Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008489 (chorea)
 2,102 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This review considers the evidence for possible involvement of central nervous system pacemaker neurons in several clinical disorders of movement. Two basic types of tremor are discussed from this point of view, i.e., 4--7/sec parkinsonian tremor, of possible thalamocortical origin, and 7--11/sec essential tremor of possible olivo-cerebellar origin. The importance of motor programs and abnormalities in their utilization are considered with reference to the loss of motor function in parkinsonism (? loss of motor programs), and the inappropriate release of such programs as a possible basis for the involuntary movements seen in other movement disorders, such as chorea, athetosis, dystonia, and hemiballismus. The possible role of pacemaker neurons controlling such programs is considered. Finally, the subject of locomotion and the pacemaker model of the spinal locomotor pattern generator for stepping are considered in relation to clinical disorders of gait. While critical evidence is lacking for pacemaker inovlvement in any of these disorders, their possible role is emphasized.
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PMID:Possible involvement of central pacemakers in clinical disorders of movement. 35 Jun 32

To identify those patients most likely to benefit from a cerebral biopsy to diagnose dementia, we reviewed a series of 14 unselected biopsies performed during a 9-year period (1980 through 1989) at Duke University Medical Center, Durham, NC. Pathognomonic features allowed a definitive diagnosis in seven specimens. Nondiagnostic abnormalities but not diagnostic neuropathologic changes were seen in five additional specimens, and two specimens were normal. Creutzfeldt-Jakob disease was the most frequent diagnosis. One patient each was diagnosed as having Alzheimer's disease, diffuse Lewy body disease, adult-onset Niemann-Pick disease, and anaplastic astrocytoma. We conclude that a substantial proportion of patients presenting clinically with atypical dementia are likely to receive a definitive diagnosis from a cerebral biopsy. However, in those with coexisting hemiparesis, chorea, athetosis, or lower motor neuron signs, cerebral biopsies are less likely to be diagnostic.
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PMID:Evaluation of cerebral biopsies for the diagnosis of dementia. 172 59

Drug-induced and tardive movement disorders represent a large number of extrapyramidal disorders seen in neurologic practice. Iatrogenically induced, most commonly by neuroleptics, these disorders can be characterized by any abnormal body movement including tremor, chorea, athetosis, dyskinesias, dystonia, myoclonus, tics, ballismus or akathisia. Parkinsonism, dyskinesias and dystonia tend to be the most common. Management of patients with drug-induced or tardive syndromes is complex. Prognosis is frequently poor as patients usually need the offending agent to manage their underlying psychiatric or medical problem. Neuroleptics and other drugs known commonly to cause movement disorders should be used cautiously and significant consideration of all risks and benefits measured before initiating therapy.
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PMID:Drug-induced and tardive movement disorders. 183 84

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

Between 1986 and 1988 a door-to-door survey was conducted on a stable rural population of 60,820 in central Ethiopia. Trained lay health workers made a complete census and identified cases with symptoms and signs of neurological disorders, using specially designed questionnaires which, in a previous pilot study, were found to have a sensitivity of 91% and specificity of 85%. Neurological disorders in the rural population were epilepsy, postpoliomyelitis paralysis, mental retardation, peripheral neuropathy (mainly due to leprosy), and deaf-mutism with prevalence rates (cases/100,000 population) of 520, 240, 170, 150 and 130, respectively. The prevalence rates of the other less common neurological disorders were 62 for hemiparesis (15 of which were for cerebrovascular accidents), 20 for cerebral palsy, 16 for optic atrophy, 12 for perceptive deafness, 10 for tropical spastic paraparesis, 7 for Parkinson's disease and 5 for motor neuron disease, ataxia and chorea/athetosis. Among related non-neurological conditions, blindness, locomotor disability and deafness were predominant. The significance and role of such a neuroepidemiological study in laying the strategies for the prevention of neurological disorders and rehabilitation of patients are discussed in the context of a developing country.
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PMID:Community-based study of neurological disorders in rural central Ethiopia. 208 51

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

Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder characterized by combined systemic degeneration of the dentatofugal and pallidofugal pathways. The patients show a variable combination of symptoms including cerebellar ataxia, involuntary movements like chorea and athetosis, myoclonic epilepsy and dementia. We found expansion of an unstable CAG repeat in the patients, and then determined the cDNA and genomic forms of the gene, as reported in Nature Genetics 6:14-18 and 8:177-182, 1994. In this review, we summarize our studies on repeat expansion of the DRPLA gene, and discuss the common and distinctive features among triplet repeat disorders.
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PMID:[Triplet repeat disorder, dentatorubral and pallidoluysian atrophy DRPLA)]. 775 62

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


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