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Bilateral pallidal deep brain stimulation in primary Meige syndrome

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Abstract

Primary Meige syndrome is an idiopathic movement disorder that manifests as craniofacial and often cervical dystonias. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as a powerful surgical option in the treatment of primary generalized or segmental dystonia. However, the experience with GPi-DBS in Meige syndrome is limited. We followed 5 patients with disabling Meige syndrome treated by bilateral GPi-DBS for 49 ± 43.7 (mean ± SD) months. All patients were assessed before surgery and at the last follow-up after surgery using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) which includes both the movement and disability scales. Bilateral GPi-DBS produced a sustained and long-lasting improvement in dystonia symptoms associated with Meige syndrome. At the last follow-up, the mean scores of BFMDRS movement and disability scales improved significantly by 84 ± 6.8% (range, 75–94%) and 89 ± 8.1% (range, 80–100%), respectively. Bilateral pallidal stimulation is a beneficial therapeutic option for long-term relief of the disabling dystonia symptoms in 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)

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The review of this paper was entirely handled by an Associate Editor, Eng-King Tan.

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