PDF(Pubmed)
Abstract：
We presented a 23-year-old patient who had experienced neuromyotonia in his left leg. Although he tested negative for anti-LGI1 and anti-CASPR2 antibodies, we diagnosed him with Isaacs syndrome due to myokymic discharges on electromyography and symptoms being relieved by intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIg). IVMP, IVIg, plasma exchange, or cyclosporine treatment did not provide a long-term response; however, rituximab showed long-term improvement. Rituximab should be considered early in the treatment of patients with antibody-negative Isaacs syndrome who are responsive to immunotherapy, including IVMP, IVIg, and plasma exchange, and have long-term symptoms that are hard to control.
摘要：
我们介绍了一名23岁的患者，他的左腿出现了神经肌强直。尽管他的抗LGI1和抗CASPR2抗体检测呈阴性，我们诊断他患有Isaacs综合征,原因是肌电图显示的肌胸腺放电,静脉注射甲基强的松龙(IVMP)和静脉注射免疫球蛋白(IVIg)可缓解症状.IVMP,IVIg,血浆置换,或环孢素治疗不能提供长期反应；然而，利妥昔单抗显示长期改善。利妥昔单抗应早期用于治疗对免疫治疗有反应的抗体阴性Isaacs综合征患者，包括IVMP,IVIg,和血浆置换，并且有难以控制的长期症状。
