重症肌无力伴Caspr2抗体相关莫旺综合征的临床特点(附1例报告)  

Clinical features of myasthenia gravis with caspr2-related morvan syndrome (report of one case)

作　　者：陆杰[1] 林柳余 杭海伦 张纪红 石静萍[1] 陈道文[1] LU Jie;LIN Liu-yu;HANG Hai-lun(Department of Neurology,Brain Hospital Affiliated 10 Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学附属脑科医院神经内科,210029

出　　处：《临床神经病学杂志》2022年第6期458-460,共3页Journal of Clinical Neurology

基　　金：国家自然科学基金青年项目(81500969);江苏省青年医学人才(QNRC2016052);南京脑科医院青年培养工程(686)。

摘　　要：目的 探讨抗乙酰胆碱受体(AChR)抗体及抗接触蛋白相关蛋白-2(Caspr2)抗体重叠综合征的发病机制、临床表现、诊断及治疗方法。方法 回顾性分析1例抗AChR抗体阳性的重症肌无力(MG),同时伴抗Caspr2抗体阳性的莫旺综合征患者的临床表现及治疗过程。结果 患者有胸腺瘤及手术切除病史,抗AChR抗体及Caspr2抗体双阳性,表现除眼睑下垂、复视,吞咽困难、肢体无力及肌无力危象等MG症状,还表现有自主神经症状、肌肉颤搐及疼痛、严重睡眠障碍和波动性生动视幻觉等莫旺综合征的典型临床表现,予丙种球蛋白、糖皮质激素等免疫治疗后症状好转,继续泼尼松、硫唑嘌呤续贯治疗,随访2年未再复发。结论 MG的抗AChR抗体及莫旺综合征的抗Caspr2抗体均于胸腺瘤密切相关,提示可能存在胸腺瘤相关抗体疾病谱。其发病机制尚待进一步研究,临床表现复杂,长程免疫治疗有助于控制病情、减少复发。Objective To investigate the pathogenesis, clinical manifestations, diagnosis and treatment of overlap syndrome of anti-acetylcholine receptor(AChR) antibody and anti-contact associated protein like 2(Caspr2) antibody. Method The clinical manifestation and treatment process of one case of myasthenia gravis(MG) with positive anti-AChR antibody and morvan’s syndrome with positive anti-Caspr2 antibody was analyzed retrospectively. Results The patient had a history of thymoma and surgical resection, and both anti-AChR and Caspr2 antibody were positive. Besides MG symptoms such as blepharoptosis, diplopia, dysphagia, limb weakness and myasthenic crisis, the patient also presented typical clinical manifestations of morvan syndrome such as autonomic nerve symptoms, myokymia and pain, severe sleep disorder and fluctuating visual hallucination. The patient was given gamma globulin and glucocorticoid and continued to be treated with prednisone and azathioprine. No recurrence was found after 2 years of follow-up. Conclusions The anti-AChR antibody of MG and anti-Caspr2 antibody of morvan syndrome are closely related to thymoma, suggesting that thymoma associated antibody disease spectrum may exist, and its pathogenesis needs further study, the clinical manifestations are complex, and long-term immunotherapy is helpful to control the disease and reduce recurrence.

关 键 词：重症肌无力 乙酰胆碱受体抗体 接触蛋白相关蛋白-2 莫旺综合征 胸腺瘤相关抗体疾病谱 

分 类 号：R746.1[医药卫生—神经病学与精神病学]