Gowda Vykuntaraju K, Vignesh Sukanya, Natarajan Balamurugan, Shivappa Sanjay K
Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
Department of Paediatrics, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.
J Pediatr Neurosci. 2021 Jul-Sep;16(3):194-198. doi: 10.4103/jpn.JPN_80_20. Epub 2021 Jul 2.
Anti--methyl-D-aspartate receptor (anti-NMDAR) encephalitis is one of the common causes of treatable encephalitis in children characterized by severe memory deficit, speech disturbances, seizures, autonomic dysfunction, and movement disorders. Hemiparesis/stroke-like episode is not a usual presenting complaint of NMDAR encephalitis. The objective of this study was to report confirmed cases of seropositive anti-NMDAR encephalitis in children who presented with hemiparesis/stroke-like episodes.
Retrospective review of charts of patients with a diagnosis of NMDAR encephalitis was performed at the pediatric neurology department attached to a tertiary care hospital for 6 years from March 2014 to February 2020. Only those case records with NMDAR seropositivity in the cerebrospinal fluid were collected and those who presented with stroke-like episode/hemiparesis were retrieved separately and the data were extracted in a predesigned proforma and analyzed.
Six children of 24 seropositive anti-NMDAR encephalitis presented with hemiparesis/stroke-like episode. All the six patients presented with hemiparesis, behavioral changes, and regression of speech. Three children had seizures and one child had Epilepsia partialis continua. Two children had dystonia and choreoathetosis. Methylprednisolone followed by oral steroids were administered in all patients. Cases 1, 2, and 4 made a full recovery within 7 days, but cases 3, 5, and 6 showed improvement after 20 days following additional IVIG. Four children have cognitive decline and behavioral problems. Case 6 had relapse and recovered with rituximab.
Anti-NMDA receptor encephalitis which is a potentially treatable disease should be considered in the differential diagnosis when a child presents with hemiparesis/stroke-like episode.
抗N-甲基-D-天冬氨酸受体（抗NMDAR）脑炎是儿童可治疗性脑炎的常见病因之一，其特征为严重记忆缺陷、言语障碍、癫痫发作、自主神经功能障碍和运动障碍。偏瘫/类卒中发作并非NMDAR脑炎常见的首发症状。本研究的目的是报告以偏瘫/类卒中发作就诊的血清学阳性儿童抗NMDAR脑炎确诊病例。
对一家三级护理医院儿科神经科2014年3月至2020年2月6年间诊断为NMDAR脑炎患者的病历进行回顾性分析。仅收集脑脊液中NMDAR血清学阳性的病例记录，并单独检索出现类卒中发作/偏瘫的病例，以预先设计的表格提取数据并进行分析。
24例血清学阳性的抗NMDAR脑炎患儿中有6例出现偏瘫/类卒中发作。所有6例患者均出现偏瘫、行为改变和言语退化。3例患儿有癫痫发作，1例患儿有持续性部分性癫痫。2例患儿有肌张力障碍和舞蹈手足徐动症。所有患者均先给予甲泼尼龙，随后给予口服类固醇。病例1、2和4在7天内完全康复，但病例3、5和6在加用静脉注射免疫球蛋白20天后有所改善。4例患儿有认知功能下降和行为问题。病例6复发，使用利妥昔单抗后康复。
当儿童出现偏瘫/类卒中发作时，鉴别诊断应考虑抗NMDA受体脑炎这一潜在可治疗的疾病。
