Fukui Kana Okazaki, Kubota Masaya, Terashima Hiroshi, Ishiguro Akira, Kashii Hirofumi
Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Japan; Center for Postgraduate Education and Training, National Center for Child Health and Development, Japan.
Division of Neurology, National Center for Child Health and Development, Japan.
Brain Dev. 2019 Aug;41(7):618-624. doi: 10.1016/j.braindev.2019.02.015. Epub 2019 Mar 9.
Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is the most prevalent encephalopathy in Japanese children. AESD is characterized by a prolonged febrile seizure on day 1 followed by secondary seizures and MRI abnormality on days 4-6, resulting in high incidence of neurological sequelae. We aimed to clarify whether early administration of vitamins (vitamin B1, vitamin B6, and l-carnitine) would improve the clinical course of AESD.
We retrospectively reviewed 34 patients with acute encephalopathy who were admitted to our hospital between January 2009 and August 2016. Of the retrospectively registered 34 patients, 22 (65%) since 2011 were treated with the drug cocktail (prescription group) within 24 h of onset, whereas 12 (35%) before 2011 were not (non-prescription group). We compared clinical course, laboratory data, and MRI findings historically in both groups.
The two groups did not differ in terms of laboratory findings except for blood lactate values. There were no differences between the two groups regarding duration of ICU admission, intubation, or the duration of seizures. Among the prescription group, two patients developed AESD while 20 had mild encephalopathy (single phasic). In contrast, seven patients inthe non-prescription group developed AESD while five did not. The incidence of AESD was lower in the prescription group (P = 0.004). As for outcomes, the rate of developmental delay and epilepsy was significantly lower in the prescription group.
Our data suggested that early administration of vitamins would improve the clinical course of acute encephalopathy. Mitochondrial rescue and neuroprotection are thought to be responsible for the favorable results.
伴有双相性癫痫发作和晚期弥散受限的急性脑病(AESD)是日本儿童中最常见的脑病。AESD的特征是在第1天出现长时间发热性癫痫发作,随后在第4至6天出现继发性癫痫发作和MRI异常,导致神经后遗症的高发生率。我们旨在阐明早期给予维生素(维生素B1、维生素B6和左旋肉碱)是否会改善AESD的临床病程。
我们回顾性分析了2009年1月至2016年8月期间入住我院的34例急性脑病患者。在回顾性登记的34例患者中,2011年以来的22例(65%)在发病后24小时内接受了药物联合治疗(处方组),而2011年之前的12例(35%)未接受治疗(非处方组)。我们对两组患者的临床病程、实验室数据和MRI结果进行了历史性比较。
除血乳酸值外,两组的实验室检查结果无差异。两组在ICU住院时间、插管时间或癫痫发作持续时间方面无差异。在处方组中,2例患者发展为AESD,而20例为轻度脑病(单相性)。相比之下,非处方组中有7例患者发展为AESD,5例未发展。处方组中AESD的发生率较低(P = 0.004)。至于预后,处方组中发育迟缓及癫痫的发生率显著较低。
我们的数据表明,早期给予维生素可改善急性脑病的临床病程。线粒体挽救和神经保护被认为是取得良好结果的原因。