Abstracts

Rationale: There are limited data on the use of non-pharmacological treatments for refractory status epilepticus (RSE). We describe a series of children (1 month to 21 years age) with use of ketogenic diet (KD) for treatment of convulsive RSE. Methods: Data was collected as an ongoing, multi-center (pediatric status epilepticus research group, pSERG), observational cohort of children with RSE (defined as failure of ≥2 anti-seizure medications), admitted to any of the 11 participating institutions from 2011-2015. The primary efficacy outcome was the proportion of patients with electrographic (EEG) seizure resolution within 7 days of starting KD. EEG seizure resolution was defined as ≥50% of the record showing suppression below 10 µV on longitudinal bipolar montage (i.e. suppression-burst ratio ≥50%). Other outcomes included time to start KD after onset of RSE, time of achieve ketosis (serum beta-hydroxybutyrate levels >20 mg/dl [=1.1 mmol/l]) after starting KD, and proportion of patients weaned off continuous infusions 2 weeks after initiation of KD. Treatment-emergent adverse effects were also recorded. Results: A total of 8 patients (8/211, 3.8%) received KD for RSE (Table 1). Out of these, 2 patients (25.0%) were already on ketogenic diet (ketogenic ratios 3:1 and 3.75:1 respectively) prior to the admission for RSE with evidence of adequate ketosis. Their ketogenic ratios were left unchanged after the onset of RSE. Of these 8 patients, 6 (75.0%) received KD via enteral route including through nasogastric and naso-jejunal tubes, and via percutaneous jejunostomy. The initial ratio was 4:1 in 5 patients, and 3:1 in 1 patient. The patient who was started on 3:1 diet was later escalated to 4:1 ratio. In patients who were not on KD prior to admission for RSE, the diet was started a median of 10.5 days (IQR 13.3) after the onset of RSE. In 3/6 patients (50.0%), KD was started within a week of onset of RSE. After starting KD, ketosis was achieved in these patients after a mean of 4.7 ± 3.0 days. Ketosis was achieved within 6 days of starting KD in 5/6 (83.3%) patients. The primary efficacy end-point of electrographic seizure resolution was achieved in 4/8 (50%) patients, within 7 days of starting KD. Within 2 weeks of starting KD, 3 of the above 4 patients (75%) were weaned off the continuous infusions. In 1 remaining patient, infusions were decreased but not completely weaned off. Infusions were also weaned off in 1 additional patient who did not meet the primary efficacy endpoint but later achieved EEG seizure remission on KD. Of the 8 patients, 2 (25.0%) also received IVIg and plasma exchange. In 1 of these 2 patients, immunotherapies were clearly not effective and seizure remission was achieved after starting KD. KD was discontinued in 3/8 (37.5%) patients before discharge due to significant elevation of triglycerides, gastrointestinal adverse effects, failure to achieve ketosis, or lack of clear efficacy. Other adverse effects included abdominal distention (n=1), and weight loss which necessitated decrease in the ketogenic ratio from 4:1 to 3:1 (n=1). One patient died due to unrelated causes. Conclusions: KD can be probably efficacious for management of pediatric RSE, given 50% patients achieving EEG seizure resolution within 7 days of starting the diet. Funding: (Funded by the Pediatric Epilepsy Research Foundation)