You X F, Hu H T, Ye J
Department of Neurology, Beijing Jishuitan Hospital, Beijing 100096, China.
Zhonghua Yi Xue Za Zhi. 2018 Mar 6;98(9):668-672. doi: 10.3760/cma.j.issn.0376-2491.2018.09.009.
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). 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. 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 (=102), the patients with AP (=18) had shorter duration and fewer numbers of optic neuritis(<0.05). There was no statistical difference in sex, onset age, course of disease (relapsing or monophasic) and EDSS scores (>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. 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.
评估与最后区（AP）相关的视神经脊髓炎谱系障碍（NMOSD）患者的临床特征以及相应的MRI和实验室检查结果。本研究对120例NMOSD患者的数据进行回顾性分析，其中18例患者伴有AP，报告了其临床表现、MRI变化、血清学标志物及治疗结果。18例患者（15%，18/120）出现AP。14例（14/18）患者以AP为首发症状，3天至7个月（中位40天）后出现视神经炎或脊髓炎。1例患者在视神经炎后出现AP。3例患者（3/18）同时出现AP和脊髓炎或视神经炎。AP症状表现为顽固性恶心、呕吐、呃逆。与无AP的患者（n = 102）相比，有AP的患者（n = 18）视神经炎的病程更短、发作次数更少（<0.05）。在性别、发病年龄、病程（复发或单相）和扩展残疾状态量表（EDSS）评分方面无统计学差异（>0.05）。MRI显示延髓有片状或线状病变。12例患者颈髓病变延伸至延髓病变（12/18）。11例患者有长节段脊髓病变，延伸超过3个脊髓节段。有无AP的患者水通道蛋白4抗体无差异（14/18 vs 75/102）。甲泼尼龙成功缓解了AP症状。AP症状/体征在NMOSD患者中常见。呕吐和呃逆可能是首发症状。延髓病变以及延伸至上颈髓的病变是NMOSD所特有的。认识AP的表现有助于早期诊断和恰当治疗，以防止进一步残疾。
