Abstracts

Rationale: Super-Refractory Status Epilepticus (SRSE) is defined as status epilepticus that continues for at least 24 hours after initiation of general anesthetic medications, and includes cases in which status epilepticus recurs with reduction or withdrawal of anesthesia. The ketogenic diet (KD) is a high-fat, low carbohydrate, sufficient protein diet that has been proven as an effective dietary therapy for some children with intractable epilepsy. Here, we report our experience at Phoenix Children’s Hospital with the utilization of KD for children in SRSE.Methods: A retrospective chart review was performed of pediatric patients with SRSE who underwent KD therapy at Phoenix Children’s Hospital from 2011 to 2014. Identified clinical data included age, gender, seizure etiology, types of KD treatment, concurrent treatments to KD, and clinical outcomes.Results: Ten children (4 female, 6 male), age 2-16 years (median 8) with SRSE were identified. 4/10 patients were found to have antibody mediated encephalitis (1/4 presumed Rasmussen encephalitis, 2/4 NMDA-R encephalitis, and 1 mycoplasma-related encephalitis). 1/10 had Lennox Gastaut Syndrome, 1/10 had non-ketotic hyperglycinemia, 1/10 had PCDH19 and GABR2 gene mutations, 1/10 had New Onset Refractory Status Epilepticus (NORSE), 1/10 was diagnosed with Febrile Infection-Related Epilepsy Syndrome (FIRES), and 1/10 had prior intractable epilepsy of unknown etiology. 3/10 patients had EEG features suggesting focal SRSE, and 7/10 had features suggestive of generalized SRSE. Hospital length ranged 7-288 days (median 61) and ICU length ranged 3-120 days (median 27). The number of antiepileptic medications used prior to KD initiation ranged from 1-6 drugs (median 3), and the number used during KD treatment ranged from 0 to 7 drugs (median 3.5). The number of anesthetic agents attempted prior to KD initiation ranged from 1-3 agents, (mean 2.2). The duration of SRSE ranged from 1-45 days (median 18) prior to initiation of KD. 9/10 patients had resolution of SRSE 1-19 days (median 7 and mean 7.89) after KD treatment was initiated. All patients received Ketocal as their KD therapy. 9/10 patients were placed on a 4:1 ratio, and 1/10 patients placed on a 5:1 ratio. 1/10 patients experienced side effects on KD including ketoacidosis, hypophosphatemia and hypokalemia, requiring supplemental therapy.Conclusions: In our experience, KD has been successful in the treatment of pediatric patients with SRSE of various etiologies. The majority of our pediatric patients had resolution of SRSE within days after initiation of KD. It was well tolerated with little to no side effects, suggesting that it is a safe treatment option for critically ill children with SRSE.