A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies
A Fulminant Case of Demyelinating Encephalitis With Extensive Cortical Involvement Associated With Anti-MOG Antibodies
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DOI：
10.3389/fneur.2020.00031
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发表时间：
2020-02-14
影响因子：
3.4
通讯作者：
Hoeftberger, Romana
中科院分区：
文献类型：
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作者：
Hochmeister, Sonja;Gattringer, Thomas;Hoeftberger, Romana
抗髓磷脂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) 和抗水通道蛋白 4 抗体血清阴性视神经脊髓炎谱系障碍 (NMOSD) 以及相关综合征（如视神经炎、脊髓炎和脑干脑炎）相关。大多数情况下，MOG-Ab 的存在与更良性的临床病程和对类固醇的良好反应相关。在此，我们介绍了一名先前健康的 52 岁女性患者，她患有暴发性脱髓鞘性脑炎，该患者在首次出现对大剂量静脉注射类固醇和渗透疗法无反应的严重脑水肿症状后一周内死亡。最终诊断是在获得血清抗 MOG 抗体结果后进行尸检得出的。大脑的组织病理学分析显示，额叶、颞叶和顶叶存在广泛的、主要是皮质脱髓鞘病变，并伴有皮质内、白皮质和软膜下斑块，与活化补体复合物明显的静脉周沉积以及以破坏性病变为特征的急性 MS 特征相关。
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.