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NeuroImage. Clinical 2022Blepharospasm is a debilitating focal dystonia characterized by involuntary eyelid spasms that can be accompanied by oromandibular muscle involvement (Meige's syndrome)....
BACKGROUND
Blepharospasm is a debilitating focal dystonia characterized by involuntary eyelid spasms that can be accompanied by oromandibular muscle involvement (Meige's syndrome). Frequently observed abnormality in functional neuroimaging hints at an important position of the thalamus, that relays involved cortico-basal ganglia-cortical and cortico-cerebello-cortical circuits, within the abnormal network in blepharospasm.
OBJECTIVE
To characterize abnormal cortico-thalamic structural/streamline connectivity (SC) patterns in the disease, as well as their potential co-occurrence with abnormal subcortico-thalamo-cortical projections using diffusion tractography.
METHODS
Diffusion imaging was obtained in 17 patients with blepharospasm (5 with mild lower facial involvement) and 17 healthy controls. Probabilistic tractography was used for quantification of SC between six cortical regions and thalamus, and voxel-level thalamic SC mapping as well as evaluation of the thalamic SC distributions' topography by center-of-gravity analysis was performed. Post-hoc, correlations of SC with clinical parameters were evaluated. Further, white matter integrity was investigated within representative segments of the dentato-thalamo-cortical and pallido-thalamo-cortical tract.
RESULTS
Connectivity mapping showed significant reduction of right (pre)motor- and left occipital-thalamic SC, as well as a topographic shift of the left occipital-thalamic SC distribution in patients. Significant positive correlation of occipital-thalamic SC with disease severity was found. Post-hoc analysis revealed significantly reduced mean fractional anisotropy in patients within the dentato-thalamo-cortical trajectory connecting to right (pre)motor and left occipital cortex.
CONCLUSION
Abnormal occipital/motor SC provides evidence for dysfunction of the thalamus-relayed visual and motor network as a key aspect in the disease. Concurrent impairment of microstructural integrity within the dentato-thalamic trajectories targeting those cortices hints at cerebellar contribution.
Topics: Basal Ganglia; Blepharospasm; Dystonic Disorders; Humans; Magnetic Resonance Imaging; Neural Pathways; Thalamus
PubMed: 35483134
DOI: 10.1016/j.nicl.2022.103013 -
Nepalese Journal of Ophthalmology : a... Jan 2021Blepharospasm is a condition of involuntary spasm of the orbicularis oculi muscle which leads to intermittent or complete closure of the eyelids. Botulinum toxin is the...
INTRODUCTION
Blepharospasm is a condition of involuntary spasm of the orbicularis oculi muscle which leads to intermittent or complete closure of the eyelids. Botulinum toxin is the currently recommended first line treatment for such blepharospasm. This study aims to find out the outcome of injection Botulinum toxin Type A in Blepharospasm.
MATERIALS AND METHODS
It was a hospital based, prospective, interventional study conducted on patients diagnosed as Benign essential blepharospasm (BEB), Meige syndrome (MS) and Hemifacial spasm (HFS) by oculoplastic surgeon at Oculoplasty department OPD, Tilganga Institute of Ophthalmology, from December 2018 to November 2019. After taking all standard precautions for botulinum toxin injections, 6 to 8 sites for injecting 2.5 to 5 IU of the toxin were given. All the patients were evaluated before and after injections according to Jankovic spasm grading and improvement in functional impairment scale and followed on one week, one month, three month and when the symptoms reappeared.
RESULTS
A total of 43 cases which included 32 cases of Benign essential Blepharospasm, 9 Hemifacial spasm and 2 Meige syndrome. The mean Jankovic severity score was 3.51 ± 0.51 (range 3-4). The mean improvement in functional score was 2.60 ± 0.54 (range 1-3), was statistically significant (p-value <0.001).The effective period of injection was 130 ± 20.82 (93 - 189) days.38 patients had repeated injections after reappearance of symptoms. 4 patients had side effects of redness and hematoma at one site.
CONCLUSION
This study concludes that Botulinum toxin type A is effective in the management of Benign essential blepharospasm, Hemifacial spasm and Meige syndrome. This along with a good safety profile justifies its role as a first line treatment therapy in blepharospasm. However, it is a temporary treatment option where the effect lasts for a short period of time and repeated injections are required.
Topics: Blepharospasm; Botulinum Toxins, Type A; Facial Muscles; Hemifacial Spasm; Humans; Prospective Studies
PubMed: 33981096
DOI: 10.3126/nepjoph.v13i1.30589 -
Clinical NeuropharmacologyMeige syndrome is a segmental form of dystonia where botulinum toxins are the preferred treatment option. However, its invasive nature, treatment costs, partial...
INTRODUCTION
Meige syndrome is a segmental form of dystonia where botulinum toxins are the preferred treatment option. However, its invasive nature, treatment costs, partial responsiveness, and benefit duration are some of their limitations.
METHODS
Six consecutive subjects with Meige syndrome were treated only with aripiprazole.
RESULTS
A dramatic response was obtained in all subjects during the first weeks of treatment. Aripiprazole mean ± SD daily dose was 7.9 ± 3.6 mg. Three subjects developed parkinsonism related to aripiprazole treatment; the former improved after reducing the dosage, without significant worsening of cranial dystonia. After a mean ± SD follow-up of 2.0 ± 0.7 years, clinical benefit persists over time, with a mean percentage reduction of Unified Dystonia Rating Score of 75.6% ± 8.4%.
CONCLUSIONS
Aripiprazole should be considered as an alternative treatment option among subjects with Meige syndrome, especially in those refractory to botulinum toxin injections. The clinical response shown in our patients may lead to treatment development.
Topics: Aripiprazole; Dystonia; Humans; Meige Syndrome; Treatment Outcome
PubMed: 34456230
DOI: 10.1097/WNF.0000000000000473 -
Journal of Neurology Nov 2017
Topics: France; History, 19th Century; History, 20th Century; Humans; Meige Syndrome; Neurology
PubMed: 28756604
DOI: 10.1007/s00415-017-8575-z -
Frontiers in Neurology 2023It has been shown in previous studies that botulinum toxin type A (BTX-A) can effectively relieve the motor symptoms of Meige syndrome. However, its effect on non-motor...
BACKGROUND
It has been shown in previous studies that botulinum toxin type A (BTX-A) can effectively relieve the motor symptoms of Meige syndrome. However, its effect on non-motor symptoms (NMS) and quality of life (QoL) has not been comprehensively studied. This study aimed to explore the effects of BTX-A on NMS and QoL and to clarify the relationship between changes in motor symptoms, NMS, and QoL after BTX-A.
METHODS
Seventy-five patients were recruited for the study. All patients were assessed by a series of clinical assessments before, one, and 3 months after BTX-A treatment. Dystonic symptoms, psychiatric disturbances, sleep disorders, and QoL were evaluated.
RESULTS
After 1 and 3 months of BTX-A treatment, the scores of motor symptoms, anxiety, and depression were significantly decreased ( < 0.05). Except for general health, the scores of the other 36-item short-form health survey QoL subitems were significantly improved after BTX-A ( < 0.05). After 1 month of treatment, the changes in anxiety and depression were not correlated with changes in motor symptoms ( > 0.05). Still, they were negatively correlated with changes in physical functioning, role-physical and mental component summary QoL ( < 0.05).
CONCLUSIONS
BTX-A effectively improved motor symptoms, anxiety, depression, and QoL. Anxiety and depression improvement did not correlate with motor symptom changes after BTX-A, and QoL improvements were strongly associated with psychiatric disturbances.
PubMed: 36846150
DOI: 10.3389/fneur.2023.1115482 -
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 -
Human Pathology Feb 2018By nature, idiopathic interstitial pneumonias have been diagnosed in a multidisciplinary manner. As classifications have been subject to significant refinement over the... (Review)
Review
By nature, idiopathic interstitial pneumonias have been diagnosed in a multidisciplinary manner. As classifications have been subject to significant refinement over the last decade, the importance of correlating clinical, radiologic, and pathologic information to arrive at a diagnosis, which will predict prognosis in any given patient, has become increasingly recognized. In 2013, the American Thoracic Society and European Respiratory Society updated the idiopathic interstitial pneumonias classification scheme, addressing the most recent updates in the field. The purpose of this review is to highlight the correlations between radiologic and pathologic findings in idiopathic interstitial pneumonias while using updated classification schemes and naming conventions.
Topics: Diagnosis, Differential; Humans; Idiopathic Interstitial Pneumonias; Meige Syndrome; Prognosis; Radiography; Treatment Outcome
PubMed: 29180253
DOI: 10.1016/j.humpath.2017.11.009 -
Neurosurgery May 2023Bilateral pallidal deep brain stimulation (DBS) has been broadly accepted as a feasible surgical procedure for treating various forms of dystonia, but its effects on...
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.
Topics: Humans; Globus Pallidus; Meige Syndrome; Dystonia; Treatment Outcome; Retrospective Studies; Deep Brain Stimulation; Quality of Life; Dystonic Disorders
PubMed: 36728352
DOI: 10.1227/neu.0000000000002335