Abstract：
New-Onset Refractory Status Epilepticus (NORSE) is a rare and severe form of refractory status epilepticus without an apparent underlying cause at presentation or prior history of epilepsy. We aimed to describe the clinical features, etiology, treatment, and outcomes of NORSE in adults in a quaternary-level hospital in Saudi Arabia.
In this retrospective cohort study, inclusion criteria involved patients over 14 years old who met the 2018 consensus definition for NORSE. Patients were identified using a combination of medical record admission labels \'status epilepticus\' and \'encephalitis\', and continuous EEG reports documenting status epilepticus. Demographic, clinical, and radiological data were collected and then analyzed for factors correlated with specific etiologies, better functional outcomes, and future diagnosis of epilepsy.
We found 24 patients presenting with NORSE between 2010 and 2021. Fever/infectious symptoms were the most common prodrome. Elevated inflammatory serum and cerebrospinal fluid markers in most patients. Brain MRI revealed T2/FLAIR hyperintensity patterns, predominantly affecting limbic and perisylvian structures. The etiology of NORSE varied, with immune-related causes being the most common. Long-term outcomes were poor, with a high mortality rate and most survivors developing drug-resistant epilepsy.
This study provides valuable insights into NORSE\'s clinical characteristics, highlighting the heterogeneity of this condition. The poor outcome is likely related to the progressive nature of the underlying disease, where refractory seizures are a clinical symptom. Thus, we propose to focus future research on the etiology rather than the NORSE acronym.
In this retrospective cohort study, inclusion criteria involved patients over 14 years old who met the 2018 consensus definition for NORSE. Patients were identified using a combination of medical record admission labels \'status epilepticus\' and \'encephalitis\', and continuous EEG reports documenting status epilepticus. Demographic, clinical, and radiological data were collected and then analyzed for factors correlated with specific etiologies, better functional outcomes, and future diagnosis of epilepsy.
We found 24 patients presenting with NORSE between 2010 and 2021. Fever/infectious symptoms were the most common prodrome. Elevated inflammatory serum and cerebrospinal fluid markers in most patients. Brain MRI revealed T2/FLAIR hyperintensity patterns, predominantly affecting limbic and perisylvian structures. The etiology of NORSE varied, with immune-related causes being the most common. Long-term outcomes were poor, with a high mortality rate and most survivors developing drug-resistant epilepsy.
This study provides valuable insights into NORSE\'s clinical characteristics, highlighting the heterogeneity of this condition. The poor outcome is likely related to the progressive nature of the underlying disease, where refractory seizures are a clinical symptom. Thus, we propose to focus future research on the etiology rather than the NORSE acronym.
摘要：
目的：新发作难治性癫痫持续状态（NORSE）是一种罕见且严重的难治性癫痫持续状态，在出现或既往有癫痫病史时没有明显的潜在病因。我们的目的是描述临床特征，病因学,治疗,沙特阿拉伯一家四级医院的成人NORSE结果。
方法：在这项回顾性队列研究中，纳入标准涉及符合2018年NORSE共识定义的14岁以上患者.患者使用医疗记录入院标签“癫痫持续状态”和“脑炎”的组合进行识别，和连续脑电图报告记录癫痫持续状态。人口统计,临床,收集放射学数据，然后分析与特定病因相关的因素，更好的功能结果，和未来的癫痫诊断。
结果：我们发现在2010年至2021年期间有24例患者出现NORSE。发热/感染症状是最常见的前驱症状。大多数患者血清炎症和脑脊液标志物升高。脑部MRI显示T2/FLAIR高强度模式,主要影响边缘和旁系结构。NORSE的病因各不相同，与免疫相关的原因是最常见的。长期结果很差，死亡率高，大多数幸存者发展为耐药性癫痫。
结论：这项研究为NORSE的临床特征提供了有价值的见解，突出了这种情况的异质性。不良结果可能与潜在疾病的进行性有关，难治性癫痫是临床症状。因此，我们建议将未来的研究重点放在病因上，而不是NORSE缩写。
方法：在这项回顾性队列研究中，纳入标准涉及符合2018年NORSE共识定义的14岁以上患者.患者使用医疗记录入院标签“癫痫持续状态”和“脑炎”的组合进行识别，和连续脑电图报告记录癫痫持续状态。人口统计,临床,收集放射学数据，然后分析与特定病因相关的因素，更好的功能结果，和未来的癫痫诊断。
结果：我们发现在2010年至2021年期间有24例患者出现NORSE。发热/感染症状是最常见的前驱症状。大多数患者血清炎症和脑脊液标志物升高。脑部MRI显示T2/FLAIR高强度模式,主要影响边缘和旁系结构。NORSE的病因各不相同，与免疫相关的原因是最常见的。长期结果很差，死亡率高，大多数幸存者发展为耐药性癫痫。
结论：这项研究为NORSE的临床特征提供了有价值的见解，突出了这种情况的异质性。不良结果可能与潜在疾病的进行性有关，难治性癫痫是临床症状。因此，我们建议将未来的研究重点放在病因上，而不是NORSE缩写。
