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自身免疫性脑炎的功能恢复:一项前瞻性观察研究。

Functional Recovery in Autoimmune Encephalitis: A Prospective Observational Study.

机构信息

Department of Neurology, Medical University of Graz, Graz, Austria.

Core Facility Computational Bioanalytics, Center for Medical Research, Medical University of Graz, Graz, Austria.

出版信息

Front Immunol. 2021 May 21;12:641106. doi: 10.3389/fimmu.2021.641106. eCollection 2021.

Abstract

BACKGROUND

Prospective observations of functional recovery are lacking in patients with autoimmune encephalitis defined by antibodies against synaptic proteins and neuronal cell surface receptors.

METHODS

Adult patients with a diagnosis of autoimmune encephalitis were included into a prospective registry. At 3, 6 and 12 months of follow-up, the patients' modified Rankin Scale (mRS) was obtained.

RESULTS

Patients were stratified into three groups according to their antibody (Ab) status: anti-NMDAR-Ab (n=12; group I), anti-LGI1/CASPR2-Ab (n=35; group II), and other antibodies (n=24; group III). A comparably higher proportion of patients in group I received plasma exchange/immunoadsorption and second line immunosuppressive treatments at baseline. A higher proportion of patients in group II presented with seizures. Group III mainly included patients with anti-GABAR-, anti-GAD65- and anti-GlyR-Ab. At baseline, one third of them had cancer. Patients in groups I and III had much higher median mRS scores at 3 months compared to patients in group II. A median mRS of 1 was found at all follow-up time points in group II.

CONCLUSIONS

The different dynamics in the recovery of patients with certain autoimmune encephalitides have important implications for clinical trials. The high proportion of patients with significant disability at 3 months after diagnosis in groups I and III points to the need for improving treatment options. More distinct scores rather than the mRS are necessary to differentiate potential neurological improvements in patients with anti-LGI1-/CASPR2-encephalitis.

摘要

背景

缺乏针对突触蛋白和神经元细胞表面受体抗体定义的自身免疫性脑炎患者的功能恢复的前瞻性观察。

方法

将诊断为自身免疫性脑炎的成年患者纳入前瞻性登记处。在随访的 3、6 和 12 个月时,获得患者的改良 Rankin 量表(mRS)。

结果

根据抗体(Ab)状态将患者分为三组:抗 NMDAR-Ab(n=12;I 组)、抗 LGI1/CASPR2-Ab(n=35;II 组)和其他抗体(n=24;III 组)。基线时,I 组中有更高比例的患者接受血浆置换/免疫吸附和二线免疫抑制治疗。II 组中更多的患者出现癫痫发作。III 组主要包括抗 GABAAR、抗 GAD65 和抗 GlyR-Ab 的患者。基线时,其中三分之一的患者患有癌症。与 II 组相比,I 组和 III 组的患者在 3 个月时的中位数 mRS 评分更高。II 组在所有随访时间点的中位数 mRS 均为 1。

结论

某些自身免疫性脑炎患者恢复的不同动态对临床试验具有重要意义。I 组和 III 组中,诊断后 3 个月有相当一部分患者存在严重残疾,这表明需要改进治疗方案。更明确的评分而不是 mRS 是区分抗 LGI1-/CASPR2-脑炎患者潜在神经改善所必需的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37fe/8175889/ebc1bd291195/fimmu-12-641106-g001.jpg

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