Background

Autoimmune encephalitis (AE) and acute disseminated encephalomyelitis (ADEM) are immune-mediated brain conditions that can cause significant neurological sequalae. Data describing the clinical characteristics, treatments, and neurological outcomes for these conditions are needed.

Methods

This is a single-center retrospective review of children diagnosed with AE or ADEM over a nine-year period with discharge outcomes measured by the Modified Rankin Score.

Results

Seventy-five patients (23 with ADEM and 52 with AE) were identified. Patients with ADEM had a higher percentage of abnormal magnetic resonance imaging findings (100% vs 60.8%; P < 0.001) and a shorter time from symptom onset to diagnosis (6 vs 14 days; P = 0.024). Oligoclonal bands and serum and cerebrospinal fluid inflammatory indices were higher in patients with AE. Nearly all patients received corticosteroids followed by plasmapheresis or intravenous immunoglobulin, and treatment strategies did not differ significantly between groups. Second-line immune therapies were commonly used in patients with AE. Finally, patients with AE had trends toward longer hospital lengths of stay (21 vs 13 days) and a higher percentage of neurological disability at hospital discharge (59.6% vs 34.8%).

Conclusions

Although patients with ADEM and AE may have similar presenting symptoms, we found significant differences in the frequency of imaging findings, symptom duration, and laboratory and cerebrospinal fluid profiles, which can assist in distinguishing between the diagnoses. Patients in both groups were treated with a combination of immunomodulating therapies, and neurological disability was common at hospital discharge.

中文翻译：

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免疫介导性脑病住院儿童的临床特征、治疗策略和结局

背景

自身免疫性脑炎 (AE) 和急性播散性脑脊髓炎 (ADEM) 是免疫介导的脑部疾病，可导致严重的神经系统后遗症。需要描述这些疾病的临床特征、治疗和神经系统结果的数据。

方法

这是一项为期 9 年期间诊断为 AE 或 ADEM 的儿童的单中心回顾性研究，出院结果通过改良 Rankin 评分来衡量。

结果

确定了 75 名患者（23 名 ADEM 和 52 名 AE）。ADEM 患者的磁共振成像异常比例较高（100% 对 60.8%；  P  < 0.001），从症状出现到诊断的时间更短（6 天对 14 天；  P  = 0.024）。AE患者的寡克隆带和血清和脑脊液炎症指数较高。几乎所有患者都接受了皮质类固醇，然后接受了血浆置换术或静脉注射免疫球蛋白，治疗策略在各组之间没有显着差异。二线免疫治疗常用于 AE 患者。最后，AE 患者的住院时间更长（21 天对 13 天），出院时神经功能障碍的比例更高（59.6% 对 34.8%）。

结论

尽管 ADEM 和 AE 患者可能有相似的症状，但我们发现影像学发现的频率、症状持续时间以及实验室和脑脊液特征存在显着差异，这有助于区分诊断。两组患者都接受了免疫调节疗法的联合治疗，出院时神经功能障碍很常见。