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 report that repeated administration of salmon calcitonin (20-40 micrograms daily for 30 days) improves dystonic symptoms in patients affected by idiopathic torsion dystonia, blepharospasm-oromandibular dystonia syndrome or writer's cramp syndrome. These results support the suggestion that calcitonin may act at central level influencing the extrapyramidal motor system.
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PMID:Generalized and focal dystonic syndromes: possible therapy with salmon calcitonin. 406 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

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 pathophysiologic mechanisms of blepharospasm are poorly understood and the condition is often misdiagnosed. In an attempt to improve our understanding of blepharospasm, 250 patients with blepharospasm were surveyed. This heretofore largest reported series of blepharospasm patients reveals findings of clinical and demographic interest. For example, there is a 3 to 1 female preponderance, the age of onset is in the fifth or sixth decade of life in two-thirds of patients, the onset is usually gradual with progressive deterioration and involuntary spasms of other portions of face and body. Although specific ocular abnormalities may predispose a patient to blepharospasm, in most cases the blepharospasm is a form of focal dystonia often associated with other involuntary movements and neurologic abnormalities. Increased awareness of blepharospasm among primary care physicians as well as ophthalmologists, neurologists, psychiatrists, and other specialists should lead to early recognition and management of this disabling condition.
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PMID:Blepharospasm: demographic and clinical survey of 250 patients. 614 83

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

Two patients with facial dystonia (blepharospasm and/or oromandibular dystonia) presented with an unusual "trick" movement. Both patients were able to inhibit blepharospasm and oromandibular dystonia by vocalizations including singing, reading, and speaking spontaneously. The significance of "trick" movements in facial dystonia is discussed.
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PMID:"Trick" movements in facial dystonia. 650 Dec 39

Relief of blepharospasm was achieved with clonazepan (CNZ, 1 mg i.v.) and benzhexol (BH, 5 mg i.m.) by acute parenteral administration in 5 cases of Meige's syndrome. Improvement was greater with CNZ, mean value on a quantitative scale 100%, than for BH (84.1%). Both drugs were less effective on the associated oromandibular dystonia (OMD) observed in 3 of the cases, relief again being greater when using CNZ (87.3%) compared to BH (58.3%). Intravenous administration of CNZ predicted the response to prolonged oral medication (6 mg/day) in 3 of the cases. Though both blepharospasm and OMD are thought to represent focal dystonia at different body sites, the extent of improvement achieved with these drugs at the dosage employed differed markedly.
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PMID:Meige's syndrome: acute and chronic responses to clonazepan and anticholinergics. 651 Apr 47

In the neurologic literature there has been a growing recognition of a syndrome of focal cranial dystonias referred to variously as blepharospasm-oromandibular dystonia, Brueghel's syndrome, or Meige's syndrome. Typically, this syndrome presents as an isolated and idiopathic dystonia of facial or oromandibular muscles occasionally prompting a referral to a speech pathologist. Our findings in a group of 10 patients with this diagnosis are presented, and the negligible effect of an intensive program of speech management for one subject is described.
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PMID:Focal cranial dystonia. 662 Oct 3

We studied six patients with clinical and radiographic evidence of rostral brainstem lesion and bilateral blepharospasm. Two patients also had other facial dystonic movements. Four patients suffered rostral brainstem strokes, and two had multiple sclerosis. None had been treated with antipsychotic drugs prior to the onset of blepharospasm. Medical treatment was helpful in two patients, and bilateral selective facial nerve section was used in another patient. Possible pathogenic mechanisms are discussed.
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PMID:Blepharospasm associated with brainstem lesions. 668 64


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