1/11. 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.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/11. 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.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
3/11. 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.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
4/11. 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.- - - - - - - - - - ranking = 5.5keywords = muscle (Clic here for more details about this article) |
5/11. 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.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/11. Levetiracetam in Meige's syndrome.The spontaneous occurrence of blepharospasm and dystonic movements in face muscles, particularly those of the perioral and mandibular regions, has been named as Meige's disease which was first described by Henry Meige in 1910. We report the case of a woman with Meige's syndrome whose symptoms improved with the use of levetiracetam.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
7/11. Severe tongue protrusion dystonia: clinical syndromes and possible treatment.We describe intermittent or sustained severe involuntary tongue protrusion in patients with a dystonic syndrome. speech, swallowing, and breathing difficulties can be severe enough to be life threatening. Causes include neuroacanthocytosis, pantothenate kinase-associated neurodegeneration, lesch-nyhan syndrome, and postanoxic and tardive dystonia. The pathophysiology of intermittent severe tongue protrusion remains unknown. tongue protrusion dystonia is often unresponsive to oral drugs but may benefit from botulinum toxin injections into the genioglossus muscle. Bilateral deep brain pallidal stimulation was beneficial in two cases.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
8/11. blepharospasm-oromandibular dystonia associated with a left cerebellopontine angle meningioma.blepharospasm-oromandibular dystonia is characterized by the presence of spasms of the orbicularis oculi (blepharospasm) and of the lower facial or oromandibular muscles. A patient with this syndrome is presented in which a left cerebellopontine angle meningioma appeared to act as a triggering mechanism for the development of this disorder. On the basis of this report, we recommend that physicians search for this tumor in patients with this disorder.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
9/11. Coexistent Meige's syndrome and myasthenia gravis. A relationship between blinking and extraocular muscle fatigue?We studied five patients with a combination of Meige's syndrome (blepharospasm-oromandibular dystonia) and myasthenia gravis. The coexistence of two disorders impairing eyelid opening led to diagnostic confusion and delayed appropriate therapy. Detailed oculographic monitoring of one patient indicated that eye position drifting due to myasthenic oculomotor fatigue was corrected by eye blinks, and that blinks tended to occur with slower saccades. Our observations suggest that fatigue of extraocular muscles may lead to synkinetic blinking and perhaps eventually to autonomous blepharospasm.- - - - - - - - - - ranking = 2.5keywords = muscle (Clic here for more details about this article) |
10/11. tongue protrusion dystonia: treatment with botulinum toxin.We report the treatment experience in a series of patients with involuntary tongue protrusion resulting from oromandibular dystonia (OMD) or Meige's syndrome. A retrospective analysis of clinical findings and results of treatment was conducted on patients treated at Vanderbilt University Medical Center between 1989 and 1995. After unsuccessful treatment with conventional oral medications, nine patients having involuntary tongue protrusion resulting from OMD or Meige's syndrome were treated with botulinum toxin type A (BTX-A) injected into the genioglossus muscle at four sites via a submandibular approach. A marked reduction in tongue protrusion was achieved in six patients (67%). Of 35 consecutive injections, 83% were successful at reducing tongue protrusion. Mild dysphagia complicated 14% of the injections. The average dose injected was 34 ( /- 3) units producing a 15 ( /- 2) week average duration of effect. Injection of the genioglossus with BTX-A may prove to be a valid treatment option for involuntary tongue protrusion related to OMD or Meige's syndrome. A double-blind, placebo-controlled trial is needed to better define efficacy and adverse events.- - - - - - - - - - ranking = 0.5keywords = muscle (Clic here for more details about this article) |
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