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Successful treatment of the Meige's syndrome with navigated repetitive transcranial magnetic stimulation: A case report

Open AccessPublished:November 17, 2021DOI:https://doi.org/10.1016/j.brs.2021.11.013

      Keywords

      1. Introduction

      Meige's syndrome is a type of segmental cranial dystonia characterized by blepharospasm and oromandibular dystonia, often complicated with abnormal movement of maxillofacial and cervical muscles [
      • Pandey S.
      • Sharma S.
      Meige's syndrome: history, epidemiology, clinical features, pathogenesis and treatment.
      ]. Current treatments for Meige's syndrome include oral medications, botulinum toxin, and deep brain stimulation (DBS). However, these therapies were reported to either have unstable efficacy or cause unwanted side effects, or be invasive [
      • Pandey S.
      • Sharma S.
      Meige's syndrome: history, epidemiology, clinical features, pathogenesis and treatment.
      ]. Repetitive transcranial magnetic stimulation (rTMS) is a non-invasive brain stimulation method which has been successfully used in movement disorders such as Parkinson's disease [
      • Latorre A.
      • Rocchi L.
      • Berardelli A.
      • Bhatia K.P.
      • Rothwell J.C.
      The use of transcranial magnetic stimulation as a treatment for movement disorders: a critical review.
      ]. However, whether it can be used in Meige's syndrome remains unknown. Here, we report the successful treatment of Meige's syndrome with navigated rTMS in a patient with Meige's syndrome who is refractory to oral medication.

      2. Material and methods

      2.1 Patient

      The patient is a 57-year-old woman who had three-years-history of blepharospasm and oromandibular dystonia complicated with abnormal maxillofacial movement before her first navigated rTMS treatment. During the first six months, she initially presented with right eyelid convulsion especially when she felt nervous, which gradually evolved bilaterally. In the following six months, the patient developed spontaneous mouth movements, inarticulacy, cervical stiffness, and resultant dysphagia. During the first year, the patient had been treated with botulinum toxin and haloperidol which was withdrawn because of side effects. In the next two years before her first rTMS treatment, she received anti-dystonia and supportive treatments, and she was prescribed medications including haloperidol, benzhexol hydrochloride and simvastatin. However, the dysphagia of the patient deteriorated, and she developed cough from water drinking. The patient refused the history of hypertension, diabetes, infectious diseases, smoking, alcohol addiction, and allergies. Written informed consent has been obtained from the patient. This study was approved by the ethics committee of Xijing Hospital and was conducted according to the Declaration of Helsinki.

      2.2 Navigated rTMS treatment

      The patient was enrolled and her first navigated rTMS treatment was carried out at the Department of Neurology, Xijing Hospital on July 27, 2020, with a CCY-1 stimulator (Yiruide Medical Equipment Company, China) using a figure 8 coil, following the safety guidelines and protocols for rTMS [
      • Rossi S.
      • Hallett M.
      • Rossini P.M.
      • Pascual-Leone A.
      Safety, ethical considerations, and application guidelines for the use of transcranial magnetic stimulation in clinical practice and research.
      ]. Navigation was performed using a neuronavigational system (visor2, ANT Neuro, Enschede, Netherlands) to guide the magnetic stimulation as previously described [
      • Zhao C.G.
      • Sun W.
      • Ju F.
      • Wang H.
      • Sun X.L.
      • Mou X.
      • et al.
      Analgesic effects of directed repetitive transcranial magnetic stimulation in acute neuropathic pain after spinal cord injury.
      ]. Briefly, before the first rTMS session, T1-weighted magnetic resonance imaging of the patient was integrated into the navigation system and a three-dimensional reconstruction of the patients' brain was generated accordingly. A 3D-printed mask with ball-like tracking points (Black Dolphin Navigation Robot, Solide Brain Medical Technology, China) was attached to the patient's head. The position of the tracking points and the location and orientation of the coil were co-registered and visualized by an infrared camera system, which allowed the operator to place the coil over the target region precisely. The targeted region was the anterior central gyrus of the frontal lobe (Brodmann area 4) corresponding to facial and cervical muscles. During the treatment, patient was asked to lie comfortably on an examine chair in a quiet room. Active rTMS was delivered in trains of 15 pulse at 5Hz with intertrain intervals of 3 seconds (total number of 1500 pulses). The intensity of stimulation was set at 90% of the resting motor threshold (RMT) of the hand area. The RMT was determined before the first rTMS session as follows: After skin preparation, an electromyography (EMG) recording electrode (approximately 1cm2) was placed over the muscle belly of musculi abductor pollicis brevis. The hand area of contralateral primary motor cortex (M1) was stimulated and the RMT was defined as the lowest stimulation intensity which could elicit motor evoked potential ≥50 μV in at least 5 of 10 consecutive stimulations. In the first and second treatment session, active rTMS was delivered once a day at the same time of the day for consecutive ten days. From the third session, active rTMS was delivered twice a day 6–8 hours apart for consecutive five days. The interval between each treatment session was three to five days.

      3. Results

      The results of clinical examination of the patient on admission were summarized in Table 1. Before the first rTMS treatment, the patient was unable to communicate orally with the doctor due to oromandibular dystonia (Video 1). After the first rTMS session, the patients’ symptoms got significantly improved as she recovered understandable oral communication with her families. Her difficulty in swallowing got relieved. After three sessions of treatment, she was able to read clearly and fluently when asked to read a paragraph (Video 2). Currently, her treatment is still ongoing, and she can speak clearly and communicate with others. She also reports normal eating and swallowing without detectable adverse effects.
      Table 1An overview of clinical examinations of the patient.
      ExaminationFindings
      Physical examinationInarticulacy, involuntary movement of mouth and bilateral eyelids
      Neurological examinationNormal
      UrinalysisBacterial quantitation 733.10/μl, WBC +++, WBC quantitation 66.30/μl, triglyceride (TG) 2.04mmol/L
      Blood testsNormal
      Stool testsNormal
      Liver and kidney functionNormal
      Myocardial enzyme testsNormal
      Blood glucose concentrationNormal
      Copper and ceruloplasmin testsNormal
      MRIDemyelination changes in bilateral frontal subcortex and the right centrum semiovale
      MRACerebral arteriosclerosis
      Carotid artery ultrasoundBilateral arteriosclerosis in CCA and intima-media thickening of right subclavian artery and right CCA
      Abbreviations: MRI, magnetic resonance imaging; MRA, magnetic resonance angiography; CCA, common carotid artery.
      The following is/are the supplementary data related to this article:
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      4. Discussion

      TMS has been harnessed as a neurophysiological therapy in the treatment of several neurological or psychiatric conditions, as well as movement disorders including Parkinson's disease, Tourette syndrome, Huntington's disease, and essential tremor [
      • Latorre A.
      • Rocchi L.
      • Berardelli A.
      • Bhatia K.P.
      • Rothwell J.C.
      The use of transcranial magnetic stimulation as a treatment for movement disorders: a critical review.
      ,
      • Valero-Cabre A.
      • Amengual J.L.
      • Stengel C.
      • Pascual-Leone A.
      • Coubard O.A.
      Transcranial magnetic stimulation in basic and clinical neuroscience: a comprehensive review of fundamental principles and novel insights.
      ]. In our case, oromandibular dystonia was succesfully treated with rTMS targeting the Brodmann area 4 corresponding to facial and cervical muscles. The therapeutic effect could be attributed to changes in synaptic plasity and neurotransmission induced by TMS. Previous studies reported that rTMS provided cumulative effects depending on the number of sessions [
      • Schulze L.
      • Feffer K.
      • Lozano C.
      • Giacobbe P.
      • Daskalakis Z.J.
      • Blumberger D.M.
      • et al.
      Number of pulses or number of sessions? An open-label study of trajectories of improvement for once-vs. twice-daily dorsomedial prefrontal rTMS in major depression.
      ]. Our case corresponds to this as suggested by the improvement in patients' symptom alongside the accumulation of treatment sessions. Moreover, in our case, the navigated rTMS is a non-invasive method with significant efficacy. This non-invasive method bear the potential to get over the invasive nature of DBS surgery in the treatment of Meige's syndrome, and to bypass the refractoriness or side effects of oral medication.

      5. Conclusions

      In conclusion, our case suggested that rTMS could be an effective and convenient noninvasive neurophysiological treatment for patients with Meige's syndrome who repond poorly to pharmacological medication. Future larger clinical studies are warrented to hopefully verify the efficacy and safety of rTMS in the treatment of Meige's syndrome, as well as to optimize the parameters to gain better efficacy.

      Funding source

      This work was supported by Xijing Hospital Discipline Boosting Program [grant number XJZT19Z24 ]. The funding source had no involvement in the study design, in the collection, analysis, and interpretation of data, in the writing of the report, or in the submission of the manuscript.

      Author contributions

      Chang-Geng Song: Conceptualizaion, Methodology, Formal analysis, Writing- Original Draft, Writing - Review & Editing.
      Xiao-Jing Shi: Data Curation, Formal analysis, Writing - Review & Editing.
      Bin Jiang: Data Curation, Formal analysis, Writing - Review & Editing.
      Rui Shi: Data Curation, Formal analysis, Writing - Review & Editing.
      Xin Guo: Data Curation, Formal analysis, Writing - Review & Editing.
      Shun Qi: Supervision, Formal analysis, Writing - Review & Editing.
      Li Li: Conceptualizaion, Supervision, Formal analysis, Funding acquisition, Writing - Review & Editing.
      All the authors have approved the manuscript to be submitted.

      Declaration of competing interest

      The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

      Acknowledgements

      We specially thank Dr. Yang Rao from Solide Brain Medical Technology, Ltd. and Brain Modulation and Scientific Research Center, China, for his assitance in the editing of this manuscript.

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