TY -的T1 Caspr2-Antibody相关疾病的临床表现(S12.008) JF -神经学乔-神经学六世- 86 - 16补充SP - S12.008盟Agnes Sond首页eren AU -海伦娜Arino AU - Mar Petit-Pedrol AU -弗兰克Leypoldt AU -彼得Kortvelyessy AU -埃里克·兰开斯特盟保罗Wirtz AU -马可Schreurs盟彼得Sillevis Smitt AU -弗朗西斯克格劳盟约瑟Dalmau AU - Maarten Titulaer Y1 - 2016/04/05 UR - //www.ez-admanager.com/content/86/16_Supplement/S12.008.abstract N2 -目的:我们描述一个大群contactin-associated蛋白2 (Caspr2)这个障碍患者和患者识别提供了一个框架。背景:Caspr2是膜蛋白密切相关电压门控钾通道(VGKC)。Caspr2抗体的临床表现多样,不知道。识别的障碍很重要,因为它是可以治愈的。方法:临床信息和病人的血清和脑脊液样本评估在两个神经免疫学中心在巴塞罗那或鹿特丹。抗体的研究调查使用之前报道的大脑免疫组织化学和细胞化验。临床作者获得的信息或提供的治疗医师在患者'informed同意。结果:38例患者中,89 (percnt)是男性。症状出现时的平均年龄是66岁。疾病的最低点是达到的平均4个月,但在30 (percnt)的患者1年之后了。莫氏综合症最常见的症状包括(29 [percnt]),边缘脑炎(LE)和其他症状(26 [percnt])或勒(16 [percnt])。 Seventy-seven percent of the patients had ≥3 of the following symptoms: encephalopathy, cerebellar symptoms, peripheral nervous hyperexcitability, autonomic dysfunction, insomnia, neuropathic pain and weight loss. The presence of an underlying tumor (usually thymoma) occurred in 19[percnt] of the patients. IgG4 subclass antibodies were present in 94[percnt] of the patients. Full or partial response to treatment occurred in 93[percnt] of the patients; overall, 73[percnt] had a favorable outcome. Relapses occurred in 25[percnt] of the patients. Conclusions: Caspr2 antibodies associate with a treatable disorder that predominantly affects elderly men. The resulting syndrome may vary among patients, but usually includes a set of well-established symptoms. The recognition of this spectrum of symptoms and the consideration of a protracted clinical course are essential for the early diagnosis of this disorder. Prompt immunotherapy and tumor therapy (if needed) often result in improvement. Disclosure: Dr. Sonderen has nothing to disclose. Dr. Arino has nothing to disclose. Dr. Petit-Pedrol has nothing to disclose. Dr. Leypoldt has received personal compensation for activities with Grifols as a speaker. Dr. Körtvelyessy has nothing to disclose. Dr. Lancaster has nothing to disclose. Dr. Wirtz has nothing to disclose. Dr. Schreurs has nothing to disclose. Dr. Sillevis Smitt has nothing to disclose. Dr. Graus has nothing to disclose. Dr. Dalmau has received personal compensation in an editorial capacity from the board of Up-To-Date. Dr. Dalmau has received royalty payments from Memorial Sloan-Kettering Cancer Center. Dr. Dalmau has received research support from Euroimmun. Dr. Titulaer has nothing to disclose.Sunday, April 17 2016, 1:00 pm-3:00 pm ER -