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 (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.
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.
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.
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.
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 analyze the risk factors for post-thymectomy myasthenic crisis (PTMC) and prolonged mechanical ventilation, in myasthenia gravis patients who underwent extended thymectomy.
MethodsWe retrospectively analyzed the clinical data of 79 patients including 38 males and 41 females who experienced PTMC and required mechanical ventilation in Daping Hospital between June 2008 and November 2014. Single factor analysis and multivariate analysis were conducted.
ResultsMorbidity of PTMC was 20.6% (79/384). Result of single-factor analysis showed that postoperative pneumonia was one of the main reasons of prolonged mechanical ventilation (P < 0.05). Result of multiple-factor analysis showed that the operation time was positively correlated with mechanical ventilation time (P < 0.05). The risk factor of prolonged mechanical ventilation time in PTMC was not associated with sex, age, disease history, myasthenic crisis history, Osserman classification, dosage of pyridostigmine before and after the operation, surgical approach, bleeding volume, other therapies besides mechanical ventilation (P > 0.05).
ConclusionMechanical ventilation is one the main therapy of PTMC, operation time, and postoperative pneumonia are the main factors to prolong mechanical ventilation time. In order to decrease morbidity of PTMC and shorten mechanical ventilation time, the operation time should be controlled and pulmonary infection should be avoided.
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）
Myasthenia gravis is an autoimmune disorder characterized by impaired neuromuscular transmission. Thymectomy is one of the therapeutic options for acetylcholine receptor antibody-positive myasthenia gravis patients. The quality of perioperative care is directly associated with surgical safety and patient outcomes. However, there is currently a lack of specialized nursing consensus or guidelines specifically addressing the care of these patients domestically or internationally. To promote the standardization and normalization of perioperative nursing care for myasthenia gravis patients undergoing thymectomy and to ensure treatment efficacy, a panel of 57 experts from relevant fields was convened. Based on evidence-based medicine and clinical practice experience, discussions were held on various aspects including condition assessment, nutritional support, medication management, and airway care, resulting in a consensus with 18 final recommendations by using the Delphi method through two rounds of expert consultation. This consensus aims to provide a scientific reference for the perioperative nursing care of myasthenia gravis patients undergoing thymectomy.
