Subthalamic nucleus deep brain stimulation in primary Meige syndrome: motor and non-motor outcomes

Abstract

Background and purpose: Deep brain stimulation (DBS) has emerged as a promising treatment for movement disorders. This prospective study aims to evaluate the effects of bilateral subthalamic nucleus DBS (STN-DBS) on motor and non-motor symptoms in patients with primary Meige syndrome.

Methods: Thirty patients who underwent bilateral STN-DBS between April 2017 and June 2020 were included. Standardized and validated scales were utilized to assess the severity of dystonia, health-related quality of life, sleep, cognitive function and mental status at baseline and at 1 year and 3 years after neurostimulation.

Results: The Burke-Fahn-Marsden Dystonia Rating Scale movement scores showed a mean improvement of 63.0% and 66.8% at 1 year and 3 years, respectively, after neurostimulation. Similarly, the Burke-Fahn-Marsden Dystonia Rating Scale disability scores improved by 60.8% and 63.3% at the same time points. Postoperative quality of life demonstrated a significant and sustained improvement throughout the follow-up period. However, cognitive function, mental status, sleep quality and other neuropsychological functions did not change after 3 years of neurostimulation. Eight adverse events occurred in six patients, but no deaths or permanent sequelae were reported.

Conclusions: Bilateral STN-DBS is a safe and effective alternative treatment for primary Meige syndrome, leading to improvements in motor function and quality of life. Nevertheless, it did not yield significant amelioration in cognitive, mental, sleep status and other neuropsychological functions after 3 years of neurostimulation.

Keywords: Meige syndrome; deep brain stimulation; dystonia; non-motor symptoms; subthalamic nucleus.

FIGURE 1

Lead placement accuracy assessed by MRI‐CT fusion images. (a) Preoperative MRI‐CT fusion images showing the targeted region of the STN. (b) Microelectrode recording of high‐amplitude and high‐frequency discharges from STN neurons. (c) Postoperative CT scans to rule out intracranial hemorrhage. (d) MRI‐CT fusion images demonstrating that electrodes were located bilaterally in the STN. (e) Three‐dimensional reconstruction of implanted electrodes using Lead‐DBS software. MRI, magnetic resonance imaging; CT, computed tomography; STN, subthalamic nucleus; DBS, deep brain stimulation; GPi, globus pallidus internus; GPe, globus pallidus externus; RN, red nucleus.

FIGURE 2

BFMDRS scores at baseline and at 1 and 3 years after neurostimulation. (a) BFMDRS total scores at baseline and at 1 and 3 years after neurostimulation. (b) BFMDRS movement scores at baseline and at 1 and 3 years after neurostimulation. (c) BFMDRS disability scores at baseline and at 1 and 3 years after neurostimulation. BFMDRS, Burke−Fahn−Marsden Dystonia Rating Scale.