Objective:

To compare clinical, diagnostic, management, and outcome factors in children with anti–N-methyl-d-aspartate receptor (NMDAR) encephalitis and a history of herpes simplex encephalitis (HSE) to children with NMDAR encephalitis without a history of HSE.

Methods:

All patients with anti-NMDAR antibodies in cerebrospinal fluid treated at our institution between 2012 and 2019 were identified and divided into those with a history of HSE (HSE+NMDAR group) and those without a history of HSE (NMDAR-only group). Demographic data, clinical characteristics, immunotherapy, and outcome data were collected on all patients and compared between the 2 groups.

Results:

Seventeen patients were identified with anti-NMDAR antibodies in cerebrospinal fluid, 6 of whom had a history of HSE. Mean age in the HSE+NMDAR cohort was significantly younger in the HSE+NMDAR cohort, as 5 of the 6 patients were infants. Of HSE+NMDAR patients, 50% had behavioral symptoms, 67% had movement disorders, and 100% had seizures at disease nadir. In the NMDAR-only group, 100% had behavioral symptoms, 73% had movement disorders, and 73% had seizures at nadir. HSE+NMDAR patients received a median of 1 immunotherapy, compared to a median of 4.5 immunotherapies in the NMDAR-only group.

Conclusion:

Behavioral symptoms were more common in NMDAR-only patients, whereas seizures were more common in HSE+NMDAR patients. Both groups had significant disability at disease nadir, with more improvement in disability over time in the NMDAR-only group. HSE+NMDAR patients received fewer immunotherapies than NMDAR-only patients. Outcomes of infants with HSE appear to primarily reflect sequelae from HSE.

中文翻译：

---

小儿 N-甲基-d-天冬氨酸 (NMDA) 受体脑炎，伴或不伴疱疹性脑炎

客观的：

比较抗N-甲基-d-天冬氨酸受体 (NMDAR) 脑炎和单纯疱疹脑炎 (HSE) 病史儿童与无 HSE 病史的 NMDAR 脑炎儿童的临床、诊断、管理和结局因素。

方法：

确定2012-2019年间在我院治疗的脑脊液中存在抗NMDAR抗体的所有患者，并分为有HSE病史（HSE+NMDAR组）和无HSE病史（仅NMDAR组）。收集所有患者的人口统计学数据、临床特征、免疫治疗和结果数据，并在两组之间进行比较。

结果：

17 名患者在脑脊液中发现抗 NMDAR 抗体，其中 6 人有 HSE 病史。HSE+NMDAR 队列的平均年龄在 HSE+NMDAR 队列中明显更年轻，因为 6 名患者中有 5 名是婴儿。在 HSE+NMDAR 患者中，50% 有行为症状，67% 有运动障碍，100% 在疾病最低点有癫痫发作。在仅 NMDAR 组中，100% 有行为症状，73% 有运动障碍，73% 有最低点癫痫发作。HSE+NMDAR 患者接受的免疫治疗中位数为 1 次，而仅 NMDAR 组的中位数为 4.5 次免疫治疗。

结论：

仅 NMDAR 患者的行为症状更常见，而 HSE+NMDAR 患者的癫痫发作更常见。两组在疾病最低点都有明显的残疾，随着时间的推移，仅 NMDAR 组的残疾改善更大。HSE+NMDAR 患者比仅 NMDAR 患者接受的免疫疗法更少。HSE 婴儿的结局似乎主要反映了 HSE 的后遗症。