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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 -
Clinical Parkinsonism & Related... 2021Dyspnoea is rarely mentioned in the clinical description of adult-onset isolated dystonia. In this study, we present the clinical features of 13 patients with Meige...
BACKGROUND
Dyspnoea is rarely mentioned in the clinical description of adult-onset isolated dystonia. In this study, we present the clinical features of 13 patients with Meige syndrome (cranio-cervical dystonia) with breathing difficulties.
METHODS
A retrospective case note review was performed of patients presenting with Meige syndrome and shortness of breath, to a neuro-laryngology MDT clinic.
RESULTS
Some patients were severely limited by their breathlessness, but others did not volunteer these symptoms. The majority of patients were referred with the assumption that the larynx was the cause of the problem; however half the patients did not have evidence of laryngeal involvement. Of the patients who had laryngeal involvement, injecting the larynx alone did not always relieve the dyspnoea. The majority of our patients responded to injection of the suprahyoid muscles, including genioglossus, digastric and mylohyoid.
CONCLUSION
We recommend routinely establishing if the patient with Meige syndrome has signs or symptoms of breathlessness, and establishing the level of the problem, as this can be treated successfully.
PubMed: 34541486
DOI: 10.1016/j.prdoa.2021.100106 -
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 -
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 -
Scientific Reports Aug 2021To contribute to the understanding of the aetiology and pathogenesis of Meige syndrome, the metabolic networks of patients with Meige syndrome were investigated using...
To contribute to the understanding of the aetiology and pathogenesis of Meige syndrome, the metabolic networks of patients with Meige syndrome were investigated using 18F-fluoro-D-glucose positron emission tomography (18F-FDG-PET) imaging of cerebral glucose metabolism. Fifty right-handed and unmedicated primary Meige syndrome patients enrolled between September 2017 and September 2020 at the Department of Neurosurgery, Peking University People's Hospital, and 50 age- and sex-matched healthy control subjects participated in the study. Metabolic connectivity and graph theory analysis were used to investigate metabolic network differences based on 18F-FDG-PET images. Glucose hypometabolism was detected in the left internal globus pallidus and parietal lobe, right frontal lobe and postcentral gyrus, and bilateral thalamus and cerebellum of patients with Meige syndrome. Clustering coefficients (Cps) (density threshold: 16-28%; P < 0.05) and shortest path lengths (Lps) (density threshold: 10-15%; P < 0.05) were higher in Meige syndrome patients than in healthy controls. Small-worldness was lower in Meige syndrome patients than in healthy controls, and centrality was significantly lower in the right superior occipital gyrus and pallidum and higher in the right thalamus. Hypometabolism in the globus pallidus and thalamus may indicate basal ganglia-thalamocortical motor circuit abnormalities as a pathogenic mechanism of Meige syndrome, providing a possible explanation for the efficacy of deep brain stimulation (DBS) in improving symptoms. Meige syndrome patients had abnormal small-world properties. Centrality changes in the right pallidus and thalamus verified the important roles of these regions in the pathogenesis of Meige syndrome.
Topics: Adult; Aged; Brain; Case-Control Studies; Female; Glucose; Humans; Male; Meige Syndrome; Metabolic Networks and Pathways; Metabolome; Middle Aged; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 34344985
DOI: 10.1038/s41598-021-95333-8 -
Movement Disorders Clinical Practice Jul 2021
PubMed: 34307753
DOI: 10.1002/mdc3.13233 -
Medicina Oral, Patologia Oral Y Cirugia... Sep 2021This retrospective study aimed in the evaluation of oral health and oral health-related quality of life (OHRQoL) of patients with oral dystonia (OD).
BACKGROUND
This retrospective study aimed in the evaluation of oral health and oral health-related quality of life (OHRQoL) of patients with oral dystonia (OD).
MATERIAL AND METHODS
Seventeen patients with OD (Meige Syndrome: n=11, Oromandibular Dystonia: n=6) were included, of which seven were examined again at three months after botulinum toxin injection. OHRQoL was assessed by the German short form of oral health impact profile (OHIP G14). Within oral examination, dental parameters, remaining teeth and periodontitis severity were assessed. A matched healthy control (HC) was composed for comparison.
RESULTS
The OD patients had significantly more carious teeth (0.94 ± 1.75 vs. 0; p<0.01), less remaining teeth (15.65 ± 9.89 vs. 22.22 ± 5.91; p=0.01) and higher dental treatment need than the HC (42.9% vs. 0%; p<0.01). The OHIP G14 sum score of 9.47± 9.82 vs. 1.58 ± 2.79 (p<0.01) as well its dimensions psychosocial impact (4.47 ± 6.45 vs. 0.53 ± 1.16; p=0.03) and oral function (4.35 ± 2.98 vs. 0.47 ± 1.34; p<0.01) were clinically relevant and statistically significant higher in OD compared to HC group. No significant differences could be detected at three months after botulinum toxin injection.
CONCLUSIONS
Patients with OD suffer from more dental diseases and have a worse OHRQoL than HC. Dental special care appears recommendable and should be fostered by everyone, who is involved in the treatment of patients with OD.
Topics: Cross-Sectional Studies; Diagnosis, Oral; Dystonia; Humans; Oral Health; Quality of Life; Retrospective Studies; Surveys and Questionnaires
PubMed: 34023846
DOI: 10.4317/medoral.24479 -
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 2021Meige syndrome (MS) is cranial dystonia characterized by the combination of upper and lower cranial involvement and including binocular eyelid spasms (blepharospasm;... (Review)
Review
Meige syndrome (MS) is cranial dystonia characterized by the combination of upper and lower cranial involvement and including binocular eyelid spasms (blepharospasm; BSP) and involuntary movements of the jaw muscles (oromandibular dystonia; OMD). The etiology and pathogenesis of this disorder of the extrapyramidal system are not well-understood. Neurologic and ophthalmic examinations often reveal no abnormalities, making diagnosis difficult and often resulting in misdiagnosis. A small proportion of patients have a family history of the disease, but to date no causative genes have been identified to date and no cure is available, although botulinum toxin A therapy effectively mitigates the symptoms and deep brain stimulation is gaining increasing attention as a viable alternative treatment option. Here we review the history and progress of research on MS, BSP, and OMD, as well as the etiology, pathology, diagnosis, and treatment.
PubMed: 33854473
DOI: 10.3389/fneur.2021.630221 -
Brain Stimulation 2021Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been shown to be a safe and effective alternative therapy for ameliorating medically refractory...
OBJECTIVE
Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been shown to be a safe and effective alternative therapy for ameliorating medically refractory primary Meige syndrome. However, the associations between DBS target position and surrounding electrophysiological properties as well as patients' clinical outcomes remains largely unknown. In a large number of patients, we investigated electrophysiological features around stimulation targets and explored their roles in predicting clinical outcomes following bilateral GPi-DBS.
METHODS
The locations of DBS active contacts along the long axis of the GPi in a standard space were calculated and compared among three groups with different clinical outcomes. The firing rates of individual neurons within the GPi were calculated for each patient and compared across the three groups.
RESULTS
Compared with the bad group (poor clinical outcome), active contacts in the good group (good clinical outcome) and the best group (best clinical outcome) were located in the more posterior GPi. The average firing rates in the good and best groups were significantly higher than in the bad group, and this difference was pronounced within the ventral GPi. For the bad group, the average firing rates were significantly lower in the ventral than in the dorsal GPi.
CONCLUSIONS
This study suggests that DBS of the posterior GPi may produce better clinical outcomes during primary Meige syndrome treatment and that higher GPi neuronal activity, particularly within the ventral part, can be used as a biomarker to guide DBS electrode implantation during surgery.
Topics: Deep Brain Stimulation; Electrodes; Globus Pallidus; Humans; Meige Syndrome; Treatment Outcome
PubMed: 33848676
DOI: 10.1016/j.brs.2021.04.005