Eremija Jelena, Patel Sanjay, Rice Sydney, Daines Michael
Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, University of Arizona, Tucson, AZ, United States.
Department of Internal Medicine, Arizona College of Osteopathic Medicine, Midwestern University, Glendale, AZ, United States.
Front Pediatr. 2023 Oct 16;11:1229150. doi: 10.3389/fped.2023.1229150. eCollection 2023.
Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) is defined by acute onset of diverse neuropsychiatric manifestations, presumably in the setting of underlying immune dysfunction We used standardized neuropsychological testing to assess how intravenous immunoglobulins (IVIG) impact neurological and cognitive functions in PANS patients by comparing pretreatment with post-treatment scores. A 5-year retrospective study was undertaken in Children's Postinfectious Autoimmune Encephalopathy Center at University of Arizona. We identified 12 children diagnosed with PANS and treated with immunomodulatory IVIG doses, who also completed neuropsychological testing before and after treatment. We tracked multiple patient characteristics, type/timeline of testing, and number of IVIG courses. Score change of 1 standard deviation in any tested domain/subdomain was considered improvement. We further reviewed records for laboratory signs of triggering infection and immune dysfunction. Improvement occurred in 11/12 patients, in one or multiple domains/subdomains, independently of time between disease onset and IVIG initiation (0-7 years). Participants received 1-7 IVIG courses. Improvement was primarily seen in memory (58%), sensory-motor (37%) and visual-motor integration (30%). In 5/12 patients we detected hypogammaglobulinemia requiring ongoing IVIG replacement, one patient had isolated low IgA. Only one patient had to discontinue IVIG therapy due to severe adverse effects. Standardized neuropsychological testing represents an important tool to objectively measure improvement in PANS patients. IVIG was tolerated well and showed efficacy in the vast majority of participants, independently from timelapse since disease onset, emphasizing impact of immunomodulation in PANS. Significant presence of baseline hypogammaglobulinemia in children with PANS emphasizes the presumed role of immune dysfunction in disease pathogenesis.
儿童急性起病神经精神综合征（PANS）的定义是多种神经精神症状急性发作，推测是在潜在免疫功能障碍的背景下发生。我们通过比较治疗前和治疗后的分数，使用标准化神经心理学测试来评估静脉注射免疫球蛋白（IVIG）对PANS患者神经和认知功能的影响。在亚利桑那大学儿童感染后自身免疫性脑病中心进行了一项为期5年的回顾性研究。我们确定了12名被诊断为PANS并接受免疫调节剂量IVIG治疗的儿童，他们在治疗前后也完成了神经心理学测试。我们跟踪了多个患者特征、测试类型/时间线以及IVIG疗程数。任何测试领域/子领域的分数变化达到1个标准差被视为改善。我们进一步审查了引发感染和免疫功能障碍的实验室指标记录。11/12名患者在一个或多个领域/子领域出现改善，与疾病发作和开始使用IVIG之间的时间（0 - 7年）无关。参与者接受了1 - 7个IVIG疗程。改善主要见于记忆（58%）、感觉运动（37%）和视觉运动整合（30%）。在5/12名患者中，我们检测到低丙种球蛋白血症需要持续进行IVIG替代，1名患者孤立性低IgA。只有1名患者因严重不良反应不得不停止IVIG治疗。标准化神经心理学测试是客观衡量PANS患者改善情况的重要工具。IVIG耐受性良好，在绝大多数参与者中显示出疗效，与疾病发作后的时间间隔无关，强调了免疫调节在PANS中的作用。PANS患儿中显著存在基线低丙种球蛋白血症，强调了免疫功能障碍在疾病发病机制中的假定作用。
