Xu Qiao, Yang Xixi, Qiu Zhandong, Li Dawei, Wang Hongxing, Ye Hong, Jiao Lidong, Zhang Jing, Di Li, Lei Peng, Dong Huiqing, Liu Zheng
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China.
Department of Neurology, The First College of Clinical Medical Science, China Three Gorges University and Yichang Central People's Hospital, Yichang 443000, China.
Mult Scler Relat Disord. 2023 Sep;77:104797. doi: 10.1016/j.msard.2023.104797. Epub 2023 Jun 18.
To assess the characteristics of Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorder (MOGAD) with brainstem involvement in the first event (BSIFE) and make comparisons with aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS).
From 2017 to 2022, this study identified MOG-IgG-positive patients with brainstem or both brainstem and cerebellum lesions in the first episode. As a comparison group, AQP4-IgG-NMOSD (n = 30) and MS (n = 30) patients with BSIFE were enroled.
Thirty-five patients (35/146, 24.0%) were the BSIFE of MOGAD. Isolated brainstem episodes occurred in 9 of the 35 (25.7%) MOGAD patients, which was similar to MS (7/30, 23.3%) but was lower than AQP4-IgG-NMOSD (17/30, 56.7%, P = 0.011). Pons (21/35, 60.0%), medulla oblongata (20/35, 57.1%) and middle cerebellar peduncle (MCP, 19/35, 54.3%) were the most frequently affected areas. Intractable nausea (n = 7), vomiting (n = 8) and hiccups (n = 2) happened in MOGAD patients, but EDSS of MOGAD was lower than AQP4-IgG-NMOSD (P = 0.001) at the last follow-up. MOGAD patients with or without BSIFE did not significantly differ in terms of the ARR (P = 0.102), mRS (P = 0.823), or EDSS (P = 0.598) at the most recent follow-up. Specific oligoclonal bands appeared in MOGAD (13/33, 39.4%) and AQP4-IgG-NMOSD (7/24, 29.2%) in addition to MS (20/30, 66.7%). Fourteen MOGAD patients (40.0%) experienced relapse in this study. When the brainstem was involved in the first attack, there was an increased likelihood of a second attack occurring at the same location (OR=12.22, 95%CI 2.79 to 53.59, P = 0.001). If the first and second events were both in the brainstem, the third event was likely to occur at the same location (OR=66.00, 95%CI 3.47 to 1254.57, P = 0.005). Four patients experienced relapses after the MOG-IgG turned negative.
BSIFE occurred in 24.0% of MOGAD. Pons, medulla oblongata and MCP were the most frequently involved regions. Intractable nausea, vomiting and hiccups occurred in MOGAD and AQP4-IgG-NMOSD, but not MS. The prognosis of MOGAD was better than AQP4-IgG-NMOSD. In contrast to MS, BSIFE may not indicate a worse prognosis for MOGAD. When patients with BSIFE, MOGAD tent to reoccur in the brainstem. Four of the 14 recurring MOGAD patients relapsed after the MOG-IgG test turned negative.
评估首次发病累及脑干的髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）的特征，并与水通道蛋白4-IgG血清阳性的视神经脊髓炎谱系障碍（AQP4-IgG-NMOSD）和多发性硬化症（MS）进行比较。
2017年至2022年，本研究纳入了首次发作时脑干或脑干及小脑均有病变的MOG-IgG阳性患者。作为对照组，纳入了首次发病累及脑干的AQP4-IgG-NMOSD患者（n = 30）和MS患者（n = 30）。
35例患者（35/146，24.0%）为MOGAD首次发病累及脑干（BSIFE）。35例MOGAD患者中有9例（25.7%）出现孤立性脑干发作，这与MS（7/30，23.3%）相似，但低于AQP4-IgG-NMOSD（17/30，56.7%，P = 0.011）。脑桥（21/35，60.0%）、延髓（20/35，57.1%）和小脑中脚（MCP，19/35，54.3%）是最常受累的区域。MOGAD患者出现顽固性恶心（n = 7）、呕吐（n = 8）和打嗝（n = 2），但在最后一次随访时，MOGAD的扩展残疾状态量表（EDSS）低于AQP4-IgG-NMOSD（P = 0.001）。在最近一次随访时，有或无BSIFE的MOGAD患者在年复发率（ARR）、改良Rankin量表（mRS）或EDSS方面无显著差异（P = 0.102、P = 0.823、P = 0.598）。除MS（20/30，66.7%）外，MOGAD（13/33，39.4%）和AQP4-IgG-NMOSD（7/24，29.2%）中出现了特异性寡克隆带。本研究中有14例（40.0%）MOGAD患者复发。当首次发作累及脑干时，第二次发作在同一部位发生的可能性增加（比值比[OR]=12.22，95%置信区间[CI] 2.79至53.59，P = 0.001）。如果第一次和第二次发作均在脑干，则第三次发作很可能发生在同一部位（OR=66.00，95%CI 3.47至1254.57，P = 0.005）。4例患者在MOG-IgG转阴后复发。
24.0%的MOGAD患者首次发病累及脑干。脑桥、延髓和小脑中脚是最常受累的区域。MOGAD和AQP4-IgG-NMOSD患者出现顽固性恶心、呕吐和打嗝，但MS患者未出现。MOGAD的预后优于AQP4-IgG-NMOSD。与MS不同，首次发病累及脑干可能并不表明MOGAD的预后更差。对于首次发病累及脑干的患者，MOGAD倾向于在脑干复发。14例复发的MOGAD患者中有4例在MOG-IgG检测转阴后复发。
