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New-Onset Refractory Status Epilepticus in Children: Etiologies, Treatments, and Outcomes
Khalil S Husari1,2, Katherine Labiner1,3, Rong Huang4
1Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX.
Insights
Most pediatric new-onset refractory status epilepticus cases lack identifiable causes. While treatments vary, outcomes like epilepsy development and neurocognitive impairment are common, highlighting the need for better diagnostic and treatment strategies.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurocritical Care
Background:
- New-onset refractory status epilepticus (NORSE) in children presents significant diagnostic and therapeutic challenges.
- Understanding the etiologies and outcomes of pediatric NORSE is crucial for improving patient management.
Purpose of the Study:
- To investigate the causes, treatments, and functional/neurocognitive outcomes of children with NORSE.
- To identify factors influencing outcomes in pediatric NORSE patients.
Main Methods:
- A retrospective study was conducted at a single tertiary care children's hospital.
- Data from 40 pediatric patients (1 month to 21 years) with NORSE admitted between 2004 and 2017 were analyzed.
- Etiologies, treatments, and outcomes (functional, neurocognitive, epilepsy development) were assessed.
Main Results:
- The etiology of NORSE remained cryptogenic in over half of the cases; viral infections were the most common identified cause (20%).
- Neuronal antibody positivity was rare. Immunotherapy was used in 50% of patients.
- 12.5% of patients died during the acute phase. Of the survivors, 65% developed epilepsy, and 58% had persistent neurocognitive impairment. No significant differences in outcomes were found based on etiology or treatment.
Conclusions:
- A significant proportion of pediatric NORSE cases lack identifiable etiologies, differing from adult presentations where neuronal antibodies are more common.
- Super-refractory status epilepticus was associated with poorer outcomes.
- Standardized diagnostic and treatment protocols, along with multicenter prospective studies, are needed to improve outcomes for children with NORSE.
Objectives:
To elucidate etiologies, treatment, functional and neurocognitive outcomes of children with new-onset refractory status epilepticus.
Design:
A single-center retrospective study.
Setting:
A tertiary care children's hospital.
Patients:
All patients between 1 month and 21 years old admitted with new-onset refractory status epilepticus between January 2004 and July 2017.
Interventions:
None.
Measurements And Main Results:
Clinical presentation, laboratory data, imaging studies, and treatments were collected during hospitalization. Outcomes were assessed at hospital discharge and follow-up in the outpatient neurology clinic based on functional and neurocognitive outcomes as well as development of epilepsy. A total of 674 unique patients presented with status epilepticus of which 40 had new-onset refractory status epilepticus. Patients were classified into either refractory status epilepticus or super-refractory status epilepticus. The etiology of most children with new-onset refractory status epilepticus remained cryptogenic. The most common identified etiology was viral (20%). None of the patients had a contributory positive neuronal antibody test. Several treatments were tried including immunotherapy which was used in half of the patients. Five patients died (12.5%) during the acute phase of their disease, with four lost to follow-up. Twenty out of the remaining 31 patients (65%) developed epilepsy and 18 (58%) had persistent neurocognitive impairment. There was no statistical significant difference in various outcome measures and various etiologies, patients' characteristics, and treatments.
Conclusions:
In this single-center cohort, more than half of the children with new-onset refractory status epilepticus did not have an identifiable etiology. Unlike adult patients, the presence of positive neuronal antibody syndrome was rare. There was no difference in outcome between those with or without an identifiable etiology. As expected, patients with super-refractory status epilepticus had worse functional and neurocognitive outcomes. More standardized diagnostic and treatment algorithms are needed along with prospective multicenter studies.
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