CASPR2/LGI1抗体双阳Isaccs综合征的临床观察  

Clinical observation of CASPR2/LGI1 antibody double positive Isaccs syndrome

作　　者：王小琴 丁岩[1] 卫华[1] Wang Xiaoqin;Ding Yan;Wei Hua(Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,北京100053

出　　处：《北京医学》2023年第5期436-438,442,共4页Beijing Medical Journal

摘　　要：目的总结接触蛋白相关样蛋白2(contactin-associated protein 2,CASPR2)/富亮氨酸胶质瘤失活蛋白1(leucine-rich glioma inactivated 1,LGI1)抗体双阳Isaacs综合征的临床表现。方法回顾性分析首都医科大学宣武医院收治的1例CASPR2/LGI1抗体双阳Isaacs综合征患者的临床表现和诊治经过。结果本例患者病程中有肌肉颤搐、大汗、体位性低血压、窦性心动过速、小便障碍、睡眠障碍、低钠血症、体质量下降等临床表现。肌电图提示肌颤搐、束颤电位,血清CASPR2/LGI1抗体双阳性。临床表现既符合Isaacs综合征,又符合轻型Morvan综合征,予以小剂量激素治疗后症状完全缓解。结论Isaacs综合征和Morvan综合征具有相同的病因和发病机制,临床表现存在重叠,鉴别较困难,均对激素及丙球治疗效果好,可能是临床表现不同的同一种综合征。Objective To summarize the clinical manifestations of antibody double-positive Isaacs syndrome with contactin-associated protein 2(CASPR2)/leucine-rich glioma activated protein 1(LGI1)antibody double-positive.Methods The clinical manifestations,diagnosis and treatment of a patient with CASPR2/LGI1 antibody double-positive Isaacs syndrome in Xuanwu Hospital,Capital Medical University were analyzed.Results The patient had clinical manifestation such as muscle twitching,sweating,postural hypotension,sinus tachycardia,dysuria,sleep disorder,hyponatremia and weight loss.Electromyography showed twitching and fascicular fibrillation potential,and serum CASPR2/LGI1 antibody was double positive.The clinical manifestations were consistent with both Isaacs syndrome and mild Morvan syndrome,and the symptoms were completely relieved after low-dose hormone treatment.Conclusions Isaacs syndrome and Morvan syndrome have the same etiology and pathogenesis,and their clinical manifestations overlap which is difficult to distinguish them.Both of them have good therapeutic effects on hormones and gamma globulin,which may be the same syndrome with different clinical manifestations.

关 键 词：ISAACS综合征 Morvan综合征 接触蛋白相关样蛋白2 富亮氨酸胶质瘤失活蛋白1 周围神经过度兴奋 

分 类 号：R745[医药卫生—神经病学与精神病学]