[Plasmapheresis in combined treatment of severe generalized myasthenia]. / Plazmaferez v kompleksnom lechenii tiazheloi generalizovannoi miastenii.

Experience in the application of plasmapheresis in various stages of treatment is analysed. It was conducted in 52 patients with severe forms of generalized myasthenia; 12 of them were males and 40 were females. It is noted that plasmapheresis is very effective in the preoperative period for the preparation of patients for operation and is ineffective in medicamentous therapy. In the late-term periods after thymectomy plasmapheresis is mainly recommended for arresting crises and precritical conditions because of the rapid and short effect of the method. In patients who underwent thymectomy and splenectomy the effect of plasmapheresis in the late-term period after the operation was of a longer duration. Research must be continued and clinical experience accumulated to identify exactly the mechanism of the effect of plasmapheresis on the myasthenic status and the immune and neuroendocrine systems and to determine the place of the method in the complex of therapeutic measures.

[Residual thymus gland in patients operated on for myasthenia]. / Ostatochnaia vilochkovaia zheleza u bol'nykh, operirovannykh po povodu miastenii.

The authors studied the causes of ineffective surgical treatment of myasthenia and found residual thymus to be one of them. From analysis of 21 cases in which part of the thymus was not removed (in 19 cases the diagnosis was verified histologically), they drew the conclusion that the causes of residual thymus may be technical errors due to inadequate choice of the surgical approach of the surgeons' insufficient experience, in removal of a thymoma failure to excise part of the thymus not involved in the tumor, incomplete knowledge of the role of the thymus in the pathogenesis of myasthenia and its incomplete removal in thymectomy in patients with generalized myasthenia. Uneventful postoperative course was noted in 11 patients, crises with respiratory disorders in 6 (prolonged artificial lung ventilation had to be resorted to in 4 of them), suppuration of the wound in 2, and pneumonia in 1 patient who was under artificial lung ventilation. The late--term results were studied in follow--up periods of 12 months to 15 years. The myasthenic status improved in all patients, the results were excellent and good in 15. In view of the difficulty in establishing the diagnosis and management, this grave category of patients must be treated at specialized centers.

[The morphological and clinico-immunological characteristics of 2 types of myasthenia]. / Morfologicheskaia i kliniko-immunologicheskaia kharakteristika dvukh tipov miastenii.

The thymus is studied morphologically in 139 cases of myasthenia (with no thymic neoplasia). Hyperplasia of true thymic parenchyma (without its intralobular perivascular spaces) was observed in 28%, no change in 40% and atrophy in 32% of cases. Two types of the disease were distinguished on the basis of morphological and clinico-immunological parameters: the 1st type with a hyperplastic or normal thymic parenchyma and the 2nd type with its atrophy. These types probably differ in their pathogenesis. The outcome of the thymectomy in patients with the 1st type of myasthenia is most favourable, while in patients with the 2nd type the outcome in 42% of patients is either satisfactory or unfavourable.

[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.

[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.