Long-Term Efficacy of Deep Brain Stimulation of Bilateral Globus Pallidus Internus in Primary Meige Syndrome

Abstract

<b><i>Objective:</i></b> Deep brain stimulation (DBS) of the internal segment of the globus pallidus (GPi) is an alternative therapy in ameliorating the clinical symptoms of primary Meige syndrome. Nevertheless, proof of its efficacy and safety is insufficient due to several case reports and small-sample clinical studies. This study aims to investigate postoperative long-term efficacy in patients undergoing DBS of the GPi for primary Meige syndrome. <b><i>Methods:</i></b> We performed a retrospective study to assess the efficacy and safety of bilateral GPi stimulation in 40 patients with primary Meige syndrome who responded poorly to medical treatments or botulinum toxin injections. All participants were postoperatively followed up at the outpatient clinic, and their motor functions were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). The severity of patients’ dystonia was evaluated before surgery and at follow-up neurostimu­lation. <b><i>Results:</i></b> The implanted stimulator was turned on 1 month after surgery. All 40 patients received monopolar stimulation using the following parameters: voltage 2.5–3.5 V (average: 2.6 ± 0.8 V), frequency 60–160 Hz (average: 88.0 ± 21.3 Hz), and pulse width 60–185 μS (average: 90.0 ± 21.1 μS). In 28 of 40 patients, the symptoms had signifi­cantly improved within 1 week of stimulation. Most of the patients had been followed up for 6–24 months (average: 15.0 ± 7.8 months). The clinical symptoms of all patients had significantly improved. At 6, 12, and 24 months after surgery, the BFMDRS subscores of eyes, mouth, speech, and swallowing were significantly lower, and subscores of mouth movement showed progressively decreased with prolonged stimulation time. The overall improvement rate was 83%. Five adverse events occurred in the 40 patients; all of these events resolved without permanent sequelae. <b><i>Conclusions:</i></b> Bilateral GPi-DBS demonstrated satisfactory long-term efficacy in the treatment of primary Meige syndrome and could serve as an effective and safe option.

Effect of Deep Brain Stimulation on Swallowing Function: A Systematic Review

“…Bilateral GPi DBS has been shown to improve the swallowing function in majority of the studies in patients with Meige syndrome, as demonstrated by improved Burke–Fahn–Masden Dystonia Rating Scale (BFMDRS) speech and swallowing scores in 12 patients who followed up to 38 months on average ( 29 ), in 11 patients who followed up for 23 months on average ( 30 ), in 6 patients who followed up to 60 months ( 31 ), and in 40 patients who followed up at 6, 12, and 24 months after surgery ( 32 ). There was one study by Limotai et al in six patients with Meige syndrome, with one unilateral and five bilateral GPi, evaluated 6 and 12 months after DSB for Unified Dystonia Rating Scale (UDRS) and BFMDR speech and swallowing function, but they did not find improvement in speech and swallowing function in this cohort ( 50 ).…”

“…Targeting GPi seemed to have positive results on Meige syndrome in the majority of the studies (29)(30)(31)(32). One of the possibilities behind the benefit is the direct effect on the pharyngeal and laryngeal dystonia by GPi, which could help to improve dysphagia symptoms.…”

“…DBS targeting the post-subthalamic area and caudal zona incerta (PSA/cZi) was thought to be associated with fewer side effects compared to ventralis intermedius (ViM) or STN (21), including the swallowing function (22)(23)(24). GPi DBS has also been used to treat various dystonia (25)(26)(27)(28), including Meige syndrome (29)(30)(31)(32), and its effect on the swallowing function is also of interest to review compared to that in PD.…”

Similar Long-Term Clinical Outcomes of Deep Brain Stimulation With Different Electrode Targets for Primary Meige Syndrome: One Institution’s Experience of 17 Cases

“…Since Houser et al reported that deep brain stimulation (DBS) could relieve the dystonia of 44-year-old women with MS in 2005 (4); increasing evidence of single-case and small-sample cohort studies has showed that DBS is an effective and safe method for primary MS (4,5). All of these studies chose the globus pallidus internus (Gpi) as the target for electrode implantation until subthalamic nucleus (STN)-DBS also was reported to be an effective treatment for patients with MS by Xiu et al in 2015 (6).…”

Metabolic Imaging of Deep Brain Stimulation in Meige Syndrome

“…Deep brain stimulation can be used to treat Meige syndrome by implanting stimulating electrodes into the basal ganglia nuclei and continuously releasing electrical signals through a pulse generator to inhibit abnormal discharge and regulate the basal ganglia motor circuits. The globus pallidus internus (GPi) is the most widely targeted region in deep brain stimulation in Meige syndrome ( Tian et al, 2019 ; Hao et al, 2020 ). Our previous study also demonstrated the safety and efficacy of subthalamic nucleus (STN) DBS ( Ouyang et al, 2021 ), revealing that patients with Meige syndrome had similar improvements in motor function, quality of life and sleep after either GPi-DBS or STN-DBS ( Liu et al, 2021a ).…”