Rinaldi Carlo, Russo Cinzia Valeria, Filla Alessandro, De Michele Giuseppe, Marano Enrico
Department of Neurological Sciences, Federico II University, Via Pansini, 5, 80131, Naples, Italy.
Neurol Sci. 2009 Jun;30(3):237-9. doi: 10.1007/s10072-009-0041-y. Epub 2009 Mar 4.
Morvan's syndrome is a rare disease characterized by peripheral nerve hyperexcitability, associated with CNS and autonomic systems involvement. High serum voltage-gated potassium channel (VGKC) antibody titers have been reported, and, till now, Morvan's syndrome has been considered as a VGKC antibody associated disease. We describe a patient with Morvan's syndrome associated with myasthenia gravis and a thymoma in his previous history, with surprisingly undetectable levels of VGKC antibodies. The clinical course is similar to those cases of Morvan's syndrome with VGKC-Ab, except for the lack of response to plasma exchange, previously considered as the first choice treatment. Nevertheless, the good response to corticosteroids therapy and the association with myasthenia confirm an autoimmune origin of the disease.
莫旺综合征是一种罕见疾病，其特征为周围神经兴奋性增高，并伴有中枢神经系统和自主神经系统受累。据报道，患者血清中电压门控钾通道（VGKC）抗体滴度较高，迄今为止，莫旺综合征一直被视为一种与VGKC抗体相关的疾病。我们描述了一名曾患重症肌无力和胸腺瘤的莫旺综合征患者，其VGKC抗体水平出奇地检测不到。该患者的临床病程与那些VGKC抗体阳性的莫旺综合征病例相似，只是对血浆置换无反应，而血浆置换此前被视为首选治疗方法。尽管如此，该患者对皮质类固醇治疗反应良好以及与重症肌无力的关联证实了该疾病的自身免疫起源。
