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更新于：2025-05-07

Meige Syndrome

Meige综合征

更新于：2025-05-07

基本信息

别名

Blepharospasm - oromandibular dystonia、Blepharospasm Oromandibular Dyskinesia、Blepharospasm Oromandibular Dystonia

+ [46]

简介

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)

关联

项与 Meige综合征相关的药物

AbobotulinumtoxinA (Ipsen Biopharm Ltd)

A型肉毒杆菌毒素（Ipsen Biopharm Ltd）

靶点

SNAP25

作用机制

SNAP25 抑制剂

在研机构

原研机构

在研适应症

非在研适应症

最高研发阶段批准上市

首次获批国家/地区

英国 [+29]

首次获批日期2009-01-28

项与 Meige综合征相关的临床试验

NCT06912282

/ RecruitingN/AIIT

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.

开始日期2025-03-06

申办/合作机构

复旦大学附属华山医院

NCT06292559

/ RecruitingN/AIIT

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.

开始日期2023-11-30

申办/合作机构

山东大学齐鲁医院

ChiCTR2500097170

/ CompletedN/AIIT

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

开始日期2023-10-30

申办/合作机构

四川大学华西医院（四川省国际医院）

100 项与 Meige综合征相关的临床结果

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100 项与 Meige综合征相关的转化医学

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0 项与 Meige综合征相关的专利（医药）

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432

项与 Meige综合征相关的文献（医药）

2025-05-01·Neuroscience

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

Article

作者: 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.

2025-04-01·The Korean Journal of Pain

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

Letter

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

2025-04-01·Neuromodulation: Technology at the Neural Interface

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

Article

作者: 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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