Institute of Health and Neurodevelopment, College of Health and Life Sciences, Aston University, Birmingham, UK.
Institute of Health and Neurodevelopment, College of Health and Life Sciences, Aston University, Birmingham, UK; Nanyang Technological University, Singapore, Singapore.
Eur J Paediatr Neurol. 2024 Nov;53:8-17. doi: 10.1016/j.ejpn.2024.09.001. Epub 2024 Sep 3.
Paediatric acquired demyelinating syndromes (pADS) attack white matter pathways in the brain during an important period of development. Affected children can experience poor functional outcomes, including deficits in specific cognitive domains. Understanding risk factors for poor outcome will guide clinical management of these children. One clinical phenotype which may differentially impact cognitive outcomes is the presence of autoantibodies to myelin oligodendrocyte glycoprotein (MOG). Preliminary research has suggested that cognitive difficulties exist in paediatric patients who test positive for MOG antibodies or MOGAD (Myelin Oligodendrocyte Glycoprotein Associated Disease) however, they experience a less severe profile compared to seronegative counterparts. The current study assesses children diagnosed with pADS who tested positive or negative for MOG-ab using standardised assessments of both intellectual functioning and academic ability. The results show that a subset of MOGAD patients experience clinically significant sequalae in intellectual functioning and academic ability. The neuropsychological profile also differed between children with and without MOG-ab positivity, with seronegative patients more likely to show a clinically relevant difficulties at the individual patient level. Whilst no differences existed at the group-level; the current study demonstrates the relative additional risk of intellectual/academic difficulty associated with MOG-ab seronegativity. This research further supports the growing perspective that MOG-positivity confers a more favourable neuropsychological outlook than is the case for their seronegative counterparts. This broadening consensus offers reassurance for clinicians, families, and patients.
儿科获得性脱髓鞘综合征(pADS)在大脑发育的重要时期攻击白质通路。受影响的儿童可能会出现功能不良的后果,包括特定认知领域的缺陷。了解不良预后的风险因素将指导对这些儿童的临床管理。一种可能对认知结果产生不同影响的临床表型是髓鞘少突胶质细胞糖蛋白(MOG)自身抗体的存在。初步研究表明,MOG 抗体或 MOGAD(髓鞘少突胶质细胞糖蛋白相关疾病)阳性的儿科患者存在认知困难,但与血清阴性患者相比,他们的病情较轻。目前的研究评估了使用智力和学业能力的标准化评估检测为 MOG-ab 阳性或阴性的 pADS 患儿。结果表明,MOGAD 患者中有一部分在智力和学业能力方面存在临床显著的后遗症。神经心理学特征也在有和没有 MOG-ab 阳性的儿童之间存在差异,血清阴性患者更有可能在个体患者层面表现出临床相关的困难。虽然在组间没有差异;但本研究表明,与 MOG-ab 血清阴性相关的智力/学业困难的相对额外风险。这项研究进一步支持了这样一种越来越多的观点,即 MOG 阳性比血清阴性患者具有更有利的神经心理学前景。这种不断扩大的共识为临床医生、家庭和患者提供了安慰。