Li Wen-Jing, Xue Chun-Ling, Zhang Yong, Wu Li-Hui, Chen Dong-Mei, Chen Feng, Xu Jing, Li Zhuo, Miao Hong-Jun
Department of Pharmacy, Children's Hospital of Nanjing Medical University, Nanjing, China.
Department of Emergency, Children's Hospital of Nanjing Medical University, Nanjing, China.
Transl Pediatr. 2021 Sep;10(9):2392-2397. doi: 10.21037/tp-21-121.
Management of frequent epileptic seizures in febrile infection-related epilepsy (FIRES) is often challenging. FIRES is an uncommon disease condition. Children with FIRES develop refractory epilepsy with severe cognitive deficits that affect the function of the temporal and frontal lobes. However, better seizure control during the acute stage of FIRES could protect against injury to the nervous system. Ketogenic diet (KD) can effectively resolve super-refractory status epilepticus (SRSE) in the acute phase and improve the prognosis of FIRES. We present the case of a previously healthy 3-year-old male with new-onset status epilepticus (SE) admitted to the paediatric intensive care unit for 55 days. Despite treatment with multiple anti-epileptic agents in addition to IV anaesthetics, the patient remained in SRSE and continued to have generalised epileptic activity on electroencephalography (EEG). KD therapy was initiated on the 14th day of the onset, and the patient achieved complete neurological recovery following the KD. Throughout the remainder of admission, the patient was successfully weaned off the ventilator, tolerated oral meals, and worked with occupational and physical therapists to return to his baseline functional status. The convulsions were well controlled after discharge. We discuss the treatment strategies for FIRES and highlight the role of KD therapy in the acute phase to control disease progression and improve the prognosis, and early diagnosis of FIRES and early initiation of KD therapy combined with anti-epileptic drugs (AEDs) could improve the prognosis.
发热感染相关性癫痫综合征（FIRES）中频繁癫痫发作的管理通常具有挑战性。FIRES是一种罕见的疾病状态。患有FIRES的儿童会发展为难治性癫痫，并伴有严重的认知缺陷，影响颞叶和额叶的功能。然而，在FIRES急性期更好地控制癫痫发作可以预防神经系统损伤。生酮饮食（KD）可以有效解决急性期的超难治性癫痫持续状态（SRSE），并改善FIRES的预后。我们报告了一例先前健康的3岁男性患儿，新发癫痫持续状态（SE），入住儿科重症监护病房55天。尽管除静脉麻醉剂外还使用了多种抗癫痫药物进行治疗，但患者仍处于SRSE状态，脑电图（EEG）上持续存在全身性癫痫活动。在发病第14天开始生酮饮食治疗，患者在接受生酮饮食后实现了完全神经功能恢复。在住院的剩余时间里，患者成功脱机，耐受口服饮食，并与职业和物理治疗师合作恢复到基线功能状态。出院后惊厥得到良好控制。我们讨论了FIRES的治疗策略，强调了生酮饮食治疗在急性期控制疾病进展和改善预后中的作用，并且FIRES的早期诊断以及生酮饮食治疗与抗癫痫药物（AEDs）的早期联合应用可以改善预后。
