Surgical treatment of myasthenia gravis and its evaluation]

30 cases of myasthenia gravis (MG) were treated with thymectomy. Before and after operation, peripheral blood lymphocyte subpopulation was determined in 10 cases and acetylcholine receptor antibody titer was determined in 6. The results confirmed the efficacy of thymectomy in treating MG. We suggest that application of hormone for regulating immune function of the body and/or plasma exchange for alleviating symptoms in patients with generalized MG before and after the operation. The results of treatment did not correlate with age, sex, length, and course of the disease. Radical operation, proper anaesthesia and appropriate antibiotics may ensure a smooth operation and high curative effect.     

Castleman's disease associated with myasthenia gravis

Castleman's disease presents as a peculiar type of lymph node hyperplasia. Traditionally, the disease has been classified on clinical grounds (solitary or multicentric) and by histologic appearance (hyaline vascular pattern, plasma cell predominance, or mixed lesions). It is now increasingly clear that there are different etiologies for each of these different subtypes. Reported associations include POEMS syndrome (polyneuropathy, organomegally, endocrinopathy, monoclonal gammopathy, and skin changes), paraneoplastic pemphigus, Hodgkin's disease, and follicular dendritic cell sarcoma. We present a case of Castleman's disease associated with myasthenia gravis, the third reported case in the literature. We discuss Castleman's disease and review the literature.     

Surgical treatment for myasthenia gravis.

A new surgical technique for thymectomy is presented. Three hundred and seventeen patients with myasthenia gravis and 20 with thymomas who had myasthenic symptoms were operated on. The new surgical approach--a small transverse sternotomy--was used in 257 cases (in 240 patients with myasthenia gravis and 17 with thymomas) and conventional median sternotomy in 80. In myasthenic patients small transverse sternotomy enabled radical thymectomy to be performed with an uneventful postoperative course and very good cosmetic results. There were no hospital deaths among patients with myasthenia gravis after thymectomy. The long term results, assessed after 18-24 months, were good: the total remission rate was 39.5%, and there was a great improvement in 48.5% and an improvement in 9%. After thymectomy about 30% of patients received supplementary treatment with prednisone. A correlation between the duration of symptoms and the result of thymectomy was established: the shorter the duration of myasthenia gravis the better the results. In the small group of 20 patients with thymomas two died in hospital. In 12 patients with encapsulated thymic tumours the long term results were similar to those in patients with myasthenia gravis, whereas in patients with infiltrating thymic tumours the results were unsatisfactory.     

Surgical treatment of myasthenia gravis in children

Thymectomy was included in the complex of therapeutic measures in the management of generalized myasthenia in 41 children. Much attention was given to preoperative management in which plasmapheresis was applied along with the traditional methods. As a result, maximum compensation of the myasthenic disorders was achieved and spontaneous respiration was restored in 9 patients who were given artificial ventilation of the lungs before the operation. The authors describe the techniques of thymectomy and the changes found in the thymus during histological study. No complications occurred. All of the children were discharged from the clinic in an improved condition. The late-term results were studied in 38 children in follow-up periods of 6 months to 4 years. Marked stable improvement was recorded in 31 (77.5%) patients.     

Surgical treatment of myasthenia gravis in children

The article deals with the treatment of myasthenia in 33 children whose ages ranged from 3 to 15 years. Myasthenia of moderate severity was diagnosed in 14, severe in 15, and extremely severe in 2 children. To make a precise diagnosis, loading, neostigmine methylsulfate, cold, and D-tubocurarine tests were conducted. Thymectomy was performed in 32 patients. The operation was carried out through a T-shaped sternotomy approach. Thymogenic myasthenia was verified in 31 patients and thymomogenic only in one patient. There were no fatal outcomes. The late-term results were studied in 12 patients in follow-up periods of 3 to 6 years. Adaptational activity of the patients, anticholinesterase agents and the results of stimulant electric myography served as the criterion in appraising the late-term results. Modern diagnostic methods and surgical intervention ensure a favourable result in 75% of patients.     

Surgical treatment of myasthenia gravis in childhood

Myasthenia gravis is considered to be an autoimmune disorder of neuromuscular transmission. Thymectomy is an effective mode of treatment which produces either remission or stabilization of symptoms especially in young female patients with short duration of disease. The present study, records the experience in ten pediatric patients with early thymectomy done via the transcervical approach. There were no operative deaths or serious complications. Follow-up for a mean of 37 months has shown one to be in complete remission requiring no medication and eight to have improved or stable symptoms. Thymectomy via the transcervical approach is an effective mode of treatment for patients with myasthenia gravis. Since this approach involves a small dissection and avoids sternotomy, the transcervical approach results in a very short postoperative recovery period. It is ideal for those patients not in respiratory failure. It can also be safely done in patients requiring intubation for acute respiratory failure preoperatively.     

Surgical treatment of thymomas in patients with generalized myasthenia gravis

Experience in surgical treatment of 2977 patients with generalized myasthenia is presented, 386 (13%) of them have undergone surgeries for thymus tumors. Organo-specific tumors (thymoma) were diagnosed in 370 (95.9%) of them. Computed and magnetic-resonance tomography has the highest sensitivity and specificity in diagnosis of thymus tumors. Thymomthymectomy was performed in 317 patients including extended one in 117 (36.9%). Postoperative lethality in thymic tumor in the last years was 0.5%, general lethality over all period--5.1%. According to used classification (WHO, 1999) types of thymoma were the following: type A--2.1%, type B--86.3%, type AB--9.6%. Prognosis of surgical treatment depends of tumor type (it is worse in type B than in types A and AB), duration of the disease and severity of myasthenia.     

Surgical aspects in the treatment of myasthenia gravis

We treated 81 patients suffering from myasthenia with thymectomy in a 10-year period (1991-2000). We think, that thymectomy must be carried out in the treatment of myasthenia gravis, unless contraindications are present. The operation is not urgent and in the preoperative period patients must reach optimal condition with the help of standard medical treatment. The result of the operation is influenced by the length of time between the beginning of the complaints and the operation. The best results can be expected when the operation is performed in less than 2 years time, however an operation performed later may also be successful. We operated on 63% (n = 51) of the patients between 4-12 months, on 18% (n = 15) between 13-24 months and on 18.6% (n = 15) more than 25 months after the beginning of the complaints. Complete thymectomy was performed in all patients through median sternotomy. We had no operative mortality. Our postoperative results were evaluated with Jaretzki classification: 75% of our patients are in remission and asymptomatic (n = 61).     

Thymolipoma associated with myasthenia gravis

A 51-year-old woman with a 3-year history of diplopia was admitted to our hospital. Repetitive stimulation of the right median nerve revealed decreasing hand muscle responses. Edrophonium chloride administration alleviated the symptoms. The serum anti-acetylcholine receptor antibody level was 3.3 nmol/l; thus, myasthenia gravis was diagnosed. A chest roentgenogram revealed a left inferior mediastinal mass, and a chest computed tomographic scan revealed a fat density mass partly containing soft tissue areas in the left side of the anterior mediastinum. Median sternotomy and extended thymectomy were performed. The pathology examination of the tumor revealed mature adipose tissue including islands of thymic tissue containing Hassall's corpuscles, without germinal centers. The tumor was a thymolipoma of the anterior mediastinum. Her postoperative course was good, and she is doing well at 1 year postoperatively. The present study is the 15th reported case of thymolipoma associated with myasthenia gravis. We did not confirm a causal relationship between the thymolipoma and myasthenia gravis.     

Surgical management of myasthenia gravis

The experience from 1940 to 1970 with thymectomy for myasthenia gravis both with and without an associated thymoma is reviewed. This operation is satisfactory when applied to selected patients without thymoma. It is indicated in every patient with myasthenia and a thymoma.     

Primary intrapulmonary thymoma associated with myasthenia gravis

Primary intrapulmonary thymomas are very rare. So far, research in the field has identified only 31 cases. In all databases, a total of two published articles describing primary intrapulmonary thymoma with myasthenia gravis were encountered between 1950 and 2010. We admitted a 58-year-old male patient with a mass in the right lower lobe of his lung. The tumor was excised, and histological findings were found to be consistent with Type AB thymoma. The patient was intubated due to respiratory distress during the postoperative period, and his acetylcholine receptor antibody was determined positive. He was diagnosed with myasthenia gravis. Pyridostigmine therapy and plasmapheresis were scheduled; yet, we could not begin therapy because of rapid deterioration of the patient's respiratory status due to myasthenia gravis and subsequently resulting in intubation-associated pneumonia. The patient's health rapidly worsened, and he died.     

A case of nephrotic syndrome associated with myasthenia gravis and malignant thymoma]

A 26-year-old woman who presented facial and lower leg edema associated with massive proteinuria was admitted to our hospital in February 1992. Nine months before this admission, she exhibited myasthenia gravis and malignant thymoma, and underwent total thymectomy. On admission, there was no symptom of myasthenia gravis. She was diagnosed as having nephrotic syndrome and the first renal biopsy was performed. The histological findings showed membranous nephropathy. Immunofluorescent microscopy revealed that IgG and C3 were stained in a granular pattern in the periphery, and subepithelial deposits were observed in the basement membrane of the glomerulus by electron microscopy. With the administration of prednisolone, proteinuria disappeared and the nephrotic syndrome remitted. She was admitted again in January 1993 due to proteinuria and lower leg edema following cystitis. The findings of the second renal biopsy were unremarkable. She was administered cyclosporin A to improve the nephrotic syndrome and to reduce the side effects of prednisolone. The proteinuria disappeared again and this effect was dependent on the dose of cyclosporin A. Since the first administration, no symptoms of myasthenia gravis or malignant thymoma have been observed. The relationships among myasthenia gravis, malignant thymoma and nephrotic syndrome were examined. Although the first renal biopsy findings showed membranous nephropathy, from the therapeutic responses of both prednisolone and cyclosporin A, the main course of proteinuria in this case may have been due to minimal change nephrotic syndrome. We consider this case of nephrotic syndrome to be important considering its etiology and the relationship between the histological findings and its clinical course.