Ito Yasuo, Abe Tatsuya, Tomioka Ryo, Komori Tetsuo, Araki Nobuo
Department of Neurology, Faculty of Medicine, Saitama Medical University.
Rinsho Shinkeigaku. 2010 Feb;50(2):103-7. doi: 10.5692/clinicalneurol.50.103.
A 19-year-old female in her 2nd trimester (17 weeks) of pregnancy became irritable a few days before admission. She became unable to open her mouth and could not talk. She was admitted to the psychiatric hospital due to a rapid change in behavior and a consciousness disturbance. She was diagnosed as having schizophrenia by a psychiatrist. Her EEG showed diffuse high voltage and slow waves. Acute encephalitis was then suspected. Her past and family histories were not suggestive of viral infection. On physical examination, she had a low grade fever. She had hyperhidrosis, autophagia, and repeated oral dyskinesia. Her consciousness level fluctuated from somnolence to stupor. Although her blood CRP level was mildly elevated and she had mild pleocytosis, HSV-PCR was negative in the cerebrospinal fluid (CSF). Abdominal ultrasound examination and MRI showed no ovarian teratoma. Computed tomography (CT) and magnetic resonance imaging (MRI) showed no brain abnormalities. Before analysis for specific nervous system antibodies, the initial diagnosis was non-herpetic limbic encephalitis. She was twice treated with a 6-day course of methylprednisolone (500 mg/day) infusion. She was also given phenobarbital since she had a tonic-clonic seizure about 1 month after admission. Finally, she had a normal delivery at 37 weeks. The baby was healthy, and the patient was discharged without sequelae. We concluded that her diagnosis was anti-N-methyl-D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis based on the presence of anti-NMDAR antibody in the CSF. This report is the first description of a patient with anti-NMDAR antibody encephalitis. The precise mechanism of this encephalitis is not clear, although there have been several reports of autoimmune encephalitis during pregnancy. The patient's CSF anti-NMDAR antibody titer during treatment was measured. Before treatment, the CSF anti-NMDAR antibody titer was strongly positive, but it decreased during treatment and then disappeared after delivery. We hypothesized that the presence of the embryo or placenta may have triggered an antigenic signal and/or antibody through inappropriate immunological modulation.
一名19岁处于妊娠中期(17周)的女性在入院前几天变得烦躁易怒。她无法张口且不能说话。由于行为迅速改变和意识障碍,她被收治入精神病院。精神科医生诊断她患有精神分裂症。她的脑电图显示弥漫性高电压和慢波。随后怀疑是急性脑炎。她的既往史和家族史均未提示病毒感染。体格检查时,她有低热。她多汗、有自伤行为且反复出现口腔运动障碍。她的意识水平在嗜睡和昏迷之间波动。尽管她的血液CRP水平轻度升高且有轻度脑脊液细胞增多,但脑脊液(CSF)中的单纯疱疹病毒聚合酶链反应(HSV-PCR)为阴性。腹部超声检查和磁共振成像(MRI)未显示卵巢畸胎瘤。计算机断层扫描(CT)和磁共振成像(MRI)未显示脑部异常。在分析特定神经系统抗体之前,初步诊断为非疱疹性边缘叶脑炎。她接受了两次甲泼尼龙(500毫克/天)静脉输注,疗程为6天。由于她在入院约1个月后出现强直阵挛发作,还给予了苯巴比妥。最后,她在37周时顺产。婴儿健康,患者出院时无后遗症。基于脑脊液中存在抗N-甲基-D-天冬氨酸(NMDA)受体(抗NMDAR)抗体,我们得出结论,她的诊断为抗NMDAR脑炎。本报告是首例抗NMDAR抗体脑炎患者的描述。尽管已有多篇关于妊娠期自身免疫性脑炎的报道,但这种脑炎的确切机制尚不清楚。对治疗期间患者脑脊液中的抗NMDAR抗体滴度进行了检测。治疗前,脑脊液抗NMDAR抗体滴度呈强阳性,但在治疗期间下降,分娩后消失。我们推测胚胎或胎盘的存在可能通过不适当的免疫调节引发了抗原信号和/或抗体。