Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Eur J Neurol. 2021 Apr;28(4):1308-1315. doi: 10.1111/ene.14654. Epub 2020 Dec 11.
Myelitis is an important clinical component of myelin oligodendrocyte glycoprotein antibody (MOG-ab)-associated disease (MOGAD) and aquaporin-4 antibody (AQP4-ab)-positive neuromyelitis optica spectrum disorder (NMOSD). The aim of this work was to evaluate the differentiating features of myelitis between the two diseases.
Myelitis-related clinical and radiologic data from 130 patients with MOGAD and 125 patients with AQP4-ab-positive NMOSD were retrospectively reviewed and compared. A scoring model was established to differentiate MOG-ab-associated myelitis from AQP4-ab-associated myelitis.
Overall, 29.2% (38/130) of patients with MOGAD and 66.4% (83/125) of patients with AQP4-ab-positive NMOSD had ever experienced myelitis. Compared with those with NMOSD, patients with MOGAD exhibited a lower frequency of myelitis, either during the first episode (p < 0.0001) or throughout the disease duration (p < 0.0001). Compared with AQP4-ab-associated myelitis, MOG-ab-associated myelitis manifested a higher male-to-female ratio (p < 0.0001), younger age at disease onset (p = 0.0004), more prodromic influenza-like symptoms (p = 0.030), more prodromic fever (p = 0.0003), more bowel and bladder dysfunction (p = 0.011), less painful tonic spasms (p < 0.0001), and lower Expanded Disability Status Scale scores after treatment (p < 0.0001). On magnetic resonance imaging, lower spinal cord lesions (p = 0.023), short-segment lesions (p = 0.021), conus involvement (p = 0.0001), and H sign (p < 0.0001) were more common in MOG-ab-associated myelitis. A scoring model with a cutoff value of 4 differentiated MOG-ab-associated myelitis from AQP4-ab-associated myelitis with a sensitivity of 87.9% and a specificity of 90.1%.
Myelitis was less commonly observed in MOGAD and exhibited distinct features compared to those of AQP4-ab-positive NMOSD.
脱髓鞘性脑脊髓炎是髓鞘少突胶质细胞糖蛋白抗体（MOG-ab）相关性疾病（MOGAD）和水通道蛋白-4 抗体（AQP4-ab）阳性视神经脊髓炎谱系疾病（NMOSD）的重要临床组成部分。本研究旨在评估这两种疾病的髓鞘炎的鉴别特征。
回顾性分析了 130 例 MOGAD 患者和 125 例 AQP4-ab 阳性 NMOSD 患者的髓鞘炎相关临床和影像学资料，并进行比较。建立评分模型以区分 MOG-ab 相关性髓鞘炎与 AQP4-ab 相关性髓鞘炎。
总体而言，29.2%（38/130）的 MOGAD 患者和 66.4%（83/125）的 AQP4-ab 阳性 NMOSD 患者曾经历过髓鞘炎。与 NMOSD 患者相比，MOGAD 患者髓鞘炎的发生率较低，无论是首发时（p<0.0001）还是整个病程中（p<0.0001）。与 AQP4-ab 相关性髓鞘炎相比，MOG-ab 相关性髓鞘炎表现为更高的男女比例（p<0.0001），发病年龄更年轻（p=0.0004），前驱性流感样症状更常见（p=0.030），前驱性发热更常见（p=0.0003），肠和膀胱功能障碍更常见（p=0.011），痛性痉挛性发作较少（p<0.0001），治疗后扩展残疾状况量表评分较低（p<0.0001）。磁共振成像上，MOG-ab 相关性髓鞘炎脊髓下段病变（p=0.023）、短节段病变（p=0.021）、圆锥受累（p=0.0001）和 H 征（p<0.0001）更常见。截断值为 4 的评分模型对 MOG-ab 相关性髓鞘炎和 AQP4-ab 相关性髓鞘炎的诊断具有 87.9%的敏感性和 90.1%的特异性。
MOGAD 中髓鞘炎较少见，与 AQP4-ab 阳性 NMOSD 相比，具有不同的特征。
