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Twin pairs with one or two myasthenic patients were selected from patients with neuromuscular pathology. 18 couples of twins were included in final selection (9 monozygotic and 9 dizygotic pairs). 4 pairs of monozygotic twins were concordant to myasthenia (MA). All dizygotic pairs were discordant to this disease. Matched concordance of monozygotic twins was 44%. Penetration of pathological gene was 61% assuming the hypothesis about monogenic heredity of MA. The coefficient of heredity was 44%. The conclusion was made about important, but not absolute role of hereditary factors in development of MA. It was necessary the presence of combination of both genetic and environmental factors for MA development.

The distribution of ABO blood groups and rhesus factor was studied in patients with myasthenia as compared with the control. There was a statistically significant association of the diseases with the rhesus-negative phenotype and that of generalized myasthenia concurrent with thymoma with the B (III) blood group. The examination revealed no other determinants of the significant association with the disease. The values of a disease risk were obtained for persons having myasthenia-associated signs. It is concluded that the Rh-negative phenotype shows a 1.3-fold increase in the risk of the disease as compared with those having Rh-positive persons.

Thirty-year experience in surgical treatment of myasthenia in children and adolescents (175 patients) is generalized. It provides evidence of delayed recognition of this disease in many patients and of prolonged, often ineffective nonoperative treatment. Operation for thymectomy should be one of the main methods for the management of myasthenia in children. Operative treatment is indicated in a disease of moderate severity or in a severe course of myasthenia, particularly in patients with the first type of immunomorphological changes. It is important to amend the myasthenic disorders before the operation by means of maximally possible doses of anticholinesterases with addition of corticosteroids, whenever necessary. Whatever the method of treatment chosen, the patients must be kept under skilled dynamic control. Excellent and good late-term results were produced in 84.6% of cases.

From experience in surgical treatment of 1,800 patients with generalized myasthenia as well as from literature data, the authors analyse the main tactical and technical errors in the treatment of this category of patients. A complex approach and a program of improving the results of treatment of patients with myasthenia were elaborated. Compulsory examination of the mediastinum for revealing possible affection of the thymus is indicated in myasthenia. Median sternotomy with ++extra-fascial removal of the whole thymus is considered the optimal approach in thymectomy. Thymomthymectomy with extensive removal of the areolar tissue of the anterior mediastinum is believed to be the operation of ++choice in tumors of the thymus. Thymus tissue which is not removed during the operation is one of the causes of recurrent myasthenia, while unjustified extension of the volume of the operation in nonneoplastic affection of the thymus is also inadvisable. Preoperative radiotherapy is not recommended. The principles elaborated in the clinic enabled the authors to reduce the frequency of assisted ventilation sevenfold and the incidence of infectious complications, pneumonia among others, to 0.6%.

The authors discuss the results of splenectomy carried out on 20 patients with generalized myasthenia in whom previous complex treatment including thymectomy, corticosteroid and azathioprine therapy, repeated courses of plasmapheresis during medication with anticholinesterase agents, did not cause any essential improvement. Different values of homeostasis and immunological status were studied before and in different periods after splenectomy. The condition improved in 65% of patients, which was confirmed by the results of myography; the doses of corticosteroids and anticholinesterase agents were reduced. Bearing in mind the severe condition of patients selected for splenectomy, it is recommended to treat them only in medical centers possessing sufficient experience in treatment of myasthenia. The indications and contraindications for splenectomy in complex treatment of myasthenia must be specified further.

The authors describe the results of clinical and electromyography examinations of 20 patients with grave generalized myasthenia, carried out within the short-term (up to 2 weeks) and long-term period (up to 1 year) after splenectomy. In these patients, the preceding therapy (thymectomy, gamma-therapy of the thymic area, the treatment with prednisolone, azathioprine, plasmapheresis, etc) had not produced any beneficial effect. The efficacy of splenectomy was assessed according to the Keens classification (A-E). In the short-term period after splenectomy the C effect was recorded in 13 out of the 20 patients. Out of 9 patients examined in the long-term period, the B effect was revealed in 3 patients, the C effect persisted in 3 patients, and 3 patients manifested the return to the initial status, namely to the E effect. Therefore in the group of patients with grave generalized myasthenia, the efficacy of splenectomy amounted to 65%. In view of this fact the inclusion of the given treatment method into the multimodality treatment of myasthenic patients is regarded advisable.

The authors describe the course of myasthenia in children whose mean age of the disease onset is 10.1 years (7.9 years in boys and 11.2 years in girls). All the children had generalized myasthenia. The use of plasmapheresis produced a beneficial effect in all the cases, especially in children with grave myasthenia.

A total of 1630 patients with various neuromuscular diseases of autoimmune genesis were treated. Glucocorticoid drugs taken every other day were shown to be highly effective. On the basis of a large experience the authors propose recommendations for the management of patients in relation to the form, severity and course of the disease with the objective of achieving the maximum therapeutic effect.

It has been established that the mean number of myoid cells in one visual field in the thymus from myasthenia patients exceeds normal approximately twofold, with significant individual fluctuations being recorded. The observation data allow one to distinguish two basic forms of changes in myoid cells in myasthenia: (1) virtually complete absence of myoid cells leading to deficiency of myoid antigens; (2) more frequently seen hypertrophy and the increased number of myoid cells responsible for excess myoid antigens in the thymus. Basing on the fact that hypertrophy of myoid elements is seen, in most cases, in the presence of a pronounced hypoplasia of the lymphoid tissue, a suggestion is made about derangement in myasthenia of a mechanism by which the ratio of lymphoid and myoid cells in the thymus is controlled.

The article shows possibilities of thermography in the study of peripheral blood circulation. Data are presented on examining the patients with diverse pathology forms by means of conjunctival biomicroscopy and television capillaroscopy of the nail bed. Evaluation of these methods and analysis of the data obtained are presented. The authors believe in more extended possibilities of applying microcirculation study methods together with thermography for disclosure of some diseases.

Deposits of granular material containing IgM, IgA, and IgG were revealed in the thymus of patients with myasthenia by direct immunofluorescence. Treatment of the thymus sections by unlabeled preparations against individual classes of human immunoglobulins inhibited the reaction of the granular material with the homologous labeled preparations. Disappearance of fluorescence of these deposits was also seen in treatment of the sections with glycine-HCl-buffer, pH 2.8. These data permitted a suggestion that granular material represented immune complexes where IgM, IgA, and IgG served as antibody, and thymus tissue components--as an antigen. The presence of bound immunoglobulin in the thymus indicated that an autoimmune process directed against the tissues of this organ occurred in myasthenia.

The circulatory dynamics was studied in the conjunctival vessels of 45 patients with ischaemic heart disease without hypertension (12 patients with myocardial infarction including) and in 24 patients with ischaemic heart disease and hypertension. The method of biomicroscopy of the conjunctiva permits to examine the shape of the microvessels, the vascular background and the density of the pattern, as well as the nature of the blood flow. The patients with ischaemic heart disease without hypertension had more distinct changes, in some cases -- with irreversible discontinuity of the flow (aggregation of the erythrocytes in myocardial infarction). In ischaemic heart disease with hypertension the flow in the venules restores completely by the end of therapy. Reversible changes in the microcirculation were demonstrated in cases of a good effect of therapy, as well as the possibilities of timely diagnosis of complications in repeated examinations of the conjunctival vessels.

Myoglobin serum levels have been measured in 130 patients with various neuromuscular diseases whose development is related to the denervational process secondary to damage to motoneurons and their axons as well as in patients with impaired neuromuscular transmission. Interest in myoglobin is explained by the fact that hypermyoglobinemia reflects either destruction or impairment of the penetrability of the membranes of muscle fibers. The data obtained indicate that the degree of myoglobinemia is correlated with the course of the process rather than with the severity of motor disturbances. There is a distinct correlation between the level of myoglobinemia and impairment of the trophic function of motor neuron axons in neuronal and axonal lesions.

In ten patients with generalized myasthenia in whom disease exacerbation was noted long after thymectomy the authors employed a multiple modality treatment including therapeutic plasmapheresis (TP). The treatment was monitored by examining the status of neuromuscular transmission and parameters of external respiration, as well as the levels of immunoglobulins. It has been shown that the inclusion of TP into the complex of therapeutic measures in the given category of patients makes it possible to arrest quickly and to a considerable degree the clinical manifestations of an exacerbation, to increase the reliability of neuromuscular transmission and decrease doses of anticholinesterase drugs.

With the aid of an indirect method of immunofluorenscence in the process of a study of 65 blood sera of patients with myasthenia, the authors, demonstrated significant prevalence of antibodies titres to myoid cells and to an epithelial thymus reticulum (especialey in patients with thymomas) in comparison with normals. These data my indicate an autoimmune character of myasthenia. A positive effect of hormonal therapy was accompanied by a distinct drop in the level of antibodies in patients with myasthenia which permits to assume a certain connection between hormonotherapy and its influence on the autoimmune processes.

On the basis of a long-term observation of 1000 patients with myasthenia the authors stress the necessity of a manifold treatment of this disease with the use of anticholinesterase preparations, by correcting the mineral metabolism and by influencing the thymus gland. In specially severe cases an intensive hormone therapy is recommended with large doses of triamcinolon and prednisolon. In 39 cases of a severe myasthenia the used treatment was successful. The paper deals with theoretical prerequisites and methods of treatment by corticosteroid preparations, their combination with other drugs and an optimal appliance of hormone therapy with preparations of kalii and veroshpiron.

It has been demonstrated by the indirect immunofluorecent technique that many of the sera of patients with myasthenia gravis react with the anticells of the human thymus epithelial tissue. Sorption of the sera with the suspension of the epidermis cells and the homogenates of the tissues of other human organs showed that the epithelial cell antigen with which the sera of patients with myasthenia reacted were epidermal heteroorganic thymus antigens, i.e. common for the thymus epithelium and skin epidermis. The presence of antibodies to the cells of the epithelial tissue of the thymus in the sera of patients suffering from myasthenia gravis permits to suppose the existence of an immunopathological process against the thymus tissue antigens (including the heteroorganic structures of its epithelium) in this disease.

It has been established that sera from myasthenia patients contain in 42% of cases IgG antibodies to the point antigenic structure on the lymphocyte surface of the human thymus, spleen and lymph nodes. This antigenic structure is less pronounced on the lymphocytes from the thymus of normal subjects as compared with lymphocytes from the spleen and lymph nodes. On the contrary, the thymocytes from myasthenia patients show an elevated content of the antigen in question and do not differ in this respect from lymphocytes of the peripheral lymphoid organs of normal man.

The authors report of their observations over 52 patients with severe and moderate myasthenia who after diverse forms of therapy (thymusectomy, x-ray therapy, prolonged use of anticholesterase preparations, etc.) did not demonstrate a stable therapeutical effectiveness. However, treatment by retabolil proved to be effective with minimal side effects. The paper contains theoretical prereqisites to therapy by anabolic hormones and the approximate scheme of its use.

The article presents an analysis of long-term follow up studies of more than 2500 patients with various forms of neuromuscular transmission impairments. The results of repeated observations, employing electromyographic and immunologic techniques made it possible to raise a question about the homogeneity and differences in the mechanisms of myasthenia formation, and also about combinations of myasthenia with other autoimmune diseases and with the myasthenic syndrome of Lambert- Iton 's type.