[Twin studies of myasthenia]. / Bliznetsovye issledovaniia miastenii.

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.

[Analysis of the association of ABO blood groups and Rhesus factor with myasthenia]. / Analiz assotsiatsii grupp krovi ABO i rezus-faktora s miasteniei.

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.

[Diagnosis and surgical treatment of myasthenia gravis in childhood]. / Diagnostika i khirurgicheskoe lechenie miastenii v detskom vozraste.

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.

[Tactical and technical errors in surgical treatment of patients with generalized myasthenia gravis]. / Takticheskie i tekhnicheskie oshibki pri khirurgicheskom lechenii bol'nykh generalizovannoi miastenii.

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%.

[Splenectomy in the treatment of severe generalized myasthenia gravis (first experience)]. / Splenektomiia v lechenii tiazheloi generalizovannoi miastenii (pervyi opyt).

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.

[Clinical characteristics of myasthenia gravis in children and the role of plasmapheresis in its complex treatment]. / Klinicheskie osobennosti miastenii u detei i rol' plazmafereza v kompleksnoi terapii.

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.

[Splenectomy in the treatment of severe forms of myasthenia gravis]. / Splenéktomiia v lechenii tiazhelykh form miastenii.

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.

[Serum myoglobulin levels in neuromuscular diseases]. / Soderzhanie syvorotochnogo mioglobina pri nervno-myshechnykh zabolevaniiakh.

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.

[Use of therapeutic plasmapheresis in patients with generalized myasthenia during exacerbations in the late period following thymectomy]. / Primenenie lechebnogo plazmafereza u bol'nykh generalizovannoi miasteniei v period obostreniia v otdalennye sroki posle timéktomii.

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.

[Strategy for using glucocorticoid preparations in neuromuscular diseases]. / Strategiia primeneniia gliukokortikoidnykh preparatov pri nervno-myshechnykh zabolevaniiakh.

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.

[Immunologic pathology in patients with disorders of neuromuscular transmission]. / Immunologicheskaia patologiia u bol'nykh s narusheniem nervno-myshechnoi peredachi.

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.

[Immunofluorescence study of changes in myoid cells in the thymus of myasthenia patients]. / Izuchenie izmenenii mioidnykh kletok timusa bol'nykh miasteniei metodom immunofluorestsentsii.

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.

[Reaction between myasthenia patients' sera and the antigenic structure of the surface of thymic lymphocytes of healthy subjects and myasthenia gravis patients]. / Reaktsiia syvorotok bol'nykh miasteniei s antigennoi strukturoi poverkhnosti limfotsitov timusa zdorovykh liudei i bol'nykh miasteniei gravis.

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.