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 clinical features and course of 14 patients with progressive supranuclear palsy (PSP) were analysed. PSP formed 2.3 percent of the parkinsonian population. Blepharospasm, hypersomnia, athetosis, action dystonia, action myoclonus and family history of dementia were the unusual features. Half of the patients had dementia at presentation. Drug therapy was uniformly disappointing. The mean duration from onset to death in 4 patients who died was 4.5 years. The histopathological features in a patient with the disease for one year and who died of acute myocardial infarction showed moderately severe changes characteristic of the disease.
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PMID:Progressive supranuclear palsy. Report of 14 cases with special reference to unusual features. 193 53

Actual phenomena of various types of involuntary movements listed below were demonstrated by moving pictures, which were followed by comments on symptomatology, in particular the fundamental characteristics of an individual involuntary movement. These characteristics are the essence of each involuntary movement, and it is necessary to recognize both its phenomenon itself and its accumulated knowledge in order to realize and interpret the involuntary movement. The following involuntary movements are treated: (1) typical tremor-at-rest in paralysis agitans, (2) atypical parkinsonian tremor, (3) essential tremor, (4) chorea, (5) ballism, (6) athetosis, (7) choreoathetosis, (8) dystonia, (9) spontaneous myoclonus at rest, (10) intention or action myoclonus, (11) intention tremor and (12) hyperkinesis.
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PMID:[Symptomatology of the involuntary movement]. 201 97

Extrapyramidal dysfunction is poorly characterized in Rett's syndrome, a neurodegenerative disorder in girls. We studied the motor and behavioral findings in 32 Rett's syndrome patients, 21 months to 30 years old. In addition to the typical stereotyped movements and scoliosis, other motor disturbances included bruxism, sialorrhea, ocular deviations, parkinsonian findings, dystonia, myoclonus, and athetosis. The types of movement disorders seemed to be age-related, with the hyperkinetic disorders occurring in the younger patients and the bradykinetic disorders occurring more frequently in the older patients.
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PMID:Extrapyramidal involvement in Rett's syndrome. 223 45

The results of stereotactic surgery in 38 patients with cerebral palsy are reviewed. A total of 67 nuclei were approached and 109 stereotactic lesions performed. The motor disorders were spasticity, athetosis, dystonia and tremor. The targets were chosen according to the motor disorder, and included the pulvinar, ventrolateral (VL), sub-VL and cerebellar dentate, and a combination of 2 or 3 of these. The results of the surgery were evaluated on the basis of objective functional improvement using a scale for task achievement. Varying degrees of overall improvement were found in over 80% of the patients; these are discussed in detail.
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PMID:Stereotactic surgery for cerebral palsy. 208 Mar 27

An Indian man with Wernicke's encephalopathy had nystagmus, pupillary changes and confusion, but the unusual and prominent features in his presentation were marked dystonia and choreo-athetosis, which responded rapidly to thiamine. The possible pathogenesis is discussed.
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PMID:Dystonia and choreo-athetosis in Wernicke's encephalopathy. A case report. 272 44

The diagnosis of postinfectious encephalomyelitis with symmetric lesions in the basal ganglia was confirmed by magnetic resonance imaging in 2 patients. A 7-year-old patient experienced severe dystonia and hyperreflexia; magnetic resonance imaging demonstrated bilateral lesions in the putamina and basis pontes. The other patient, a 2-year-old female, manifested hypotonia, facial grimacing, and athetosis. Symmetric lesions in the globus pallidus and substantia nigra were demonstrated by imaging studies. The nature and monophasic course of illness in these 2 patients, as well as the symmetric involvement of specific regions of the basal ganglia, may result from an immune-mediated postinfectious demyelinating process.
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PMID:Postinfectious encephalomyelitis with localized basal ganglia involvement. 280 90

Authors report a study concerning 12 dyskinetic patients with cerebral palsy. The clinical pre-operative examination shows that many signs and symptoms are associated: volitional and postural dyskinesia, athetosis and dystonia, pyramidal deficit and spasticity. Talairach's stereotactic methodology has been used for bifocal (VPL thalamic nucleus and internal pallidum) Yttrium 90 implantation. After stereotactic bifocal lesions, involuntary movements have been reduced in 45.5% of cases and have disappeared in 27% of cases. Impairment of previous motor deficit has been observed in 3% of cases; volitional and postural dyskinesia seems to be the most curable symptomatology. Clinical results in athetoid involuntary movements and dystonia are less rewarding. Because of important anatomical modifications often observed cerebral palsy patients, the authors stress the interest of individual acute neurophysiological study and discuss about the stereotactic targets and the modalities of destruction. They insist upon the necessity of rigorous selection of indications based on acute clinical examination in the perspective of improvement of global functional capacities.
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PMID:[Value of bifocal stereotaxic destruction in case of dyskinesia in patients with a motor deficit of cerebral origin]. 332 99

A novel substance, tiaprid (Tiapridex), from the family of the substituted benzamides, can be used in an attempt to block the dopaminergic receptors of the corpus striatum without inducing the psychiatric side effects on the mesolimbic system associated with, for instance, neuroleptics. Since hyperkinetic illness of the brain-stem ganglions accompanied by myoclonia, athetosis and dystonia are believed to stem from a state of imbalance between dopaminergic and cholinergic processes in which dopaminergic activity predominates, the application of tiaprid was tested on patients with etiologically different illnesses in an open clinical study. The course of treatment was documented by measurements and a record of clinical findings. No tolerance problems were encountered, and the substance had no grave side effects. Its effects proved to be good to very good on myoclonic hyperkinesis and good to moderate on athetosis, but dystonia seemed to be resistant to this form of treatment.
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PMID:[The possibility of pharmacologic therapy of extrapyramidal hyperkineses with tiapride]. 622 30

6 cases with tremor-athetotic type cerebral palsy and 2 cases with moderate dystonia-tremor type cerebral palsy were treated by selective stereotactic thalamotomy. In the former group, postural-movement type tremor in the upper limb gradually progressed with age while athetosis remained unchanged. In the latter group, dystonia in the truncal muscles predominated over the irregular tremulous movement of the upper limbs. In all cases, the intelligence was almost normal. Stereotactic selective thalamotomy (Vim for tremor athetosis, VL-Vim for dystonia tremor) was performed under local anesthesia with the aid of radiological and neurophysiological control methods. The results of the operations were satisfactory in regard to the tremor relief and concomitant improvement of motor performances in most of the cases. Stereotactic treatment might be an effective way to make possible a one-step progress in these handicapped cases. The importance of postoperative physical therapy is also emphasized.
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PMID:Stereotactic selective thalamotomy for the treatment of tremor type cerebral palsy in adolescence. 634 49

The descriptive aspects of all types of movement disorders and their related syndromes and terminologies used in the literature are reviewed and described. This comprises the features of (a) movement disorders secondary to neurological diseases affecting the extrapyramidal motor system, such as: athetosis, chorea, dystonia, hemiballismus, myoclonus, tremor, tics and spasm, (b) drug induced movement disorders, such as: akathisia, akinesia, hyperkinesia, dyskinesias, extrapyramidal syndrome, and tardive dyskinesia, and (c) abnormal movements in psychiatric disorders, such as: mannerism, stereotyped behaviour and psychomotor retardation. It is intended to bring about a more comprehensive overview of these movement disorders from a phenomenological perspective, so that clinicians can familiarize with these features for diagnosis. Some general statements are made in regard to some of the characteristics of movement disorders.
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PMID:Clinical features of movement disorders. 662 43


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