PDF(Pubmed)
Abstract：
UNASSIGNED: Both myelin oligodendrocyte glycoprotein-IgG associated disorders (MOGAD) and neuromyelitis optica spectrum disorder (NMOSD) are demyelinating diseases of the central nervous system. They present similar clinical manifestations such as optica neuritis, myelitis and area postrema syndrome (APS). The distinctions of optica neuritis (ON) and myelitis between them have been elaborated to great length while their differences in APS remain to be elucidated. We aim to report the frequency of APS in patients with MOGAD as well as NNOSD patients, and to compare the characteristics of APS between patients with MOGAD and those with NMOSD.
UNASSIGNED: Seven MOG-IgG positive APS patients were retrospectively identified between 2017 and 2022. APS phenotypes have been previously described. The similarities and differences between MOGAD and NMOSD patients with APS was compared, including the frequency and duration of APS between the two diseases, and their incidences of accompanied subtentorial lesions have also been described and compared.
UNASSIGNED: We reviewed a cohort of 218 MOG-IgG-positive patients, and 396 patients with NMOSD. 200 MOGAD patients and 332 NMOSD patients were included in this study. In the cohort, seven patients with MOG-IgG-positive antibody presented with APS were analyzed, four of whom had disease onset with APS. Of the 332 patients with NMOSD, 47 had APS attacks while 31 had APS at disease onset. In patients with MOGAD, 2 had nausea, 3 had vomiting, 5 had hiccups, and 1 patient presented with all three symptoms above. In patients with NMOSD, 70.2 % had nausea, vomiting and hiccups at the same time during APS attacks. Apart from the medulla oblongata, other subtentorial regions were also affected in 6/7 MOGAD patients while 14/47 NMOSD patients had other subtentorial regions involved. During an APS attack, the incidence of concomitant lesions in the brainstem and other regions was significantly greater in MOGAD than in the NMOSD cohort (P = 0.008*).
UNASSIGNED: APS is a rare, but not isolated clinical manifestation of MOGAD. APS happened more frequently with other supratentorial and subtentorial lesions in MOGAD. The symptoms of NVH (nausea, vomiting, hiccups) tended to happen respectively in MOGAD compared with NMOSD. The phenotype or mechanism of APS in MOGAD may differ from that in NMOSD.
UNASSIGNED: Seven MOG-IgG positive APS patients were retrospectively identified between 2017 and 2022. APS phenotypes have been previously described. The similarities and differences between MOGAD and NMOSD patients with APS was compared, including the frequency and duration of APS between the two diseases, and their incidences of accompanied subtentorial lesions have also been described and compared.
UNASSIGNED: We reviewed a cohort of 218 MOG-IgG-positive patients, and 396 patients with NMOSD. 200 MOGAD patients and 332 NMOSD patients were included in this study. In the cohort, seven patients with MOG-IgG-positive antibody presented with APS were analyzed, four of whom had disease onset with APS. Of the 332 patients with NMOSD, 47 had APS attacks while 31 had APS at disease onset. In patients with MOGAD, 2 had nausea, 3 had vomiting, 5 had hiccups, and 1 patient presented with all three symptoms above. In patients with NMOSD, 70.2 % had nausea, vomiting and hiccups at the same time during APS attacks. Apart from the medulla oblongata, other subtentorial regions were also affected in 6/7 MOGAD patients while 14/47 NMOSD patients had other subtentorial regions involved. During an APS attack, the incidence of concomitant lesions in the brainstem and other regions was significantly greater in MOGAD than in the NMOSD cohort (P = 0.008*).
UNASSIGNED: APS is a rare, but not isolated clinical manifestation of MOGAD. APS happened more frequently with other supratentorial and subtentorial lesions in MOGAD. The symptoms of NVH (nausea, vomiting, hiccups) tended to happen respectively in MOGAD compared with NMOSD. The phenotype or mechanism of APS in MOGAD may differ from that in NMOSD.
摘要：
髓磷脂少突胶质细胞糖蛋白-IgG相关病症(MOGAD)和视神经脊髓炎谱系病症(NMOSD)都是中枢神经系统的脱髓鞘疾病。他们表现出相似的临床表现，如视神经炎，脊髓炎和后区综合征（APS）。视神经神经炎（ON）和脊髓炎之间的区别已被详细阐述，而它们在APS中的差异仍有待阐明。我们的目的是报告APS在MOGAD患者以及NNOSD患者中的频率。并比较MOGAD患者和NMOSD患者的APS特征。
■在2017年至2022年之间回顾性确定了7例MOG-IgG阳性APS患者。先前已经描述了APS表型。比较MOGAD和NMOSD患者APS的异同,包括APS在两种疾病之间的频率和持续时间，并对其伴发幕下病变的发生率进行了描述和比较。
■我们回顾了218名MOG-IgG阳性患者的队列，396例NMOSD患者。本研究包括200名MOGAD患者和332名NMOSD患者。在队列中，分析了7例出现APS的MOG-IgG抗体阳性患者，其中4人因APS发病。在332名NMOSD患者中，47人患有APS发作，而31人在疾病发作时患有APS。在MOGAD患者中，2有恶心，3有呕吐，5打嗝，1例患者出现上述三种症状。在NMOSD患者中，70.2%有恶心,在APS发作期间同时呕吐和打嗝。除了延髓,6/7MOGAD患者的其他幕下区域也受到影响，而14/47NMOSD患者的其他幕下区域也受到影响。在APS攻击期间，MOGAD中脑干和其他区域伴随病变的发生率明显高于NMOSD队列(P=0.008*).
■APS是一种罕见的，但不是MOGAD的孤立临床表现。MOGAD中其他幕上和幕下病变发生APS的频率更高。NVH的症状（恶心，呕吐,打嗝）与NMOSD相比，在MOGAD中倾向于分别发生。APS在MOGAD中的表型或机制可能与NMOSD中的表型或机制不同。
■在2017年至2022年之间回顾性确定了7例MOG-IgG阳性APS患者。先前已经描述了APS表型。比较MOGAD和NMOSD患者APS的异同,包括APS在两种疾病之间的频率和持续时间，并对其伴发幕下病变的发生率进行了描述和比较。
■我们回顾了218名MOG-IgG阳性患者的队列，396例NMOSD患者。本研究包括200名MOGAD患者和332名NMOSD患者。在队列中，分析了7例出现APS的MOG-IgG抗体阳性患者，其中4人因APS发病。在332名NMOSD患者中，47人患有APS发作，而31人在疾病发作时患有APS。在MOGAD患者中，2有恶心，3有呕吐，5打嗝，1例患者出现上述三种症状。在NMOSD患者中，70.2%有恶心,在APS发作期间同时呕吐和打嗝。除了延髓,6/7MOGAD患者的其他幕下区域也受到影响，而14/47NMOSD患者的其他幕下区域也受到影响。在APS攻击期间，MOGAD中脑干和其他区域伴随病变的发生率明显高于NMOSD队列(P=0.008*).
■APS是一种罕见的，但不是MOGAD的孤立临床表现。MOGAD中其他幕上和幕下病变发生APS的频率更高。NVH的症状（恶心，呕吐,打嗝）与NMOSD相比，在MOGAD中倾向于分别发生。APS在MOGAD中的表型或机制可能与NMOSD中的表型或机制不同。
