Hacohen Yael, Banwell Brenda
Department of Neuroinflammation, Queen Square MS Centre, UCL Institute of Neurology, London, UK.
Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.
Curr Treat Options Neurol. 2019 Jan 22;21(1):2. doi: 10.1007/s11940-019-0541-x.
The purpose of this review is to summarize current understanding regarding the treatment of myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated demyelination in children. Emphasis is placed on the unique obstacles we face when predicting the risk of relapse and the important implications of such challenges when planning treatment protocols.
MOG-Abs are consistently identified in a range of acquired demyelinating syndromes (ADS) in adults and children with a clinical phenotype distinct of MS and AQP4-Ab neuromyelitis optica spectrum disorder. Although initially thought to be associated with a benign disease, recent reports of children who are treatment-resistant and developed progressive disability over time raise important questions about how children with relapsing MOG-Ab disease should be managed. MOG-Abs are common in children with ADS with both monophasic and relapsing disease courses. Treatment of patients with MOG-Ab-associated demyelination includes management of acute relapses and chronic immunotherapy for those with relapsing disease. Emerging consensus supports distinction of treatment strategies from those typically used for relapsing remitting MS, and several groups debate whether to follow treatment protocols akin to those for AQP4-Ab NMOSD. A key challenge remains predicting the severity of the disease at onset. Collaborative international consensus to derive shared clinical evaluative platforms standardized biological and neuroimaging protocols which can be used clinically, and partnered research programs are required to advance personalized treatment for children with MOG-Ab-associated demyelination.
本综述旨在总结目前对儿童髓鞘少突胶质细胞糖蛋白抗体(MOG-Ab)相关脱髓鞘疾病治疗的认识。重点关注预测复发风险时所面临的独特障碍,以及在制定治疗方案时这些挑战的重要影响。
在成人和儿童一系列获得性脱髓鞘综合征(ADS)中持续检测到MOG抗体,其临床表型不同于多发性硬化症(MS)和水通道蛋白4抗体(AQP4-Ab)视神经脊髓炎谱系障碍(NMOSD)。尽管最初认为与良性疾病相关,但近期有报道称,一些儿童对治疗耐药并随着时间推移出现进行性残疾,这引发了关于如何管理复发性MOG-Ab疾病患儿的重要问题。MOG抗体在单相和复发病程的ADS患儿中很常见。MOG-Ab相关脱髓鞘疾病患者的治疗包括急性复发的管理以及对复发性疾病患者的慢性免疫治疗。新出现的共识支持将治疗策略与复发缓解型MS通常使用的策略区分开来,并且几个研究小组在讨论是否遵循类似于AQP4-Ab NMOSD的治疗方案。一个关键挑战仍然是预测疾病起病时的严重程度。需要国际合作达成共识,以建立标准化的生物学和神经影像学方案的共享临床评估平台,这些平台可用于临床,并且需要合作研究项目来推进针对MOG-Ab相关脱髓鞘疾病患儿的个性化治疗。