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)

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

Blepharospasm and oromandibular dystonia are clinically similar to other hyperkinetic movement disorders. Dopaminergic antagonist (neuroleptic) and purported cholinergic agonist (deanol) treatment improved symptoms, whereas dopaminergic agonist (carbidopa/levodopa) and cholinergic antagonist (benztropine) drugs worsened symptoms in two patients. This suggested that the syndrome is also pharmacologically related to the hyperkinetic dyskinesias. Symptoms worsened substantially during carbidopa/levodopa but temporarily resolved in one patient and improved in another when the drug was discontinued. This suggests that the pathophysiology of these symptoms involves an idiopathic form of receptor hypersensitivity that can be modified by agonist treatment. The effect of cholinergic agents was less than the effect of dopaminergic drugs, implying that dopamine plays a predominant role in the pathophysiology.
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PMID:Pharmacology of blepharospasm-oromandibular dystonia syndrome. 719 Feb 36

We studied central cholinergic systems in 13 patients with idiopathic adult-onset blepharospasm/oromandibular dystonia (Meige syndrome). Six patients studied acutely, and 12 of 13 patients studied chronically, improved after administration of centrally acting anticholinergic agents. These data suggest that Meige syndrome is pharmacologically similar to other dystonic disorders, in which central cholinergic antagonism is more consistently of benefit than manipulation of central dopaminergic systems.
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PMID:Meige disease: acute and chronic cholinergic effects. 720 Nov 18

Isolated idiopathic oromandibular dystonia and blepharospasm (Meige's syndrome) has generally been described as a nonfamilial disorder. This report describes Meige's syndrome in 2 sisters free of other neurological and psychiatric disease.
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PMID:Blepharospasm and oromandibular dystonia (Meige's syndrome) in sisters. 723 35

A patient with Brueghel's syndrome is described, who died following a seven year history of oromandibular dystonia with blepharospasm. Postmortem examination of the central nervous system revealed no abnormalities.
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PMID:Brueghel's syndrome, report of a case with postmortem studies. 726 92

Over recent years botulinum toxin type A has emerged as a safe and effective treatment for a number of previously refractory conditions associated with excessive muscle activity. The list of indications is expanding, but at present it is generally considered to be the treatment of choice for focal dystonias such as blepharospasm, torticollis, laryngeal dystonia, and oromandibular dystonia, as well as hemifacial spasm, strabismus, and some forms of limb spasticity. Carefully targeted intramuscular injections of a small amount of the toxin block the release of acetylcholine at the neuromuscular junction, producing a chemical denervation, with the aim of reducing excessive muscle activity without producing significant functional weakness. In some situations electrophysiological assessment and localisation of the muscles for injection is necessary. Treatment is symptomatic, with effects lasting 3 to 4 months and most patients requiring up to 4 injections per year to maintain the beneficial effect. Appropriate use of the toxin requires both an understanding of the physiological action of the potential muscles involved in each situation, together with a knowledge of the likely dose necessary to reduce muscle activity to the required level. Botulinum toxin represents a major advance in the management of these conditions, many of which responded poorly to previously available forms of therapy.
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PMID:Botulinum toxin in clinical practice. 753 96

A prospective open study of botulinum toxin A treatment for patients with various movement disorders at Siriraj Hospital, Mahidol University was analysed to evaluate its efficacy. The grand total of 900 patients comprised of a) 592 patients (65.78 per cent) with hemifacial spasm; b) 92 patients (10.22 per cent) with occupational cramp; c) 79 patients (8.78 per cent) with blepharospasm and Meige syndrome; d) 72 patients (8.00 per cent) with spasmodic torticollis; e) 19 patients (2.11 per cent) with hemidystonia and generalised dystonia; f) 11 patients (1.22 per cent) with spasmodic dysphonia; g) 10 patients (1.11 per cent) with spastic hemiparesis; and h) 25 patients (2.78 per cent) with miscellaneous group (i.e. tics, Gilles de la Tourette, facial myokimia, benign fasciculation, etc.). The results of treatment for hemifacial spasm were classified as excellent in 486 patients (82.09 per cent), moderate improvement in 60 patients (10.14 per cent), mild improvement in 39 patients (6.59 per cent) and no improvement or worse in 7 patients (1.18 per cent). There were complications of mild transient facial weakness in 50 patients (8.45 per cent) and mild ptosis in 12 patients (2.02 per cent). The effect of botulinum toxin treatment lasted 3-6 months. In occupational cramp and spasmodic torticollis the good response rate was around two-thirds of all patients, whereas, blephalospasm, spasmodic dysphonia, spastic hemiparesis and tics responsed in 79-88 per cent of the patients. Botulinum toxin A injection is thus a simple, safe, and effective out-patient treatment for patients with various kinds of movement disorders but it is a costly therapy.
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PMID:Treatment of various movement disorders with botulinum A toxin injection: an experience of 900 patients. 756 52

We evaluated the prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. The population of the area was 244,935 on October 1, 1992. Because four patients with blepharospasm and three patients with writer's cramp did not visit any hospitals or clinics in 1993 and did not reply to our question letter, we could not confirm their present condition: with or without focal dystonia in 1993. Four patients with facial dystonia including blepharospasm and oromandibular dystonia, seven with spasmodic torticollis, and four with writer's cramp were observed. The prevalence of focal dystonias was 6.12 per 100,000 persons, which may be lower than that in western countries. Although the reasons for this difference are still unclear, a genetic factor may be one implication.
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PMID:Prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. 872 61

Glabellar frown lines and crow's feet are wrinkles of facial expression related to an underlying muscular activity, which is particularly strong during facial expression. Classic treatments of these wrinkles only give partially satisfactory are associated with results, and secondary effects, whether they involve skin and muscle lifting, surgical section of muscles, dermal stimulation by thread or injectable fillers, chemical or mechanical abrasion, transient or permanent soft tissue augmentation with various materials. The authors studied the efficacy and safety of intramuscular injections botulinum A Exotoxin in glabellar and crow's feet areas in 19 well-informed and consenting patients. Botulinum toxin injections have been used since 1980 in the treatment of focal dystonia (blepharospasm, oromandibular dystonia, spasmodic torticollis, spasmodic dysphonia and writer's cramp) and safety hemifacial spasm. Their wide use in these indications has highlighted their excellent and efficacy, and the need to repeat injections every 3 to 4 months. The dose required was progressively adjusted around glabellar and orbital areas, while injections of the peri-buccal and forehead areas are still being evaluated. The 19 patients were examined clinically, filmed and photographed every month over a period of 12 to 24 months, and skin prints were performed. Evaluation criteria included the percentage improvement as assessed by the patients themselves, and also evaluation by the investigators of the data of clinical examination, and blind comparison of photographic, videoscopic, and prints. The authors obtained a significant decrease of wrinkles of the areas studied, with a "smoothing" effect during the period of activity of the toxin, which lased an average of 3 to 4 months at the beginning, and 6 to 9 months after several injections. No secondary effects, either general or local, were observed. The product's specificity means that the operator must have a complete mastery of the injection technique and a thorough knowledge of its pharmacology.
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PMID:[Botulinum toxin in the treatment of frontoglabellar and periorbital wrinkles. An initial study]. 766 8


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