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 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.
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 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.
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 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.
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.
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.
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 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.