Pretkalnina D, Grinvalde S, Kalnina E
Faculty of Doctoral Studies, Riga Stradins University, Riga, Latvia.
Department of Neurosurgery and Neurology, Childrens Clinical University Hospital, Riga, Latvia.
Neuropediatrics. 2024 Oct;55(5):321-326. doi: 10.1055/s-0044-1788259. Epub 2024 Jul 15.
Autoimmune encephalitis (AE) is the third most common encephalitis in children. Diagnosis can be challenging due to overlapping and diverse clinical presentations as well as various investigation results. This study aims to characterize the clinical, diagnostic features, as well as treatment and outcomes of AE in children and determine the incidence of pediatric AE in Latvia.
The study was conducted at the Children's Clinical University Hospital in Riga. The study participants were patients under the age of 18 years diagnosed with AE from 2014 to 2022. Data regarding clinical characteristics, investigation findings, treatment strategy, and outcomes were retrospectively collected from the medical history data system.
We included 18 pediatric patients diagnosed with AE. The mean incidence of pediatric AE in Latvia was 0.56 per 100,000 children. Most patients (66.6%) had seronegative AE. In the seropositive group, the most common was anti-methyl-D-aspartate receptor AE, with two patients having other antibodies. The most prevalent clinical features were personality change, cognitive impairment, autonomic dysfunction, and movement disorders. The majority of patients (58.8%) received first-line treatment only. More than half (55.6%) of our AE patient group had long-term sequelae.
Our study shows that the pediatric AE incidence in Latvia is similar to what has been previously reported in other studies. A relatively high proportion of seronegative AE was present in our cohort, indicating that awareness of possible misdiagnosis should be raised. Further research is needed to better understand the underlying mechanisms, characterize clinical features, and determine the treatment of choice in different situations to improve long-term outcomes.
自身免疫性脑炎（AE）是儿童中第三常见的脑炎。由于临床表现重叠且多样以及各种检查结果，诊断可能具有挑战性。本研究旨在描述儿童AE的临床、诊断特征以及治疗和结局，并确定拉脱维亚儿童AE的发病率。
该研究在里加的儿童临床大学医院进行。研究参与者为2014年至2022年期间诊断为AE的18岁以下患者。有关临床特征、检查结果、治疗策略和结局的数据是从病史数据系统中回顾性收集的。
我们纳入了18例诊断为AE的儿科患者。拉脱维亚儿童AE的平均发病率为每10万名儿童0.56例。大多数患者（66.6%）患有血清阴性AE。在血清阳性组中，最常见的是抗N-甲基-D-天冬氨酸受体AE，有两名患者有其他抗体。最常见的临床特征是人格改变、认知障碍、自主神经功能障碍和运动障碍。大多数患者（58.8%）仅接受了一线治疗。我们的AE患者组中超过一半（55.6%）有长期后遗症。
我们的研究表明，拉脱维亚儿童AE的发病率与先前其他研究报告的相似。我们的队列中存在相对较高比例的血清阴性AE，这表明应提高对可能误诊的认识。需要进一步研究以更好地了解潜在机制、描述临床特征并确定不同情况下的治疗选择，以改善长期结局。
