Related Experiment Video
Updated: Oct 6, 2025
Author Spotlight: Advancing Pediatric Epilepsy Surgery in Children Through Novel Biomarkers and Enhanced Localization
Published on: September 20, 2024
Specific characteristics and current diagnostic and treatment modalities performance of super refractory status
George Briassoulis1, Chrysavgi Stefanogianni1, Ioannis Zaganas2
1Pediatric Intensive Care Unit, University Hospital, School of Medicine, University of Crete, Heraklion, Crete, Greece.
Insights
Super-refractory status epilepticus (SRSE) in children leads to poor outcomes and prolonged hospital stays. Early diagnosis and treatments like immunotherapy or ketogenic diets show promise for better seizure control and functional recovery.
Area of Science:
- Pediatric intensive care
- Neurology
- Epileptology
Background:
- Super-refractory status epilepticus (SRSE) presents significant challenges in pediatric intensive care units (PICUs), associated with high morbidity and mortality.
- Understanding the clinical spectrum and outcomes of SRSE compared to status epilepticus (SE) and refractory SE (RSE) is crucial for improving patient management.
Purpose of the Study:
- To explore the clinical characteristics, treatment modalities, and outcomes of pediatric patients with SRSE admitted to a PICU.
- To compare the performance of diagnostic and treatment approaches in SRSE against SE and RSE cohorts.
- To identify predictors of poor outcomes in pediatric SRSE patients.
Main Methods:
- Retrospective analysis of 86 pediatric patients with convulsive SE admitted to a PICU between 2009 and 2019.
- Classification of patients into SE, RSE, and SRSE groups, with identification of New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES).
- Evaluation of functional outcomes using the modified Rankin scale and analysis of diagnostic and treatment modalities.
Main Results:
- SRSE patients (n=20) experienced longer mechanical ventilation, hospital stays, and poorer outcomes compared to SE (n=13) and RSE (n=53) patients.
- High prevalence of NORSE (n=13) and FIRES (n=9) was associated with SRSE. Abnormal MRI findings and epilepsy-related pathogenic variants via whole-exome sequencing (WES) were more common in SRSE.
- Plasmapheresis and ketogenic diet were more effective for seizure control in SRSE than intravenous immunoglobulins or steroid pulses. SRSE and diagnosis were significant predictors of poor outcome.
Conclusions:
- Pediatric SRSE is linked to substantial functional decline and morbidity.
- WES analysis can identify epilepsy-related variants, and early aggressive immunotherapy or ketogenic diets may improve outcomes.
- Further multicenter studies are required to develop robust outcome prediction models for pediatric SRSE.
Objective:
Super-refractory status epilepticus (SRSE) is associated with significant morbidity and mortality in children. We explored the clinical spectrum, specific characteristics, and outcome in SRSE patients admitted in a pediatric intensive care unit (PICU) and investigated how well current diagnostic or treatment modalities perform compared to Status Epilepticus (SE) and Refractory SE (RSE) patients.
Methods:
Retrospective analysis of PICU patients admitted with convulsive SE during 2009-2019. Eighty-six patients were classified as SE, RSE, and SRSE. New-onset RSE (NORSE) and febrile infection-related epilepsy syndrome (FIRES) were also identified. Functional outcome was evaluated by the modified Rankin scale.
Results:
Patients with SRSE (n = 20) had longer weaning off anesthetics (p = 0.014), length of stay, mechanical ventilation duration, higher illness severity scores, and poorer outcome compared to SE (n = 13) or RSE (n = 53) patients (all p < 0.001). Diagnosis, mainly expressed by high prevalence of NORSE (n = 13) and FIRES (n = 9), was independently associated with SRSE (p = 0.024). Abnormal MRI findings (p = 0.005), and epilepsy-related pathogenic variants identified by whole-exome sequencing (WES) were mostly found in SRSE patients. Compared to intravenous immunoglobulins and steroid pulses, plasmapheresis and ketogenic diet, more often used in SRSE (p < 0.01), contributed better to seizure control. Only SRSE (AUROC > 0.80, 95% CI = 0.68-0.94, p < 0.001) and diagnosis (AUROC > 0.70, 95% CI = 0.55-0.83, p = 0.02) could predict a poor outcome.
Conclusion:
The majority of SRSE patients are characterized by considerable functional decline and morbidity. WES analysis may reveal epilepsy-related pathogenic variants while early aggressive immunotherapy and/or ketogenic diet might prove beneficial. Multicenter studies for prediction models of outcome are needed.
More Related Videos
08:43Long-term Continuous EEG Monitoring in Small Rodent Models of Human Disease Using the Epoch Wireless Transmitter System
Published on: July 21, 2015
10:22Interictal High Frequency Oscillations Detected with Simultaneous Magnetoencephalography and Electroencephalography as Biomarker of Pediatric Epilepsy
Published on: December 6, 2016
Related Concept Videos
01:24Epilepsy and Seizures: Overview
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
01:13Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types: