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.
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.
ObjectiveTo analyze the clinical outcomes of extended thymectomy for myasthenia gravis (MG) patients under different surgical approaches, and to determine the factors affecting the prognosis of MG. MethodsThe MG patients who underwent extended thymectomy from January 2014 to March 2021 in our hospital were retrospectively collected. According to the surgical approach, they were divided into a subxiphoid group and an intercostal group, and the perioperative results and prognosis were compared between the two groups. A “good outcome” was defined as complete stable remission (CSR), pharmacological remission (PR) or minimal manifestations state (MMS); a “poor outcome” was defined as outcomes worse than MMS. Univariate and multivariate logistic regression analyses were performed to assess the factors associated with the good outcomes. ResultsA total of 187 MG patients were included in the study, including 82 males and 105 females, with a median age of 50 (36, 60) years. There were 134 patients in the intercostal group and 53 patients in the subxiphoid group. Compared with the intercostal group, although the operation time of the subxiphoid group was longer [200.0 (172.0, 232.0) min vs. 141.0 (118.0, 169.0) min, P<0.001], the intraoperative blood loss was less [10.0 (10.0, 20.0) mL vs. 20.0 (10.0, 50.0) mL, P<0.001], the postoperative hospital stay was shorter [3.0 (2.5, 4.0) d vs. 5.0 (3.0, 7.0) d, P<0.001], and the incidence of complications was lower [1 (1.9%) vs. 26 (19.4%), P=0.001]. A total of 159 (85.0%) patients were followed up for a median period of 46 (13, 99) months, with a good outcome rate of 90.6% and CSR rate of 33.3%. There were no statistical differences in PR, MMS or overall good outcome rates between the two groups (P>0.05). Multivariate logistic analysis showed that age≤50 years was an independent predictor for "good outcome" of MG patients. ConclusionExtended thymectomy via subxiphoid for MG is a safe, feasible and effective surgical approach.
[Abstract]This study reports a case of an 83-year-old female patient with systemic severe myasthenia gravis (American Myasthenia Foundation class Ⅳb) and multiple comorbidities who achieved minimal clinical status through preoperative alemtuzumab treatment (10 mg/kg, once per week for 4 consecutive weeks). After undergoing robotic thymectomy, the patient remained clinically stable in the early postoperative period until the 5th day after surgery when she developed dysphagia. Despite intravenous immunoglobulin and other therapeutic interventions, the patient’s myasthenic symptoms continued to worsen, coughing was impaired, respiratory insufficiency intensified, non-invasive ventilation support was required, and pulmonary infection occurred. Clinical symptoms further deteriorated, with diarrhea, urinary tract infection, and progressive respiratory tract infection. She was subsequently transferred to the intensive care unit for invasive mechanical ventilation and therapeutic plasmapheresis. These interventions failed to halt the disease progression, which eventually led to multiple organ dysfunction syndrome, and the patient died. Notably, among the other 12 patients receiving alemtuzumab treatment during the same period, none experienced such severe complications. This case is exceptional, and a direct causal relationship between alemtuzumab and mortality risk cannot be established at this time. Further research is needed to clarify perioperative immune management strategies.
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.
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.
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.
Myasthenia gravis (MG) is an autoimmune disease with indefinite pathogenesis. MG is closely related to thymic diseases, and thymectomy is an important way for MG treatment. However, there are some controversies regarding thymectomy, including indications, operation opportunities, operative procedures, surgical approaches, perioperative managements, and efficacy evaluations, etc. Therefore, based on the literature and the experience of Chinese experts, this consensus has been written after careful discussion and inquiry and 29 recommendations have been made, aiming to guide surgical treatment of MG and improve the clinical outcomes.
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.
