Department of Ophthalmology.
Department of Neurology.
Curr Opin Ophthalmol. 2019 Nov;30(6):418-425. doi: 10.1097/ICU.0000000000000607.
To summarize recent developments in the classification, investigation and management of pediatric optic neuritis (PON).
A recent surge in interest surrounding antibodies to myelin oligodendrocyte glycoprotein antibody (MOG-Ab) has instigated a paradigm shift in our assessment of children with PON. This serological marker is associated with a broad spectrum of demyelinating syndromes that are clinically and radiologically distinct from multiple sclerosis (MS) and aquaporin-4 antibody positive neuromyelitis optica spectrum disorder (AQP4+NMOSD). Optic neuritis is the most common presenting phenotype of MOG-Ab positive-associated disease (MOG+AD). MOG-Ab seropositivity is much more common in the pediatric population and it predicts a better prognosis than MS or AQP4+NMOSD, except in the subset that exhibit a recurrent phenotype.
A better grasp of MOG+AD features and its natural history has facilitated more accurate risk stratification of children after a presenting episode of PON. Consequently, the initial investigation of PON has broadened to include serology, along with neuroimaging and cerebrospinal fluid analysis. Acute treatment of PON and chronic immunotherapy is also becoming better tailored to the suspected or confirmed diagnoses of MS, AQP4+NMOSD and MOG+AD.
总结小儿视神经炎（PON）分类、研究和管理的最新进展。
髓鞘少突胶质细胞糖蛋白抗体（MOG-Ab）抗体的研究兴趣最近激增，促使我们对 PON 患儿的评估发生了范式转变。这种血清标志物与广泛的脱髓鞘综合征相关，其临床表现和影像学特征与多发性硬化症（MS）和水通道蛋白 4 抗体阳性视神经脊髓炎谱系障碍（AQP4+NMOSD）不同。视神经炎是 MOG-Ab 阳性相关疾病（MOG+AD）最常见的表现型。MOG-Ab 阳性在儿科人群中更为常见，与 MS 或 AQP4+NMOSD 相比，其预后更好，但在表现为复发性表型的亚组中除外。
更好地掌握 MOG+AD 的特征及其自然病史有助于在 PON 发作后对患儿进行更准确的风险分层。因此，对 PON 的初始检查已扩大到包括血清学检查，以及神经影像学和脑脊液分析。PON 的急性治疗和慢性免疫治疗也越来越针对 MS、AQP4+NMOSD 和 MOG+AD 的疑似或确诊诊断进行个体化治疗。
