严重、难治性癫痫发作：新发难治性癫痫持续状态和热性感染相关性癫痫综合征。

Severe, Refractory Seizures: New-Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome.

机构信息

Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Division of Epilepsy and Clinical Neurophysiology, Program in Neuroimmunology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

出版信息

Med Clin North Am. 2024 Jan;108(1):201-213. doi: 10.1016/j.mcna.2023.05.020. Epub 2023 Jul 26.

DOI:10.1016/j.mcna.2023.05.020

PMID:37951651

Abstract

NORSE (new-onset refractory status epilepticus) and FIRES (febrile infection-related epilepsy syndrome) represent presentations of new-onset status epilepticus without apparent underlying structural, metabolic, or toxic etiology. The cause of NORSE/FIRES remains cryptogenic in up to half of cases, and an abnormal response of the innate immune system has been implicated. Consensus guidelines recommend broad diagnostic investigation and empiric treatment with immunotherapy. NORSE/FIRES is associated with poor outcomes including cognitive impairment and epilepsy, but early recognition and treatment may be important for improving outcomes.

摘要

NORSE（新发难治性癫痫持续状态）和 FIRES（热性感染相关癫痫综合征）均代表无明显潜在结构、代谢或毒性病因的新发癫痫持续状态。多达一半的 NORSE/FIRES 病例病因仍不明，且先天免疫系统的异常反应已被牵涉其中。共识指南建议广泛进行诊断性检查，并采用免疫疗法进行经验性治疗。NORSE/FIRES 与不良结局相关，包括认知障碍和癫痫，但早期识别和治疗可能对改善结局至关重要。