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Scientific Reports Nov 2022To 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...
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.
Topics: Humans; Meige Syndrome; Globus Pallidus; Deep Brain Stimulation; Treatment Outcome; Dystonia
PubMed: 36411289
DOI: 10.1038/s41598-022-23156-2 -
Clinical Parkinsonism & Related... 2022Oromandibular dystonia (OMD) is a form of focal dystonia that involves the masticatory, lower facial, labial, and lingual musculature. It is a disabling disorder which... (Review)
Review
Oromandibular dystonia (OMD) is a form of focal dystonia that involves the masticatory, lower facial, labial, and lingual musculature. It is a disabling disorder which had limited treatment options until the recent introduction of botulinum toxin (BoNT) as the recommended first-line therapy by most experts and evidence-based literature. Owing to the complex relationship between the muscles of mastication and surrounding muscles, there is a wide variety of dynamic clinical presentations, making clinical recognition and the corresponding approach to BoNT injection therapy difficult. In this review, the authors provide a framework for practical clinical approaches, beginning with the recognition of clinical subtypes of OMD (jaw-opening, jaw-closing, jaw-deviating, lingual, -oral, and/or pharyngeal dystonias), followed by patient selection and clinical evaluation to determine function interferences, with injection techniques illustrated for each subtype. Careful stepwise planning is recommended to identify the muscles that are primarily responsible and employ a conservative approach to dosing titration. Treating physicians should be diligent in checking for adverse events, especially for the first few injection cycles, as muscles involved in OMD are small, delicate, and situated in close proximity. It is recommended that future studies should aim to establish the clinical efficacy of each subtype, incorporating muscle targeting techniques and patient-centred outcome measures that are related to disturbed daily functions.
PubMed: 36033904
DOI: 10.1016/j.prdoa.2022.100160 -
The British Journal of Ophthalmology Jun 1997
Topics: Botulinum Toxins, Type A; Humans; Keratoconjunctivitis Sicca; Meige Syndrome; Sjogren's Syndrome
PubMed: 9274400
DOI: 10.1136/bjo.81.6.426 -
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 -
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 -
Frontiers in Neuroscience 2021Uric acid (URIC) is a natural antioxidant, and it has been shown that low levels of URIC could be a risk factor for the development of Parkinson's disease. Our aim was...
Uric acid (URIC) is a natural antioxidant, and it has been shown that low levels of URIC could be a risk factor for the development of Parkinson's disease. Our aim was to investigate whether URIC also plays a role in Meige's syndrome (MS). We conducted a cohort study to compare serum URIC levels between patients with MS and healthy controls. In addition, we analyzed the impact of URIC on the risk of MS and symptom severity. Compared with normal subjects, URIC content was remarkably decreased in MS patients. In addition, URIC was regarded as a protective factor for MS, as verified by multivariate logistic regression models. We also found non-linear relationships between the levels of serum URIC and the incidence rate of MS and the Burke-Fahn-Marsden dystonia rating scale score. Our study is the first to show a connection between serum URIC levels and MS. Low serum URIC levels indicate an increased risk of MS incidence and more severe clinical symptoms. Our findings provide new insights into the prevention and treatment of MS.
PubMed: 34658782
DOI: 10.3389/fnins.2021.755056 -
Revue Medicale de Liege Sep 2021Blepharospasm is a particularly distressing form of focal dystonia, specifically affecting the eyelids and causing involuntary closing of the eyes or an increased...
Blepharospasm is a particularly distressing form of focal dystonia, specifically affecting the eyelids and causing involuntary closing of the eyes or an increased blinking. Despite its frequency, the diagnosis of this condition is often delayed. Diagnostic criteria are mainly clinical, but sometimes further investigations may be necessary as blepharospasm may be included in a complex clinical picture.
Topics: Blepharospasm; Humans
PubMed: 34477342
DOI: No ID Found -
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 -
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