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 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.
ObjectiveTo assess the long-term efficacy of extended thymectomy in the treatment of myasthenia gravis(MG) and the factors influencing the results. MethodsWe retrospectively analyzed the clinical data of 45 MG patients who underwent extended thymectomy in our hospital from January 2005 through December 2010. There were 11males and 34 females at age of 32.6(16-50) years. The overall effective rate was calculated. Univariate analysis was used to analyze the risk factors including sex, age, Osserman classification, and pathological type for outcomes. ResultsAll the patients were followed up for a mean of 58.3 months(ranged from 36 to 108 months). The overall effective rate was 80.0%(36/45) and rose with the extention of time. Age(P=0.019), pathological type(P=0.024) of thymus and Osserman classification(P=0.043) of MG were the main factors influencing the outcomes of the treatment except sex(P=0.666). ConclusionExtended thymectomy in the treatment of MG has better outcomes. And the overall effective rate rises with the extention of time.
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 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.
ObjectiveTo investigate the perioperative safety of patients with myasthenia gravis who take high doses of oral corticosteroids. MethodsA retrospective analysis was conducted on the clinical data of patients with myasthenia gravis who received oral corticosteroids and underwent thoracoscopic thymectomy at the Department of Thoracic Surgery, the University of Hong Kong-Shenzhen Hospital from April 2013 to October 2019. Patients were divided into a high-dose steroid group and a medium-to-low dose steroid group based on the dosage of oral steroids, and the clinical data of the two groups were compared. ResultsA total of 102 patients were included, including 19 (18.62%) males and 83 (81.37%) females, with an average age of (32.25±9.83) years. All patients in both groups successfully completed the surgery without major intraoperative bleeding, conversion to open chest surgery, delayed extubation, severe infection, or perioperative death. The daily oral steroid dose for the high-dose steroid group was (35.81±4.29) mg, and for the medium-to-low dose steroid group it was (15.29±2.17) mg. There was no statistical difference in the operation time [(124.69±23.51) min vs. (117.89±21.46) min, P=0.172] and intraoperative blood loss [(21.19±3.48) mL vs. (20.56±3.41) mL, P=0.419] between the two groups. Postoperatively, 12 (11.76%) patients developed complications: one patient of myasthenic crisis (medium-to-low dose steroid group), which was improved after short-term respiratory support and intravenous immunoglobulin treatment; 11 patients of respiratory/swallowing difficulties (9 in the low-dose steroid group and 2 in the high-dose steroid group), which were improved after anticholinergic treatment to reduce oral secretions and sputum suction, and the patients were discharged smoothly. There was no statistical difference in the incidence of postoperative complications between the two groups (P=0.637). ConclusionOn the basis of good perioperative management, it is safe and feasible for patients with myasthenia gravis who take high dose of oral steroids to undergo thymectomy, and they have the same perioperative safety as patients with medium-to-low dose steroids.
Objective To compare three surgical treatments for mediastinal mass with myasthenia gravis. Methods Retrospective analysis was performed on the clinical data of 53 patients who underwent extended thymectomy between January 2010 and December 2017 in our hospital. There were 29 males and 24 females, aged 17-73 years. Patients were divided into three groups according to the surgical methods: a group A (video-assisted thoracoscopic surgery with the da Vinci robotic system, n=22), a group B (video-assisted thoracoscopic surgery, n=12) and a group C (median sternotomy, n=19). The gender distribution, age, intraoperative blood loss, operation time, postoperative extubation time, postoperative hospital stay, Osserman classification of myasthenia gravis, postoperative myasthenic remission rate, etc were compared in three groups. Results No perioperative death was observed in 53 patients. One patient in the group C suffered from postoperative myasthenic crisis and improved after active treatment. One patient with video-assisted thoracoscopic surgery was converted to median sternotomy due to the intraoperative injury of the left brachiocephalic vein. Compared with the group B and group C, the group A had shorter operation time, less intraoperative blood loss and drainage on the first postoperative day and fewer days of extubation. Postoperative hospital stay was less in the group A than that in the group C (P<0.05). The postoperative myasthenic remission rate was higher in the group A than that in the other two groups, but there was no statistical difference. Conclusion Because of the robot’s unique minimally invasive advantage, in this study, the outcome of patients with myasthenia gravis treated with Da Vinci robots and thymectomy is better than that of the remaining two groups in terms of perioperative outcomes and myasthenic remission rate. But long-term results and a large of number matching experiments are needed to confirm. However, it is undeniable that robotic surgery must be the future of the minimally invasive surgery.
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 role of T follicular helper cells (TFH) and their expression of α7 nicotinic acetylcholine receptor (α7nAChR) in patients with Myasthenia gravis (MG). Methods Fifteen MG patients who underwent surgical treatment in the Myasthenia Gravis Comprehensive Diagnosis and Treatment Center of Henan Provincial People's Hospital from June 2022 to June 2023 were selected as the MG group, including 7 males and 8 females, aged 12-30 years. Twelve healthy individuals who underwent partial thymectomy without obstruction during cardiac surgery in Fuwai Central China Cardiovascular Hospital from June 2022 to June 2023 were selected as the healthy control group, including 5 males and 7 females aged 20-35 years. Thymus single cell suspension was obtained by grinding the thymus tissue, and flow cytometry was used to detect the expression of α7nAChR in TFH cells. The thymus cell suspension was purified using density gradient centrifugation, followed by immunomagnetic bead separation to obtain CD4+T cells. CXCR5 antibody and coupled magnetic beads were added to isolate TFH cells. Real-time fluorescent polymerase chain reaction (RT-PCR) and Western blotting were performed to further investigate the expression of α7nAChR in TFH cells. Results Compared with the healthy control group, there was a significant decrease in α7nAChR levels within thymic TFH cells in the MG group (P<0.001), along with significantly decreased mRNA and protein expression levels of α7nAChR within these cells (P<0.01). Conclusion The findings suggest that there is a reduced expression of α7nAChR within thymic TFH cells in MG patients, leading to weakened immunosuppressive function which may indirectly contribute to disease onset and progression.
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.