[Vascular purpura and Guillain--Barre syndrome in patients with severe Wilson--Konovalov disease].
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Biomedical subjects
Publications and source records attributed to Iu V Mozolevskiĭ.
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A two-central randomised single-blind placebo-controlled study was conducted to assess efficacy of intravenous administration of trometamol salt of thioctic (alpha-lipoic) acid (Thioctacid 600, "ASTA Medica", Germany) in 200 ml of physiological solution in 40 non-insulin dependent diabetic patients with symptomatic diabetic neuropathy. 10 patients of the control group received a physiological solution stained with 1% solution of riboflavin mononucleate (B2 vitamin) as placebo. Intravenous infusion was administered once daily during a period of 3 weeks. Diabetic neuropathy score was assessed at the entry as well as on days 7, 14 and 21 of therapy. Indicators of lipid peroxidation (oxidative stress)--malonic dialdehyde levels in plasma and membranes of erythrocytes. Electroneuromyography were performed. Most of the patients (29) noted symptomatic improvement after 7-10 infusions and on day 14 a significant reduction of the diabetic neuropathy score was noted in comparison to a baseline (p < 0.05). The further improvement of clinical picture had been pronounced by day 21 (p < 0.001). Improvement of symptom score was obtained in 39 patients (97.5%). The response rate in the control group was 40%. Parameters of oxidative stress and electroneuromyography improved significantly in the main group of patients, while these in placebo group remained without changes.
Upon the analysis of literature data and original experience, the authors suggest that painful ophthalmoplegia (PO) is rather a syndrome than a disease. The most prevalent PO syndromes are characterized clinically. PO patients need a purposeful examination to identify the underlying disease. It is the latter that should be treated first of all. Early administration of corticosteroids in PO without careful examination of patients is not justified. The scheme of such examination is presented.
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Motor, sensitive and vegetative disorders were subjected to a clinical analysis in 40 adult patients with Landry-Guillain-Barre syndrome in the acute disease stage and during rehabilitation. Investigation of the cerebrospinal fluid and of the rate of the nervous impulses conduction in the peripheral nerves has demonstrated that in the majority of the patients, the clinical findings did not correlate with the laboratory ones. Early diagnosis and rational therapy with plasmapheresis and glucocorticoids ensured a favourable prognosis.
A study was made of the clinical and electrophysiological data on 33 patients. In all the patients, motor disorders played the leading part. 18 patients manifested distal tetraparesis, 7 diffuse, 3 tetraplegia, and 5 had multiple mononeuropathy. The progressive, recurrent and monophasic types of the disease course were distinguished. The electrophysiological study revealed pathology primarily in muscles of the distal parts of the limbs and local areas of the blocking of excitation conduction in different nerves. The prognosis and treatment of the patients with glucocorticoids, cytostatics and plasmapheresis are under discussion. The catamnesis is 5 years.
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Two cases of the X-chromosome-linked adult bulbospinal neuronopathy are reported. Patients displayed major signs of the disease: the onset at adult age, slowly progressing involvement of limb, facial and tongue muscles, light bulbar disorders, generalized fasciculation and neuronal signs in EMG investigation. Observed were also bilateral gynecomastia, fascicular tremor. Clinical differences in the 2 patients could be accounted for by different expression of the pathological gene.
Clinico-electromyographic characterization of 20 patients with different forms of the spinal muscular atrophy (bulbospinal in 8 patients, chronic proximal in 5, distal in 7) is reported. The patients displayed the characteristic clinical signs and neuronal changes in EMG. The latter data are specified for each form. The authors infer that the spinal muscular atrophy in adults, children and adolescents fall into one group, though differing in the onset age, type of inheritance, clinical features, severity and prognosis.
The authors analyzed 32 cases of verified craniospinal tumors (CST) which were observed at a neurosurgical department over the last decade. These tumors were subdivided into neurinomas (n = 14), meningiomas (n = 11) and gliomas (n = 7). All patients underwent surgery. The pathogenesis of symptoms observed in CST is discussed. Clinical symptoms of importance for the early diagnosis of craniospinal tumors are outlined. When CST is suspected the recommended procedures include analysis of the CSF, X-ray examination of the cervical portion of the vertebral column, ascending positive myelography and computer-aided tomography of the cranium and vertebral column.
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Two clinical cases of post-poliomyelitic progressive amyotrophy (PPA) are described. One patient had serological tests of the blood and cerebrospinal fluid for viruses of poliomyelitis, measles, simple herpes and tick-borne encephalitis which were negative. The authors suggest that PPA develops in patients with a peculiar genetic predisposition and altered homeostasis and is a degenerative-dystrophic disease of the motor nerve.
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Two cases with expressed signs of a permanent muscular tension in the extremities with fasciculation (the Isaacs syndrome) and difficulties in self-service are described. In both cases the diagnosis was verified by typical EEG changes and a good effect from the use of a diphenylhydantoin or finlepsin.
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