新诊断标准下视神经脊髓炎谱系疾病与多发性硬化的 临床特点分析
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国家自然科学基金项目(81301208)


Clinical characteristics of the neuromyelitis eptica spectrum disorders and multiple sclerosis under the new criteria for the diagnosis
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    目的 研究视神经脊髓炎谱系疾病(NMOSD)的一般临床特点及与多发性硬化(MS)的区 别。方法 收集2016 年1 月—2017 年12 月于宣武医院神经内科住院的中枢神经系统脱髓鞘疾病患者, 依据2015 年NMOSD 诊断标准及2017 年McDonald 诊断标准进行筛选,对NMOSD 及MS 患者临床资料进 行回顾性分析,并比较NMOSD 及MS 患者临床特点。结果 纳入NMOSD 患者233 例,MS 患者125 例, 平均发病年龄分别为(43.0±14.7)岁、(38.1±11.2)岁,男女比例分别为1∶4.5、1∶2.6。NMOSD 多以视 神经受损症状起病,顽固性瘙痒、恶心、呕吐可能是其特征性临床特征;MS 则多以脑损伤起病。大多 数NMOSD 和MS 患者的首次发病无明显诱因,可能的诱因有上呼吸道感染、劳累、疫苗接种、产后等。 NMOSD 及MS 脑脊液压力方面无明显区别,相比MS 患者,NMOSD 患者脑脊液白细胞计数高于正常。血 水通道蛋白4 抗体(AQP4-Ab)与脑脊液寡克隆区带(OB)为鉴别二者重要生物标志物。结论 NMOSD 最 多被误诊的疾病是MS,尤其是疾病初期及对于AQP4-IgG 抗体测试阴性的NMOSD 患者。顽固性瘙痒、 恶心、呕吐可能是NMOSD 的一个被低估的特征。血AQP4-Ab 与脑脊液OB 分别为NMOSD 及MS 重要的 生物标志物,对鉴别两种疾病意义重大。

    Abstract:

    Objectives To study the general clinical characteristics of the neuromyelitis eptica spectrum disorders( NMOSD) and the difference from multiple sclerosis( MS). Methods Patients with central nervous system demyelinating diseases admitted to the Neurology Department of Xuanwu Hospital from January 2016 to December 2017 were screened according to the diagnostic criteria of NMOSD( 2015) and the diagnostic criteria of McDonald( 2017). A retrospective analysis was conducted to compare the clinical features of NMOSD and MS patients. Results Totals of 233 NMOSD patients and 125 MS patients were induded. The average onset age of NMOSD and MS were( 43.0±14.7) and( 38.1±11.2) years respectively, and the male to female ratio was( 1/4.5) and( 1/2.6). NMOSD mostly begin with the optic nerve damage symptom. Intractable pruritus and intractable nausea and vomiting may be characteristic clinical features. The MS mostly begin with brain damage. Most NMOSD and MS patients have no obvious causes of first onset. Possible causes are upper respiratory tract infection, fatigue, vaccination, postpartum and so on. There was no significant difference between NMOSD and MS in cerebrospinal fluid pressure. Compared with MS patients, NMOSD patients has higher white blood cell count than normal.Blood AQP4-Ab and cerebrospinal fluid OB were important biomarkers to distinguish from the two diseases. Conclusions NMOSD is the most likely to be misdiagnosed as MS, especially in the early stage of the disease and NMOSD patients with negative AQP-4 antibody test. Intractable itching and intractable nausea and vomiting may be underestimated features of NMOSD. Blood AQP4-Ab and cerebrospinal fluid OB are important biomarkers of NMOSD and MS, which are significant for the identification of the two diseases.

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刘江红 邢怡 高乐虹 侯月.新诊断标准下视神经脊髓炎谱系疾病与多发性硬化的 临床特点分析[J].神经疾病与精神卫生,2019,19(5):
DOI :10.3969/j. issn.1009-6574.2019.05.015.

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  • 在线发布日期: 2019-08-22