Frequency and characteristics of isolated psychiatric episodes in anti–N-methyl-d-aspartate receptor encephalitis.

Frequency and characteristics of isolated psychiatric episodes in anti–N-methyl-d-aspartate receptor encephalitis.
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DOI:
10.1001/jamaneurol.2013.3216
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发表时间:
2013-09-01
期刊:
影响因子:
29
通讯作者:
Dalmau, Josep
Dalmau, Josep
中科院分区:
医学1区
文献类型:
--
作者:
Kayser, Matthew S.;Titulaer, Maarten J.;Gresa-Arribas, Nuria;Dalmau, Josep

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抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者常出现明显的精神症状。无论是作为疾病的初始表现还是作为复发,孤立的精神疾病发作(没有神经参与的纯精神症状)的频率和类型尚不清楚。目的:确定一组抗NMDAR脑炎患者中孤立精神发作的频率、症状和结局。在5年内确诊的患者的观察性队列(中位数随访2年)。571例抗NMDAR NR1亚单位抗体阳性的患者被纳入研究。抗体研究在宾夕法尼亚大学和巴塞罗那大学进行,临床信息由作者或推荐医生获得。抗NMDAR脑炎和孤立精神症状患者的频率、症状类型和转归。571例患者中有23例(4%)出现孤立的精神症状,5例在发病时,18例在复发期间。在所有23名患者中,年龄(中位数20岁)、性别(91%女性)和肿瘤相关性(43%,卵巢畸胎瘤)与总体人群相似。主要症状包括妄想症(74%)、情绪障碍(70%,通常为躁狂)和攻击性(57%)。脑MRI异常者10/22(45%),脑脊液细胞增多者17/22(77%)。83%的患者在适当的时候接受免疫治疗和肿瘤切除后完全/实质性恢复。复发后,17/18(94%)患者恢复到复发前相似或更好的功能水平。孤立的精神病发作很少见,但可以作为抗NMDAR脑炎的初发或复发发生。认识到这些发作很重要,因为它们对免疫治疗有反应。在新发精神病患者中,脑炎病史、轻微神经症状和/或辅助测试异常应提示筛查NMDAR抗体。
Patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis often develop prominent psychiatric manifestations. The frequency and type of isolated psychiatric episodes (pure psychiatric symptoms without neurological involvement) either as initial presentation of the disease or as relapse are unknown. To determine the frequency, symptoms, and outcome of isolated psychiatric episodes in a cohort of patients with anti-NMDAR encephalitis. Observational cohort of patients diagnosed over a 5 year period (median follow-up 2 years). 571 patients with IgG antibodies against the NR1 subunit of the NMDAR were included in the study. Antibody studies were performed at the Universities of Pennsylvania and Barcelona, and clinical information was obtained by the authors or referring physicians. Frequency, type of symptoms, and outcome of patients with anti-NMDAR encephalitis and isolated psychiatric manifestations. 23/571 patients (4%) developed isolated psychiatric episodes, 5 at disease onset and 18 during relapses. For all 23 patients, age (median 20 years), gender (91% female), and tumor association (43%, ovarian teratoma) were similar to the population at large. Predominant symptoms included, delusional thinking (74%), mood disturbances (70%, usually manic), and aggression (57%). Brain MRI was abnormal in 10/22 (45%) and CSF showed pleocytosis in 17/22 (77%). Eighty three percent of the patients had full/substantial recovery after immunotherapy and tumor resection when appropriate. After relapse, 17/18 (94%) patients returned to a similar or better pre-relapse functional level. Isolated psychiatric episodes are rare but can occur as initial onset or relapse of anti-NMDAR encephalitis. Recognition of these episodes is important because they respond to immunotherapy. In patients with new onset psychosis, history of encephalitis, subtle neurological symptoms, and/or abnormal ancillary tests should prompt screening for NMDAR antibodies.
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