Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Department of Neurology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan.
Medicine (Baltimore). 2021 Jul 30;100(30):e26660. doi: 10.1097/MD.0000000000026660.
Steroid pulse therapy is widely used to treat virus-associated acute encephalopathy, especially the cytokine storm type; however, its effectiveness remains unknown. We sought to investigate the effectiveness of early steroid pulse therapy for suspected acute encephalopathy in the presence of elevated aspartate aminotransferase (AST) levels.We enrolled children admitted to Hyogo Children's Hospital between 2003 and 2017 with convulsions or impaired consciousness accompanied by fever (temperature >38°C). The inclusion criteria were: refractory status epilepticus or prolonged neurological abnormality or hemiplegia at 6 hours from onset, and AST elevation >90 IU/L within 6 hours of onset. We excluded patients with a neurological history. We compared the prognosis between the groups with or without steroid pulse therapy within 24 hours. A good prognosis was defined as a Pediatric Cerebral Performance Category Scale (PCPC) score of 1-2 at the last evaluation, within 30 months of onset. Moreover, we analyzed the relationship between prognosis and time from onset to steroid pulse therapy.Fifteen patients with acute encephalopathy and 5 patients with febrile seizures were included in this study. Thirteen patients received steroid pulse therapy within 24 hours. There was no between-group difference in the proportion with a good prognosis. There was no significant correlation between PCPC and timing of steroid pulse therapy (rs = 0.253, P = .405). Even after excluding 2 patients with brainstem lesions, no significant correlation between PCPC and steroid pulse therapy timing (rs = 0.583, P = .060) was noted. However, the prognosis tended to be better in patients who received steroid pulse therapy earlier.Steroid pulse therapy within 24 hours did not improve the prognosis in children with suspected acute encephalopathy associated with elevated AST. Still, even earlier administration of treatment could prevent the possible neurological sequelae of this condition.
类固醇脉冲疗法广泛用于治疗与病毒相关的急性脑病,特别是细胞因子风暴型;然而,其疗效仍不清楚。我们旨在研究在天门冬氨酸氨基转移酶(AST)升高的情况下,早期使用类固醇脉冲疗法治疗疑似急性脑病的疗效。
我们纳入了 2003 年至 2017 年期间在兵库儿童医院住院的伴有发热(体温>38°C)的惊厥或意识障碍的儿童。纳入标准为:发病 6 小时后出现难治性癫痫持续状态或持续神经功能异常或偏瘫,且发病 6 小时内 AST 升高>90 IU/L。我们排除了有神经病史的患者。我们比较了发病 24 小时内是否使用类固醇脉冲疗法的两组患者的预后。预后良好定义为最后一次评估时的小儿脑功能表现分类量表(PCPC)评分为 1-2,发病后 30 个月内。此外,我们分析了预后与发病至类固醇脉冲疗法时间之间的关系。
本研究纳入了 15 例急性脑病患者和 5 例热性惊厥患者。13 例患者在 24 小时内接受了类固醇脉冲疗法。预后良好的患者比例在两组间无差异。PCPC 与类固醇脉冲疗法时间之间无显著相关性(rs=0.253,P=0.405)。即使排除了 2 例脑干病变患者,PCPC 与类固醇脉冲疗法时间之间也无显著相关性(rs=0.583,P=0.060)。然而,较早接受类固醇脉冲疗法的患者预后较好。
在 AST 升高的疑似急性脑病患者中,发病后 24 小时内使用类固醇脉冲疗法并不能改善预后。尽管如此,尽早进行治疗可能有助于预防这种疾病的潜在神经后遗症。