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

Achalasia is a neuromuscular functional disturbance with dystonia and dyscinesia of the entire tubular oesophagus of unknown aetiology characterised by loss of cardial opening reflex. In the usual functional state dysphagia and moderate stenosis are observed, but occasionally an organic form with marked mega-dolicho-oesophagus will develop. Therapeutically an attempt is made to reduce the hypertonus of the terminal oesophagus. In mild or moderate cases this may be achieved by dilatation alone. If conservative treatment fails or in severe forms an operative correction is mandatory, we advise preoperative dilatation together with an extramucous myotomy of the distal anterior oesophageal wall and a fundoplication to prevent the gastro-oesophageal reflux. This abdominal procedure demands great care but is not traumatising and ensures satisfactory early and late results even in patients with considerable surgical risks. Operative indication and technique in 63 cases are demonstrated.
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PMID:[Achalasia of the oesophagus. Operative treatment by abdominal myotomy and fundoplication (author's transl)]. 71 70

Using previously obtained data concerning the effectivity of amedin in motor and speech disorders due to muscular dystonia the drug was used for the treatment of 50 children from 3.5 months to 18 years, with motor disorders of an extrapyramidal and labyrinth nature. A high therapeutical effectivity of amedin in an increased pathological activity of tonic cervical and labyrinth reflexes in infants and in torsion spasm of school age children is demonstrated.
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PMID:[Experience with using amedin in children with movement disorders of extrapyramidal and labyrinth origin]. 71 25

Central hemodynamics was studied by means of echocardiography in 7 patients suffering from neurocirculatory dystonia with a high cardiac index but normal arterial pressure, and in 41 patients with essential hypertension prior to, and after treatment. Two groups of patients suffering from essential hypertension were distinguished: with a hyperkinetic type of circulation and with a normal cardiac index. In the first group there was a statistically significant increase in the stroke index and the index of diastolic left ventricular diameter with a normal ejection fraction. The increase in the cardiac index in the hyperkinetic type of essential hypertension is caused by an increase in the stroke output according to the Frank--Starling law. The authors believe that the increase in peripheral resistance is the principal pathogenetic mechanism of hypertension with any level of the minute volume.
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PMID:[Hemodynamic types of hypertensive disease according to echocardiographic data]. 72 44

The state of the psyche was studied in 128 patients with cardiac-type neurocirculator dystonia. The results of clinical examination and the Minnesota test (MMT) showed that with the increase of the subjective manifestations of the cardiac pain syndrome and extension of the zones of pain sense disorders in the left half of the chest, a clear tendency to an increase of the neurotic changes was revealed, which were found, respectively, in 61.9, 89,7 and 100% of patients examined with grade I, grade II and grade III cardialgia. The psychic disorders were predominantly of the hysterical and hypochondriac types, which should be borne in mind when prescribing treatment.
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PMID:[Personality characteristics of patients with the cardiac type of neurocirculatory dystonia]. 73 86

Our objective has been to trace Joseph's disease to its geographic origins and to determine the spectrum of clinical manifestations. This goal we have achieved by documenting type I and II disease within the Joseph and Sousa families. The major neuropathologic findings are a progressive neuronal loss involving the striatum, nigra, dentate nucleus of the cerebellum, and lower motor neurons in the brainstem and spinal cord. The homozygote form of the disease produces type I disease with onset in early childhood of progressive dystonia, athetosis, and spasticity. Type I disease tends to have its onset by age 25 years in heterozygotes and lasts about 15 years on the average. Type II disease, which we consider the result of a single dose of the mutant gene, usually begins somewhat later and runs its course over a 20-year period. Type III disease documented in the Thomas family is the most benign. Its onset is often in the fifth decade, and it progresses slowly into the eighth decade. Patients may benefit from antiparkinson medication including dihydroxyphenylalanine and anticholinergic agents (e.g., amantadine). A molecular marker for the disease is being sought actively, and several interesting patterns have already been documented by means of patient fibroblast cultures and two-dimensional acrylamide gel protein separations. The mutant gene is clearly outside the HLA complex but may be linked to it. The only biochemical change noted thus far is a reduced CSF level of HVA that probably reflects the loss of dopamine-synthesizing neurons in the substantia nigra and is thus a secondary effect of disease. Although the disease is a very old one which we can trace back to the early 19th century on the island of Flores, it may be recurring de novo by new gene mutations at an unstable gene locus in a genetically vulnerable population. Now that the spectrum of clinical expression has been identified and the mode of inheritance established as an autosomal dominant wherever the disease has been found, it is believed that its true incidence will become more evident by virtue of better detection and that the true incidence will actually increase because of increased assimilation of affected persons into other ethnic groups.
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PMID:Joseph's disease: an autosomal dominant neurological disease in the Portuguese of the United States and the Azores Islands. 73 30

The therapeutic effectiveness of sulpiride on various types of neurosis was compared with that of chlordiazepoxide on a double-blind basis. Global improvement, effectiveness on various types of neurosis, and side effects were studied for a period of two weeks. The subjects consisted of 41 males and 32 females. Neurosis including borderline case and vegetative dystonia was divided into eight different subtypes comprising borderline, neurasthenic state, hypochondria, obsessive neurosis or phobia, depressive neurosis, anxiety neurosis, vegetative dystonia, and others. A newly devised matched-pair method of comparison was employed to achieve even distribution of the eight subtypes of neurosis between the two drug groups. As a result 40 patients fell in the sulpiride group and 33 patients in the chlordiazepoxide group. The backgrounds of the paired patients matched closely. The daily dose was uniformly 150 mg for sulpiride and 30 mg for chlordiazepoxide in the first week but was raised (or lowered in some cases) to 225 mg and 45 mg, respectively, in the second week according to severity. The rate of global improvement was 79% for the sulpiride group and 90% for chlordiazepoxide group, and the difference did not reach statistical significance. Improvement by manifestation (13 symptom items) and type of neurosis also matched. Side effects occurred at a rate of 28% (sulpiride group) and 30% (chlordiazepoxide group), and also matched closely in incidence and variety. The authors concluded that sulpiride in appropriate doses is useful in the treatment of neurosis without causing extrapyramidal side effects.
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PMID:A double-blind comparison of sulpiride with chlordiazepoxide in neurosis. 78 20

The ST length and T wave were investigated electrocardiographically and clinically in 200 patients with a short PQ interval in the electrocardiogram, independent of the length and shape of the QRS complex. In group A (Lown-Ganong-Syndrome LGL), we often found a narrow, pointed, positive P wave and a narrow QRS complex in the electrocardiogram in addition to the short PQ interval, and also a tendency to respiratory arrhythmia. Clinically this was frequently a matter of young women with a tendency to paroxysmal tachycardia and autonomic dystonia. A James bundle or even a short AV conduction pathway with a small heart might be considered as the origin of this LGL syndrome. A particular classification of patients with short PQ intervals and S-T deformation (Group B) in organic heart disease has not yet been described in the literature to our knowledge. The results of our investigations in group C (WPW syndrome) correspond in the main to those given in the literature.
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PMID:[Short PQ interval with particular reference to the Lown-Ganong-Levine syndrome (author's transl)]. 80 14

In this chapter, we have attempted to demonstrate that chronic manganese intoxication, in both animals and man, is a better model of dystonia than of Parkinson's disease. It is proposed that many of the monoamine and endocrine changes in dystonia may be the result of disturbances in manganese metabolism. A search for such modifications in human dystonia victims is presently underway. Preliminary studies indicate that head and pubic hair manganese concentrations are elevated in dystonia patients.
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PMID:Role of manganese in dystonia. 82 21

In three cebus monkeys the chronic daily administration of haloperidol (0.5 mg/kg/day orally) created sedation and parkinsonism during the first 5-7 weeks. Later the animals developed signs reminiscent of acute dystonia, as seen in the clinic during treatment with neuroleptics. These signs were dose-dependent and in extreme cases included widespread tonic and clonic seizures. After 3 and 12 months, respectively, two of the cebus monkeys developed buccolingual signs (grimacing and tongue protrusion), similar to tardive dyskinesia in the clinic. The tardive dyskinesia symptoms were reduced in a dose-dependent manner after each haloperidol administration, being most pronounced in the morning before haloperidol was given. Biperiden reduced acute dystonia but reinstated signs of tardive dyskinesia, which had been abolished by haloperidol. It is suggested that cebus monkeys may provide a useful animal model for the study of neurologic long-term complications from neuroleptic drugs.
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PMID:Haloperidol-induced tardive dyskinesia in monkeys. 82 68

Motor disorders reported in the present paper do not result from cortical ablations stricto sensu since some white matter was excised in every patient. However they appear to suggest that, as suggested by Walshe (1935), the central region and premotor area are a functional entity, i.e. they work as a whole. The extensive lesions of the premotor area, leaving untouched the motor region, have the same motor and tonic consequences as lesions limited to the central region. This point which appears specific for man does not imply that the premotor region subserves activities similar to those subserved by the central region. Rather it may suggest a deafferentiation of the central region, the consequences of which would be more important than is generally assumed. Extensive central or premotor lesions determine various tonic disorders: a well known spasticity, with exaggeration of the stretch reflex, associated with an increase in passive swinging of segments of limbs and in extensibility of joints. These two latter phenomena are usually defined as hypotonia. With premotor and precentral lesions the hypotonia disappears and a hemiplegic posture is observed. This hemiplegic posture is a dystonia which apparently does not result directly from the exaggeration of the stretch reflex. Anatomically it appears to result from lesions of both central and premotor regions. This is in agreement with Denny-Brown's (1966) contention that an extrapyramidal region lies rostral to the prerolandic sulcus. As suggested by Evarts (1973) motor regions appear to control automatic as well as voluntary movements. They probably play a role in the trophic function of muscle, since, despite rehabilitation, amyotrophy was present in every case reported in the present paper.
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PMID:Motor consequences of motor area ablations in man. 83 9


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