摘要
目的探讨重症抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点及预后。方法回顾性分析9例重症抗NMDAR脑炎患者的临床资料。结果 9例患者(女7例,男2例)均入住ICU治疗;平均发病年龄27.7岁;平均病程22.4 d,ICU平均住院时间50.9 d。主要临床表现有发热(7例)、精神行为异常(9例)、癫痫发作(9例)、意识障碍(8例)、运动功能异常(7例)、自主神经功能障碍(9例)及低通气(6例)。3例合并畸胎瘤。所有患者CSF抗NMDAR抗体均阳性,6例血清抗NMDAR抗体阳性,7例CSF-Ig A升高。3例患者头颅MRI示颞叶或海马异常信号。5例患者EEG示异常慢波。9例患者接受糖皮质激素、丙种球蛋白或血浆置换等免疫调节治疗。5例患者完全恢复,4例患者症状改善伴残留症状。结论重症抗NMDAR脑炎常表现为进展迅速的精神行为改变、癫痫等症状,还有意识障碍、运动障碍、自主神经功能障碍,合并肿瘤并不多见。多数重症抗NMDAR脑炎患者积极免疫治疗预后较好。
Objective To observe the clinical characteristic and prognostic analysis of severe anti-N-methyl-Daspartate receptor（ anti-NMDAR） encephalitis. Methods The clinical data of 9 patients with severe anti-NMDAR encephalitis were retrospective analyzed. Results Nine patients（ two male and seven females） were taken care at ICU. They were 27. 7 years old at average. The median course was 22. 4 d and median length of stay in ICU was 50.9 d. The mainly clinical characteristics were fever（ 7 cases）,psychiatric behavioral symptoms（ 9 cases）,seizures（ 9cases）,decreased consciousness（ 8 cases）,abnormal movement（ 7 cases）,automatic instability（ 9 cases） and hypoventilation（ 6 cases）. Three cases had ovarian teratoma. Anti-NMDAR antibodies in CSF of all patients were testing positive,while in serum were 6 cases. CSF-Ig A in 7 cases were increased. The brain MRI indicated abnormal signals in temporal lobe,hippocampus in 3 patients. The EEG showed slowing wave abnormal in 5 patients. All patients were treated with combined first-line immunomodulatory therapies with steroids,intravenous immunoglobulin,or plasma exchange. Five patients were fullly recoveried,4 patients were improved with residual symptoms.Conclusions Severe anti-NMDAR encephalitis may present progress quickly mental behavior change,epilepsy,and disturbance of consciousness,movement disorders,autonomic nerve dysfunction. Which accompanied with tumor is rare. Most patients with active immunotherapy can get good prognosis.
出处
《临床神经病学杂志》
CAS
北大核心
2016年第2期101-104,共4页
Journal of Clinical Neurology
