American University of Beirut Medical Center, Lebanon.
American University of Beirut Medical Center, Lebanon.
Neuromuscul Disord. 2022 Aug;32(8):682-686. doi: 10.1016/j.nmd.2022.06.001. Epub 2022 Jun 2.
Morvan Syndrome (MoS) is an autoimmune disorder characterized by peripheral nerve hyperexcitability, autonomic dysfunction, and encephalopathy. We describe the case of a man with a history of thymoma diagnosed with a paraneoplastic MoS with a severe painful neuropathy refractory to immunoglobulins and steroids who had a dramatic and lasting response following treatment with rituximab. We also reviewed the clinical features, comorbidities, laboratory findings, treatment responses, relapses, and long-term outcomes of all published cases of MoS treated with rituximab. This drug appears promising for the treatment of patients with MoS who failed first line therapy with immunoglobulins and steroids.
莫旺综合征（MoS）是一种自身免疫性疾病，其特征为周围神经兴奋性过高、自主神经功能障碍和脑病。我们描述了一例患有胸腺瘤的男性患者，被诊断为副肿瘤性 MoS，伴有严重的疼痛性神经病，对免疫球蛋白和类固醇治疗无效，而利妥昔单抗治疗后出现了显著且持久的反应。我们还回顾了所有已发表的用利妥昔单抗治疗的 MoS 病例的临床特征、合并症、实验室检查结果、治疗反应、复发和长期结局。这种药物似乎有望治疗一线免疫球蛋白和类固醇治疗失败的 MoS 患者。
