Explorations Neurophysiologiques, Département de Neurologie, Hôpital Pierre-Paul-Riquet, Purpan, CHU de Toulouse, Toulouse, France; Centre de Recherche Cerveau et Cognition, Université de Toulouse, Université Paul-Sabatier Toulouse, Toulouse, France; CerCo, UMR 5549, Centre National de la Recherche Scientifique, Toulouse Mind and Brain Institute, Toulouse, France.
Unité Cognition, Épilepsie, Mouvements Anormaux, Département de Neurologie, Hôpital Pierre-Paul-Riquet, Purpan, CHU de Toulouse, Toulouse, France.
Rev Neurol (Paris). 2020 Jun;176(6):408-426. doi: 10.1016/j.neurol.2019.12.010. Epub 2020 Apr 21.
Status epilepticus (SE) is a potentially serious condition that can affect vital and functional prognosis and requires urgent treatment. Etiology is a determining factor in the patient's functional outcome and in almost half of all cases justifies specific treatment to stop progression. Therefore, identifying and addressing the cause of SE is a key priority in SE management. However, the etiology can be difficult to identify among acute and remote causes, which can also be multiple and interrelated. The most common etiologies are the discontinuation of antiepileptic medication in patients with a prior history of epilepsy, and acute brain aggression in cases of new onset SE (cerebrovascular pathologies are the most common). The list of remaining possible etiologies includes heterogeneous pathological contexts. Refractory SE and especially New-Onset Refractory Status Epilepticus (NORSE) lead to an extension of the etiological assessment in the search for encephalitis of autoimmune or infectious origin in adults and in children, as well as a genetic pathology in children in particular. This is an overview of current knowledge of SE etiologies and a pragmatic approach for carrying out an etiological assessment based on the following steps: - Which etiological orientation is identified according to the field and clinical presentation?; - Which etiologies to look for in an inaugural SE?; - Which first-line assessment should be carried out? The place of the biological, EEG and imaging assessment is discussed; - Which etiologies to look for in case of refractory SE?
癫痫持续状态（SE）是一种潜在的严重疾病，可能会影响重要的和功能性的预后，并需要紧急治疗。病因是患者功能预后的决定因素，在近一半的病例中，需要进行特定的治疗以阻止病情进展。因此，确定和解决 SE 的病因是 SE 管理的关键优先事项。然而，在急性和远程病因之间，病因可能难以识别，而且病因也可能是多个且相互关联的。最常见的病因是有癫痫病史的患者停止抗癫痫药物治疗，以及新发 SE 的急性脑损伤（最常见的是脑血管病变）。剩余可能病因的列表包括异质的病理情况。难治性 SE 特别是新发性难治性癫痫持续状态（NORSE）导致在寻找成人和儿童自身免疫或感染性脑炎以及儿童特别是遗传病理学时，对病因评估的扩展。这是对 SE 病因的当前知识的概述，以及根据以下步骤进行病因评估的实用方法：
根据领域和临床表现确定哪些病因方向？
在新发 SE 中应寻找哪些病因？
应进行哪些一线评估？讨论了生物学、EEG 和影像学评估的位置；
在难治性 SE 的情况下应寻找哪些病因？
