Leel Nellihela, Thakkar Hemanshoo S, Drake David, Bouhadiba Nordeen
Department of Paediatric Surgery, Guy's and St Thomas's NHS Foundation Trust, London, UK.
Paediatric Surgery, Evelina Children's Hospital, London, UK.
BMJ Case Rep. 2018 Mar 13;2018:bcr-2017-220333. doi: 10.1136/bcr-2017-220333.
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immune-mediated encephalopathy with a known association with ovarian teratomas. We present the first case in the UK of a 7-year-old patient presenting with this condition. Having been previously fit and healthy, the patient presented with an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported diffuse background slowing, supporting a diagnosis of encephalopathy. Extensive autoimmune screening was done and found to be positive for anti-NMDAR antibodies. A whole body MRI found a right adnexal lesion consistent with ovarian teratoma. At laparoscopy, the right ovary was excised and histology confirmed a mature cystic teratoma with neural tissue. There were no postoperative complications with the patient's neurology recovering progressively at follow-up.
抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种副肿瘤性、免疫介导的脑病，已知与卵巢畸胎瘤有关。我们报告了英国首例患此病的7岁患者。该患者此前身体健康，此次急性起病，出现认知障碍。最初怀疑是感染性脑病；然而，腰椎穿刺结果为阴性。脑电图报告显示弥漫性背景减慢，支持脑病诊断。进行了广泛的自身免疫筛查，发现抗NMDAR抗体呈阳性。全身MRI发现右侧附件区有一个与卵巢畸胎瘤相符的病变。在腹腔镜检查中，切除了右侧卵巢，组织学证实为含有神经组织的成熟囊性畸胎瘤。术后无并发症，随访时患者神经功能逐渐恢复。
