Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A dopamine agonist (apomorphine) and a cholinomimetic drug (physostigmine) were administered to five patients with blepharospasm and oromandibular dystonia (Meige disease). The effects of haloperidol and levodopa were also assessed. Apomorphine lessened and physostigmine aggravated the facial dyskinesias in all patients, while placebo injections had no consistent effect. Levodopa did not modify the symptoms, but haloperidol attentuated the facial dystonia. Dysfunction of the basal ganglia, characterized by a state of striatal dopamine preponderance, probably underlies the dystonic spasms in Meige disease. The prominent cholinergic enhancement of facial dyskinesias may distinguish this disorder pharmacologically from tardive dyskinesia, a differentiation which has practical therapeutic implications.
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PMID:Meige disease: striatal dopaminergic preponderance. 37 89

We studied 115 Japanese patients with idiopathic cranial dystonia (Meige disease), using surface electromyography (EMG) focused on the orbicularis oculi muscles to classify the findings of the abnormal involuntary movements of this disease and to evaluate the pathophysiology of blepharospasm (BS). Surface EMGs at rest and at voluntary eyelid opening after eyelid closing were investigated. We found 62 (53.9%) patients exhibiting the overblinking type, 37 (32.2%) the tonic BS type, and 16 (13.9%) the normal type of behavior, considering the frequency of spontaneous blinking and presence of spasms. The present results suggest that BS is not a summation of blinking but a spatial and temporal extension of the orbicularis oculi muscle activity engaging in blinking, and the classification of the present study can support the investigation of the temporal characteristics of patients with this disease.
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PMID:Surface electromyographic study of idiopathic cranial dystonia focused on the orbicularis oculi muscles. 150 71

Two patients developed either blepharospasm or blepharospasm-oromandibular dystonia following chronic therapy with chlorpromazine, haloperidol, or thioridazine. In one patient, appearance of the movement disorder was associated with neuroleptic withdrawal, and in the other patient, the movement disorder began while neuroleptic therapy continued. Because of the age of one patient and the severe intermittent psychosis in the other, these Meige-like symptoms were attributed to chronic neuroleptic use rather than to spontaneously occurring Meige syndrome. The symptoms occurring as part of a tardive dyskinesia suggest that dopaminergic mechanisms play a role in idiopathic Meige syndrome.
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PMID:Meige syndrome (blepharospasm-oromandibular dystonia) after long-term neuroleptic therapy. 611 44

Most classifications of movement disorders emphasize their differential diagnosis and epidemiology according to clinical history and neurological examination. This review of movement disorders is organized according to the hypothesis of basal ganglia neurotransmitter imbalance in order to emphasize current research based on the pharmacology of these disorders. Specifically, dopamine (DA) excess and acetylcholine (ACh) deficiency may characterize part of the pathology of several hyperkinetic movement disorders including tardive dyskinesia, Huntington disease, Gilles de la Tourette syndrome, l-dopa dyskinesias, tardive Tourette syndrome, and toxic Tourette syndrome. The mirror image of this paradigm, namely DA deficiency and ACh excess, may characterize several rigid-dystonic movement disorders including Parkinson disease, drug-induced dystonias, and dystonia musculorum deformans. Finally, the unique combination of DA excess with ACh excess may characterize idiopathic orofacial dyskinesia (also known as Meige dystonia, Brueghel syndrome, and blepharospasm-oromandibular dystonia). Evidence supporting this formulation of movement disorders is reviewed, the limitations of this hypothesis are discussed, and new data from our own studies are presented.
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PMID:The neuropharmacology of tardive dyskinesia, spontaneous dyskinesia, and other dystonias. 612 51

The clinical pharmacological, and neuroradiological observations in six patients with spontaneous blepharospasm-oromandibular dystonia (Meige's) syndrome are recorded. This group consisted of five males and one female, mean age at onset being 50.3 years. The duration of symptoms ranged from three months to 12 years, three patients having had symptoms for over four years. The dyskinesia was arrhythmic and asymmetrical in the orbicularis oculi and masseter muscles electrophysiologically. Pharmacological studies evinced no consistent response to parenteral physostigmine, no response to oral levodopa and no significant improvement in the dyskinesia following oral haloperidol. Lumbar air encephalogram was done in five patients, and showed frontal cortical atrophy without ventricular dilation in three. It is concluded that Meige's syndrome is a distinct nosological entity, and that physostigmine test is unlikely to be helpful in the differential diagnosis from neuroleptic-induced tardive dyskinesia. Neurotransmitter imbalance in the basal ganglia in this disorder remains to be established, and at present there is no satisfactory drug treatment for this progressively disabling movement disorder.
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PMID:Meige's syndrome: clinical, pharmacological and radiological observations. 627 56

We evaluated prospectively 100 patients, the largest reported series, with blepharospasm and orofacial-cervical dystonia, or Meige syndrome. The mean age at onset was 51.7 years, and 81% presented between the ages of 40 and 70. Women outnumbered men three to two. Blepharospasm was the initial symptom in 58 patients, but only 23 had involuntary movements localized to the orbicularis oculi. Sixty-one patients had the complete syndrome, blepharospasm and oromandibular dystonia, and 60 had neck or generalized dystonia in addition to the orofacial movements. Twenty-one patients with spasmodic dysphonia were included; in 12 of these patients, spasmodic dysphonia was part of the complete (Meige) syndrome, and 16 of these patients had neck or generalized dystonia or essential tremor. An organic cause of Meige syndrome is supported by a high correlation with essential tremor and other movement disorders and by positive family history in some patients. Response to medication was inconsistent, but 69% of patient trials resulted in some improvement; in 22% the benefit was marked and persistent. Tetrabenazine, lithium, and trihexyphenidyl were most useful for the treatment of oromandibular dystonia, and clonazepam was useful in some patients with blepharospasm.
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PMID:Blepharospasm and orofacial-cervical dystonia: clinical and pharmacological findings in 100 patients. 683 74

Idiopathic orofacial dyskinesia, also called Brueghel's syndrome, blepharospasm-oromandibular dystonia, and Meige dystonia, is characterized by involuntary facial movements. Since this disorder can be difficult to distinguish from tardive dyskinesia, we have generated a neuropharmacologic profile of Meige dystonia. Symptoms were improved by antagonists of both dopamine and acetylcholine and worsened by the cholinergic agonist physostigmine, consistent with a hypothesis of relative excess in both dopamine and acetylcholine neuronal activities. Since tardive dyskinesia is hypothesized to be characterized by dopamine excess and acetylcholine deficiency, a physostigmine infusion may help differentiate these two disorders by exacerbating Meige dystonia but improving tardive dyskinesia.
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PMID:Pharmacologic characteristics of Meige dystonia: differentiation from tardive dyskinesia. 717 19

Seventeen patients with prominent orofacial dystonia of unknown cause (idiopathic orofacial dystonia: Meige's disease) were examined and several clinical features seen that, to my knowledge, had previously not been recognized. These include a family history of dystonia or other extrapyramidal disorders, a high incidence of depression, and frequent extension of spasms beyond the orofacial muscles. The course of the muscle spasms varies: rapid progression (eg, two months) to maximal disability occurred in some patients, and clear improvement after years of severe disability was observed in others. In addition to the muscle spasms, neurological abnormalities that suggest dysfunction of the basal ganglia were frequently present. The "spasm facial median" of Meige may be a distinct dystonic disorder, unrelated to idiopathic torsion dystonia.
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PMID:Clinical features of Meige's disease (idiopathic orofacial dystonia): a report of 17 cases. 746 47

The authors describe the Meige's Syndrome also known as blepharospasm or mandibulo-oral dystonia. This Syndrome rather known by Neurologists and Ophthalmologists than by Stomatologists actually benefits by a specific treatment based on botulin toxins.
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PMID:[Oromandibular dystonia and botulinum toxins]. 900 16

Meige's Syndrome is a combination of blepharospasm and oromandibular dystonia (cranial dystonia) that can affect the pharyngeal and respiratory musculature. We are reporting a case of a 61 year-woman with this diagnosis who presented a laryngeal stenosis due to sinequia on posterior commissure. The symptoms were an association of hoarseness and dyspnea with a clear movement in all pharyngo-laryngeal structures because of her illness. She was operated by Laser surgery with section of such sinequia and going on treatment in Neurology.
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PMID:[Posterior laryngeal stenosis in patient with Meige's Syndrome]. 1538 86


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