a dominant theta/alpha activity similar to that observed in other hyperkinetic disorders. 5,6 In the present study, taking advantage of the capabilities of the new Medtronic PERCEPT implantable pulse generator (Medtronic, Minneapolis, MN), 7 we obtained LFPs of the GPi during DBS-OFF and DBS-ON in a gene-mutation carrier (CAG = 43), 43-year-old woman with symptomatic HD, characterized by pharmacologically uncontrollable choreatic movements in both the upper and lower extremities, dystonia, constant right-arm complex motor stereotypes, and vocalizations (Unified Huntington's Disease Rating Scale [UHDRS] total motor score = 82).…”

Cerebellar gray matter alterations predict deep brain stimulation outcomes in Meige syndrome

“…As for the diagnosis of dystonia, the GM morphological network properties ( Li et al, 2021 ), brain resting-state FC ( Battistella et al, 2016 , Pan et al, 2021 , Pan et al, 2019 ), local field potentials in the GPi ( Zhu et al, 2018 ) have been recognized as latent biomarkers to distinguish dystonic patients from HCs with satisfactory accuracy and sensitivity. Notably, a study compared the performance of anatomical, diffusional, and functional biomarkers, concluding that GM volume outperformed functional metrics and contributed more to the disease classification ( Schouten et al, 2016 ).…”