Background:Myasthenia gravis(MG)and neuromyelitis optica(NMO,also known as Devic disease)are rare autoimmune disorders,with upper-limit prevalence estimates in the general population of 15 per 100 000 and 5 per 100 00...Background:Myasthenia gravis(MG)and neuromyelitis optica(NMO,also known as Devic disease)are rare autoimmune disorders,with upper-limit prevalence estimates in the general population of 15 per 100 000 and 5 per 100 000,respectively.To our knowledge,an association between these diseases has not been previously reported.Objectives:To describe 4 patients with MG who developed NMO after thymectomy and to analyze possible causes of apparent increased prevalence of NMO among patients with MG.Design:Case series.Patients:Four patients with MG who underwent thymectomy.Interventions:None.Results:The prevalence of MG within the published cohort of patients with NMO is more than 150 times higher than that in the general population.Conclusion:Dysregulation of B-cell autoimmunity in myasthenia,possibly exacerbated by loss of control over autoreactive cells as a result of thymectomy,may predispose patients to the development of NMO.展开更多
Background: Antibodies to a muscle-specific receptor tyrosine kinase (MuSK) h ave been found in approximately 40%of patients with generalized myasthenia grav is who are seronegative for the antiacetylcholine receptor ...Background: Antibodies to a muscle-specific receptor tyrosine kinase (MuSK) h ave been found in approximately 40%of patients with generalized myasthenia grav is who are seronegative for the antiacetylcholine receptor antibody. Many of the patients with anti-MuSK antibodies have prominent oculobulbar symptoms or weak ness of the neck and respiratory muscles, but patients with ocular myasthenia ha ve not been described. Objective: To report a case of ocular myasthenia due to a nti-MuSK antibodies. Patient: A young woman with ocular myasthenia and antibodi es to MuSK. Results: Anti-MuSK antibody was detected by radioimmunoassay using highly purified MuSK recombinant antigen. Conclusion: Ocular myasthenia gravis i s a presentation of the anti-MuSK antibody syndrome.展开更多
Objective: To assess the efficacy and side effects of cyclophosphamide on refractory myasthenic crisis. Methods: Five patients of myasthenic crisis refractory to usual comprehensive treatment, entered an open addition...Objective: To assess the efficacy and side effects of cyclophosphamide on refractory myasthenic crisis. Methods: Five patients of myasthenic crisis refractory to usual comprehensive treatment, entered an open additional study with cyclophosphamide 200 mg VD q. d or 400 mg VD q. o. d with 6-10 g of total dosage. The patients were followed up for 1-8 years. Results: All the 5 patients were effectively treated with obvious remission in 3 and improvement in 2. Two patients have returned to partial work. The side effects were tolerable. Conclusion: The present clinical trial showed that cyclophosphamide was effective, particularly in a long term as an additional therapy for treating MG patients with refractory crisis of myasthenia gravis.展开更多
Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in ...Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.展开更多
文摘Background:Myasthenia gravis(MG)and neuromyelitis optica(NMO,also known as Devic disease)are rare autoimmune disorders,with upper-limit prevalence estimates in the general population of 15 per 100 000 and 5 per 100 000,respectively.To our knowledge,an association between these diseases has not been previously reported.Objectives:To describe 4 patients with MG who developed NMO after thymectomy and to analyze possible causes of apparent increased prevalence of NMO among patients with MG.Design:Case series.Patients:Four patients with MG who underwent thymectomy.Interventions:None.Results:The prevalence of MG within the published cohort of patients with NMO is more than 150 times higher than that in the general population.Conclusion:Dysregulation of B-cell autoimmunity in myasthenia,possibly exacerbated by loss of control over autoreactive cells as a result of thymectomy,may predispose patients to the development of NMO.
文摘Background: Antibodies to a muscle-specific receptor tyrosine kinase (MuSK) h ave been found in approximately 40%of patients with generalized myasthenia grav is who are seronegative for the antiacetylcholine receptor antibody. Many of the patients with anti-MuSK antibodies have prominent oculobulbar symptoms or weak ness of the neck and respiratory muscles, but patients with ocular myasthenia ha ve not been described. Objective: To report a case of ocular myasthenia due to a nti-MuSK antibodies. Patient: A young woman with ocular myasthenia and antibodi es to MuSK. Results: Anti-MuSK antibody was detected by radioimmunoassay using highly purified MuSK recombinant antigen. Conclusion: Ocular myasthenia gravis i s a presentation of the anti-MuSK antibody syndrome.
文摘Objective: To assess the efficacy and side effects of cyclophosphamide on refractory myasthenic crisis. Methods: Five patients of myasthenic crisis refractory to usual comprehensive treatment, entered an open additional study with cyclophosphamide 200 mg VD q. d or 400 mg VD q. o. d with 6-10 g of total dosage. The patients were followed up for 1-8 years. Results: All the 5 patients were effectively treated with obvious remission in 3 and improvement in 2. Two patients have returned to partial work. The side effects were tolerable. Conclusion: The present clinical trial showed that cyclophosphamide was effective, particularly in a long term as an additional therapy for treating MG patients with refractory crisis of myasthenia gravis.
文摘Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.
