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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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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PMID:Oral rehabilitation with osseointegrated implants in a patient with oromandibular dystonia with blepharospasm (Brueghel's syndrome): a patient history. 1128 Mar 56

Two cases with blepharospasm followed by Meige syndrome were described. In the first magnetic resonants revealed bilateral hypodensive foci in thalamus. During the few years general dystonia has been developed after several relapses of disease diagnosed as multiple infarcts followed by aphasia, hemiparesis and asynergia. In the other one, who died, loss of neurons in striatum, especially in caudate nuclei was found. It is concluded that blepharospasm could be a syndrome of different origin and only the phase of Meige syndrome.
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PMID:[Blepharospasm and Meige's syndrome--a contribution to it's pathogenesis]. 1132 May 88

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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PMID:Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy. 1148 13

The results of deep brain stimulation (DBS) of the globus pallidus internus (Gpi) in six patients with generalized, focal, and segmental dystonia are presented. Pre- and postoperative assessments are given for one patient with generalized inherited dystonia and for five patients with idiopathic segmental or cervical dystonia. Clinical symptoms were evaluated before and 3-12 months after surgery using the Burke-Fahn-Marsden (BFM) dystonia rating scale for primary torsion dystonia and the Tsui scale for cervical dystonia. The Short-Form Health Survey (SF-36) was completed by each patient to document preoperative and postoperative health status. Also, neurological status was documented by video before and during chronic stimulation. Magnetic resonance imaging studies were performed to show the anatomical localization of the electrode leads. Five patients showed a progressive improvement within 7 days. One patient with cervical dystonia and Meige's syndrome showed no improvement for 3 months, but beneficial effects were observed after 12 months. On average, the BFM movement scale scores decreased by 72.5% and Tsui scale scores by 63%. SF-36 showed an improvement in health status by an average of 36% according to eight different health categories. We conclude that chronic high-frequency Gpi stimulation in different types of dystonia is a very effective and safe treatment.
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PMID:Chronic high-frequency globus pallidus internus stimulation in different types of dystonia: a clinical, video, and MRI report of six patients presenting with segmental, cervical, and generalized dystonia. 1183 51

Laryngeal dystonia is a focal dystonia occurring more often than it is diagnosed. Adductor type dystonia was described in 5 patients. In 3 cases it manifested itself as blepharospasm, which later developed into Meige's syndrome. The patients were treated with botulinum toxin A injections under EMG control administering 10 i.m. into laryngeal muscles on both sides with good and very good results. There were no serious side effects.
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PMID:[Laryngeal dystonia--personal experience with botulin toxin treatment]. 1205 8

The renaissance of functional neurosurgery in the treatment of Parkinson's disease has sparked also the interest in other movement disorders which are refractory to medical treatment. Deep brain stimulation (DBS) has been used only since a few years in dystonia patients. This review summarizes the available data on pallidal and thalamic DBS for various dystonic syndromes. The major advantage of DBS as compared to radiofrequency lesioning is that it allows performing contemporaneous bilateral surgery with relatively low morbidity in these patients. The posteroventral lateral globus pallidus internus (GPi) has been the preferred target in most instances, thus far. While phasic dystonic movements may improve early after surgery, the response of tonic dystonic movements to chronic stimulation may be delayed. The most beneficial results have been achieved in patients with primary genetic generalized and segmental dystonia, myoclonic dystonia, and complex cervical dystonia. Outcome has been varied in patients with other dystonic disorders, in particular those with secondary dystonia. Most studies have reported on relatively short follow-up periods, on single cases, or were retrospective. Pallidal DBS has been shown to be effective in complex cervical dystonia yielding both symptomatic and functional benefit for up to 2.5 years of follow-up. Dramatic improvement has been obtained in children and in adults with DYT1 positive dystonia. Also, patients with non DYT1 genetic dystonia achieved sustained benefit for up to 2 years of follow-up. Preliminary experience indicates that choreoathetosis in patients with cerebral palsy responds less well to pallidal DBS, and that it may not be effective at all in some patients. In single instances unilateral pallidal DBS has been shown to yield valuable benefit in patients with hemidystonia. The experience with DBS for treatment of Meige syndrome and other focal dystonias has been explored only recently. There is much less experience with thalamic DBS for dystonia. Thalamic DBS has been shown to be effective in single cases with posttraumatic dystonia, postanoxic dystonia and paroxysmal nonkinesigenic dystonia. Future perspectives of DBS for treatment of dystonia include the development of new technology, the evaluation of the possible role of other targets, and carefully planned studies to further establish the role of surgery.
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PMID:Deep brain stimulation for dystonia in adults. Overview and developments. 1265 41

For some years the dystonias have been the subject of major studies and, as far as the generalised dystonias are concerned, of major therapeutic advances. The opposite is true of the so-called focal or functional dystonias, which include conditions such as Meige's syndrome, spasmodic torticollis, writer's cramp, dystonias using instruments especially in musicians, and spasmodic dysphonia. For the last group, the term functional dysphonia would seems to us to be more appropriate. It would appear that what is involved is a disorder not of a muscle group, but rather of a function. Consequently stuttering can, in our opinion, be legitimately considered as a dystonia affecting the articulation of speech, within the global context of a new neurological grouping which could be called 'dysfunctional neurology'.
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PMID:[Is stuttering a functional dystonia?]. 1274 Dec 96

The liability to develop parkinsonian symptoms was evaluated in 105 outpatients with idiopathic blepharospasm (IBS; 54 cases) or IBS associated to oromandibular dystonia (Meige's syndrome; 51 cases) mean age 70.3 +/- 9.6 years, and compared with an age- and sex-matched population. Eleven patients developed Parkinson's disease in the blepharospasm group, whereas only 2 of 105 patients were affected in the control group. Our results suggest that patients with IBS either isolated or associated with oromandibular dystonia are more prone to develop parkinsonian symptoms.
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PMID:Development of Parkinson's disease in patients with blepharospasm. 1537 98

Meige's syndrome presents with a combination of upper and lower facial motor dysfunction, including eye closing spasms and oromandibular dystonia. While the pathophysiology of eye closing spasms has been extensively studied using the blink reflex and other trigeminofacial reflexes, very few studies have been carried out with regard to the abnormal perioral movements. We hypothesized that action-related dystonic features could be revealed by the analysis of the semiautomatic rhythmic movements required for chewing and swallowing. A total of 7 patients with Meige's syndrome that complained of chewing problems and 7 age-matched healthy volunteers were studied. Subjects were instructed to munch and swallow a small muffin while surface electromyographic (EMG) activity of masseter (MAS) and orbicularis oris (OOr) of the dominant side was recorded. In healthy subjects, MAS and OOr showed a rhythmic alternating phasic EMG pattern during chewing, which changed to a tonic cocontraction of both muscles during swallowing. Mean duration of MAS and OOr EMG bursts was, respectively, 297 +/- 28 msec and 328 +/- 29 msec. Patients exhibited the following alterations: excess duration of muscle activity, frequent cocontraction, loss of rhythmicity during chewing, and abnormalities in the chewing to swallowing transition phase. These abnormalities, similar in type to those encountered in other forms of focal dystonia, may be the expression of an abnormal motor control of basal ganglia over mastication-related movement pattern generators of the brainstem.
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PMID:Chewing pattern in patients with Meige's syndrome. 1539 39

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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PMID:Substantial improvement in a Meige's syndrome patient with levetiracetam treatment. 1539 69


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