TY - T1的临床特征、治疗和结果的500例Anti-NMDA受体脑炎(PL01.001) JF -神经学乔-神经病学SP - PL01.001 LP - PL01.001六世- 78 - 1补充AU - Maarten Titulaer AU -林首页赛McCracken盟尼Gabilondo Cuellar盟Eugenia马丁斯AU -弗朗西斯克格劳盟丽塔Balice-Gordon盟——约瑟Dalmau Y1 - 2012/04/25 UR - //www.ez-admanager.com/content/78/1_Supplement/PL01.001.abstract N2 -目的:提供的临床特征和治疗指南建议anti-NMDA-receptor脑炎。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;36% <18 and 4% >45 years). 42% had a tumor (95% teratomas). 55% of females >12 years had ovarian teratoma(s) versus 8% <12 years. In patients <12 years old the most frequent initial symptoms were abnormal behavior, seizures, and movement disorders (36%, 35%, 14%), while in adults were abnormal behavior and memory problems (70%, 13%). 90% of patients had >3 of the following: psychiatric symptoms, memory, speech disorders, seizures, dyskinesias, decreased level of consciousness, autonomic instability, or hypoventilation. Within the first month, movement disorders and ataxia were more frequent in children (92% and 17% vs 70% and 2%, p<0.001 both), while memory problems and hypoventilation predominated in adults (84% and 42% vs 68% and 16%, p=0.008 and 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 ER -