Fulminant demyelinating encephalomyelitis Insights from antibody studies and neuropathology
Fulminant demyelinating encephalomyelitis Insights from antibody studies and neuropathology
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DOI：
10.1212/nxi.0000000000000175
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发表时间：
2015-12-01
影响因子：
8.8
通讯作者：
Berger, Thomas
中科院分区：
文献类型：
--
作者：
Di Pauli, Franziska;Hoftberger, Romana;Berger, Thomas
目的：髓鞘少突胶质细胞糖蛋白（MOG）抗体可检测到炎性脱髓鞘中枢神经系统疾病，和MOG抗体相关疾病似乎有一个更好的预后，尽管偶尔严重presentation.Methods：我们报告的情况下，71岁的患者急性视力和步态障碍，急剧恶化，双边黑蒙，四肢瘫痪，呼吸功能不全在几天内。MRI显示多个进行性脑和脊髓病变，扩散受限（包括视神经）和边缘对比增强。常规血液和CSF测量（包括寡克隆条带）正常。在疾病发作时，检测到MOG免疫球蛋白G（血清滴度1：1，280，相应的CSF滴度为1：20），并在患者血清中保持阳性。水通道蛋白-4抗体在疾病发作时不存在，但在第9周血清转化为阳性。此外，CSF胶质细胞酸性蛋白和髓鞘碱性蛋白水平在发病时非常高，但在病程中下降。4个月后，尽管进行了免疫调节治疗，患者仍死亡。尸检神经病理学检查显示急性多发性硬化症（MS）定义为多发性脱髓鞘病变，具有明显的破坏性成分和星形胶质细胞的损失。视交叉的病变模式符合MS模式II，其特征在于抗体和补体介导的demyelination.Conclusion：急性脱髓鞘性脑脊髓炎的临床表现的情况下，主要的光学和脊髓受累，缺乏寡克隆带，急性MS模式II和水通道蛋白-4抗体的发展的组织病理学扩展了MOG抗体相关脑脊髓炎的频谱。虽然MOG抗体被怀疑是一个良好的预后，暴发性疾病的过程是可能的，并保证积极的免疫治疗。
Objectives: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are detectable in inflammatory demyelinating CNS diseases, and MOG antibody-associated diseases seem to have a better prognosis despite occasionally severe presentations.Methods: We report the case of a 71-year-old patient with acute visual and gait disturbance that dramatically worsened to bilateral amaurosis, tetraplegia, and respiratory insufficiency within a few days.Results: MRI showed multiple progressive cerebral and spinal lesions with diffusion restriction (including both optic nerves) and marginal contrast enhancement. Routine blood and CSF measures including oligoclonal bands were normal. At disease onset, MOG immunoglobulin G was detected (serum titer 1: 1,280, corresponding CSF titer was 1: 20) and remained positive in patient serum. Aquaporin-4 antibodies were absent at disease onset but seroconverted to positive at week 9. In addition, CSF glial fibrillary acid protein and myelin basic protein levels were very high at onset but decreased during disease course. After 4 months, the patient died despite immunomodulatory treatment. Postmortem neuropathologic examination revealed an acute multiple sclerosis (MS) defined by multiple demyelinating lesions with a pronounced destructive component and loss of astrocytes. Lesion pattern of optic chiasm met MS pattern II characterized by antibody and complement-mediated demyelination.Conclusion: The case with the clinical presentation of an acute demyelinating encephalomyelitis with predominant optic and spinal involvement, absent oligoclonal bands, a histopathology of acute MS pattern II and development of aquaporin-4 antibodies extends the spectrum of MOG antibody-associated encephalomyelitis. Although, MOG antibodies are suspected to indicate a favorable prognosis, fulminant disease courses are possible and warrant an aggressive immunotherapy.