Department of Neurology, Erasmus Medical Center, Rotterdam, the Netherlands.
Department of Paediatrics and Adolescent Medicine, Division of Paediatric Neurology, Medical University of Vienna, Austria.
Eur J Paediatr Neurol. 2020 Nov;29:32-40. doi: 10.1016/j.ejpn.2020.10.007. Epub 2020 Nov 4.
There is increasing knowledge on the role of antibodies against myelin oligodendrocyte glycoprotein (MOG-abs) in acquired demyelinating syndromes and autoimmune encephalitis in children. Better understanding and prediction of outcome is essential to guide treatment protocol decisions. Therefore, this part of the Paediatric European Collaborative Consensus provides an oversight of existing knowledge of clinical outcome assessment in paediatric MOG-ab-associated disorders (MOGAD). The large heterogeneity in disease phenotype, disease course, treatment and follow-up protocols is a major obstacle for reliable prediction of outcome. However, the clinical phenotype of MOGAD appears to be the main determinant of outcome. Patients with a transverse myelitis phenotype in particular are at high risk of accruing neurological disability (motor and autonomic), which is frequently severe. In contrast, having a single episode of optic neuritis any time during disease course is broadly associated with a lower risk of persistent disability. Furthermore, MOG-ab-associated optic neuritis often results in good functional visual recovery, although retinal axonal loss may be severe. The field of cognitive and behavioural outcome and epilepsy following demyelinating episodes has not been extensively explored, but in recent studies acute disseminated encephalomyelitis (-like) phenotype in the young children was associated with cognitive problems and epilepsy in long-term follow-up. In conclusion, main domains of importance in determining clinical outcome in paediatric MOGAD are visual, motor, autonomic and cognitive function. A standardised evaluation of these outcome domains in all children is of importance to allow adequate rehabilitation and follow-up.
人们对髓鞘少突胶质细胞糖蛋白(MOG-ab)抗体在儿童获得性脱髓鞘综合征和自身免疫性脑炎中的作用的了解越来越多。更好地理解和预测预后对于指导治疗方案决策至关重要。因此,本儿科欧洲协作共识的这一部分概述了儿科 MOG-ab 相关疾病(MOGAD)的临床结局评估的现有知识。疾病表型、病程、治疗和随访方案的巨大异质性是可靠预测预后的主要障碍。然而,MOGAD 的临床表型似乎是结局的主要决定因素。特别是横贯性脊髓炎表型的患者有发生神经功能障碍(运动和自主)的高风险,而且往往很严重。相比之下,在疾病过程中的任何时间发生单次视神经炎通常与持续残疾的风险较低相关。此外,MOG-ab 相关视神经炎通常会导致良好的功能视觉恢复,尽管视网膜轴索丢失可能很严重。认知和行为结局以及脱髓鞘发作后的癫痫领域尚未得到广泛探讨,但在最近的研究中,幼儿的急性播散性脑脊髓炎(样)表型与长期随访中的认知问题和癫痫有关。总之,决定儿科 MOGAD 临床结局的主要重要领域是视力、运动、自主和认知功能。对所有儿童进行这些结局领域的标准化评估对于进行适当的康复和随访很重要。