Treatment options in pediatric super-refractory status epilepticus
Palita Arayakarnkul1, Krisnachai Chomtho2
1Department of Pediatrics, King Chulalongkorn Memorial Hospital, Thailand.
Insights
Super-refractory status epilepticus (SRSE) in children has varied causes, often not epilepsy-related. Treatment should include immunomodulation and metabolic therapies, with ketogenic diets considered early for better outcomes.
Area of Science:
- Pediatric Neurology
- Critical Care Medicine
- Epileptology
Background:
- Super-refractory status epilepticus (SRSE) is a severe neurological emergency characterized by prolonged seizures (>24 hours) despite anesthesia.
- SRSE in children is challenging to manage and associated with poor prognoses, with limited data on optimal therapeutic strategies.
- Understanding the specific etiologies and treatment responses in pediatric SRSE is crucial for improving patient outcomes.
Purpose of the Study:
- To investigate the diverse etiologies contributing to SRSE in pediatric patients.
- To evaluate the efficacy and safety of various treatment modalities employed for pediatric SRSE.
- To assess the clinical outcomes and complications associated with SRSE in children.
Main Methods:
- A retrospective review of medical records for pediatric patients (<15 years) diagnosed with SRSE between 2007 and 2017.
- Data collection included demographic information, seizure etiology, anesthetic agents used, adjunctive therapies, complications, and discharge status.
- Statistical analysis of treatment patterns and patient outcomes.
Main Results:
- Immune-mediated encephalitis and epilepsy were the leading causes of SRSE in 17 pediatric patients.
- Midazolam and propofol were common anesthetic agents; immunomodulatory therapy and ketogenic diets were frequently used (76.5%).
- Common complications included hypotension (61.5%) and drug hypersensitivity (32.5%); the mortality rate was 17.6%, with survivors seizure-free at discharge.
Conclusions:
- Pediatric SRSE often stems from non-epileptic causes, necessitating a broad therapeutic approach beyond traditional antiepileptic drugs.
- Treatments targeting immunomodulation and specific metabolic pathways, such as ketogenic diets, are vital.
- While multiple anesthetics can be managed with close monitoring, early consideration of ketogenic diets is recommended, showing relatively better outcomes in children compared to adults.
Background:
Super-refractory status epilepticus (SRSE) is a seizure that continues >24 h after anesthesia, or recurs on the reduction of anesthesia. SRSE is extremely difficult-to-control and associated with poor outcome. To date, optimal therapy and outcome data in children is limited.
Objective:
To assess etiology, treatment options and outcome in pediatric SRSE patients.
Method:
We reviewed medical records of children <15 years old with SRSE during 2007-2017 at King Chulalongkorn Memorial Hospital. Demographic data, etiology, treatment, complications and discharge outcome were recorded.
Results:
Seventeen patients, aged 1 month-13 years were included. The leading etiology was immune-mediated encephalitis (29.4%) and epilepsy (29.4%). The most common anesthetic agents were midazolam (94.1%) and propofol (52.9%) with the average maximal dose of 1.3 and 6.9 mg/kg/h respectively. Other treatments included immunological therapy (76.5%), ketogenic diet (76.5%), pyridoxine/pyridoxal-5-phosphate (70.5%). The most common complications were hypotension (61.5%), drug hypersensitivity (32.5%). Median length of anesthetic and intensive care were 9 and 23 days. The mortality rate was 17.6%, and 2 of 3 febrile infection-related epilepsy syndrome cases died. At discharge, all survivors were seizure free.
Conclusion:
The majority of pediatric SRSE does not have epilepsy and the etiology is various. Treatment should expand from antiepileptic drugs to other modalities targeting different possible mechanisms such as immunomodulation or specific metabolic treatment. Multiple anesthetic drugs could be tolerated with close monitoring. Ketogenic diet, via enteral or parenteral route, could be considered early if requiring multiple anesthetic drugs. Initial outcome in children is relatively better than in adults.
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