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

Background: 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 have conducted a series of case studies to investigate the treatment options and prognosis of NORSE. Methods Seven adult patients with cryptogenic NORSE who were treated at the Third Hospital of Shanxi Medical University from June 2016 to April 2022 were reviewed. We collected basic information and clinical data of the study subjects, including demographic characteristics, clinical manifestations, laboratory tests and imaging data, medication use, and prognostic regression; the prognosis was assessed by a modified Rankin Scale score (MRS). Results 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. 6 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), with 2 patients dying 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. Conclusion In a retrospective description of our cohort high-dose phenobarbital and a ketogenic diet were found to be probably safe and effective in the treatment of cryptogenic NORSE.