髓鞘少突胶质细胞糖蛋白自身抗体对小儿获得性脱髓鞘疾病的影响:智力功能和学习成绩

IF 2.3 3区 医学 Q3 CLINICAL NEUROLOGY European Journal of Paediatric Neurology Pub Date : 2024-09-03 DOI:10.1016/j.ejpn.2024.09.001
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引用次数: 0

摘要

小儿获得性脱髓鞘综合征(pADS)会在发育的重要时期攻击大脑白质通路。受影响的儿童可能会出现不良的功能结果,包括特定认知领域的缺陷。了解不良后果的风险因素将为这些儿童的临床治疗提供指导。髓鞘少突胶质细胞糖蛋白(MOG)自身抗体是一种可能对认知结果产生不同影响的临床表型。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)检测呈阳性的儿科患者存在认知障碍,但与血清反应阴性的患者相比,他们的认知障碍程度较轻。本研究通过对智力功能和学习能力进行标准化评估,对 MOG-ab 检测呈阳性或阴性的被诊断患有 pADS 的儿童进行评估。研究结果表明,一部分 MOGAD 患者在智力功能和学习能力方面出现了具有临床意义的后遗症。MOG-ab阳性和非阳性儿童的神经心理学特征也有所不同,血清阴性患者更有可能在个体层面上出现临床相关的困难。虽然在群体层面不存在差异,但本研究表明,MOG-ab 血清阴性会相对增加智力/学业困难的风险。这项研究进一步支持了越来越多的观点,即与血清阴性患者相比,MOG 阳性患者的神经心理学前景更为乐观。这一不断扩大的共识为临床医生、家属和患者提供了保证。
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Impact of autoantibodies against myelin oligodendrocyte glycoprotein in paediatric acquired demyelinating disease: Intellectual functioning and academic performance

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.

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来源期刊
CiteScore
6.30
自引率
3.20%
发文量
115
审稿时长
81 days
期刊介绍: The European Journal of Paediatric Neurology is the Official Journal of the European Paediatric Neurology Society, successor to the long-established European Federation of Child Neurology Societies. Under the guidance of a prestigious International editorial board, this multi-disciplinary journal publishes exciting clinical and experimental research in this rapidly expanding field. High quality papers written by leading experts encompass all the major diseases including epilepsy, movement disorders, neuromuscular disorders, neurodegenerative disorders and intellectual disability. Other exciting highlights include articles on brain imaging and neonatal neurology, and the publication of regularly updated tables relating to the main groups of disorders.
期刊最新文献
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