视神经脊髓炎谱系疾病相关极后区综合征临床研究

A clinical study of area postrema syndrome of neuromyelitis optica spectrum disorders

目的通过对水通道蛋白4(aquaporin protein-4,AQP4)抗体阳性及阴性的视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)相关极后区综合征(area postrema syndrome,APS)患者的临床表现、实验室和影像学检查、治疗和预后的分析研究,提高临床对APS的认识和早期诊治能力。方法回顾性分析2016年4月至2021年12月在广西医科大学第一附属医院神经内科治疗并持续随访,以原因不明性的持续性恶心、呕吐、呃逆等为早期主要或首发症状而确诊NMOSD的APS患者,根据AQP4抗体结果,分为AQP4抗体阳性组和AQP4抗体阴性组,比较分析临床表现、实验室和影像学检查、治疗和预后。结果纳入NMOSD相关APS的41例患者中,AQP4抗体阳性32例(78.05%),阴性9例(21.95%)。AQP4抗体阳性组16例和阴性组1例首次发病为孤立极后区症状(16/32 vs.1/9,P=0.039)。所有AQP4抗体阴性组及96.88%(31/32)的阳性组头颅MRI可见延髓背侧极后区病变。在单独使用激素冲击并序贯减量治疗,以及使用激素联合免疫抑制剂治疗的患者比例和有效率上,阳性组和阴性组之间比较均无统计学差异(P>0.05)。AQP4抗体阳性组和阴性组的疾病高峰期与免疫治疗1个月后缓解期扩展残疾状况评分(extended disability status score,EDSS)之差分别为1.0(1.0,1.5)分和1.5(0.5,2.0)分,二者存在统计学差异(P=0.032);经6~48个月[中位数40.5(28.5,45.8)个月]随访,AQP4抗体阳性组和阴性组分别有8例和2例复发,无统计学差异(P>0.05)。结论AQP4抗体阳性患者首次发病为孤立极后区综合征的比例高,尽早完善AQP4抗体和MRI检查有利于疾病的早期诊断和预后判断;无论是否存在AQP4抗体,急性期使用免疫治疗可改善患者预后,但仍存在较高的复发率。

Objective To improve the clinical understanding and early diagnosis of posterior region syndrome(APS) though retrospectively analyzing the data of clinical manifestations,laboratory and imaging examination,treatment and prognosis of patients with positive and negative aquaporin 4(AQP4) IgG antibody associated APS of neuromyelitis optica spectrum disease(NMOSD).Methods From April 2016 to December 2021,APS patients with unexplained persistent nausea,vomiting,hiccup,etc.,who were diagnosed with NMOSD in the Department of Neurology of the First Affiliated Hospital of Guangxi Medical University were retrospectively analyzed.According to AQP4 antibody results,the patients were divided into AQP4 antibody positive group and AQP4 antibody negative group.The clinical manifestations,laboratory and imaging examinations,treatment and prognosis were compared and analyzed.Results Among 41 cases of NMOSD-associated APS,32 cases(78.05%) were positive for AQP4 antibody and 9 cases(21.95%) were negative.Sixteen cases in the AQP4 antibody positive group and one case in the negative group presented isolated symptoms of posterior polar region at first onset(16/32,50% vs.1/9,11.11%,P=0.039).All AQP4 antibody negative group and 96.88%(31/32)positive group showed lesions in the dorsal pole region of medulla oblongata on MDI.There were no statistically significant differences between the positive and negative groups in the usage and effective rates of intravenous methylprednisolone therapy(IVMP) with sequential reduction therapy alone,as well as those of IVMP combined with immunosuppressive therapy(P>0.05).The difference points of expanded disability status scale(EDSS) of the disease peak and immunotherapy after 1 month remission between AQP4 antibody positive group and negative group were 1.0(1.0,1.5) and 1.5(0.5,2.0)respectively.There was a difference between them(P=0.032).After 6-48 months of follow-up [median 40.5(28.50,45.75)months],8 cases and 2 cases relapsed in AQP4 antibody positive group and AQP4 antibody negative group respectively,with no statistical difference(P>0.05).Conclusion Patients with positive AQP4 antibody has a high incidence of isolated ASP at the first onset.It is necessary to improve the AQP4 antibody and MRI examination as soon as possible,which is beneficial to the early diagnosis and prognosis of the disease.Regardless of the presence or absence of AQP4 antibody,immunotherapy in the acute phase can improve the prognosis of patients,but there is still a high recurrence rate.