Department of Neurology, University of Chicago, Chicago, IL, USA.
Neurology. 2013 Jan 29;80(5):e55. doi: 10.1212/WNL.0b013e31827f0fa1.
A 50-year-old woman with a history of metastatic malignant thymoma presented with diffuse neuropathic pain involving the extremities and torso prior to chemotherapy and radiation. She also developed episodic diarrhea, diaphoresis, fevers, insomnia, and encephalopathy. Examination revealed rippling muscles (video on the Neurology® Web site at www.neurology.org). Prolonged afterdischarges were noted in motor nerve studies, suggestive for nerve hyperexcitability (figures 1 and 2). Electromyography (approximately 8 years after mediastinal radiation) revealed fasciculations, doublets, triplets, and myokymic discharges. Elevated serum antibodies for voltage-gated potassium channel (0.30 nmol/L, normal <0.02) and striational muscle (1:30,720, normal <1:60) were suggestive of paraneoplastic Morvan syndrome, which includes all of the features noted in our case.(1,2) The CNS features of Morvan syndrome differentiate it from Isaac syndrome, which is most often due to an autoimmune etiology. A substantial proportion of Morvan syndrome cases are paraneoplastic, the majority of which are due to thymomas.(1).
一位 50 岁的女性，有转移性恶性胸腺瘤病史，在化疗和放疗前出现四肢和躯干弥漫性神经病理性疼痛。她还出现了间歇性腹泻、出汗、发热、失眠和脑病。检查发现肌肉波纹（神经病学®网站上的视频，网址为：www.neurology.org）。运动神经研究中发现了延长后的放电，提示神经兴奋性过高（图 1 和 2）。肌电图（纵隔放疗后约 8 年）显示肌束颤动、二联、三联和肌痉挛放电。电压门控钾通道（0.30nmol/L，正常<0.02）和横纹肌（1:30,720，正常<1:60）的血清抗体升高提示副肿瘤性莫旺综合征，包括我们病例中的所有特征。（1,2）莫旺综合征的中枢神经系统特征使其与艾萨克综合征区分开来，后者通常是由于自身免疫性病因引起的。相当一部分莫旺综合征病例是副肿瘤性的，其中大多数是由于胸腺瘤引起的。（1）。
