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Results for “Dystonia Musculorum Deformans”

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At least 19 recordsLinked to original sources
PubMed1979

MMPI characteristics associated with cerebral palsy and dystonia musculorum deformans.

The origins of dystonia musculorum deformans are now considered to be organic. However, misdiagnosis of dystonia as a functional psychiatric disorder--usually conversion reaction--has persisted. The present study describes personality traits as measured by the Minnesota Multiphasic Personality Inventory in 30 persons with dystonia and in a control group of 37 persons with cerebral palsy. The data, examined by diagnosis, level of disability, and sex, showed no differences for diagnostic groups or levels of disability. Males scored in the direction of greater psychopathology than did females. The male dystonics showed the highest elevations of MMPI scales of all the groups. Although only one person with dystonia musculorum deformans and none with cerebral palsy produced the profile usually associated with conversion reaction, 36% of all profiles showed two scales above a T score of 70. This finding suggested that young adults with a physically disabling disease may be at higher risk for developing maladaptive personality traits.

Adult↗
PubMed1979

Polygraphic analysis of sleep in dystonia musculorum deformans.

The functional state of non-specific brain systems was studied in 27 patients with torsion dystonia through a complex of techniques, which included clinical, experimental psychological and electrophysiological examination of night sleep. Changes of involved nature have been identified in the various elements of non-specific systems in different clinical forms of the disease in its early and advanced stages, which allows to consider torsion dystonia as a functional organic psychomotor syndrome in the origination of which a great role belongs to non-specific integrative systems of the brain.

Adolescent↗
PubMed1979

[Treatment and prognosis of the therapeutic effect of L-DOPA in dystonia musculorum deformans].

Clinico-biochemical examinations of 55 patients suffering from various clinical forms of deforming myodystonia were performed. L-DOPA was used in 47 cases. A correlation between the therapeutic effect and the level of phenylacetylglutamine excretion was revealed. The increase of the phenylacetylglutamine excretion was an evidence of a positive result of the treatment that was noted in 36 patients (77%). A resistance to the drug was observed in cases when the phenylacetylglutamine excretion did not exceed its normal level. The quantitative determination of phenylacetylglutamine in the daily portion of the urine can be used as a prognostic test of the therapeutic efficacy of L-DOPA in patients with deforming myodystonia.

Adolescent↗
PubMed1978

[Functional state of non-specific brain systems in dystonia musculorum deformans].

The paper deals with the results of an electropolygraphical study of night-sleep structure, as well as a clinical and experimental-psychological study of 27 patients with deforming muscular dystony. The quantitative and qualitative characteristics of functional shifts in different links of the nonspecific system in the known clinical forms and at different stages of the disease are given. The data were obtained with the aid of correlational statistical methods and were used for a clinico-physiological analysis of the syndrome of deforming muscular dystony. It is demonstrated that changes of a functional state of the nonspecific brain systems correlate with motor, emotional-personal vegetative and electroencephalographical manifestations of deforming muscular dystony.

Adolescent↗
PubMed1977

Familial spasmodic torticollis.

Presented are four cases of familial spasmodic torticollis, comprising members of three families. The age of onset tended to be family-specific, and no afflicted family member had evidence of more widespread disease (dystonia musculorum deformans). Familial incidence supports the conclusion that spasmodic torticollis is organic in origin. Familial spasmodic torticollis occurs more often than has been generally recognized.

Adult↗
PubMed1978

Treatment of spasmodic torticollis by dorsal column stimulation.

A new method is described to treat spasmodic torticollis with the implantation of a dorsal column stimulator at the C1--2 level or with transcutaneous stimulation. 22 patients were evaluated. 3 had sufficient relief to be treated with transcutaneous stimulation only. An additional 6 patients had surgically implanted dorsal column stimulators. It was empirically determined that a frequency of 800--1,100 Hz gave the best relief from torticollis. 1 patient had an excellent result; 3 have had good results; 1 had a fair result, and 1 had a poor result. An additional patient with dystonia musculorum deformans was considerably improved by the use of dorsal column stimulation.

Adult↗
PubMed1978

Experimental analysis of EMG feedback in treating dystonia.

Recent clinical reports have suggested that electromyographic feedback offers promise as a palliative therapy for neuromuscular disorders. This study utilized a single-subject experimental analysis to evaluate the effects of EMG feedback training on a 20-year-old man with adult-onset idiopathic dystonia to achieve control of orofacial dysfunctions. Feedback training produced reductions in levels and variability of tension in facial and forearm muscles, as measured by EMG. Reliable blind observations of facial control indicated that feedback training enabled the patient to produce normal facial relaxation in extraclinical environments.

Adult↗
PubMed1975

[Long-term treatment with L-DOPA in a case of torsion dystonia (authors transl)].

A 36-year old woman had been able to move from place to place only by crawling until 1971 after she had been treated with L-DOPA, the dose of which was increased to a maximum of 2.5 g daily. Within 4 weeks there was a diminution of her increased muscle tone and she experienced a dramatic improvement in her daily living activities, being able to walk without assistance for the first time in 20 years. This improvement has continued with a daily maintenance dose of 1.25 g. For patients with torsion dystonia, where the so-called myostatic component preponderates, cautious application of L-DOPA seems to be justified and further clinical studies are suggested.

Adult↗
PubMed1977

Neurosurgical treatment of the dyskinesias.

I have attempted to review what I consider to be the principal conclusions of a 25 year study of the neurosurgical treatment of the dyskinesias. The concept that the pathological disinhibition which is responsible for the production of these syndromes can be modified by destructive lesions within the thalamus or by prosthetic mobilization of inhibitory mechanisms of the cerebellum is supported by the results presented in this report. Neurosurgical treatment of the dyskinesias is potentially a relatively safe and efficacious means of alleviating incapacitation of a large group of patients. However, meticulous attention to patient selection as well as adherence to the general principles of surgical technique which have been reviewed are essential if the full potential of these approaches is to be realized.

Adult↗
PubMed1978

Somatostatin in the treatment of patients with extra-pyramidal disorders and patients with EEG abnormalities.

The growth hormone release inhibiting hormone acts probably both as a true hypophysiotropic hormone and as a neuroregulatory substance. Neurophysiological studies have shown that somatostatin is a CNS depressant. Somatostatin might therefore be of therapeutic significance in neurological diseases. Sixteen patients with different extrapyramidal disorders and seven patients with various EEG abnormalities were tested with 2-hour somatostatin infusions and control infusions with saline. Somatostatin did not induce any improvement or deterioration of symptoms, signs, or EEG abnormalities in any patient.

Adult↗