González-Toro M Cristina, Jadraque-Rodríguez Rocío, Sempere-Pérez Ángela, Martínez-Pastor Pedro, Jover-Cerdá Jenaro, Gómez-Gosálvez Francisco
Hospital General Universitario de Alicante, 03010 San Vicente del Raspeig, Espana.
Rev Neurol. 2013 Dec 1;57(11):504-8.
Encephalitis associated to anti-N-methyl D-aspartate (NMDA) receptor antibodies is an autoimmune neurological pathology that has been reported increasingly more frequently in the paediatric population in recent years. We report two cases from our own experience with similar clinical pictures.
Case 1: a 5-year-old girl who began with clinical signs and symptoms of convulsions and altered consciousness, associated to movement disorders and regression of previously acquired abilities that developed into autism. Case 2: a 13-year-old girl who presented left-side hemiparesis, abnormal movements, conduct disorder and dysautonomia. In both cases positive anti-NMDA receptor antibodies were obtained in cerebrospinal fluid and they were diagnosed with anti-NMDA receptor encephalitis. In the first case, treatment was established with intravenous perfusion of corticoids and immunoglobulins, and rituximab also had to be associated. In the second case, treatment consisted in corticoids and immunoglobulins. Progress was favourable in both cases, with a slight language disorder as a sequela in the first case and a relapse in the second case, with full resolution.
Anti-NMDA receptor encephalitis is a treatable disorder and early diagnosis and treatment are crucial, since this improves the prognosis and diminishes the chances of relapses.
抗N-甲基-D-天冬氨酸(NMDA)受体抗体相关脑炎是一种自身免疫性神经病理学疾病,近年来在儿科人群中的报道越来越频繁。我们报告两例来自我们自身经验的具有相似临床表现的病例。
病例1:一名5岁女孩,最初出现惊厥和意识改变的临床症状和体征,伴有运动障碍以及先前获得的能力退化,发展为自闭症。病例2:一名13岁女孩,出现左侧偏瘫、异常运动、行为障碍和自主神经功能障碍。两例患者脑脊液中均检测到抗NMDA受体抗体阳性,均被诊断为抗NMDA受体脑炎。第一例患者采用静脉输注皮质类固醇和免疫球蛋白进行治疗,还必须联合使用利妥昔单抗。第二例患者的治疗包括皮质类固醇和免疫球蛋白。两例患者病情进展均良好,第一例患者遗留轻微语言障碍后遗症,第二例患者复发,但最终完全康复。
抗NMDA受体脑炎是一种可治疗的疾病,早期诊断和治疗至关重要,因为这可改善预后并减少复发几率。