RARE DISEASE
Blepharospasm-oromandibular dystonia syndrome
Blepharospasm-oromandibular dystonia syndrome
Blepharospasm-oromandibular dystonia syndrome
Synonyms: Meige dystonia, Meige syndrome
Synonyms: Meige dystonia, Meige syndrome
Synonyms: Meige dystonia, Meige syndrome
Drug discovery
2
drugs
With orphan designations
Overview
Blepharospasm-oromandibular dystonia syndrome (BOS), also termed Meige syndrome, is an adult-onset focal dystonia characterized by involuntary contractions of orbicularis oculi (blepharospasm) and jaw/tongue muscles (oromandibular dystonia) [1][5][16]. Primarily idiopathic, it arises from basal ganglia dysfunction, with symptoms exacerbated by stress, light, or movement [6][9]. Diagnosis is clinical, and management focuses on symptom control [5][7].
Burden
Causes functional blindness, dysphagia, and speech impairment, severely impacting daily activities [4][6].
Associated with anxiety/depression in 30–60% of patients due to social stigma and chronic disability [6][9].
Requires lifelong multidisciplinary care, with 90% of patients reliant on repeat botulinum toxin injections [5][7].
Therapies
First-line: Botulinum toxin injections (e.g., onabotulinumtoxinA) targeting orbicularis oculi, masseter, and temporalis muscles [5][7][11].
Adjuncts: Anticholinergics (trihexyphenidyl), benzodiazepines (clonazepam), or gabapentinoids [7][19].
Refractory cases: Surgical myectomy or deep brain stimulation of the globus pallidus interna [7][11].
Categories: rare genetic diseases, rare neurological diseases
Research Papers
372 drug discovery papers about Blepharospasm-oromandibular dystonia syndrome, with 1 first-in-class and 2 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
372 drug discovery papers about Blepharospasm-oromandibular dystonia syndrome, with 1 first-in-class and 2 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-08-14 | Use of explainable cluster analysis to identify distinct subtypes of Meige syndrome patients and associated biomarker profiles.
Meige syndrome (MS), a complex form of segmental craniocervical dystonia, currently lacks objective diagnostic biomarkers.This study aims to identify biological biomarkers that can stratify patients with MS and to reveal the complex pathophysiological heterogeneity of this disorder, with particular attention to the peripheral immune-metabolic profiles that may reflect underlying neuroimmune dysfunction. We retrospectively collected clinical and laboratory data from 1,782 patients with MS during their first hospitalization at the MS Center of The Third People's Hospital of Henan Province between 2023 and 2025. A total of 66 clinical and laboratory indicators were included. An interpretable clustering analysis framework was applied to perform a series of pipeline analyses, including imputation, dimensionality reduction, outlier removal, and clustering. Next, feature importance was assessed. A multi-class classification model was then constructed and evaluated using XGBoost. Finally, the Shapley Additive exPlanations(SHAP) algorithm was employed to interpret the contribution of each feature. The preprocessing pipeline effectively imputed missing values (ranging from 0.06% to 14.14%) and removed outliers. Unsupervised clustering analysis revealed three heterogeneous subtypes of MS. A total of 23 important features were selected to construct a multi-class XGBoost classification model. The model demonstrated excellent performance, with a macro-average AUC of 0.9790 and an Obuchowski index of 0.9784. The SHAP analysis further elucidated the unique hematological and biochemical metabolic characteristics of each subtype. This study establishes a novel, data-driven subtyping system for MS based on objective blood and metabolic profiles. The three identified subtypes display distinct immune-metabolic signatures: a hypercoagulable-inflammatory profile, a metabolic-excess profile with impaired vascular protection, and a frailty-associated antioxidant-deficient profile. These peripheral biomarker patterns suggest testable hypotheses regarding neuroimmune mechanisms-including fibrinogen-driven microglial activation, oxidized lipid-mediated blood-brain barrier disruption, and uric acid-dependent antioxidant depletion-that may contribute to the pathophysiology of different MS subtypes. This framework may inform future prospective investigations into precision medicine approaches for MS, though substantial validation is required before clinical translation.
2026-07-28 | A Case of Meige Syndrome in a Pediatric Patient With Schizophrenia.
Long-term treatment with multiple antipsychotic medications for schizophrenia may increase the risk of developing tardive dystonia, including Meige syndrome. Although clozapine has been suggested as a way of improving dystonia symptoms, there is no established consensus regarding its use in pediatric patients with treatment-resistant schizophrenia, and careful consideration is required. A 12-year-old boy with schizophrenia experienced blepharospasm and oromandibular twitching while undergoing treatment with multiple antipsychotic medications. We stopped giving him the same medications that had been used during his previous hospitalizations (risperidone, olanzapine, aripiprazole, lurasidone, and paliperidone, paliperidone palmitate (a long-acting injectable), asenapine, and blonanserin). We then started giving him clozapine, gradually increasing the dose to 125 mg. Following this treatment intervention, the dystonic symptoms resolved completely, and the psychiatric symptoms stabilized.
2026-01-01 | Advances in the treatment of Meige syndrome
Meige syndrome is a form of segmental craniofacial dystonia characterized predominantly by blepharospasm and frequently accompanied by oromandibular and lower facial dystonia. It is associated with substantial symptom burden, considerable clinical heterogeneity, and persistent challenges in long-term management, including marked variability in treatment response, fragmented evidence, and the absence of standardized stratified therapeutic approaches. Against this background, this Review aims to provide a comprehensive and critical update on current therapeutic advances in Meige syndrome. First, we focus on the major established treatment modalities, including botulinum toxin therapy—with particular attention to formulation selection, dose optimization, injection strategies, safety, and efficacy assessment—systemic pharmacotherapy, including its appropriate clinical indications, potential benefits, and inherent limitations, and deep brain stimulation (DBS) for refractory cases, with emphasis on clinical efficacy, safety, target selection, and parameter optimization. In parallel, we discuss how objective tools such as neuroimaging and electrophysiological techniques may help clarify the associations between specific therapeutic strategies and disease subtypes, symptom profiles, and clinical outcomes. Second, we summarize the available evidence for adjunctive or alternative interventions, including acupuncture, radiofrequency-based approaches, and transcranial magnetic stimulation, in order to present a more complete picture of the therapeutic landscape in Meige syndrome. Finally, we highlight the translational potential of neuromodulatory strategies in this disorder, particularly their prospective role in improving refractory symptoms, refining individualized treatment pathways, and advancing precision-based management. By integrating evidence across these domains, we aim to provide a clearer framework for clinical decision-making in Meige syndrome, while identifying key gaps in the current literature and informing future efforts toward standardized outcome measures, prospective study design, and the development of novel neuromodulatory therapies.
2025-11-10 | Comparative efficacy and safety of GPi-DBS versus STN-DBS in Meige syndrome: a retrospective propensity score-matched cohort study.
Although deep brain stimulation (DBS) targeting the globus pallidus internus (GPi) or the subthalamic nucleus (STN) has shown efficacy for Meige syndrome (MS), there is limited comparative evidence available. This retrospective cohort study consecutively enrolled patients with primary MS who underwent bilateral GPi-DBS or STN-DBS at our center between March 2015 and February 2025. Propensity score matching (PSM, 1.5:1) was employed to balance baseline covariates. The primary outcomes were improvements in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore (BFMDRS-M) at 3-month (short-term) and at the final follow-up (long-term). Additionally, stimulation parameters, programming adjustments, and adverse events (AEs) were analyzed. The study cohort comprised 254 eligible patients (GPi-DBS: 176; STN-DBS: 78), with 14 patients (4.8%) lost to follow-up. The overall cohort consisted of 178 women (70.1%) with a mean surgical age of 57.7 ± 8.6 years. After PSM, 157 patients were matched (89 GPi-DBS, 68 STN-DBS). At 3-month follow up, the mean improvement rate of BFMDRS-M total score was 51.0% [95% confidence interval (CI): 46.7-55.3%] for GPi-DBS and 54.6% (95% CI: 49.3-59.8%) for STN-DBS, with no significant intergroup difference (P = 0.235). After a mean follow-up of 42.4 ± 23.6 months, the mean improvement was 61.0% (95% CI: 56.4-65.5%) for GPi-DBS and 65.2% (95% CI: 59.2-71.1%) for STN-DBS, again indicating no significant difference ( P = 0.192). STN-DBS required lower stimulation parameters but significantly more programming adjustments than GPi-DBS (mean sessions: 5.49 vs 4.33; P < 0.001). AE incidence was similar between groups (GPi-DBS vs STN-DBS: 5.4% vs 3.6%; P = 0.731). Both GPi-DBS and STN-DBS provided significant and sustained motor symptom improvement in refractory MS, with no statistically significant difference between groups.
2025-09-15 | A Case Report on the Management of Meige Syndrome
Meige syndrome is a rare neurological movement disorder characterized by involuntary, forceful and often painful contractions of the facial muscles. It typically presents as a combination of blepharospasm and oromandibular dystonia. This case report discusses a 53-year-old female patient, diagnosed with Meige syndrome, who presented to the Outpatient Department of Government Ayurveda Medical College, Thiruvananthapuram, with complaints of pain over the temporomandibular joint, repetitive jaw movements and involuntary, recurrent closure of both eyelids for the past 5 years. The treatment principle adopted in this case was based on Ardhita Chikitsa. She was given internal medications such as Indukantham ghritam, Dhanadanayanadi Kashayam, Dhanwantaram 101A, Yogaraja guggulu tablets, along with external procedures like Thalam, Jaloukavacharana, Nasya, Ksheeradhooma, Shirodhara, Gandusham, Shashtika Pinda Swedam and Agnikarma. The treatment duration was one month, with follow-ups continued for three months and changes were assessed at the third follow-up. Follow-up medicines were Murdhini Tailam and mukhabhyangam with Balatailam, Pratimarsha Nasyam with Dhanwantaram 41A and Tablet Yogaraja Guggulu as internal medicine. The changes were assessed after the third follow-up using the Jankovic Rating Scale (JRS) and the Oromandibular Dystonia Rating Scale (OMDRS). This management approach suggests that Ayurvedic interventions may serve as a promising and effective therapeutic modality in the management of Meige syndrome.
proteins
2026-05-25 | Intervention effects of different brands of botulinum toxin type A on Meige syndrome with anxiety symptoms.
Patients with Meige syndrome are often affected by anxiety symptoms, and drug therapy is the primary treatment option, with botulinum toxin A being the most frequently used medication. This study analyzes the intervention effects of different brands of botulinum toxin A on Meige syndrome with anxiety symptoms. The findings aim to provide a theoretical basis for the rational selection of clinical treatment options. A retrospective analysis was conducted on 148 patients treated between January 2021 and December 2023. Patients were divided into two groups based on the BoNT-A brand received: Chinese botulinum toxin type A (Hengli) group (n = 80) and onabotulinumtoxinA (Botox) group (n = 68). Both groups received local injections at the sites of muscle spasms. The outcomes included the degree of spasm relief, duration of efficacy, scores on the Self-Rating Anxiety Scale (SAS) and Self-Rating Depression Scale (SDS), polysomnography parameters [sleep efficiency (SE), total sleep time (TST), sleep latency (SL), number of wakefulness (AI), rapid eye movement sleep time (REMS)], and adverse reactions. There were no statistically significant differences in the total remission rate and efficacy maintenance time between the two groups (p > 0.05). The differences in the scores of the SAS, SDS, SE, TST, SL, AI, and REMS before and after treatment between the two groups were not statistically significant (p > 0.05). There was no statistically significant difference between the two groups when comparing the incidence of adverse reactions such as incomplete eyelid closure, facial muscle weakness, and drooping of the corners of the mouth during expression muscle activity (p > 0.05). Local injection of botulinum toxin type A is a safe and effective treatment for Meige syndrome with anxiety symptoms, and there is no significant difference in the efficacy, duration of action, and improvements in psychological state and sleep quality between different brands of botulinum toxin type A. Therefore, clinical practice should be based on practical considerations to reasonably select the appropriate brand of botulinum toxin type A for treatment.
2026-01-28 | Geometric Microstructural Characteristics of White Matter Differentiate Patients With Facial Dyskinesias and Palsy.
The heterogeneous and homogeneous clinical manifestations of peripheral facial palsy (FP), hemifacial spasm (HFS), and Meige's syndrome (MS) complicate the differentiation of diagnoses for these facial motor diseases. To comprehensively investigate the white matter microstructural characteristics in patients with facial dyskinesias and palsy using geometric and integrity metrics in DTI. In this prospective study conducted from September 2020 to January 2022, patients with FP, HFS, and MS, as well as sex-matched healthy control subjects, underwent 3.0 T MRI. Geometric metrics (i.e., splay, bend, twist, and total distortion) based on "Director Field Analysis" and fractional anisotropy (FA) and mean diffusivity (MD) were calculated from DTI data. Cross-sectional tract-based spatial statistics were performed among FP, HFS, MS patients, and healthy controls. The correlation between disease severity and DTI metrics was evaluated. Additionally, the geometric microstructural properties combining FA and MD were used to classify FP, HFS, and MS patients using machine learning methods. Geometric metrics and FA/MD were widely altered across white matter in FP and HFS patients compared with healthy controls. However, in MS patients only DFA metrics were significantly altered. FA and DFA values strongly correlated with the severity of facial movement disorder in FP patients. Combing conventional FA/MD value with DFA metrics enabled the diagnostic differentiation of FP and HFS from MS. Our findings demonstrated that the geometric microstructural information of white matter fibers could provide novel insight into the underlying pathological changes in facial dyskinesias and palsy.
2025-03-10 | Experience of the application of botulinum toxin type A as a treatment of facial hyperkinetic disorders in a Mexican ophthalmological center.
Blepharospasm is characterized by involuntary, sustained spasms of the orbicularis oculi, corrugator, and procerus muscles. Hemifacial spasm (HFS) is characterized by the involuntary tonic-clonic contraction of the muscles of a hemiface. The main role of botulinum toxin type A (BoNT-A) in the treatment of blepharospasm and HFS is recognized by guidelines around the world. In this study, we evaluated the applicability of botulinum toxin type A in patients with periocular hyperkinetic disorders at the Instituto de Oftalmología Fundación Conde de Valenciana, so that we can share the Mexican experience with the international evidence. The clinical records of patients diagnosed with facial hyperkinetic disorders treated with botulinum toxin type A (BoNT-A) that were treated from January 2015 to September 2021 were analyzed. A total of 140 records of patients diagnosed with facial hyperkinetic disorder and treated with BoNT-A were analyzed. Facial hyperkinetic disorders that occurred included 5 (3.6%) patients with orbicularis oculi myokymia, 72 (51.4%) with blepharospasm, 62 (44.3%) with hemifacial spasm, and 1 (0.7%) with Meige syndrome. One hundred and twenty patients (85.7%) reported remission of initial symptoms after BoNT-A therapy during the period covered by this study. Botulinum toxin type A is effective and safe as a treatment for facial hyperkinetic disorders in the Mexican population.
2024-04-01 | Effects of onabotulinum toxin type A injections in patients with Meige's syndrome
Abstract Background Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type A (OBTA) injections are the standard treatment, evidence of their effectiveness and safety in this scenario is still lacking. Objective Our research aimed to evaluate the improvement and occurrence of side effects following injections of onabotulinum toxin type A (OBTA) in patients with Meige's syndrome. Methods Patients with Meige's syndrome undergoing botulinum toxin injections were enrolled in this study. We assessed dystonia intensity before and 14 days after OBTA injection using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) to measure the response of symptoms in the eyes (blepharospasm) and mouth (oromandibular dystonia). Other variables, such as dosage, side effects, and demographic data, were also recorded. Results The study included 41 participants, with a mean age of 67.7 years and a female-to-male ratio of 3.5:1. The mean BFMDRS score before the injections was 8.89, and after 14 days, it was 2.88. The most reported side effect was ptosis, with a 7.3% incidence. OBTA significantly reduced dystonia severity (p < 0.0001). The clinical response for the blepharospasm component was superior to the oromandibular dystonia component. Conclusion Our results support that OBTA seems to be an effective and safe therapeutic option for treating Meige's syndrome. The effect of OBTA was more pronounced in the treatment of blepharospasm than in oromandibular dystonia.
2023-02-03 | Mechanism and clinical use of botulinum neurotoxin in head and facial region
Botulinum neurotoxin (BoNT) is a biological toxin produced by Clostridium botulinum. BoNT is a potent toxin extensively used in therapeutic interventions. This review provides an updated overview of the mechanisms of action and clinical applications of BoNT in head and facial region.MEDLINE/PubMed searches were conducted using the terms "botulinum neurotoxin" and "dentistry" along with a combination of other related terms. In addition, studies were manually selected from reference lists of the selected articles.The Food and Drug Administration in the United States initially approved BoNT to treat strabismus, blepharospasm, and hemifacial spasms. The use of BoNT in dermatology and cosmetics has been widely established and has created a revolution in these fields. Over the years, its applications in various medical specialties have expanded widely. Owing to its safety, efficacy, and long duration of action, it is well-accepted by patients. BoNT/A and BoNT/B are widely used in clinical practice. Several off-label uses of BoNT in the dental fraternity have yielded promising results. We have elaborated on the speculated mechanism of action, dosage, effective sites of injection, and adverse effects of each therapeutic application. The various clinical indications for BoNT include bruxism, myofascial pain, temporomandibular joint dislocation, hemifacial pain, orofacial dystonia, facial paralysis, chronic migraine, and trigeminal neuralgia.BoNT is a safe treatment that can be used effectively, provided that the clinician has adequate knowledge regarding the mechanism, injection techniques, and local and systemic side effects and that it is administered cautiously and purposefully.
cell therapies
2026-05-01 | Subthalamic nucleus deep brain stimulation in Meige syndrome: mapping the optimal stimulation sites and network targets.
The aim of this study was to identify the optimal stimulation sites for subthalamic nucleus (STN) deep brain stimulation (DBS) in treating Meige syndrome using long-term follow-up data from a large sample cohort, evaluate the whole-brain functional connectivity patterns associated with favorable treatment responses, and validate these findings in an independent cohort. The authors retrospectively analyzed long-term outcomes in 65 patients with Meige syndrome who underwent bilateral STN-DBS in two centers. The local stimulation effects within the STN and the distributed functional connectivity associated with motor improvement were investigated using advanced imaging and modeling tools, including the Lead-Group Toolbox, DBS Sweet Spot Mapping Explorers, and DBS Network Mapping Explorers. To ensure the model's reliability and generalizability, both internal validation through multiple cross-validation strategies and external validation using independent cohorts were conducted. STN-DBS yielded significant and sustained motor improvements in both cohorts, with mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score reductions of 63% in the training cohort (n = 50) and 56% in the validation cohort (n = 15) (p < 0.001). At the local level, the optimal stimulation sites were consistently located in the dorsolateral sensorimotor subregion of the STN, extending bilaterally toward the associative subregion and centered at MNI coordinates x = ±12, y = -13, z = -6. At the network level, favorable outcomes were primarily associated with positive functional connectivity to the cerebellum and negative connectivity to the somatosensory cortex. Both the sweet spot and connectivity models developed using the training cohort showed significant correlations with clinical outcomes in the independent validation cohort (R = 0.59, p = 0.020; R = 0.74, p = 0.002, respectively) and remained robust across different cross-validation strategies. The optimal therapeutic efficacy of STN-DBS for Meige syndrome depends on precise targeting within the dorsolateral STN and modulation of a distributed functional network involving the cerebellum and sensorimotor cortex. These findings may aid in developing personalized targeting strategies and adaptive programming paradigms, ultimately improving the therapeutic efficacy of DBS in this challenging disorder.
2026-02-09 | Case Report: Effective management of a Meige syndrome patient with subthalamic stimulation-induced dyskinesia through timed stimulation programming of different contacts.
Meige syndrome is a rare adult-onset segmental dystonia characterized by blepharospasms and oromandibular dystonia. Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an established treatment, but it can lead to stimulation-induced dyskinesia (SID) in some patients. Refractory SID in Meige syndrome after STN-DBS is clinically challenging. We report a case of a Meige syndrome patient who developed refractory SID following STN-DBS and was successfully managed using a novel timed-stimulation programming strategy employing different contacts. A 47-year-old female with a two-year history of Meige syndrome developed refractory SID after the treatment of STN-DBS. Various programming strategies were attempted, including monopolar stimulation, interleaved stimulation, bipolar stimulation et al., but none achieved a balance between symptom control and SID. A novel approach involving timed alternation between ventral contacts (contacts 3 and 7) and dorsal contacts (contacts 4 and 8) was implemented. The stimulation was gradually programmed, the duration of ventral stimulation was increased while decreasing dorsal stimulation. Eventually, the patient achieved significant symptom improvement without SID. The reconstruction of the volume of tissue activated (VTA) revealed that this stimulation strategy likely modulates the neural circuits of pallidothalamic fibers (PTF) to suppress SID. This case demonstrates that this noval timed stimulation programming can effectively manage refractory SID in Meige syndrome patients, offering a viable alternative when conventional methods fail. The findings suggest that PTF stimulation plays a key role in SID suppression, and this strategy warrants further investigation in larger cohorts.
2025-09-07 | Optimal stimulation site and fiber tracts in subthalamic deep brain stimulation for Meige syndrome.
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has emerged as an effective therapy for Meige syndrome (MS). However, the optimal stimulation site within STN and the most effective stimulation fiber tracts have not been investigated. Based on the discovery cohort (n = 65), we first identified the optimal stimulation site within the STN using the sweet spot mapping method. Second, we screened for the fiber tracts accounting for optimal clinical outcomes by the fiber filtering approach. Third, based on the above findings, we constructed outcome prediction models and estimated their predictive performance in the discovery cohort and an independent validation cohort (n = 20). Finally, we introduced two prospective cases to illustrate if and how the optimal stimulation site and fiber tracts could facilitate precise electrode targeting and postoperative programming. The optimal stimulation site was mapped to the anterodorsal portion of the STN-motor subregion. Superior STN-DBS outcomes were positively correlated with stimulation of the fibers projecting to the primary motor cortices, the supplementary motor areas, and the globus pallidus internus. Notably, spatial overlap between individual stimulation volumes and the resultant sweet spot or fiber filtering models could cross-predict symptom improvement in out-of-model patients. Moreover, the models could guide electrode implantation and active contact selection in prospective cases. Our study underscores the potential of optimizing stimulation sites and fibers to predict clinical improvement, and provides new insights into the ongoing efforts of precise surgical targeting and computer-assisted DBS programming.
2025-08-16 | Subthalamic nucleus deep brain stimulation for cranial-cervical dystonia: optimal stimulation sites and networks.
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an effective treatment for medically refractory cranial-cervical dystonia (CCD or Meige syndrome). However, clinical responses vary substantially across individuals, likely due to differences in electrode placement and modulation of target neural circuits. We retrospectively analyzed 51 patients with CCD treated with STN-DBS at a single center. Pre- and postoperative imaging was used to reconstruct electrode locations and model patient-specific electric fields. We then performed (i) voxel-wise sweet spot mapping to identify optimal stimulation sites, (ii) fiber filtering using normative tractography to determine white matter pathways associated with clinical improvement, and (iii) network mapping based on resting-state fMRI to identify functional connectivity patterns predictive of DBS response. Voxel-wise correlation analysis revealed that the optimal stimulation localized to the STN motor subregion (R = 0.52, p < 0.001). Normative structural connectivity analysis showed that symptom improvement correlated strongly with modulation of fibers projecting to the cranial and cervical regions of sensorimotor cortex (R = 0.52, p < 0.001) and sensorimotor-associated basal ganglia pathways (R = 0.62, p < 0.001). Functional network mapping further revealed connectivity to the sensorimotor cortex as significantly associated with clinical improvement (R = 0.43, p = 0.002). These findings inform refinement of STN targeting strategies in DBS for CCD. The involvement of cranial and cervical sensorimotor regions highlights the importance of symptom-based dystonia classification for individualized neuromodulation approaches.
2025-03-14 | Clinical outcomes and prognostic factors in patients with primary Meige syndrome undergoing subthalamic nucleus deep brain stimulation: a retrospective study of 65 cases.
Subthalamic nucleus deep brain stimulation (STN-DBS) for primary Meige syndrome has been increasingly reported in recent years. Despite the potential of this therapeutic approach, only a limited number of studies have evaluated its clinical benefits. Moreover, the efficacy of STN-DBS varies among patients with Meige syndrome, and stable prognostic predictors are scarce. In this study, the authors assessed the therapeutic effect of STN-DBS for Meige syndrome and explored reliable prognostic indicators to facilitate patient selection and postoperative programming. The authors enrolled 65 consecutive patients with Meige syndrome who underwent bilateral STN-DBS at their institution. Preoperative and postoperative motor symptoms were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability (BFMDRS-D) subscales. Leads were reconstructed in the standard space by using the Lead-DBS toolbox, and the volume of tissue activated (VTA) was calculated for each lead. Group comparisons and multivariate logistic regression analyses were conducted to evaluate clinical and demographic factors influencing the improvement rates in BFMDRS-M scores. Significant postoperative improvements in the BFMDRS-M score (59.17% ± 28.0%, p < 0.001) and in the BFMDRS-D score (65.05% ± 38.9%, p < 0.001) were observed. Group comparisons indicated that the y-axis value of active contacts, the overlapping volume between VTAs and the STN sensorimotor region, as well as the distance from the center of active contacts to the surface of the STN sensorimotor region were significantly associated with the improvement rate of BFMDRS-M scores. Multivariate logistic regression analyses revealed that both the overlapping volume between VTAs and bilateral STN sensorimotor regions-along with the involvement of the left STN limbic region-emerged as independent prognostic indicators for the improvement in BFMDRS-M scores. Bilateral STN-DBS proved to be a safe and effective treatment for Meige syndrome, and the STN sensorimotor region tended to be a desirable target. This study provided deeper insights into the clinical efficacy, patient selection, and targeting precision of STN-DBS treatment for Meige syndrome.
small molecules
2026-08-14 | Use of explainable cluster analysis to identify distinct subtypes of Meige syndrome patients and associated biomarker profiles.
Meige syndrome (MS), a complex form of segmental craniocervical dystonia, currently lacks objective diagnostic biomarkers.This study aims to identify biological biomarkers that can stratify patients with MS and to reveal the complex pathophysiological heterogeneity of this disorder, with particular attention to the peripheral immune-metabolic profiles that may reflect underlying neuroimmune dysfunction. We retrospectively collected clinical and laboratory data from 1,782 patients with MS during their first hospitalization at the MS Center of The Third People's Hospital of Henan Province between 2023 and 2025. A total of 66 clinical and laboratory indicators were included. An interpretable clustering analysis framework was applied to perform a series of pipeline analyses, including imputation, dimensionality reduction, outlier removal, and clustering. Next, feature importance was assessed. A multi-class classification model was then constructed and evaluated using XGBoost. Finally, the Shapley Additive exPlanations(SHAP) algorithm was employed to interpret the contribution of each feature. The preprocessing pipeline effectively imputed missing values (ranging from 0.06% to 14.14%) and removed outliers. Unsupervised clustering analysis revealed three heterogeneous subtypes of MS. A total of 23 important features were selected to construct a multi-class XGBoost classification model. The model demonstrated excellent performance, with a macro-average AUC of 0.9790 and an Obuchowski index of 0.9784. The SHAP analysis further elucidated the unique hematological and biochemical metabolic characteristics of each subtype. This study establishes a novel, data-driven subtyping system for MS based on objective blood and metabolic profiles. The three identified subtypes display distinct immune-metabolic signatures: a hypercoagulable-inflammatory profile, a metabolic-excess profile with impaired vascular protection, and a frailty-associated antioxidant-deficient profile. These peripheral biomarker patterns suggest testable hypotheses regarding neuroimmune mechanisms-including fibrinogen-driven microglial activation, oxidized lipid-mediated blood-brain barrier disruption, and uric acid-dependent antioxidant depletion-that may contribute to the pathophysiology of different MS subtypes. This framework may inform future prospective investigations into precision medicine approaches for MS, though substantial validation is required before clinical translation.
2026-07-28 | A Case of Meige Syndrome in a Pediatric Patient With Schizophrenia.
Long-term treatment with multiple antipsychotic medications for schizophrenia may increase the risk of developing tardive dystonia, including Meige syndrome. Although clozapine has been suggested as a way of improving dystonia symptoms, there is no established consensus regarding its use in pediatric patients with treatment-resistant schizophrenia, and careful consideration is required. A 12-year-old boy with schizophrenia experienced blepharospasm and oromandibular twitching while undergoing treatment with multiple antipsychotic medications. We stopped giving him the same medications that had been used during his previous hospitalizations (risperidone, olanzapine, aripiprazole, lurasidone, and paliperidone, paliperidone palmitate (a long-acting injectable), asenapine, and blonanserin). We then started giving him clozapine, gradually increasing the dose to 125 mg. Following this treatment intervention, the dystonic symptoms resolved completely, and the psychiatric symptoms stabilized.
2026-01-01 | Advances in the treatment of Meige syndrome
Meige syndrome is a form of segmental craniofacial dystonia characterized predominantly by blepharospasm and frequently accompanied by oromandibular and lower facial dystonia. It is associated with substantial symptom burden, considerable clinical heterogeneity, and persistent challenges in long-term management, including marked variability in treatment response, fragmented evidence, and the absence of standardized stratified therapeutic approaches. Against this background, this Review aims to provide a comprehensive and critical update on current therapeutic advances in Meige syndrome. First, we focus on the major established treatment modalities, including botulinum toxin therapy—with particular attention to formulation selection, dose optimization, injection strategies, safety, and efficacy assessment—systemic pharmacotherapy, including its appropriate clinical indications, potential benefits, and inherent limitations, and deep brain stimulation (DBS) for refractory cases, with emphasis on clinical efficacy, safety, target selection, and parameter optimization. In parallel, we discuss how objective tools such as neuroimaging and electrophysiological techniques may help clarify the associations between specific therapeutic strategies and disease subtypes, symptom profiles, and clinical outcomes. Second, we summarize the available evidence for adjunctive or alternative interventions, including acupuncture, radiofrequency-based approaches, and transcranial magnetic stimulation, in order to present a more complete picture of the therapeutic landscape in Meige syndrome. Finally, we highlight the translational potential of neuromodulatory strategies in this disorder, particularly their prospective role in improving refractory symptoms, refining individualized treatment pathways, and advancing precision-based management. By integrating evidence across these domains, we aim to provide a clearer framework for clinical decision-making in Meige syndrome, while identifying key gaps in the current literature and informing future efforts toward standardized outcome measures, prospective study design, and the development of novel neuromodulatory therapies.
2025-11-10 | Comparative efficacy and safety of GPi-DBS versus STN-DBS in Meige syndrome: a retrospective propensity score-matched cohort study.
Although deep brain stimulation (DBS) targeting the globus pallidus internus (GPi) or the subthalamic nucleus (STN) has shown efficacy for Meige syndrome (MS), there is limited comparative evidence available. This retrospective cohort study consecutively enrolled patients with primary MS who underwent bilateral GPi-DBS or STN-DBS at our center between March 2015 and February 2025. Propensity score matching (PSM, 1.5:1) was employed to balance baseline covariates. The primary outcomes were improvements in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore (BFMDRS-M) at 3-month (short-term) and at the final follow-up (long-term). Additionally, stimulation parameters, programming adjustments, and adverse events (AEs) were analyzed. The study cohort comprised 254 eligible patients (GPi-DBS: 176; STN-DBS: 78), with 14 patients (4.8%) lost to follow-up. The overall cohort consisted of 178 women (70.1%) with a mean surgical age of 57.7 ± 8.6 years. After PSM, 157 patients were matched (89 GPi-DBS, 68 STN-DBS). At 3-month follow up, the mean improvement rate of BFMDRS-M total score was 51.0% [95% confidence interval (CI): 46.7-55.3%] for GPi-DBS and 54.6% (95% CI: 49.3-59.8%) for STN-DBS, with no significant intergroup difference (P = 0.235). After a mean follow-up of 42.4 ± 23.6 months, the mean improvement was 61.0% (95% CI: 56.4-65.5%) for GPi-DBS and 65.2% (95% CI: 59.2-71.1%) for STN-DBS, again indicating no significant difference ( P = 0.192). STN-DBS required lower stimulation parameters but significantly more programming adjustments than GPi-DBS (mean sessions: 5.49 vs 4.33; P < 0.001). AE incidence was similar between groups (GPi-DBS vs STN-DBS: 5.4% vs 3.6%; P = 0.731). Both GPi-DBS and STN-DBS provided significant and sustained motor symptom improvement in refractory MS, with no statistically significant difference between groups.
2025-09-15 | A Case Report on the Management of Meige Syndrome
Meige syndrome is a rare neurological movement disorder characterized by involuntary, forceful and often painful contractions of the facial muscles. It typically presents as a combination of blepharospasm and oromandibular dystonia. This case report discusses a 53-year-old female patient, diagnosed with Meige syndrome, who presented to the Outpatient Department of Government Ayurveda Medical College, Thiruvananthapuram, with complaints of pain over the temporomandibular joint, repetitive jaw movements and involuntary, recurrent closure of both eyelids for the past 5 years. The treatment principle adopted in this case was based on Ardhita Chikitsa. She was given internal medications such as Indukantham ghritam, Dhanadanayanadi Kashayam, Dhanwantaram 101A, Yogaraja guggulu tablets, along with external procedures like Thalam, Jaloukavacharana, Nasya, Ksheeradhooma, Shirodhara, Gandusham, Shashtika Pinda Swedam and Agnikarma. The treatment duration was one month, with follow-ups continued for three months and changes were assessed at the third follow-up. Follow-up medicines were Murdhini Tailam and mukhabhyangam with Balatailam, Pratimarsha Nasyam with Dhanwantaram 41A and Tablet Yogaraja Guggulu as internal medicine. The changes were assessed after the third follow-up using the Jankovic Rating Scale (JRS) and the Oromandibular Dystonia Rating Scale (OMDRS). This management approach suggests that Ayurvedic interventions may serve as a promising and effective therapeutic modality in the management of Meige syndrome.
proteins
2026-05-25 | Intervention effects of different brands of botulinum toxin type A on Meige syndrome with anxiety symptoms.
Patients with Meige syndrome are often affected by anxiety symptoms, and drug therapy is the primary treatment option, with botulinum toxin A being the most frequently used medication. This study analyzes the intervention effects of different brands of botulinum toxin A on Meige syndrome with anxiety symptoms. The findings aim to provide a theoretical basis for the rational selection of clinical treatment options. A retrospective analysis was conducted on 148 patients treated between January 2021 and December 2023. Patients were divided into two groups based on the BoNT-A brand received: Chinese botulinum toxin type A (Hengli) group (n = 80) and onabotulinumtoxinA (Botox) group (n = 68). Both groups received local injections at the sites of muscle spasms. The outcomes included the degree of spasm relief, duration of efficacy, scores on the Self-Rating Anxiety Scale (SAS) and Self-Rating Depression Scale (SDS), polysomnography parameters [sleep efficiency (SE), total sleep time (TST), sleep latency (SL), number of wakefulness (AI), rapid eye movement sleep time (REMS)], and adverse reactions. There were no statistically significant differences in the total remission rate and efficacy maintenance time between the two groups (p > 0.05). The differences in the scores of the SAS, SDS, SE, TST, SL, AI, and REMS before and after treatment between the two groups were not statistically significant (p > 0.05). There was no statistically significant difference between the two groups when comparing the incidence of adverse reactions such as incomplete eyelid closure, facial muscle weakness, and drooping of the corners of the mouth during expression muscle activity (p > 0.05). Local injection of botulinum toxin type A is a safe and effective treatment for Meige syndrome with anxiety symptoms, and there is no significant difference in the efficacy, duration of action, and improvements in psychological state and sleep quality between different brands of botulinum toxin type A. Therefore, clinical practice should be based on practical considerations to reasonably select the appropriate brand of botulinum toxin type A for treatment.
2026-01-28 | Geometric Microstructural Characteristics of White Matter Differentiate Patients With Facial Dyskinesias and Palsy.
The heterogeneous and homogeneous clinical manifestations of peripheral facial palsy (FP), hemifacial spasm (HFS), and Meige's syndrome (MS) complicate the differentiation of diagnoses for these facial motor diseases. To comprehensively investigate the white matter microstructural characteristics in patients with facial dyskinesias and palsy using geometric and integrity metrics in DTI. In this prospective study conducted from September 2020 to January 2022, patients with FP, HFS, and MS, as well as sex-matched healthy control subjects, underwent 3.0 T MRI. Geometric metrics (i.e., splay, bend, twist, and total distortion) based on "Director Field Analysis" and fractional anisotropy (FA) and mean diffusivity (MD) were calculated from DTI data. Cross-sectional tract-based spatial statistics were performed among FP, HFS, MS patients, and healthy controls. The correlation between disease severity and DTI metrics was evaluated. Additionally, the geometric microstructural properties combining FA and MD were used to classify FP, HFS, and MS patients using machine learning methods. Geometric metrics and FA/MD were widely altered across white matter in FP and HFS patients compared with healthy controls. However, in MS patients only DFA metrics were significantly altered. FA and DFA values strongly correlated with the severity of facial movement disorder in FP patients. Combing conventional FA/MD value with DFA metrics enabled the diagnostic differentiation of FP and HFS from MS. Our findings demonstrated that the geometric microstructural information of white matter fibers could provide novel insight into the underlying pathological changes in facial dyskinesias and palsy.
2025-03-10 | Experience of the application of botulinum toxin type A as a treatment of facial hyperkinetic disorders in a Mexican ophthalmological center.
Blepharospasm is characterized by involuntary, sustained spasms of the orbicularis oculi, corrugator, and procerus muscles. Hemifacial spasm (HFS) is characterized by the involuntary tonic-clonic contraction of the muscles of a hemiface. The main role of botulinum toxin type A (BoNT-A) in the treatment of blepharospasm and HFS is recognized by guidelines around the world. In this study, we evaluated the applicability of botulinum toxin type A in patients with periocular hyperkinetic disorders at the Instituto de Oftalmología Fundación Conde de Valenciana, so that we can share the Mexican experience with the international evidence. The clinical records of patients diagnosed with facial hyperkinetic disorders treated with botulinum toxin type A (BoNT-A) that were treated from January 2015 to September 2021 were analyzed. A total of 140 records of patients diagnosed with facial hyperkinetic disorder and treated with BoNT-A were analyzed. Facial hyperkinetic disorders that occurred included 5 (3.6%) patients with orbicularis oculi myokymia, 72 (51.4%) with blepharospasm, 62 (44.3%) with hemifacial spasm, and 1 (0.7%) with Meige syndrome. One hundred and twenty patients (85.7%) reported remission of initial symptoms after BoNT-A therapy during the period covered by this study. Botulinum toxin type A is effective and safe as a treatment for facial hyperkinetic disorders in the Mexican population.
2024-04-01 | Effects of onabotulinum toxin type A injections in patients with Meige's syndrome
Abstract Background Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type A (OBTA) injections are the standard treatment, evidence of their effectiveness and safety in this scenario is still lacking. Objective Our research aimed to evaluate the improvement and occurrence of side effects following injections of onabotulinum toxin type A (OBTA) in patients with Meige's syndrome. Methods Patients with Meige's syndrome undergoing botulinum toxin injections were enrolled in this study. We assessed dystonia intensity before and 14 days after OBTA injection using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) to measure the response of symptoms in the eyes (blepharospasm) and mouth (oromandibular dystonia). Other variables, such as dosage, side effects, and demographic data, were also recorded. Results The study included 41 participants, with a mean age of 67.7 years and a female-to-male ratio of 3.5:1. The mean BFMDRS score before the injections was 8.89, and after 14 days, it was 2.88. The most reported side effect was ptosis, with a 7.3% incidence. OBTA significantly reduced dystonia severity (p < 0.0001). The clinical response for the blepharospasm component was superior to the oromandibular dystonia component. Conclusion Our results support that OBTA seems to be an effective and safe therapeutic option for treating Meige's syndrome. The effect of OBTA was more pronounced in the treatment of blepharospasm than in oromandibular dystonia.
2023-02-03 | Mechanism and clinical use of botulinum neurotoxin in head and facial region
Botulinum neurotoxin (BoNT) is a biological toxin produced by Clostridium botulinum. BoNT is a potent toxin extensively used in therapeutic interventions. This review provides an updated overview of the mechanisms of action and clinical applications of BoNT in head and facial region.MEDLINE/PubMed searches were conducted using the terms "botulinum neurotoxin" and "dentistry" along with a combination of other related terms. In addition, studies were manually selected from reference lists of the selected articles.The Food and Drug Administration in the United States initially approved BoNT to treat strabismus, blepharospasm, and hemifacial spasms. The use of BoNT in dermatology and cosmetics has been widely established and has created a revolution in these fields. Over the years, its applications in various medical specialties have expanded widely. Owing to its safety, efficacy, and long duration of action, it is well-accepted by patients. BoNT/A and BoNT/B are widely used in clinical practice. Several off-label uses of BoNT in the dental fraternity have yielded promising results. We have elaborated on the speculated mechanism of action, dosage, effective sites of injection, and adverse effects of each therapeutic application. The various clinical indications for BoNT include bruxism, myofascial pain, temporomandibular joint dislocation, hemifacial pain, orofacial dystonia, facial paralysis, chronic migraine, and trigeminal neuralgia.BoNT is a safe treatment that can be used effectively, provided that the clinician has adequate knowledge regarding the mechanism, injection techniques, and local and systemic side effects and that it is administered cautiously and purposefully.
cell therapies
2026-05-01 | Subthalamic nucleus deep brain stimulation in Meige syndrome: mapping the optimal stimulation sites and network targets.
The aim of this study was to identify the optimal stimulation sites for subthalamic nucleus (STN) deep brain stimulation (DBS) in treating Meige syndrome using long-term follow-up data from a large sample cohort, evaluate the whole-brain functional connectivity patterns associated with favorable treatment responses, and validate these findings in an independent cohort. The authors retrospectively analyzed long-term outcomes in 65 patients with Meige syndrome who underwent bilateral STN-DBS in two centers. The local stimulation effects within the STN and the distributed functional connectivity associated with motor improvement were investigated using advanced imaging and modeling tools, including the Lead-Group Toolbox, DBS Sweet Spot Mapping Explorers, and DBS Network Mapping Explorers. To ensure the model's reliability and generalizability, both internal validation through multiple cross-validation strategies and external validation using independent cohorts were conducted. STN-DBS yielded significant and sustained motor improvements in both cohorts, with mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score reductions of 63% in the training cohort (n = 50) and 56% in the validation cohort (n = 15) (p < 0.001). At the local level, the optimal stimulation sites were consistently located in the dorsolateral sensorimotor subregion of the STN, extending bilaterally toward the associative subregion and centered at MNI coordinates x = ±12, y = -13, z = -6. At the network level, favorable outcomes were primarily associated with positive functional connectivity to the cerebellum and negative connectivity to the somatosensory cortex. Both the sweet spot and connectivity models developed using the training cohort showed significant correlations with clinical outcomes in the independent validation cohort (R = 0.59, p = 0.020; R = 0.74, p = 0.002, respectively) and remained robust across different cross-validation strategies. The optimal therapeutic efficacy of STN-DBS for Meige syndrome depends on precise targeting within the dorsolateral STN and modulation of a distributed functional network involving the cerebellum and sensorimotor cortex. These findings may aid in developing personalized targeting strategies and adaptive programming paradigms, ultimately improving the therapeutic efficacy of DBS in this challenging disorder.
2026-02-09 | Case Report: Effective management of a Meige syndrome patient with subthalamic stimulation-induced dyskinesia through timed stimulation programming of different contacts.
Meige syndrome is a rare adult-onset segmental dystonia characterized by blepharospasms and oromandibular dystonia. Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an established treatment, but it can lead to stimulation-induced dyskinesia (SID) in some patients. Refractory SID in Meige syndrome after STN-DBS is clinically challenging. We report a case of a Meige syndrome patient who developed refractory SID following STN-DBS and was successfully managed using a novel timed-stimulation programming strategy employing different contacts. A 47-year-old female with a two-year history of Meige syndrome developed refractory SID after the treatment of STN-DBS. Various programming strategies were attempted, including monopolar stimulation, interleaved stimulation, bipolar stimulation et al., but none achieved a balance between symptom control and SID. A novel approach involving timed alternation between ventral contacts (contacts 3 and 7) and dorsal contacts (contacts 4 and 8) was implemented. The stimulation was gradually programmed, the duration of ventral stimulation was increased while decreasing dorsal stimulation. Eventually, the patient achieved significant symptom improvement without SID. The reconstruction of the volume of tissue activated (VTA) revealed that this stimulation strategy likely modulates the neural circuits of pallidothalamic fibers (PTF) to suppress SID. This case demonstrates that this noval timed stimulation programming can effectively manage refractory SID in Meige syndrome patients, offering a viable alternative when conventional methods fail. The findings suggest that PTF stimulation plays a key role in SID suppression, and this strategy warrants further investigation in larger cohorts.
2025-09-07 | Optimal stimulation site and fiber tracts in subthalamic deep brain stimulation for Meige syndrome.
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has emerged as an effective therapy for Meige syndrome (MS). However, the optimal stimulation site within STN and the most effective stimulation fiber tracts have not been investigated. Based on the discovery cohort (n = 65), we first identified the optimal stimulation site within the STN using the sweet spot mapping method. Second, we screened for the fiber tracts accounting for optimal clinical outcomes by the fiber filtering approach. Third, based on the above findings, we constructed outcome prediction models and estimated their predictive performance in the discovery cohort and an independent validation cohort (n = 20). Finally, we introduced two prospective cases to illustrate if and how the optimal stimulation site and fiber tracts could facilitate precise electrode targeting and postoperative programming. The optimal stimulation site was mapped to the anterodorsal portion of the STN-motor subregion. Superior STN-DBS outcomes were positively correlated with stimulation of the fibers projecting to the primary motor cortices, the supplementary motor areas, and the globus pallidus internus. Notably, spatial overlap between individual stimulation volumes and the resultant sweet spot or fiber filtering models could cross-predict symptom improvement in out-of-model patients. Moreover, the models could guide electrode implantation and active contact selection in prospective cases. Our study underscores the potential of optimizing stimulation sites and fibers to predict clinical improvement, and provides new insights into the ongoing efforts of precise surgical targeting and computer-assisted DBS programming.
2025-08-16 | Subthalamic nucleus deep brain stimulation for cranial-cervical dystonia: optimal stimulation sites and networks.
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an effective treatment for medically refractory cranial-cervical dystonia (CCD or Meige syndrome). However, clinical responses vary substantially across individuals, likely due to differences in electrode placement and modulation of target neural circuits. We retrospectively analyzed 51 patients with CCD treated with STN-DBS at a single center. Pre- and postoperative imaging was used to reconstruct electrode locations and model patient-specific electric fields. We then performed (i) voxel-wise sweet spot mapping to identify optimal stimulation sites, (ii) fiber filtering using normative tractography to determine white matter pathways associated with clinical improvement, and (iii) network mapping based on resting-state fMRI to identify functional connectivity patterns predictive of DBS response. Voxel-wise correlation analysis revealed that the optimal stimulation localized to the STN motor subregion (R = 0.52, p < 0.001). Normative structural connectivity analysis showed that symptom improvement correlated strongly with modulation of fibers projecting to the cranial and cervical regions of sensorimotor cortex (R = 0.52, p < 0.001) and sensorimotor-associated basal ganglia pathways (R = 0.62, p < 0.001). Functional network mapping further revealed connectivity to the sensorimotor cortex as significantly associated with clinical improvement (R = 0.43, p = 0.002). These findings inform refinement of STN targeting strategies in DBS for CCD. The involvement of cranial and cervical sensorimotor regions highlights the importance of symptom-based dystonia classification for individualized neuromodulation approaches.
2025-03-14 | Clinical outcomes and prognostic factors in patients with primary Meige syndrome undergoing subthalamic nucleus deep brain stimulation: a retrospective study of 65 cases.
Subthalamic nucleus deep brain stimulation (STN-DBS) for primary Meige syndrome has been increasingly reported in recent years. Despite the potential of this therapeutic approach, only a limited number of studies have evaluated its clinical benefits. Moreover, the efficacy of STN-DBS varies among patients with Meige syndrome, and stable prognostic predictors are scarce. In this study, the authors assessed the therapeutic effect of STN-DBS for Meige syndrome and explored reliable prognostic indicators to facilitate patient selection and postoperative programming. The authors enrolled 65 consecutive patients with Meige syndrome who underwent bilateral STN-DBS at their institution. Preoperative and postoperative motor symptoms were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability (BFMDRS-D) subscales. Leads were reconstructed in the standard space by using the Lead-DBS toolbox, and the volume of tissue activated (VTA) was calculated for each lead. Group comparisons and multivariate logistic regression analyses were conducted to evaluate clinical and demographic factors influencing the improvement rates in BFMDRS-M scores. Significant postoperative improvements in the BFMDRS-M score (59.17% ± 28.0%, p < 0.001) and in the BFMDRS-D score (65.05% ± 38.9%, p < 0.001) were observed. Group comparisons indicated that the y-axis value of active contacts, the overlapping volume between VTAs and the STN sensorimotor region, as well as the distance from the center of active contacts to the surface of the STN sensorimotor region were significantly associated with the improvement rate of BFMDRS-M scores. Multivariate logistic regression analyses revealed that both the overlapping volume between VTAs and bilateral STN sensorimotor regions-along with the involvement of the left STN limbic region-emerged as independent prognostic indicators for the improvement in BFMDRS-M scores. Bilateral STN-DBS proved to be a safe and effective treatment for Meige syndrome, and the STN sensorimotor region tended to be a desirable target. This study provided deeper insights into the clinical efficacy, patient selection, and targeting precision of STN-DBS treatment for Meige syndrome.
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Drug Discovery Landscape
2 orphan drug designations for Blepharospasm-oromandibular dystonia syndrome.
2 orphan drug designations for Blepharospasm-oromandibular dystonia syndrome.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Botulinum toxin type A | proteins | FDA | 1991-12-05 | — | Ipsen Biopharmaceuticals, Inc. |
Botulinum toxin type A | proteins | FDA | 1989-03-23 | — | Porton International, Inc. |
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