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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 -
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 -
Altered coupling of resting-state cerebral blood flow and functional connectivity in Meige syndrome.Frontiers in Neuroscience 2023Meige syndrome (MS) is an adult-onset segmental dystonia disease, mainly manifested as blepharospasm and involuntary movement caused by dystonic dysfunction of the...
INTRODUCTION
Meige syndrome (MS) is an adult-onset segmental dystonia disease, mainly manifested as blepharospasm and involuntary movement caused by dystonic dysfunction of the oromandibular muscles. The changes of brain activity, perfusion and neurovascular coupling in patients with Meige syndrome are hitherto unknown.
METHODS
Twenty-five MS patients and thirty age- and sex-matched healthy controls (HC) were prospectively recruited in this study. All the participants underwent resting-state arterial spin labeling and blood oxygen level-dependent examinations on a 3.0 T MR scanner. The measurement of neurovascular coupling was calculated using cerebral blood flow (CBF)-functional connectivity strength (FCS) correlations across the voxels of whole gray matter. Also, voxel-wised analyses of CBF, FCS, and CBF/FCS ratio images between MS and HC were conducted. Additionally, CBF and FCS values were compared between these two groups in selected motion-related brain regions.
RESULTS
MS patients showed increased whole gray matter CBF-FCS coupling relative to HC ( = 2.262, = 0.028). In addition, MS patients showed significantly increased CBF value in middle frontal gyrus and bilateral precentral gyrus.
CONCLUSION
The abnormal elevated neurovascular coupling of MS may indicate a compensated blood perfusion in motor-related brain regions and reorganized the balance between neuronal activity and brain blood supply. Our results provide a new insight into the neural mechanism underlying MS from the perspective of neurovascular coupling and cerebral perfusion.
PubMed: 37207180
DOI: 10.3389/fnins.2023.1152161 -
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 and Behavior Jan 2018Blepharospasm is characterized by involuntary eyelid spasms. It can be associated with perioral dystonia (Meige's syndrome or orofacial dystonia). We aimed at studying...
INTRODUCTION
Blepharospasm is characterized by involuntary eyelid spasms. It can be associated with perioral dystonia (Meige's syndrome or orofacial dystonia). We aimed at studying resting-state functional brain connectivity in these patients and its potential modulation by therapeutic botulinum toxin injections.
METHODS
We performed resting-state functional MRI and a region of interest-based analysis of functional connectivity in 13 patients with blepharospasm/Meige's syndrome in comparison to 13 healthy controls. Patients were studied before and 4 weeks after botulinum toxin treatment. Simultaneous facial electromyography was applied to control for involuntary facial movements.
RESULTS
Before botulinum toxin treatment, patients showed reduced functional connectivity between caudate and primary sensorimotor, somatosensory association and visual cortices as well as between putamen and parietal association cortex. Cerebellar areas displayed decreased functional connectivity to somatosensory and visual association cortices. On the cortical level, connectivity was reduced between the cingulate cortex and the primary sensorimotor/premotor and parietal association cortex, between premotor areas and the primary somatosensory cortices, and between the postcentral gyrus and temporoparietal, secondary somatosensory, cingular, and cerebellar regions. Botulinum toxin treatment modulated functional connectivity, especially between cerebellum and visual cortices.
CONCLUSIONS
Patients with blepharospasm/Meige's syndrome show altered functional connectivity at rest in widespread brain regions including basal ganglia, cerebellar, primary/secondary sensorimotor, and visual areas. Functionally, this may reflect a predisposition for defective movement inhibition and sensorimotor integration. Botulinum toxin treatment could modulate brain connectivity in blepharospasm by altering visual and sensory input.
Topics: Aged; Blepharospasm; Botulinum Toxins; Brain; Brain Mapping; Dystonia; Dystonic Disorders; Electromyography; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neurotoxins; Treatment Outcome
PubMed: 29568690
DOI: 10.1002/brb3.894 -
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 -
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 -
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 -
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 -
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