(456) Ketogenic Diet for New-Onset Refractory Status Epilepticus

Introduction: New-onset refractory status epilepticus (NORSE) is a presentation of refractory status epilepticus (RSE) that occurs in patients without prior history of epilepsy or neurological disorders, and with no identifiable structural, toxic, or metabolic cause. Optimal treatment remains uncertain, but typically requires continuous infusion antiepileptics and immunotherapy if presumed autoimmune etiology. Adjunctive therapies are being increasingly used, including the ketogenic diet, which has shown potential benefit for RSE.

Description: A 73-year-old man with minimal medical history was admitted to the intensive care unit (ICU) with RSE. He received conventional treatment for RSE with antiepileptic drugs (AED), including continuous AED infusions. Comprehensive workup of neoplastic, autoimmune, and infectious causes was performed during admission, however no clear etiology could be determined. He was initiated on immune therapies with intravenous immune globulin, high-dose corticosteroids, and rituximab in accordance with the international consensus recommendations for NORSE. On ICU day 21, the ketogenic diet was initiated with specialized enteral nutrition. Nutrition consisted of a high fat, very-low carbohydrate enteral nutrition formula. Carbohydrates, including in medications, were strictly minimized. Close monitoring of serum beta-hydroxybutyrate was done to ensure adequate ketosis. Within eight days, seizure activity resolved, and his AED regimen was reduced. On ICU day 48, the patient was transferred out of the ICU and required three additional days of acute hospitalization. He was then discharged to an outpatient rehabilitation hospital and returned home with good functional recovery after one month while remaining on the ketogenic diet.

Discussion: While our patient received many treatments for NORSE, there was a temporal improvement after starting the ketogenic diet. The ketogenic diet appears to be a safe and feasible treatment option for NORSE. Primary barriers include lack of awareness and/or knowledge regarding the ketogenic diet. This case supports the ketogenic diet as an adjunct treatment modality and can assist other clinicians regarding a potentially beneficial treatment for a severe, difficult-to-treat, and life-threating condition.