钱颖　曹秉振

[摘要] 目的 探討抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎的临床表现、磁共振（MRI）图像、脑脊液（CSF）特点及治疗转归。 方法 选取2011年1月～2017年3月济南军区总医院、山东大学齐鲁医院、山东省立医院、山东大学第二医院29例抗NMDAR脑炎患者的临床资料，分析病例特点，随访病情转归。 结果 29例患者年龄为2～70岁，平均（27.83±13.46）岁。其中男性患者12例，2例随访发现肿瘤；女性患者17例，3例随访发现肿瘤。本研究29例患者临床症状表现为精神症状（89.7%）、癫痫发作（69.0%）、运动障碍（65.5%）、中枢性低通气（20.7%）、意识水平下降（44.8%）及自主神经功能障碍（58.6%）。CSF常规、生化检查阳性率为70.0%，96.6%患者血清抗NMDAR抗体阳性，所有患者CSF中抗NMDAR抗体阳性。82.8%出现脑电图异常，多表现为双额、颞、中央导联为主的轻度（20.7%）至中重度（62.1%）慢波。55.2%患者头颅MRI检查有异常表现，多见于额颞叶T2-Flair异常信号。所有患者均给予免疫治疗，5例患者接受利妥昔单抗、环磷酰胺或硫唑嘌呤治疗，1例患者接受血浆置换治疗，1例合并小细胞癌患者行化疗，2例合并畸胎瘤患者行手术治疗。3例患者随访死亡，26例患者预后良好（改良Rankin量表评分0～2分）占89.7%。 结论 CSF抗NMDAR抗体阳性为其主要实验室检查异常项。绝大部分患者脑电图异常，并与病情严重程度相关。大多数患者早期一线免疫治疗效果良好，合并肿瘤的患者及早的手术切除及化疗可以减轻病情，改善预后。

[关键词] 抗N-甲基-D-天冬氨酸受体脑炎；临床表现；脑脊液；核磁共振；治疗转归

[中图分类号] R742.9 [文献标识码] A [文章编号] 1673-7210（2018）09（b）-0054-05

[Abstract] Objective To explore the clinical characteristics of anti-NMDAR encephalitis， including clinical manifestation， magnetic resonance imaging （MRI） findings， cerebrospinal fluid changes （CSF） and treatment outcome. Methods Clinical data of 29 cases with anti-NMDAR encephalitis from January 2011 to March 2017 were collected in four medical centers， including Ji′nan Military General Hospital， Qilu Hospital of Shandong University， Shandong Provincial Hospital， and the Second Hospital of Shandong University. The characteristics of cases were analyzed and the patients were followed up. Results The age of 29 patients was ranged from 2 to 70 years， with a mean value of （27.8±13.46）. There were 12 males and 17 females. Tumors were detected in 2 males and 3 females during the follow-up periods. The clinical manifestations included psychiatric symptoms （89.7%）， seizures （69.0%）， dyskinesia （65.5%）， hypoventilation （20.7%）， recognizant levels drop （44.8%） and autonomic instability （58.6%）. Positive rate of CSF routine and biochemistry tests was 70.0%， 96.6% patients with positive anti-NMDA receptor antibodies in serum， and all patients with positive anti-NMDA receptor antibodies in CSF. The EEG records were abnormal in 82.8% patients， with mild degree （20.7%） and middle and serious degree （62.1%） in generalized or frontal to temporal areas. Abnormal MRI changes were observed in 55.2% patients， in which the most common manifestations were T2-FLAIR hyper intensities in frontal and temporal lobes. All patients were treated with immunotherapy， among of whom 5 patitents received immunosuppressive agent （Rituximab， Cyclophosphamide or Azathioprine）， 1 patitent received plasmapheresis， 1 patitent diagnosed with small cell carcinoma received chemotherapy， 2 patitents were diagnosed with teratoma and received surgical resection. Death occurred in 3 patitents - during the follow-up period， the remaining 26 （89.7%） cases experienced well recoveries （modified Ranking scale 0-2）. Conclusion In anti-NMDAR encephalitis patients， the positive anti-NMDAR antibody in CSF is the most common laboratory findings. The abnormal electroencephalogram （EEG） records occur in most cases and correlated with the severity of anti-NMDAR encephalitis. Most patients receive a substantial recovery by early stage first-line immunosuppressive treatments. Surgical resection and chemotherapy improve prognosis for the cases with tumors.

[Key words] Anti-N-methyl-D-aspartate receptor encephalitis； Clinical features； CSF； MRI； Treatment outcom

抗N-甲基-D-天冬氨酸受体（N-methyl-D-aspartate receptor，NMDAR）脑炎属于自身免疫性脑炎的一种，最早由Dalmau等[1-2]因在患者体内发现抗海马和前额叶神经细胞的NMDAR抗体而提出。……