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莫尔万综合征与电压门控钾通道复合物多种成分的抗体相关。

Morvan's syndrome associated with antibodies to multiple components of the voltage-gated potassium channel complex.

机构信息

Limassol General Hospital, Limassol, Cyprus.

出版信息

J Neurol Sci. 2012 Jan 15;312(1-2):52-6. doi: 10.1016/j.jns.2011.08.024. Epub 2011 Sep 8.

Abstract

We describe a patient presenting with a combination of muscle fasciculations, paresthesias, hyperhidrosis, as well as insomnia, agitation and confusion. He went on to develop psychosis and respiratory failure requiring intensive care. Electromyography confirmed the presence of neuromyotonia and CSF showed mild pleocytosis. Routine testing for voltage-gated potassium channel complex (VGKC-complex) antibodies was highly positive, confirming the clinical diagnosis of Morvan's syndrome. The patient improved after treatment with intravenous immunoglobulin and methylprednisolone. Further investigation of the antigenic targets using immunohistochemistry and cell-based assays revealed that he had autoantibodies targeting Lgi1, Caspr2 and Contactin-2/Tag-1, all proteins known to be complexed with VGKC in peripheral nerves and CNS. This is the first case of Morvan's syndrome from Cyprus and illustrates the clinical features as well as the emerging complexity of antigenic targets involved in the pathogenesis.

摘要

我们描述了一位患者,其表现为肌肉抽搐、感觉异常、多汗,同时伴有失眠、烦躁和意识混乱。他随后出现了精神病和呼吸衰竭,需要重症监护。肌电图证实存在神经肌强直,脑脊液显示轻度白细胞增多。针对电压门控钾通道复合物 (VGKC-complex) 抗体的常规检测呈高度阳性,证实了莫旺综合征的临床诊断。患者在接受静脉注射免疫球蛋白和甲基强的松龙治疗后有所改善。使用免疫组化和基于细胞的检测进一步研究抗原靶点,显示他自身抗体针对 Lgi1、Caspr2 和 Contactin-2/Tag-1,这些蛋白均已知与周围神经和中枢神经系统中的 VGKC 复合物。这是来自塞浦路斯的首例莫旺综合征病例,说明了发病机制中涉及的抗原靶点的临床特征和新兴复杂性。

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