Objective To summarize experiences of surgical treatment and long-term results of myasthenia gravis (MG). Methods Two hundred thirty-six patients underwent thymectomy for MG in our department from Jan.1978 to Dec. 2002. The perioperative management, relative factors of postoperative crisis and long-term results were analysed. Results In 236 patients postoperative crisis took place in 44 cases accounted for 18.6%. The occurrence of postoperative crisis was related to preoperative management, modified Osserman clinical classification and combination with thymoma. Three cases died in the postoperative periods. Among them, one died of acute respiratory distress syndrome induced by aspiration and the other died of crisis. The effective rate in 1, 3, 5 years was 84.6%, 91.0% and 89.0% respectively. Conclusions Thymectomy for MG is safe and effective. Delayed extubation could decrease the needs of tracheotomy in patients with high risk factors for postoperative crisis. The partial sternotomy approach is less traumatic but the long-term effects of surgery are identical to those reported by the most authors.
ObjectiveTo investigate the effect of noninvasive ventilation (NIV) in patients with myasthenic crisis after thymectomy. Methods31 myasthenic crisis patients after thymectomy who initially used NIV,admitted in the First Affiliated Hospital of Guangzhou Medical University between January 2011 and June 2013,were analyzed retrospectively.They were assigned to two groups according to the successful application of NIV or not,with 13 patients in the NIV success group and 18 patients in the NIV failure group.The related factors including gender,age,APACHEⅡ score when admitted to ICU,the results of blood gas analysis before NIV,thymoma or not,the history of myasthenic crisis,the history of chronic lung disease,and minute ventilation accounted for the largest percentage of predicted value (MVV%pred)were analyzed. ResultsThere were no significant differences in age,gender,or APACHEⅡ score between two groups (P>0.05).The PaCO2 in the NIV success group was lower than that in the NIV failure group.The preoperative MVV%pred in the NIV success group was higher than that in the NIV failure group.There were no significant differences between two groups in pH,PO2,thymoma or not,the history of myasthenic crisis,or the history of chronic lung disease (P>0.05).If using the 45 mm Hg as the cut-off value of PaCO2 and 60% as the cut-off value of MVV%pred,the incidence of PaCO2<45 mm Hg and the incidence of MVV%pred>60% were higher in the NIV success group than those in the NIV failure group (84.6% vs.33.3%, P<0.05;100% vs. 55.6%,P<0.05).Logistic regression analysis revealed that PaCO2<45 mm Hg was an independent influence factor for successful application of NIV in patients with myasthenic crisis after thymectomy. ConclusionPaCO2<45 mm Hg can be a predictor of successful application of NIV in patients with myasthenic crisis after thymectomy.For the patients underwent NIV whose PaCO2<45 mm Hg or MVV%pred<60%,the clinician should predict the possibility of failure and prepared for intubation.
ObjectiveTo compare clinical effects of enlarged thymectomy for the treatment of myasthenia gravis (MG) complicated with thymoma via subxiphoid and subcostal arch thoracoscopic resection versus median sternotomy resection. MethodsWe retrospectively analyzed the clinical data of patients with MG complicated with thymoma admitted in Tangdu Hospital of the Air Force Military Medical University between December 2011 and December 2021. Patients who underwent subxiphoid and subcostal arch thoracoscopic enlarged thymectomy were allocated to a SR group, and patients who underwent median sternotomy enlarged thymectomy were allocated to a MR group. Perioperative outcomes were compared between the two groups. ResultsA total of 456 patients were collected. There were 51 patients in the MR group, including 30 males and 21 females aged 23-66 (49.5±11.8) years. There were 405 patients in the SR group, among whom 51 patients were matched to the MR group by propensity score matching, including 28 males and 23 females aged 26-70 (47.2±12.2) years. The operations were accomplished successfully in all patients, and no conversion to thoracotomy occurred in the SR group. The SR group had advantages in the operation time, intraoperative blood loss, chest drainage duration, hospital stay time, patients’ satisfaction level, pain score and complications (all P<0.05). No statistical difference was found in the number of intraoperative lymph node dissection stations, number of intraoperative lymph nodes dissected or remission of MG between the two groups (P>0.05). ConclusionSubxiphoid and subcostal arch thoracoscopic enlarged thymectomy and lymphadenectomy is a safe, effective and feasible minimally invasive procedure for the treatment of MG complicated with thymoma.
Abstract: Objective To investigate the clinical characteristics of thymoma and thymoma with myasthenia gravis(MG). Methods From Oct.1979 to July 2004,185 patients with thymoma were surgically treated. Among these patients, comparative analysis was made between 94 cases of thymoma (thymoma group) and 91 cases of thymus tumor with MG(thymoma with MG group).155 patients underwent radical operation (83.8%),16 patients underwent palliative operation (8.6%),and 14 patients underwent exploratory operation (7.6%). Clinical characteristics was analyzed in two groups. The factors affecting prognosis was analyzed by Masaoka’s stage system, with the lifttable method. Results Five patients died after operation, others had complete remission or symptomatic improvement. There was statistically difference of Masaoka’s stage system in two groups (χ2=53.14, P＜0.05). There were no statistically difference in pathological type of thymoma and clinical type of MG and pathologic period (χ2=8.21, P＞0.05). 57 cases of thymoma group were followed up, the duration of follow-up was 1 to 10 years, average follow-up was 40.7 months, and the patients with 1-, 3- and 5-year survival rates were 70.2% (40/57), 66.7% (22/33), 593% (16/27) respectively. 55 cases of thymoma with MG group were followed up. The patients’ survival rates were 98.2% (54/55), 86.4% (38/44), 81.6% (31/38) at 1-, 3-and 5-year respectively. There was no statistically difference of survival rates in two groups (χ2=0.83, P＞0.05). Totally, 112 patients were followed up in two groups, by Masaoka’s stage system, the 5-year survival rates were 93.7% for stage Ⅰ, 79.2% for stageⅡ, 51.4% for stage Ⅲ and 0% for stage Ⅳ respectively. Result of asaoka’s stage system evidence was statistically significant (χ25-year=51.62, P＜0.01). Conclusions Pathological type of thymoma isn’t related to modified Osserman’s classification, prognosis of thymoma is obviously related to Masaoka’s stage and isn’t related to MG. Generalized MG is the major type in MG patients accompanied by thymomas, and the major pathological type is lymphocytic. Chest CT can increase the accuracy early diagnosis of thymoma. The principal treatment is to resect the tumor as completely as possible, and proper administration of postoperative radiotherapy or chemotherapy according to the surgical status. Operative program and tumor stage are the most important prognostic factors.
ObjectiveTo analyze the risk factors for post-thymectomy myasthenic crisis (PTMC) and prolonged mechanical ventilation, in myasthenia gravis patients who underwent extended thymectomy. MethodsWe retrospectively analyzed the clinical data of 79 patients including 38 males and 41 females who experienced PTMC and required mechanical ventilation in Daping Hospital between June 2008 and November 2014. Single factor analysis and multivariate analysis were conducted. ResultsMorbidity of PTMC was 20.6% (79/384). Result of single-factor analysis showed that postoperative pneumonia was one of the main reasons of prolonged mechanical ventilation (P < 0.05). Result of multiple-factor analysis showed that the operation time was positively correlated with mechanical ventilation time (P < 0.05). The risk factor of prolonged mechanical ventilation time in PTMC was not associated with sex, age, disease history, myasthenic crisis history, Osserman classification, dosage of pyridostigmine before and after the operation, surgical approach, bleeding volume, other therapies besides mechanical ventilation (P > 0.05). ConclusionMechanical ventilation is one the main therapy of PTMC, operation time, and postoperative pneumonia are the main factors to prolong mechanical ventilation time. In order to decrease morbidity of PTMC and shorten mechanical ventilation time, the operation time should be controlled and pulmonary infection should be avoided.
Thymectomy is a major surgical procedure for patients with non-thymomatous myasthenia gravis，and can enhance their symptomatic remission rate and cure rate. There is still much controversy about appropriate surgical approach and extent of resection of thymectomy. The majority of thoracic surgeons believe that the completeness of thymectomy is closely associated with clinical symptom improvement，and perform complete resection of encapsulated thymus and surroun-ding fat tissues via mid-sternotomy. But minimally invasive thymectomies are often more acceptable by patients. On the contrary，in view of common existence of ectopic thymus tissue，some thoracic surgeons advocate a combination of cervical incision and sternotomy in order to further completely remove all thymus tissue.
Objective To exploring the effectiveness of perioperative application of new surgical clinical classification and staging for myasthenia gravis (MG) in reducing the incidence of postoperative myasthenic crisis (MC). Methods The clinical data of patients with generalized MG admitted to the Comprehensive Treatment Center for Myasthenia Gravis of Henan Provincial People’s Hospital from January 2018 to June 2022 were retrospectively analyzed, who were scored with myasthenia gravis-activities of daily living (MG-ADL) score and quantification of the myasthenia gravis (QMG) score at the first visit, 1 day before surgery, and 3 days after surgery. The patients were divided into a group A (typeⅡ) and a group B (typeⅢ+Ⅳ+Ⅴ) by the new surgical clinical classification and staging of MG according to the disease progression process, and all patients underwent expanded thoracoscopic thymus (tumor) resection after medication and other interventions to control symptoms in remission or stability. The incidence of MC and the efficiency rate after surgery were analyzed. The normal distribution method and percentile method were used to calculate the unilateral 95% reference range of the QMG score and MG-ADL score. Results Finally 126 patients were enrolled, including 62 males and 64 females, aged 13-71 years, with an average age of 46.00±13.00 years. There were 95 patients in the group A and 31 patients in the group B, and the differences of the preoperative baseline data between the two groups were not statistically significant (P>0.05). The incidence of postoperative MC was 1.05% (1/95) in the group A and 3.23% (1/31) in the group B (P>0.05). The effective one-sided 95% reference range of the QMG score and MG-ADL score 1 day before surgery was 0-7.75 and 0-5.00, and there was no postoperative death in both groups. Conclusion The new surgical clinical classification and staging of MG can guide the timing of surgery, which can benefit patients undergoing surgery for MG and greatly reduce the incidence of postoperative MC.
ObjectiveTo explore the clinical features of myasthenia gravis (MG) harboring both acetylcholine receptor antibody (AChRAb) and muscle-specific tyrosine kinase antibody (MuSKAb) positivity.MethodsWe searched PubMed, Web of Science, Embase and China National Knowledge Infrastructure databases (from inception to November 2016), to collect the case reports of MG with both AChRAb and MuSKAb positivity. Along with one case discovered in Department of Neurology, West China Hospital, the clinical data of the cases were retrospectively analyzed.ResultsA total of 13 double-seropositive MG patients were enrolled in this study, demonstrating a marked female predominance (including 1 male and 12 females) and a younger age at onset [(31.07±24.77) years]. During the disease course, 10 of the included patients presented severe bulbar involvement, dyspnea and neck weakness, with myasthenic crisis in 6 individuals. Among the 11 patients with detailed records, abnormal thymus glands comprised 4 thymus hyperplasia and one thymoma. While the response to oral pyridostigmine was unsatisfactory in 11 double-seropositive MG patients, ranging from mild benefit to overt intolerance; the patients treated with plasma exchange (3/3), rituximab (1/1) or corticosteroid (7/12) improved dramatically, with other immumosuppressants and intravenous immunoglobulin partially responsive. Moreover, 5 patients undergoing thymectomy improved markedly or partially.ConclusionsCompared with MG patients with MuSKAb positivity merely, the condition of the double-seropositive MG patients seem to be more severe and further inclined to myasthenic crisis. The incidence of abnormal thymus, such as thymus hyperplasia, is higher. Thymectomy may be an effective treatment for such patients.
Myasthenia gravis is an autoimmune neuromuscular junction disorder primarily mediated by autoantibodies against the acetylcholine receptor (AChR). It is now widely recognized that the total titer of anti-AChR antibodies does not correlate directly with clinical severity and shows significant interindividual variability. This review focuses on the structure of the AChR, the three major pathogenic mechanisms mediated by anti-AChR antibodies, the pathogenic differences associated with distinct antigenic epitopes, the characteristics of various immunoglobulin subclasses, and the limitations of current antibody detection methods. It further explores future directions in antibody profiling and functional assessment. By systematically analyzing the complexity and heterogeneity of anti-AChR antibodies, this article underscores the critical role of precision medicine in the management of myasthenia gravis.
In recent years, immune checkpoint inhibitors have begun to be used in targeted cancer therapy. Despite the favorable results in terms of oncological outcomes, these treatments have been associated with a variety of immune-related adverse events. Neuromuscular disease is more common among adverse events involving the nervous system. With the increasing use of immune checkpoint inhibitors, the early recognition and treatment of neuromuscular immune-related adverse events are very important. In this review, we are focused on the epidemiology, pathogenesis, clinical manifestations, evaluation, diagnosis, and treatment of neuromuscular diseases (including peripheral neuropathy, myasthenia gravis, and myositis) caused by immune checkpoint inhibitors, aiming to provide a theoretical basis for improving the diagnosis and treatment ability of users of immune checkpoint inhibitors for such neuromuscular diseases and reducing the disability rate and mortality rate caused by immune checkpoint inhibitors.