Hochmeister Sonja, Gattringer Thomas, Asslaber Martin, Stangl Verena, Haindl Michaela Tanja, Enzinger Christian, Höftberger Romana
Department of Neurology, Medical University Graz, Graz, Austria.
Department of Pathology, Medical University Graz, Graz, Austria.
Front Neurol. 2020 Feb 14;11:31. doi: 10.3389/fneur.2020.00031. eCollection 2020.
Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies (MOG-Abs) are commonly associated with clinical presentations as acute disseminated encephalomyelitis (ADEM) in both adults and children and anti-aquaporin 4 antibody-seronegative neuromyelitis optica spectrum disorder (NMOSD) and related syndromes such as optic neuritis, myelitis, and brainstem encephalitis. Most often, the presence of MOG-Abs is associated with a more benign clinical course and a good response to steroids. Here, we present a case report of a previously healthy 52-year-old female patient with fulminant demyelinating encephalitis, leading to death within a week after the first presenting symptoms from a massive brain edema irresponsive to high-dose intravenous steroids as well as osmotic therapy. The final diagnosis was only made postmortem after serum anti-MOG-Abs results were available. Histopathological analysis of the brain revealed extensive, predominantly cortical demyelinating lesions in the frontal, temporal, and parietal lobes with intracortical, leukocortical, and subpial plaques, associated with pronounced perivenous deposition of activated complement complex as well as features of acute MS characterized by destructive lesions.
抗髓鞘少突胶质细胞糖蛋白（MOG）抗体（MOG-Abs）通常与成人和儿童的急性播散性脑脊髓炎（ADEM）临床表现相关，以及与抗水通道蛋白4抗体血清阴性的视神经脊髓炎谱系障碍（NMOSD）和相关综合征如视神经炎、脊髓炎和脑干脑炎有关。大多数情况下，MOG-Abs的存在与更良性的临床病程以及对类固醇的良好反应相关。在此，我们报告一例先前健康的52岁女性患者，患有暴发性脱髓鞘性脑炎，在首次出现症状后一周内死于大剂量静脉注射类固醇及渗透性治疗均无效的大面积脑水肿。最终诊断仅在死后获得血清抗MOG-Abs结果后才得以明确。大脑的组织病理学分析显示额叶、颞叶和顶叶广泛的、主要为皮质脱髓鞘病变，伴有皮质内、白质皮质和软脑膜下斑块，伴有活化补体复合物明显的静脉周围沉积以及以破坏性病变为特征的急性多发性硬化的特征。
