Cases reported "Meige Syndrome"

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1/36. Treatment of Meige's syndrome with ECT.

    A 62-year-old woman with Meige's syndrome failed to respond to several pharmacologic interventions. Her dystonias improved significantly after treatment with bilateral electroconvulsive therapy (ECT). However, the effect was not durable, lasting < or = 72 h. ECT is an effective treatment for many movement disorders including dystonias of differing etiologies. Its efficacy for Meige's syndrome is questionable.
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ranking = 1
keywords = dystonia
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2/36. Oral rehabilitation with osseointegrated implants in a patient with oromandibular dystonia with blepharospasm (Brueghel's syndrome): a patient history.

    Oromandibular dystonia with blepharospasm (also known as Brueghel's syndrome, Meige's syndrome, or idiopathic orofacial dystonia) is characterized by intense and involuntary spasms of the orofacial muscles, with a frequent loss of teeth and occlusal alterations that worsen the dystonic manifestations and cause mucosal lesions that can lead to complete edentulism. The history of a patient with oromandibular dystonia who was rehabilitated with mandibular overdentures supported by endosteal implants is presented. Oral rehabilitation with implant-supported overdentures improved the situation, despite serious problems with instability. Mandibular overdentures supported by endosteal implants were satisfactorily used to re-establish occlusion, ensuring prosthetic stability and improving the dynamics of the masticatory muscles.
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ranking = 3.5266864576983
keywords = dystonia, orofacial, idiopathic
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3/36. meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.

    BACKGROUND: meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on meige syndrome and the relationship between dystonias and respiratory compromise is presented. methods: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.
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ranking = 2.5003281334571
keywords = dystonia, idiopathic
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4/36. Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from meige syndrome refractory to bilateral thalamotomy.

    meige syndrome is an adult-onset dystonic movement disorder that predominantly involves facial muscles, while some patients with this syndrome develop spasmodic dysphonia and dystonia of the neck, trunk, arms, and legs. We report that all dystonic symptoms that had been refractory to both pharmacotherapy and bilateral thalamotomy were markedly alleviated by bilateral pallidal stimulation in a patient with segmental axial dystonia advanced from meige syndrome.
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ranking = 3.0013125338285
keywords = dystonia, idiopathic
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5/36. The natural history of embouchure dystonia.

    Focal task-specific dystonias are unusual disorders of motor control, often affecting individuals who perform complex repetitive movements. Musicians are especially prone to develop these disorders because of their training regimens and intense practice schedules. Task-specific dystonia occurring in keyboard or string instrumentalists usually affects the hand. In contrast, there have been few descriptions of musicians with task-specific dystonia affecting the muscles of the face and jaw. We report detailed clinical observations of 26 professional brass and woodwind players afflicted with focal task-specific dystonia of the embouchure (the pattern of lip, jaw, and tongue muscles used to control the flow of air into a mouthpiece). This is the largest and most comprehensively studied series of such patients. patients developed embouchure dystonia in the fourth decade, and initial symptoms were usually limited to one range of notes or style of playing. Once present, dystonia progressed without remission and responded poorly to oral medications and botulinum toxin injection. patients with embouchure dystonia could be separated by the pattern of their abnormal movements into several groups, including embouchure tremor, involuntary lip movements, and jaw closure. dystonia not infrequently spread to other oral tasks, often producing significant disability. Effective treatments are needed for this challenging and unusual disorder.
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ranking = 5.5
keywords = dystonia
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6/36. jaw-opening dystonia (Brueghel's syndrome) associated with cavum septi pellucidi and Verga's ventricle - a case report.

    jaw-opening dystonia (oromandibular dystonia with jaw-opening; Brueghel's syndrome) is a rare condition, and only a limited number of cases have been reported in the literature. However, many patients may remain undiscovered or misdiagnosed, like a patient described previously. A case (40-year-old man) of jaw-opening dystonia (oromandibular dystonia with jaw-opening; Brueghel's syndrome) is reported. In this case, brain anomalies, cavum septi pellucidi and Verga's ventricle, were observed on magnetic resonance imaging of the brain. This case and a review of the literature indicate the presence of organic factors in the etiology of Brueghel's syndrome. The etiological relationship of brain anomalies in Bruegel's syndrome is discussed.
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ranking = 4
keywords = dystonia
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7/36. Smothering dystonia in a patient with oromandibular dystonia.

    A case report is presented of a patient with pathologically confirmed striatonigral degeneration who experienced episodic syncope as a result of oromandibular dystonia obstructing inhalation through her mouth and nose.
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ranking = 4.5
keywords = dystonia
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8/36. Substantial improvement in a Meige's syndrome patient with levetiracetam treatment.

    We report on a woman with idiopathic Meige's syndrome whose dystonia improved with the use of levetiracetam (LEV, Keppra, UCB Pharma, Smyrna, GA). This report and data from an animal model of paroxysmal dystonia suggest that LEV might be helpful in the treatment of dystonia.
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ranking = 1.5003281334571
keywords = dystonia, idiopathic
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9/36. Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology.

    OBJECTIVE AND IMPORTANCE: We report the intraoperative results, subsequent course, and 1-year follow-up evaluation of a patient with medication-refractory craniofacial dystonia for whom we planned bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) implantation but delayed the left GPi DBS implantation because of robust intraoperative effects of right GPi DBS. CLINICAL PRESENTATION: A 47-year-old patient had a 5-year history of progressively severe, bilateral craniofacial dystonia with blepharospasm (Meige's syndrome) that was refractory to medications and to botulinum toxin (A and B) injections. blepharospasm interfered with his ability to perform his duties as a Special Forces soldier and ended his military career. INTERVENTION: Under stereotactic guidance (magnetic resonance imaging and computed tomographic image fusion, Cosman-Roberts-Wells frame, and University of florida surgical navigation software) and with detailed microelectrode mapping (four microelectrode passes), a DBS electrode was implanted in the right posteroventral GPi. Microelectrode recordings were taken to document electrophysiological activity of neurons in the region, and intraoperative macrostimulation was performed. The patient was followed up for 6 months with right unilateral GPi DBS, and later a left GPi DBS electrode was placed. CONCLUSION: Although DBS for primary generalized dystonia is commonly performed by simultaneously implanting bilateral GPi electrodes, it may be reasonable in cases of refractory blepharospasm and/or craniofacial dystonia to use a staged procedure for implantation in selected patients. Additionally, the physiology, especially that encountered in the striatum, may help to elucidate the pathophysiological basis for refractory blepharospasm and Meige's syndrome. More cases will be needed to determine the significance of the results reported in this article.
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ranking = 4
keywords = dystonia
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10/36. meige syndrome and pallidal deep brain stimulation.

    The cause of primary meige syndrome is unknown, and although gender and age predilections are different from idiopathic torsion dystonia, most investigators consider meige syndrome a variant of that disorder. Interest in the use of stereotactic brain surgery for refractory forms of dystonia is thus increasing. There is little experience with the use of deep brain stimulation (DBS) in focal dystonias, and reports of its use in meige syndrome are very rare. We report on a case of meige syndrome successfully treated with bilateral pallidal DBS.
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ranking = 1.5003281334571
keywords = dystonia, idiopathic
(Clic here for more details about this article)
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