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.
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.
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.
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.
Objective To investigate application values and techniques of robot-assisted extended thymectomy for the treatment of typeⅠmyasthenia gravis （MG） using Da Vinci S system. Methods We retrospectively analyzed clinical data of 3 patients with MG who underwent robot-assisted extended thymectomy in General Hospital of Shenyang Military Command from March 2012 to September 2012. All the patients were ocular MG （typeⅠ） including 2 men （33 years and 66 years old respectively） and 1 woman （21 years old）. Surgical outcomes were analyzed. Results All the 3 patients successfully received robot-assisted extended thymectomy without accessorial incisions. None of the patients required converting to open sternotomy or postoperative reexploration for bleeding. Intraoperative blood loss was 5-10 ml．Overall operation time was95-138 minutes, and thymoma dissection time was 26-80 minutes. No myasthenic crisis or other major postoperative complic-ation occurred. Postoperative chest drainage duration was 3-9 days and postoperative hospital stay was 10-15 days. Two patientswere followed up for 6-12 months after discharge without MG recurrence. Conclusion Robot-assisted extended thymectomy is safe and feasible for the treatment of typeⅠMG with satisfactory results．
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.
ObjectiveA two-sample Mendelian randomization analysis was used to explore the causal associations between four basic body indices (basal metabolic rate, body fat percentage, BMI and hip circumference) and myasthenia gravis (MG). MethodsPooled gene-wide association study (GWAS) data were obtained from large publicly searchable databases, and four basic body indices were selected as the exposure factors and myasthenia gravis as the outcome factors, and single nucleotide polymorphisms (SNPs), which were strongly correlated with the phenotype of the exposure factors, were screened as the instrumental variables, and two-sample Mendelian randomization analyses were performed in order to assess the potential causal relationship between the exposure and the disease. ResultsInverse variance weighting (IVW) analysis showed that increased basal metabolic rate (OR=1.39, 95%CI 1.00 to 1.93, P=0.047), body fat percentage (OR=1.61, 95%CI 1.06 to 2.44, P=0.024), and hip circumference (OR=1.67, 95%CI 1.29 to 2.17, P<0.001) increased the risk of MG. But there was no significant causal relationship between BMI and MG. ConclusionBasal metabolic rate, body fat percentage and hip circumference have a positive causal relationship with MG, while BMI does not have a significant causal relationship with MG.
Objective To investigate the effects of inflammatory reaction of thymomas with myasthenia gravis (MG) treated by traditional thoracotomy and minimally invasive surgery. Methods A total of 40 thymomas patients (Mssaoka Ⅰ or Ⅱ) with myasthenia gravis from August 2014 to June 2015 were treated by traditional thoracotomy (n=20) or video-assisted thoracoscopic surgery (n=20). The serum levels of IL-6, IL-8, TNF-α, CRP and CORT were measured by enzyme-linked immunosorbent assay (ELISA) methods at before anesthesia (T1), after anesthesia (T2), 2 h after skin cut (T3), 24 h post-operation (T4), 48 h post-operation (T5) and 72 h post-operation (T6) respectively. Perioperative parameters were also reported. The statistics analysis was performed by SPSS 17.0 software. Results The serum levels of IL-6, IL-8, TNF-α, CRP and CORT had no significant difference between T1 and T2, T2 and T3 (allP value>0.05) in both groups. But the serum levels of these factors after operation were obviously higher than that of before operation, commonly the highest level was reached at T4 (allP value>0.01), and also was higher at T6 than that of before the operation (allP value<0.01), except the level of TNF-α recovered rapidly to the level of before operation (allP value>0.05) in the VATS group. The operation time, postoperative drainage tube indwelling time and incision healing time in the VATS were lower than that in the control group (allP value<0.05). Conclusion VATS could be widely applied in clinical practice with lowering operative trauma and reducing the degree of inflammatory reaction.
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 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.