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Published on: December 13, 2017
Pediatric refractory and super-refractory status epilepticus
Alejandra Vasquez1, Raquel Farias-Moeller2, William Tatum3
1Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, United States.
Insights
Pediatric refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) are severe neurological emergencies with limited treatment options. Collaborative research is needed to improve patient outcomes and evaluate current therapies.
Area of Science:
- Pediatric Neurology
- Critical Care Medicine
- Epileptology
Background:
- Refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) are life-threatening neurological emergencies in children.
- These conditions are characterized by prolonged seizures unresponsive to standard antiepileptic drug treatments.
- RSE and SRSE are associated with significant long-term neurological deficits and high mortality rates.
Purpose of the Study:
- To review and summarize current evidence on pediatric RSE and SRSE.
- To focus on epidemiology, causes, treatment strategies, and outcomes.
- To highlight challenges in managing these complex neurological conditions.
Main Methods:
- A narrative review of existing medical literature.
- Searches conducted using the MEDLINE database.
Main Results:
- RSE is defined by failure to respond to first- and second-line antiepileptic drugs; SRSE involves persistent or recurrent seizures despite anesthesia.
- Management is challenging due to unrecognized etiologies and limited therapeutic options, often relying on case series and expert opinions.
- Continuous anesthetic infusion is a common treatment, but optimal protocols are unclear; non-pharmacological therapies have low evidence levels.
- Patients face neurological and systemic complications from prolonged seizures, polypharmacy, and extended hospitalizations.
Conclusions:
- Pediatric RSE and SRSE are critical neurological emergencies with limited therapeutic avenues.
- There is a need for multi-national collaborative studies to assess the safety and efficacy of existing treatments.
- Such collaborative efforts are essential to potentially improve patient outcomes in RSE and SRSE.
Purpose:
To summarize the available evidence related to pediatric refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE), with emphasis on epidemiology, etiologies, therapeutic approaches, and clinical outcomes.
Methods:
Narrative review of the medical literature using MEDLINE database.
Results:
RSE is defined as status epilepticus (SE) that fails to respond to adequately used first- and second-line antiepileptic drugs. SRSE occurs when SE persist for 24 h or more after administration of anesthesia, or recurs after its withdrawal. RSE and SRSE represent complex neurological emergencies associated with long-term neurological dysfunction and high mortality. Challenges in management arise as the underlying etiology is not always promptly recognized and therapeutic options become limited with prolonged seizures. Treatment decisions mainly rely on case series or experts' opinions. The comparative effectiveness of different treatment strategies has not been evaluated in large prospective series or randomized clinical trials. Continuous infusion of anesthetic agents is the most common treatment for RSE and SRSE, although many questions on optimal dosing and rate of administration remain unanswered. The use of non-pharmacological therapies is documented in case series or reports with low level of evidence. In addition to neurological complications resulting from prolonged seizures, children with RSE/SRSE often develop systemic complications associated with polypharmacy and prolonged hospital stay.
Conclusion:
RSE and SRSE are neurological emergencies with limited therapeutic options. Multi-national collaborative efforts are desirable to evaluate the safety and efficacy of current RSE/SRSE therapies, and potentially impact patients' outcomes.
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