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Super-Refractory Status Epilepticus in Children: A Retrospective Cohort Study
Alejandra Vasquez1,2, Raquel Farias-Moeller3, Iván Sánchez-Fernández1,4
1Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Insights
Pediatric super-refractory status epilepticus (SRSE) is associated with delayed treatment, increased complications, and higher mortality compared to non-SRSE. Treatment for SRSE is highly variable, indicating a need for clearer clinical guidelines.
Area of Science:
- Neurology
- Pediatric Critical Care
Background:
- Super-refractory status epilepticus (SRSE) is a rare but severe neurological emergency in children.
- Characterizing the pediatric SRSE population is crucial for understanding treatment variability and outcomes.
Purpose of the Study:
- To compare clinical characteristics and outcomes between pediatric SRSE and non-SRSE patients.
- To describe treatment variability within the pediatric SRSE population.
Main Methods:
- Retrospective cohort study using prospectively collected data from 17 US academic hospitals (June 2011-January 2019).
- Included pediatric patients (1 month-21 years) with convulsive refractory status epilepticus.
- Defined SRSE as seizures lasting ≥24 hours after continuous infusion initiation.
Main Results:
- 31 SRSE patients were identified among 281 total.
- SRSE patients experienced delayed initiation of non-benzodiazepine antiseizure medications and continuous infusions.
- SRSE was associated with prolonged seizure duration, longer ICU stays, more medical complications, lower return to baseline function, and higher mortality.
- Treatment for SRSE was heterogeneous, with varying numbers of continuous infusions required for resolution.
Conclusions:
- Pediatric SRSE patients face worse outcomes, including higher mortality and complications, compared to non-SRSE patients.
- Delayed treatment initiation and heterogeneous therapeutic approaches characterize pediatric SRSE.
- Further research is needed to establish evidence-based guidelines for managing pediatric SRSE.
Objectives:
To characterize the pediatric super-refractory status epilepticus population by describing treatment variability in super-refractory status epilepticus patients and comparing relevant clinical characteristics, including outcomes, between super-refractory status epilepticus, and nonsuper-refractory status epilepticus patients.
Design:
Retrospective cohort study with prospectively collected data between June 2011 and January 2019.
Setting:
Seventeen academic hospitals in the United States.
Patients:
We included patients 1 month to 21 years old presenting with convulsive refractory status epilepticus. We defined super-refractory status epilepticus as continuous or intermittent seizures lasting greater than or equal to 24 hours following initiation of continuous infusion and divided the cohort into super-refractory status epilepticus and nonsuper-refractory status epilepticus groups.
Interventions:
None.
Measurements And Main Results:
We identified 281 patients (157 males) with a median age of 4.1 years (1.3-9.5 yr), including 31 super-refractory status epilepticus patients. Compared with nonsuper-refractory status epilepticus group, super-refractory status epilepticus patients had delayed initiation of first nonbenzodiazepine-antiseizure medication (149 min [55-491.5 min] vs 62 min [33.3-120.8 min]; p = 0.030) and of continuous infusion (495 min [177.5-1,255 min] vs 150 min [90-318.5 min]; p = 0.003); prolonged seizure duration (120 hr [58-368 hr] vs 3 hr [1.4-5.9 hr]; p < 0.001) and length of ICU stay (17 d [9.5-40 d] vs [1.8-8.8 d]; p < 0.001); more medical complications (18/31 [58.1%] vs 55/250 [22.2%] patients; p < 0.001); lower return to baseline function (7/31 [22.6%] vs 182/250 [73.4%] patients; p < 0.001); and higher mortality (4/31 [12.9%] vs 5/250 [2%]; p = 0.010). Within the super-refractory status epilepticus group, status epilepticus resolution was attained with a single continuous infusion in 15 of 31 patients (48.4%), two in 10 of 31 (32.3%), and three or more in six of 31 (19.4%). Most super-refractory status epilepticus patients (30/31, 96.8%) received midazolam as first choice. About 17 of 31 patients (54.8%) received additional treatments.
Conclusions:
Super-refractory status epilepticus patients had delayed initiation of nonbenzodiazepine antiseizure medication treatment, higher number of medical complications and mortality, and lower return to neurologic baseline than nonsuper-refractory status epilepticus patients, although these associations were not adjusted for potential confounders. Treatment approaches following the first continuous infusion were heterogeneous, reflecting limited information to guide clinical decision-making in super-refractory status epilepticus.
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