在过去的几年里,抗髓磷脂少突胶质细胞糖蛋白(MOG-abs)自身抗体作为脱髓鞘性中枢神经系统疾病新的候选生物标志物的作用引起了越来越多的兴趣.现在已经在各种脱髓鞘综合征中发现了MOG-abs,在儿科患者中占主导地位。这些MOG-ab相关疾病(MOGAD)的临床范围仍在扩大,儿科和成年患者之间存在差异。儿科欧洲合作共识的第一部分强调了儿科患者与MOG-abs相关的临床表型的多样性,并详细讨论了这些相关的临床表型。典型的MOGAD介绍包括脱髓鞘综合征,包括年轻的急性播散性脑脊髓炎(ADEM),大龄儿童的视神经炎(ON)和/或横贯性脊髓炎(TM)。一部分患者经历复发性疾病过程,呈现为ADEM,然后是ON(ADEM-ON)的一个或多个情节,多相播散性脑脊髓炎(MDEM),复发性ON(RON)或复发性视神经脊髓炎谱系障碍(NMOSD)样综合征。最近,疾病谱已经扩大了临床和放射学表型,包括脑炎样,脑白质营养不良样,和其他不可分类的演示文稿。本综述的结论是在儿科患者MOG-abs血清学检测专家达成共识后提出的建议。其存在会对长期监测产生影响,复发风险,治疗,为病人和家属提供咨询。此外,我们提出了儿科MOGAD的临床分类,包括临床定义和关键特征。这些是可操作的,需要测试,然而,对于未来的儿科MOGAD研究至关重要。
Over the past few years, increasing interest in the role of autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs) as a new candidate biomarker in demyelinating central nervous system diseases has arisen. MOG-abs have now consistently been identified in a variety of demyelinating syndromes, with a predominance in paediatric patients. The clinical spectrum of these MOG-ab-associated disorders (MOGAD) is still expanding and differs between paediatric and adult patients. This first part of the Paediatric European Collaborative
Consensus emphasises the diversity in clinical phenotypes associated with MOG-abs in paediatric patients and discusses these associated clinical phenotypes in detail. Typical MOGAD presentations consist of demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM) in younger, and optic neuritis (ON) and/or transverse myelitis (TM) in older children. A proportion of patients experience a relapsing disease course, presenting as ADEM followed by one or multiple episode(s) of ON (ADEM-ON), multiphasic disseminated encephalomyelitis (MDEM), relapsing ON (RON) or relapsing neuromyelitis optica spectrum disorders (NMOSD)-like syndromes. More recently, the disease spectrum has been expanded with clinical and radiological phenotypes including encephalitis-like, leukodystrophy-like, and other non-classifiable presentations. This review concludes with recommendations following expert
consensus on serologic testing for MOG-abs in paediatric patients, the presence of which has consequences for long-term monitoring, relapse risk, treatments, and for counselling of patient and families. Furthermore, we propose a clinical classification of paediatric MOGAD with clinical definitions and key features. These are operational and need to be tested, however essential for future paediatric MOGAD studies.