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Frontiers in Aging Neuroscience 2022The subthalamic nucleus (STN) has been shown to be a safe and effective deep brain stimulation (DBS) surgical target for the treatment of Meige syndrome. The aim of this...
OBJECTIVES
The subthalamic nucleus (STN) has been shown to be a safe and effective deep brain stimulation (DBS) surgical target for the treatment of Meige syndrome. The aim of this study was to compare changes in brain metabolism before and 6 months after STN-DBS surgery.
METHODS
Twenty-five patients with primary Meige syndrome underwent motor function assessment, including the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability subscale (BFMDRS-D) and positron emission tomography with an 18[F]-fluorodeoxyglucose scan before and 6 months after STN-DBS surgery. For the voxelwise metabolic change assessment, the -value was controlled for multiple comparisons using the familywise error rate.
RESULTS
There was a significant decrease in BFMDRS-M scores 6 months after STN-DBS, from 10.02 ± 3.99 to 4.00 ± 2.69 ( < 0.001). The BFMDRS-D scores also decreased significantly from 4.52 ± 2.90 to 0.64 ± 1.29 ( < 0.001). In the left hemisphere, hypermetabolism was found in the occipital lobe, superior parietal gyrus, postcentral gyrus and thalamus. In the right hemisphere, hypermetabolism was found in the lentiform nucleus, precuneus and precentral gyrus in patients with Meige syndrome receiving DBS. In addition, the bilateral inferior temporal gyrus and middle frontal gyrus exhibited glucose hypermetabolism.
CONCLUSION
Our findings indicate that STN-DBS has a significant effect on metabolic level in the brain, which may be an important mechanism for the treatment of Meige syndrome using STN-DBS.
PubMed: 35370610
DOI: 10.3389/fnagi.2022.848100 -
Journal of Neurology Jan 2020Thirty years after their approval, botulinum toxin injections still are the first-line therapy for blepharospasm. The aim of our study was to analyze long-term data... (Observational Study)
Observational Study
INTRODUCTION
Thirty years after their approval, botulinum toxin injections still are the first-line therapy for blepharospasm. The aim of our study was to analyze long-term data concerning safety and efficacy in a large cohort over decades.
METHODS
Treatment data of all patients with blepharospasm and Meige´s syndrome in our outpatient clinic having undergone at least three subsequent treatment sessions with current onabotulinumtoxinA or abobotulinumtoxin A were analyzed with respect to the course of dose, effect duration, side effects, patients´ satisfaction and occurrence/reasons for treatment discontinuation.
RESULTS
The observation period was up to 18 years for onabotulinumtoxinA and 29 years for abobotulinumtoxinA with a total of 1778 and 9319 treatment sessions in 69 patients with onabotulinumtoxinA, 281 with abobotulinumtoxin A and 2 of these having used both products. The dose increased in the first years followed by a stable dose in the following years. The mean dose was 39.1/198.7 mouse units (onabotulinumtoxinA/abobotulinumtoxinA). In over 25% of all sessions, inhibition of the eyelid opening was effectively treated with pretarsal injections. The most common adverse events included ptosis (4%/5%), epiphora/sicca (4%/5%), double vision (1%/1%) and facial asymmetry (1%/1%). Reasons for therapy discontinuation were change to a nearby doctor, age, other diseases, spontaneous improvement, side effects or possible treatment failure. Only one patient was tested positive for neutralizing antibodies against botulinum toxin A.
CONCLUSION
The treatment of blepharospasm and Meige's syndrome with onabotulinumtoxinA and abobotulinumtoxinA is safe and effective, also over a long observation period of up to 29 years.
Topics: Aged; Blepharospasm; Botulinum Toxins, Type A; Female; Humans; Longitudinal Studies; Male; Meige Syndrome; Middle Aged; Neuromuscular Agents; Outcome Assessment, Health Care
PubMed: 31630241
DOI: 10.1007/s00415-019-09581-w -
Neuroreport Feb 2023Meige's syndrome and hemifacial spasm (HFS) are two different forms of dystonic movement disorder, but their difference in terms of resting state functional connectivity...
Meige's syndrome and hemifacial spasm (HFS) are two different forms of dystonic movement disorder, but their difference in terms of resting state functional connectivity (rsFC) remains unclear. The present study applied resting state fMRI on the patients and quantified their functional connectivity with graph theoretical measures, including the degree centrality and the betweenness centrality. Fifteen Meige's syndrome patients and 19 HFS patients matched in age and gender were recruited and their MRI data were collected. To analyze the rsFC, we adopted the Anatomical Automatic Labeling (AAL) template, a brain atlas system including 90 regions of interest (ROIs) covering all the brain regions of cerebral cortex. For each participant, the time-course of each ROI was extracted, and the corresponding degree centrality and betweenness centrality of each ROI were computed. These measures were then compared between the Meige's syndrome patients and the HFS patients. Meige's syndrome patients showed higher betweenness centrality and degree centrality of bilateral superior medial frontal cortex, the left cerebellum cortex, etc. than the HFS patients. Our results suggest that the rsFC pattern in Meige's syndrome patients might become more centralized toward the prefrontal and vestibular cerebellar systems, indicating less flexibility in their functional connections. These results preliminarily revealed the characteristic abnormality in the functional connection of Meige's patients and may help to explore better treatment.
Topics: Humans; Hemifacial Spasm; Meige Syndrome; Dystonic Disorders; Movement; Cerebellum
PubMed: 36608166
DOI: 10.1097/WNR.0000000000001865 -
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 -
Dystonia (Lausanne, Switzerland) 2022Blepharospasm is a type of dystonia where the diagnosis is often delayed because its varied clinical manifestations are not well recognized. The purpose of this study...
OBJECTIVE
Blepharospasm is a type of dystonia where the diagnosis is often delayed because its varied clinical manifestations are not well recognized. The purpose of this study was to provide a comprehensive picture of its clinical features including presenting features, motor features, and non-motor features.
METHODS
This was a two-part study. The first part involved a systematic literature review that summarized clinical features for 10,324 cases taken from 41 prior reports. The second part involved a summary of clinical features for 884 cases enrolled in a large multicenter cohort collected by the Dystonia Coalition investigators, along with an analysis of the factors that contribute to the spread of dystonia beyond the periocular region.
RESULTS
For cases in the literature and the Dystonia Coalition, blepharospasm emerged in the 50s and was more frequent in women. Many presented with non-specific motor symptoms such as increased blinking (51.9%) or non-motor sensory features such as eye soreness or pain (38.7%), photophobia (35.5%), or dry eyes (10.7%). Non-motor psychiatric features were also common including anxiety disorders (34-40%) and depression (21-24%). Among cases presenting with blepharospasm in the Dystonia Coalition cohort, 61% experienced spread of dystonia to other regions, most commonly the oromandibular region and neck. Features associated with spread included severity of blepharospasm, family history of dystonia, depression, and anxiety.
CONCLUSIONS
This study provides a comprehensive summary of motor and non-motor features of blepharospasm, along with novel insights into factors that may be responsible for its poor diagnostic recognition and natural history.
PubMed: 36248010
DOI: 10.3389/dyst.2022.10359 -
Annals of Indian Academy of Neurology 2021Non-motor symptoms are an essential cause of comorbidity in generalized and focal dystonia. However, there are few studies on dystonia involving the craniofacial regions.
INTRODUCTION
Non-motor symptoms are an essential cause of comorbidity in generalized and focal dystonia. However, there are few studies on dystonia involving the craniofacial regions.
METHODS
We studied non-motor symptoms in patients with oromandibular dystonia (OMD) and Meige syndrome using a questionnaire, and validated instruments for depression, anxiety, REM behaviour disorder, restless leg syndrome, sleep quality, excessive daytime sleepiness, and self-esteem. The severity of dystonia and blepharospasm was also studied.
RESULTS
Nineteen patients with OMD were recruited into the study. Among patients with OMD, depression was seen in 63.6% ( = 7), sleep impairment in 27.3% ( = 3), excessive daytime sleepiness in 27.3% ( = 3), and poor self- esteem in 18.2% ( = 2) of the patients. Among patients with Meige syndrome, depression was seen in 37.5% ( = 3), sleep impairment in 12.5% ( = 1), excessive daytime sleepiness in 25% ( = 2), low self-esteem in 25% ( = 2) of the patients.
CONCLUSION
This study highlights the significant frequency of depression and sleep disturbances in patients with idiopathic OMD and Meige syndrome.
PubMed: 34446996
DOI: 10.4103/aian.AIAN_906_20 -
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 -
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 -
Scientific Reports Sep 2020To investigate the changes and clinical significance of brain structural abnormalities in patients with Meige syndrome and related depressive symptoms. We...
To investigate the changes and clinical significance of brain structural abnormalities in patients with Meige syndrome and related depressive symptoms. We retrospectively analysed clinical data, imaging examinations, and Hamilton Depression Rating scale scores in 46 patients with Meige syndrome from January 2017 to January 2019. We compared the Meige syndrome group with the healthy control group, and the definite depression group with the non-definite depression group. Voxel-based morphometry (VBM) was used to compare grey matter (GM) volumes. We conducted two-sample t-tests corrected for subject age and gender. We tested at a level of significance of p < 0.001 with a false discovery rate (FDR) correction. VBM demonstrated decreased GM volume (p < 0.001 and cluster size > 50 voxels) in the left hemisphere in the middle frontal orbital gyrus, temporal pole (superior temporal gyrus) and insula and in the right hemisphere in the temporal pole (middle temporal gyrus), precuneus, inferior parietal, inferior temporal and olfactory cortices in the Meige syndrome group. Comparing VBM-MRI measures in Meige syndrome patients with and without depression, decreased GM volume was found in the left hemisphere in the cuneus and hippocampus and in the right hemisphere in the angular gyrus, middle frontal gyrus and middle occipital gyrus in the definite depression group. Unlike other dystonia studies that have suggested an involvement of the basal ganglia and motor cortex in the pathophysiology of the disorder , we believe that the precuneus is involved in the development of Meige syndrome. Additionally, our findings suggest that the hippocampus plays a role in the pathogenesis of depression in patients with Meige syndrome.
Topics: Aged; Female; Gray Matter; Humans; Magnetic Resonance Imaging; Male; Meige Syndrome; Middle Aged; Motor Cortex; Retrospective Studies
PubMed: 32884000
DOI: 10.1038/s41598-020-71479-9 -
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