Department of Pediatrics, Kiang Wu Hospital, Macau; Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
Department of Pediatrics, Min-Sheng Hospital, TaoYuan, Taiwan.
Epilepsy Behav. 2019 Jun;95:117-123. doi: 10.1016/j.yebeh.2019.02.033. Epub 2019 Apr 28.
The febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy which developed the refractory status epilepticus following or during a nonspecific febrile illness. To analyze the short-term and long-term outcome of FIRES in the children, we retrospectively analyzed the related data.
The motor outcome was evaluated by modified Rankin scale (mRS). Poor motor outcome was defined as a mRS score of 4 or higher at discharge. Significant motor decline was defined as the mRS difference more than 2 before hospital admission and at discharge.
We totally enrolled 25 patients for analysis. Four patients were expired during hospitalization, and one patient was lost to follow-up after discharge. Therefore, a total 20 patients were finally analyzed. The age of disease onset ranged from 1.6 to 17.2 years (mean: 9.6 ± 4.4 years). Newly acquired epilepsy and cognitive deficit occurred in 100% and 61%, respectively. The duration of the anesthetic agents ranged from 7 to 149 days (mean: 34.2 ± 36.1 days). The duration of anesthetic agent usage (p = 0.011), refractory epilepsy (p = 0.003), and the use of ketogenic diet (p = 0.004) were significantly associated with the poor long-term motor outcome, and the number of anesthetic agents tended to be associated with the poor long-term motor outcome (p = 0.050). In-hospital mortality was 16%. Significant functional decline at discharge occurred in 100%. However, there was improvement in long-term follow-up.
The outcome of FIRES is poor with significant mortality and morbidities. Refractory epilepsy with cognitive deficit in survived cases is common, but improvement is possible.
发热相关感染性癫痫综合征（FIRES）是一种灾难性的癫痫性脑病，在非特异性发热疾病后或期间发展为难治性癫痫持续状态。为了分析 FIRES 患儿的短期和长期预后，我们回顾性分析了相关数据。
采用改良 Rankin 量表（mRS）评估运动结局。出院时 mRS 评分≥4 定义为运动结局不良。显著运动恶化定义为入院前和出院时 mRS 评分差值＞2。
共纳入 25 例患者进行分析。4 例患者在住院期间死亡，1 例患者出院后失访。因此，最终分析了 20 例患者。发病年龄为 1.6 至 17.2 岁（平均 9.6±4.4 岁）。新获得的癫痫和认知缺陷分别发生在 100%和 61%的患者中。麻醉药物的使用时间为 7 至 149 天（平均 34.2±36.1 天）。麻醉药物的使用时间（p=0.011）、难治性癫痫（p=0.003）和生酮饮食的使用（p=0.004）与长期运动结局不良显著相关，麻醉药物的使用数量与长期运动结局不良也有相关性（p=0.050）。住院期间死亡率为 16%。出院时 100%的患者出现显著功能下降。但在长期随访中有改善。
FIRES 的预后较差，死亡率和发病率较高。存活患者常伴有难治性癫痫和认知缺陷，但可能会改善。
