Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Management.
Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease: Current Insights into the Disease Pathophysiology, Diagnosis and Management.
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DOI：
10.3390/ijms22010100
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发表时间：
2020-12-24
影响因子：
5.6
通讯作者：
Kalinowska A
中科院分区：
文献类型：
--
作者：
Ambrosius W;Michalak S;Kozubski W;Kalinowska A
髓鞘少突胶质细胞糖蛋白（MOG）相关疾病（MOGAD）是一种罕见的抗体介导的中枢神经系统（CNS）炎性脱髓鞘疾病，具有从视神经炎开始的各种表型，通过横肌炎到急性脱髓鞘脑脊髓炎（ADEM）和皮质脑炎。尽管有时这种疾病的临床表现与视神经肌病谱系障碍（NMOSD）的表现相似，但大多数专家认为MOGAD是一种具有不同免疫系统病理学的独特实体。MOG是在髓鞘的外膜上检测到的分子，并且主要在脑、脊髓以及视神经内表达。其功能尚未完全了解，但这种糖蛋白可能作为细胞表面受体或细胞粘附分子。髓鞘的特定最外位置使其成为脱髓鞘过程中自身免疫抗体和细胞介导的反应的潜在靶点。视神经炎似乎是成人和儿童ADEM中最常见的表现型。在成人中，病程是多相的，随后的复发会增加残疾。在儿童中，ADEM通常表现为一次性事件。幸运的是，急性免疫疗法非常有效，严重残疾（行走和视力）的发生率低于NMOSD。可靠诊断的一个关键要素是用准确、特异和灵敏的方法检测致病性血清抗体MOG，最好是用优化的基于细胞的测定法（CBA）。MRI成像也可以帮助区分MOGAD与其他神经炎性疾病。关于随机对照试验的报告有限，但观察性开放标签经验表明，大剂量类固醇和血浆置换在治疗急性发作中发挥作用，免疫抑制治疗，如类固醇，口服免疫抑制剂和利妥昔单抗作为维持治疗。在这篇综述中，我们提出了最新的临床，免疫学，放射学，组织病理学资料有关MOGAD和总结的实际方面的诊断和管理患者与这种疾病。
Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis (ADEM) and cortical encephalitis. Even though sometimes the clinical picture of this condition is similar to the presentation of neuromyelitis optica spectrum disorder (NMOSD), most experts consider MOGAD as a distinct entity with different immune system pathology. MOG is a molecule detected on the outer membrane of myelin sheaths and expressed primarily within the brain, spinal cord and also the optic nerves. Its function is not fully understood but this glycoprotein may act as a cell surface receptor or cell adhesion molecule. The specific outmost location of myelin makes it a potential target for autoimmune antibodies and cell-mediated responses in demyelinating processes. Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. In adults, the disease course is multiphasic and subsequent relapses increase disability. In children ADEM usually presents as a one-time incident. Luckily, acute immunotherapy is very effective and severe disability (ambulatory and visual) is less frequent than in NMOSD. A critical element of reliable diagnosis is detection of pathogenic serum antibodies MOG with accurate, specific and sensitive methods, preferably with optimized cell-based assay (CBA). MRI imaging can also help in differentiating MOGAD from other neuro-inflammatory disorders. Reports on randomised control trials are limited, but observational open-label experience suggests a role for high-dose steroids and plasma exchange in the treatment of acute attacks, and for immunosuppressive therapies, such as steroids, oral immunosuppressants and rituximab as maintenance treatment. In this review, we present up-to-date clinical, immunological, radiographic, histopathological data concerning MOGAD and summarize the practical aspects of diagnosing and managing patients with this disease.
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MOG 抗体具有脱髓鞘表型并影响少突胶质细胞骨架。
DOI：
10.1212/nxi.0000000000000012
发表时间：
2014-06
期刊：
Neurology(R) neuroimmunology & neuroinflammation
影响因子：
--
作者：
Dale RC;Tantsis EM;Merheb V;Kumaran RY;Sinmaz N;Pathmanandavel K;Ramanathan S;Booth DR;Wienholt LA;Prelog K;Clark DR;Guillemin GJ;Lim CK;Mathey EK;Brilot F
通讯作者：
Brilot F
影响因子：
4
作者：
Ciotti, John R.;Eby, Noah S.;Cross, Anne H.
通讯作者：
Cross, Anne H.
影响因子：
3.4
作者：
Hochmeister, Sonja;Gattringer, Thomas;Hoeftberger, Romana
通讯作者：
Hoeftberger, Romana
影响因子：
6
作者：
Budhram, A.;Mirian, A.;Nicolle, M. W.
通讯作者：
Nicolle, M. W.
影响因子：
6
作者：
Cobo-Calvo, Alvaro;Ruiz, Anne;Marignier, Romain
通讯作者：
Marignier, Romain