目的:儿童急性发作神经精神综合征(PANS)患者在感染或其他触发因素后会出现神经精神症状。虽然PANS通常被描述为复发缓解,2017年一项基于社区的大型研究揭示了一系列课程.本研究检查了症状持续的临床预测因子,以%无症状天数计算,在同一个样本中。方法:一项146个问题的在线调查收集了历史(感染和其他诱因,医学和发育合并症),症状学,干预措施,以及PANS患者的预后(包括学校功能)。应用多变量分析来检查这些变量与整个病程中无症状天数%之间的关联。结果:在646名受试者中,发现更大的症状持续性和更高的医疗合并症发生率之间存在显着关系(尤其是皮疹,头痛,慢性鼻窦炎,频繁腹泻,和免疫缺陷),发育诊断,和受访者感知的发展滞后。症状持续较大的受试者更有可能报告与密切接触者感染相关的PANS恶化。疫苗接种,环境触发因素,以及合并症的恶化,更有可能报告由爱泼斯坦巴尔病毒引发的PANS复发,支原体,和鼻窦感染。更持久的PANS也与某些症状(睡眠障碍,尿失禁,肌肉疼痛,脑雾,感觉防御性,烦躁,和侵略相关症状),静脉注射免疫球蛋白在对抗症状方面的有效性较低,上学更困难。结论:我们的结果表明,PANS的高症状持续性与更普遍的医学和神经精神症状有关。症状持续性的差异与内在的(例如,免疫能力)和外在(例如,感染,治疗)因素。因为外在因素是可以改变的,至关重要的是,提供者必须了解当前的PANS评估和治疗指南.
Objective: Individuals with Pediatric Acute Onset Neuropsychiatric Syndrome (PANS) experience neuropsychiatric symptoms following an infection or other trigger. Although PANS is typically described as relapsing-remitting, a large community-based 2017 study revealed a range of courses. The present study examined clinical predictors of symptom persistence, measured as % days symptom-free, in this same sample. Methods: A 146-question online survey gathered histories (infections and other triggers, medical and developmental comorbidities), symptomatology, interventions, and outcomes (including school functioning) of PANS patients. Multivariate analyses were applied to examine associations between these variables and % days symptom-free across the disease course. Results: Among the 646 subjects included, significant relationships were found between greater symptom persistence and higher rates of medical comorbidities (especially rashes, headaches, chronic sinusitis, frequent diarrhea, and immune deficiencies), developmental diagnoses, and respondent-perceived developmental lags. Subjects with greater symptom persistence were significantly more likely to report PANS exacerbations associated with infections in close contacts, vaccinations, environmental triggers, and exacerbations of comorbidities and were more likely to report PANS recurrences triggered by Epstein Barr Virus, mycoplasma, and sinus infections. More persistent PANS was also associated with significantly higher frequencies of certain symptoms (sleep disturbance, urinary incontinence, muscle pain, brain fog, sensory defensiveness, irritability, and aggression-related symptoms), less effectiveness of intravenous immunoglobulin in combating symptoms, and more difficulty attending school. Conclusions: Our results suggest high symptom persistence in PANS to be associated with more pervasive medical and neuropsychiatric symptoms. Differences in symptom persistence are associated with both intrinsic (e.g., immune competence) and extrinsic (e.g., infections, treatment) factors. Because extrinsic factors are potentially modifiable, it is critical that providers be aware of current guidelines on PANS evaluation and treatment.