Department of Neurosciences, Apollo Speciality Hospitals, Madurai, India.
Department of Pediatrics, Apollo Speciality Hospitals, Madurai, India.
Epilepsia Open. 2021 Jan 15;6(1):22-27. doi: 10.1002/epi4.12454. eCollection 2021 Mar.
A 14-year-old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection-Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable.
一位 14 岁男孩出现前驱性呼吸道感染，随后出现难治性癫痫持续状态。诊断为发热感染相关性癫痫综合征（FIRES）。最初的 MRI 研究和 CSF 分析正常。由于癫痫发作的难治性，他需要使用多种抗癫痫药物。疾病的过程相当汹涌，伴有各种医疗问题，如肝功能障碍、脓毒症、血液动力学和血液学异常，这在管理方面带来了诸多挑战。噬血细胞性淋巴组织细胞增生症（HLH）被确定为疾病的病因，并进行了治疗，但没有成功。该病例报告强调了为治疗该疾病而采用的几种免疫调节策略，但最终结果并不理想。
