Myasthenia gravis (MG) is a common antibody mediated, cell-mediated, and complement dependent neuromuscular junction immune disease. The treatment mainly includes drug therapy (symptomatic therapy, non-specific immunosuppressive therapy, targeted immunotherapy), immune regulation (intravenous injection of human immunoglobulin and plasma exchange), and thymectomy. With the continuous deepening of research on MG treatment, targeted immune regulation of B cells, complement system, and neonatal Fc receptors has become a current research hotspot in the treatment of MG. Compared with traditional immunosuppressants, MG patients have better tolerance to new biological agents. This article elaborates on the research of MG targeted therapy related drugs and summarizes their efficacy and safety in MG treatment, aiming to find more treatment options.
Objective To present the preliminary clinical experience of robot assisted trans-subxiphoid (extended) thymectomy in patients with thymic neoplasms or myasthenia gravis. Methods A total of 62 patients (34 males and 28 females at an average age of 38±11 years) suffering from thymic neoplasms or myasthenia gravis who underwent robotic (extended) thymectomy via subxiphoid approach were included in our department between August 2016 and August 2017. All of the operation were completed through 4 ports. In details, the observation hole was created just below the xiphoid process, two ports for arm 1 and arm 2 were created below bilateral subcostal arch at the midclavicular line, and trocar for arm 3 was placed in the 5th or 6th intercostal space at the anterior axillary line, respectively. Patients with thymic neoplasms received thymectomy. Patients with myasthenia gravis received extended thymectomy. Results All the patients experienced uneventful operations. The mean operative time was 116.0±34.0 min. The mean intraoperative blood loss was 5.6±4.3 ml. The mean postoperative hospital stay was 4.0±2.2 days. There was no intra-operational massive hemorrhage, mortality, conversion or postoperative complication during the postoperative and follow-up period. Conclusion Robotic trans-subxiphoid thymectomy is safe and feasible, which is a promising technique for extensive application.
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 analyze the surgical efficacy and influencing factors of myasthenia gravis (MG) patients with thymic atrophy after thymectomy. MethodsThe clinical data of MG patients with thymic atrophy undergoing thymectomy between October 2014 and May 2018 in Daping Hospital of Army Medical University and Shijiazhuang People Hospital were retrospectively analyzed. ResultsA total of 71 patients were collected, including 40 males and 31 females with a mean age of 45.17±12.42 years. All patients received the surgery successfully. After the surgery, 20 (28.17%) patients were stable remission, 12 (16.90%) patients were minimal manifestation status,19 (26.76%) patients were improved, 5 (7.04%) patients showed no change, 3 (4.23%) patients were worsened, 10 (14.08%) patients were exacerbated and 2 (2.82%) patients were dead. Multivariate logistic regression analysis showed that the preoperative illness duration (OR=4.61, 95%CI 1.13-18.85, P=0.03), and postoperative pyridostigmine combined with immunosuppressive (OR=0.12, 95%CI 0.03-0.45, P=0.00) were independent risk factors for long-term efficacy of thymectomy for MG patients with thymic atrophy. ConclusionEarly surgery after diagnosis of MG and postoperative pyridostigmine combined with immunosuppressive treatment is beneficial to the prognosis of MG patients with thymic atrophy.
ObjectiveTo analyze the risk factors of myasthenia gravis crisis after thymectomy with myasthenia gravis (MG).MethodsSixty-five myasthenia gravis patients who had myasthenia crisis after thymectomy in Xuanwu Hospital, Capital Medical University from June 2006 to June 2019 were retrospectively enrolled, including 31 males and 34 females, aged 15-78 (45.7±17.8) years. The relationship between myasthenia crisis after thymectomy and surgical option, operation time, pathological type, et al. were anylyzed.ResultsOperation time and pathological type were the predictive factors of postoperative myasthenic crisis. The area under receiver operating characteristic curve (AUC) of MG type (Osserman) was 0.676, the cut-off value wasⅡB type, the sensitivity was 37.5%, the specificity was 90.5%, and the Youden’s index was 0.280. The AUC of thymoma stage (Masaoka) was 0.682, cut-off value was stageⅡ, sensitivity was 62.5%, specificity was 66.7%, and Youden’s index was 0.292. The AUC of blood loss was 0.658, the cut-off value was 90 mL, the sensitivity was 87.5%, the specificity was 69.6%, and the Youden’s index was 0.304.ConclusionPreoperative MG classification, pathological type, operation time and blood loss are the risk factors of postoperative myasthenic crisis. Therefore, adequate preoperative preparation, rapid and careful intraoperative operation and active postoperative management can reduce the occurrence of postoperative myasthenic crisis.
Objective To investigate the value of ice test in the diagnosis of ptosis of myasthenia gravis(MG). Methods A total of 32 patients with myasthenic ptosis and 33 with nonmyasthenic ptosis underwent ice and rest test which were performed alternately twice within 1 day on each patient. Besides, neostigmine test was performed on the patients with myasthenic ptosis after ice and rest test . Two observers who didnrsquo;t know the clinical diagnosis were asked to evaluate the improvement of eyelid elevation by measuring the width between the midpoints of upper and lower eyelid with a 20mm steel rule (precision of 0.5 mm). The average of margin of palpebral fissure width after double ice or rest tests subtrac ted from the one before the tests in one patient was the standard of the improve ment of eyelid elevation. Results Ice and rest test improved myasthenic ptosis but not nonmyasthenic ptosis with the specificity of 100% in both of the tests. In addition, ice test improved myasthenic ptosis more effectively with a higher sensitivity of 78%, and it could also improve the palpebral fissure width in pa i tents with complete myasthenic ptosis apparently. Compared with the neostigmine test, ice test had lower sensitivity, cost shorter time, didnt need injection which avoided the discomfort, and had no side effects. Conclusion Ice test is a simple and safe means with high sensitivity and specificity to diagnose myasthenic ptosis, which is valuable in clinical application. (Chin J Ocul Fundus Dis, 2006,22:382-384）
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.
Objective To evaluate the risk factors affecting the prognosis in patients with myasthenia gravis after thymeetomy. Methods Therapeutic effects were evaluated with Relative Counting Method in 136 cases with myasthenia gravis who receivingthymectomy. Six clinical factors including sex, age, preoperative course, et al. were converted into quantitative parameters and used for analysis. Cumulative logit model for ordinal response was employed to investigate the therapeutic effects of various factors. Results Sex, age and preoperative course exerted significant effects on postoperative prognosis in patients with myasthenia gravis, while clinical subtype, pathological changes and preoperative administration of cholinesterase inhibitors had no significant impact on therapeutic effects. Conclusion Some factors may lead to the remission and improvement in postoperative patients with myasthenia gravis, therefore, they should be included for the evaluation of prognosis. Early diagnosis and early thymeetomy may improve the therapeutic effects and shorten the duration of amelioration.
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.
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.