表现为最后区综合征的视神经脊髓炎谱系疾病临床分析

摘要

Objective To evaluate the clinical characteristics and the corresponding MRI and laboratory findings in patients with neuromyelitis optica spectrum disorder(NMOSD)associated with area postrema(AP).Methods The study was a retrospective analysis of data from 120 NMOSD patients, and 18 cases were with AP out of these patients, The clinical presentation, MRI changes, serological markers and treatment outcome were reported.Results AP occurred in 18 patients(15%,18/120).AP was the onset symptom in 14(14/18)patients and 3 days to 7 months(median 40 days)later, optic neuritis or myelitis was involved.One patient presented AP after optic neuritis.Three patients(3/18)had AP and myelitis or optic neuritis simultaneously.AP symptom presented as intractable nausea and vomiting, hiccups.Compared to the patients without AP(n=102), the patients with AP(n=18)had shorter duration and fewer numbers of optic neuritis(P<0.05).There was no statistical difference in sex, onset age,course of disease(relapsing or monophasic)and EDSS scores(P>0.05).The MRI revealed flake or linear lesions in medulla.Twelve patients had cervical cord lesions extending to medulla lesions(12/18). Eleven patients had long cord lesions extending more than 3 spinal cords.The AQP4-antibody did not differ in patients with or without AP(14/18 vs 75/102).The symptom of AP was successfully relieved with methylprednisolone.Conclusion AP symptoms/signs are common in patients with NMOSD.Vomiting and hiccups can be the first symptoms.The medulla lesions and the lesions extending to upper cervical cord are unique to NMOSD.Awareness of AP presentations is helpful for early diagnosis and proper treatment to prevent further disability.%目的 研究表现为最后区综合征的视神经脊髓炎谱系疾病(NMOSD)临床特点、影像学特征、实验室检查和预后.方法 回顾性分析2006—2016年在北京积水潭医院神经内科和首都医科大学宣武医院神经内科就诊的120例NMOSD患者,对其中表现为最后区综合征的18例患者分析其临床特点,影像学特征以及与水通道蛋白4(AQP4)抗体的关系.结果 最后区损害组18例(15%,18/120).最后区症状可作为疾病的首发症状(14/18),间隔3 d至7个月(中位数40 d)出现视神经或脊髓受累.视神经炎后出现最后区症状1例.最后区和视神经或脊髓症状同时出现3例(3/18).最后区症状表现为难治性恶心、呕吐、呃逆.最后区损害组(18例)与无最后区损害组(102例)相比,病程较短,视神经受累较少,两组差异有统计学意义(P<0.05).两组性别、起病年龄、单一病程与复发病程比例、EDSS评分差异无统计学意义.脊髓MRI显示延髓病灶呈片状或线样损害,12例患者颈髓延髓交界区受累(12/18),11例伴有超过3个节段的长节段脊髓损害.最后区组AQP4抗体阳性率(14/18),无最后区损害组AQP4抗体阳性率73.5%(75/102),两组差异无统计学意义.激素治疗后最后区症状好转.结论 NMOSD可表现为最后区综合征,呃逆、呕吐可作为首发症状,延髓颈髓延续病灶是较为特异的影像学表现,早期识别有助于诊断和治疗.