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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients suffering from dystonia and athetosis have been treated by means of chronic electrical stimulation of the paleocerebellum and were followed for over two and a half years. One of the patients showed signs of slight improvement, while no improvement at all was observed in the other patient. Some observations and problems are discussed.
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PMID:Chronic paleocerebellar stimulation in dystonia and athetosis. Report of two cases. 696 21

Six patients who developed extrapyramidal manifestations following poisoning with the organophosphorus (OP) insecticide fenthion are reported. The extrapyramidal features, in order of frequency, were dystonia, rest tremor, cog-wheel rigidity, and choreo-athetosis. The delay in onset of these signs, following poisoning, varied from 4 to 40 days, and they disappeared spontaneously in about 1 to 4 weeks in those who survived. The human extrapyramidal system is rich in cholinergic neurons and acetylcholinesterase (AChE). Inhibition of AChE by fenthion, which has ready access to central neurons on account of its lipid solubility, is postulated as the mechanism underlying the extrapyramidal manifestations.
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PMID:Extrapyramidal manifestations complicating organophosphorus insecticide poisoning. 757 21

Involuntary movements originate from different parts of the nervous system. The character of movements depends upon the site of the lesion and the type of pathological change. The presence of more than one type of involuntary movement in a patient can cause confusion and difficulty in the proper classification of movement disorders, which then leads to problems in the differential diagnosis and appropriate treatment. This work was planned to estimate the prevalence and to study the aetiological factors of chorea, dystonia, athetosis and hemiballismus in Assiut, a representative community of Upper Egypt. This study was carried out on 7,000 families (42,000 subjects) representing different types of communities (2,000 families from urban, 2,000 families from suburban and 3,000 families from rural communities). All members of these samples were personally interviewed at home. Full clinical assessment and special investigations required for the diagnosis of different types of chorea, dystonia and athetosis were carried out in Assiut University Hospital. The prevalence rate for rheumatic chorea was 62/100,000 population and it was significantly higher (p < 0.01) among rural than urban and suburban populations, whereas Huntington's chorea had a prevalence rate of 21/100,000 with no significant difference between different areas studied (urban, suburban and rural). The other two common types of chorea were reported with prevalence rates of 12/100,000 for the encephalitic type and 17/100,000 for the atherosclerotic type. No single case of generalized dystonia was recorded and all cases were of the focal type of dystonia with a prevalence rate of 26/100,000 population. No significant differences were recorded between the different areas studied (urban, suburban and rural).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevalence of chorea, dystonia and athetosis in Assiut, Egypt: a clinical and epidemiological study. 796 3

Dystonia is a persistent attitude or posture in one or other of the extremes of athetoid movement. It may take the form of an over-extension or over-flexion of the hand, torsion of the spine, with arching and twisting of the back or forceful closure of the eyes and a fixed grimace. Dystonia is classified into idiopathic and symptomatic dystonia. Idiopathic dystonia is further divided into generalized, focal and segmental dystonia. Generalized dystonia covers classical torsion dystonia, paradoxical dystonia, myoclonic dystonia, dystonia with diurnal variation and Dopa-responsive dystonia. Dystonic tic, paroxysmal dystonia and hypnotic dystonia show a dystonic posture, although they are also accompanied by various other involuntary movements such as athetosis or chorea. Torticollis, writer's cramp or blepharospasm is assigned to the focal dystonia and Meige syndrome to the segmental dystonia. Symptomatic dystonia is observed in various neurological disorders, including cerebrovascular diseases, Parkinson's disease and Wilson's disease.
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PMID:[Dystonia]. 827 58

Athetosis is a peculiar involuntary movement resulting from pathologic involvement of the basal ganglia. Although mechanism of this movement is still far from established, athetosis is clinically differentiated from chorea and dystonia. The purpose of this article is to review and summarise the classification of this involuntary movement disorder. This movement disorder is classified into double athetosis, chorea-athetosis, unilateral athetosis and pseudo-athetosis. The double athetosis is featured by increased muscle tonus and irregular small amplitude movement, which appears the most frequently in patients of cerebral palsy. In chorea-athetosis, irregular abnormal movement is more prominent and larger than double athetosis. This type of movement appears commonly in patients other than cerebral palsy. Unilateral and pseudo-athetosis are derived not from disturbance of the basal ganglia but from impared sensory pathways of the deep sensation due to cerebro-vascular lesion. Stereotactic VL-thalamotomy is effective to relieve increased muscle tonus but not to decrease involuntary movement.
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PMID:[Athetosis]. 827 61

A variety of inheritable metabolic disorders produce movement disorders. A lists of conditions associated with tremor, athetosis, chorea, dystonia and myoclonus are presented as a guide for the differential diagnosis of such abnormal involuntary movements. The list includes aminoacidopathies, lipidoses, mucopolysaccharidoses, mucolipidoses, organic acidemias, mitochondrial cytopathies and disorders of carbohydrate, purine, and metal metabolism. Clinical, pathological and biochemical features of movement disorders of three typical examples, Wilson's disease, Lesch-Nyhan syndrome and glutaric acidemia type 1, are described.
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PMID:[Movement disorders in miscellaneous disorders--inherited metabolic diseases]. 827 72

From 1983 to 1991, 13 patients were identified with a clinical radiologic association characterized by acute or persistent neurologic dysfunction and bilateral lesions in the basal ganglia region demonstrated by ultrasound, computed tomography, or magnetic resonance imaging. Initial clinical manifestations of this group of patients were characterized by extrapyramidal signs (i.e., dystonia 9, hypotonia 2, athetosis 1, rigidity 1), altered state of consciousness in 5, and seizures in 3. The outcomes of most of these patients were poor: 10 had motor sequelae, 9 cognitive impairment, and 4 died. The outcomes of 2 patients, however, were much better than what was expected from the initial presentation. Based on current and previous reports, the diagnostic approach and classification of patients with neurologic dysfunction and bilateral striatal lesions are presented.
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PMID:Bilateral striatal lesions in childhood. 829 9

In normal subjects the execution of single rapid one-joint movements is characterized by an electromyographic (EMG) pattern composed of three discrete bursts of activity; two bursts (first and second agonist bursts, or AG1 and AG2) are present in the agonist muscle separated by an almost complete period of electrical silence. During this pause, another burst (antagonist burst, or ANT) occurs in the antagonist muscle. If a rapid movement is executed during tonic activation of the agonist muscle, tonic activity is inhibited just prior to AG1 onset (agonist inhibition). Similarly, if the movement is performed during tonic activation of the antagonist muscle, such activity is also inhibited prior to AG1 onset (antagonist inhibition). Antagonist inhibition also starts prior to AG1 onset and lasts until ANT onset. A general descriptor of the kinematic features related to the EMG pattern described above is a symmetrical and unimodal velocity profile that is bell-shaped and shows an acceleration time roughly equal to the deceleration time. This holds true for movements performed under low accuracy constraints; as accuracy demands become stricter and stricter, the peak velocity decreases but, as long as the movement is made with one continuous trajectory, the velocity profile remains roughly symmetrical. In general terms, the function of AG1 is to provide the impulsive force to start the movement; the function of ANT is to halt the movement at the desired end-point; and the function of AG2 is to dampen out the oscillations which might occur at the end of the movement. The timing and size of the bursts vary according to the speed and amplitude of the movement. The origin of the EMG pattern is a central programme, but afferent inputs can modulate the voluntary activity. In this paper, we also review the EMG and kinematic abnormalities that are present during the execution of single-joint, rapid arm movements in patients with Parkinson's disease, Huntington's disease, Sydenham's chorea, dystonia, athetosis, cerebellar deficits, upper motor neuron syndrome, essential tremor and large-fibre sensory neuropathy. The data from these studies lead us to the following conclusions: (i) the basal ganglia have a role in scaling the size of AG1, reinforcing the voluntary command and inhibiting inappropriate EMG activity; (ii) the cerebellum has a role in timing the voluntary bursts and probably in implementing muscle force phasically; (iii) the corticospinal tract has a role in determining spatial and temporal recruitment of motor units; (iv) proprioceptive feedback is not necessary to produce the triphasic pattern but it contributes to the accuracy of both the trajectory and the end-point of rapid movements.
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PMID:Single-joint rapid arm movements in normal subjects and in patients with motor disorders. 880 Sep 55

Athetosis and dystonia are well known clinical signs, described in disorders of basal ganglia. As opposed to pseudoathetosis, true athetosis was hitherto not reported in cord lesions. We here report three patients with athetosis and dystonia of hands due to intramedullary lesions of cervical cord: two patients with syringomyelia and one with glioma. Even though pseudoathetosis can be produced by lesions of posterior columns and likely to be confused with the involuntary movements of our patients, they had clinical and EMG findings consistent with true athetosis. A possible explanation for the athetosis and dystonia due to cord lesion is being postulated.
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PMID:Athetosis-dystonia in intramedullary lesions of spinal cord. 884 36

We describe the first Danish family with dentatorubral-pallidoluysian atrophy (DRPLA), containing 16 clinically affected individuals in five generations. Inheritance is autosomal dominant. The disorder was diagnosed as Huntington's disease (HD), but analysis of the IT15 gene for HD revealed normal alleles. The diagnosis of DRPLA was based on the finding of elongated CAG repeats in the B37 gene on chromosome 12 in affected individuals. The age at onset ranged from 13 to 60 years, with the most severe clinical picture being associated with onset in childhood. Clinical features included varying combinations of dementia, euphoria, visuomotor disturbances, speech problems, ataxia, tremor, epilepsy and involuntary movements presenting as chorea, athetosis, and dystonia. We discuss characteristics of DRPLA that may enable the differentiation from HD on a clinical basis. In conclusion, DRPLA should be considered and DNA analysis is recommended in patients manifesting varying combinations of extrapyramidal and cerebellar symptoms, especially when clinical features show pronounced intrafamilial variability, and dyscoordination, tremor, myoclonus, epilepsy, and euphoria are part of the syndrome.
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PMID:Dentatorubral-pallidoluysian atrophy. Clinical features of a five-generation Danish family. 886 94


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