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)

R1 and R2 blink reflex responses to single and paired stimuli were investigated in 23 control subjects, 21 patients with blepharospasm (BSP), 20 patients with torticollis spasmodica (TS), and 23 with hemifacial spasm (HFS). For paired stimuli, we compared measurements of area and peak responses at two and three times R2 threshold. R1 and R2 indices were calculated as the average of the recovery values at 0.5-, 0.3-, and 0.21-s interstimulus intervals to test individual patients. Peak amplitude measurements at three times R2 threshold were optimal. The R2 index was abnormal in 67% of BSP patients, 37% of TS patients, and 50% of HFS patients on the affected side and 20% on the unaffected side. A normal R2 index in one third of patients with BSP may indicate that different pathophysiological mechanisms are involved in this type of focal dystonia.
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PMID:Blink reflex recovery curves in blepharospasm, torticollis spasmodica, and hemifacial spasm. 853 55

The effects of botulinum toxin type A were studied in 33 patients with dystonia (12 blepharospasms, 10 hemifacial spasms and 11 spasmodic torticollis). A rate scale was used to evaluate the severity of the dystonic movements, before and two weeks after each injection. Among blepharospasm patients, eight were female and four were male; the mean age was 57.7 years; the mean time of the disease duration was four years. Three had familial history for similar disease; nine were essential and three had used neuroleptic drugs (tardive dystonia). The mean dose used was 51.3 U, with a mean time of beneficial effects of 2.8 months. For 22 injections and reinjections, 14 (63.7%) showed an excellent result, five (22.7%) good and three (13.6%) null. In the hemifacial spasm group, eight were female and two male; the mean age was 52.6 years; the mean time of the disease duration was 7.4 years; eight were essential and two post-paralytic. The mean dose used was 32 U. From the total of 15 injections and reinjections, all of them (100%) had an excellent result, with a mean time of beneficial effect of 3.4 months. Among the cervical dystonic patients, eight were male and three female; the mean age was 44.2 years; the mean time of the disease duration was 12.2 years; six had essential dystonia, three had used neuroleptic drugs and two had familial history for similar disease. The mean dose used was 238.6 U, with the mean duration of effect of 3.5 months. From the total of 20 injections and reinjections, 18 (90%) had good result, one (5%) mild and one (5%) null.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Botulinum toxin in blepharospasm, in hemifacial spasm, and in cervical dystonia: results in 33 patients]. 854 Aug 13

Fifty-five patients were treated with botulin injections into the muscles showing dystonia, contracture or tremor. Twenty two of them had torticollis, 21 had blepharospasm, 10 had hemifacial spasm, and 2 had tremor. In all, 112 injections were done with good result in 64%, slight effect in 27% and without effect in 9% of the cases. Similar results have been reported from other centers in the world. Adverse effects were not significant and disappeared after several days or weeks. They included ptosis, speech and deglutition disturbances, general weakness and neurotic reactions. These adverse effects developed in 12 cases. In cases of tremor the dose as well as the technique of injections must be individualized. The method is an important therapeutic advance and can be applied in outpatient clinics.
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PMID:[Own experience with botulinum treatment of dystonia]. 854 26

Clinical, neurological, endoscopic, psychological findings, questionnaire data on vegetative sphere, diaphragm x-ray, articulation test and Viene test system evidence obtained on 25 patients with phonic spasm confirm organic neurological nature of spastic dysphonia as focal muscular dystonia. This condition can be accompanied with tremor, rotatory, winking and writers' spasms, oromandibular dystonia. As indicated by positive treatment outcomes, combined treatment of phonic spasm with GABA-ergic drugs of clonazepam (antelepsin) and baclofen, orthophonic voice correction, physiotherapy is pathogenetically justified.
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PMID:[Clinical features of spastic dysphonia]. 855 15

We report on the psychopathology and illness-related changes of life in patients with benign essential blepharospasm (BEB) or hemifacial spasm (HFS). Fifty-six patients with BEB and 40 patients with HFS completed the SCL 90R, a screening instrument for psychiatric symptomatology, and the Freiburg Questionnaire for Dystonia (FQD), a questionnaire about psychosocial changes in subjects with movement disorders. In both BEB and HFS patients, the mean scores on all but one subscale of the SCL 90R remained within the double standard deviation of normal. In comparing BEB to HFS patients in illness-related changes of life, BEB patients were more severely disabled in all areas of life examined. Psychological distress in BEB, but not in HFS, correlated with physical disability and in particular with impairment of vision.
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PMID:Relative absence of psychopathology in benign essential blepharospasm and hemifacial spasm. 871 Jan 22

Botulinum toxin has become the initial treatment of choice for the management of essential blepharospasm, hemifacial spasm and other craniocervical dystonias. Numerous studies have confirmed a 90% to 95% response rate. Although a number of common side effects have been reported, the occurrence and incidence of rare local complications remains poorly understood. More importantly, the acute and chronic distant effects of botulinum toxin have not been clearly elucidated. A better understanding of such effects is essential if clinicians are to appropriately advise patients on the use of this therapeutic modality. This article is based on the Duke University experience in the management of over 500 patients with craniocervical spasm disorders, combined with a review of the published literature. These disorders include essential blepharospasm, oromandibular dystonia, hemifacial spasm, and torticollis. The incidence of side effects following more than 6000 treatments with botulinum toxin is presented. Pertinent research relating to the causes of these complications is also reviewed. The most common complications of treatment with botulinum toxin are related to acute local effects resulting from chemodenervation. The most important clinical effect in this group is weakening of the levator muscle resulting in ptosis, and the corneal consequences of lagophthalmos. The latter includes exposure keratitis, dry eyes, blurred vision, and hypersecretion epiphora. Less common local effects include facial numbness, diplopia, and ectropion. Some distant effects are being observed with increasing frequency. These include pruritus, dysphagia, nausea, and a flu-like syndrome. Most significant, however, are the rare reports of generalized weakness and the documentation of EMG abnormalities distant to the site of toxin injection. This has been seen with injections for both blepharospasm and torticollis. Until further studies on the long-term distant complications of botulinum toxin are available, it is recommended that patients receive as few life-time doses of toxin as possible, consistent with adequate management of their spasms. The practice of reinjecting patients routinely every three months, or at the first return of mild spasms should be discouraged.
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PMID:Botulinum-A toxin in the treatment of craniocervical muscle spasms: short- and long-term, local and systemic effects. 882 30

Dystonia and particularly spasmodic torticollis are neuromuscular disorders that are extremely resistant to most therapies (physical, medical, or surgical). Torticollis is a unilateral spasm of the neck muscles, particularly of the sternocleidomastoid, that produces violent, tonic turning of the head to one side. The etiology remains uncertain, although the role of psychogenic factors has been emphasized. This article reviews the literature and reports four cases of spasmodic torticollis treated successfully with hypnosis. In all four cases, psychogenic causes were involved. Postural hypnosis (i.e., hypnosis in the standing position) was employed to counteract and minimize muscle spasms due to postural reflexes. A hypnobehavioral approach was adopted along with hypnotic strategies that included hierarchical desensitization, sensory-imaging conditioning, ego-boosting suggestions, and hypnosis-facilitated differential muscle retraining. In two cases, a combined hypnosis and electromyographic-biofeedback approach was used to equilibrate and retrain affected neck muscles. Although the hypnotherapeutic process took several months to induce and stabilize significant changes, outcome results were good to excellent in all cases, with marked reduction of the torticollis and the hypertrophy of the neck muscles as well as a reduced interference of symptoms in daily living.
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PMID:Hypnosis and spasmodic torticollis--report of four cases: a brief communication. 888 29

Botulinum toxin, the most potent of the neurotoxins, produces paralysis by blocking presynaptic release of the neurotransmitter (acetylcholine) at the neuromuscular junction, with reversible chemical denervation of the muscle fibre, thereby inducing partial paralysis and atrophy. Because chemical denervation is reversible, botulinum toxin has temporary effects, the muscle being progressively reinnervated by nerve sproutings. Type A botulinum toxin (Bix-A) is available under two dosage forms: Botox and Dysport. Although the initial clinical indication was strabismus, subsequent studies have demonstrated the efficacy of Btx-A, mainly in dystonia, hemifacial spasm and spasticity. However, botulinum toxin has been successfully used in various other clinical indications. In regard to spasticity associated with cerebral palsy, Btx-A is a promising treatment requiring a multidisciplinary approach. Btx-A injections lead to effective reduction of muscle hyperactivity with minor side-effects. They are painless, even though electromyographic guidance may be required for the injection of deep muscles. However, the production of antibodies to Btx-A may compromise the effect of long-term treatment.
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PMID:[Mechanism of action, clinical indication and results of treatment of botulinum toxin]. 897 11

Botulinum toxin is a presynaptic neuromuscular blocking agent that, when injected intramuscularly in minute quantities, can produce selective muscle weakness. This property is employed therapeutically to provide symptomatic relief in conditions related to excessive muscle activities in strabismus, blepharospasm, hemifacial spasm, cervical dystonia, spasmodic dysphonia (adductor type), and jaw closing dystonia. It is investigational for a long list of medical conditions. It is a marketed drug in a number of countries in the world, but its use has only been approved by different regulatory agencies for use in a limited number of conditions. The long-term effects, appropriate dose for children, and in pregnancy, and maximum dose without causing toxicity remain unclear.
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PMID:Botulinum toxin as a therapeutic agent. 898 68

Botulinum toxin was introduced as a safe and effective treatment for strabismus and other disorders of the extraocular muscles in the early 1980s. Approved by the U.S. Food and Drug Administration for use in the treatment of strabismus, blepharospasm and seventh cranial nerve disorders, botulinum toxin has also been shown to be effective in the treatment of a wide range of disorders associated with involuntary muscle contraction and spasm, including focal dystonia, hemifacial spasm and spasmodic torticollis. Neurologic syndromes associated with excessive muscle contraction or involuntary movement, disturbances of sphincter function and musculoskeletal pain are other conditions that can be successfully treated with botulinum toxin.
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PMID:Therapeutic uses of botulinum toxin. 905 23


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