视神经脊髓炎谱系障碍（neuromyelitis optic spectrum disease，NMOSD）是一种自身免疫性神经系统疾病，其特征是复发性视神经炎和纵向广泛横行性脊髓炎。NMOSD患者妊娠后易发生不良妊娠结局、分娩期并发症、NMOSD复发和致残。本文通过分析1例NMOSD患者妊娠及分娩期间病情加重、发生产后出血伴凝血功能障碍，最终抢救成功的案例，提出分娩期间积极给予类固醇激素及丙种球蛋白是控制病情的有效方案，同时快速补充凝血因子，及时纠正凝血功能以避免不良结局，总结了该类患者孕期保健及分娩相关风险，为管理流程的优化提供经验。

Neuromyelitis optica spectrum disorder （NMOSD） is an autoimmune neurological condition characterized by relapsing optic neuritis and longitudinally extensive transverse myelitis. Patients with NMOSD are prone to adverse pregnancy outcomes， intrapartum complications， high relapse rates， and significant disability following pregnancy. This paper analyzes a case of NMOSD experienced exacerbation of symptoms during pregnancy and childbirth， postpartum hemorrhage accompanied by coagulopathy， and ultimately successful resuscitation. We propose that active administration of corticosteroids and intravenous immunoglobulin during labor is an effective and feasible strategy for controlling the disease. Rapid replenishment of coagulation factors and timely correction of coagulation function are essential to prevent adverse outcomes. The paper summarizes the prenatal care and childbirth-related risks for such patients， and provides insights for the optimization of management protocols.