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
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
BACKGROUND Coronary artery spasm(CAS)is a rare but critical condition during surgery.Clinical manifestations can vary from only subtle electrocardiography change to sudden death.In this case report,we present the case...BACKGROUND Coronary artery spasm(CAS)is a rare but critical condition during surgery.Clinical manifestations can vary from only subtle electrocardiography change to sudden death.In this case report,we present the case of a patient with myasthenia gravis(MG)who developed refractory CAS-related cardiogenic shock during thymoma surgery.CASE SUMMARY A 61-year-old man had a history of cigarette smoking and coronary artery disease with a bare metal stent placed.Three months ago,he suffered from coronary spasms,with three vessels involved,after surgery for cervical spine injury.He started having progressive dysphagia 4 wk prior and was diagnosed with MG via serologic tests,and computed tomography declared a thymoma in the anterior mediastinum.After the symptoms of MG subsided,he was referred for thy-mectomy.The operation was uneventful until the closing of the sternal wound.Electrocardiography showed sudden onset ST elevation,followed by ventricular tachycardia and severe hypotension.Cardiopulmonary cerebral resuscitation was initiated immediately with electrical defibrillation,extracorporeal membrane oxygenation was performed due to refractory cardiogenic shock,and the patient was transferred to an angiography room.Angiography showed diffuse CAS with three vessels involved.Intracoronary isosorbide dinitrate and adenosine were administered,and then the patient was transferred to the intensive care unit.CONCLUSION Our case highlights the importance of being prepared for clinical situations such as the one described here and suggests the necessity of developing an appropriate anesthesia plan that includes proactive analgesia and preemptive coronary vaso-dilators.展开更多
In recent decades, the treatment of myasthenia gravis has been extensively developed, but a standardized standard still needs to be used. Its treatment strategy is associated with patient prognosis, economic costs, an...In recent decades, the treatment of myasthenia gravis has been extensively developed, but a standardized standard still needs to be used. Its treatment strategy is associated with patient prognosis, economic costs, and complications. This article reviews the pathogenesis, treatment methods, and complications of myasthenia gravis, providing new ideas for diagnosing and treating myasthenia gravis and fully embodies the principle of safety and precision.展开更多
Background:To investigate the effectiveness of the progressive resistance training(PRT)using thera band in improving muscle strength in myasthenia gravis(MG).Methods:In this prospective study,12 MG patients with gener...Background:To investigate the effectiveness of the progressive resistance training(PRT)using thera band in improving muscle strength in myasthenia gravis(MG).Methods:In this prospective study,12 MG patients with generalised stable disease performed progressive resistance training with thera band for four times per week for 24 weeks.The isometric muscle force of shoulder abductors,biceps brachii and knee extensors using handheld dynamometer,Myasthenia Gravis Composite(MGC)score,Quantitative myasthenia gravis score(QMG),were assessed before and after the training period.Results:Progressive resistance training was well tolerated,and the isometric muscle strength was significantly improved in shoulder abductors,biceps brachii and knee extensors(P<0.05).The disease course(QMG and MGC)was slowed down and improved(P<0.05).Conclusion:Progressive resistance training is effective in improving muscle strength specifically in most affected muscles in MG.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
Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction."Warming yang and...Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction."Warming yang and invigorating qi" acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis.However,few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction.Here,we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following "warming yang and invigorating qi" acupuncture therapy.Needles were inserted at acupressure points Shousanli(LI10),Zusanli(ST36),Pishu(BL20),and Shenshu(BL23) once daily for 7 consecutive days.The treatment was repeated after 1 day of rest.We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment.This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide.These findings suggest that "warming yang and invigorating qi" acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.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
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 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
Cardiovascular adverse events in patients with myasthenia gravis (MG) are rare, but the early recognition of such events is crucial. We describe a case of a noncoronary myocardial infarction (MI) during the initial tr...Cardiovascular adverse events in patients with myasthenia gravis (MG) are rare, but the early recognition of such events is crucial. We describe a case of a noncoronary myocardial infarction (MI) during the initial treatment period with pyridostigmine bromide in a female patient with MG. Clinicians should be cautious about the appearance of potential MI in patients with MG. A baseline electrocardiogram is advocated, when the early recognition of the MI clinical signs and the laboratory findings (myocardial markers) are vital to the immediate and appropriate management of this medical emergency, as well as to prevent future cardio-vascular events. In this case report possible causes of myocardial adverse events in the context of MG, which may occur during the ongoing treatment and the clinical course of the disease, are discussed.展开更多
Myasthenia gravis (MG) is an autoimmune disorder involving the neuromuscular junction that frequently affects the extra-ocular muscles (EOMs). It has been described as a very rare cause of bilateral EOM atrophy, but h...Myasthenia gravis (MG) is an autoimmune disorder involving the neuromuscular junction that frequently affects the extra-ocular muscles (EOMs). It has been described as a very rare cause of bilateral EOM atrophy, but histological analysis of such cases is lacking. A 66-year-old man presented with two months of right eyelid drooping and vertical diplopia. Examination showed bilateral ophthalmoparesis and complete right ptosis. The remainder of his exam was normal, and an MRI showed small EOMs. Acetylcholine receptor antibodies were elevated, establishing the diagnosis of MG. Oral corticosteroids and pyridostigmine followed by azathioprine improved his ptosis, but not his ophthalmoparesis. One year later he had surgical correction of his diplopia, and the resected superior rectus muscle showed complete replacement of EOM by connective tissue. MG can rarely cause bilateral EOM atrophy, which is characterized histologically by fibrosis in the muscle itself. Atrophy in the EOMs of a myasthenic patient may indicate a poor response to medical management alone.展开更多
Objective To present the clinical experience in treating thymomas and thymomas with myasthenia gravis (MG). Methods Between 1975 to 1997,285 patients with thymoma were surgically treated. 168 cases of thymus tumor (TT...Objective To present the clinical experience in treating thymomas and thymomas with myasthenia gravis (MG). Methods Between 1975 to 1997,285 patients with thymoma were surgically treated. 168 cases of thymus tumor (TT,Group Ⅰ ) were compared to 90 cases of thymus tumor with myasthenia gravis (TTMG, Group Ⅱ ). Results In group Ⅰ , the average age was 38. 4 years, the youngest was 4 years old. In 93. 4%, the diameter of tumors was 5 cm. 61. 2% were in stages Ⅲ and Ⅳ of clinical pathology. In group Ⅱ ,the average age was 46.2 with the youngest of 20 years old,the diameter of tumor under 3 cm and 5 cm was 34.6 % and 65.6 % . 55.2 % was in stage Ⅰ of clinical pathology. Conclusion Many cases of thymoma in early stage received early treatment. The diagnostic standard of thymoma in early stage is: (1) the diameter of tumor is below 3 cm. (2) In stage I of clinical pathology. The clinical characteristics of TTMG are: short history, the rapid progress, serious MG symptoms,and the high incidence of thymus-展开更多
Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra f...Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman’s standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.展开更多
objective: To analyze the effects of thymectomy for children with myasthenia gravis (MG).Methods: Thirty-six children with MG were treated by thymectomy from 1984 to 1997, their age ranging from 4 to 14 years, with sy...objective: To analyze the effects of thymectomy for children with myasthenia gravis (MG).Methods: Thirty-six children with MG were treated by thymectomy from 1984 to 1997, their age ranging from 4 to 14 years, with symptom duration from 2 months to 8 years. According to Osserman’s classifica tion, 27 case belongs to class Ⅰ, 6 class Ⅱa, 2 class Ⅱb and 1 class Ⅲ. The outcome was graded as "much im proved", "improved", "unchanged" and "deteriorated or death". Results: 13. 9% of patients were much im proved, 69. 4% improved, so the better response to thymectomy (much improved or improved) is 83. 3%, which is significantly higher than the control group (without surgical treatment) 44. 8% (P<0. 001), and no one exacerbated or died. Conclusion: Thymectomy for children with MG is safe and effective, and the out come after thymectomy relates to the duration of symptoms and pathologic classification, but irrelevant to sex and age, and the follow-up results were satisfactory.展开更多
The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding...The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.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.
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.
文摘BACKGROUND Coronary artery spasm(CAS)is a rare but critical condition during surgery.Clinical manifestations can vary from only subtle electrocardiography change to sudden death.In this case report,we present the case of a patient with myasthenia gravis(MG)who developed refractory CAS-related cardiogenic shock during thymoma surgery.CASE SUMMARY A 61-year-old man had a history of cigarette smoking and coronary artery disease with a bare metal stent placed.Three months ago,he suffered from coronary spasms,with three vessels involved,after surgery for cervical spine injury.He started having progressive dysphagia 4 wk prior and was diagnosed with MG via serologic tests,and computed tomography declared a thymoma in the anterior mediastinum.After the symptoms of MG subsided,he was referred for thy-mectomy.The operation was uneventful until the closing of the sternal wound.Electrocardiography showed sudden onset ST elevation,followed by ventricular tachycardia and severe hypotension.Cardiopulmonary cerebral resuscitation was initiated immediately with electrical defibrillation,extracorporeal membrane oxygenation was performed due to refractory cardiogenic shock,and the patient was transferred to an angiography room.Angiography showed diffuse CAS with three vessels involved.Intracoronary isosorbide dinitrate and adenosine were administered,and then the patient was transferred to the intensive care unit.CONCLUSION Our case highlights the importance of being prepared for clinical situations such as the one described here and suggests the necessity of developing an appropriate anesthesia plan that includes proactive analgesia and preemptive coronary vaso-dilators.
文摘In recent decades, the treatment of myasthenia gravis has been extensively developed, but a standardized standard still needs to be used. Its treatment strategy is associated with patient prognosis, economic costs, and complications. This article reviews the pathogenesis, treatment methods, and complications of myasthenia gravis, providing new ideas for diagnosing and treating myasthenia gravis and fully embodies the principle of safety and precision.
文摘Background:To investigate the effectiveness of the progressive resistance training(PRT)using thera band in improving muscle strength in myasthenia gravis(MG).Methods:In this prospective study,12 MG patients with generalised stable disease performed progressive resistance training with thera band for four times per week for 24 weeks.The isometric muscle force of shoulder abductors,biceps brachii and knee extensors using handheld dynamometer,Myasthenia Gravis Composite(MGC)score,Quantitative myasthenia gravis score(QMG),were assessed before and after the training period.Results:Progressive resistance training was well tolerated,and the isometric muscle strength was significantly improved in shoulder abductors,biceps brachii and knee extensors(P<0.05).The disease course(QMG and MGC)was slowed down and improved(P<0.05).Conclusion:Progressive resistance training is effective in improving muscle strength specifically in most affected muscles in MG.
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.
基金supported by the National Natural Science Foundation of China,No.81173344
文摘Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction."Warming yang and invigorating qi" acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis.However,few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction.Here,we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following "warming yang and invigorating qi" acupuncture therapy.Needles were inserted at acupressure points Shousanli(LI10),Zusanli(ST36),Pishu(BL20),and Shenshu(BL23) once daily for 7 consecutive days.The treatment was repeated after 1 day of rest.We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment.This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide.These findings suggest that "warming yang and invigorating qi" acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.
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.
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.
文摘Cardiovascular adverse events in patients with myasthenia gravis (MG) are rare, but the early recognition of such events is crucial. We describe a case of a noncoronary myocardial infarction (MI) during the initial treatment period with pyridostigmine bromide in a female patient with MG. Clinicians should be cautious about the appearance of potential MI in patients with MG. A baseline electrocardiogram is advocated, when the early recognition of the MI clinical signs and the laboratory findings (myocardial markers) are vital to the immediate and appropriate management of this medical emergency, as well as to prevent future cardio-vascular events. In this case report possible causes of myocardial adverse events in the context of MG, which may occur during the ongoing treatment and the clinical course of the disease, are discussed.
文摘Myasthenia gravis (MG) is an autoimmune disorder involving the neuromuscular junction that frequently affects the extra-ocular muscles (EOMs). It has been described as a very rare cause of bilateral EOM atrophy, but histological analysis of such cases is lacking. A 66-year-old man presented with two months of right eyelid drooping and vertical diplopia. Examination showed bilateral ophthalmoparesis and complete right ptosis. The remainder of his exam was normal, and an MRI showed small EOMs. Acetylcholine receptor antibodies were elevated, establishing the diagnosis of MG. Oral corticosteroids and pyridostigmine followed by azathioprine improved his ptosis, but not his ophthalmoparesis. One year later he had surgical correction of his diplopia, and the resected superior rectus muscle showed complete replacement of EOM by connective tissue. MG can rarely cause bilateral EOM atrophy, which is characterized histologically by fibrosis in the muscle itself. Atrophy in the EOMs of a myasthenic patient may indicate a poor response to medical management alone.
文摘Objective To present the clinical experience in treating thymomas and thymomas with myasthenia gravis (MG). Methods Between 1975 to 1997,285 patients with thymoma were surgically treated. 168 cases of thymus tumor (TT,Group Ⅰ ) were compared to 90 cases of thymus tumor with myasthenia gravis (TTMG, Group Ⅱ ). Results In group Ⅰ , the average age was 38. 4 years, the youngest was 4 years old. In 93. 4%, the diameter of tumors was 5 cm. 61. 2% were in stages Ⅲ and Ⅳ of clinical pathology. In group Ⅱ ,the average age was 46.2 with the youngest of 20 years old,the diameter of tumor under 3 cm and 5 cm was 34.6 % and 65.6 % . 55.2 % was in stage Ⅰ of clinical pathology. Conclusion Many cases of thymoma in early stage received early treatment. The diagnostic standard of thymoma in early stage is: (1) the diameter of tumor is below 3 cm. (2) In stage I of clinical pathology. The clinical characteristics of TTMG are: short history, the rapid progress, serious MG symptoms,and the high incidence of thymus-
文摘Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman’s standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.
文摘objective: To analyze the effects of thymectomy for children with myasthenia gravis (MG).Methods: Thirty-six children with MG were treated by thymectomy from 1984 to 1997, their age ranging from 4 to 14 years, with symptom duration from 2 months to 8 years. According to Osserman’s classifica tion, 27 case belongs to class Ⅰ, 6 class Ⅱa, 2 class Ⅱb and 1 class Ⅲ. The outcome was graded as "much im proved", "improved", "unchanged" and "deteriorated or death". Results: 13. 9% of patients were much im proved, 69. 4% improved, so the better response to thymectomy (much improved or improved) is 83. 3%, which is significantly higher than the control group (without surgical treatment) 44. 8% (P<0. 001), and no one exacerbated or died. Conclusion: Thymectomy for children with MG is safe and effective, and the out come after thymectomy relates to the duration of symptoms and pathologic classification, but irrelevant to sex and age, and the follow-up results were satisfactory.
文摘The myasthenia gravis is an autoimmune disease which can be associated frequently with a thymoma and also with autoimmune pathologies. We report the case of a 64-year-old man affected an overlap syndrome corresponding to the association of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). He also presented concomitant myasthenia gravis and thymoma. The physical examination notes only a disturbed voice. The liver tests showed: alkaline phosphatase 600 U/l, alanine aminotransferase 1280 U/l, and aspartate aminotransferase 985 U/l. Viral serologies of hepatitis were all negative, as well as antinuclear, antimitochondrial and antismooth muscle antibodies. The diagnosis of an overlap syndrome was posed according to the biological and hepatic histhological results. An associated myasthenia was confirmed by the results of the EMG and the positivity of the anti-acetylcholine receptor antibody. A chest CT revealed a thymoma. The treatment consisted of ursodeoxycholic acid, prednisone, azathioprine and pyridostigmine. And the patient improved his voice and the liver function. Thymectomy was practiced without incidents.
