Myasthenia gravis(MG)is an autoimmune disorder that affects the neuromuscular junction.The primary pathology in MG involves the presence of autoantibodies to acetylcholine receptors(AChRs),which results in qualitative...Myasthenia gravis(MG)is an autoimmune disorder that affects the neuromuscular junction.The primary pathology in MG involves the presence of autoantibodies to acetylcholine receptors(AChRs),which results in qualitative and quantitative reductions in the availability of functional AChRs.Cardiac muscles are also affected,resulting in various perioperative cardiac complications.Antistriational antibodies are commonly reported in MG cases with cardiac involvement.In the presence of thymoma,the prevalence of cardiac manifestations in patients with MG increases to approximately 10%-15%.Cardiac involvement in MG may range from asymptomatic electrocardiogram changes to ventricular tachycardia,myocarditis,conduction disorders,heart failure,and sudden death.Increased incidence of atrial fibrillation,ventricular and supraventricular extra systoles,and prolonged QTc have also been reported in patients with MG.Clinicians should consider the evaluation of autonomic dysfunction and risk of cardiovascular disease in patients with MG.展开更多
This editorial aimed to consolidate the current evidence in literature on the association between myasthenia gravis(MG)and cardiac involvement,focusing on the impact of thymoma,antistriational antibodies,and late-onse...This editorial aimed to consolidate the current evidence in literature on the association between myasthenia gravis(MG)and cardiac involvement,focusing on the impact of thymoma,antistriational antibodies,and late-onset MG.Additionally,the study aimed to explore the influence of genetic differences among populations on the association with cardiac disease.We conducted a review of existing literature in PubMed and Google Scholar to find relevant studies on cardiac involvement in MG.We created search criteria using a combination of free text words,including MG,antistriational antibodies,thymectomy,cardiomyopathy,myocarditis,arrhythmias,autonomic dysfunction.Relevant articles published in English language were analyzed and incorporated.The findings indicate a strong association between thymoma,myasthenic crisis,antistriational antibodies,and late-onset MG with cardiac involvement.The study also revealed that genetic differences among populations influence the risk of cardiac disease and electrocardiography(ECG)abnormalities in MG patients.Autonomic dysfunctions altered cardiac autonomic response and increased susceptibility to arrhythmias and sudden cardiac death in MG patients.The study supports the significance of thymoma,antistriational antibodies,and late-onset MG as key factors associated with cardiac involvement in MG patients.It emphasizes the importance of ECG as the initial test in managing MG patients,particularly in the perioperative period,to identify and genetic testing if needed to address their cardiac risk effectively.展开更多
Background: Our study aimed to investigate the clinical value of platelet parameters and the number of Monocytes (MONO) in the peripheral blood of patients with Ocular Myasthenia Gravis (OMG). Methods: Fifty patients ...Background: Our study aimed to investigate the clinical value of platelet parameters and the number of Monocytes (MONO) in the peripheral blood of patients with Ocular Myasthenia Gravis (OMG). Methods: Fifty patients discharged from the Department of Neurology of the First People’s Hospital of Jingzhou City with the diagnosis of OMG from January 1, 2020, to December 31, 2023, were selected as the experimental group, and healthy medical examiners were selected as the control group during the same period. The differences in peripheral blood mononuclear cell count and platelet-related parameters between the two groups were compared. ROC curves were drawn to analyze the clinical value of the studied indexes in patients with OMG. Results: Patients with OMG had significantly higher monocyte counts and narrower platelet distributions compared with normal adults;further logistic regression analysis revealed that higher monocyte counts and narrower Platelet Distribution Width (PDW) may be independent influences on the occurrence of OMG (p Conclusion: Peripheral blood mononuclear cell count and PDW have some diagnostic value in OMG.展开更多
BACKGROUND Myasthenia gravis(MG)is an autoimmune disorder caused by neuromuscular junction failure characterized by muscle weakness and fatigability.We herein report a case of MG that received intravascular laser irra...BACKGROUND Myasthenia gravis(MG)is an autoimmune disorder caused by neuromuscular junction failure characterized by muscle weakness and fatigability.We herein report a case of MG that received intravascular laser irradiation of blood(ILIB)interventions and regained muscle power and better quality of life.To our knowledge,no previous study has investigated the benefits of ILIB treatment on patients with MG.We also evaluated the changes in brain perfusion scan and the MG activities of daily living(MG-ADL)and quantitative MG(QMG)scales.CASE SUMMARY A 59-year-old man presented to our outpatient hospital experiencing ptosis,diplopia,fibromyalgia,muscle fatigue,and fluctuating weakness in his limbs for 1 year.Based on his history,physical examination,and laboratory investigations,the final diagnosis was a flare-up of MG with poor endurance and muscle fatigue.The patient agreed to receive ILIB.Brain single-photon emission computed tomography(SPECT)was performed both before and after ILIB therapy.After receiving three courses of ILIB,the brain SPECT images showed greatly increased perfusion of the frontal lobe and anterior cingulate gyri.The patient’s MG-ADL scale score decreased markedly from 17/24 to 3/24.The QMG scale score also decreased remarkably from 32/39 to 9/39.The symptoms of MG became barely detectable and the patient was able to perform his activities of daily living and regain muscle power.CONCLUSION ILIB might have beneficial effects on MG,and brain SPECT images provided direct evidence of a positive correlation between ILIB and clinical performance.展开更多
Objective To explore effective therapeutic method for the treatment of ocular myasthenia gravis. Methods Sixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī （大椎 GV 14...Objective To explore effective therapeutic method for the treatment of ocular myasthenia gravis. Methods Sixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī （大椎 GV 14）, Bǎihuì （百会 GV 20）, Shàngxīng （上星 GV 23） of Governor Vessel, and its crossing points with Yinqiao and Yangqiao Meridian, such as Jīngmíng （睛明 BL 1）, shēnmài （申脉 BL 62）, Dìcāng （地仓 ST 4）, Jūliáo （巨髎 ST 3）, Chéngqì （承泣 ST 1 ）, etc. Besides, ginger-partition moxibustion was applied at Yángbái （阳白 GB 14） and Sìbái （四白 ST 2）. The plum-blossom needle was applied at upper limb distribution of lung channel of hand taiyin, heart channel of hand shaoyin and pericardium channel of hand jueyin. The patients’ clinical symptoms, potential degradation percentages of repetitive nerve stimulation （RNS） in low frequency were observed. Results 8 cases were clinically cured, 20 cases were basically cured, 12 cases were remarkably effective, 15 cases were effective and 5 cases were failed. The total effective rate was 91.7%. Compared with before the treatment, the potential degradation percentages of repetitive nerve stimulation in low frequency were lowered [（19.44±6.28）% vs （8.78±3.12）%, （P0.05）]. Conclusion Acupuncture and moxibustion at extra-meridian and collateral for ocular myasthenia gravis could achieve positive efficacy.展开更多
Objective： To investigate the relationship among the latest WHO classification of thymoma, myasthenia gravis （MG） and clinical stages. Methods： To review the pathological sections of 74 patients with thymoma from ...Objective： To investigate the relationship among the latest WHO classification of thymoma, myasthenia gravis （MG） and clinical stages. Methods： To review the pathological sections of 74 patients with thymoma from 1980-2004 using WHO classification （1999）, the statistical software was used to analyze the relationship among the WHO classification, MG and clinical stages. Results： （1） Two cases of type A, 23 cases of type AB, 4 cases of type B1, 27 cases of type B2, 16 cases of type B3 and 2 cases of type C were classified. Type B2 more likely accompanied MG （P〈0.05）, while none with MG occurred for type C. （2） One patient was in stage Ⅰ, 30 were in stage Ⅱ, 38 were in stage Ⅲ, and 5 were in stage Ⅳ. The latest histologic classification was significantly correlated with Masaoka stages （P〈0.01）. Conclusion： The latest WHO classification was correlated with occurrence of MG and finely reflected clinical stage. It can also evaluate the prognosis of patients.展开更多
Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junctionthat has higher incidence in younger women than men, which could be related todifferences in sex hormones physiology and immune system function...Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junctionthat has higher incidence in younger women than men, which could be related todifferences in sex hormones physiology and immune system functioning betweenmales and females. MG can first present during pregnancy and variably affectpregnancy, labor, and postpartum period. In this paper, we had an updatedoverview on our understanding about MG presentation and its effect onpregnancy and vice versa, therapeutic options for MG pregnant women,management of pregnancy or labor complications in MG patients, and finally fetaland neonatal considerations in MG pregnant women. A multidisciplinaryapproach, involving obstetricians/gynecologists, neurologists, and anesthesiologists,plays a pivotal role in improving the clinical outcomes in both MGmothers and their infants during pregnancy, delivery and postpartum.展开更多
Myasthenia gravis （MG） is an autoimmune neuromuscular junction disease mediated by antibodies against the acetylcholine receptor （AChR）. The etiology and immunopathogenesis of MG remain unclear. Recent research ha...Myasthenia gravis （MG） is an autoimmune neuromuscular junction disease mediated by antibodies against the acetylcholine receptor （AChR）. The etiology and immunopathogenesis of MG remain unclear. Recent research has shown the involvement of autoantibodies, lymphocytes, cytokines and chemokines, in the pathogenesis of MG. Systematic factors are also demonstrated, such as inheritance and endocrine. This review indicates the research development in immunopathogenesis of MG.展开更多
Complete resection could be achieved in virtually all myasthenic patients with Masaoka stage I and II thymoma us- ing the trans-sternal technique. Whether this is appropriate for minimally invasive approach is not yet...Complete resection could be achieved in virtually all myasthenic patients with Masaoka stage I and II thymoma us- ing the trans-sternal technique. Whether this is appropriate for minimally invasive approach is not yet clear. We evalu- ated the feasibility of complete video-assisted thoracoscopic surgery （VATS） thymectomy for the treatment of Ma- saoka stage I and Ⅱ thymoma-associated myasthenia gravis, compared to conventional trans-sternal thymectomy. We summarized 33 patients with Masaoka stage I and II thymoma-associated myasthenia gravis between April 2006 and September 2011. Of these, 15 patients underwent right-sided complete VATS （the VATS group） by us- ing adjuvant pneuomomediastinum, comparing with 18 patients using the trans-sternal approach （the T3b group）. No intraoperative death was found and no VATS case required conversion to median sternotomy. Significant differences between the two groups regarding duration of surgery and volume of intraoperative blood loss （P = 0.001 and P 〈 0.001, respectively） were observed. Postoperative morbidities were 26.7% and 33.3% for the VATS and T3b groups, respectively. All 33 patients were followed up for 12 to 61 months in the study. The cumulative probabilities of reaching complete stable remission and effective rate were 26.7% （4/15） and 93.3% （14/15） in the VATS group, which had a significantly higher complete stable remission and effective rate than those in the T3b group （P = 0.026 and P = 0.000, respectively）. We conclude that VATS thymectomy utilizing adjuvant pneuomo- mediastinum for the treatment of stage I and II thymoma-associated myasthenia gravis is technically feasible but deserves further investigation in a large series with long-term follow-up.展开更多
Thymoma is the most common mediastinal tumor. They have varied presentation ranging from asymptomatic incidental mediastinal masses to locally extensive tumor with compressive symptoms and distant metastases. They hav...Thymoma is the most common mediastinal tumor. They have varied presentation ranging from asymptomatic incidental mediastinal masses to locally extensive tumor with compressive symptoms and distant metastases. They have frequent association with various paraneoplastic syndromes(PNS). The most common PNS associated with thymoma is myasthenia gravis(MG). Patients of thymoma with MG have a favourable outcome due to early disclosure of the disease. Histologically they are classified into five subtypes and MasaokaKoga staging system is used for staging. Surgery, chemotherapy and radiotherapy play an important role along with anti-myasthenia drugs. This review would like to highlight the association of thymoma with MG and associated clinical and therapeutic issues.展开更多
Myasthenia gravis(MG) is a prototypical antibody-mediated neurological autoimmune disease with the involvement of humoral immune responses in its pathogenesis. T follicular helper(Tfh) cells have been implicated in ma...Myasthenia gravis(MG) is a prototypical antibody-mediated neurological autoimmune disease with the involvement of humoral immune responses in its pathogenesis. T follicular helper(Tfh) cells have been implicated in many autoimmune diseases. However, whether and how Tfh cells are involved in MG remain unclear.Here, we established and studied a widely-used and approved animal model of human MG, the rat model with acetylcholine receptor alpha(AChRa) subunit(RAChR97–116)-induced experimental autoimmune myasthenia gravis(EAMG). This model presented mild bodyweight loss 10 days after the first immunization(representing the early stage of disease) and more obvious clinical manifestations and body-weight loss 7 days after the second immunization(representing the late stage of disease). AChR-specific pre-Tfh cells and mature Tfh cells were detected in these two stages, respectively. In cocultures of Tfh cells and B cells, the number of IgG2 bsecreting B cells and the level of anti-AChR antibodies in the supernatant were higher in the cultures containing EAMG-derived Tfh cells. In immunohistochemistry and immunofluorescence assays, a substantial number of CD4^+/Bcl-6^+ T cells and a greater number of larger germinal centers were observed in lymph node tissues resected from EAMG rats. Based on these results, wehypothesize that an AChR-specific Tfh cell-mediated humoral immune response contributes to the development of EAMG.展开更多
Objective To analyze the relationship between tumor necrosis factor-alpha (TNFα) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFα in peripheral blood mononuclear ce...Objective To analyze the relationship between tumor necrosis factor-alpha (TNFα) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFα in peripheral blood mononuclear cells (PBMC) in MG patients. Methods A biallelic polymorphism at position -308 in the promoter of TNFα gene was screened by PCR amplification and NcoI recognition site. One hundred and twenty-three MG cases and 115 healthy controls were included in this study. MG patients were classified to different groups according to clinical type, age at onset, and sex respectively. PBMC were isolated from 20 patients and 20 healthy controls, and then cultured in the presence or absence of phytohemag- glutinin (PHA) and acetycholine receptors (AchR). The supernatants were harvested after incubation and stored until TNFα was assayed by enzyme-linked immunosorbent assay. Results The frequency of TNFα-308 allele 2 (A) was found significantly increase in MG patients and showed a trend especially in late onset (≥ 40 years) and male patients (P < 0.05). The allele A had no relationship with thymic pathogenesis in MG patients. But frequency of allele A was significantly higher in general type than in ocular type (P < 0.05). MG patients had a higher inducible level of TNFα by PHA and AchR, and could be down regulated after treatment. Conclusion Polymorphism in TNFα gene promoter -308 is associated with onset of MG. The microsatellite allele TNFα2 confer risk for the development of MG in Chinese patients. MG patients have a higher inducible level of TNFα.展开更多
Myasthenia gravis is an acquired,humoral immunity-mediated autoimmune disease characterized by the production of autoantibodies that impair synaptic transmission at the neuromuscular junction.The intervention-mediated...Myasthenia gravis is an acquired,humoral immunity-mediated autoimmune disease characterized by the production of autoantibodies that impair synaptic transmission at the neuromuscular junction.The intervention-mediated clearance of immunoglobulin G(IgG)was shown to be effective in controlling the progression of the disease.The neonatal Fc receptor(FcRn)plays a key role in prolonging the serum half-life of IgG.Antagonizing FcRn to prevent its binding to IgG can accelerate the catabolism of the latter,resulting in decreased levels of IgG,including pathogenic autoantibodies,thereby achieving a therapeutic effect.In this review,we detail the substantial research progress,both basic and clinical,relating to the use of FcRn inhibitors in the treatment of myasthenia gravis.展开更多
Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodie...Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.展开更多
OBJECTIVE: To investigate the effect of Jianpiyiqi granule, prepared according to a Traditional Chinese Medicine(TCM) formula, on the ocular type of myasthenia gravis(MG) caused by a defect in synaptic transmission at...OBJECTIVE: To investigate the effect of Jianpiyiqi granule, prepared according to a Traditional Chinese Medicine(TCM) formula, on the ocular type of myasthenia gravis(MG) caused by a defect in synaptic transmission at the neuromuscular junction.METHODS: A total of 155 children with ocular MG were recruited from January 2008 to January 2015.All individuals received ineffective glucocorticoid treatment prior to admission, and were given Jianpiyiqi granules(two doses per day) for 12 months.Plasma levels of acetylcholine receptor autoantibodies(ACh R-Ab), cytokines, immune parameters and clinical score were analyzed.RESULTS: After intervention with Jianpiyiqi granule for 12 months, the levels of cytokines [including interleukin(IL)-6, IL-12, IL-17], immune parameters[including immunoglobulin G(Ig G), Ig M, free triiodothyronine, free thyroxine] and ACh R-Ab were sig-nificantly decreased(P = 0.042, P = 0.049, P = 0.011,P = 0.02, P = 0.04, P = 0.03, P = 0.01; F = 21.60, P =0.000). In contrast, IL-4 levels were significantly increased(P = 0.049). The absolute clinical score after treatment declined significantly compared with before treatment(F = 33.24, P = 0.000). The effective cure rate and the total effective rate gradually increased to a maximum of 149(96.2%) and 151(97.4%) in MG patients after 12 months of treatment, respectively.CONCLUSION: The Jianpiyiqi granule treatment lowered ACh R-Ab levels and improved cytokine and other immune parameter levels, which suggests that the granule could be an ancillary treatment for ocular MG in children caused by a defect in synaptic transmission at the neuromuscular junction.展开更多
Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the m...Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine receptor.Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse.This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis.The review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional dynamics.The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.展开更多
An important problem in management of the case with myasthenia gravis (MG) is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a ...An important problem in management of the case with myasthenia gravis (MG) is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a case of MG exacerbation and diarrhea associated with erythromycin treatment.展开更多
There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis （MG）. This systematic review was undertaken to determine whether thymectomy was effective in...There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis （MG）. This systematic review was undertaken to determine whether thymectomy was effective in non-thymomatous MG. We retrieved articles published between January 1980 and September 2013. Sixteen cohort studies were included. Given the considerable het- erogeneity, we used a descriptive method instead of statistical synthesis. The median relative rates （RRs） and their interquartile ranges were used to estimate the magnitude of benefit. Compared to conserva- tively treated MG patients, thymectomized patients had higher survival, clinical remission, pharma- cologic remission and improvement rates, and RRs were 1.07 （1.01-1.17）, 1.83 （0.82-2.99）, 1.55 （1.22-1.95） and 1 （1.00-1.09）, respectively. Subgroup analyses showed that patients with moderate to severe generalized MG benefited more from thymeetomy, with RRs of survival and pharmacologic re- mission increasing to 1.35 （1.24-1.49） and 2.68 （1.73-4.17）, respectively. These results suggested that thymectomy might be an effective procedure in non-thymomatous MG patients. The patients with mod- erate to severe generalized MG might benefit more. Taking into account the poor methodological qual- ity of present studies, more well-designed prospective randomized controlled trials （RCTs） are still re- quired to reach uneouivocal conclusion.展开更多
To investigate the underlying mechanism of the exacerbation of myasthenia gravis by aminoglycoside antibiotics. C57/BL6 mice were immunized with acetylcholine receptor (AChR), extracted from electric organ of Narcine ...To investigate the underlying mechanism of the exacerbation of myasthenia gravis by aminoglycoside antibiotics. C57/BL6 mice were immunized with acetylcholine receptor (AChR), extracted from electric organ of Narcine timilei according to Xu Haopeng's methods, in complete Fruend's adjuvant (CFA) to establish experimental autoimmune myasthenia gravis (EAMG). EAMG mice were divided randomly into 5 groups: MG group, NS group and three antibiotics groups. The clinical symptom scores of mice were evaluated on d7 after the last immunization and d14 of antibiotics treatment. Repetitive nerve stimulation (RNS) was performed and the levels of anti-AChR antibody (AChR-Ab) were tested at the same time. The mean clinical symptom grades of gentamycin group (1.312, 2.067), amikacin group (1.111, 1.889) and etimicin group (1.263, 1.632) were significantly higher than those of MG group (1.000, 1.200) (P<0.05). The positive rates of RNS of three antibiotics groups were 69.23 %, 58.82 % and 63.16 % respectively, which were significantly higher than those of MG group and NS group (40.00 %, 40.00 %, P<0.05). The AChR-Ab level in serum and the expression of AChR on neuromuscular junction (NMJ) of mice in three antibiotics groups were also higher than those of MG group. Our results indicated that aminoglycoside antibiotics could aggravate the symptom of myasthenia gravis. The exacerbation of myasthenia gravis by these antibiotics probably involves competitively restraining the release of acetylcholine from presynaptic membrane, impairing the depolarization of postsynaptic membrane, depressing the irritability of myocyte membrane around the end-plate membrane and consequently leading to the blockade of neuromuscular junction.展开更多
Objective: To investigate the expression of both thymic regulatory T cells (CD4+CD25+Foxp3+cells, Treg) and thymic stromal lymphopoietin (TSLP) in thymomas accompanying myasthenia gravis. Methods: We used immunohistoc...Objective: To investigate the expression of both thymic regulatory T cells (CD4+CD25+Foxp3+cells, Treg) and thymic stromal lymphopoietin (TSLP) in thymomas accompanying myasthenia gravis. Methods: We used immunohistochemistry and real-time reverse trancription polymerase chain reaction (real-time RT-PCR) techniques to determine Foxp3+ Treg counts and the expression levels of Foxp3 mRNA and TSLP mRNA in thymomas of 23 MG patients and thymuses of 4 healthy controls. Results: The CD4+ Foxp3+ nTreg (natural regulatory T cells) counts in thymomas were significantly lower than those in normal thymuses (P<0.01), and the expression levels of Foxp3 mRNA and TSLP mRNA were also lower in thymomas(P<0.01). Among the thymoma types, type B1 thymoma had the highest Foxp3+ nTreg count and standard values of Foxp3 mRNA and TSLP mRNA. There was a strong positive correlation between the mRNA transcriptional levels of Foxp3 and TSLP. Conclusion: The insufficient expression of Foxp3 in thymoma, which may be caused by decreased transcription of TSLP, may result in the reduction of Tregs and cause autoimmune disorders.展开更多
文摘Myasthenia gravis(MG)is an autoimmune disorder that affects the neuromuscular junction.The primary pathology in MG involves the presence of autoantibodies to acetylcholine receptors(AChRs),which results in qualitative and quantitative reductions in the availability of functional AChRs.Cardiac muscles are also affected,resulting in various perioperative cardiac complications.Antistriational antibodies are commonly reported in MG cases with cardiac involvement.In the presence of thymoma,the prevalence of cardiac manifestations in patients with MG increases to approximately 10%-15%.Cardiac involvement in MG may range from asymptomatic electrocardiogram changes to ventricular tachycardia,myocarditis,conduction disorders,heart failure,and sudden death.Increased incidence of atrial fibrillation,ventricular and supraventricular extra systoles,and prolonged QTc have also been reported in patients with MG.Clinicians should consider the evaluation of autonomic dysfunction and risk of cardiovascular disease in patients with MG.
文摘This editorial aimed to consolidate the current evidence in literature on the association between myasthenia gravis(MG)and cardiac involvement,focusing on the impact of thymoma,antistriational antibodies,and late-onset MG.Additionally,the study aimed to explore the influence of genetic differences among populations on the association with cardiac disease.We conducted a review of existing literature in PubMed and Google Scholar to find relevant studies on cardiac involvement in MG.We created search criteria using a combination of free text words,including MG,antistriational antibodies,thymectomy,cardiomyopathy,myocarditis,arrhythmias,autonomic dysfunction.Relevant articles published in English language were analyzed and incorporated.The findings indicate a strong association between thymoma,myasthenic crisis,antistriational antibodies,and late-onset MG with cardiac involvement.The study also revealed that genetic differences among populations influence the risk of cardiac disease and electrocardiography(ECG)abnormalities in MG patients.Autonomic dysfunctions altered cardiac autonomic response and increased susceptibility to arrhythmias and sudden cardiac death in MG patients.The study supports the significance of thymoma,antistriational antibodies,and late-onset MG as key factors associated with cardiac involvement in MG patients.It emphasizes the importance of ECG as the initial test in managing MG patients,particularly in the perioperative period,to identify and genetic testing if needed to address their cardiac risk effectively.
文摘Background: Our study aimed to investigate the clinical value of platelet parameters and the number of Monocytes (MONO) in the peripheral blood of patients with Ocular Myasthenia Gravis (OMG). Methods: Fifty patients discharged from the Department of Neurology of the First People’s Hospital of Jingzhou City with the diagnosis of OMG from January 1, 2020, to December 31, 2023, were selected as the experimental group, and healthy medical examiners were selected as the control group during the same period. The differences in peripheral blood mononuclear cell count and platelet-related parameters between the two groups were compared. ROC curves were drawn to analyze the clinical value of the studied indexes in patients with OMG. Results: Patients with OMG had significantly higher monocyte counts and narrower platelet distributions compared with normal adults;further logistic regression analysis revealed that higher monocyte counts and narrower Platelet Distribution Width (PDW) may be independent influences on the occurrence of OMG (p Conclusion: Peripheral blood mononuclear cell count and PDW have some diagnostic value in OMG.
文摘BACKGROUND Myasthenia gravis(MG)is an autoimmune disorder caused by neuromuscular junction failure characterized by muscle weakness and fatigability.We herein report a case of MG that received intravascular laser irradiation of blood(ILIB)interventions and regained muscle power and better quality of life.To our knowledge,no previous study has investigated the benefits of ILIB treatment on patients with MG.We also evaluated the changes in brain perfusion scan and the MG activities of daily living(MG-ADL)and quantitative MG(QMG)scales.CASE SUMMARY A 59-year-old man presented to our outpatient hospital experiencing ptosis,diplopia,fibromyalgia,muscle fatigue,and fluctuating weakness in his limbs for 1 year.Based on his history,physical examination,and laboratory investigations,the final diagnosis was a flare-up of MG with poor endurance and muscle fatigue.The patient agreed to receive ILIB.Brain single-photon emission computed tomography(SPECT)was performed both before and after ILIB therapy.After receiving three courses of ILIB,the brain SPECT images showed greatly increased perfusion of the frontal lobe and anterior cingulate gyri.The patient’s MG-ADL scale score decreased markedly from 17/24 to 3/24.The QMG scale score also decreased remarkably from 32/39 to 9/39.The symptoms of MG became barely detectable and the patient was able to perform his activities of daily living and regain muscle power.CONCLUSION ILIB might have beneficial effects on MG,and brain SPECT images provided direct evidence of a positive correlation between ILIB and clinical performance.
文摘Objective To explore effective therapeutic method for the treatment of ocular myasthenia gravis. Methods Sixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī （大椎 GV 14）, Bǎihuì （百会 GV 20）, Shàngxīng （上星 GV 23） of Governor Vessel, and its crossing points with Yinqiao and Yangqiao Meridian, such as Jīngmíng （睛明 BL 1）, shēnmài （申脉 BL 62）, Dìcāng （地仓 ST 4）, Jūliáo （巨髎 ST 3）, Chéngqì （承泣 ST 1 ）, etc. Besides, ginger-partition moxibustion was applied at Yángbái （阳白 GB 14） and Sìbái （四白 ST 2）. The plum-blossom needle was applied at upper limb distribution of lung channel of hand taiyin, heart channel of hand shaoyin and pericardium channel of hand jueyin. The patients’ clinical symptoms, potential degradation percentages of repetitive nerve stimulation （RNS） in low frequency were observed. Results 8 cases were clinically cured, 20 cases were basically cured, 12 cases were remarkably effective, 15 cases were effective and 5 cases were failed. The total effective rate was 91.7%. Compared with before the treatment, the potential degradation percentages of repetitive nerve stimulation in low frequency were lowered [（19.44±6.28）% vs （8.78±3.12）%, （P0.05）]. Conclusion Acupuncture and moxibustion at extra-meridian and collateral for ocular myasthenia gravis could achieve positive efficacy.
文摘Objective： To investigate the relationship among the latest WHO classification of thymoma, myasthenia gravis （MG） and clinical stages. Methods： To review the pathological sections of 74 patients with thymoma from 1980-2004 using WHO classification （1999）, the statistical software was used to analyze the relationship among the WHO classification, MG and clinical stages. Results： （1） Two cases of type A, 23 cases of type AB, 4 cases of type B1, 27 cases of type B2, 16 cases of type B3 and 2 cases of type C were classified. Type B2 more likely accompanied MG （P〈0.05）, while none with MG occurred for type C. （2） One patient was in stage Ⅰ, 30 were in stage Ⅱ, 38 were in stage Ⅲ, and 5 were in stage Ⅳ. The latest histologic classification was significantly correlated with Masaoka stages （P〈0.01）. Conclusion： The latest WHO classification was correlated with occurrence of MG and finely reflected clinical stage. It can also evaluate the prognosis of patients.
文摘Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junctionthat has higher incidence in younger women than men, which could be related todifferences in sex hormones physiology and immune system functioning betweenmales and females. MG can first present during pregnancy and variably affectpregnancy, labor, and postpartum period. In this paper, we had an updatedoverview on our understanding about MG presentation and its effect onpregnancy and vice versa, therapeutic options for MG pregnant women,management of pregnancy or labor complications in MG patients, and finally fetaland neonatal considerations in MG pregnant women. A multidisciplinaryapproach, involving obstetricians/gynecologists, neurologists, and anesthesiologists,plays a pivotal role in improving the clinical outcomes in both MGmothers and their infants during pregnancy, delivery and postpartum.
文摘Myasthenia gravis （MG） is an autoimmune neuromuscular junction disease mediated by antibodies against the acetylcholine receptor （AChR）. The etiology and immunopathogenesis of MG remain unclear. Recent research has shown the involvement of autoantibodies, lymphocytes, cytokines and chemokines, in the pathogenesis of MG. Systematic factors are also demonstrated, such as inheritance and endocrine. This review indicates the research development in immunopathogenesis of MG.
文摘Complete resection could be achieved in virtually all myasthenic patients with Masaoka stage I and II thymoma us- ing the trans-sternal technique. Whether this is appropriate for minimally invasive approach is not yet clear. We evalu- ated the feasibility of complete video-assisted thoracoscopic surgery （VATS） thymectomy for the treatment of Ma- saoka stage I and Ⅱ thymoma-associated myasthenia gravis, compared to conventional trans-sternal thymectomy. We summarized 33 patients with Masaoka stage I and II thymoma-associated myasthenia gravis between April 2006 and September 2011. Of these, 15 patients underwent right-sided complete VATS （the VATS group） by us- ing adjuvant pneuomomediastinum, comparing with 18 patients using the trans-sternal approach （the T3b group）. No intraoperative death was found and no VATS case required conversion to median sternotomy. Significant differences between the two groups regarding duration of surgery and volume of intraoperative blood loss （P = 0.001 and P 〈 0.001, respectively） were observed. Postoperative morbidities were 26.7% and 33.3% for the VATS and T3b groups, respectively. All 33 patients were followed up for 12 to 61 months in the study. The cumulative probabilities of reaching complete stable remission and effective rate were 26.7% （4/15） and 93.3% （14/15） in the VATS group, which had a significantly higher complete stable remission and effective rate than those in the T3b group （P = 0.026 and P = 0.000, respectively）. We conclude that VATS thymectomy utilizing adjuvant pneuomo- mediastinum for the treatment of stage I and II thymoma-associated myasthenia gravis is technically feasible but deserves further investigation in a large series with long-term follow-up.
文摘Thymoma is the most common mediastinal tumor. They have varied presentation ranging from asymptomatic incidental mediastinal masses to locally extensive tumor with compressive symptoms and distant metastases. They have frequent association with various paraneoplastic syndromes(PNS). The most common PNS associated with thymoma is myasthenia gravis(MG). Patients of thymoma with MG have a favourable outcome due to early disclosure of the disease. Histologically they are classified into five subtypes and MasaokaKoga staging system is used for staging. Surgery, chemotherapy and radiotherapy play an important role along with anti-myasthenia drugs. This review would like to highlight the association of thymoma with MG and associated clinical and therapeutic issues.
基金supported by the National Natural Science Foundation of China(81000536,81471227,31371079,31671112 and 81430035)the China Postdoctoral Science Foundation(20100471094)+3 种基金the Returned Overseas Scholars Foundation of the Natural Science Foundation of Heilongjiang Province,China(LC2015029,QC2015022)the Science and Technology Research Project of the Education Department of Heilongjiang Province,China(12541z008)the Heilongjiang Province Postdoctoral Science Foundation(LBH-Q131111)the Open Topic of Key Laboratory of Neurobiology,General Colleges and Universities in Heilongjiang Province,China(2013HLJKLNT-05)
文摘Myasthenia gravis(MG) is a prototypical antibody-mediated neurological autoimmune disease with the involvement of humoral immune responses in its pathogenesis. T follicular helper(Tfh) cells have been implicated in many autoimmune diseases. However, whether and how Tfh cells are involved in MG remain unclear.Here, we established and studied a widely-used and approved animal model of human MG, the rat model with acetylcholine receptor alpha(AChRa) subunit(RAChR97–116)-induced experimental autoimmune myasthenia gravis(EAMG). This model presented mild bodyweight loss 10 days after the first immunization(representing the early stage of disease) and more obvious clinical manifestations and body-weight loss 7 days after the second immunization(representing the late stage of disease). AChR-specific pre-Tfh cells and mature Tfh cells were detected in these two stages, respectively. In cocultures of Tfh cells and B cells, the number of IgG2 bsecreting B cells and the level of anti-AChR antibodies in the supernatant were higher in the cultures containing EAMG-derived Tfh cells. In immunohistochemistry and immunofluorescence assays, a substantial number of CD4^+/Bcl-6^+ T cells and a greater number of larger germinal centers were observed in lymph node tissues resected from EAMG rats. Based on these results, wehypothesize that an AChR-specific Tfh cell-mediated humoral immune response contributes to the development of EAMG.
文摘Objective To analyze the relationship between tumor necrosis factor-alpha (TNFα) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFα in peripheral blood mononuclear cells (PBMC) in MG patients. Methods A biallelic polymorphism at position -308 in the promoter of TNFα gene was screened by PCR amplification and NcoI recognition site. One hundred and twenty-three MG cases and 115 healthy controls were included in this study. MG patients were classified to different groups according to clinical type, age at onset, and sex respectively. PBMC were isolated from 20 patients and 20 healthy controls, and then cultured in the presence or absence of phytohemag- glutinin (PHA) and acetycholine receptors (AchR). The supernatants were harvested after incubation and stored until TNFα was assayed by enzyme-linked immunosorbent assay. Results The frequency of TNFα-308 allele 2 (A) was found significantly increase in MG patients and showed a trend especially in late onset (≥ 40 years) and male patients (P < 0.05). The allele A had no relationship with thymic pathogenesis in MG patients. But frequency of allele A was significantly higher in general type than in ocular type (P < 0.05). MG patients had a higher inducible level of TNFα by PHA and AchR, and could be down regulated after treatment. Conclusion Polymorphism in TNFα gene promoter -308 is associated with onset of MG. The microsatellite allele TNFα2 confer risk for the development of MG in Chinese patients. MG patients have a higher inducible level of TNFα.
基金supported by the National Natural Science Foundation of China,No.U1604181the Joint Project of Medical Science and Technology Research Program of Henon Province,No.LHGJ20190078+1 种基金Henan Medical Education Research Project,No.Wjlx2020531Henan Province Key R&D and Promotion Special Project(Science and Technology Tackle),No.212102310834(all to JW)。
文摘Myasthenia gravis is an acquired,humoral immunity-mediated autoimmune disease characterized by the production of autoantibodies that impair synaptic transmission at the neuromuscular junction.The intervention-mediated clearance of immunoglobulin G(IgG)was shown to be effective in controlling the progression of the disease.The neonatal Fc receptor(FcRn)plays a key role in prolonging the serum half-life of IgG.Antagonizing FcRn to prevent its binding to IgG can accelerate the catabolism of the latter,resulting in decreased levels of IgG,including pathogenic autoantibodies,thereby achieving a therapeutic effect.In this review,we detail the substantial research progress,both basic and clinical,relating to the use of FcRn inhibitors in the treatment of myasthenia gravis.
基金supported by the National Natural Science Foundation of China (31170864 and 81171183)
文摘Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.
基金Supported by Shijiazhuang Science and Technology Bureau Foundation(No.131460613)Science and Technology Agency Foundation of Hebei Province(grant no.14277758D)+1 种基金Natural Science Foundation of Hebei Province(No.H2015106020)Key Project of Hebei Provincial Administration of Traditional Chinese Medicine(No.2014221)
文摘OBJECTIVE: To investigate the effect of Jianpiyiqi granule, prepared according to a Traditional Chinese Medicine(TCM) formula, on the ocular type of myasthenia gravis(MG) caused by a defect in synaptic transmission at the neuromuscular junction.METHODS: A total of 155 children with ocular MG were recruited from January 2008 to January 2015.All individuals received ineffective glucocorticoid treatment prior to admission, and were given Jianpiyiqi granules(two doses per day) for 12 months.Plasma levels of acetylcholine receptor autoantibodies(ACh R-Ab), cytokines, immune parameters and clinical score were analyzed.RESULTS: After intervention with Jianpiyiqi granule for 12 months, the levels of cytokines [including interleukin(IL)-6, IL-12, IL-17], immune parameters[including immunoglobulin G(Ig G), Ig M, free triiodothyronine, free thyroxine] and ACh R-Ab were sig-nificantly decreased(P = 0.042, P = 0.049, P = 0.011,P = 0.02, P = 0.04, P = 0.03, P = 0.01; F = 21.60, P =0.000). In contrast, IL-4 levels were significantly increased(P = 0.049). The absolute clinical score after treatment declined significantly compared with before treatment(F = 33.24, P = 0.000). The effective cure rate and the total effective rate gradually increased to a maximum of 149(96.2%) and 151(97.4%) in MG patients after 12 months of treatment, respectively.CONCLUSION: The Jianpiyiqi granule treatment lowered ACh R-Ab levels and improved cytokine and other immune parameter levels, which suggests that the granule could be an ancillary treatment for ocular MG in children caused by a defect in synaptic transmission at the neuromuscular junction.
文摘Myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary muscles.The classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine receptor.Other variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral synapse.This review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia gravis.The review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional dynamics.The hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.
文摘An important problem in management of the case with myasthenia gravis (MG) is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a case of MG exacerbation and diarrhea associated with erythromycin treatment.
基金supported by grants from the National Nature Science Foundation of China(Nos.81271406 and 81000537)Program for New Century Excellent Talents in University of China(No.NCET-09-0394)
文摘There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis （MG）. This systematic review was undertaken to determine whether thymectomy was effective in non-thymomatous MG. We retrieved articles published between January 1980 and September 2013. Sixteen cohort studies were included. Given the considerable het- erogeneity, we used a descriptive method instead of statistical synthesis. The median relative rates （RRs） and their interquartile ranges were used to estimate the magnitude of benefit. Compared to conserva- tively treated MG patients, thymectomized patients had higher survival, clinical remission, pharma- cologic remission and improvement rates, and RRs were 1.07 （1.01-1.17）, 1.83 （0.82-2.99）, 1.55 （1.22-1.95） and 1 （1.00-1.09）, respectively. Subgroup analyses showed that patients with moderate to severe generalized MG benefited more from thymeetomy, with RRs of survival and pharmacologic re- mission increasing to 1.35 （1.24-1.49） and 2.68 （1.73-4.17）, respectively. These results suggested that thymectomy might be an effective procedure in non-thymomatous MG patients. The patients with mod- erate to severe generalized MG might benefit more. Taking into account the poor methodological qual- ity of present studies, more well-designed prospective randomized controlled trials （RCTs） are still re- quired to reach uneouivocal conclusion.
文摘To investigate the underlying mechanism of the exacerbation of myasthenia gravis by aminoglycoside antibiotics. C57/BL6 mice were immunized with acetylcholine receptor (AChR), extracted from electric organ of Narcine timilei according to Xu Haopeng's methods, in complete Fruend's adjuvant (CFA) to establish experimental autoimmune myasthenia gravis (EAMG). EAMG mice were divided randomly into 5 groups: MG group, NS group and three antibiotics groups. The clinical symptom scores of mice were evaluated on d7 after the last immunization and d14 of antibiotics treatment. Repetitive nerve stimulation (RNS) was performed and the levels of anti-AChR antibody (AChR-Ab) were tested at the same time. The mean clinical symptom grades of gentamycin group (1.312, 2.067), amikacin group (1.111, 1.889) and etimicin group (1.263, 1.632) were significantly higher than those of MG group (1.000, 1.200) (P<0.05). The positive rates of RNS of three antibiotics groups were 69.23 %, 58.82 % and 63.16 % respectively, which were significantly higher than those of MG group and NS group (40.00 %, 40.00 %, P<0.05). The AChR-Ab level in serum and the expression of AChR on neuromuscular junction (NMJ) of mice in three antibiotics groups were also higher than those of MG group. Our results indicated that aminoglycoside antibiotics could aggravate the symptom of myasthenia gravis. The exacerbation of myasthenia gravis by these antibiotics probably involves competitively restraining the release of acetylcholine from presynaptic membrane, impairing the depolarization of postsynaptic membrane, depressing the irritability of myocyte membrane around the end-plate membrane and consequently leading to the blockade of neuromuscular junction.
文摘Objective: To investigate the expression of both thymic regulatory T cells (CD4+CD25+Foxp3+cells, Treg) and thymic stromal lymphopoietin (TSLP) in thymomas accompanying myasthenia gravis. Methods: We used immunohistochemistry and real-time reverse trancription polymerase chain reaction (real-time RT-PCR) techniques to determine Foxp3+ Treg counts and the expression levels of Foxp3 mRNA and TSLP mRNA in thymomas of 23 MG patients and thymuses of 4 healthy controls. Results: The CD4+ Foxp3+ nTreg (natural regulatory T cells) counts in thymomas were significantly lower than those in normal thymuses (P<0.01), and the expression levels of Foxp3 mRNA and TSLP mRNA were also lower in thymomas(P<0.01). Among the thymoma types, type B1 thymoma had the highest Foxp3+ nTreg count and standard values of Foxp3 mRNA and TSLP mRNA. There was a strong positive correlation between the mRNA transcriptional levels of Foxp3 and TSLP. Conclusion: The insufficient expression of Foxp3 in thymoma, which may be caused by decreased transcription of TSLP, may result in the reduction of Tregs and cause autoimmune disorders.
