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Heliyon Mar 2024Globus pallidus internus (GPi) and subthalamic nucleus (STN) are two common deep brain stimulation (DBS) targets. This meta-analysis was to compared the efficacy and...
BACKGROUND
Globus pallidus internus (GPi) and subthalamic nucleus (STN) are two common deep brain stimulation (DBS) targets. This meta-analysis was to compared the efficacy and safety of these two DBS targets for the treatment of Meige syndrome (MS).
METHODS
A systematic search was performed using EMBASE, MEDLINE, the Cochrane Library, and ClinicalTrials.gov to identify DBS trials for MS. Review Manager 5.3 was used to perform meta-analysis and the mean difference (MD) was analyzed and calculated with a random effect model. Pearson's correlation coefficients and meta-regression analyses were utilized to identify relevant predictive markers.
RESULTS
Twenty trials involving 188 participants with GPi-DBS and 110 individuals with STN-DBS were eligible. Both groups showed improvement of the Burke-Fahn-Marsden Dystonia Rating Scale-Movement (BFMDRS-M) and Disability (BFMDRS-D) scores (BFMDRS-M: MD = 10.57 [7.74-13.41] for GPi-DBS, and MD = 8.59 [4.08-13.11] for STN-DBS; BFMDRS-D: MD = 5.96 [3.15-8.77] for GPi-DBS, and MD = 4.71 [1.38-8.04] for STN-DBS; all < 0.001) from baseline to the final follow-up, while no notable disparity in improvement rates was observed between them. Stimulation-related complications occurrence was also similar between two groups (38.54 ± 24.07% vs. 43.17 ± 29.12%, P = 0.7594). Simultaneously, preoperative BFMDRS-M score and disease duration were positively connected with the relative changes in BFMDRS-M score at the final visit.
CONCLUSION
Both GPi-DBS and STN-DBS are effective MS therapies, with no differences in efficacy or the frequency of stimulation-related problems. Higher preoperative scores and longer disease duration probably predict greater improvement.
PubMed: 38510025
DOI: 10.1016/j.heliyon.2024.e27945 -
Scientific Reports Apr 2021Deep-brain stimulation (DBS) is an effective treatment for patients with Meige syndrome. The globus pallidus interna (GPi) and the subthalamic nucleus (STN) are accepted...
Deep-brain stimulation (DBS) is an effective treatment for patients with Meige syndrome. The globus pallidus interna (GPi) and the subthalamic nucleus (STN) are accepted targets for this treatment. We compared 12-month outcomes for patients who had undergone bilateral stimulation of the GPi or STN. Forty-two Asian patients with primary Meige syndrome who underwent GPi or STN neurostimulation were recruited between September 2017 and September 2019 at the Department of Neurosurgery, Peking University People's Hospital. The primary outcome was the change in motor function, including the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability subscale (BFMDRS-D) at 3 days before DBS (baseline) surgery and 1, 3, 6, and 12 months after surgery. Secondary outcomes included health-related quality of life, sleep quality status, depression severity, and anxiety severity at 3 days before and 12 months after DBS surgery. Adverse events during the 12 months were also recorded. Changes in BFMDRS-M and BFMDRS-D scores at 1, 3, 6, and 12 months with DBS and without medication did not significantly differ based on the stimulation target. There were also no significant differences in the changes in health-related quality of life (36-Item Short-Form General Health Survey) and sleep quality status (Pittsburgh Sleep Quality Index) at 12 months. However, there were larger improvements in the STN than the GPi group in mean score changes on the 17-item Hamilton depression rating scale (- 3.38 vs. - 0.33 points; P = 0.014) and 14-item Hamilton anxiety rating scale (- 3.43 vs. - 0.19 points; P < 0.001). There were no significant between-group differences in the frequency or type of serious adverse events. Patients with Meige syndrome had similar improvements in motor function, quality of life and sleep after either pallidal or subthalamic stimulation. Depression and anxiety factors may reasonably be included during the selection of DBS targets for Meige syndrome.
Topics: Aged; Deep Brain Stimulation; Female; Globus Pallidus; Humans; Male; Meige Syndrome; Middle Aged; Retrospective Studies; Subthalamic Nucleus
PubMed: 33888857
DOI: 10.1038/s41598-021-88384-4 -
Parkinsonism & Related Disorders Jan 2019Subthalamic nucleus (STN) deep brain stimulation (DBS) has recently been shown to be an effective treatment for Meige syndrome but efficacy of symptomatic improvement...
BACKGROUND
Subthalamic nucleus (STN) deep brain stimulation (DBS) has recently been shown to be an effective treatment for Meige syndrome but efficacy of symptomatic improvement and its relationship to factors in DBS remains to be explored.
OBJECTIVES
This study explored the relationship of electrode contact location in bilateral STN-DBS with clinical efficacy in Meige syndrome patients through retrospective analysis.
METHODS
Pre- and post-operative magnetic resonance (MR) images of Meige syndrome patients (n = 15) were analysed. Clinical outcomes were evaluated with the Burke-Fahn-Marsden Dystonia Scale (BFMDRS). The location of active contacts in Montreal Neurological Institute (MNI) standard space and volume of activated STN tissue were determined and related to clinical outcomes.
RESULTS
At the last follow up (mean = 14.8 ± 4.0 months; range = 11-24 months), Meige syndrome patients (n = 14) showed improved BFMDRS scores (mean improvement = 70.9%, p = 0.001) compared to pre-operative assessment. Active contacts of stimulation given from coordinates in the MNI space (mean left side: x = -12.5 ± 1.2 mm, y = -13.3 ± 1.7 mm, z = -5.5 ± 2.5 mm; mean right side: x = 12.7 ± 1.4 mm, y = -12.7 ± 1.7 mm, z = -6.4 ± 2.4 mm) were found mainly clustered in the dorsolateral STN. While there were no significant differences in patients grouped by their degree of symptomatic improvement (<30%, 30-70% and >70%) with their respective coordinates, the volume of activated tissue within the STN of patients was significantly correlated to the BFMDRS improvement (R = 0.6, p = 0.02).
CONCLUSIONS
These findings further support the stimulation of the dorsolateral STN for effective alleviation of symptoms in Meige syndrome patients and indicate that specific factors of DBS can be considered to predict clinical efficacy.
Topics: Adult; Aged; Deep Brain Stimulation; Electrodes, Implanted; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Meige Syndrome; Middle Aged; Outcome Assessment, Health Care; Subthalamic Nucleus
PubMed: 30217540
DOI: 10.1016/j.parkreldis.2018.05.014 -
Neuromodulation : Journal of the... Dec 2016The aim of this study was to report on four patients with craniocervical dystonia (CCD) treated with deep brain stimulation (DBS). In addition, we investigated the... (Review)
Review
OBJECTIVES
The aim of this study was to report on four patients with craniocervical dystonia (CCD) treated with deep brain stimulation (DBS). In addition, we investigated the treatment efficacy and surgical outcome predictors by the review and analysis of previously published studies.
METHODS
Four patients with CCD underwent DBS of the globus pallidus internus (Gpi) or subthalamus nucleus (STN). PubMed and MEDLINE searches were performed to obtain detailed information on patients who underwent DBS for CCD. The primary efficacy endpoint was the change in the Burke-Fahn-Marsden Dystonia Rating Scale (movement and disability scores, BFMDRS-M/D) after surgery.
RESULTS
Seventy-five patients were included in the pooled analysis, including 69 patients with Gpi-DBS and 6 patients with STN-DBS. The mean follow-up of time was 28.0 months after surgery. The mean BFMDRS-M score was 24.5 ± 11.2 preoperatively and 8.1 ± 5.7 postoperatively at the final follow-up evaluation, with a mean improvement of 66.9% (p < 0.001). The mean BFMDRS-D score was 8.1 ± 4.6 preoperatively and 3.6 ± 2.5 postoperatively, with a mean percentage improvement of 56.0% (p < 0.01). Positive correlations were found between each of the preoperative movement and disability scores and percentage of postoperative improvement (r = 0.247, p = 0.034; r = 0.331, p = 0.034, respectively).
CONCLUSION
GPi/STN-DBS is an effective treatment for patients with medically refractory CCD, including those with severe preoperative symptoms. The age at CCD onset and the disease duration do not predict improvement in movement scores.
Topics: Adult; Aged; Craniofacial Abnormalities; Databases, Bibliographic; Deep Brain Stimulation; Female; Globus Pallidus; Humans; Male; Middle Aged; Osteochondrodysplasias; Subthalamic Nucleus; Treatment Outcome
PubMed: 26399458
DOI: 10.1111/ner.12345 -
Journal of Neurology, Neurosurgery, and... Dec 2020To study the efficacy and safety of bilateral globus pallidus internus deep brain stimulation (GPi-DBS) in refractory Meige syndrome (MS) and evaluate the psychiatric...
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.
Topics: Aged; Articulation Disorders; Deep Brain Stimulation; Deglutition Disorders; Depression; Dizziness; Female; Gait Disorders, Neurologic; Globus Pallidus; Humans; Hypesthesia; Implantable Neurostimulators; Male; Meige Syndrome; Middle Aged; Postoperative Complications; Quality of Life; Retrospective Studies; Sleep; Treatment Outcome
PubMed: 33028603
DOI: 10.1136/jnnp-2020-323701 -
Tremor and Other Hyperkinetic Movements... 2017Hyperkinetic dysarthria is characterized by abnormal involuntary movements affecting respiratory, phonatory, and articulatory structures impacting speech and... (Review)
Review
BACKGROUND
Hyperkinetic dysarthria is characterized by abnormal involuntary movements affecting respiratory, phonatory, and articulatory structures impacting speech and deglutition. Speech-language pathologists (SLPs) play an important role in the evaluation and management of dysarthria and dysphagia. This review describes the standard clinical evaluation and treatment approaches by SLPs for addressing impaired speech and deglutition in specific hyperkinetic dysarthria populations.
METHODS
A literature review was conducted using the data sources of PubMed, Cochrane Library, and Google Scholar. Search terms included 1) hyperkinetic dysarthria, essential voice tremor, voice tremor, vocal tremor, spasmodic dysphonia, spastic dysphonia, oromandibular dystonia, Meige syndrome, orofacial, cervical dystonia, dystonia, dyskinesia, chorea, Huntington's Disease, myoclonus; and evaluation/treatment terms: 2) Speech-Language Pathology, Speech Pathology, Evaluation, Assessment, Dysphagia, Swallowing, Treatment, Management, and diagnosis.
RESULTS
The standard SLP clinical speech and swallowing evaluation of chorea/Huntington's disease, myoclonus, focal and segmental dystonia, and essential vocal tremor typically includes 1) case history; 2) examination of the tone, symmetry, and sensorimotor function of the speech structures during non-speech, speech and swallowing relevant activities (i.e., cranial nerve assessment); 3) evaluation of speech characteristics; and 4) patient self-report of the impact of their disorder on activities of daily living. SLP management of individuals with hyperkinetic dysarthria includes behavioral and compensatory strategies for addressing compromised speech and intelligibility. Swallowing disorders are managed based on individual symptoms and the underlying pathophysiology determined during evaluation.
DISCUSSION
SLPs play an important role in contributing to the differential diagnosis and management of impaired speech and deglutition associated with hyperkinetic disorders.
Topics: Deglutition Disorders; Humans; Hyperkinesis; Speech Disorders; Speech-Language Pathology
PubMed: 28983422
DOI: 10.7916/D8Z32B30 -
Acta Neurochirurgica Jul 2017Meige syndrome (MS) is usually described as a combination of blepharospasm with oromandibular dystonia. There are a large number of case reports of deep brain...
Meige syndrome (MS) is usually described as a combination of blepharospasm with oromandibular dystonia. There are a large number of case reports of deep brain stimulation (DBS) of the globus pallidus internus (GPI) for MS and only one report of unilateral pallidotomy (PT). We report the first case of staged bilateral PT for treatment of a patient with MS using intraoperative high-frequency stimulation in order to predict and prevent postoperative deficit. There was a significant improvement of the Burk-Fahn-Marsden dystonia rating scale from 26 to 3. There were no adverse postoperative neurological and neuropsychological events.
Topics: Aged; Humans; Male; Meige Syndrome; Pallidotomy; Postoperative Complications
PubMed: 28424916
DOI: 10.1007/s00701-017-3178-0 -
Journal of Neurology Nov 2019Deep brain stimulation (DBS) is an effective intervention for Meige syndrome, a type of dystonia characterized by blepharospasm, facial, and oromandibular dystonia. This... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Deep brain stimulation (DBS) is an effective intervention for Meige syndrome, a type of dystonia characterized by blepharospasm, facial, and oromandibular dystonia. This individual patient-level data meta-analysis was to identify the potential outcome predictors, compare the stimulation targets and summarize the efficacy of DBS for Meige syndrome.
METHODS
Three electronic databases (PubMed, Web of Science and Embase) were searched with no publication data restriction to identify studies regarding DBS for Meige syndrome. The primary outcome was the improvement in BFMDRS-M score. Pearson's correlation coefficients and a stepwise multivariate regression analysis were used to identify the potential prognostic factors.
RESULTS
Twenty-three studies (115 patients, 94 with pallidal stimulation and 21 with subthalamic stimulation) were eligible. Patients showed significant improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) (21.5 ± 11.0 vs 8.6 ± 6.9, P < 0.001) and disability (BFMDRS-D) (6.4 ± 5.1 vs 2.9 ± 2.4, P < 0.001) scores at the last follow-up visit (31.9 ± 30.7 months), compared with scores at baseline. Preoperative BFMDRS-M and BFMDRS-D scores were positively correlated with the relative changes in BFMDRS-M score at the last follow-up visit. On the stepwise multivariate regression, only the preoperative BFMDRS remained significant in the best predictive model.
CONCLUSIONS
Based on the existing evidence, pallidal/subthalamic stimulation is an effective therapy for even the refractory Meige syndrome. Higher preoperative scores probably indicate larger improvement. Stimulation targets or other clinical factors do not constitute the outcome predictive factors.
Topics: Deep Brain Stimulation; Humans; Meige Syndrome
PubMed: 31302747
DOI: 10.1007/s00415-019-09462-2 -
Frontiers in Neuroscience 2022Meige' s syndrome, a rare form of dystonia, lacks effective treatment. The purpose of this study was to determine the effects of CT-guided percutaneous extracranial...
BACKGROUND
Meige' s syndrome, a rare form of dystonia, lacks effective treatment. The purpose of this study was to determine the effects of CT-guided percutaneous extracranial radiofrequency ablation of the facial and/or trigeminal nerves in the treatment of Meige's syndrome.
METHODS
A total of 10 patients were enrolled in this study, with the numbers of blepharospasm dystonia syndrome (BDS), oromandibular dystonia syndrome (ODS), and blepharospasm combined with oromandibular dystonia syndrome (B-ODS) being 7, 1, and 2, respectively. BDS patients underwent radiofrequency ablation of the bilateral stylomastoid foramen facial nerve; ODS patients underwent radiofrequency ablation of the bilateral foramen oval trigeminal mandibular branch, and B-ODS patients underwent radiofrequency ablation of the bilateral stylomastoid foramen facial nerve and foramen oval trigeminal mandibular branch. The therapeutic effects and complications were observed.
RESULTS
All 10 patients in this series experienced improved Meige's syndrome-related symptoms after extracranial radiofrequency ablation of the cranial and/or mandibular branches of the extracranial trigeminal nerve. Adverse events included class II-III facial paralysis and/or mandibular skin numbness. Two patients had recurrences at the 18th and 22nd months postoperatively, respectively; the other patients were being followed up.
CONCLUSION
These results shown that CT-guided radiofrequency ablation of bilateral stylomastoid foramen facial nerve and/or oval foramen trigeminal mandibular branch can effectively treat the corresponding types of Meige's syndrome. According to preliminary observations, the therapeutic effect may last more than 18 months.
PubMed: 36278012
DOI: 10.3389/fnins.2022.1013555 -
NeuroImage. Clinical 2023The physiopathologic mechanism of Meige syndrome (MS) has not been clarified, and neuroimaging studies centering on cerebellar changes in MS are scarce. Moreover, even...
BACKGROUND
The physiopathologic mechanism of Meige syndrome (MS) has not been clarified, and neuroimaging studies centering on cerebellar changes in MS are scarce. Moreover, even though deep brain stimulation (DBS) of the subthalamic nucleus (STN) has been recognized as an effective surgical treatment for MS, there has been no reliable biomarker to predict its efficacy.
OBJECTIVE
To characterize the volumetric alterations of gray matter (GM) in the cerebellum in MS and to identify GM measurements related to a good STN-DBS outcome.
METHODS
We used voxel-based morphometry and lobule-based morphometry to compare the regional and lobular GM differences in the cerebellum between 47 MS patients and 52 normal human controls (HCs), as well as between 31 DBS responders and 10 DBS non-responders. Both volumetric analyses were achieved using the Spatially Unbiased Infratentorial Toolbox (SUIT). Further, we performed partial correlation analyses to probe the relationship between the cerebellar GM changes and clinical scores. Finally, we plotted the receiver operating characteristic (ROC) curve to select biomarkers for MS diagnosis and DBS outcomes prediction.
RESULTS
Compared to HCs, MS patients had GM atrophy in lobule Crus I, lobule VI, lobule VIIb, lobule VIIIa, and lobule VIIIb. Compared to DBS responders, DBS non-responders had lower GM volume in the left lobule VIIIb. Moreover, partial correlation analyses revealed a positive relationship between the GM volume of the significant regions/lobules and the symptom improvement rate after DBS surgery. ROC analyses demonstrated that the GM volume of the significant cluster in the left lobule VIIIb could not only distinguish MS patients from HCs but also predict the outcomes of STN-DBS surgery with high accuracy.
CONCLUSION
MS patients display bilateral GM shrinkage in the cerebellum relative to HCs. Regional GM volume of the left lobule VIIIb can be a reliable biomarker for MS diagnosis and DBS outcomes prediction.
Topics: Humans; Gray Matter; Meige Syndrome; Deep Brain Stimulation; Magnetic Resonance Imaging; Cerebellum
PubMed: 36610311
DOI: 10.1016/j.nicl.2023.103316