摘要
儿童抗N-甲基-D-天冬氨酸受体抗体脑炎（anti—N-methyl-D—aspartatereceptorencephalitis，N加AR）是一种抗NMDA受体抗体相关性自身免疫性脑炎。该病发病、临床表现具有儿童其自身特点。成年患者多与肿瘤相关，患儿肿瘤相关性低，多与感染、遗传因素有关。其发病机制主要是抗NMDAR抗体选择性、可逆性的减少神经元突触后膜表面的NMDAR密度。临床主要表现为精神症状、语言障碍、运动障碍、睡眠障碍及自主神经功能紊乱等。治疗以肿瘤切除和免疫治疗为主。该病儿童病例报道少，易误诊，部分患儿病情重，易留下后遗症，故早期诊断、治疗具有重要意义。该文就儿童抗NMDAR脑炎发病机制、临床表现及诊断治疗作一综述。
Anti-N-methyl-D-aspartate receptor encephalitis （Anti-NMDAR-encephalitis）in children is a type of autoimmune encephalitis associated with NMDA receptor. Its etiology and clinical manifestations are spe- cific in children. This disease is associated with teratoma more in adult than in children. However,its etiology is more associated with infection and heredity in children. The pathogenesis is that the anti-NMDAR-antibody can combine with N-MDAR of neurons selectively and reversibly. The clinical manifestations include psychiatric syn- drome,language,motor, sleep and autonomic nervous disorders. Tumor resection and immunotherapy are chief treatments. This disorder is often misdiagnosed as it is rarely reported in children. It is valuable to make early di- agnosis and treatment. This paper reviews the mechanism, clinical features, diagnosis and treatment of anti- NMDAR-encephalitis of children.
出处
《国际儿科学杂志》
2013年第5期481-485,共5页
International Journal of Pediatrics
