Cases reported "meige syndrome"

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1/47. 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. ( info)

2/47. Conjunctival edema and alopecia of the external third of the eyebrows in a patient with meige syndrome.

    PURPOSE: To describe a patient with meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. methods: Case report. RESULTS: Histological examination of the conjunctival and skin specimens showed dermal edema and a slight reduction in the number of severely ectatic lymphatics in the reticular dermis. The vessel were identified as lymphatics on the basis of immunohistochemical evidence of discontinuity and/or absence of basement membrane. CONCLUSIONS: Clinical and histological findings suggest that the etiopathogenesis of the edema in meige syndrome is related to a structural ectatic defect of lymphatics. This anomaly seems to involve both skin and other sites, such as conjunctival mucosa. ( info)

3/47. 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. ( info)

4/47. 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. ( info)

5/47. 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. ( info)

6/47. Effect of clonazepam treatment on antipsychotic drug-induced meige syndrome and changes in plasma levels of GABA, HVA, and MHPG during treatment.

    We demonstrated the effect of clonazepam (2 mg/day) on meige syndrome in two schizophrenic patients under continuous treatment with antipsychotic drugs, and changes in the plasma levels of gamma-aminobutyric acid (GABA), homovanillic acid (HVA), and 3-methoxy-4-hydroxyphenylglycol (MHPG) in these cases. The plasma levels of HVA and MHPG during treatment with clonazepam were decreased in the responder, while not changed in the non-responder to clonazepam. A difference between the responder and the non-responder was not found in the plasma GABA levels. These results suggest that hyperactivities of the central dopaminergic and noradrenergic neurones are involved in the pathophysiology of meige syndrome. ( info)

7/47. Three silent periods in the orbiculari oculi muscles of man: normal findings and some clinical vignettes.

    PURPOSE: To investigate how many true silent periods could be found in the orbiculari oculi muscles of man. MATERIAL AND methods: 10 subjects, clinically healthy (5 male, 5 female), with a mean age of 34 years-old (range: 23 to 48) were evaluated by mean of the blink reflex at resting and during contraction of the orbiculari oculi reflex according to protocols validated internationally. RESULTS: Three responses called R1, R2 and R3 were obtained in the orbicular oculi muscle at resting state which had latencies and amplitudes within normal limits. What was new was to obtain three silent periods when the subjects were evaluated during muscle contraction. The duration of the first silent period was statistically longer than the second one (p < 0.004) and shorter than the third silent period (p < 0.0001). In addition, this test was found useful in detecting more specific findings in patients with hemifacial spasm and Meigge syndrome. CONCLUSION: This is by the first time that three silent periods in the orbicular oculi muscles are consistently demonstrated. The refractoriness of the alpha motoneurons and the action of gamma-collateral activity seem to be the main conditions leasing to display the first two periods of muscle suppression. The modification of gamma motoneurons firing as well as a pause of muscle spindles in facial muscles due to the action of nociceptive stimuli traveling unmyelinated C fibers of the supraorbital nerve might be the most important mechanisms involved in the production of the third silent period. These results enables further clinical application of this test. ( info)

8/47. 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. ( info)

9/47. meige syndrome with apraxia of lid opening after the discontinuation of sulpiride treatment.

    We report a case of meige syndrome with apraxia of lid opening that lasted for about seven months after discontinuation of sulpiride treatment. To our knowledge, this is the first report demonstrating that meige syndrome with apraxia of lid opening is induced by sulpiride, and that the condition persists. ( info)

10/47. 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. ( info)
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