Pooni Rajdeep, Zheng Wynne, Ma Meiqian, Silverman Melissa, Xie Yuhuan, Farhadian Bahare, Thienemann Margo, Mellins Elizabeth, Frankovich Jennifer
Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, School of Medicine, Stanford University, Palo Alto, CA, United States.
Stanford Immune Behavioral Health Clinic and Research Program, Palo Alto, CA, United States.
Front Behav Neurosci. 2024 Aug 19;18:1342486. doi: 10.3389/fnbeh.2024.1342486. eCollection 2024.
This study characterizes cerebral spinal fluid (CSF) indices including total protein, the albumin quotient, IgG index and oligoclonal bands in patients followed at a single center for pediatric acute-neuropsychiatric syndrome (PANS) and other psychiatric/behavioral deteriorations.
In a retrospective chart review of 471 consecutive subjects evaluated for PANS at a single center, navigational keyword search of the electronic medical record was used to identify patients who underwent lumbar puncture (LP) as part of the evaluation of a severe or atypical psychiatric deterioration. Psychiatric symptom data was ascertained from parent questionnaires and clinical psychiatric evaluations. Inclusion criteria required that subjects presented with psychiatric deterioration at the time of first clinical visit and had a lumbar puncture completed as part of their evaluation. Subjects were categorized into three subgroups based on diagnosis: PANS (acute-onset of severe obsessive compulsive disorder (OCD) and/or eating restriction plus two other neuropsychiatric symptoms), autoimmune encephalitis (AE), and "other neuropsychiatric deterioration" (subacute onset of severe OCD, eating restriction, behavioral regression, psychosis, etc; not meeting criteria for PANS or AE).
71/471 (15.0 %) of patients underwent LP. At least one CSF abnormality was seen in 29% of patients with PANS, 45% of patients with "other neuropsychiatric deterioration", and 40% of patients who met criteria for autoimmune encephalitis. The most common findings included elevated CSF protein and/or albumin quotient. Elevated IgG index and IgG oligoclonal bands were rare in all three groups.
Elevation of CSF protein and albumin quotient were found in pediatric patients undergoing LP for evaluation of severe psychiatric deteriorations (PANS, AE, and other neuropsychiatric deteriorations). Further studies are warranted to investigate blood brain barrier integrity at the onset of the neuropsychiatric deterioration and explore inflammatory mechanisms.
本研究对在单一中心随访的小儿急性神经精神综合征(PANS)及其他精神/行为恶化患者的脑脊液(CSF)指标进行了特征分析,这些指标包括总蛋白、白蛋白商、IgG指数和寡克隆带。
在对单一中心连续评估的471例PANS患者进行的回顾性病历审查中,通过电子病历的导航关键词搜索,识别出作为严重或非典型精神恶化评估一部分而接受腰椎穿刺(LP)的患者。精神症状数据来自家长问卷和临床精神评估。纳入标准要求受试者在首次临床就诊时出现精神恶化,并作为评估的一部分完成了腰椎穿刺。根据诊断将受试者分为三个亚组:PANS(严重强迫症(OCD)和/或饮食限制急性发作加另外两种神经精神症状)、自身免疫性脑炎(AE)和“其他神经精神恶化”(严重OCD、饮食限制、行为倒退、精神病等亚急性发作;不符合PANS或AE标准)。
71/471(15.0%)的患者接受了LP。在29%的PANS患者、45%的“其他神经精神恶化”患者和40%符合自身免疫性脑炎标准的患者中发现了至少一项CSF异常。最常见的发现包括CSF蛋白和/或白蛋白商升高。IgG指数升高和IgG寡克隆带在所有三组中均很少见。
在因严重精神恶化(PANS、AE和其他神经精神恶化)接受LP评估的儿科患者中发现了CSF蛋白和白蛋白商升高。有必要进一步研究神经精神恶化发作时血脑屏障的完整性,并探索炎症机制。