新起难治性癫痫持续状态（NORSE）和病因不明的热性感染相关癫痫综合征（FIRES）：不可比因素的比较？

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) of unknown aetiology: A comparison of the incomparable?

机构信息

Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Arnold-Heller-Street 3, House C, Kiel 24105, Germany.

Department of Neurology, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany.

出版信息

Seizure. 2022 Mar;96:18-21. doi: 10.1016/j.seizure.2022.01.006. Epub 2022 Jan 11.

DOI:10.1016/j.seizure.2022.01.006

PMID:35042004

Abstract

PURPOSE

The discussion is ongoing whether new-onset refractory status epilepticus (NORSE) in adults and febrile infection-related epilepsy syndrome (FIRES) in children are one syndrome if the aetiology is unknown. In this study we will compare an adult cohort with NORSE and a paediatric cohort with FIRES in order to determine if they are similar or different.

METHODS

We retrospectively compared 18 adults with NORSE versus 48 children with FIRES, both cohorts without identifiable cause despite extensive investigations. We analyzed demographic and clinical data using Mann-Whitney-U and χ- tests.

RESULTS

NORSE affected more women (78% vs. 42%; P = 0.009) than in FIRES. Median acute hospital stay was longer in FIRES (35 days [interquartile range, IQR=36] vs. 20 days [IQR=19]; P<0.001). FIRES was treated more frequently with coma therapy (82% vs. 28%; P<0.001) and with a higher median number of antiseizure medicines (7 [IQR=5] vs. 4 [IQR=2]; P<0.001). Children with FIRES showed a higher cerebrospinal fluid (CSF) cell count (10 cells/μl; P = 0.002) but a lower CSF protein level than adults with NORSE (48 mg/dl; P = 0.028). Immunotherapy was administered more frequently in FIRES (73% vs. 22%; P<0.001) than in NORSE. Group differences in number of antiseizure medicines after hospital stay (P = 0.229) and in overall mortality (P = 0.327) were not significant.

CONCLUSION

In our explorative comparison, differences prevailed. NORSE and FIRES should be compared prospectively in age-matched cohorts.

摘要

目的

目前仍在讨论成人新发难治性癫痫持续状态（NORSE）和儿童热性惊厥相关癫痫综合征（FIRES）是否为病因不明时的同一种综合征。本研究将比较成人 NORSE 队列和儿童 FIRES 队列，以确定它们是否相似或不同。

方法

我们回顾性比较了 18 例成人 NORSE 患者和 48 例儿童 FIRES 患者，尽管进行了广泛的检查，但两组均未发现可识别的病因。我们使用 Mann-Whitney-U 和 χ 检验分析了人口统计学和临床数据。

结果

NORSE 组中女性比例高于 FIRES 组（78% vs. 42%；P=0.009）。FIRES 组的急性住院时间中位数更长（35 天[四分位距，IQR=36] vs. 20 天[IQR=19]；P<0.001）。FIRES 更常接受昏迷治疗（82% vs. 28%；P<0.001）和中位数更多的抗癫痫药物治疗（7 种[IQR=5] vs. 4 种[IQR=2]；P<0.001）。FIRES 患儿的脑脊液（CSF）细胞计数较高（10 个/μl；P=0.002），但 CSF 蛋白水平较低（48mg/dl；P=0.028）。免疫治疗在 FIRES 中的应用更为频繁（73% vs. 22%；P<0.001）。住院后抗癫痫药物数量的组间差异（P=0.229）和总体死亡率（P=0.327）无显著差异。