McCoy Bláthnaid, Akiyama Tomoyuki, Widjaja Elysa, Go Cristina
The Hospital for Sick Children, Toronto, Ontario, Canada.
J Child Neurol. 2011 Feb;26(2):218-22. doi: 10.1177/0883073810378536. Epub 2010 Dec 23.
Limbic encephalitis, first described in the 1960s as a paraneoplastic condition, has emerged as an autoimmune condition, occurring often without evidence of an underlying tumor. Many novel autoantibodies have been identified, and this diagnosis is increasingly being made in the pediatric population. This article reports the case of a 16-year-old boy who presented following gastrointestinal illness with subacute evolution of neuropsychiatric symptoms. Brain magnetic resonance imaging revealed progressive hippocampal signal abnormality and swelling. N-methyl-D-aspartate (NMDA) receptor antibody was detected in serum. The patient responded well to pharmacological immunotherapy but has residual cognitive deficits. The available literature on this condition is reviewed. Limbic encephalitis should be considered in the differential diagnosis in children presenting with encephalopathy, particularly with neuropsychiatric manifestations. Long-term surveillance and close follow-up are required to accurately clarify tumor risk and natural history of this condition in children and balance these factors with risks of radiation exposure through imaging.
边缘性脑炎最早在20世纪60年代被描述为一种副肿瘤性疾病，如今已成为一种自身免疫性疾病，常无潜在肿瘤的证据。许多新型自身抗体已被识别，且该诊断在儿科人群中越来越常见。本文报告了一名16岁男孩的病例，他在胃肠道疾病后出现神经精神症状的亚急性进展。脑部磁共振成像显示海马信号逐渐异常和肿胀。血清中检测到N-甲基-D-天冬氨酸（NMDA）受体抗体。患者对药物免疫治疗反应良好，但仍有认知缺陷。本文对关于该疾病的现有文献进行了综述。对于出现脑病的儿童，尤其是有神经精神表现的儿童，鉴别诊断时应考虑边缘性脑炎。需要进行长期监测和密切随访，以准确明确儿童患该疾病的肿瘤风险和自然病程，并在这些因素与影像学辐射暴露风险之间取得平衡。
