Changes in the treatment of pediatric acute encephalopathy in Japan between 2015 and 2021: A national questionnaire-based survey

Background: Although acute encephalopathy (AE) is the most serious disorder associated with a viral infection in childhood and often causes death or neurological sequelae, standard treatments have not been established. In 2016, the Japanese Society of Child Neurology published the "Guidelines for the Diagnosis and Treatment of Acute Encephalopathy in Childhood 2016" (AE GL 2016). We conducted a questionnaire survey to evaluate the status of the treatment of pediatric AE in 2021 and the changes in treat-ment before and after the publication of the AE GL 2016.Methods: In October 2021, questionnaires were mailed via the web to members of two mailing lists who were involved in the practice of pediatric neurological disorders.Results: Most Japanese physicians (98 %) engaged in the treatment of pediatric AE used the AE GL 2016 as a clinical reference. From 2015 to 2021, the number of institutions that implemented targeted temperature management (TTM), vitamin administration, and continuous electroencephalographic monitoring increased significantly. Regarding the targeted temperature for TTM, the pro-portion of patients who were treated with normothermia (36.0-37.0 degrees C) increased from 2015 (55 %) to 2021 (79 %). The use of cor-ticosteroids in patients with AE caused by a cytokine storm, which is recommended in the AE GL 2016, had already been implemented in most institutions by 2015.Conclusion: The AE GL 2016 could be used to disseminate the knowledge accumulated to date. Evidence of the efficacy and proper indication criteria for the treatment of AE is insufficient and must be further accumulated.(c) 2022 Published by Elsevier B.V. on behalf of The Japanese Society of Child Neurology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

背景：尽管急性脑病（AE）是儿童期与病毒感染相关的最严重疾病，常导致死亡或神经后遗症，但标准治疗方法尚未确立。2016年，日本儿童神经病学学会发布了《2016年儿童急性脑病诊断和治疗指南》（AE GL 2016）。我们进行了一项问卷调查，以评估2021年儿童AE的治疗状况以及AE GL 2016发布前后治疗的变化。 方法：2021年10月，通过网络将问卷邮寄给两个涉及儿童神经系统疾病诊疗的邮件列表成员。 结果：大多数从事儿童AE治疗的日本医生（98%）将AE GL 2016作为临床参考。从2015年到2021年，实施目标温度管理（TTM）、维生素给药和持续脑电图监测的机构数量显著增加。关于TTM的目标温度，接受正常体温（36.0 - 37.0℃）治疗的患者比例从2015年（55%）增加到2021年（79%）。AE GL 2016中推荐的对细胞因子风暴引起的AE患者使用糖皮质激素，到2015年大多数机构已经实施。 结论：AE GL 2016可用于传播迄今为止积累的知识。AE治疗的有效性证据和适当的适应证标准不足，必须进一步积累。 （c）2022由爱思唯尔公司代表日本儿童神经病学学会出版。这是一篇在CC BY - NC - ND许可（http://creativecommons.org/licenses/by - nc - nd/4.0/）下的开放获取文章。