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.
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 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）
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.
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.
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.
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.
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.
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.