Lee Yun-Jin, Nam Sang Ook, Ko Ara, Kong JuHyun, Byun Shin Yun
Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University College of Medicine, Yangsan, Korea.
Clin Exp Pediatr. 2021 Mar;64(3):103-110. doi: 10.3345/cep.2019.01305. Epub 2020 May 14.
Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.
炎症性或免疫介导的中枢神经系统(CNS)脱髓鞘综合征包括广泛的临床表型和不同的重叠疾病。在这些脱髓鞘疾病的某些病例中,特别是儿童患者中,发现了针对髓鞘少突胶质细胞糖蛋白(MOG-Ab)的抗体。MOG-Ab与更广泛的临床表型相关,不限于视神经脊髓炎谱系障碍,大多数患者表现为视神经炎、急性播散性脑脊髓炎(ADEM)或伴有脑脱髓鞘病变的ADEM样脑炎和/或脊髓炎。通过特定的基于细胞的检测方法,MOG-Ab正成为中枢神经系统炎症性脱髓鞘疾病的潜在生物标志物。最近在单相疾病和复发/多相临床进展中发现了针对MOG的体液免疫反应,特别是在儿科患者中。本综述总结了有关MOG-Ab作为区分这些不同的中枢神经系统脱髓鞘疾病的潜在生物标志物的数据,并讨论了MOG-Ab相关疾病的免疫发病机制的最新进展、临床应用和研究结果。