Description of Gastrointestinal Symptoms in Patients with New-onset Refractory Status Epilepticus (NORSE)
Anima Shrestha1, Lori Silveira1, Divya Thomas3, Jarrett Leech2, Yi-Chen Lai4, James Riviello5, Eyal Muscal3, Jason Soden6, Krista Eschbach7
1University of Colorado School of Medicine, 2Department of Neurology, University of Colorado School of Medicine, 3Department of Pediatrics, Division of Rheumatology, 4Department of Pediatrics, Division of Critical Care Medicine, 5Departments of Pediatrics and Neurology, Baylor College of Medicine, 6Department of Pediatrics, Section of Gastroenterology, Hepatology, and Nutrition, Children’s Hospital Colorado, 7Department of Pediatrics, Section of Child Neurology, Children's Hospital Colorado
Objective:
To characterize gastrointestinal (GI) symptoms in patients with a diagnosis of new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES).
Background:
NORSE and FIRES are rare clinical presentations in which previously healthy patients develop refractory status epilepticus that does not have a clear structural, toxic, genetic, or metabolic cause, without or with a preceding febrile illness. Anecdotally, some patients experience significant GI concerns after the onset of status epilepticus and initiation of therapy.
Design/Methods:
This is a multicenter retrospective study of pediatric and adult patients with a diagnosis of NORSE or FIRES. Data from initial hospitalization and follow-up was collected via REDCap surveys. Wilcoxon rank-sum tests and Fisher’s exact test were used to compare continuous and categorical variables, respectively. Analysis was performed using R-4.2.1.
Results:
The study included 20 patients with a median age of 11.25 years (IQR 6.75-15.80) at status epilepticus onset. Two were diagnosed with NORSE and 18 with FIRES. Median length of ICU stay was 35.00 days (IQR 15.75-68.25). GI symptoms occurred during initial hospitalization in 90% (n=18). Of these, most frequently reported were vomiting (80%, n=16), constipation (75%, n=15), and abdominal pain (50%, n=10). Seven patients had GI procedures, including colonoscopy, gastrostomy tube placement, and small bowel resection. At median follow-up of 37 months (IQR 10-41), GI symptoms were present in 35% (n=6) of 17 surviving patients. Time in burst suppression (r=0.37, p=0.133) and ketogenic diet use (p=0.298) were not associated with GI symptoms. Number of anti-seizure medications during initial hospitalization was moderately associated with GI symptoms (r=0.47, p=0.042).
Conclusions:
GI symptoms are a common manifestation for patients with NORSE or FIRES during initial hospitalization, and for many may persist after discharge. Further research is needed to determine if symptoms are secondary to the critical illness and treatment or part of the underlying pathogenesis of NORSE and FIRES.