Alqassmi Amal A, Alaklabi Faisal, Alzomor Omar A, Salih Mustafa A
Department of Pediatric Neurology, Children's Hospital, King Saud Medical City, Riyadh, Saudi Arabia.
Department of Infectious Diseases, Children's Hospital, King Saud Medical City, Riyadh, Saudi Arabia.
Sudan J Paediatr. 2021;21(1):76-81. doi: 10.24911/SJP.106-1601560207.
Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an immune-mediated syndrome that is still under-recognised, with grave consequences if not treated early. A multidisciplinary team approach is required in the process of diagnosis and management of this potentially treatable and reversible disorder. We report on a 26-month-old Sudanese girl who presented with focal seizures associated with fever (temperature = 38.9°C) and history of trivial head trauma a day before. Viral encephalitis was suspected, and she was started on acyclovir and ceftriaxone. Cranial computed tomography revealed small high density in the right frontal lobe, and magnetic resonance imaging showed the features of cortical haemorrhagic lesion at the right frontoparietal lobe. Polymerase chain reaction for herpes simplex virus 1 and 2 revealed negative results. Her condition worsened over the course of 1 week, with recurrent seizures, insomnia, violent chorea and orofacial dyskinesia. Electroencephalography showed diffuse slow activity and the presence of 'extreme delta brush' pattern, a specific abnormality seen in anti-NMDA receptor (NMDAR) encephalitis. Cerebrospinal fluid was positive for anti-NMDAR antibodies (titre = 1:100). She was treated with intravenous (IV) corticosteroids, IV immune globulin, plasma exchange and rituximab. Her condition improved gradually, with full recovery when last seen 19 months after the onset of the disease.
抗 N-甲基-D-天冬氨酸（抗 NMDA）受体脑炎是一种免疫介导的综合征，目前仍未得到充分认识，如果不及早治疗会导致严重后果。对于这种潜在可治疗且可逆的疾病，在诊断和管理过程中需要多学科团队协作。我们报告了一名 26 个月大的苏丹女孩，她出现局灶性癫痫发作，伴有发热（体温 = 38.9°C），且一天前有轻微头部外伤史。怀疑为病毒性脑炎，遂开始使用阿昔洛韦和头孢曲松治疗。头颅计算机断层扫描显示右额叶有小的高密度影，磁共振成像显示右额顶叶有皮质出血性病变的特征。单纯疱疹病毒 1 和 2 的聚合酶链反应结果为阴性。在 1 周内她的病情恶化，出现反复癫痫发作、失眠、剧烈舞蹈症和口面部运动障碍。脑电图显示弥漫性慢波活动以及“极端δ刷”模式，这是抗 NMDA 受体（NMDAR）脑炎中可见的一种特定异常。脑脊液抗 NMDAR 抗体呈阳性（滴度 = 1:100）。她接受了静脉注射（IV）皮质类固醇、IV 免疫球蛋白、血浆置换和利妥昔单抗治疗。她的病情逐渐好转，在疾病发作 19 个月后最后一次就诊时已完全康复。
