Gastrointestinal symptoms are common in patients with new-onset refractory status epilepticus (NORSE)

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation in which patients develop refractory status epilepticus without a clear cause. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE preceded by febrile illness. Prolonged critical illness and treatments carry a risk of gastrointestinal (GI) disturbance. This study aimed to characterize GI symptoms in patients with NORSE.

This is a multicenter retrospective study of NORSE patients. Data from initial hospitalization and follow-up was collected via REDCap. Analysis was performed using R-4.2.1.

The study included 19 patients with a median age of 11.20 years (IQR 6.70-15.75) at status epilepticus onset, 2 diagnosed with NORSE and 17 with FIRES. GI symptoms occurred during initial hospitalization in 89% of patients. Most frequently reported were vomiting (79%), constipation (73%), and abdominal pain (47%). Seven patients had GI procedures, including colonoscopy and small bowel resection. At median follow-up of 37 months (IQR 10-41), GI symptoms were present in 31% of 16 surviving patients. Time in burst suppression (r = 0.34, p = 0.052), duration of status epilepticus (r=-0.11, p = 0.670), and ketogenic diet (p = 0.213) were not associated with GI symptoms. Number of anti-seizure medications (r = 0.48, p = 0.043) and highest pentobarbital rate (r = 0.51, p = 0.029) were associated.

GI symptoms are a common manifestation for NORSE patients during hospitalization, and for some may persist after discharge. One limitation is potential underreporting of subjective GI symptoms due to altered levels of consciousness and cognitive impairment. Further research may determine if symptoms are secondary to critical illness or part of the underlying pathogenesis of NORSE.