Departments of Neurology and Pathology (Neuropathology), University of Pittsburgh School of Medicine, Pittsburgh, PA.
J Clin Neuromuscul Dis. 2020 Jun;21(4):195-204. doi: 10.1097/CND.0000000000000285.
Four retrospective studies from the United States, Europe, and Asia address outcomes in juvenile myasthenia gravis. Common features include earlier onset with ocular myasthenia gravis (MG) and generally good outcomes overall. Patients who were seronegative, had equivocal acetylcholine receptor (AChR) antibodies, or had only clustered AChR antibodies had better outcomes. An article highlighting the utility of median nerve slow-repetitive stimulation is reviewed and another showing the high sensitivity of repetitive nerve stimulation in myasthenic crisis is covered. Two articles address the thymus and thymectomy in non-AChR antibody-positive autoimmune MG. Longer term data on eculizumab as well as studies of cyclophosphamide, rituximab, and tacrolimus are summarized. Other topics include the possible role of statins in MG and central nervous system autoimmune comorbidities.
四项来自美国、欧洲和亚洲的回顾性研究探讨了青少年重症肌无力的结局。共同特征包括眼肌型重症肌无力(MG)的发病年龄更早,总体预后良好。血清阴性、乙酰胆碱受体(AChR)抗体不确定或仅存在聚集性 AChR 抗体的患者预后更好。一篇强调正中神经慢重复刺激作用的文章和另一篇显示重复神经刺激在肌无力危象中高度敏感的文章被涵盖。两篇文章探讨了非 AChR 抗体阳性自身免疫性 MG 中的胸腺和胸腺切除术。关于依库珠单抗的更长期数据以及环磷酰胺、利妥昔单抗和他克莫司的研究也进行了总结。其他主题包括他汀类药物在 MG 中的可能作用以及中枢神经系统自身免疫合并症。