Clinical analysis of 7 cases of cryptogenic new-onset refractory status epilepticus

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.