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)

We describe clinical experiences in the management of three patients with laryngopharyngeal dystonia causing severe breathing problems. In contrast to spasmodic dysphonia, which presents with action-induced involuntary spasm of laryngeal muscles during speaking, all three patients showed laryngopharyngeal spasms primarily during respiration. In analogy to spasmodic dysphonia we propose the term spasmodic laryngeal dyspnea for this rare condition. Localized unilateral botulinum toxin injected into the thyroarytenoid muscle and/or ventricular folds reduced the quantity and quality of spasms and led to a pronounced improvement of breathing problems.
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PMID:Spasmodic laryngeal dyspnea: a rare manifestation of laryngeal dystonia. 919 49

Dystonia is a rare neurologic disorder of the basal ganglia presenting with involuntary twisting or turning spasm of muscles. Movements localized to the face, eyes, or neck generally present during late adulthood. Cranial dystonia is usually idiopathic but may be caused by trauma or medications. Of 148 patients with focal dystonia referred to Indiana University over four years, four women had the onset of face and neck symptoms eight days to 34 months after completing treatment with chemotherapy alone or combined with radiation therapy. Two patients were treated with 5-FU, one received doxorubicin and one was treated with both. Both drugs have been associated with transient parkinsonism, but no chemotherapeutic medications have been reported to cause dystonia. Three patients remain free of demonstrable malignancy. A possible association of chemotherapy and focal dystonia has not been previously described.
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PMID:Focal dystonia after chemotherapy: a case series. 921 64

Despite the clinical potential of botulinum toxin type B (BTXB) for treating focal dystonia, hemifacial spasm, and other movement disorders, particularly in those resistant to botulinum toxin type A (BTXA), no objective human data exist to compare the muscle paralysis resulting from these two botulinum toxin subtypes. To objectively compare the human muscle paralysis resulting from intramuscular injections of BTXB with that from BTXA, we measured the extensor digitorum brevis (EDB) M wave amplitude four times before and six times after injection with 17 different doses of BTXB (from 1.25 to 480 units) in 17 healthy volunteers. This established a dose-response curve that we compared with the previously published BTXA dose-response curve. After the establishment of the dose-response curve, we injected 10 new volunteers with five different doses of BTXB and BTXA measuring EDB M wave amplitude 4 times before and 13 times over 57 weeks after injection. The volunteers were randomized by dose and received BTXA and BTXB in opposite EDB muscles. The effect of the toxin in all volunteers was expressed as percent decline in M wave amplitude postinjection (% paralysis). The maximal paralysis 2 weeks postinjection with 320 to 480 mouse units (MU) of BTXB was 50 to 75%, whereas maximal paralysis was 70 to 80% with 7.5 to 10 MU of BTXA. Postexercise M wave facilitation on day 9 postinjection averaged 63% for BTXB and 20% for BTXA. Seven weeks postinjection, BTXB-induced paralysis had improved by 66% with complete improvement by 11 weeks postinjection, whereas BTXA-induced paralysis had improved by only 6% at 7 weeks, and at 57 weeks postinjection 22% of the original muscle paralysis was still present. Thus, human muscle paralysis resulting from BTXB injection is not as complete or long-lasting as that resulting from BTXA.
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PMID:Human response to botulinum toxin injection: type B compared with type A. 922 89

Fifty five patients with Meige's syndrome were examined for clinical and demographic features. The mean age of onset was 52.3 years. The peak age of onset was in the sixth decade with a male to female ratio of 1.11:1. The mean duration of illness was 3.7 years. Commonest initial symptom was increased blinking, seen in 30 cases (54.5%). Twenty five patients (45.4%) had complete syndrome of blepharospasm with oromandibular dystonia, whereas 24 patients (43.6%) had blepharospasm alone and the rest (6 patients, 10.9%) had oromandibular dystonia. The extension of spasm beyond cranial muscles was observed in 10 patients (18.1%). Eleven patients had family history of dystonia or other extrapyramidal disorders. Incidence of depression was high in these cases.
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PMID:Clinical and demographic features of Meige's syndrome. 925 78

Dystonia is a neurologic disease characterized by involuntary repetitive muscular contractions which frequently causes torsion, spasmodic movements or abnormal postures. Pharmacologic and surgical treatment had been unsatisfactory in the majority of the cases. Intramuscular botulinic toxin application in focal dystonia disorders, achieve relief in approximately 90% of the cases by quimiodenervation. We included 35 patients with focal dystonia treated between November 1994 through June 1996: 14 facial hemispasm, nine blepharospasm, five cervical dystonia, two writer's cramps, one oromandibular dystonia and four patients with mixed focal dystonia. Improvement between 50-90% was observed in 32/34 (94%) of the patients; one case (2.8%) with therapeutic failure and one case (2.8%) never returned to quantify the response. Actually, botulinic toxin is the first choice treatment of focal dystonia and other diseases related to involuntary muscle spasm.
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PMID:[Treatment of focal dystonia with botulinic toxin]. 928 Sep 25

Therapeutic effect of botulinum toxin A was studied in a group of pediatric patients (n = 28) aged between 6 months and 18 years. The patients were diagnosed with cervical dystonia (n = 6), adductor spasm of the hip (n = 8), spastic drop foot (n = 7) and various other focal motor problems associated with spastic muscular hyperactivity (n = 7). The mean dose of botulinum toxin A (Dysport) used to inject into the affected muscle was 22 U/kg body weight. Reduced muscular hyperactivity with a significant increase in joint mobility was achieved for dystonic (p < 0.0001) as well as for spastic conditions in patients with adductor spasm (p < 0.0002). For these patients the improved joint mobility represented a significant benefit for both daily activities and nursing care. Local paresis and local hematoma were observed in 1/28 and 1/28 patients, respectively; 1/28 patients developed a secondary non-response. However, apart from these side effects, no other adverse reactions to botulinum toxin A treatment were recorded during the treatment and observation period (12-64 months). Our results suggest that botulinum toxin A represents an effective and safe therapeutic substance for the treatment of pediatric patients suffering of focal motor problems due to dystonic or spastic muscular hyperactivity.
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PMID:Interventional neuropediatrics: treatment of dystonic and spastic muscular hyperactivity with botulinum toxin A. 945 27

EMG can be used for both dystonic muscles selection and performing injections of botulinum toxin. The indications for performing injections under EMG guidance in different forms of movement disorders are discussed. There is no need of EMG control in the treatment of blepharospasm and hemifacial spasm. In cervical dystonia the use of EMG guidance can improve the results of treatment in cases of head tilt, retrocollis, shoulder elevation and in more complex forms of dystonia. The injections should be performed under EMG control in patients with limb, oro-mandibular and laryngeal dystonia and with palatal myoclonus.
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PMID:[Botulinum toxin injections under electromyographic guidance]. 960 57

Head, neck, or shoulder trauma is an occasional antecedent event before the appearance of cervical dystonia. A clinically distinctive syndrome of acute-onset posttraumatic cervical dystonia characterized by markedly restricted range of neck motion, absence of phasic involuntary movements, and poor response to treatment has previously been described. Patients with cervical dystonia attending a movement disorder clinic were reviewed for history of trauma before onset of symptoms. Patients with symptom onset within 4 weeks of trauma were compared with patients who developed symptoms between 3 months and 1 year after trauma. Acute-onset cervical dystonia was characterized by markedly reduced cervical mobility; prominent shoulder elevation with trapezius hypertrophy in most patients, absence of involuntary movements, sensory tricks, or activation maneuvers; and poor response to botulinum toxin injection. By contrast, delayed-onset cervical dystonia was clinically indistinguishable from nontraumatic idiopathic cervical dystonia. Acute-onset posttraumatic cervical dystonia is similar to limb dystonia after peripheral trauma and may represent a form of nondystonic muscle spasm similar to torticollis associated with musculoskeletal injuries of the cervical spine and craniocervical junction.
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PMID:Comparison of acute- and delayed-onset posttraumatic cervical dystonia. 961 41

Botulinum toxin A is the more efficient therapy of focal dystonias and hemifacial spasm. Our experience with botulinum toxin A injections in 115 patients is reported. Marked or total improvement was achieved in all 45 patients with hemifacial spasm, in 70% of 20 patients with essential blepharospasm and in 71.4% of 14 patients with Meige's syndrome. In 65.2% of 23 patients with cervical dystonia marked but no total improvement was obtained. The worse results were seen in the 6 patients with hand dystonia (writers cramp), in whom marked improvement was obtained in just two. Mild and transient complications occurred in up to 24.4%, eyelid ptosis and eyelid weakness being the most frequent. One patient with Meige's syndrome had an aspiration pneumonia following dysphagia. Our results are in agreement with others, showing that botulinun toxin A is a useful and safe treatment for these conditions.
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PMID:[Botulinum toxin A: experience in the treatment of 115 patients]. 962 4

Intramuscular injections of botulinum neurotoxin type A cause reversible chemodenervation and subsequent paralysis by blocking the presynaptic release of acetylcholine. Botulinum toxin type A has emerged as the most effective form of symptomatic treatment for abnormabilities in muscle movement (blepharospasm, hemifacial spasm, torticollis) and has been approved for use in these conditions. First results in the treatment of patients suffering from oromandibular dystonia, myogenic craniomandibular dysfunction and recurrent dislocation of the temporomandibular joint are presented. In most cases, therapeutic effects occurred within 1-6 days post-injection. Muscular hyperfunction was reliably reduced and involuntary activity patterns gradually ceased. No severe side effects of the local injections were noted.
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PMID:[New techniques in maxillofacial surgery: local injection treatment with botulinum toxin A]. 965 37


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