Structural network topologies are associated with deep brain stimulation outcomes in Meige syndrome.

Deep brain stimulation (DBS) is an effective therapy for Meige syndrome (MS). However, the DBS efficacy varies across MS patients and the factors contributing to the variable responses remain enigmatic. We aim to explain the difference in DBS efficacy from a network perspective. We collected preoperative T1-weighted MRI images of 76 MS patients who received DBS in our center. According to the symptomatic improvement rates, all MS patients were divided into two groups: the high improvement group (HIG) and the low improvement group (LIG). We constructed group-level structural covariance networks in each group and compared the graph-based topological properties and interregional connections between groups. Subsequent functional annotation and correlation analyses were also conducted. The results indicated that HIG showed a higher clustering coefficient, longer characteristic path length, lower small-world index, and lower global efficiency compared with LIG. Different nodal betweennesses and degrees between groups were mainly identified in the precuneus, sensorimotor cortex, and subcortical nuclei, among which the gray matter volume of the left precentral gyrus and left thalamus were positively correlated with the symptomatic improvement rates. Moreover, HIG had enhanced interregional connections within the somatomotor network and between the somatomotor network and default-mode network relative to LIG. We concluded that the high and low DBS responders have notable differences in large-scale network architectures. Our study sheds light on the structural network underpinnings of varying DBS responses in MS patients.

Deep brain stimulation of the subthalamic nucleus for primary Meige syndrome: clinical outcomes and predictive factors.

OBJECTIVE: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has demonstrated efficacy against multiple types of dystonia, but only a few case reports and small-sample studies have investigated the clinical utility of STN-DBS for Meige syndrome, a rare but distressing form of craniofacial dystonia. Furthermore, the effects of DBS on critical neuropsychological sequelae, such as depression and anxiety, are rarely examined. In this study, the authors investigated the therapeutic efficacy of STN-DBS for both motor and psychiatric symptoms of Meige syndrome. METHODS: The authors retrospectively reviewed consecutive patients with Meige syndrome receiving bilateral STN-DBS at their institution from January 2016 to June 2023. Motor performance and nonmotor features including mood, cognitive function, and quality of life (QOL) were evaluated using standardized rating scales at baseline and at final postoperative follow-up. Clinical and demographic factors influencing postoperative motor outcome were evaluated by uni- and multivariable linear regression models. RESULTS: Fifty-one patients were ultimately included, with a mean ± SD follow-up duration of 27.3 ± 18.0 months. The mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score improved from 12.9 ± 5.2 before surgery to 5.3 ± 4.2 at the last follow-up (mean improvement 58.9%, p < 0.001) and the mean BFMDRS disability score improved from 5.6 ± 3.3 to 2.9 ± 2.9 (mean improvement 44.6%, p < 0.001). Hamilton Depression and Anxiety Rating Scale scores also improved by 35.3% and 34.2%, respectively, and the postoperative 36-item Short-Form Health Survey score indicated substantial QOL enhancement. Global cognition remained stable after treatment. Multiple linear regression analysis identified disease duration (ß = -0.241, p = 0.027), preoperative anxiety severity (ß = -0.386, p = 0.001), and volume of activated tissue within the dorsolateral (sensorimotor) STN (ß = 0.483, p < 0.001) as independent predictors of motor outcome. CONCLUSIONS: These findings support STN-DBS as an effective and promising therapy for both motor and nonmotor symptoms of Meige syndrome. Timely diagnosis, treatment of preoperative anxiety, and precise electrode placement within the dorsolateral STN are essential for optimal clinical outcome.

Subthalamic nucleus deep brain stimulation in primary Meige syndrome: motor and non-motor outcomes.

BACKGROUND AND PURPOSE: Deep brain stimulation (DBS) has emerged as a promising treatment for movement disorders. This prospective study aims to evaluate the effects of bilateral subthalamic nucleus DBS (STN-DBS) on motor and non-motor symptoms in patients with primary Meige syndrome. METHODS: Thirty patients who underwent bilateral STN-DBS between April 2017 and June 2020 were included. Standardized and validated scales were utilized to assess the severity of dystonia, health-related quality of life, sleep, cognitive function and mental status at baseline and at 1 year and 3 years after neurostimulation. RESULTS: The Burke-Fahn-Marsden Dystonia Rating Scale movement scores showed a mean improvement of 63.0% and 66.8% at 1 year and 3 years, respectively, after neurostimulation. Similarly, the Burke-Fahn-Marsden Dystonia Rating Scale disability scores improved by 60.8% and 63.3% at the same time points. Postoperative quality of life demonstrated a significant and sustained improvement throughout the follow-up period. However, cognitive function, mental status, sleep quality and other neuropsychological functions did not change after 3 years of neurostimulation. Eight adverse events occurred in six patients, but no deaths or permanent sequelae were reported. CONCLUSIONS: Bilateral STN-DBS is a safe and effective alternative treatment for primary Meige syndrome, leading to improvements in motor function and quality of life. Nevertheless, it did not yield significant amelioration in cognitive, mental, sleep status and other neuropsychological functions after 3 years of neurostimulation.

Sjogren's syndrome meets Meige's syndrome.

This report details a rare case where a patient simultaneously suffered from Sjogren's syndrome (SS) and Meige's syndrome (MS). SS, an autoimmune disorder, and MS, a rare neurological condition characterized by involuntary eyelid closure, presented in a 73-year-old male. The patient had been experiencing dry eye symptoms for the past 5 years, with the onset of eyelid spasms and tetanic eye closure occurring 3 years ago. Traditional treatments, including subthalamic nucleus deep brain stimulation, provided only temporary relief. Diagnostic evaluations, including blood tests and imaging, confirmed SS and MS coexistence. Treatment involved a combination of steroids, immunosuppressants, and immunoglobulin, leading to significant symptom relief. This case suggests a potential association between SS and the development of MS, highlighting the importance of immunomodulatory therapy in managing neurological symptoms. Further research is needed to explore the relationship between these two conditions and to develop more effective treatment strategies.

Deep Brain Stimulation for Focal or Segmental Craniocervical Dystonia in Patients Who Have Failed Botulinum Neurotoxin Therapy-A Narrative Review of the Literature.

(1) Background: The first-line treatment for patients with focal or segmental dystonia with a craniocervical distribution is still the intramuscular injection of botulinum neurotoxin (BoNT). However, some patients experience primary or secondary treatment failure from this potential immunogenic therapy. Deep brain stimulation (DBS) may then be used as a backup strategy in this situation. (2) Methods: Here, we reviewed the current study literature to answer a specific question regarding the efficacy and safety of the use of DBS, particularly for cervical dystonia (CD) and Meige syndrome (MS) in patients with documented treatment failure under BoNT. (3) Results: There are only two studies with the highest level of evidence in this area. Despite this clear limitation, in the context of the narrowly defined research question of this paper, it is possible to report 161 patients with CD or MS who were included in studies that were able to show a statistically significant reduction in dystonic symptoms using DBS. Safety and tolerability data appeared adequate. However, much of the information is based on retrospective observations. (4) Conclusions: The evidence base in this area is in need of further scientific investigation. Most importantly, more randomized, controlled and double-blind trials are needed, possibly including a head-to-head comparison of DBS and BoNT.

[Efficacy of CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves in the treatment of Meige syndrome].

Objective: To evaluate the efficacy of CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves in the treatment of Meige syndrome. Methods: The Clinical data of 56 patients with Meige syndrome in the Department of Pain Medicine, Affiliated Hospital of Jiaxing University from June 2019 to January 2023 were retrospectively analyzed [19 males and 37 females, aged 42-76 (58.6±8.3) years], including 51 cases of blepharospasm, 3 cases of oromandibular dystonia and 2 cases of blepharospasm concomitant with oromandibular dystonia. CT-guided partial radiofrequency ablation of bilateral responsible cranial nerves was performed on different types of Meige syndrome. And the efficacy and complications of the technique were observed. Results: Fifty-one patients with blepharospasm Meige syndrome underwent CT-guided radiofrequency of facial nerve through bilateral stylomastoid foramen punctures, the symptoms of blepharospasm disappeared completely, leaving bilateral mild and moderate facial paralysis symptoms. Three patients with oral-mandibular dystonia underwent CT-guided radiofrequency therapy by bilateral foramen ovale puncture of mandibular branches of trigeminal nerve, masticatory muscle spasm disappeared, the patients had no difficulty opening the mouth, and the skin numbness in bilateral mandibular nerve innervation area was left. Two cases of Meige syndrome with blepharospasm concomitant with oromandibular dystonia were treated by radiofrequency of facial nerve and mandibular branch of trigeminal nerve, and all symptoms disappeared. The patients were followed up for 1-44 months after the operation, and the symptoms of mild and moderate facial paralysis disappeared at (3.2±0.8) months after the operation, but the numbness did not disappear. Three patients with blepharospasm recurred at the 14, 18 and 22 months after the operation, respectively, while the rest cases did not recur. Conclusions: According to different types of Meige syndrome, CT-guided partial radiofrequency ablation of responsible cranial nerves can effectively treat the corresponding type of Meige syndrome. The complications are only mild and moderate facial paralysis which can be recovered, and/or skin numbness in the mandibular region.

Bilateral Pallidal Deep Brain Stimulation in Meige Syndrome: Effects on Motor Function, Neuropsychological Status, and Mood.

BACKGROUND: Bilateral pallidal deep brain stimulation (DBS) has been broadly accepted as a feasible surgical procedure for treating various forms of dystonia, but its effects on motor function, neuropsychological status, and mood in patients with Meige syndrome have rarely been examined. OBJECTIVE: To evaluate the effects of bilateral globus pallidus internus DBS (GPi-DBS) on the motor performance, quality of life, neuropsychological status, and mood of patients with primary Meige syndrome. METHODS: Between January 2015 and April 2019, the database of 35 patients with Meige syndrome who underwent bilateral GPi-DBS in our institution was retrospectively reviewed. The severity of dystonia, health-related quality of life, cognitive function, and mood were assessed using standardized and validated rating scales at baseline. Repeat assessment of the same domains was performed at 1 year and 3 years after neurostimulation in a similar manner. RESULTS: One year and 3 years after bilateral GPi-DBS, Burke-Fahn-Marsden Dystonia Rating Scale movement scores were improved by 65% and 72% and Burke-Fahn-Marsden Dystonia Rating Scale disability scores were improved by 49% and 57%, respectively. The significant improvement in health-related quality of life observed at 1 year was sustained at 3 years. Relative to baseline and to the 1-year assessment, cognitive functions and mood remained stable after 3 years of neurostimulation. No deaths or life-threatening events were reported over the study period. CONCLUSION: Bilateral GPi-DBS is a safe and effective approach for medically refractory Meige syndrome that can improve motor function and quality of life without cognitive and mood side effects.

Volume of tissue activated within subthalamic nucleus and clinical efficacy of deep brain stimulation in Meige syndrome.

OBJECTIVE: The clinical efficacy of deep brain stimulation (DBS) relies on the optimal electrode placement in a large extent. Subthalamic nucleus (STN) DBS was recognized as clinically effective for Meige syndrome. This study identified the correlations of volume of tissue activated (VTA) within the motor STN and the final efficacy of the surgical procedure. METHODS: Clinical outcomes of the patients (n=25) were evaluated with the percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) scores at the last follow-up (LFU) visit. Pearson's correlation coefficients were calculated to identify the relationship of the final clinical outcomes with the VTA within the STN, VTA within the different STN territories, and other clinical variables. RESULTS: On the whole, the patients showed an average of 59.21% improvement at the LFU visit relative to the baseline (5.72 ± 7.31 vs. 13.70 ± 7.36, P ˂ 0.001). Active electrode contacts mainly clustered in the STN motor territories. There were significant positive correlations between the BFMDRS-M percentage improvement and VTA within the STN (Pearson r = 0.434, P = 0.039) and the STN motor territories (r = 0.430, P = 0.041), but not associative or limbic STN. Other basic clinical characteristics including age, disease duration, and preoperative scores were not significantly correlated with the final outcomes. CONCLUSIONS: Our study further validated the efficacy of STN-DBS in even the cases with intractable Meige syndrome. Furthermore, VTA within the motor STN could serve as a potential prognostic factor for the final clinical outcomes.

[The efficacy and safety of nerve loop blocking in the treatment of blepharospasm in Meige syndrome].

Objective: To evaluate the efficacy and safety of nerve loop blocking in the treatment of blepharospasm caused by Meige syndrome. Methods: It was a retrospective case series study. Patients with Meige syndrome characterized by blepharospasm or blepharospasm-oromandibular dystonia who underwent nerve loop blocking in the Ophthalmology Department of Henan No. 3 Provincial People's Hospital from April 2018 to January 2020 were included. Before and after surgery, blepharospasm was graded, and the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) was used to score the symptoms of ocular and oral dystonia. The improvement rate of ocular spasm was calculated after surgery according to the scores. The postoperative complications were observed. The Wilcoxon rank test was used to compare the BFMDRS movement subscale scores before and after surgery. The independent sample Mann-Whitney U test was used to compare the improvement rates of eye spasm between male and female patients and between patients with and without combined oromandibular dystonia. Results: Among the 199 patients included, 64 (32.2%) were males, and 135 (67.8%) were females, aged 58 (51, 64) years (22-79 years). The postoperative follow-up period was 24 (21, 28) months. Preoperatively, blepharospasm was graded as grade 3 in 12 patients (6.0%) and grade 4 in 187 patients (94.0%), while the postoperative blepharospasm grades were grade 0 in 100 patients (50.3%), grade 1 in 64 patients (32.2%), grade 2 in 31 patients (15.6%), and grade 3 in 4 patients (2.0%). There was statistically significant difference in the BFMDRS scores of ocular dystonia before and after surgery [8.0 (8.0, 8.0) vs. 0.0 (0.0, 1.0); Z=-12.41, P<0.001]. The improvement rate of blepharospasm in all patients was 100% (87.5%, 100%), ranging from 43.8% to 100%, with no statistically significant difference between patients of different genders and between patients with and without combined oral dystonia (both P>0.05). Statistically significant difference existed in the scores of oral dystonia before and after surgery [2.0 (0.5, 4.5) vs. 1.0 (0.5, 2.0); Z=-4.38, P<0.001], with 25 of 65 patients (38.5%) having their oral symptoms improved. Postoperative complications included eyelid valgus (7.5%, 15/199), frontal numbness (100%, 199/199) and tearing (89.9%, 179/199). Conclusion: Nerve loop blocking is a relatively safe and effective method in the treatment of blepharospasm symptoms of Meige syndrome.

Micro lesion effect of pallidal deep­brain stimulation for meige syndrome.

To analyse the microlesion effect (MLE) in the globus pallidus interna (GPi) of deep brain stimulation (DBS) in patients with Meige syndrome. Thirty-two patients with primary Meige syndrome who underwent GPi-DBS in this study. Burke-Fahn-Marsden Dystonia Rating Scale scores (BFMDRS-M) were obtained for the evaluation of clinical symptoms at 3 days before DBS (baseline), 24 h after DBS surgery, once weekly for 1 month until electrical stimulation, 6 months postoperatively and 12 months after surgery. Twenty-seven patients had MLE after GPi-DBS. The mean time of BFMDRS-M scores maximal improvement from MLE was 35.9 h postoperatively (range, 24-48 h), and the mean scores improved by 49.35 ± 18.16%. At 12 months after surgery, the mean BFMDRS-M scores improved by 50.28 ± 29.70%. There was a positive correlation between the magnitude of MLE and the motor score at 12 months after GPi-DBS (R2 = 0.335, p < 0.05). However, there was no correlation between the duration of MLE and DBS improvement. Most Meige syndrome patients who underwent GPi-DBS and had MLE benefited from MLE. For Meige syndrome, MLE might be a predictive factor for patient clinical symptom improvement from DBS.

Henry Meige: The man and his understanding of dystonia, at the turn of the 19th to 20th century.

BACKGROUND: Henry Meige (1866-1940), a French neurologist, the pupil of Charcot, is remembered for the eponym, the Meige syndrome, describing the clinical picture of craniocervical dystonia. This historical review highlights the controversies from his essay on "Le Juif Errant" (the Wandering Jew), and the evolution of his understanding of the movement disorders of the face and neck at the time of the encephalitis lethargica. RESULTS: His thesis reported 5 patients from Eastern Europe, presenting with functional neurological disorders following traumatic life experiences. He wrote with Feindel the first book on movement disorders "Les tics et leur traitement". He pioneered the concept of focal dystonia and distinguished the facial median spasm as a dystonic movement disorder of the face. He highlighted the co-existence of psychopathology and the influence of the mental on tics and dystonia. He coined with Brissaud and Feindel the term "geste antagoniste" in cervical dystonia. He emphasized the importance of self-management and psycho-motor retraining for focal dystonia. CONCLUSION: Meige made an invaluable contribution to our understanding of movement disorders, during his long medical career. The eponym Meige syndrome should be retained to describe an individual clinical entity.

Experience to prevent wire tethering in deep brain stimulation from a single center.

OBJECTIVE: To analyze the causes of wire tethering in deep brain stimulation (DBS) and propose ways to prevent it. METHODS: A total of 70 consecutive patients (140 electrodes) operated for DBS in our department from September 2017 to December 2019 were analyzed to document wire tethering, respectively, in the initial period (September 2017-June 2018) and the late period (July 2018-December 2019). The patients come back to our clinic 1 month postoperatively to turn on the equipment and followed up any time postoperatively face to face. RESULTS: Wire tethering was divided into mild, moderate and severe. The frequency of mild wire tethering was 12.5% (2/16) in the initial period and 9.3% (5/54) in the late period. The frequency of moderate wire tethering was 12.5% (2/16) in the initial period and 3.7% (2/54) in the late period. There was only one patient suffered from severe wire tethering in the initial period and none in the late period. There was a significant difference between the initial (31.3%) and the late (13%) periods in the frequency of total wire tethering. CONCLUSIONS: Wire tethering is a rare but serious hardware complication in DBS which should be noteworthy. Improving surgical skill when implanted the extension wire and inventing new material covering extension wire can prevent wire tethering.

Electrophysiological signatures predict clinical outcomes after deep brain stimulation of the globus pallidus internus in Meige syndrome.

OBJECTIVE: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been shown to be a safe and effective alternative therapy for ameliorating medically refractory primary Meige syndrome. However, the associations between DBS target position and surrounding electrophysiological properties as well as patients' clinical outcomes remains largely unknown. In a large number of patients, we investigated electrophysiological features around stimulation targets and explored their roles in predicting clinical outcomes following bilateral GPi-DBS. METHODS: The locations of DBS active contacts along the long axis of the GPi in a standard space were calculated and compared among three groups with different clinical outcomes. The firing rates of individual neurons within the GPi were calculated for each patient and compared across the three groups. RESULTS: Compared with the bad group (poor clinical outcome), active contacts in the good group (good clinical outcome) and the best group (best clinical outcome) were located in the more posterior GPi. The average firing rates in the good and best groups were significantly higher than in the bad group, and this difference was pronounced within the ventral GPi. For the bad group, the average firing rates were significantly lower in the ventral than in the dorsal GPi. CONCLUSIONS: This study suggests that DBS of the posterior GPi may produce better clinical outcomes during primary Meige syndrome treatment and that higher GPi neuronal activity, particularly within the ventral part, can be used as a biomarker to guide DBS electrode implantation during surgery.

Rescue Subthalamic Deep Brain Stimulation for Refractory Meige Syndrome.

Meige syndrome is a segmental form of dystonia. It is a disabling disease, especially when refractory to treatment with botulinum toxin. A well-established therapeutic option is deep brain stimulation (DBS), and the target in bilateral globus pallidus internus (GPi DBS) demonstrated satisfactory short- and long-term efficacy. However, some patients present minor or suboptimal responses after GPi DBS, and in those cases, rescue DBS may be appropriate. The present case illustrates a good outcome after subthalamic nucleus (STN) and not after GPi DBS (considering that both were well positioned and had adequate programming). The larger dimension of the GPi and its somatotopic organization, with the stimulation outside the "face region," could explain our outcomes.

Stimulation-Induced Dyskinesia After Subthalamic Nucleus Deep Brain Stimulation in Patients With Meige Syndrome.

OBJECTIVES: Deep brain stimulation of the subthalamic nucleus (STN-DBS) is increasingly used to treat Meige syndrome (MS) and markedly improves symptoms. Stimulation-induced dyskinesia (SID), which adversely affects surgical outcomes and patient satisfaction, may, however, occur in some patients. This study attempts to explore possible causes of SID. MATERIALS AND METHODS: Retrospectively collected clinical data on 32 patients who underwent STN-DBS between October 2016 and April 2019 were analyzed. Clinical outcomes were assessed pre- and post-surgery, using the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). Patients were divided into a dyskinesia group and a non-dyskinesia group, according to whether or not they experienced persistent SID during follow-up. The coordinates of the active contacts were calculated from post-operative computerized tomography or magnetic resonance imaging, using the inter-commissural line as a reference. At final follow-up, the main stimulatory parameters for further study included pulse width, voltage, and frequency. RESULTS: At final follow-up (mean = 16.3 ± 7.2 months), MS patients had improved BFMDRS total scores compared with pre-surgical scores (mean improvement = 79.0%, p < 0.0001). The mean improvement in BFMDRS total scores in the dyskinesia (n = 10) and non-dyskinesia (n = 22) groups were 81.6 ± 8.8% and 77.9 ± 14.2%, respectively. The mean minimum voltage to induce dyskinesia was 1.7 ± 0.3 V. The programmed parameters of both groups were similar. When compared with the non-dyskinesia group, active stimulatory contact coordinates in the dyskinesia group were inferior (mean left side: z = -2.3 ± 1.7 mm vs. z = -1.2 ± 1.5 mm; p = 0.0282; mean right side: z = -2.7 ± 1.9 mm vs. z = -2.3 ± 1.7 mm; p = 0.0256). The x and y coordinates were similar. CONCLUSION: STN-DBS is an effective intervention for MS, providing marked improvements in clinical symptoms; SID may, however occur in the subsequent programming control process. Comparing patients with/without dyskinesia, the active contacts were located closer to the inferior part of the STN in patients with dyskinesia, which may provide an explanation for the dyskinesia.

Subthalamic Nucleus Deep Brain Stimulation in Primary Meige Syndrome: A 1-Year Follow-Up Study.

OBJECTIVE: To investigate the efficacy of bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) in patients with Meige syndrome. MATERIALS AND METHODS: Fifteen consecutive patients who underwent STN-DBS at the Peking University People's Hospital between September 2017 and June 2018 were included in this study. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score and the BFMDRS disability score were obtained prior to surgery, and at specific time points after surgery. Patients' sleep status was also assessed before and after surgery. RESULTS: The BFMDRS movement scores decreased from 15.3 ± 4.6 to 5.2 ± 6.2 after STN-DBS, with a mean improvement of 68.6% (p < 0.05). The BFMDRS disability scores were also significantly decreased, from 6.9 ± 3.3 to 3.5 ± 2.9, with a mean improvement of 51.7% (p < 0.05). The eye, mouth, speech, and swallowing movement scores also decreased significantly after STN-DBS compared to baseline (p < 0.05). The sleep quality of the patients was also improved after surgery. CONCLUSIONS: These findings demonstrate that the STN is an effective brain target for the treatment of patients with Meige syndrome. STN-DBS was not only able to improve patients' motor symptoms, but also their sleep status.

Similar Long-Term Clinical Outcomes of Deep Brain Stimulation With Different Electrode Targets for Primary Meige Syndrome: One Institution's Experience of 17 Cases.

OBJECTIVE: Deep brain stimulation (DBS) is considered an effective and safe treatment for patients with primary Meige syndrome (MS). Both the subthalamic nucleus (STN) and globus pallidus pars internus (Gpi) have been shown to be optional targets for electrode implantation to improve clinical symptoms, but the relationship between clinical outcomes and target is still unclear. The current study aims to compare the clinical outcomes of DBS with different electrode targets for primary MS. MATERIALS AND METHODS: We performed a retrospective study to assess the clinical outcomes for 17 consecutive patients with primary MS in Wuhan Union Hospital from January 2016 to September 2019. Six patients were treated by Gpi-DBS and 11 patients were treated by STN-DBS. All patients were assessed before surgery and at the last follow-up after surgery. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) including the movement and disability scales was used to evaluate the dystonia severity of the eyes, the mouth, speech, and swallowing. The median follow-up duration was 30.1 ± 13.1 months (range 6 months-52 months). RESULTS: In our study, DBS improved the BFMDRS-M scores by 70.52 ± 7.45% and the BFMDRS-D scores by 70.51 ± 8.38% for patients with MS. STN-DBS and Gpi-DBS had similar effects not only on the BFMDRS-M and BFMDRS-D scores, but also on the subitems including eyes, mouth, speech, and swallowing. The stimulation voltage for the Gpi was significantly higher than that for the STN. The improvements were similar in the general anesthesia and local anesthesia groups (p > 0.05). CONCLUSION: The curative effects of STN-DBS and Gpi-DBS on patients with primary MS are similar. Both the STN and Gpi could be effective targets of DBS for primary MS.

Pallidal deep brain stimulation in primary Meige syndrome: clinical outcomes and psychiatric features.

OBJECTIVES: To study the efficacy and safety of bilateral globus pallidus internus deep brain stimulation (GPi-DBS) in refractory Meige syndrome (MS) and evaluate the psychiatric disorders before and after surgery. METHODS: Twenty-two patients with MS treated with bilateral GPi-DBS were retrospectively analysed before surgery and after continuous neurostimulation. Before surgery, patients were assessed by the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), Self-Rating Depression Scale, Medical Outcomes Study 36-Item Short-Form General Health Survey (SF-36) and Pittsburgh Sleep Quality Index (PQSI), which corresponded to motor symptoms, depressive state, quality of life and sleep quality, respectively. The implantable pulse generator of each patient was activated at 1 month after surgery. At 1 month, 3 months, 6 months and 12 months after continuous neurostimulation, all patients were evaluated by the same scales above. RESULTS: The BFMDRS movement scores decreased from 15.0±5.3 before surgery to 3.5±4.5 at 12 months after neurostimulation, with a mean improvement of 78% (p<0.001). The BFMDRS disability scores improved from 7.4±4.9 before surgery to 4.0±4.6 at 12 months after neurostimulation, with a mean improvement of 56% (p<0.001). The postoperative SF-36 scores had the remarkable improvement compared with baseline scores. Impaired sleep quality was found in 82% of patients and depression in 64% before surgery, which didn't neither obtained amelioration after continuous neurostimulation. CONCLUSIONS: Bilateral pallidal neurostimulation is a beneficial therapeutic option for refractory MS, which could improve the motor symptoms except for depression and sleep quality.