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Query: UMLS:C0013421 (dystonia)
8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The occurence of extranuchal dystonia, facial spasm, parkinsonian symptoms (facial masking, bradykinesia, rigidity), tremor and family history of tremor was tabulated in a group of 30 patients with IST. The incidence of extranuchal dystonia increased as severity of IST increased. There was a strong trend for severity of extranuchal dystonia to increase as severity of torticollis increased, which was significant (p less than 0.001). There was a similar trend for severity of facial spasm to increase with increasing severity of torticollis (p less than 0.025). Parkinsonian features were seen in 10 of 30 patients, and in three the diagnosis of Parkinson's disease could be entertained. Tremor was seen in 26 of 30 patients being mild in 12, moderate in 11, and severe in three. A family history of tremor was present in 16 of 28 cases for whom history was available (12 primary, four secondary relations). The results are most consistent with the hypothesis that IST is a variant of DMD with tremor as an integral part of the disease and tremor represents a forme of the disease in family members.
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PMID:Dystonia and tremor in spasmodic torticollis. 94 73

Thirty-nine patients with the idiopathic blepharospasm-oromandibular dystonia syndrome are described. All presented in adult life, usually in the sixth decade; women were more commonly affected than men. Thirteen had blepharospasm alone, nine had oromandibular dystonia alone, and 17 had both. Torticollis or dystonic writer's camp preceded the syndrome in two patients. Eight other patients developed toritocollis, dystonic posturing of the arms, or involvement of respiratory muscles. No cause or hereditary basis for the illness were discovered. The evidence to indicate that this syndrome is due to an abnormality of extrapyramidal function, and that it is another example of adult-onset focal dystonia akin to spasmodic torticollis and dystonic writer's cramp, is discussed.
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PMID:Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia? 101 Oct 31

67 cases of various functional disorders of the diencephalon were examined by EMI scanner. The patients were composed of 38 cases of parkinsonism, 7 cases of thalamic syndrome, 6 cases of choreoathetoid movement, 2 cases of dystonia, 11 cases of involuntary movement of unknown etiology and 1 case of torticollis, tic, and ballismus, respectively. In parkinsonism, 79% showed diffuse cerebral atrophy, 5% had focal low density in the substantia nigra and the thalamus, whereas 16% remained normal. Pre- and postoperative assessment with CT scan was briefly discussed with reference to stereotactic surgery of the diencephalon.
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PMID:Role of computerized transverse axial tomography on stereotactic surgery of the diencephalon. A preliminary report of 67 cases. 105 97

In summary, then, without consideration of specific circuits or transmitter agents, one can conceive of a hypothetical model that involves both learning and the functional nature of the defect in torticollis and focal dystonia to describe the results obtained. The model must be further elaborated upon and tested, preferably in a quantitative manner. Naturally, the specific finding of a defective transmitter agent (e.g., GABA) such as described in parkinsonian syndrome (dopamine) or the interruption of a specific pathway that causes and improves a dyskinesia is desirable. In this chapter we have described the use of integrated EMG feedback for the treatment of focal dystonia or spasmodic torticollis. Although we have achieved significant results, it remains clear that further research in the treatment of these disorders is required. However, since this treatment does not require medication or surgery and the possibility for significant improvement is greater than 40%, it should be attempted in patients with focal dystonia or torticollis prior to other forms of therapy. SFT should be considered as a standard mode in the medical armamentarium used for the treatment of these disorders, either primarily or in conjunction with other forms of medical, surgical, and physical therapy.
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PMID:Integrated EMG feedback in the management of spasmodic torticollis and focal dystonia: a prospective study of 80 patients. 108 48

Advances in the understanding of the relationship of proprioceptive (kinesthetic) feedback to motor physiology have prompted the study of therapeutic effects of audiovisual displays of EMG activity. Patients with various manifestations of disturbed neuromotor control were studied prospectively for three years. This group included 114 patients with hemiparesis, torticollis, dystonia, and spinal cord or peripheral nerve injury. Initially, all but one of these patients had some residual volitional motor activity, which was insufficient for adequate function, and all patients had had conventional therapy with little or no functional recovery. Prior to EMG feedback therapy, the duration of illness was from three months to 35 years. The shaping of a patient's motor responses usually occurred gradually, often over an 8 to 12 week period. This modification was accomplished by feeding processed audio-visual signals back to the patient. These signals were proportional to the degree of activity of the muscles responsible for the defective function. The concept of microvolt-second, as a unit of muscle activity, is introduced and defined. Patterned movements, which were previously defective were observed to improve to varying degrees. Following the initial course of treatment, reinforcement was required by some patients. The mechanisms of improvement after EMG feeback therapy are not well understood; however, some hypotheses are presented. The results of this study indicate that EMG feedback therapy may induce significant functional recovery in patients with disturbed neuromotor control.
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PMID:EMG feedback therapy: review of treatment of 114 patients. 125 43

Botulin A has been introduced for the treatment of local dystonia especially blepharospasm and torticollis. Three cases of blepharospasm and 5 cases of torticollis were treated with botulin injections directly into the muscles by a method presented in detail. Good effects were obtained in blepharospasm but very poor in torticollis, which may have been due to too low doses of the toxin and inadequate choice of injection points. The method is safe and in only 1 case transient weakness of the masseters was noted.
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PMID:[Botulin in the treatment of local dystonia]. 130 5

Intramuscular injections of botulinum toxin (Botox) are followed by a dose-dependent focal paresis which can be used to treat several focal movement disorders. Botox injections are recommended as effective for the treatment of blepharospasm, hemifacial spasm, and cervical dystonia (torticollis). Focal dystonias elsewhere (for example, writer's cramp) can often be treated with similar success. Others, such as oromandibular dystonia, are more difficult to treat. In the case of more generalized dystonias, some focal muscle spasms can be treated with success by local intramuscular injections. New indications are still being investigated, for example in focal tremors and spasticity. Side effects are in general slight and disappear at the end of toxin effect. In general, it is necessary to repeat the injections after a couple of months, due to a cessation of effect after regrowth of nerve terminals. New injections have similar effects even over years of treatment.
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PMID:[Treatment of movement disorders using botulinum toxin]. 141 87

Striatal 18F-Dopa uptake and glucose metabolism were studied by positron emission tomography with 6-L-[18F]fluorodopa and [18F]fluorodeoxyglucose, respectively, in 8 patients with idiopathic dystonia. Patients with abnormal findings on the brain CT and MRI were excluded from this study. The clinical diagnosis consisted of torsion dystonia in 3 patients, focal dystonia limited in the arm in 3 and cervical dystonia (spasmodic torticollis) in 2. The 18F-Dopa uptake, corrected by nonspecific retention in the cerebellum, at 120 min post-administration was evaluated, and increased 18F-Dopa uptake in the putamen and in the caudate head was observed in the patients with idiopathic dystonia compared to the normal controls. The striatal glucose metabolism in the patients with idiopathic dystonia showed no difference with the normal controls. These findings suggest that pathogenetic mechanism of idiopathic dystonia involves increased presynaptic activity of the dopaminergic system in the striatum.
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PMID:Increased striatal 18F-dopa uptake and normal glucose metabolism in idiopathic dystonia syndrome. 143 86

Dystonias can be classified by etiology (idiopathic or symptomatic), by age of onset (childhood, adolescence or adult), and by anatomical involvement (focal, segmental or generalized). Cervical dystonia (torticollis) is one of the most common focal dystonias. We describe our experience in the treatment of 15 consecutive cervical dystonia patients by chemodenervation with botulinum A toxin (BOTOX), with significant improvement being objectively measured. Botox is accepted as a safe and efficacious modality for the treatment of cervical dystonia.
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PMID:Treatment of cervical dystonia (torticollis) in adults with botulinum A toxin. 151 80

One hundred and twenty-six patients with different forms of focal dystonia (89 with cervical dystonia, 12 with hand cramps and 25 with laryngeal dystonia) were treated with localised injections of botulinum toxin. Mean doses per muscle were 200 mouse units (m.u.) for treating cervical dystonia, 40-120 m.u. for forearm muscles in writers' cramp and 3.7 m.u. for the thyroarytenoid muscle in laryngeal dystonia. Responder rates have been above 80% in all patient groups and beneficial effects could be reproduced over follow-up periods of up to 4 years. The commonest side-effects were dysphagia after treatment of spasmodic torticollis, weakness of neighbouring muscles after injections for hand cramps and breathiness and hypophonia following laryngeal injections. All these were transient and generally well tolerated. It is concluded that botulinum toxin injections are a safe and effective treatment in all three types of focal dystonia.
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PMID:Treatment of cervical dystonia hand spasms and laryngeal dystonia with botulinum toxin. 154 63


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