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)

Laryngeal dystonia (spasmodic dysphonia) is a movement disorder characterized by involuntary contractions of laryngeal muscles involved with vocalization. The introduction of botulinum toxin in the treatment of laryngeal dystonia had a major clinical impact due to the striking improvement of symptoms. We report the preliminary results of therapeutical use of botulinum toxin in the treatment of twelve patients with laryngeal dystonia. After an extensive clinical evaluation, the patients underwent a videostroboscopic exam for diagnostic confirmation. Botulinum toxin was injected in the cricothyreoid membrane, directed towards the thyreoaritenoid muscle, with the aid of eletromyography needles. Most of patients who underwent botulinum toxin injection had a significant improvement of their symptoms (83%), with effects lasting for four months in average and without important side effects.
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PMID:[Use of botulinum toxin in the treatment of laryngeal dystonia (spasmodic dysphonia): preliminary study of twelve patients]. 1159 95

Meige syndrome is an adult-onset dystonic movement disorder that predominantly involves facial muscles, while some patients with this syndrome develop spasmodic dysphonia and dystonia of the neck, trunk, arms, and legs. We report that all dystonic symptoms that had been refractory to both pharmacotherapy and bilateral thalamotomy were markedly alleviated by bilateral pallidal stimulation in a patient with segmental axial dystonia advanced from Meige syndrome.
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PMID:Bilateral pallidal stimulation for idiopathic segmental axial dystonia advanced from Meige syndrome refractory to bilateral thalamotomy. 1148 13

Adductory spasmodic dysphonia is a focal dystonia of laryngeal muscles. Patients with this disorder typically have severe vocal difficulties, with significant functional, social, and emotional consequences. There is no widely accepted cure for this condition, however, botulinum toxin injections of the thyroarytenoid muscles are considered by most voice clinicians to be the state of the art treatment. Based on extensive experience treating patients for adductory spasmodic dysphonia, we feel that traditional means of voice assessment do not adequately measure either the disease severity or the treatment outcomes. That is, listening to or acoustically analyzing limited phonatory samples does not capture the functional, social, and emotional consequences of this disorder. These consequences will be reflected in a patient's voice-related quality of life (V-RQOL). Using a validated voice outcomes instrument, the V-RQOL Measure, the purpose of this study was to quantify longitudinal changes in the V-RQOL of patients with adductory spasmodic dysphonia who are undergoing botulinum toxin injections. Twenty-seven consecutive new patients presenting with dysphonia to our institution during an 18-month period were diagnosed with adductory spasmodic dysphonia, and treated patients were evaluated prospectively using the V-RQOL Measure. Results indicated that (1) V-RQOL was initially very low for these patients, (2) botulinum toxin injections improved it significantly for each injection cycle studied, and (3) the magnitude of the treatment effect appears to change across injections.
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PMID:Longitudinal effects of botulinum toxin injections on voice-related quality of life (V-RQOL) for patients with adductory spasmodic dysphonia. 1179 36

Spasmodic Dysphonia (SD) is a dystonia involving laryngeal musculature thus causing a characteristic voice disorder. Two main types of SD have been described. The adductor type is the commonest and it is characterized by a strain-strangle, choked voice. The abductor type can be distinguished from the previous one by episodes of a blown and whispering voice, interrupting speech. Botulism toxin (BTX) has demonstrated to be the most effective treatment for this condition. Thirty patients diagnosed of SD (twenty-nine adductor type/one abductor type) were included. Their degree of dysphonia was evaluated using both functional and visual-analogue scales. They were treated with BTX vocal cord injections using a percutaneous technique under EMG guidance. Improvements up to a 100% of the normal vocal function were obtained, with an average of 82% in the adductor type. The adverse effects were mild and transient. Hypophonia affected 61.3% of patients lasting an average of 11.3 days. Dysphagia was reported in 44.1% of cases lasting an average of 5.8 days.
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PMID:[Results of using botulism toxin in the treatment of spasmodic dysphonia]. 1199 15

We performed a service-based epidemiological study of dystonia in Munich, Germany. Due to favourable referral and treatment patterns in the Munich area, we could provide confident data from dystonia patients seeking botulinum toxin treatment. A total of 230 patients were ascertained, of whom 188 had primary dystonia. Point prevalence ratios were estimated to be 10.1 (95% confidence interval 8.4-11.9) per 100,000 for focal and 0.3 (0.0-0.6) for generalised primary dystonia. The most common focal primary dystonias were cervical dystonia with 5.4 (4.2-6.7) and essential blepharospasm with 3.1 (2.1-4.1) per 100,000 followed by laryngeal dystonia (spasmodic dysphonia) with 1.0 (0.4-1.5) per 100,000.
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PMID:Service-based survey of dystonia in munich. 1206 83

Symptomatic dystonia can be the result of various metabolic, degenerative diseases, the consumption of certain medications or exposure to toxic agents. However, only symptomatic dystonia with focal structural lesion provides a significant "window" for, at least indirect, perception of aetiopathogenesis and pathomorphological substratum of idiopathic dystonia. Our study included 57 patients with symptomatic dystonia, which as a base had focal or multifocal lesions, of whom 7 patients had generalized dystonia, 18 hemidystonia, 6 segmental dystonia, 7 torticollis, 6 blepharospasm, 7 hand dystonia, 3 spasmodic dysphonia, and 3 had oromandibular dystonia. Stroke was highly statistically the most frequent cause of structural lesions (33/57 or 58%). Relevant pathomorphological changes were present in 50/57 (88%) patients, of whom 25 (50%) had lesion in the lenticular nucleus (including individual damage of the putamen and globus pallidus), 12/50 (24%) had damage of the thalamus and 6/50 (12%) had damage of the brainstem. Generalized dystonia was most frequently associated with bilateral lesion of the putamen, hemidystonia with lesion of contralateral putamen, torticollis with damage of the caudate nucleus, hand dystonia with lesion of the thalamus and blepharospasm with lesion of the upper brainstem.
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PMID:[Clinico-pathomorphologic correlations in patients with symptomatic dystonias]. 1239 40

Although treatment with botulinum toxin type A (BTXA) has become the standard of care for most patients with laryngeal dystonia, its use is limited by the development of resistance to the toxin in some patients. Botulinum toxin type B (BTXB) has been found to be safe and effective in the treatment of cervical dystonia, but it has not been used previously to treat spasmodic dysphonia. Our experience with BTXB in a patient who developed resistance to BTXA suggests that BTXB may be safe and effective for the treatment of laryngeal dystonia, as well.
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PMID:Botulinum toxin type B for treatment of spasmodic dysphonia: a case report. 1239 95

Botulinum toxin is a dreaded biological toxin elaborated by Clostridium botulinum. The action of this toxin is to cause paralysis of both voluntary and involuntary muscles. The unique property of paralysing capability of muscles has been used for the benefit of human beings. Dr Allan Scot, an ophthalmologist, first used the toxin in a patient with squint in 1981 and since then the botulinum toxin is being used in various disorders characterised by muscle overactivity such as spasticity in both children and adult, dystonic conditions such as blepharospasm, cervical dystonia, spasmodic dysphonia, writer's cramp, etc, hemifacial spasm and headache. Its main action is at the terminal nerve endings of myoneural junction and it prevents release of acetylcholine from vesicles thus causing chemical denervation. Its action persists for 3 to 4 months on an average. Its side effects such as drooping, diplopia, dysphagia, depending on the sites of injection, are few and usually transient. Generalised anaphylaxis is almost unknown. Now botulinum toxin is being used in non-neurological conditions where muscles are under spasmodic state such as achalasia cardia, anal fissure, spasm of urethral sphincter, etc. Because of wider safety range and fewer complications, botulinum toxin has been an important therapeutic armamentarium in different branches of medicine and surgery.
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PMID:Botulinum toxin: a dreaded toxin for use in human being. 1245 15

For some years the dystonias have been the subject of major studies and, as far as the generalised dystonias are concerned, of major therapeutic advances. The opposite is true of the so-called focal or functional dystonias, which include conditions such as Meige's syndrome, spasmodic torticollis, writer's cramp, dystonias using instruments especially in musicians, and spasmodic dysphonia. For the last group, the term functional dysphonia would seems to us to be more appropriate. It would appear that what is involved is a disorder not of a muscle group, but rather of a function. Consequently stuttering can, in our opinion, be legitimately considered as a dystonia affecting the articulation of speech, within the global context of a new neurological grouping which could be called 'dysfunctional neurology'.
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PMID:[Is stuttering a functional dystonia?]. 1274 Dec 96

Dystonia has been described in various diseases affecting mitochondrial function but spasmodic dysphonia, a form of focal dystonia, has not. We present a patient with action myoclonus affecting the hands and arms who carried the most common mutation in mitochondrial DNA causing the myoclonic epilepsy and ragged red fibers (MERRF) syndrome (the A-->G substitution at nucleotide 8344 in the tRNA(Lys) gene). This patient also had spasmodic dysphonia that was responsive to treatment with intralaryngeal botulinum toxin.
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PMID:Spasmodic dysphonia in a patient with the A to G transition at nucleotide 8344 in mitochondrial DNA. 1278 81


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