2015年视神经脊髓炎谱系疾病诊断标准临床应用评估

目的 评估2015年视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)诊断标准的临床应用.方法 回顾性分析作者医院2008-3-2016-8收治的605例中枢神经系统脱髓鞘疾病患者临床资料,并分别采用2015年NMOSD诊断标准及2006年NMO诊断标准对其进行诊断分析.结果 共有176例患者符合2015年NMOSD诊断标准,其中108例(61.4％)符合2006年NMO诊断标准.AQP4-IgG阳性患者139例(79.0％).当假设该139例患者AQP4-IgG水平未知时,共有91例(65.5％)符合2015年NMOSD诊断标准,而仅有55例(39.6％)满足2006年NMO诊断标准(P＜0.05).176例患者中有39例同时满足2006年NMO诊断标准和201 5年NMOSD诊断标准且以临床孤立病灶起病,在疾病早期通过2006年NMO诊断标准尚不能与多发性硬化相鉴别,但已符合2015年NMOSD诊断标准.在核心症状方面,NMOSD患者中以急性脊髓炎(50.6％)和视神经炎(31.8％)起病者所占比例最高,其次为最后区综合征(5.7％).结论 2015年NMOSD诊断标准较2006诊断标准扩大了疾病的诊断范围,且在AQP4-IgG水平未知的情况下仍具有更高的诊断敏感性,对于疾病早期与多发性硬化的鉴别中具有重要作用,且更加强调了疾病的核心症状.

Clinical application evaluation of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorder

Objective To evaluate the clinical application of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorder (NMOSD).Methods A retrospective analysis of the clinical data was performed in 605 cases with central nervous system demyelinating diseases in our hospital from March 2008 to August 2016.The 2015 NMOSD diagnostic criteria and 2006 NMO criteria were used respectively to analyze the cases.Results A total of 176 patients fulfilled the diagnostic criteria of 2015 criteria and 108 patients (61.4％) met the 2006 NMO criteria.AQP4-IgG positive patients were 139 (79.0％).And when we assumed an unknown AQP4-IgG status of these cases,91 (65.5 ％) patients were diagnosed with NMOSD by 2015 diagnostic criteria,but only 55 (39.6 ％) patents met the 2006 diagnostic criteria (P ＜0.05).In addition,39 of 176 patients were diagnosed with NMOSD by 2015 criteria,before distinguished from multiple sclerosis using 2006 diagnostic criteria in the early stages of disease.In terms of the core clinical characteristics of all patients with NMOSD,acute myelitis (50.6％) and optic neuritis (31.8％) were the most common initial clinical manifestation,followed by area postrema syndrome (5.7％).Conclusions The 2015 diagnostic criteria for NMOSD extended the diagnostic scope of the spectrum disorder and had more sensitive effect of diagnosis even in patients without AQP4-1gG result,compared with the 2006 criteria.Furthermore,the criteria had considerable effect for early identification of disease and emphasized the core clinical characteristics clearly.