Castillo Rocha Pamela A, Hon Kam Lun E, Mohanty Mugdha, Torres Alcy R
Boston University School of Medicine, USA.
Departamentof Pediatric, The Chinese University of Hong Kong, China.
Medicina (B Aires). 2022 Aug 30;82 Suppl 3:46-50.
Autoimmune encephalitis can be defined as central nervous system inflammation, secondary to multiple causes, where we can possibly identify the formation of auto-antibody against neurotransmitter receptors or neuronal surface proteins. Approximately 50% of patients are seropositive; the auto-antibody against N-methyl-D-aspartate receptor (NMDAR) are the most common. In the pediatric population, the clinical presentation is characterized by movement disorders and seizures, psychiatric manifestations are more commonly found in young adults. An early intervention is associated with a better prognosis in these patients. In contrast to the seropositive group, seronegative autoimmune encephalitis is linked with less movement alterations and is related with a worse cognitive outcome. Much remains to be discovered about possible etiologies, molecular processes, detection, and interaction of yet undescribed antibodies,as well as increasing our knowledge about clinical manifestations in early disease and new diagnostic techniques that could improve the diagnosis of autoimmune encephalitis. The main goal of this document is to review the updates of the molecular field about the antibody against GluK2 and its clinical presentation in pediatric population; COVID-19 as a possible cause of autoimmune encephalitis; recognize the importance of psychiatric manifestation in early disease, especially catatonia as a marker of severity; additionally consider new imaging diagnostic method such as positron emission tomography (PET), which has shown to be more sensible than MRI (goal standard).
自身免疫性脑炎可定义为继发于多种病因的中枢神经系统炎症，在此过程中我们有可能识别出针对神经递质受体或神经元表面蛋白的自身抗体形成。约50%的患者血清学呈阳性；针对N-甲基-D-天冬氨酸受体（NMDAR）的自身抗体最为常见。在儿童群体中，临床表现以运动障碍和癫痫发作为特征，精神症状在年轻成人中更为常见。早期干预与这些患者的较好预后相关。与血清学阳性组相比，血清学阴性的自身免疫性脑炎运动改变较少，但认知结局较差。关于可能的病因、分子过程、检测以及尚未描述的抗体的相互作用，仍有许多有待发现之处，同时也需要增加我们对疾病早期临床表现以及可改善自身免疫性脑炎诊断的新诊断技术的了解。本文献的主要目的是回顾关于抗GluK2抗体的分子领域进展及其在儿童群体中的临床表现；新冠病毒病作为自身免疫性脑炎的一种可能病因；认识精神症状在疾病早期的重要性，尤其是紧张症作为病情严重程度的一个指标；此外，考虑新的成像诊断方法，如正电子发射断层扫描（PET），其已显示出比磁共振成像（金标准）更敏感。
