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)

Five of seven patients with chronic nonfluent aphasia and hemiparesis due to a focal ischemic infarction developed painful hemidystonia during treatment with a high dose of bromocriptine. All seven patients had cortical damage, but four also had basal ganglia and one thalamic involvement. While lesion location did not differ between dystonic and nondystonic patients, the dystonic patients had more weakness than those without dystonia.
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PMID:Bromocriptine-induced dystonia in patients with aphasia and hemiparesis. 823 49

We treated focal hand dystonia in 53 patients with botulinum toxin injections for up to 6 years. Eighty-one percent of the patients improved with at least one injection session. Sixty-five percent of the injections produced transient weakness. We followed 37 of the patients for at least 2 years from the start of treatment, 24 of whom discontinued treatment because of inadequate response, loss of response, inaccessibility of a treatment provider, or the expense of the toxin. Women, who had a greater extent and longer duration of benefit than men, were more likely to continue treatment. The mean interval between injection sessions was 6 months. In most patients, we injected the toxin into the same combination of muscles at each session. The dose of toxin generally fluctuated within a range of 20 units. Side effects were mild and transient and unrelated to the long-term use of botulinum toxin. Botulinum toxin injection is safe and effective for the long-term management of focal hand dystonia.
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PMID:Long-term botulinum toxin treatment of focal hand dystonia. 829 95

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

A 5-year-old Chinese boy presented with difficulty in walking and weakness of his lower limbs for one year, especially towards the evening. Bilateral equinovarus posturing of the feet and tremors of the upper limbs were noted on physical examination. Dopa-responsive dystonia was diagnosed after a remarkable symptomatic response to levodopa. This disorder is reported here to highlight an often misdiagnosed condition is children which is important because it is treatable. Dopa-responsive dystonia should be considered in the differential diagnosis of gait disturbance in children.
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PMID:Dopa-responsive dystonia of childhood: a case report. 855 5

A 29-year-old man suffered from dystonic writer's cramp for over three years. When he wrote, typed and did other tasks using right hand, dystonic involuntary movement triggered medial rotation of the arm, wrist extension and shoulder elevation. Medication, biofeedback, and botulinum injection were performed without much success. We tried to block the sensory input from muscles by using lidocaine and ethanol. We made injections of 0.5% lidocaine 50ml and 99% ethanol 5ml into muscles with abnormal activity at the frequency of twice a week for about six months. After the treatment, dystonic movement was remarkably improved and he was then able to write, type and perform other tasks with the right hand. Side effects included pain of the injection site, nausea and dizziness, which lasted for a few hours. This "muscle afferent block" did not cause muscle weakness. We speculate that muscle afferent plays a pivotal role in dystonia so that its blocking may be of clinical use.
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PMID:[Muscle afferent block for the treatment of writer's cramp]. 872 Mar 30

We report the results of treatment of 16 patients (14 women, two men; 18-81 years old) with nonoccupational limb and trunk dystonia with botulinum toxin A (BTX; Dysport). A total of 18 clinical problems were identified. Outcomes were assessed in terms of pain relief and improvement in posture and function by the combined observations of the patient and physician. Patients' satisfaction with treatment was high--the benefit in 15 of 18 problems was rated as good to excellent. Reduction in pain was achieved in nine of 10 painful problems, with total relief in four cases. Some normalisation of posture was obtained in 17 of 18; it was complete in three cases. Functional improvement was less common (10 of 18). Excessive weakness was the most common side effect, affecting five patients, but it was disabling in only two. We conclude that BTX can provide substantial benefit with minimal side effects in the majority of patients with these conditions, particularly with pain relief and postural improvements.
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PMID:Treatment of nonoccupational limb and trunk dystonia with botulinum toxin. 881 16

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

A 49-year-old man reported several periods of acute paresis of different nerves after exposure to pressure. All palsies showed a good recovery over a period of days to months. The suspected diagnosis of a hereditary neuropathy with liability to pressure palsies was confirmed by the histology of a suralis nerve biopsy, which showed a distinct tomaculous swelling of the myelin. Nine years ago he noticed an acute, distally pronounced palsy of the right arm. A brachial plexus lesion was diagnosed. His profession required written work, thereby forcing him to compensate this weakness by increasing the coactivation of the elbow and upper arm. This coactivation persisted even after complete recovery from the plexus palsy. Thus, the patient showed the typical feature of classical writer's cramp, with broad coactivation of the arm muscles, which has continued to the present. At the same time, however, the patient was able to perform other motoric tasks well, such as painting. After several practice sessions with a special program that makes use of preserved motor abilities to reestablish writing, the patient was again able to write with a normal kinematic profile as documented by the recorded handwriting. This suggests that the "writer's cramp" of our patient is one form of task-related dystonia that may be related more to the perserveration of a misleading motor strategy than to a general inability of the motor system to control the movement properly.
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PMID:Persisting "writer's cramp" as a result of compensation of a temporary palsy due to a hereditary neuropathy with liability to pressure palsies. 886 1

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

The appearance of involuntary movements in the clinical course of reflex sympathetic dystrophy (DSR) constitutes a rare clinical entity. In this context, the most frequent changes in movements are muscle spasms and focal dystonia, although postural tremor, muscle weakness and rhythmic myoclonus have also been described. The disorder is more frequent in young women and in the lower limbs. It may have a focal, segmental, multifocal, hemicorporal or symmetrical distribution. It is almost always secondary to local trauma. The pathogenesis and most effective treatment are unknown. We present the case of a 62 year old woman with muscle spasms of both legs and feet as a complication of spontaneously appearing DSR. The electromyogram showed continuous non-rhythmic discharges with morphologically normal motor unit potentials in both anterior tibial muscles. The clinical course and symptomatic improvement following treatment with benzodiazepine seems to suggest that the disorder is of central origin.
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PMID:[Muscular spasms associated with a reflex sympathetic dystrophy]. 898 30


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