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Frontiers in Neurology 2023Oscillatory patterns in local field potentials (LFPs) have been recognized as disease-specific physiomarkers, particularly in the context of Parkinson's disease and...
OBJECTIVES
Oscillatory patterns in local field potentials (LFPs) have been recognized as disease-specific physiomarkers, particularly in the context of Parkinson's disease and cervical dystonia. This characteristic oscillatory feature is currently employed in adaptive deep brain stimulation (aDBS). However, for other types of dystonia, especially Meige syndrome, a distinct physiomarker of this nature is yet to be identified.
METHODS
Local field potentials were recorded during microelectrode-guided deep brain stimulation surgery from 28 patients with primary Meige syndrome. Before surgery, the severity of patients' motor syndrome were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale-Motor (BFMDRS-M). An instantaneous oscillation detection method was employed to identify true narrowband oscillations. Subsequently, a linear mixed effects model was utilized to examine the relationship between oscillatory activities (including power amplitude and burst duration) and symptom severity.
RESULTS
The focal peaks of "oscillatory activities" detected were predominantly concentrated in the narrow theta band (4-8 Hz), constituting 81.5% of the total detected oscillations in all recording sites near active DBS contacts in the globus pallidus internus (GPi). The linear mixed effects model revealed a positive correlation between the theta burst duration and the severity of preoperative motor impairment, but no correlation with postoperative motor scores. Additionally, there was no significant lateralization effect observed between the left and right GPi.
CONCLUSION
Our findings suggest that the exaggerated narrowband theta activity (mainly the burst duration) in the GPi is predictive of dystonia symptom severity and may be used as a physiomarker for optimized DBS target during surgery and adaptive DBS for the treatment of Meige syndrome.
PubMed: 38178893
DOI: 10.3389/fneur.2023.1286634 -
Brain Stimulation 2023
Topics: Humans; Meige Syndrome; Pallidotomy; Globus Pallidus; Dystonic Disorders; Deep Brain Stimulation; Treatment Outcome
PubMed: 37774913
DOI: 10.1016/j.brs.2023.09.023 -
Toxins Oct 2023(1) Background: The first-line treatment for patients with focal or segmental dystonia with a craniocervical distribution is still the intramuscular injection of... (Review)
Review
(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.
Topics: Humans; Botulinum Toxins; Deep Brain Stimulation; Dystonic Disorders; Meige Syndrome; Randomized Controlled Trials as Topic; Retrospective Studies; Torticollis; Treatment Outcome
PubMed: 37888637
DOI: 10.3390/toxins15100606 -
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 -
Heliyon Apr 2024Individual differences were observed in the clinical efficacy of Botulinum toxin A (BoNT-A) in the treatment of the primary Meige syndrome. Our study aimed to explore...
OBJECTIVE
Individual differences were observed in the clinical efficacy of Botulinum toxin A (BoNT-A) in the treatment of the primary Meige syndrome. Our study aimed to explore the potential associations between the clinical efficacy of BoNT-A in the treatment of the primary Meige syndrome and variants of , and , which are involving in the translocation of the BoNT-A in vivo.
METHODS
Patients with the primary Meige syndrome treated with BoNT-A were enrolled. Clinical efficacy was evaluated by the maximum improvement rate of motor symptoms and the duration of efficacy. Variants of , and were obtained by Sanger sequencing. Another cohort diagnosed with primary cervical dystonia was also enrolled in the replication stage.
RESULTS
Among the 104 primary Meige syndrome patients, 80 patients (76.9%) had a good efficacy (the maximum improvement rate of motor symptoms ≥30%) and 24 (23. 1%) had a poor (the maximum improvement rate of motor symptoms <30%). As to the duration of efficacy, 52 patients (50.0%) had a long duration of efficacy (≥4 months), and 52 (50.0%) had a short (<4 months). In terms of primary Meige syndrome, rs6104571 was found associating with the maximum improvement rate of motor symptoms (Genotype: = 0.02, OR = 0.26; Allele: = 0.013, OR = 0.29), and rs31244 was found associating with the duration of efficacy (Genotype: = 0.024, OR = 0.13; Allele: = 0.012, OR = 0.13). Besides, we also conducted the association analyses between the variants and BoNT-A-related adverse reactions. Although, there was no statistical difference between the allele of rs31244 and BoNT-A-related adverse reactions, there was a trend ( = 0.077, OR = 2.56). In the replication stage, we included 39 patients with primary cervical dystonia to further expanding the samples' size. Among the 39 primary cervical dystonia patients, 25 patients (64.1%) had a good efficacy (the maximum improvement rate of motor symptoms ≥50%) and 14 (35.9%) had a poor (the maximum improvement rate of motor symptoms <50%). As to the duration of efficacy, 32 patients (82.1%) had a long duration of efficacy (≥6 months), and 7 (17.9%) had a short (<6 months). Integrating primary Meige syndrome and primary cervical dystonia, rs31244 was still found associating with the duration of efficacy (Genotype: = 0.002, OR = 0. 23; Allele: = 0.001, OR = 0. 25).
CONCLUSION
In our study, rs6104571 was associated with the maximum improvement rate of motor symptoms in patients with primary Meige syndrome treated with BoNT-A, and patients carrying this variant had a lower improvement rate of motor symptoms. rs31244 was associated with duration of treatment in patients with primary Meige syndrome treated with BoNT-A and patients carrying this variant had a shorter duration of treatment. Patients with primary Meige syndrome carrying rs31244 G allele have an increase likelihood of BoNT-A-related adverse reactions. Involving 39 patients with primary cervical dystonia, the results further verify that rs31244 was associated with duration of treatment and patients carrying this variant had a shorter duration of treatment.
PubMed: 38628704
DOI: 10.1016/j.heliyon.2024.e28543 -
Arquivos de Neuro-psiquiatria Apr 2024Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type...
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 ( < 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.
Topics: Humans; Male; Female; Aged; Botulinum Toxins, Type A; Blepharospasm; Dystonia; Meige Syndrome; Dystonic Disorders
PubMed: 38641339
DOI: 10.1055/s-0044-1785691 -
Structural network topologies are associated with deep brain stimulation outcomes in Meige syndrome.Neurotherapeutics : the Journal of the... Apr 2024Deep 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...
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.
PubMed: 38679556
DOI: 10.1016/j.neurot.2024.e00367 -
Radiology Case Reports Sep 2023Multiple system atrophy (MSA) is a rare form of adult-onset α-synucleinopathy. Meige syndrome, identified as bilateral blepharospasm and oromandibular dystonia, is a...
Multiple system atrophy (MSA) is a rare form of adult-onset α-synucleinopathy. Meige syndrome, identified as bilateral blepharospasm and oromandibular dystonia, is a type of focal dystonic movement disorder. This case report aims to highlight the clinical features of multiple system atrophy associated with Meige syndrome in a patient. Additionally, we aim to provide the treatment experience in a patient with Meige syndrome as this is an extremely rare clinical case.
PubMed: 37448600
DOI: 10.1016/j.radcr.2023.04.055 -
Cureus Oct 2023Oromandibular dystonia is a focal dystonia characterized by involuntary movements of the jaw, oropharynx, lips, and tongue. The diagnosis of oromandibular dystonia is...
Oromandibular dystonia is a focal dystonia characterized by involuntary movements of the jaw, oropharynx, lips, and tongue. The diagnosis of oromandibular dystonia is clinical and can be complex. For effective treatment, it is essential to understand its underlying etiology. A 70-year-old man was referred to our center with a diagnosis of Meige's syndrome, which had been present for five and a half years, for receiving botulinum toxin-A (BoNT-A) injections. Upon physical examination, he exhibited oromandibular dystonia, with a score of 177 points on the Oromandibular Dystonia Rating Scale (OMDRS). He had a history of taking methotrexate for six years, as he was diagnosed with psoriatic arthritis during that time. The possibility of methotrexate-induced dystonia was considered. A switch from methotrexate to sulfasalazine was initiated. Subsequently, the patient showed progressive improvement in his symptoms, as reflected by an OMDRS score of 103 points. After eight weeks, the medical team decided to supplement the treatment with BoNT-A injections, resulting in an OMDRS score of 75. While there is currently no definitive evidence linking the use of methotrexate to the development of dystonia, it is advisable to consider oromandibular dystonia as a potential side effect of methotrexate until more robust evidence becomes available.
PubMed: 38022297
DOI: 10.7759/cureus.47248