Cases reported "Meige Syndrome"

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1/17. 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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2/17. 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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3/17. 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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4/17. 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.
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5/17. Idiopathic blepharospasm-oromandibular dystonia syndrome (Meige's syndrome) presenting as chronic temporomandibular joint dislocation.

    A case of idiopathic blepharospasm-oromandibular dystonia (Meige's syndrome) is reported, presenting as chronic bilateral dislocation of the temporomandibular joints. The nature of the syndrome is discussed, together with the difficulties in diagnosis and management.
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6/17. 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.
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7/17. Meige's syndrome during long-term neuroleptic treatment.

    Two patients developed difficulties in eyelid opening following long-term neuroleptic treatment of more than 6-8 years. Tardive dyskinesia and dystonia apart from the face were not found in either case. The symptoms fluctuated in their severities on a daily basis and were easily aggravated by various stimuli, e.g., stress, walking, reading and watching television. Electromyographic studies of their faces clearly indicated that the symptoms resulted from spontaneous blepharospasm and were analogous to idiopathic Meige's syndrome. Therefore, the patients' difficulties in opening their eyes were considered to be the so-called drug-induced Meige's syndrome and/or facial tardive dystonia. It must be stressed that this syndrome is extremely distressing to patients and is a severe complication accompanying a long-term neuroleptic treatment.
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8/17. Meige's syndrome associated with neuroleptic treatment.

    Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. Three cases are presented; two were associated with long-term neuroleptic administration. This drug-induced syndrome may be a variant of tardive dystonia, and prompt discontinuation of neuroleptic treatment may be therapeutic.
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9/17. Use of botulinum toxin in Meige's disease.

    Four patients with severe Meige's disease (blepharospasm-oromandibular dystonia) have been treated, after having given an informed consent, by local injections of purified botulinum toxin type "A". Previous systemic therapy with anticholinergics, dopamine antagonists and other drugs had been unsuccessful in all these subjects. Each patient was treated by saline solution injected with the same method as botulinum toxin, just once. The self-evaluation of patients and the clinical evaluation that some of us- unaware of the kind of therapy which had been performed- gave to the symptoms on the basis of videotapes, for each session of injection, showed that the injections of botulinum toxin are effective in the treatment of such disorder. The duration of the beneficial effect was slightly shorter in these patients than in patients with blepharospasm treated by the same method.
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10/17. Continuous dopaminergic stimulation in cranial dystonia.

    Meige's disease is a distressing complaint, the treatment of which often poses a challenge to the neurologist. The patient described here had blepharospasm-oromandibular dystonia, which responded transiently to oral lisuride. On three occasions, drug holidays successfully restored efficacy but thereafter further trials proved fruitless. Continuous subcutaneous lisuride administration in 0.35 mg doses per day, by means of a portable infusion pump, led to sustained improvement for 7 months. No major side effects were observed. Our findings suggest that this treatment deserves further trials.
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