Michael Seaman, MD
Resident Physician
University of North Dakota, United States
Disclosure information not submitted.
Title: Ketogenic Diet in the Treatment of FIRES-associated SRSE: Fad or Fact?
Case Report Body:
Introduction: The ketogenic diet (KD) has been used to treat drug-resistant epilepsy for many years. However, its use in the treatment of Febrile Infection-Related Epilepsy Syndrome (FIRES) and Super-Refractory Status Epilepticus (SRSE) has been controversial. Despite its low cost and safety, it is often initiated only as a last resort. Although the exact mechanism through which the KD leads to resolution of seizure activity is unknown, it appears to exhibit anti-epileptic, anti-inflammatory, and neuroprotective effects making it an ideal therapy in FIRES.
Description: A 17-year-old male was admitted to the PICU after presenting in status epilepticus with acute respiratory failure requiring intubation. For several days prior to his presentation, he had a febrile gastrointestinal illness. He was sedated but at times required up to seven AEDs to suppress his epileptiform activity. No cause was identified for his SRSE, which led to a diagnosis of Cryptogenic FIRES. Various treatments were initiated to control his seizures including corticosteroids, rituximab, plasma exchange, cyclophosphamide, and anakinra. However, all treatments failed, and he continued to experience breakthrough seizure activity. A KD was implemented approximately 3 months into his hospitalization. Subsequently, he was successfully weaned from all anesthetic agents. Although he still required several AEDs to eliminate seizure activity, his neurocognitive status gradually improved enough to be discharged.
Discussion: SRSE in FIRES represents a neurologic emergency and is associated with high morbidity and mortality. Rapid control and cessation of the epileptic activity is key to preventing long-term disability. In the past, treatment options have focused on AEDs and sedation to accomplish this goal. However, these have significant systemic toxicity especially with prolonged exposure. Studies have attempted to delineate the efficacy of KD in SRSE and early implementation has been associated with a favorable prognosis. Nevertheless, widespread application has yet to occur due to continued skepticism. This case provides a unique insight into the potential relationship as the KD was implemented after other therapies had been exhausted. Achievement of ketosis was associated with control of seizures and neurocognitive improvement.