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)

Meige's disease is a form of focal dystonia characterized by symmetric dystonic spasms of facial muscles, sometimes associated with dystonic movements of other midline muscle groups. The etiology and pathophysiology of the disease have not been established. There is evidence that Meige's disease may result from striatal dopaminergic preponderance coupled with cholinergic overactivity. Several authors have noted a high prevalence of depression in patients with Meige's disease, suggesting a common neurochemical abnormality. Depression, in turn, is associated with decreased pineal melatonin secretion. We propose, based on studies demonstrating that melatonin regulates dopaminergic, cholinergic and GABA-ergic functions, that alterations in melatonin functions may enhance the development of the disease. This hypothesis may open new avenues toward understanding the pathophysiology of the disease and developing newer therapeutic strategies.
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PMID:Melatonin secretion and the pathophysiology of Meige's disease (idiopathic orofacial dystonia): a hypothesis. 197 65

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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PMID:Use of botulinum toxin in Meige's disease. 324 65

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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PMID:Continuous dopaminergic stimulation in cranial dystonia. 340 58

The spontaneous occurrnce of blepharospasm and dystonic movements in face muscles, particularly those of the perioral and mandibular regions, has been named Meige's disease. Other dystonic features as spasmodic torticollis, dysphagia, spasmodic dysphonia and segmental dystonia of the limbs may, eventually, be present in the same patient. There is very little knowledge about the pathology of this disease. Many hypotheses concerning the pathophysiology of this entity have been put forward, most of them correlating the clinical response to several drugs with known action on the neurotransmitter system of the brain. There are some evidences that it may exist a dopaminergic preponderance in the disease. In the nigro-striatal pathway, one of the retrograde loops in the feed-back control of dopamine synthesis by nigral neurons is dependent on GABA. Increasing GABA activity through GABA agonists that cross the blood-brain barrier could result in a decreased dopaminergic action in the nigro-striatal pathway and, thus, ameliorate the dystonic symptoms which might have been produced by its increased function. We have used baclofen, a GABA-agonist drug, to treat five patients with Meige's disease, in a single-blinded trial. These were four females and one male, with age ranging from 50 to 63 years. The drug was started at 20mg/day, being increased by 10mg each three days reaching a maximum dose of 70mg/day. One of the patients showed marked improvement of blepharospasm and orofacial dystonia and a second patient had a moderate improvement in the same symptoms. Another patient showed moderate improvement of limb dystonia, but had no benefit in the facial movements.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Treatment of Meige disease with a GABA receptor agonist]. 409 37

A patient with Meige's disease was given several pharmacological trials, including muscle relaxants, dopaminergic antagonists, monoamine depletors, anticholinergics, dopamine agonists, cholinergics, benzodiazepines, tricyclic antidepressants, and lithium. Improvement of dystonic movements was best obtained by a combined treatment, haloperidol-benztropine. A review of the literature on Meige's disease is presented. The hypothesis of a simultaneous dopaminergic and cholinergic overactivity is discussed and the use of physostigmine for diagnostic purposes is suggested.
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PMID:Neuropharmacological profile of Meige's disease: overview and a case report. 666 28

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 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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PMID:Levetiracetam in Meige's syndrome. 1677 30