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有助于预测急性脑炎患者自身抗体存在的临床变量。

Clinical variables that help in predicting the presence of autoantibodies in patients with acute encephalitis.

机构信息

Department of Neurology, FCM, University of Campinas (UNICAMP), and the Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), Campinas, SP, Brazil.

Neuroimmunology Unit, IB, Department of Genetics, Evolution and Bioagents, University of Campinas (UNICAMP), Campinas, SP, Brazil.

出版信息

Seizure. 2021 Aug;90:117-122. doi: 10.1016/j.seizure.2021.02.023. Epub 2021 Feb 24.

DOI:10.1016/j.seizure.2021.02.023
PMID:33658153
Abstract

OBJECTIVE

To identify clinical variables that could predict the presence of autoantibodies in patients with acute encephalitis.

METHODS

An observational, retrospective study from May 2011 to May 2017. Clinical, EEG, brain MRI data, and antibodies against human neuronal antigens (NMDAR, GABAR, AMPAR, LGI1, CASPR2, and GAD) from 158 patients with criteria for possible autoimmune encephalitis were analyzed to create a predictive model for this disease.

RESULTS

We analyzed 158 samples, of which 18 cases were positive for anti-NMDAR, 2 for anti-LGI1, and 2 for anti-GAD. Seven of the 18 positive NMDAR patients were children, and 12 were female. Behavioral disorder, epileptic seizures, movement disorder, and altered level of consciousness were the frequent symptoms with >75 % sensitivity in positive anti-NMDAR patients. Other symptoms, such as language disorder, psychosis, hypoventilation, altered wake and sleep cycle, and cognitive impairment, had a sensitivity >55 %. Abnormal EEG findings had a high sensitivity (99.4 %). Brain MRI suggestive of encephalitis was observed in 7 of the positive cases for NMDAR. Abnormal CSF findings were reported in 12 patients positive for this receptor (sensitivity 70.6 %). With 7 of these symptoms, we obtained a sensitivity of 70 % and specificity of 81 % for the presence of anti-NMDAR antibodies (ROC Area 82 %). However, to predict that a patient with subacute encephalitis may have an autoimmune cause, the patient should include clinical manifestations such as movement disorder, behavioral disorder, hypoventilation, dysautonomia, and alteration of the wake and sleep cycle. Children were significantly more likely than adults with autoimmune encephalitis to experience chorea and status epilepticus (p < 0.05).

CONCLUSIONS

Anti-NMDAR encephalitis was more frequent in females and children. The repertoire of autoimmune encephalitis in children is different from adults. The presence of subacute behavioral changes, epileptic seizures, movement disorders, altered consciousness, hypoventilation, dysautonomia, and altered wake and sleep cycle predicted autoimmune encephalitis in our series.

摘要

目的

确定可能预测急性脑炎患者自身抗体存在的临床变量。

方法

这是一项 2011 年 5 月至 2017 年 5 月的观察性、回顾性研究。对 158 例符合自身免疫性脑炎标准的患者的临床、脑电图、脑 MRI 数据以及针对人类神经元抗原(NMDAR、GABAR、AMPA、LGI1、CASPR2 和 GAD)的抗体进行分析,为该疾病建立预测模型。

结果

我们分析了 158 例样本,其中 18 例抗 NMDAR 阳性,2 例抗 LGI1 阳性,2 例抗 GAD 阳性。18 例抗 NMDAR 阳性患者中有 7 例为儿童,12 例为女性。行为障碍、癫痫发作、运动障碍和意识改变是抗 NMDAR 阳性患者常见的敏感性超过 75%的症状。其他症状,如语言障碍、精神病、通气不足、觉醒和睡眠周期改变以及认知障碍,敏感性超过 55%。异常脑电图发现具有高敏感性(99.4%)。抗 NMDAR 阳性患者中,7 例出现磁共振成像提示脑炎。12 例抗 NMDAR 阳性患者报告脑脊液异常(敏感性 70.6%)。有 7 种这些症状,我们得到抗 NMDAR 抗体存在的敏感性为 70%,特异性为 81%(ROC 面积 82%)。然而,要预测亚急性脑炎患者可能患有自身免疫性疾病,患者应包括运动障碍、行为障碍、通气不足、自主神经功能障碍和睡眠-觉醒周期改变等临床表现。儿童患自身免疫性脑炎比成人更有可能出现舞蹈病和癫痫持续状态(p<0.05)。

结论

抗 NMDAR 脑炎在女性和儿童中更为常见。儿童自身免疫性脑炎的表现与成人不同。在我们的系列研究中,亚急性行为改变、癫痫发作、运动障碍、意识改变、通气不足、自主神经功能障碍和睡眠-觉醒周期改变的存在预测了自身免疫性脑炎。

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