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)

Fluctuations in performance in levodopa-treated Parkinsonians pose frequent and difficult problems of managment. Controlled trials with two recently introduced drugs, bromocriptine and L-deprenyl, have been performed in an attempt to clarify their use in Parkinsonian oscillations. Bromocriptine, partially substituted for levodopa, was helpful in 4 of 6 patients with early morning dystonia, but did not benefit 9 patients with end-of-dose deterioration and 5 patients with "on-off changes. L-Deprenyl 10mg daily gave substantial benefit to 19 of 39 patients with end-of-dose deterioration, but to only 1 of 10 patients with "on-off' phenomena. Neither L-deprenyl nor bromocriptine helped patients disabled by freezing episodes or levodopa-induced involuntary movements.
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PMID:Oscillations in performance in levodopa-treated parkinsonians: treatment with bromocriptine and L-deprenyl. 12 6

An unusual neurovisceral lipid storage disorder in two unrelated juvenile patients manifested itself by dystonia and involuntary movements, with facial grimacing, dysarthria, gait difficulty, and impaired manual dexterity. Supranuclear paresis of vertical gaze and splenomegaly were present. Absent were seizures, major intellectual deterioration, spasticity, or blindness. Histiocytes showed lysosomal storage of various phospholipids, cholesterol, neutral lipids, and autofluorescent material. Appendiceal neurons showed only an increse of phospholipids by histochemistry. Neuronal deposits differed ultrastructurally from these in histiocytes. Leukocyte sphingomyelinase activity was normal. The nosology of this disease and its relationship to so-called juvenile types of Niemann-Pick disease is discussed. The primary metabolic defect in these patients remains unknown.
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PMID:Juvenile dystonic lipidosis: an unusual form of neurovisceral storage disease. 18 51

A case of involuntary plantar flexion of the toes and sole with no other neurological pathological sign is reported. The electromyographic activity of some muscles of the involved limb. the behavior of the phenomenon during the sleep-wakefulness cycle and spinal reflex excitability have been studied. According to the clinical and neurophysiological findings, the phenomenon appears to be a dystonia. Such a dystonia may occur as the only pathological neurological sign or together with the other signs of central nervous system involvement. Particular care has to be taken to distinguish 'tonic foot' from hypertonus of sole flexor muscles occurring almost always in all spastic hemipareses.
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PMID:Tonic foot: clinical and neurophysiological study of a case. 22 49

Scintigraphy of the myocardium with pyrophosphate-99mTc was used in the examination of 45 patients with chronic ischemic heart disease and 3 patients with vegetovascular dystonia. A direct dependence of the character of the scintigram on the anginous attack was revealed. Accumulation of pyrophosphate-99mTc in the myocardium was observed in all patients with the agent injected just before the attack, during the attack or in the first 8 hours after it. Pyrophosphate-99mTc accumulation in the myocardium demonstrated on the scintigrams of the myocardium was less intense but more diffuse and extensive in chronic ischemic heart disease than in the focus of acute myocardial infarction.
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PMID:[Myocardial scintigraphy with pyrophosphate-99mTc in transitory myocardial ischemia in chronic ischemic heart disease]. 22 67

Scintigraphy with pyrophosphate 99mTc was performed in 230 patients with various forms of ischemic heart disease and in 15 persons with pain in the region of the heart caused by osteochondrosis of the cervical and thoracic spinal segments or vegetovascular dystonia (control group). It was found that labelled purophosphate accumulated in the myocardium in necrosis of the heart muscle or when coronary insufficiency takes a course in which necrosis of the myocardial cells is quite possible. Positive results of scintigraphy with pyrophosphate 99mTc are not a strict criterion of acute myocardial infarction because they are also encountered in a chronic course of ischemic heart disease and are evidence in this case that "a state of risk" has occurred during the disease.
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PMID:[Importance of pyrophosphate-99mTc scintigraphy in the diagnosis of acute myocardial infarct]. 22

Pheylalanine metabolism was studied in 56 children with various forms of hyperkinesias. It was found that in the development of slow and fast hyperkinesias a certain role belongs to dihydroxyphenylalanine (DOPA). It is probable, that in patients with Turett's syndrome the synthesis of DOPA is increased while in patients the excretion of phenylacetylglutamine was found to be disturbed: it was decreased in the patients with the fast and increased in the children with the slow hyperkinesias. Phenylalanine load led to a lowering of the DOPA level in the patients with Turett's syndrome; an intensification of the synthesis of phenylacetylglutamine and diminution of the intensity of hyperkinesias. L-glutamine load resulted in detoxication of the toxic phenylalanine metabolites which inhibited the DOPA synthesis, as well as in a short-time increase in the phenylacetylglutamine excretion and a moderation of tonic hyperkinesias that manifested by athetosis and dystonia. All this points out that in the development of various forms of hyperkinesias a certain role belongs to amino acid metabolites.
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PMID:[Pathogenesis of hyperkinesis in children]. 29 Dec 44

Seventy-four cases of tic syndromes were classified into four groups: chronic multiple tics, subacute multiple tics, chronic simple tics and transient simple tics, and 37 cases of chronic multiple tics (Tourette syndrome) were investigated. Clinical evaluation suggested that a transition existed between the four groups. Posture abnormalities were found in 27% of Tourette syndrome and a relation to dystonia was implied. Clinical evaluation and studies of catecholamine blockers' effectiveness suggested the validity of subtyping Tourette syndrome into four groups whose topographical or biochemical abnormalities differ. It was argued that the neurochemical basis of Tourette syndrome might lie in a multiplicity of biochemical abnormalities including disturbances of dopaminergic and noradrenergic pathways.
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PMID:Gilles de la Tourette syndrome in Oriental children. 29 28

This review covers recent advances in a variety of dyskinesias. Introduction of new drugs for the treatment of myoclonus and sensory biofeedback therapy for focal dystonia are expanding our concepts of these types of movement disorders. Progress in the treatment of action myoclonus is especially noteworthy and has led to the implication of serotonin deficit in the pathophysiology of this syndrome. Knowledge of the biochemical pathology of Huntington's chorea has outpaced therapy for this disorder, but new forms of therapy have been proposed based on the chemical findings. Basic pharmacologic studies suggest pathophysiologic mechanisms for the syndrome known as tardive dyskinesia, but treatment is still far from ideal for this disorder. Other movement disorders with recent therapeutic advances include essential tremor and hemiballism. This review will cover only those dyskinesias in which new therapies have been advanced in the last few years. Aside from parkinsonism, which will not be discussed here, progress in the treatment of movement disorders has been slow, but steady. New drugs are being tested constantly, and the purpose of this review is to call attention to the ongoing evaluation in this field. Descriptions and etiologies for these dyskinesias are covered elsewhere (Fahn, 1976a) and therefore are not repeated here.
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PMID:New approaches in the management of hyperkinetic movement disorders. 30 60

Haloperidol treatment for stuttering was examined in a double-blind cross-over study of 26 adult volunteers with long-standing stuttering. Most had had unsuccessful speech and drug therapy. During the three-month study, their speech and stuttering patterns were repeatedly evaluated from videotaped readings of a standard passage and during spontaneous speaking. Of the 18 patients completing the trial 11 were significantly more improved on haloperidol than on placebo, three benefited equally from both, and four were unchanged. Improvement over placebo was indicated by fewer dysfluencies, increased speed of speaking, and reduced secondary "struggle" phenomena. Side effects were common on a dosage of 3 mg haloperidol daily. Poor concentration, akathisia and dystonic movements caused 8 patients to discontinue the trial despite significant improvement in 5 of them. Although "statistically significant" improvement occurred in most patients on haloperidol, the "clinical significance" of this form of therapy will be limited by the partial response, the need for continuous medication, the side effects of haloperidol and the attitude of stutters to this type of therapy.
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PMID:Haloperiodl in the treatment of stuttering. 32 82

The cerebral pareses occurring in early childhood or produced subsequently by trauma or spontaneous cerebral haemorrhages, present a heterogenous symptomatic picture. In the forefront we find the spastic pareses associated with extra-pyramidal motor hyperkinesia (athetosis, dystonia, ballism). 36 C.P.-patients were surveyed from 1--4 years after operative intervention. It is shown that better results can be obtained when multilocular lesions during a single session in correspondence with the dominant group of symptoms are performed (thalamic and subthalamic target points of the extrapyramidal motor nuclei and dentate nucleus). The results are presented. Continuous physical therapy after the operation and a reasonable intellectual level are essential for improvement.
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PMID:Multilocular lesions in the therapy of cerebral palsy. 33


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