Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.
Department of Neurology, West China Hospital, Sichuan University, Chengdu, China.
J Neuroimmunol. 2021 Apr 15;353:577494. doi: 10.1016/j.jneuroim.2021.577494. Epub 2021 Jan 23.
At present, patients positive for aquaporin-4 antibody (AQP4-Ab) or myelin oligodendrocyte glycoprotein antibody (MOG-Ab) are diagnosed as neuromyelitis optica spectrum disorder (NMOSD) and MOG-Ab-associated diseases, respectively. However, some patients who meet the diagnostic criteria for NMOSD and show demyelination of the central nervous system cannot be clearly classified.
We performed a prospective cohort study to evaluate the clinical characteristics and prognoses of double-seronegative patients with NMOSD.
A total of 594 patients were included in the cohort, including 26 patients with MOG-Ab, 517 with AQP4-Ab, and 51 with double seronegativity. Compared to AQP4-Ab-positive patients, double-seronegative patients experienced less severe clinical attacks (51.0% vs. 78.1%; P < 0.01), either visual (23.5% vs. 42.6%; P = 0.024) or motor attacks (39.2% vs. 59.8%; P = 0.015), and had a better median Expand Disability Status Scale (EDSS) score at the last follow-up (2.0 vs. 3.0; P = 0.012) and a lower proportion of disability (11.8% vs. 30.9%; P = 0.015). Furthermore, lower risks of visual and motor disability were also observed by Kaplan-Meier analyses (P = 0.031 and 0.038, respectively). Both the MOG-Ab and double-seronegative groups had lower frequencies of severe clinical attacks, especially motor attacks, better EDSS scores at the last visit, and a lower proportion of disability than was found in the AQP4-Ab group (all P values and corrected P values <0.05).
In patients who met the diagnostic criteria for NMOSD, compared with AQP4-Ab-seropositive patients, double-seronegative and MOG-Ab-seropositive patients had less severe clinical attacks and better prognoses, including lower EDSS scores and a lower proportion of disability.
目前，AQP4 抗体（AQP4-Ab）或髓鞘少突胶质细胞糖蛋白抗体（MOG-Ab）阳性的患者分别被诊断为视神经脊髓炎谱系疾病（NMOSD）和 MOG-Ab 相关疾病。然而，一些符合 NMOSD 诊断标准且存在中枢神经系统脱髓鞘的患者无法明确分类。
我们进行了一项前瞻性队列研究，以评估 NMOSD 双阴性患者的临床特征和预后。
该队列共纳入 594 例患者，其中 26 例为 MOG-Ab 阳性，517 例为 AQP4-Ab 阳性，51 例为双阴性。与 AQP4-Ab 阳性患者相比，双阴性患者的临床发作更轻（51.0% vs. 78.1%；P < 0.01），无论是视觉（23.5% vs. 42.6%；P = 0.024）还是运动发作（39.2% vs. 59.8%；P = 0.015），末次随访时的中位扩展残疾状况量表（EDSS）评分也更好（2.0 vs. 3.0；P = 0.012），残疾比例更低（11.8% vs. 30.9%；P = 0.015）。Kaplan-Meier 分析还显示，视觉和运动残疾的风险也较低（P = 0.031 和 0.038）。MOG-Ab 阳性和双阴性两组的严重临床发作频率均较低，特别是运动发作，末次随访时 EDSS 评分更好，残疾比例也低于 AQP4-Ab 阳性组（均 P 值和校正 P 值<0.05）。
在符合 NMOSD 诊断标准的患者中，与 AQP4-Ab 阳性患者相比，双阴性和 MOG-Ab 阳性患者的临床发作更轻，预后更好，包括 EDSS 评分更低，残疾比例更低。
