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 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

We present two women with Meige's syndrome, a condition in which the clinical presentation differs from tardive dyskinesia by the lack of exposure to neuroleptic drgus, greater severity of blepharospasms, and more prolonged dystonic contractions of oromandibular muscles. In this condition we used triaxial accelerometry to detect dystonia, which may also appear in limb and respiratory muscles. Although psychologic factors may affect the symptoms, the basic pathogenesis of this syndrome does not seem to be psychogenic. We think that biochemical abnormalities in the basal ganglia are responsible for the dyskinesias and submit data suggesting a reduction of dopamine turnover in the central nervous system of one patient. Both patients have evidence of autoimmune diseases, and one patient's dystonic movements responded to immunosuppressive therapy, suggesting that autoimmune processes contribute to the pathogenic mechanism of Meige's syndrome in some instances.
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PMID:Meige's syndrome. 738 44

Dystonia as cause of pharyngo-laryngeal motility disorders has not been adequately considered in most clinical ENT practices. This case study of a patient with spasmodic torticollis, Meige's syndrome and pharyngo-laryngeal motility disorder was found to be due to dystonia as the underlying cause. The possibility of local symptomatic therapy with botulinum toxin injections has currently provided the physician with an effective means for alleviating the disorder.
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PMID:[Dystonia as the cause of pharyngolaryngeal motility disorders]. 755 8

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 report the results of botulinum toxin type A (Dysport, Porton Products, UK) treatment over 5 years in 107 patients with blepharospasm, Meige's syndrome, oromandibular dystonia, hemifacial spasm, cervical dystonia, and writer's cramp. Electromyography was used to localize dystonic muscles and guide Dysport injections in Meige's syndrome, oromandibular dystonia, cervical dystonia, and writer's cramp. All but 2 Meige's syndrome and 2 writer's cramp patients responded to treatment. Improvement was dramatic in blepharospasm (79%) and hemifacial spasm (90%); pronounced in cervical dystonia (74%); and moderate in Meige's syndrome (53%), oromandibular dystonia (57%), and writer's cramp (34%). Although Dysport doses were 50-75% lower than usually reported, response and improvement rates as well as relapse intervals were similar to those of others. To treat cervical dystonia relapses, only 50% of the initial dose was required for continued optimal relief of symptoms. Low-dose Dysport was associated with a very low incidence of dysphagia in cervical dystonia.
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PMID:Five-year experience in the treatment of focal movement disorders with low-dose Dysport botulinum toxin. 778 62

We report the first Indian experience of botulinum toxin A in the treatment of blepharospasm and hemifacial spasm. Sixteen patients, 7 with essential blepharospasm, 5 with Meige syndrome and 4 with hemifacial spasm received botulinum toxin A injection. One patient received 3 courses of injections, 2 received 2 courses and the rest received only one course. The effect was observed after a latent period of less than 48 hours in all patients and lasted for a mean of 16.65 weeks. More than 70% improvement occurred after 17/20 injections (85%). Poor response was more often seen when blepharospasm was associated with oromandibular dystonia (2/5 injections). Though the duration of response and subjective score of improvement was best in patients with hemifacial spasm, the numbers were very small for any statistical evaluation. The side effects were local, transient, mild and well tolerated. The commonest side effect was blepharoptosis.
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PMID:Botulinum toxin A in blepharospasm and hemifacial spasm. 786 May 9

We report a patient with Meige syndrome (segmental cranial dystonia) who had neuropathologic changes of Parkinson's disease on postmortem examination. Neuropathologic examination showed typical and atypical Lewy bodies in the pigmented nuclei of the brainstem (substantia nigra, locus ceruleus), the nucleus basalis of Meynert, and the nucleus ambiguus. Neurochemical analysis of postmortem brain tissue showed evidence for decreased dopamine turnover in the substantia nigra, striatum, and nucleus accumbens. We propose that some cases of Meige syndrome may be included in the spectrum of Lewy body disease.
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PMID:Meige syndrome in the spectrum of Lewy body disease. 805 44

A case of Meige's syndrome is reported who presented with blepharospasm and oromandibular dystonia along with dislocation of jaw, due to severe dystonia. Such severe form of dystonia are very rare.
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PMID:Meige's syndrome. 822 4

Dystonia is a persistent attitude or posture in one or other of the extremes of athetoid movement. It may take the form of an over-extension or over-flexion of the hand, torsion of the spine, with arching and twisting of the back or forceful closure of the eyes and a fixed grimace. Dystonia is classified into idiopathic and symptomatic dystonia. Idiopathic dystonia is further divided into generalized, focal and segmental dystonia. Generalized dystonia covers classical torsion dystonia, paradoxical dystonia, myoclonic dystonia, dystonia with diurnal variation and Dopa-responsive dystonia. Dystonic tic, paroxysmal dystonia and hypnotic dystonia show a dystonic posture, although they are also accompanied by various other involuntary movements such as athetosis or chorea. Torticollis, writer's cramp or blepharospasm is assigned to the focal dystonia and Meige syndrome to the segmental dystonia. Symptomatic dystonia is observed in various neurological disorders, including cerebrovascular diseases, Parkinson's disease and Wilson's disease.
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PMID:[Dystonia]. 827 58


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