Chan Avis, Gao Jaynelle, Houston Madison, Willett Theresa, Farhadian Bahare, Silverman Melissa, Tran Paula, Jaradeh Safwan, Thienemann Margo, Frankovich Jennifer
Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, United States.
Stanford PANS/Immune Behavioral Health Clinic and PANS Research Program at Lucile Packard Children's Hospital, Palo Alto, CA, United States.
Front Neurol. 2022 Apr 26;13:819636. doi: 10.3389/fneur.2022.819636. eCollection 2022.
Pediatric acute-onset neuropsychiatric syndrome (PANS) is characterized by an abrupt-onset of severe psychiatric symptoms including OCD, anxiety, cognitive difficulties, and sleep issues which is thought to be a post-infection brain inflammatory disorder. We observed postural orthostatic tachycardia syndrome (POTS) which resolved with immunomodulation in a patient with Pediatric acute-onset neuropsychiatric syndrome (PANS). Here, we aim to present a case of POTS and to examine the prevalence of (POTS) in our PANS cohort, and compare the clinical characteristics of patients with and without POTS.
We conducted this cohort study of patients meeting PANS criteria who had at least three clinic visits during the study period. We included data from prospectively collected questionnaires and medical record review. We present a case followed by statistical comparisons within our cohort and a Kaplan-Meier analysis to determine the time-dependent risk of a POTS diagnosis.
Our study included 204 patients: mean age of PANS onset was 8.6 years, male sex (60%), non-Hispanic White (78%). Evidence of POTS was observed in 19/204 patients (9%) with 5/19 having persistent POTS defined as persistent abnormal orthostatic vitals, persistent POTS symptoms, and/or continued need for pharmacotherapy for POTS symptoms for at least 6 months). In this PANS cohort, patients with POTS were more likely to have comorbid joint hypermobility (63 vs 37%, = 0.04), chronic fatigue (42 vs 18%, = 0.03), and a family history of chronic fatigue, POTS, palpitations and syncope. An unadjusted logistic regression model showed that a PANS flare (abrupt neuropsychiatric deterioration) was significantly associated with an exacerbation of POTS symptoms (OR 3.3, 95% CI 1.4-7.6, < 0.01).
Our study describes a high prevalence of POTS in patients with PANS (compared to the general population) and supports an association between POTS presentation and PANS flare within our cohort.
儿童急性起病神经精神综合征（PANS）的特征是突然出现包括强迫症、焦虑、认知困难和睡眠问题在内的严重精神症状，被认为是一种感染后脑炎性疾病。我们观察到一名儿童急性起病神经精神综合征（PANS）患者的体位性直立性心动过速综合征（POTS）通过免疫调节得以缓解。在此，我们旨在呈现一例POTS病例，并研究我们的PANS队列中POTS的患病率，比较有和没有POTS的患者的临床特征。
我们对符合PANS标准且在研究期间至少有三次门诊就诊的患者进行了这项队列研究。我们纳入了前瞻性收集的问卷数据和病历回顾。我们先呈现一个病例，然后在我们的队列中进行统计比较，并进行Kaplan-Meier分析以确定POTS诊断的时间依赖性风险。
我们的研究纳入了204名患者：PANS起病的平均年龄为8.6岁，男性占60%，非西班牙裔白人占78%。在204名患者中有19名（9%）观察到POTS证据，其中5名（19名中的）患有持续性POTS，定义为持续性异常直立生命体征、持续性POTS症状和/或至少6个月持续需要针对POTS症状进行药物治疗。在这个PANS队列中，患有POTS的患者更可能合并关节活动过度（63%对37%，P = 0.04）、慢性疲劳（42%对18%，P = 0.03），以及有慢性疲劳、POTS、心悸和晕厥的家族史。一个未调整的逻辑回归模型显示，PANS发作（突然的神经精神恶化）与POTS症状加重显著相关（比值比3.3，95%置信区间1.4 - 7.6，P < 0.01）。
我们的研究描述了PANS患者中POTS的高患病率（与一般人群相比），并支持在我们的队列中POTS表现与PANS发作之间存在关联。
