Gagliano Antonella, Carta Alessandra, Tanca Marcello G, Sotgiu Stefano
Department of Health Science, "Magna Graecia" University of Catanzaro, Catanzaro, Italy.
Department of Biomedical Sciences, University of Cagliari & "A. Cao" Paediatric Hospital, Child & Adolescent Neuropsychiatry Unit, Cagliari, Italy.
Neuropsychiatr Dis Treat. 2023 May 24;19:1221-1250. doi: 10.2147/NDT.S362202. eCollection 2023.
Pediatric acute-onset neuropsychiatric syndrome (PANS) features a heterogeneous constellation of acute obsessive-compulsive disorder (OCD), eating restriction, cognitive, behavioral and/or affective symptoms, often followed by a chronic course with cognitive deterioration. An immune-mediated etiology is advocated in which the CNS is hit by different pathogen-driven (auto)immune responses. This narrative review focused on recent clinical (ie, diagnostic criteria, pre-existing neurodevelopmental disorders, neuroimaging) and pathophysiological (ie, CSF, serum, genetic and autoimmune findings) aspects of PANS. We also summarized recent points to facilitate practitioners with the disease management. Relevant literature was obtained from PubMed database which included only English-written, full-text clinical studies, case reports, and reviews. Among a total of 1005 articles, 205 were pertinent to study inclusion. Expert opinions are converging on PANS as the effect of post-infectious events or stressors leading to "brain inflammation", as it is well-established for anti-neuronal psychosis. Interestingly, differentiating PANS from either autoimmune encephalitides and Sydenham's chorea or from alleged "pure" psychiatric disorders (OCD, tics, Tourette's syndrome), reveals several overlaps and more analogies than differences. Our review highlights the need for a comprehensive algorithm to help both patients during their acute distressing phase and physicians during their treatment decision. A full agreement on the hierarchy of each therapeutical intervention is missing owing to the limited number of randomized controlled trials. The current approach to PANS treatment emphasizes immunomodulation/anti-inflammatory treatments in association with both psychotropic and cognitive-behavioral therapies, while antibiotics are suggested when an active bacterial infection is established. A dimensional view, taking into account the multifactorial origin of psychiatric disorders, should suggest neuro-inflammation as a possible shared substrate of different psychiatric phenotypes. Hence, PANS and PANS-related disorders should be considered as a conceptual framework describing the etiological and phenotypical complexity of many psychiatric disorders.
小儿急性起病神经精神综合征(PANS)具有一系列异质性症状,包括急性强迫症(OCD)、饮食限制、认知、行为和/或情感症状,通常随后会发展为伴有认知衰退的慢性病程。目前主张其病因是免疫介导的,即中枢神经系统受到不同病原体驱动的(自身)免疫反应的攻击。本叙述性综述聚焦于PANS近期的临床(即诊断标准、既往神经发育障碍、神经影像学)和病理生理学(即脑脊液、血清、基因和自身免疫结果)方面。我们还总结了近期要点,以帮助从业者进行疾病管理。相关文献从PubMed数据库获取,该数据库仅包括英文撰写的全文临床研究、病例报告和综述。在总共1005篇文章中,205篇与研究纳入相关。专家意见逐渐趋于一致,认为PANS是感染后事件或应激源导致“脑部炎症”的结果,就像抗神经元精神病一样已得到充分证实。有趣的是,将PANS与自身免疫性脑炎和 Sydenham 舞蹈病或与所谓的“单纯”精神障碍(OCD、抽动、妥瑞氏综合征)区分开来时,发现它们之间的重叠和相似之处多于差异。我们的综述强调需要一种综合算法,以在患者急性痛苦阶段帮助患者,并在医生进行治疗决策时提供帮助。由于随机对照试验数量有限,对于每种治疗干预的优先级尚未达成完全一致。目前PANS的治疗方法强调免疫调节/抗炎治疗,并结合精神药物和认知行为疗法,而当确定存在活动性细菌感染时建议使用抗生素。考虑到精神障碍的多因素起源的维度观点,应将神经炎症视为不同精神表型可能的共同基础。因此,PANS及与PANS相关的疾病应被视为一个概念框架,用于描述许多精神障碍的病因和表型复杂性。