Short communicationBilateral pallidal deep brain stimulation in primary Meige syndrome☆
Introduction
Primary Meige syndrome is an idiopathic dystonia that involves craniofacial and often cervical muscles. This adult-onset movement disorder manifests as blepharospasm and oromandibular dystonia, but dystonia may also occur in the upper extremities, trunk, and neck [1], [2]. Meige syndrome can be disabling despite the best medical therapy. Botulinum toxin injections constitute the standard treatment for Meige syndrome, but its effectiveness often diminishes over time. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as a powerful surgical option in the treatment of primary generalized and segmental dystonias [3], and interest in the use of GPi-DBS for refractory dystonia symptoms in Meige syndrome is increasing [4], [5], [6], [7]. However, the beneficial effects of GPi-DBS in patients with Meige syndrome remain to be established, because the data currently available is based on a small series of patients with short-term follow-up. To further elucidate the therapeutic efficacy of pallidal stimulation, we assessed surgical outcome in 5 patients suffering from disabling Meige syndrome who underwent bilateral GPi-DBS.
Section snippets
Subjects
The clinical characteristics of the patients included in this study are summarized in Table 1. None of the patients had a family history of dystonia or prior exposure to neuroleptics, and their preoperative brain magnetic resonance images appeared normal. Before surgery, written informed consents were obtained from all patients and their families. At the time of surgery, the mean age of the patients was 65 ± 7.2 (mean ± SD) years (range, 54–72 years) and the mean disease duration was 12 ± 4.2
Stimulation settings
For all patients, optimal results were obtained at the final stimulator settings with the mean amplitude of 2.6 ± 1.1 V (range, 1.0–3.9 V), mean frequency of 84 ± 27.2 Hz (range, 60–130 Hz), and pulse width of 392 ± 98.1 μs (range, 210–450 μs) (see Table 1). We applied a continuous monopolar mode using 1 or 2 active contacts in all patients except patient 3, for whom a bipolar mode with contacts 1 (cathode) and 3 (anode) was used.
Assessment with BFMDRS
As shown in Table 1, the mean follow-up period was 49 ± 43.7
Discussion
Clinical studies in patients with primary generalized or segmental dystonia have shown the beneficial effects of bilateral GPi-DBS for both motor symptoms and disability caused by dystonia [3]. However, experience with GPi-DBS in other forms of dystonia such as Meige syndrome is limited. Moreover, long-term outcome of patients with Meige syndrome treated with GPi-DBS remain to be elucidated. In this study, we showed that bilateral pallidal stimulation produced a long-lasting suppression of
Acknowledgements
This work was supported by grant from the Ministry of Education, Culture, Sports, Science and Technology of Japan (grant-in-aid for Scientific Research, 20591025).
References (12)
- et al.
Meige syndrome: primary and secondary forms
Adv Neurol
(1988) Meige syndrome: what’s in a name?
Parkinsonism Relat Disord
(2009)Treatment of hyperkinetic movement disorders
Lancet Neurol
(2009)- et al.
Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy
Mov Disord
(2001) - et al.
Meige syndrome and pallidal deep brain stimulation
Mov Disord
(2005) - et al.
Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome)
Mov Disord
(2007)
Cited by (59)
Subthalamic Nucleus Deep Brain Stimulation in Primary Meige Syndrome: A 1-Year Follow-Up Study
2021, NeuromodulationCitation Excerpt :The globus pallidus internus (GPi) has been shown to be an effective target of DBS for the relief of Meige syndrome symptoms. It has also been reported that subthalamic nucleus (STN)-DBS is an effective treatment for primary dystonia patients (10–13), making the STN a promising alternative target for dystonia treatment. However, relatively few reports have substantiated these findings, and most are limited to small-scale studies.
Principles and Practice of Movement Disorders
2021, Principles and Practice of Movement DisordersThe psychopharmacology of catatonia, neuroleptic malignant syndrome, akathisia, tardive dyskinesia, and dystonia
2019, Handbook of Clinical NeurologyPost-operative electrode location and clinical efficacy of subthalamic nucleus deep brain stimulation in Meige syndrome
2019, Parkinsonism and Related DisordersThe impact of deep brain stimulation on health related quality of life and disease-specific disability in Meige Syndrome (MS)
2018, Clinical Neurology and NeurosurgeryCitation Excerpt :Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is indicated in medically refractory generalized dystonias and there is growing evidence of its potential benefits in segmental dystonia, including MS [8–30]. However, few reports assess the impact of DBS on disease-specific disability and health-related quality of life (HRQoL) [16,17,25–27,29,31]. Therefore, our main aim is to report our experience with bilateral GPi-DBS in two cases of medically refractory MS with emphasis on the effects on disability and HRQoL.
- ☆
The review of this paper was entirely handled by an Associate Editor, Eng-King Tan.