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)

In this review, the authors present a critical overview of the historical development, indications, complications, operative techniques, and results of procedures for the alleviation of the major dyskinesias. Emphasis is placed upon recent refinement of technique, particularly stereotaxis, as well as neurophysiologic stimulation and recording, computerized tomographic scanning (CT) and magnetic resonance imaging (MRI). Specific disorders that may be amenable to surgical therapy include spasticity secondary to spinal cord pathology, cerebral palsy, and multiple sclerosis; the tremor and rigidity of Parkinson's disease; essential tremor; dystonia; spasmodic torticollis; post-traumatic and postinfarction intention tremor; cerebral palsy with tremor; hemiballismus; myoclonus; and dyskinesias induced by L-DOPA.
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PMID:Neurosurgical management of spasticity, rigidity, and tremor. 332 80

Examination of brains from four individuals with the clinical diagnosis of primary dystonia revealed histopathologic abnormalities in two cases. A 29-year-old man with a 15-year history of dystonia musculorum deformans (DMD) had numerous neurofibrillary tangles (NFT) and mild neuronal loss within the locus ceruleus; occasional NFT were also recognized in the substantia nigra pars compacta, pedunculopontine nucleus, and dorsal raphe nucleus. A 68-year-old man with a 35-year history of Meige syndrome had moderate-to-severe neuronal loss in several brainstem nuclei, including the substantia nigra pars compacta, locus ceruleus, raphe nuclei, and pedunculopontine nucleus. Infrequent NFT were also noted in substantia nigra. An examination of these and other brain regions in a 10-year-old boy with a 6-year history of DMD and a 50-year-old woman with a 3-year history of spasmodic torticollis did not disclose similar abnormalities.
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PMID:Pathology in brainstem regions of individuals with primary dystonia. 336 65

In 131 patients treated exclusively by selective denervation during the past 10 years, all or almost all the abnormal movements of spasmodic torticollis were suppressed in 115 (88%) while preserving posture and mobility. This approach was also used in certain forms of adult-onset dystonia. An appreciable amount of abnormal movements remained in the other patients, either due to residual innervation or because of limitation of denervation necessary to preserve normal movements (laterocollis) or neck stability (retrocollis). A medio-lateral approach to the posterior cervical region in the sitting position using stimulation under light anaesthesia is recommended.
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PMID:Observations and analysis of results in 131 cases of spasmodic torticollis after selective denervation. 345 Feb 40

Regional cerebral glucose metabolism was studied in 16 patients with idiopathic torticollis, using positron emission tomography. Analysis of subcortical regions revealed no consistent focal abnormality of cerebral metabolic rate for glucose, but there was a bilateral breakdown of the normal relationships between the thalamus and basal ganglia. The findings suggest disruption of the pallidothalamic projections in this focal dystonia and may imply a disturbance of GABA.
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PMID:PET studies of cerebral glucose metabolism in idiopathic torticollis. 348 80

Medical treatment of dystonia usually results in an incomplete response and is frequently unsuccessful. Peripheral surgical therapy is available for some focal dystonias, but may only offer temporary relief and may have unacceptable complications. We have used local injections of botulinum toxin into the appropriate muscles for treatment of disabling focal or segmental dystonia in 93 patients with torticollis, blepharospasm, oromandibular dystonia (OMD), limb dystonia, lingual dystonia, and dystonia adductor dysphonia, in addition to four patients with hemifacial spasm. Significant relief of motor symptoms was seen in 69% of the patients with blepharospasm and 64% of patients with torticollis; 74% of the latter group with pain experience relief. Relief of symptoms was noted in most patients with OMD and limb dystonia, and all with lingual dystonia, dystonic adductor spastic dysphonia, and those with hemifacial spasm. Benefit averaged 2 1/2-3 months initially; however some patients experienced longer relief with subsequent injections. Adverse effects were transient, although 2 patients developed antibodies against the toxin, and we documented evidence for distant effects in others. This approach of chemically weakening contracting muscles in focal dystonia offers many advantages over pharmacotherapy and surgical therapy. Additional experience is needed to explore the proper doses, and potential for long term adverse effects.
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PMID:Localized injections of botulinum toxin for the treatment of focal dystonia and hemifacial spasm. 350 53

A 76-year-old man is reported with advanced progressive supranuclear palsy (PSP) who developed a persistent, gradually progressive torticollis over a period of several months. Blepharospasm and dysfluency of the extrapyramidal type antedated the torticollis. This first report of torticollis in PSP reinforces previous notions that torticollis is related to pathologic changes in the striatum and brainstem. In addition, the combination of torticollis and blepharospasm in our patient supports the previous concept that these two "focal dystonias" have a common pathophysiologic mechanism. This also suggests that dysfluency in PSP may be an expression of a focal dystonia involving the muscles of articulation.
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PMID:Progressive supranuclear palsy: report of a case with torticollis, blepharospasm, and dysfluency. 379 10

Lisuride hydrogen maleate, 0.4 to 5 (mean, 3) mg/d, was given orally to 42 subjects with various types of dystonia. In seven of the eight patients who improved (one with segmental dystonia, one with myoclonic dystonia, two with spasmodic torticollis, two with cranial dystonia, and two with tardive dystonia), the response was confirmed by double-blind placebo substitution. No patients with a suspected structural brain lesion improved. There was no consistent pattern of response among those patients with different forms of idiopathic (primary) dystonia. Lisuride improved some patients, but had no effect on other, apparently identical, patients.
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PMID:Lisuride in dystonia. 388 15

We reviewed the frequency of spontaneous remissions in spasmodic torticollis (ST). One hundred sixteen patients with idiopathic ST (72 F, 44 M) were examined. The age at onset ranged from 9 to 69 (mean, 38.1 +/- 1.3). Twenty-one patients (18%) were Jewish. Eleven patients (9%) had a history of familial dystonia. Remissions longer than 1 year unrelated to treatment were observed in 14 patients (12%) (9 F, 5 M). They occurred in the first year of ST in 13 patients (93%) and in the eighth year in 1. Duration of remissions ranged from 1 to 20 years (mean, 6.5 +/- 1.6). Two patients had three remissions, and another had two. The mean age at the onset of ST in patients with remission was 26.4 +/- 3.3 (SEM) and ranged from 9 to 49. The age at the onset in the patients without remissions was 39.7 +/- 1.4, ranging from 10 to 69 (p less than 0.01). In the remission group, 3 patients were Jewish (21%); in the non-remission group, 18 (18%) were Jewish. There was a familial history in 1 case with remission (7%) and in 10 cases (10%) without remission. Spontaneous remissions in the course of ST seem to be more frequent in patients with early onset, and they occur usually during the first year.
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PMID:Spontaneous remissions in spasmodic torticollis. 395 8

An intravenous infusion of a 250-ml, 10% ethanol solution decreased dystonic scores in five of seven patients with spasmodic torticollis, but had no effect in patients with Meige syndrome, tardive dystonia, or generalized torsion dystonia. Alcohol may temporarily decrease some forms of dystonia.
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PMID:Effect of alcohol on dystonia. 396 14

In a case of therapy-resistant spasmodic torticollis administration of nitrous oxide (N2O) resulted in a dramatic transient amelioration of the dystonic movements. We suggest that manipulations of the opioid system may be useful in the therapy of this condition. Furthermore, our results with N2O may provide insight into the pathogenesis of the disease.
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PMID:Nitrous oxide ameliorates spasmodic torticollis. 405 76


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