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Last update 08 May 2025

Meige Syndrome

Last update 08 May 2025

Basic Info

Synonyms

Blepharospasm - oromandibular dystonia, Blepharospasm Oromandibular Dyskinesia, Blepharospasm Oromandibular Dystonia

+ [46]

Introduction

A syndrome characterized by orofacial DYSTONIA; including BLEPHAROSPASM; forceful jaw opening; lip retraction; platysma muscle spasm; and tongue protrusion. It primarily affects older adults, with an incidence peak in the seventh decade of life. (From Adams et al., Principles of Neurology, 6th ed, p108)

Drugs associated with Meige Syndrome

AbobotulinumtoxinA (Ipsen Biopharm Ltd)

Target

SNAP25

Mechanism

SNAP25 inhibitors

Active Org.

Ipsen SA [+2]

Originator Org.

Ipsen Biopharm Ltd.

Active Indication

Facial Dermatoses [+8]

Inactive Indication

Low Back Pain [+9]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United Kingdom [+29]

First Approval Date28 Jan 2009

Clinical Trials associated with Meige Syndrome

NCT06912282

/ RecruitingNot ApplicableIIT

Evaluation of Bilateral Single-Electrode VO Combined With STN-DBS Stimulation in the Treatment of Meige Syndrome: A Prospective, Single-Center, Double-Blind, Randomized Controlled Exploratory Clinical Study

Meige syndrome is a rare and debilitating movement disorder characterized by blepharospasm, oromandibular dystonia, and cervical muscle contractions, often refractory to pharmacological therapies. Deep brain stimulation (DBS), initially developed to treat essential tremor and Parkinson's disease, has been explored in recent years as a potential treatment for various dystonias, including Meige's syndrome. This clinical study aims to evaluate the safety and efficacy of bilateral single-electrode ventralis oralis (VO) nucleus stimulation combined with subthalamic nucleus deep brain stimulation (STN-DBS) in patients with Meige syndrome.

Start Date06 Mar 2025

Sponsor / Collaborator

Shanghai Huashan Hospital

NCT06292559

/ RecruitingNot ApplicableIIT

A Randomized Prospective Study Between STN-DBS and GPi-DBS in Meige Syndrome

Deep-brain stimulation (DBS) is an effective treatment for patients with Meige syndrome. The globus pallidus interna (GPi) and the subthalamic nucleus (STN) are both accepted targets for this treatment. Researchers designed this study to compare the 12-month outcomes for patients who undergo bilateral stimulation of the GPi or STN.

Start Date30 Nov 2023

Sponsor / Collaborator

Qilu Hospital of Shandong University

ChiCTR2500097170

/ CompletedNot ApplicableIIT

Research on aided diagnosis technology for movement disorders based on multi-source data fusion

Start Date30 Oct 2023

Sponsor / Collaborator

West China Hospital

100 Clinical Results associated with Meige Syndrome

100 Translational Medicine associated with Meige Syndrome

0 Patents (Medical) associated with Meige Syndrome

432

Literatures (Medical) associated with Meige Syndrome

01 May 2025·Neuroscience

Progressive gray matter alterations in the Meige’s syndrome and across sub-types

Article

Author: Yao, Chen ; Xu, Jinping ; Wang, Lin ; Zhang, Haoran ; Cheng, Qinxiu ; Wang, Ning ; Meng, Dawei ; Shen, Hailiang ; Lin, Jiatai

Meige Syndrome (MS) is a form of segmental dystonia, categorized into four subtypes. The common and specific neuro-mechanisms among these subtypes remain to be elucidated. Herein, 3D T1-weighted MRI images were obtained from 159 patients with primary MS (31 with MS-I, 92 with MS-II, and 36 with MS-IV). Voxel-based morphometry (VBM), surface-based morphometry (SBM), and causal structural covariance network (CaSCN) were utilized to investigate morphological variations and causal relationships in structural changes across subtypes. The study revealed a decremental trend in gray matter volume (GMV) of the right precentral gyrus (PreCG.R), right calcarine cortex (CAL.R), left parietal association cortex (PAL.L), and left hippocampus (HIP.L) from MS-I to MS-IV, which negatively correlated with BFMDRS scores. The progression of GMV atrophy was followed by a trajectory from HIP.L to PAL.L with disease duration and from PAL.R to HIP.L/CAL.L/PreCG.R with increasing BFMDRS scores. Support vector machine (SVM) analysis indicated that these GMV changes might be served as potential biomarkers for diagnosing MS subtypes, with areas under the curve (AUC) of 0.935, 0.864, and 0.882, respectively. The results suggest that the PreCG.R is a key region affected early in MS, with GMV reductions extending to other brain areas as the disease progresses, indicating a hierarchy of structural brain changes associated with disease duration in MS progression. Our study further provides evidence for the association of MS with extensive gray matter abnormalities.

01 Apr 2025·The Korean Journal of Pain

CT-guided extracranial radiofrequency of multiple groups of cranial nerves for the treatment of compound Meige's syndrome

Letter

Author: Wang, Chunxiao ; Xin, Binyue ; Peng, Huiling ; Huang, Bing

01 Apr 2025·Neuromodulation: Technology at the Neural Interface

Long-term Efficacy of Bilateral Globus Pallidus Stimulation in the Treatment of Meige Syndrome

Article

Author: Zhang, Junmei ; Liu, Dingyang ; Yang, Zhuanyi ; Yang, Zhiquan ; Du, Liangchao ; Fu, Shiyu ; He, Xinghui

OBJECTIVEThis study aimed to investigate the long-term efficacy and prognosis of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with benign essential blepharospasm (BEB) and complete Meige syndrome, and to search for the best therapeutic subregion within the GPi.MATERIALS AND METHODSData were collected for 36 patients with Meige syndrome who underwent bilateral GPi-DBS surgery at our hospital between March 2014 and February 2022. Using the Burk-Fahn-Marsden Dystonia Rating Scale (BFMDRS)-Movement (BFMDRS-M) and BFMDRS-Disability (BFMDRS-D), the severity of the symptoms of patients with complete Meige syndrome was evaluated before surgery and at specific time points after surgery. Patients with BEB were clinically evaluated for the severity of blepharospasm using BFMDRS-M, the Blepharospasm Disability Index (BDI), and Jankovic Rating Scale (JRS). Three-dimensional reconstruction of the GPi-electrode was performed in some patients using the lead-DBS software, and the correlation between GPi subregion volume of tissue activated (VTA) and symptom improvement was analyzed in patients six months after surgery. The follow-up duration ranged from six to 99 months.RESULTSCompared with preoperative scores, the results of all patients at six months after surgery and final follow-up showed a significant decrease (p < 0.05) in the mean BFMDRS-M score. Among them, the average BFMDRS-M improvement rates in patients with BEB at six months after surgery and final follow-up were 60.3% and 69.7%, respectively, whereas those in patients with complete Meige syndrome were 54.5% and 58.3%, respectively. The average JRS and BDI scores of patients with BEB also decreased significantly (p < 0.05) at six months after surgery and at the final follow-up (JRS improvement: 38.6% and 49.1%, respectively; BDI improvement: 42.6% and 57.4%, respectively). We were unable to identify significantly correlated prognostic factors. There was a significant correlation between GPi occipital VTA and symptom improvement in patients at six months after surgery (r = 0.34, p = 0.025).CONCLUSIONSOur study suggests that bilateral GPi-DBS is an effective treatment for Meige syndrome, with no serious postoperative complications. The VTA in the GPi subregion may be related to the movement score improvement. In addition, further research is needed to predict patients with poor surgical outcomes.

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Meige Syndrome

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