Su Lei, Kuchling Joseph, Gao Chenyang, Niendorf Thoralf, Finke Carsten, Zhang Zhe, Guo Ai, Jing Jing, Tian De-Cai, Li Yu-Jing, Zhang Mengting, Shi Xiaoyu, Liu Xinyao, Wang Huabing, Liu Yaou, Chien Claudia, Levy Michael, Duan Yunyun, Paul Friedemann, Shi Fu-Dong
Tiantan Neuroimaging Center of Excellence, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Ann Clin Transl Neurol. 2025 Jul;12(7):1431-1441. doi: 10.1002/acn3.70080. Epub 2025 May 22.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can radiographically mimic multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). The disease hallmarks cortical lesion, central vein sign (CVS) and paramagnetic rim lesions identified in MS have not yet been comprehensively investigated in MOGAD.
We have characterized 45 patients with MOGAD using 7.0 Tesla (7 T) MRI at two academic research hospitals in China and Germany. 7 T MRI, laboratory, and clinical data were collected. The classification of cortical lesions, proportion of CVS, and the phase shifts of lesions on susceptibility weighted imaging were analyzed.
Of the 45 patients enrolled with MOGAD, 282 lesions were identified. We further detected 31 (11%) cortical lesions including leukocortical, intracortical, and subpial types, of which intracortical lesions (16/31, 52%) were frequently involved. CVS was identified in 53 (19%) lesions of 21 (47%) patients, 154 (55%) lesions showed multiple veins sign (MVS) in 30 (67%) patients. The number (4.3 ± 6.0 vs. 1.5 ± 2.1, p = 0.0049) and percentage (52% vs. 18%, p < 0.0001) of MVS lesions for each MOGAD patient were higher than those of CVS. Eight patients (18%) had 39 (14%) lesions of hypointense signal with paramagnetic phase shifts on SWI, showing nodular phase changes and irregular borders in appearance.
In our observational MOGAD cohort, all three types of cortical lesions were recognized, with intracortical lesions being the most common. The number and proportion of lesions with MVS were higher than those with CVS. Lesions with paramagnetic phase changes were rare and non-rim-like in appearance. These findings provide a better understanding of the underlying pathology of MOGAD and will help in the differentiation of MOGAD from other demyelinating disorders.
髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)在影像学上可模仿多发性硬化(MS)和视神经脊髓炎谱系障碍(NMOSD)。MS中识别出的疾病特征性皮质病变、中央静脉征(CVS)和顺磁性边缘病变在MOGAD中尚未得到全面研究。
我们在中国和德国的两家学术研究医院对45例MOGAD患者进行了7.0特斯拉(7T)磁共振成像(MRI)检查。收集了7T MRI、实验室及临床数据。分析了皮质病变的分类、CVS的比例以及病变在磁敏感加权成像上的相位偏移。
在纳入研究的45例MOGAD患者中,共识别出282个病变。我们进一步检测到31个(11%)皮质病变,包括白质皮质、皮质内和软脑膜下类型,其中皮质内病变(16/31,52%)最为常见。21例(47%)患者的53个(19%)病变中发现了CVS,30例(67%)患者的154个(55%)病变出现了多静脉征(MVS)。每位MOGAD患者的MVS病变数量(4.3±6.0 vs. 1.5±2.1,p = 0.0049)和百分比(52% vs. 18%,p < 0.0001)均高于CVS。8例(18%)患者的39个(14%)病变在磁敏感加权成像上表现为低信号且具有顺磁性相位偏移,外观呈结节状相位变化且边界不规则。
在我们观察的MOGAD队列中,识别出了所有三种类型的皮质病变,其中皮质内病变最为常见。MVS病变的数量和比例高于CVS。具有顺磁性相位变化的病变罕见且外观非边缘样。这些发现有助于更好地理解MOGAD的潜在病理,并有助于将MOGAD与其他脱髓鞘疾病相鉴别。