Abstract Details

Title Description of Gastrointestinal Symptoms in Patients with New-onset Refractory Status Epilepticus (NORSE)

Topic Epilepsy/Clinical Neurophysiology (EEG)

Presentation(s) P6 - Poster Session 6 (8:00 AM-9:00 AM)

Poster/Presentation
Number 1-004

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.

Authors/Disclosures
Anima Shrestha, MD PRESENTER	Miss Shrestha has nothing to disclose.
Lori Silveira (University of Colorado Anschutz)	No disclosure on file
Divya Thomas (Baylor College of Medicine/ Texas Children's Hospital)	No disclosure on file
Jarrett Leech, MD (University of Colorado Denver)	Dr. Leech has nothing to disclose.
Yi-Chen Lai	No disclosure on file
James J. Riviello, Jr., MD (Texas Children'S Hospital)	Dr. Riviello has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biomarin. Dr. Riviello has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for J. Kiffin Penry Foundation. An immediate family member of Dr. Riviello has received publishing royalties from a publication relating to health care. Dr. Riviello has received personal compensation in the range of $500-$4,999 for serving as a Visiting Professor with University of Utah. Dr. Riviello has received personal compensation in the range of $500-$4,999 for serving as a Visiting Professor with University of Calgary.
Eyal Muscal	Eyal Muscal has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sobi. An immediate family member of Eyal Muscal has stock in pfizer.
Jason Soden	No disclosure on file
Krista L. Eschbach, MD (Childrens Hospital Colorado)	The institution of Dr. Eschbach has received research support from Neurocrine Biosciences. The institution of Dr. Eschbach has received research support from UCB Biopharma. The institution of Dr. Eschbach has received research support from Pediatric Epilepsy Research Foundation. Dr. Eschbach has a non-compensated relationship as a Medical Science Advisory Board Member with NORSE Institute that is relevant to AAN interests or activities.

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