% 0期刊文章% Maarten Titulaer %林赛McCracken %一个尼Gabilondo Cuellar %一个弗朗西斯克格劳Eugenia马丁斯·% % Josep Dalmau Rita Balice-Gordon % % T临床特点,治疗,和500名患者的结果Anti-NMDA受体脑炎(PL01.001) % D J神经病学2012% % P PL01.001-PL01.001 % V 78% N 1补充% X目的:提供的临床特征和治疗指南建议anti-NMDA-receptor脑炎。首页背景Anti-NMDA-receptor脑炎是最常见的特征和最佳抗体介入脑炎。我们提供的临床特征、治疗和随访的500例患者。设计/方法:队列研究,分析人口、发病、治疗和长期随访。结果:82%是女性。平均年龄为21岁(范围1 - 85;< 18 - 4% > 45年36%)。有42%的肿瘤(95%畸胎瘤)。55%的女性> 12年卵巢畸胎瘤(s)和8% < 12年。< 12岁的病人最常见的初期症状是异常行为,癫痫,和运动障碍(36%、35%、14%),而在成人异常行为和记忆问题(70%,13%)。90%的患者进行了> 3以下:精神病症状,记忆、言语障碍、癫痫、运动困难,减少的意识水平,自主不稳定,或肺换气不足。在第一个月,运动障碍和共济失调儿童更频繁(92%和17% vs 70%和2%,p < 0.001),而记忆问题和肺换气不足成为主流成人(84%和42% vs 68%和16%,p = 0.008, p < 0.001)。 Immunotherapy (93%) and tumor removal (when appropriate) resulted in full recovery or substantial improvement in 61% of patients at 8 months, and 77% at 24 month follow-up; 7% died. Early treatment (1st month) led to better outcome (75% vs 64%, p=0.001). If 1st line immunotherapy (steroids, immunoglobulins and/or plasma exchange) failed, 2nd line treatment (rituximab or cyclophosphamide) significantly improved outcome compared with no treatment or repeating 1st line drugs (56% vs 27%, p=0.006). Relapses occurred in 14%, 73% of them in patients without teratoma.Conclusions: Anti-NMDA-receptor encephalitis is a severe but treatable disorder of predominantly young individuals. Prompt treatment improves outcome. If initial immunotherapy fails, second-line treatment is usually effective. 75% of patients have full/substantial improvement although the process of recovery can take >24 months.Supported by: In part by grants from the National Institutes of Health and National Cancer Institute RO1CA89054 (Dalmau), 1RC1NS068204-01 (Balice-Gordon and Dalmau), a McKnight Neuroscience of Brain Disorders award (Balice-Gordon and Dalmau), a KWF fellowship 2009-4451 of the Dutch Cancer Society (Titulaer), and a grant from the Fondo de Investigaciones Sanitarias, FIS, Spain (Martinez-Hernandez).Disclosure: Dr. Titulaer has nothing to disclose. Dr. McCracken has nothing to disclose. Dr. Gabilondo Cuellar has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Dr. Graus has nothing to disclose. Dr. Balice-Gordon has nothing to disclose. Dr. Dalmau has received personal compensation in an editorial capacity for Up To Date, Inc.Dr. Dalmau has received research support from Euroimmun.Wednesday, April 25 2012, 09:00 am-12:00 pm %U