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Neurology India 2018
Topics: Blepharospasm; Botulinum Toxins; Deep Brain Stimulation; Dystonic Disorders; Humans; Meige Syndrome; Neuromuscular Agents
PubMed: 29322952
DOI: 10.4103/0028-3886.222827 -
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 -
European Journal of Neurology Mar 2011To characterize patients with benign essential blepharospasm (BEB) by diagnosis, environmental risk factors, and family history.
BACKGROUND AND PURPOSE
To characterize patients with benign essential blepharospasm (BEB) by diagnosis, environmental risk factors, and family history.
METHODS
Two hundred and forty patients with BEB were evaluated through a clinical examination and questionnaire. The questionnaire reviewed personal medical history, demographic factors, risk factors for the development of blepharospasm and family history of dystonia and other neurological conditions.
RESULTS
Benign essential blepharospasm was more commonly found in women (2.8:1) and 93% of the patients were Caucasian. Fifty percent had pure BEB, 31% had BEB/Meige's syndrome, and 4% had BEB and eyelid opening apraxia (+/- Meige's syndrome). A minority of patients reported preceding photophobia (25%) or other eye conditions (22%). The majority were non-smokers, had no exposure to anti-emetic or antipsychotic agents, had a normal birth history, and had no history of head trauma. Seventy-two percent did report a stressful event immediately prior to the development of symptoms. Treatments reported included botulinum toxin (BoNT), oral medications, surgical procedures, and acupuncture. Thirty-two percent of patients reported a family history of focal dystonia, and BEB was the most commonly reported.
CONCLUSION
This study confirms previous reports of usual age, sex, caffeine and tobacco use, and family history in patients with blepharospasm. New findings include a report on occupation, lower reports of preceding eye conditions and photophobia, and higher reported stressful events. Further, this study shows a change in treatment with an increase in BoNT use and decrease in surgical procedures.
Topics: Adult; Age of Onset; Blepharospasm; Dystonic Disorders; Female; Humans; Male; Middle Aged; Pedigree; Risk Factors
PubMed: 20649903
DOI: 10.1111/j.1468-1331.2010.03161.x -
CMAJ : Canadian Medical Association... Nov 1988Blepharospasm, the most frequent feature of cranial dystonia, and hemifacial spasm are two involuntary movement disorders that affect facial muscles. The cause of... (Review)
Review
Blepharospasm, the most frequent feature of cranial dystonia, and hemifacial spasm are two involuntary movement disorders that affect facial muscles. The cause of blepharospasm and other forms of cranial dystonia is not known. Hemifacial spasm is usually due to compression of the seventh cranial nerve at its exit from the brain stem. Cranial dystonia may result in severe disability. Hemifacial spasm tends to be much less disabling but may cause considerable distress and embarrassment. Patients affected with these disorders are often mistakenly considered to have psychiatric problems. Although the two disorders are quite distinct pathophysiologically, therapy with botulinum toxin has proven very effective in both. We review the clinical features, proposed pathophysiologic features, differential diagnosis and treatment, including the use of botulinum toxin, of cranial dystonia and hemifacial spasm.
Topics: Adult; Aged; Basal Ganglia Diseases; Blepharospasm; Botulinum Toxins; Diagnosis, Differential; Evaluation Studies as Topic; Eyelid Diseases; Facial Muscles; Facial Nerve; Female; Humans; Injections, Intramuscular; Male; Meige Syndrome; Middle Aged; Sex Factors; Spasm
PubMed: 3052771
DOI: No ID Found -
Radiology Case Reports Sep 2023Multiple system atrophy (MSA) is a rare form of adult-onset α-synucleinopathy. Meige syndrome, identified as bilateral blepharospasm and oromandibular dystonia, is a...
Multiple system atrophy (MSA) is a rare form of adult-onset α-synucleinopathy. Meige syndrome, identified as bilateral blepharospasm and oromandibular dystonia, is a type of focal dystonic movement disorder. This case report aims to highlight the clinical features of multiple system atrophy associated with Meige syndrome in a patient. Additionally, we aim to provide the treatment experience in a patient with Meige syndrome as this is an extremely rare clinical case.
PubMed: 37448600
DOI: 10.1016/j.radcr.2023.04.055 -
Cureus Sep 2020Meige syndrome, also known as blepharospasm-oromandibular dystonia, is a neurological movement disorder that involves the involuntary muscle contractions of the eyes,...
Meige syndrome, also known as blepharospasm-oromandibular dystonia, is a neurological movement disorder that involves the involuntary muscle contractions of the eyes, mouth, tongue, and jaw. It is often associated with other disorders, such as Parkinson's disease. We describe a case of an 87-year-old man with Meige syndrome who was successfully treated with oral baclofen.
PubMed: 33101815
DOI: 10.7759/cureus.10570 -
Structural network topologies are associated with deep brain stimulation outcomes in Meige syndrome.Neurotherapeutics : the Journal of the... Apr 2024Deep brain stimulation (DBS) is an effective therapy for Meige syndrome (MS). However, the DBS efficacy varies across MS patients and the factors contributing to the...
Deep brain stimulation (DBS) is an effective therapy for Meige syndrome (MS). However, the DBS efficacy varies across MS patients and the factors contributing to the variable responses remain enigmatic. We aim to explain the difference in DBS efficacy from a network perspective. We collected preoperative T1-weighted MRI images of 76 MS patients who received DBS in our center. According to the symptomatic improvement rates, all MS patients were divided into two groups: the high improvement group (HIG) and the low improvement group (LIG). We constructed group-level structural covariance networks in each group and compared the graph-based topological properties and interregional connections between groups. Subsequent functional annotation and correlation analyses were also conducted. The results indicated that HIG showed a higher clustering coefficient, longer characteristic path length, lower small-world index, and lower global efficiency compared with LIG. Different nodal betweennesses and degrees between groups were mainly identified in the precuneus, sensorimotor cortex, and subcortical nuclei, among which the gray matter volume of the left precentral gyrus and left thalamus were positively correlated with the symptomatic improvement rates. Moreover, HIG had enhanced interregional connections within the somatomotor network and between the somatomotor network and default-mode network relative to LIG. We concluded that the high and low DBS responders have notable differences in large-scale network architectures. Our study sheds light on the structural network underpinnings of varying DBS responses in MS patients.
PubMed: 38679556
DOI: 10.1016/j.neurot.2024.e00367 -
Toxins Nov 2012The safety and efficacy of botulinum toxin for the treatment of focal hand and cranial dystonias are well-established. Studies of these adult-onset focal dystonias... (Review)
Review
The safety and efficacy of botulinum toxin for the treatment of focal hand and cranial dystonias are well-established. Studies of these adult-onset focal dystonias reveal both shared features, such as the dystonic phenotype of muscle hyperactivity and overflow muscle contraction and divergent features, such as task specificity in focal hand dystonia which is not a common feature of cranial dystonia. The physiologic effects of botulinum toxin in these 2 disorders also show both similarities and differences. This paper compares and contrasts the physiology of focal hand and cranial dystonias and of botulinum toxin in the management of these disorders.
Topics: Blepharospasm; Botulinum Toxins, Type A; Dystonic Disorders; Facial Muscles; Hand; Humans; Injections, Intramuscular; Meige Syndrome; Neuromuscular Agents
PubMed: 23202323
DOI: 10.3390/toxins4111404 -
Cureus Oct 2023Oromandibular dystonia is a focal dystonia characterized by involuntary movements of the jaw, oropharynx, lips, and tongue. The diagnosis of oromandibular dystonia is...
Oromandibular dystonia is a focal dystonia characterized by involuntary movements of the jaw, oropharynx, lips, and tongue. The diagnosis of oromandibular dystonia is clinical and can be complex. For effective treatment, it is essential to understand its underlying etiology. A 70-year-old man was referred to our center with a diagnosis of Meige's syndrome, which had been present for five and a half years, for receiving botulinum toxin-A (BoNT-A) injections. Upon physical examination, he exhibited oromandibular dystonia, with a score of 177 points on the Oromandibular Dystonia Rating Scale (OMDRS). He had a history of taking methotrexate for six years, as he was diagnosed with psoriatic arthritis during that time. The possibility of methotrexate-induced dystonia was considered. A switch from methotrexate to sulfasalazine was initiated. Subsequently, the patient showed progressive improvement in his symptoms, as reflected by an OMDRS score of 103 points. After eight weeks, the medical team decided to supplement the treatment with BoNT-A injections, resulting in an OMDRS score of 75. While there is currently no definitive evidence linking the use of methotrexate to the development of dystonia, it is advisable to consider oromandibular dystonia as a potential side effect of methotrexate until more robust evidence becomes available.
PubMed: 38022297
DOI: 10.7759/cureus.47248 -
Movement Disorders Clinical Practice Feb 2021
PubMed: 33816652
DOI: 10.1002/mdc3.13138