Diao Haiyan, Qu Haixia, Wang Yanhong
Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences "Tongji Shanxi Hospital", Taiyuan, China.
Medicine (Baltimore). 2025 Apr 4;104(14):e39214. doi: 10.1097/MD.0000000000039214.
There are relatively few studies related to new-onset refractory status epilepticus (NORSE), in which patients with cryptogenic NORSE have more frequent and longer duration seizures than patients with a clear etiology, are more likely to develop drug-refractory epilepsy and have a more likely prognosis of severe cognitive impairment. We conducted a series of case studies to investigate the treatment options and prognosis of NORSE. Seven adult patients with cryptogenic NORSE treated at the Third Hospital of Shanxi Medical University between June 2016 and April 2022 were reviewed. We collected basic information and clinical data of the study subjects, including demographic characteristics, clinical manifestations, laboratory test and imaging data, medication use, and prognostic regression, using a modified Rankin Scale score (MRS). The median age of the 7 patients with cryptogenic NORSE was 32 years (interquartile range 25.00-39.00), and 4 patients (57.1%) were male. Six patients (85.7%) had a history of prodromal fever; 2 (28.6%) patients received high-dose propofol and midazolam and 5 (71.4%) patients received high-dose phenobarbital, of which 4 patients (57.1%) were treated with a ketogenic diet KD. The overall mortality rate was 28.6% (2/7), and 2 patients died during hospitalization (both treated with high-dose propofol and midazolam). Of all surviving patients at discharge, 3 patients (60%) had an mRS score of 2 and 2 patients (40%) had an mRS score of 4. In the 3-month post-discharge follow-up data, all surviving patients had an mRS score of 2. In a retrospective study of our cohort high-dose phenobarbital combined with a ketogenic diet may be safe and effective in the treatment of cryptogenic NORSE.
与新发难治性癫痫持续状态（NORSE）相关的研究相对较少，其中隐源性NORSE患者比病因明确的患者癫痫发作更频繁、持续时间更长，更易发展为药物难治性癫痫，且严重认知障碍的预后可能性更大。我们进行了一系列病例研究以探讨NORSE的治疗选择和预后。回顾了2016年6月至2022年4月在山西医科大学第三医院接受治疗的7例成年隐源性NORSE患者。我们使用改良Rankin量表评分（MRS）收集了研究对象的基本信息和临床数据，包括人口统计学特征、临床表现、实验室检查和影像学数据、用药情况以及预后回归情况。7例隐源性NORSE患者的中位年龄为32岁（四分位间距25.00 - 39.00），4例（57.1%）为男性。6例（85.7%）有前驱发热史；2例（28.6%）患者接受了大剂量丙泊酚和咪达唑仑治疗，5例（71.4%）患者接受了大剂量苯巴比妥治疗，其中4例（57.1%）患者接受了生酮饮食（KD）治疗。总死亡率为28.6%（2/7），2例患者在住院期间死亡（均接受了大剂量丙泊酚和咪达唑仑治疗）。出院时所有存活患者中，3例（60%）的MRS评分为2，2例（40%）的MRS评分为4。在出院后3个月的随访数据中，所有存活患者的MRS评分为2。在对我们队列的一项回顾性研究中，大剂量苯巴比妥联合生酮饮食治疗隐源性NORSE可能是安全有效的。
