[Super-refractory status epilepticus: treatment with ketogenic diet in pediatrics]

Area of Science:

• Pediatric Neurology
• Epileptology
• Metabolic Therapies

Background:

• Super-refractory status epilepticus (SRSE) is a severe, persistent seizure state unresponsive to standard treatments.
• Existing pharmacological interventions, including induced coma, often fail to resolve SRSE.
• The ketogenic diet has been explored as an alternative therapeutic strategy for refractory epilepsy.

Purpose of the Study:

• To evaluate the efficacy and safety of the ketogenic diet in pediatric patients with SRSE.
• To assess the ketogenic diet's role when conventional anticonvulsant therapies are ineffective.

Main Methods:

• Retrospective analysis of five pediatric patients (ages 1-14) with SRSE.
• Patients included those with febrile infection-related epilepsy syndrome (FIRES) and refractory symptomatic partial epilepsy.
• All patients underwent multiple prior treatments, including barbiturate-induced coma, before initiating a classical ketogenic diet.

Main Results:

• The ketogenic diet led to the cessation of clinical and electroencephalographic seizures in four out of five patients.
• Three patients had FIRES, and two had refractory symptomatic partial epilepsy.
• The diet was well-tolerated by the responding patients; one patient did not respond and unfortunately died.

Conclusions:

• The ketogenic diet is a viable and effective therapeutic option for pediatric SRSE when other treatments fail.
• This dietary intervention demonstrates significant efficacy and a favorable safety profile in managing this critical neurological condition.