PT -期刊文章盟艾格尼丝·Sonderen AU -海伦娜Arino盟- Mar Petit-Pedrol AU -弗兰克Leypoldt AU -彼得Kortvelyessy盟Klaus-Peter扫读非盟-埃里克·兰开斯特盟-保罗·w·Wirtz AU -马尔科•j•Schreurs AU -彼得A.E. Sillevis Smitt AU -弗朗西斯克格劳盟约瑟Dalmau AU - Maarten j . Titulaer TI的临床表现Caspr2 antibody-associated疾病- 10.1212 / WNL的援助。0000000000002917 DP - 2016年8月02 TA -神经病首页学第六PG - 521 - 528 - 87 IP - 5 4099 - //www.ez-admanager.com/content/87/5/521.short 4100 - //www.ez-admanager.com/content/87/5/521.full所以Neurology2016 8月02;87 AB -目的:报告一大群患者抗体contactin-associated蛋白质像2 (Caspr2),并提供这种疾病的临床表现。方法:血清和脑脊液样本评估2神经免疫学中心在巴塞罗那和鹿特丹。患者包括如果Caspr2抗体被证实与2独立技术,包括大脑免疫组织化学和细胞试验。临床作者获得的信息或由医生治疗病人的知情同意后提供。结果:平均年龄在症状出现是66年。38例,34岁男性。最低点的疾病的平均时间为4个月(30%在1年)。最常见的症状包括边缘脑炎(42%)和Morvan综合症(29%)。百分之七十七的病人有≥3以下的症状:脑病(认知缺陷/癫痫发作),小脑功能障碍,周围神经系统兴奋过度,神经异常、失眠、神经性疼痛,或减肥。一个肿瘤,大多是胸腺瘤,发生在19%的病人。在所有患者免疫球蛋白抗体G4子类在场; 63% also had immunoglobulin G1 antibodies. Treatment response occurred in 93% of the patients and 25% had clinical relapses.Conclusions: Caspr2 antibodies associate with a treatable disorder that predominantly affects elderly men. The resulting syndrome may vary among patients but it usually includes a set of well-established symptoms. Recognition of this spectrum of symptoms and consideration of the protracted clinical course are important for early diagnosis of this disorder. Prompt immunotherapy and tumor therapy (if needed) often result in improvement.Caspr2=contactin-associated protein-like 2; CBA=cell-based assay; FLAIR=fluid-attenuated inversion recovery; IgG=immunoglobulin G; IHC=immunohistochemistry; IVIg=IV immunoglobulin; LE=limbic encephalitis; LGI1=leucine-rich glioma-inactivated 1; MG=myasthenia gravis; NMDAR=NMDA receptor; PNH=peripheral nerve hyperexcitability; RIA=radioimmunoassay; VGKC=voltage-gated potassium channel

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