Seki M, Suzuki S, Iizuka T, Shimizu T, Nihei Y, Suzuki N, Dalmau J
Department of Neurology, School of Medicine, Keio University, Tokyo, Japan.
J Neurol Neurosurg Psychiatry. 2008 Mar;79(3):324-6. doi: 10.1136/jnnp.2007.136473. Epub 2007 Nov 21.
We report an 18-year-old woman with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, who developed psychiatric symptoms, progressive unresponsiveness, dyskinesias, hypoventilation, hypersalivation and seizures. Early removal of an ovarian teratoma followed by plasma exchange and corticosteroids resulted in a prompt neurological response and eventual full recovery. Serial analysis of antibodies to NR1/NR2B heteromers of the NMDAR showed an early decrease of serum titres, although the cerebrospinal fluid titres correlated better with clinical outcome. The patients' antibodies reacted with areas of the tumour that contained NMDAR-expressing tissue. Search for and removal of a teratoma should be promptly considered after the diagnosis of anti-NMDAR encephalitis.
我们报告了一名18岁患有抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的女性,她出现了精神症状、进行性无反应性、运动障碍、通气不足、唾液分泌过多和癫痫发作。早期切除卵巢畸胎瘤,随后进行血浆置换和使用皮质类固醇,导致迅速的神经学反应并最终完全康复。对NMDAR的NR1/NR2B异聚体抗体的系列分析显示血清滴度早期下降,尽管脑脊液滴度与临床结果的相关性更好。患者的抗体与肿瘤中含有表达NMDAR组织的区域发生反应。在抗NMDAR脑炎诊断后,应立即考虑寻找并切除畸胎瘤。