Intensive Care Unit, Meyer Children's Hospital, Florence, Italy.
Nephrology Department, Meyer Children's Hospital, University of Florence, Florence, Italy.
Epilepsia Open. 2021 Dec;6(4):788-792. doi: 10.1002/epi4.12545. Epub 2021 Oct 20.
Febrile infection-related epilepsy syndrome (FIRES) is a devastating immune inflammatory-mediated epileptic encephalopathy. Herein, we discuss a previously healthy 8-year-old boy with FIRES in whom high dosages of conventional and nonconventional anesthetics were ineffective in treating SE, as were ketogenic diet, intravenous corticosteroids, and immunoglobulins. After 29 days of prolonged SRSE, the patient was successfully treated with sevoflurane paired with plasma exchange, for a total of five days, thus obtaining a stable EEG suppression burst pattern with no adverse events. Anakinra at the dosage of 100 mg b.i.d. was started seven days after sevoflurane and plasma exchange had been discontinued and was effective in ensuring non-recurrence of SE. Sevoflurane as bridge therapy for immunosuppressive treatment could be considered an early, safe, and effective option in treating convulsive SE in which an autoimmune-inflammatory etiology can reasonably be hypothesized.
发热相关感染性癫痫综合征(FIRES)是一种具有破坏性的免疫炎症介导性癫痫性脑病。本文讨论了一位先前健康的 8 岁男孩,患有 FIRES,大剂量常规和非常规麻醉药物、生酮饮食、静脉注射皮质类固醇和免疫球蛋白均不能有效治疗 SE。在长达 29 天的持续性 SRSE 后,患者成功接受七氟醚联合血浆置换治疗,共 5 天,从而获得稳定的 EEG 抑制爆发模式,无不良反应。在停用七氟醚和血浆置换后 7 天,开始给予患者剂量为 100mg bid 的阿那白滞素,有效防止 SE 再次发作。对于可以合理假设自身免疫炎症病因的惊厥性 SE,七氟醚作为免疫抑制治疗的桥梁治疗可能是一种早期、安全、有效的选择。