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)

The "off" painful dystonia (OPD), usually concerning the feet, is a type of abnormal involuntary movement, induced by the chronic use of levodopa. It is mostly observed in the advanced stage of Parkinson's disease (PD), particularly in the early morning, in the evening, and late at night. Indeed, some patients have experienced OPD also during "on" periods when dystonic posture of the foot alternates with dyskinesia. The pain probably is due to sustained muscle contraction, which causes prolonged muscle spasm, as in primary dystonia or torticollis. Dopaminergic drugs like bromocriptine, pergolide, and especially apomorphine (s.c. infusions, or bolus), can dramatically improve the OPD. Anticholinergics baclofen and lithium are alos used in the management of OPD with some benefit. On the other hand, clinical experience shows that in many cases, these therapeutic procedures are not always enough to produce the expected results. Thirty PD patients (22 men and eight women) with OPD of the foot were treated with botulinum toxin (Botox, Btx) using electromyograms to guide injections. Dystonia was evaluated using a quantitative rating scale. The selection of the muscles for Btx treatment was carried out on the basis of foot posture. We injected Btx into tibialis posterior, tibialis anterior, gastrocnemius, flexor digitorum longus, and extensores hallucis longus with a median dose 40 IU for each muscle, distributed in two sites. In all patients, the pain improved within 10 days, whereas in 21 patients, the pain disappeared completely for 4 months (range, 3-7 months); a concomitant improvement in intensity of the dystonic spasm was also observed. No side effects were reported. Seven patients with associated "on" foot dystonia described an improvement of foot posture on walking. In conclusion, in this uncontrolled study, the use of Btx in OPD seemed a promising tool to improve pain linked to foot dystonia; however, because of the well-known underlying dopaminergic defect in OPD, the Btx therapy should be considered only if the dopaminergic treatment established for the management of OPD has failed.
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PMID:"Off" painful dystonia in Parkinson's disease treated with botulinum toxin. 765 52

Glabellar frown lines and crow's feet are wrinkles of facial expression related to an underlying muscular activity, which is particularly strong during facial expression. Classic treatments of these wrinkles only give partially satisfactory are associated with results, and secondary effects, whether they involve skin and muscle lifting, surgical section of muscles, dermal stimulation by thread or injectable fillers, chemical or mechanical abrasion, transient or permanent soft tissue augmentation with various materials. The authors studied the efficacy and safety of intramuscular injections botulinum A Exotoxin in glabellar and crow's feet areas in 19 well-informed and consenting patients. Botulinum toxin injections have been used since 1980 in the treatment of focal dystonia (blepharospasm, oromandibular dystonia, spasmodic torticollis, spasmodic dysphonia and writer's cramp) and safety hemifacial spasm. Their wide use in these indications has highlighted their excellent and efficacy, and the need to repeat injections every 3 to 4 months. The dose required was progressively adjusted around glabellar and orbital areas, while injections of the peri-buccal and forehead areas are still being evaluated. The 19 patients were examined clinically, filmed and photographed every month over a period of 12 to 24 months, and skin prints were performed. Evaluation criteria included the percentage improvement as assessed by the patients themselves, and also evaluation by the investigators of the data of clinical examination, and blind comparison of photographic, videoscopic, and prints. The authors obtained a significant decrease of wrinkles of the areas studied, with a "smoothing" effect during the period of activity of the toxin, which lased an average of 3 to 4 months at the beginning, and 6 to 9 months after several injections. No secondary effects, either general or local, were observed. The product's specificity means that the operator must have a complete mastery of the injection technique and a thorough knowledge of its pharmacology.
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PMID:[Botulinum toxin in the treatment of frontoglabellar and periorbital wrinkles. An initial study]. 766 8

Botulinum A toxin injection is the most recent and effective treatment of various movement disorders especially focal dystonia. Spasmodic torticollis is one focal dystonia which responds poorly to both medication and surgery. Botulinum A toxin injection has been adopted as a treatment procedure at the Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand since 1989 (before the American Food and Drug Administration approval) as a research protocal for Thai patients. This report is the first ever study of this treatment for Thai patients with spasmodic torticollis. Fifty six spasmodic torticollis patients who had been treated with botulinum A toxin injection at the Movement Disorder Clinic, Siriraj Hospital were analysed. Thirty six patients were male and the male to female ratio was 1.8:1. Most of the patients (76.79 per cent) were aged between 20-49 years and half of them were from Bangkok. Twelve patients (21.43 per cent) were classified as simple torticollis, 35 patients (62.5 per cent) were combined torticollis, 7 patients (12.5 per cent) were retrocollis, and 2 patients (3.57 per cent) were lateral collis. Three patients had generalised dystonia and 2 patients had segmental dystonia. Duration of suffering in each patient ranged from 1 month to 25 years with the mean duration of 3.70 (S.D. 5.09) years. Only four patients (7.14 per cent) refused botulinum A toxin injection due to their mild symptoms. The remaining 52 patients were given botulinum A toxin injection of 30-120 international units into the most overactive group of muscles which were responsible for abnormal neck posture (mainly sternocleidomastoid and splenius capitis). Eight patients (15.38 per cent) were lost to follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Botulinum A toxin treatment in spasmodic torticollis: report of 56 patients. 770 65

To investigate the pathophysiology of dystonia, we recorded contingent negative variation (CNV) in 12 patients with cervical dystonia and in 12 age-matched normal subjects. In a simple reaction time paradigm, the subjects were given a pair of a warning stimulus and a subsequent stimulus that triggered head rotation to either side or extension of the fingers. In normal subjects, CNVs for head rotation were not affected by neck muscle pre-activation simulating torticollis, and were always symmetrical with equally high amplitudes over the frontal and central leads. By contrast, CNVs for finger movement had the maximum over the central lead and showed a characteristic distribution; those for the right finger movement had the left hemisphere dominance, whereas those for the left finger movement had similar amplitudes over both hemispheres. In patients with rotatory torticollis (rotatocollis), the components of CNVs for head rotation were markedly attenuated in all the leads, except for the initial negative deflection (orienting response). As a whole, cervical dystonia patients had significantly lower amplitudes of late CNVs for head rotation than normal subjects (P < 0.001), whereas late CNV amplitudes in finger extension did not differ in the two groups. Their reaction times for head rotation were similar, but durations of EMG activities were prolonged in the patients because of co-contractions of the antagonists. The task-specific CNV amplitude loss is therefore not explained by reaction times or by the abnormal neck muscle activities prior to the movement, but it reflects a failure of neural activities preparing for a phasic neck movement, resulting in co-contraction of the agonists and the antagonist. Dystonia may be associated with defective retrieval or retaining of specific motor programmes or subroutines in response to sensory stimuli.
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PMID:Physiological study of cervical dystonia. Task-specific abnormality in contingent negative variation. 773 91

We reviewed the histories of 115 patients who had idiopathic dystonia and who were 21 years of age and younger at onset of symptoms and 472 patients with onset older than age 21. Patients with onset in the lower extremities tended to be younger at onset (mean age 8.4 years), have rapid spread of symptoms to other body parts, and to develop generalized dystonia. Patients with onset in the upper extremities tended to be older at onset (mean age 11.2 years) and were less likely to develop generalized dystonia, but were more likely to experience spread of symptoms many years after the disease began. Almost 20% of patients younger than age 22 began with torticollis, and 67% of these remained focal after a mean 14.9 years. In adults, dystonic symptoms remained focal in the majority, but approximately 15-30% of patients presenting with blepharospasm, torticollis, laryngeal or brachial dystonia eventually experienced dystonia outside the initially involved segment. Almost 50% of children and adolescents with dystonia reported a family history of dystonia, compared with approximately 10-13% of patients with torticollis, laryngeal or brachial dystonia. Less than 3% of patients with blepharospasm reported a family history of dystonia. There were only minor differences in the pattern of spread of dystonic symptoms between Jewish and non-Jewish patients. Although younger patients were more likely to report a family history of dystonia, patterns of spread were the same for familial and sporadic patients in the same age range.
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PMID:Spread of symptoms in idiopathic torsion dystonia. 775 56

Dystonia is characterized by slow, repetitive, involuntary, often twisting movements leading to sustained abnormal postures and can be focal or generalised. In most cases, etiology remains unrevealed, some cases are hereditary, others have metabolic origin or can be attributed to focal brain lesions. The most common types of dystonia, spasmodic torticollis and blepharospasm, can now be treated successfully, albeit symptomatically, with Botulinum toxin injections into the affected muscles. A practical guide to differential diagnostic and therapeutic strategies is presented.
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PMID:[Clinical aspects and therapy of dystonias]. 775 50

This paper reports on a case of spasmodic torticollis after longterm treatment with neuroleptics. This form of dystonia is called tardive dystonia to distinguish it from tardive dyskinesia. Its pathophysiology is unknown. Pathological changes are described in the basal ganglia. Increased signal was found on magnetic resonance imaging on both sides of the basal ganglia reflecting structural lesions. These structural changes, together with neuroleptic medication, represent predisposing factors for the manifestation of tardive dystonia.
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PMID:[Tardive dystonia. A rare neuroleptic-induced disease picture]. 781 58

The authors made a meta-analysis of the results of botulinum toxin injection for the treatment of spasmodic torticollis. They concluded that botulinum toxin is effective for the treatment of cervical dystonia.
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PMID:[Botulinum toxin the treatment of spasmodic torticollis: a meta-analysis]. 782 61

A 45-year-old white male with a positive family history for various neurological disorders (not including dystonias), and a long history of neuroleptic exposure on account of a chronic schizoaffective illness, developed severe torsion trunkal movement to the left which were accompanied by inversion of the left arm and outward extension of the right arm, as well as left torticollis. The axial dystonia, which was refractory to treatment, was disabling interfering with the activities of daily living, posture and gait. Radiological studies revealed marked dextroscoliosis. The administration of clozapine in dosages of 200 mg p.o. in the morning and 250 mg p.o. at bedtime, resulted in a significant improvement of the neck and trunk dystonia. After the discontinuation of the clozapine, an exacerbation of the movement disorder back to baseline levels prior to the use of this agent, was observed. Subsequent therapy with thioridazine in a dosage of 600 mg/day did not mask or treat the dystonia. The clinical implications of these findings are discussed.
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PMID:Improvement of axial dystonia with the administration of clozapine. 792 26

Twenty index patients with hereditary essential tremor and their kindreds were studied to define the phenotype of this condition. Ninety-three first degree and 38 more distant relatives were examined; 53 definite and 18 possible secondary cases were identified. The age of tremor onset was bimodally distributed with a median at approximately 15 years. Segregation analysis indicated autosomal dominant inheritance and penetrance was virtually complete by the age of 65 years. There were no examples of the disease skipping a generation. Men and women were affected in equal proportions. About 50% of cases were alcohol responsive. In the majority of families alcohol responsiveness was either consistently present or did not occur, but in 20% of kindreds definite heterogeneity of responsiveness was encountered within each family. The typical phenotype was a mild symmetrical postural tremor of the upper limbs. Tremor of the legs, head, facial muscles, voice, jaw and tongue occurred but never in isolation and rest, task specific (e.g. primary writing tremor) and primary orthostatic tremors were not found. Head tremor was invariably mild and 75% was of a 'no-no' type. Dystonia (e.g. torticollis and writer's cramp) were not encountered, a finding which strongly suggests that many previous studies of 'essential tremor' were contaminated by cases of idiopathic or hereditary torsion dystonia. No association with Parkinson's disease was found but classical migraine occurred in approximately 26% of cases and co-segregated with tremor. The severity of arm tremor (assessed using a clinical rating scale and by scoring tremor in Archimedes spirals) and disability increased with advancing age and increasing tremor duration, but there was no correlation between age at tremor onset and either tremor severity or disability. Men and women were affected with equal severity. The sex of the affected parent had no influence on the severity of tremor or the degree of disability experienced by an affected child. Disability commenced in the second decade and progressively increased. All the index patients and 59% of the definite secondary cases had tremor induced disabilities. Eighty-five percent of index patients and 38% of secondary cases also reported some degree of social handicap. Twenty-five percent of index patients and 12% of secondary cases had been compelled to change jobs or retire. Biological fitness was normal.
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PMID:A study of hereditary essential tremor. 792 67


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