视神经脊髓炎谱系疾病14例临床特征分析

Clinical analysis of fourteen cases with neuromyelitis optica spectrum disorders

目的探讨视神经脊髓炎谱系疾病（NMOSD）的临床表现、影像学特征、实验室检查及治疗和预后情况，以提高广大临床医生对该病的认识。方法对2015年6月至2016年6月在郑州大学人民医院神经内科诊治的符合NMOSD诊断标准的14例患者的临床表现、影像学特点、实验室检查及治疗和预后情况等资料进行回顾性分析。结果14例患者，发病年龄15～62岁，平均39．64岁，男：女=3：11。其中1例有甲状腺功能亢进症病史，3例有干燥综合征病史。首发症状为视力减退4例（4／14），恶心、呕吐4例（4／14），肢体麻木无力6例（6／14）。14例患者均进行了脑脊液和血清的抗水通道蛋白4抗体（AQP-4）检测，结果强阳性6例（6／14），阳性7例（7／14），弱阳性1例（1／14）。磁共振（MRI）检查可见广泛大脑皮质、第三脑室、中脑导水管、延髓、桥脑及颈、胸段脊髓内存在异常信号影。14例患者均使用糖皮质激素冲击治疗，其中8例加用免疫抑制剂治疗。随访14例患者，9例患者遗留有不同程度后遗症。结论视神经脊髓炎谱系疾病好发于青壮年女性，对急性或亚急性出现的视物模糊、恶心呕吐、肢体或躯干麻木无力及精神症状等临床表现的患者，需考虑有无视神经脊髓炎谱系疾病的可能。对于临床表现不特殊的患者不能轻易排除视神经脊髓炎谱系疾病的诊断，应尽可能完善相关检查结合最新的诊断标准来做出正确诊断。

Objective To analysis the clinical manifestation, radiological data, laboratory findings of neuromyelitis optica spectrum disorders （NMOSD） to help physicians have a deeper understanding of the disease. Methods The clinical manifestations, radiological data, laboratory findings of NMOSD patients from People＇s Hospital of Zhengzhou University between June 2015 and June 2016 were retrospectively analyzed. Results Fourteen patients with NMOSD were enrolled, consisting of 3 males and 11 females. The onset age ranged from 15 to 62 years, in average 39.64. Among them, there was 1 ease with history of hyperthyroidism, 3 cases with Sjsgren syndrome （SS）. Major clinical manifestations were vision loss in 4 cases, nausea and vomiting in 4 cases, limbs numbness and weakness in 6 cases. All the 14 patients were received the cerebrospinal fluidcell surface antigen antibody detection, and the results were strong positive 6 cases （6/14）, positive 7 cases （7/14）, weak positive 1 case （1/14）. Abnormal signals in the thirdventricle, midbrain aqueduct, oblongata, pons and cervical, thoracic spinal cord were found by Magnetic Resonance Imaging （MRI）. Fourteen patients were treated with glucocorticoid, among them 8 received immunosuppressive therapy. All the 14 patients were followed up, and 9 patients had different degrees of sequelae, including vision decline in 4 cases, numbness and weakness in 5 cases. Conclusions Neuromyelitis optica spectrum disorders frequently occur in young female. Clinicians need to consider the possibility of NMOSD for acute or subacute blurred vision, nausea, vomiting, numbness and weakness of limbs or trunk and psychiatric symptoms. NMOSD can not be easily excluded for those special patients with atypical clinical manifestations, and relevant inspections should be performed as far as possible in order to make the correct diagnosis.