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

We report the results of botulinum toxin type A (Dysport, Porton Products, UK) treatment over 5 years in 107 patients with blepharospasm, Meige's syndrome, oromandibular dystonia, hemifacial spasm, cervical dystonia, and writer's cramp. Electromyography was used to localize dystonic muscles and guide Dysport injections in Meige's syndrome, oromandibular dystonia, cervical dystonia, and writer's cramp. All but 2 Meige's syndrome and 2 writer's cramp patients responded to treatment. Improvement was dramatic in blepharospasm (79%) and hemifacial spasm (90%); pronounced in cervical dystonia (74%); and moderate in Meige's syndrome (53%), oromandibular dystonia (57%), and writer's cramp (34%). Although Dysport doses were 50-75% lower than usually reported, response and improvement rates as well as relapse intervals were similar to those of others. To treat cervical dystonia relapses, only 50% of the initial dose was required for continued optimal relief of symptoms. Low-dose Dysport was associated with a very low incidence of dysphagia in cervical dystonia.
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PMID:Five-year experience in the treatment of focal movement disorders with low-dose Dysport botulinum toxin. 778 62

A 55-year-old woman with known retinitis pigmentosa for 25 years was progressively clumsy in gait and activities of daily living over the past 30 years. She was able to manage house work and social activities, but she developed swallowing disturbance associated with involuntary neck muscle spasm for 2 weeks, which brought her to our clinic on September 7, 1990. General physical examination was normal except for dry skin. Neurological examination was compatible with sensory-dominant polyneuropathy, showing distal dominant sensory impairment together with absent vibration sense and areflexia in lower limbs, but no gross muscular weakness. There were neck dystonia and bilateral poor visual acuity due to secondary optic atrophy of retinitis pigmentosa. The former responded to the combination of tiapride and trihexyphenidyl. She was admitted twice for further evaluation. Complete blood count and blood chemistry tests including lipids were all within normal limits, and so was cerebrospinal fluid. Pyruvate and lactate before and after exercise loading were also normal. Malignancy workup was negative. To our surprise, serum vitamin E level turned out very low (1.89 micrograms/ml), normal range being 4.7-20.3 micrograms/ml. Oral vitamin E administration test by 2g of alpha-tocopherol showed abnormal absorption curve followed fast clearance in serum. Stool was occasionally positive for fat corpuscles by Sudan III staining, but 99Tc-HSA leakage into the intestines was not detected.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A sporadic case of essential vitamin E deficiency manifested by sensory-dominant polyneuropathy and retinitis pigmentosa]. 782 14

We report the first Indian experience of botulinum toxin A in the treatment of blepharospasm and hemifacial spasm. Sixteen patients, 7 with essential blepharospasm, 5 with Meige syndrome and 4 with hemifacial spasm received botulinum toxin A injection. One patient received 3 courses of injections, 2 received 2 courses and the rest received only one course. The effect was observed after a latent period of less than 48 hours in all patients and lasted for a mean of 16.65 weeks. More than 70% improvement occurred after 17/20 injections (85%). Poor response was more often seen when blepharospasm was associated with oromandibular dystonia (2/5 injections). Though the duration of response and subjective score of improvement was best in patients with hemifacial spasm, the numbers were very small for any statistical evaluation. The side effects were local, transient, mild and well tolerated. The commonest side effect was blepharoptosis.
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PMID:Botulinum toxin A in blepharospasm and hemifacial spasm. 786 May 9

Focal dystonias include blepharospasm, torticollis, writer's cramp and laryngeal dystonia. They are not uncommon and are disabling. Botulinum toxin has recently been developed for injection into the muscles in spasm and can relieve symptoms in many patients.
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PMID:Consensus statement for the management of focal dystonias. 812 47

The use of botulinum-A toxin will be described in two conditions--the extrapyramidal syndrome of dystonia and the pyramidal deficit, spasticity. There is no cure for dystonia and its cause is unknown. Drug therapy is unpredictable and dose-limiting side effects frequently occur with little or no alleviation of symptoms. Spasticity of adductor muscles in the lower limbs causes profound disability and major nursing problems in patients with chronic disorders of the pyramidal tract. As in the case with dystonia, drug therapy is unsatisfactory. At the UBC Movement Disorders Clinic treatment with botulinum-A has been applied to over 400 patients since 1985. The results of the first studies using this treatment in spasmodic torticollis (the most common form of focal dystonia) and spasticity (in late stage multiple sclerosis) will be discussed. As well the effects of long term treatment will be addressed. Botulinum-A toxin is approved treatment for strabismus, blepharospasm and hemifacial spasm. Approval for its use in other focal dystonias is anticipated. The very nature of the agent used for treatment requires that patients be well prepared and reassured before they undergo their first treatment. There is a wide gulf between the patients' preconceived notions about the treatment and reality.
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PMID:Local treatment of dystonia and spasticity with injections of botulinum-A toxin. 827 87

In six Centers belonging to the Italian Movement Disorder Study Group, the efficacy of botulinum toxin treatment was evaluated in an open collaborative study in 251 patients with focal dystonia and hemifacial spasm. The percentage of functional improvement ranged from 66% to 81% in patients with blepharospasm, from 40% to 51% in patients with spasmodic torticollis and from 73% to 81% in those with hemifacial spasm. Good results were also obtained in patients with oromandibular dystonia, laryngeal dystonia and writer's cramp. Side effects were mild and transient. Local botulinum toxin injection is the first choice symptomatic treatment in focal dystonia and hemifacial spasm.
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PMID:Botulinum toxin treatment in patients with focal dystonia and hemifacial spasm. A multicenter study of the Italian Movement Disorder Group. 835 32

Local injections of botulinum toxin constitute the only truly effective treatment of certain abnormal movements and focal dystonias. The authors describe its indications and report on their personal experience. One hundred and seventeen patients were treated: 48 for blepharospasm, 46 for hemifacial spasm and 23 for spasmodic torticollis. The results were evaluated by means of a score taking into account the effectiveness of treatment, the duration of this effectiveness, the side-effects, if any, observed, and the course of the neurological disorder after several series of injections. The results were good or excellent in 91 percent of patients with hemifacial spasm and 79 percent of patients with blepharospasm. Spasmodic torticollis was much improved in 35 percent of the cases and less, but satisfactorily, improved in 48 percent. In this disease, the muscles which antagonize those responsible for the dystonia must absolutely be re-educated.
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PMID:[Treatments by local injections of botulinum toxin in neurology. Indications and results]. 836 20

A new surgical procedure with potential application for the treatment of adductor spasmodic dysphonia was performed on ten rabbits to assess surgical effects on laryngeal function. Using an external approach, partial unilateral thyroarytenoid (TA) muscle excision was performed through a thyroplasty cartilage window. The contralateral side was left undisturbed as a control. The animals were studied acutely and at 3 months using videolaryngoscopy. Electrophysiologic measurements were recorded at 3 months. The procedure was well tolerated by all animals, with no postoperative infection or aspiration. At 3 months, spontaneous and evoked (recurrent laryngeal nerve stimulation) TA muscle electromyographic potentials were measurable bilaterally. TA compound muscle action potential amplitudes were reduced on the side of myectomy. The threshold of recurrent laryngeal nerve stimulation needed to produce observable vocal fold adduction was increased on the side operated on. Perioperative and long-term (3 months) videolaryngoscopy demonstrated preservation of laryngeal competence with good true vocal cord adduction. Histologic analysis with whole organ sections showed replacement of excised muscle with loose fibroareolar tissue. No evidence of muscle regeneration was observed. The vocal ligament and vocal fold mucosa were intact and undistorted in all specimens. This procedure is technically simple and appears to effectively result in a functional yet weakened TA muscle. Because myectomy includes motor unit end-plate excision, problems associated with reinnervation may be circumvented. TA myectomy may be applicable in patients with focal laryngeal dystonia to decrease muscle spasm.
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PMID:Partial thyroarytenoid myectomy: an animal study investigating a proposed new treatment for adductor spasmodic dysphonia. 846 39


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