Objective:Pediatric acquired demyelinating syndromes (PADS) include a heterogeneous group of diagnoses, including acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorders (NMOSD), optic neuritis (ON) and transverse myelitis (TM). Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is often associated with demyelinating conditions, but may also present with encephalopathy without demyelinating lesions. Approximately 30% of patients diagnosed with MOGAD experience a relapse. Neurocognitive outcomes in PADS have reduced performance on tasks related to attention, processing speed, visual motor, and fine motor functioning. Psychosocial problems include anxiety, depression, and fatigue. Neurocognitive and psychosocial impacts of MOGAD events for the pediatric population are sparse. The current study sought to characterize neurocognitive sequelae from MOGAD (MAGAD+) compared to patients diagnosed with PADS without MOGAD (MOGAD-).Participants and Methods:Twenty children and adolescents (6–18 years) diagnosed with PADS were recruited using a clinic convenience sample of patients. Study participants completed a neurocognitive battery and parents completed questionnaires of behavioral and emotional functioning. Demographic and medical variables were collected via retrospective chart review. Chi square and t-test analyses were used to compare MOGAD+ and MOGAD- groups. Performance on neuropsychological and behavioral questionnaires were compared to established sex and age norms to assess the degree to which group means deviate from normative expectations.Results:MOGAD+ and MOGAD- groups did not significantly differ based on demographic, neurocognitive, or parent reported social and behavioral functioning. Neurocognitive testing documented mean scores that were in the average range between groups. Notable variability in performance was observed within both MOGAD+ and MOGAD- groups. Bilateral fine motor deficits, visual motor, visual perception attention, and executive functioning deficits were notable for the combined PADS group, with 30-50% performing >1.5 SD below the mean. The number of white matter lesions or hospital duration were not significantly associated with performance on neurocognitive measures. However, older age of onset of PADS was significantly correlated with lower performance on visual motor integration and visual perception tasks (r(18) = -.50 p = .026; r(18) = -.53 p = .016). Findings also revealed associations of shorter hospitalization stays with higher behavioral symptoms on a parent measure of social/behavioral functioning (r(18) = -.47 p = .037).Conclusions:Consistent with the PADS literature, relative to control norms, lower performance on tasks related to attention, executive functioning, visual motor, and fine motor skills, irrespective of MOGAD status, are observed in the current study. The variability of functioning and heterogeneity observed across PADS diagnoses warrants further study to better understand the impact of clinical course, treatment outcomes, and neuropsychological sequelae over time in this population. Higher behavioral distress with shorter hospital stays may indicate a potential opportunity for patient and family education preparing for return to home/community. The current study was limited by small sample size, variable time since hospitalization, and heterogeneous diagnoses within PADS that make it difficult to generalize findings. Future studies could prospectively follow patients over time to better understand the trajectory of recovery, identify predictors for relapse, and those at greatest risk of neurocognitive and behavioral deficits.

中文翻译：

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28 伴有或不伴有髓磷脂少突胶质细胞糖蛋白抗体病 (MOGAD) 的儿童获得性脱髓鞘综合征的神经认知概况

目的：小儿获得性脱髓鞘综合征 (PADS) 包括一组异质性诊断，包括急性播散性脑脊髓炎 (ADEM)、视神经脊髓炎谱系疾病 (NMOSD)、视神经炎 (ON) 和横贯性脊髓炎 (TM)。髓鞘少突胶质细胞糖蛋白抗体病 (MOGAD) 通常与脱髓鞘疾病相关，但也可能表现为无脱髓鞘病变的脑病。大约 30% 诊断为 MOGAD 的患者会出现复发。PADS 的神经认知结果降低了与注意力、处理速度、视觉运动和精细运动功能相关的任务的表现。心理社会问题包括焦虑、抑郁和疲劳。MOGAD 事件对儿科人群的神经认知和社会心理影响很少。当前的研究旨在比较 MOGAD (MAGAD+) 与诊断为无 MOGAD (MOGAD-) 的 PADS 患者 (MOGAD-) 的神经认知后遗症的特征。 参与者和方法：使用诊所便利样本招募了 20 名诊断为 PADS 的儿童和青少年（6-18 岁）的患者。研究参与者完成了神经认知电池测试，家长完成了行为和情绪功能的问卷调查。通过回顾性图表审查收集人口统计和医疗变量。使用卡方和 t 检验分析来比较 MOGAD+ 和 MOGAD- 组。将神经心理学和行为问卷的表现与既定的性别和年龄标准进行比较，以评估群体平均值偏离规范期望的程度。结果：MOGAD+ 和 MOGAD- 组在人口统计学、神经认知或父母报告的社会和行为方面没有显着差异发挥作用。神经认知测试记录的平均分数处于各组之间的平均范围内。MOGAD+ 组和 MOGAD- 组的表现存在显着差异。联合 PADS 组的双侧精细运动缺陷、视觉运动、视觉感知注意力和执行功能缺陷非常显着，其中 30-50% 的表现比平均值低 1.5 个标准差以上。白质病变的数量或住院时间与神经认知测量的表现没有显着相关。然而，PADS 发病年龄较大与视觉运动整合和视觉感知任务的较低表现显着相关 (r(18) = -.50p= .026；r(18) = -.53p=.016）。研究结果还显示，根据家长对社交/行为功能的衡量，较短的住院时间与较高的行为症状之间存在关联 (r(18) = -.47p= .037）。结论：与 PADS 文献一致，相对于控制规范，当前研究中观察到，无论 MOGAD 状态如何，与注意力、执行功能、视觉运动和精细运动技能相关的任务表现较低。PADS 诊断中观察到的功能变异性和异质性值得进一步研究，以更好地了解临床过程、治疗结果和神经心理学后遗症随时间的推移对该人群的影响。较高的行为困扰和较短的住院时间可能表明患者和家人有可能接受教育，为返回家庭/社区做好准备。目前的研究受到样本量小、住院后时间变化以及 PADS 内诊断异质性的限制，使得研究结果难以推广。未来的研究可以前瞻性地跟踪患者一段时间，以更好地了解康复轨迹，确定复发的预测因素，以及神经认知和行为缺陷风险最大的患者。