Encephalitis and AMPA receptor antibodies Novel findings in a case series of 22 patients
Encephalitis and AMPA receptor antibodies Novel findings in a case series of 22 patients
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DOI：
10.1212/wnl.0000000000001682
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发表时间：
2015-06-16
期刊：
影响因子：
9.9
通讯作者：
Dalmau, Josep
中科院分区：
文献类型：
--
作者：
Hoeftberger, Romana;van Sonderen, Agnes;Dalmau, Josep
目的：我们报告了22例新发现的alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic酸受体抗体阳性患者的临床特征、合并症和转归。结果：患者的平均年龄为62岁(23-81岁，女性14例)。表现为边缘脑炎12例(55%)，边缘功能障碍伴多灶性/弥漫性脑病8例(36%)，伴有运动障碍的边缘脑炎1例，双相精神障碍1例。14例(%)经病理证实(肺5例，胸腺瘤4例，乳腺2例，卵巢畸胎瘤2例)或放射学证实(1例肺)。另有7例患者(3例肿瘤相关抗体，2例细胞表面抗体，1例肿瘤相关抗体和1例肿瘤相关抗体)均伴有神经系统症状或肿瘤，反映同时存在自身免疫。21例患者可获得治疗和结果(中位随访72周，范围5-266周)：5例对免疫治疗和肿瘤治疗反应良好，10例部分反应，6例无改善。最终有5名患者死亡；所有患者都有肿瘤或与肿瘤神经抗体相关的副肿瘤症状。神经细胞抗体阳性预示预后不良(p=0.009)。结论：抗AMPAR脑炎多表现为LE，可伴有其他症状或精神障碍，%的病例为副肿瘤。完全和令人印象深刻的神经学改善是可以发生的，但大多数患者都有部分恢复。筛查肿瘤和肿瘤细胞抗体很重要，因为它们的检测会影响结果。
Objective: We report the clinical features, comorbidities, and outcome of 22 newly identified patients with antibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR).Methods: This was a retrospective review of patients diagnosed between May 2009 and March 2014. Immunologic techniques have been reported previously.Results: Patients' median age was 62 years (range 23-81; 14 female). Four syndromes were identified: 12 (55%) patients presented with distinctive limbic encephalitis (LE), 8 (36%) with limbic dysfunction along with multifocal/diffuse encephalopathy, one with LE preceded by motor deficits, and one with psychosis with bipolar features. Fourteen patients (64%) had a tumor demonstrated pathologically (5 lung, 4 thymoma, 2 breast, 2 ovarian teratoma) or radiologically (1 lung). Additional antibodies occurred in 7 patients (3 onconeuronal, 1 tumor-related, 2 cell surface, and 1 tumor-related and cell surface), all with neurologic symptoms or tumor reflecting the concurrent autoimmunity. Treatment and outcome were available from 21 patients (median follow-up 72 weeks, range 5-266): 5 had good response to immunotherapy and tumor therapy, 10 partial response, and 6 did not improve. Eventually 5 patients died; all had a tumor or additional paraneoplastic symptoms related to onconeuronal antibodies. Coexistence of onconeuronal antibodies predicted a poor outcome (p = 0.009).Conclusion: Anti-AMPAR encephalitis usually manifests as LE, can present with other symptoms or psychosis, and is paraneoplastic in 64% of cases. Complete and impressive neurologic improvement can occur, but most patients have partial recovery. Screening for a tumor and onconeuronal antibodies is important because their detection influences outcome.