Salvucci Alana, Devine Irisa M, Hammond David, Sheth Raj D
Alfred I. duPont Children's Hospital - Pediatric Neurology, Talleyville, Delaware.
Mayo Clinic Florida - Jacksonville, Jacksonville, Florida; Nemours Children's Clinic - Jacksonville, Jacksonville, Florida.
Pediatr Neurol. 2014 May;50(5):507-10. doi: 10.1016/j.pediatrneurol.2014.01.012. Epub 2014 Jan 6.
We report the clinical features and course of pediatric patients presenting with anti-N-methyl D-aspartate receptor (NMDA-R) encephalitis.
Single-center 4-year observational study of pediatric encephalitis associated with NMDA-R antibodies in the serum and/or the cerebrospinal fluid.
Three girls with anti-NMDA-R encephalitis were identified. All presented with an acute hyperkinetic movement disorder and seizures, expressive aphasia, and emotional lability requiring inpatient treatment for 1-3 months. Imaging and electroencephalogram findings were nondiagnostic. None had an underlying tumor or ovarian teratoma. All received immune-modulatory therapy, including one or more of the following: high-dose methyl-prednisolone, plasma exchange, intravenous immunoglobulin or mycophenolate mofetil. Two of the three patients relapsed within 6 months of presentation and required retreatment with plasma exchange. All have remained in subsequent remission, with two of the three requiring second-line immunotherapy with rituximab.
Hyperkinetic movements in pediatric patients presenting with acute encephalopathy and prominent psychiatric symptoms should elicit a search for NMDA-R antibodies early in the evaluation. Relapses require aggressive immunomodulatory treatment for remission. This series highlights a unique positron emission tomography scan finding of hypermetabolism in one of the patients that correlated with her clinical symptoms. Recovery and rehabilitation can be prolonged, often taking years after the initial diagnosis. Early identification and treatment is likely to reduce relapses and limit morbidity associated with this potentially devastating but treatable encephalitis.
我们报告了表现为抗 N-甲基-D-天冬氨酸受体（NMDA-R）脑炎的儿科患者的临床特征及病程。
对血清和/或脑脊液中存在 NMDA-R 抗体的儿科脑炎进行单中心 4 年观察性研究。
确定了 3 名患有抗 NMDA-R 脑炎的女孩。她们均表现为急性运动增多性运动障碍、癫痫发作、表达性失语和情绪不稳定，需要住院治疗 1 - 3 个月。影像学和脑电图检查结果均无诊断意义。无人有潜在肿瘤或卵巢畸胎瘤。所有患者均接受了免疫调节治疗，包括以下一种或多种：大剂量甲泼尼龙、血浆置换、静脉注射免疫球蛋白或霉酚酸酯。3 名患者中有 2 名在发病后 6 个月内复发，需要再次进行血浆置换治疗。此后所有患者均保持缓解状态，3 名患者中有 2 名需要用利妥昔单抗进行二线免疫治疗。
对于出现急性脑病和突出精神症状的儿科患者，其运动增多应在评估早期就促使医生寻找 NMDA-R 抗体。复发需要积极的免疫调节治疗以实现缓解。本系列研究突出了一名患者独特的正电子发射断层扫描结果，即高代谢，这与她的临床症状相关。恢复和康复过程可能会延长，通常在初始诊断后需要数年时间。早期识别和治疗可能会减少复发，并限制与这种潜在致命但可治疗的脑炎相关的发病率。
