抗 NMDA 受体脑炎45例特点和临床转归分析

摘要

Objective To investigate the clinical characteristics of anti-N-methyl-D-aspartate receptor (NMDAR)encephalitis in the group. Methods The clinical data of 45 cases with anti-NMDAR encephalitis at neurology department of Beijing Youanmen Hospital and Beijing Xuanwu hospital from October 2012 to April 2015 were collected. The clinical features and followed-up outcomes were analyzed retrospectively. Results In the study of 45 patients, the outbreak age was between 14-61 years old, the mean age was 32.2±13.24 years. Twenty-seven patients were male, three of who(11%)had an underlying tumors. Eighteen were female, five of who(28%)had ovarian teratomas. Prodiomal symptoms were noted in 23(51%)of patients. The main clinical manifestations include psychiatric symptoms(91%), seizures(76%), involuntary movement(42%), hypoventilation(24%), decreased level of consciousness(47%)and autonomic instability(40%).The CSF routine test and biochemistry panel were abnormal in 89% patients and test for anti-NMDA receptor antibodies in CSF were positive in all patients. The EEG records main show generalized or frontal to temporal slow waves wild mild degree(21%)to middle degree(59%) abnormal. Brain CT or magnetic resonance imaging(MRI)were abnormal in 53% of patients and majority of them showing that T2 or FLAIR hyperintensities within frontal lobes and temporal lobes. All patients were treated with a combination of corticosteroids and IVIg .Six patients were received immunosuppressive agent(Clclophosphamide or Mycophenolate Mofetil). Except one was lost to follow-up, 86% experienced complete or near-complete recovery (modified rankin scale[MRS]0-2). Conclusion The incidence of anti-NMDAR encephalitis is not uncommon in men, and this disease is less associated with tumor. Anti-NMDAR encephalitis is characterized by psychiatric-behavior abnormality, seizures and anti-NMDAR antibody positive in CSF. 79% patients received a substantial recovery by early first-line immunosuppressive treatments. An underlying tumor is associated with more severe clinical course and poor outcome. Patients treated with ovarian teratomas resection cannot prevent from the disease completely, but can relieve the severity of anti-NMDAR encephalitis.%目的：探讨本组抗 NMDA 受体脑炎患者的临床特点。方法收集北京丰台右安门医院和北京宣武医院神经内科2012年10月至2015年4月收治的45例抗 NMDA 受体脑炎患者的临床资料，分析病例特点，随访病情转归。结果本组的45例患者年龄为14~61岁，平均年龄32.2±13.24岁；男性27例，其中3例（11%）合并肿瘤。女性18例，其中5例（28%）合并卵巢畸胎瘤。23例（51%）出现前驱症状。本组45例临床症状表现为精神症状（91%）、癫痫发作（76%）、不自主运动（42%）、中枢性低通气（24%）、意识水平下降（47%）及自主神经功能障碍（40%）。脑脊液（CSF）常规、生化检查阳性率为89%，所有患者 CSF 抗 NMDA 受体抗体阳性。脑电图（EEG）多表现为双额、颞、中央导联为主的轻度（21%）至中度（59%）慢波。53%患者头颅 CT 平扫或 MRI 检查有异常表现，多见于额颞叶 T2-Flair 异常信号。所有患者均给予激素和丙种球蛋白治疗，6例患者接受环磷酰胺或吗替麦考酚治疗。除1例失访，44例患者预后良好（MRS 评分0-2分）者占86％。结论抗 NMDAR 脑炎发病男性并不少见，肿瘤合并率低。以精神行为异常、癫痫发作、CSF 抗 NMDA 受体抗体阳性为其主要临床特点。绝大部分患者 EEG 异常，并与病情严重程度相关。79%患者一线免疫治疗效果良好。8例合并肿瘤患者病情严重且预后不良，畸胎瘤摘除术不能完全预防该病发生，但能减轻病情严重程度。