Abstracts

Rationale: Refractory status epilepticus (SE) is defined as status epilepticus lasting >60 minutes. It occurs in 12-43% of SE cases. However, in a subset of patients, seizures can last >24 hours and may require further treatment. Given the paucity of published data in this area, we present our experience in a pediatric population. Methods: A retrospective review of our EEG database and the medical record was performed on patients seen at Children's Medical Center Dallas/UTSW from 2003 and 2012, who were in super-refractory status epilepticus. This was defined as refractory SE lasting longer than 24 hours despite treatment. We reviewed age, gender, number of days in SE, duration of ICU stay, etiology, and outcome. We also reviewed number of antiepileptic drugs (AEDs), including anesthetic agents and/or immune therapy. Results: 22 patients were identified, 7 girls and 15 boys, ranging from 2 weeks to 16 years old. The duration of status epilepticus ranged from 24 hours to 88 days with an average ICU stay of 17.4 days. Etiologies associated with SRSE included infection (8; 36.4%), autoimmune disease (4; 18.2%), known epilepsy (3; 13.6%), stroke (2; 9%), trauma (2; 9%), unknown (2; 9%), and malignancy (1; 4.5%). The average number of AEDs used was 3.1 (range 1 to 7). Treatment also included at least one general anesthetic agent in 9/15, specifically pentobarbital (7), thiopental (1), and midazolam (6). In patients with a suspected autoimmune process, nonbacterial encephalitis, and those with unknown etiology despite extensive workup, immunotherapy was administered. This included high-dose steroids (8), IVIG (3), plasmapheresis (5), and chemotherapy (1). In one case, ketogenic diet was attempted but child could not achieve adequate ketosis in the ICU. Vagal nerve stimulator was placed in two children. Conclusions: Super-refractory status epilepticus in the pediatric population is associated with a high morbidity and mortality. In our experience, several conclusions were identified, including: (1)In our patients whose SRSE resolved and survived, the majority had significant morbidity including subsequent development of epilepsy, new learning disabilities, developmental regression, and feeding difficulties. (2) There was a 38.3% mortality (8/22 patients). Of note, in 7 of these 8 patients, the underlying medical cause for the status epilepticus likely resulted in their death. (3) In addition, careful consideration needs to be given regarding the potential adverse effects of our interventions. (4)Despite standardization in initial treatment protocols for early and evolving status epilepticus, in patients who progress to SRSE, no universally agreed upon treatment approach exists. Given the diverse etiologies encountered, protocol driven therapy may not be feasible. However, lack of standardization can make treatment decisions difficult in these multidisciplinary care settings. (5) Given significant adverse effects of medications, non-medication alternative therapies such as Ketogenic diet or VNS should be considered for SRSE.