Kim Eunsil, Park Eu Gene, Lee Jiwon, Lee Munhyang, Kim Jihye, Lee Jeehun
Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Pediatrics, Uijeongbu St. Mary's Hospital, The Catholic University School of Medicine, Seoul, Korea.
J Epilepsy Res. 2018 Jun 30;8(1):41-48. doi: 10.14581/jer.18007. eCollection 2018 Jun.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a rare autoimmune disorder manifesting as seizures, movement disorders, and psychiatric changes. However, there have been few case reports concerning this disorder in South Korean children. The current case report describes a pediatric patient with anti-NMDAR encephalitis. A 13-year-old female patient developed clonic movements of the right arm followed by aphasia, paresthesia, and right-sided hemiparesis. The electroencephalogram (EEG) results indicated electroclinical seizures arising from the left temporal area. Brain magnetic resonance imaging (MRI) revealed high signal intensity and cortical swelling in left temporal lobe. Anti-NMDAR antibodies were detected in the cerebrospinal fluid (CSF). The patient was treated with intravenous immunoglobulin and high-dose methylprednisolone and showed partial improvement in language skills, paresthesia, and motor power. The brain MRI and EEG results also indicated improvement. However, anti-NMDAR antibodies persisted in the CSF. After four doses of rituximab, the patient exhibited complete recovery of language and motor skills, and was seizure free under treatment with antiepileptic medication. There were no residual anti-NMDAR antibodies in the CSF at her 24-month follow-up visit. This case report elucidates the benefits of early intervention using rituximab to improve neurological deficits and achieve baseline recovery in patients with anti-NMDAR encephalitis.
抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种罕见的自身免疫性疾病，表现为癫痫发作、运动障碍和精神状态改变。然而，关于韩国儿童患此病的病例报告很少。本病例报告描述了一名患抗NMDAR脑炎的儿科患者。一名13岁女性患者出现右臂阵挛性运动，随后出现失语、感觉异常和右侧偏瘫。脑电图（EEG）结果显示癫痫发作源自左侧颞叶区域。脑磁共振成像（MRI）显示左侧颞叶有高信号强度和皮质肿胀。脑脊液（CSF）中检测到抗NMDAR抗体。该患者接受了静脉注射免疫球蛋白和大剂量甲泼尼龙治疗，语言技能、感觉异常和运动能力有部分改善。脑MRI和EEG结果也显示有改善。然而，脑脊液中抗NMDAR抗体仍然存在。在使用4剂利妥昔单抗后，患者语言和运动技能完全恢复，在接受抗癫痫药物治疗期间无癫痫发作。在其24个月的随访中，脑脊液中没有残留抗NMDAR抗体。本病例报告阐明了使用利妥昔单抗进行早期干预对改善抗NMDAR脑炎患者神经功能缺损并实现基线恢复的益处。
