Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas.
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas.
Pediatr Neurol. 2021 Mar;116:20-26. doi: 10.1016/j.pediatrneurol.2020.11.014. Epub 2020 Nov 28.
Autoimmune encephalitis (AE) and acute disseminated encephalomyelitis (ADEM) are immune-mediated brain conditions that can cause substantial neurological sequalae. Data describing the clinical characteristics, treatments, and neurological outcomes for these conditions are needed.
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.
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%).
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.
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，可导致严重的神经后遗症。需要描述这些疾病的临床特征、治疗方法和神经学结局的数据。
这是一项为期九年的单中心回顾性研究，纳入了诊断为 AE 或 ADEM 的儿童患者，出院结局采用改良 Rankin 评分（mRS）进行评估。
共纳入 75 例患者（23 例 ADEM，52 例 AE）。ADEM 患者的磁共振成像异常比例更高（100% vs 60.8%；P<0.001），症状出现至诊断的时间更短（6 天 vs 14 天；P=0.024）。AE 患者的寡克隆带和血清及脑脊液炎症指标更高。几乎所有患者均接受了皮质类固醇治疗，随后进行了血浆置换或静脉免疫球蛋白治疗，两组之间的治疗策略无显著差异。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 vs 13 天），出院时神经功能残疾的比例更高（59.6% vs 34.8%）。
尽管 ADEM 和 AE 患者可能具有相似的临床表现，但我们发现影像学表现、症状持续时间以及实验室和脑脊液特征的频率存在显著差异，有助于鉴别这两种诊断。两组患者均接受了免疫调节治疗的联合治疗，出院时神经功能残疾较为常见。
