Analysis of clinical and electrophysiological characteristics of generalized myasthenia gravis / 中风与神经疾病杂志

ABSTRACT

@#Objective To explore the differences of clinical and electrophysiological characteristics of different subgroups of generalized myasthenia gravis（MG），and analyze the correlation between electrophysiological results and disease severity.Methods A retrospective analysis of 101 generalized MG patients who accepted diagnosis and treatment in the department of neurology of Shenzhen Hospital of Hongkong University from July 2016 to July 2020 was performed. According to their clinical feature，these patients were divided into early-onset generalized MG group with Acetylcholine receptor（AchR） antibodies（52 cases），late-onset generalized MG group with AchR antibodies（23 cases），thymoma-associated MG group（22 cases） and MuSK associated MG group（4 cases）. We compared the demographic characteristic，Quantitative Myasthenia Gravis score（QMGs），MG Foundation America（MGFA） Clinical Classification，repetitive nerve stimulation（RNS） results of different subgroups and analyze the correlation between RNS results and QMGs.Results In different subgroups of generalized MG without positive MuSK antibodies，the rate of female patients of early-onset MG with AchR antibodies was highest（71.2% vs 65.2% vs 36.4%，P<0.05），the rate of patients with respiratory/bulbar muscles dominantly impaired of thymoma-associated MG was highest（28.8% vs 43.5% vs 63.6%，P<0.05），the rate of patients with RNS positive of whole muscles detected of early-onset MG with AchR antibodies was highest（44.2% vs 17.4% vs 22.7%，P<0.05），the positive correlation between QMGs and highest or whole RNS decrement was demonstrated（P<0.05）；In MuSK associated MG，the rate of patients with respiratory/bulbar muscles dominantly impaired or moderately and severely impaired were relatively higher（both 75%），the rate of patients with RNS positive of whole muscles detected were relatively lower（0%）.Conclusion The population distribution，gender proportion，dominantly impaired muscles and severity and distribution of impaired muscles of different subgroups of generalized MG were significantly different，which would be valuable in the classification diagnosis of generalized MG；The high correlation between RNS results and disease severity existed，which demonstrated the significant values of RNS in severity evaluation of generalized MG.