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Clinical Characteristics and Prognosis of Antibody-Negative Autoimmune Encephalitis in Children: A Single-Center Retrospective Study
Pediatric Neurology ( IF 3.8 ) Pub Date : 2022-05-25 , DOI: 10.1016/j.pediatrneurol.2022.05.007
Qingyang Dou 1 , Changjian Yang 2 , Maoqiang Tian 2 , Xing Yuan 2 , Renke Li 2 , Xiaomei Shu 2
Affiliation  

Background

Autoimmune encephalitis (AE) is a group of immune-mediated brain diseases. However, new diagnostic criteria for AE in children indicate that partial pediatric patients with AE may be diagnosed without evidence of positive autoantibodies. Therefore, the clinical characteristics and prognosis of children with antibody-negative but probable AE require further investigation.

Methods

Forty-one children with AE admitted to our hospital from April 2014 to January 2021 were retrospectively enrolled in this study. Children were divided into two groups according to positive or negative antibody tests. Clinical characteristics, cerebrospinal fluid, video electroencephalography, brain magnetic resonance imaging, and prognosis were analyzed, and the correlation between modified Rankin scale (mRS) and neutrophil-to-lymphocyte ratio (NLR) was examined.

Results

Of 41 children, 16 cases tested positive for autoantibodies. The main features were psychiatric symptoms, cognitive disturbances, speech disturbances, movement disorders, and seizures. All the children were given a combination of intravenous methylprednisolone pulses with intravenous immunoglobulin therapy; 26 cases (63%) had a good outcome, and 15 cases (37%) had a poor outcome. Antibody-positive and antibody-negative but probable AE were analyzed by univariate analysis and showed lower lymphocyte counts and higher NLR and mRS scores in the antibody-negative group (P < 0.05). The Spearman rank correlation analysis showed a positive correlation between NLR level and mRS scores (P < 0.05).

Conclusions

Antibody-negative but possible AE is frequent in children who may have a more severe neurological impairment and higher NLR than antibody-positive AE. Aggressive immunotherapy in antibody-negative AE is essential to achieve a good prognosis.



中文翻译:

儿童抗体阴性自身免疫性脑炎的临床特征和预后:一项单中心回顾性研究

背景

自身免疫性脑炎(AE)是一组免疫介导的脑部疾病。然而,儿童 AE 的新诊断标准表明,部分儿科 AE 患者可能在没有自身抗体阳性证据的情况下被诊断出来。因此,抗体阴性但可能发生AE的儿童的临床特征和预后需要进一步调查。

方法

回顾性纳入2014年4月至2021年1月我院收治的41例AE患儿。根据抗体检测阳性或阴性,将儿童分为两组。分析临床特征、脑脊液、视频脑电图、脑磁共振成像及预后,并检测改良Rankin量表(mRS)与中性粒细胞与淋巴细胞比值(NLR)的相关性。

结果

41 名儿童中,16 例自身抗体检测呈阳性。主要特征是精神症状、认知障碍、言语障碍、运动障碍和癫痫发作。所有儿童均接受静脉注射甲基强的松龙联合静脉注射免疫球蛋白治疗;26例(63%)预后良好,15例(37%)预后不良。抗体阳性和抗体阴性但可能发生AE的单因素分析显示,抗体阴性组淋巴细胞计数较低,NLR和mRS评分较高(P  < 0.05)。Spearman秩相关分析显示NLR水平与mRS评分呈正相关(P  < 0.05)。

结论

抗体阴性但可能的 AE 常见于可能比抗体阳性 AE 具有更严重神经功能障碍和更高 NLR 的儿童。抗体阴性 AE 的积极免疫治疗对于获得良好预后至关重要。

更新日期:2022-05-25
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