Abstracts

Rationale: Super-refractory status is defined as "status epilepticus (SE) that continues or recurs 24 hours or more after the onset of anesthesia, including those cases in which SE recurs on the reduction or withdrawal of anesthesia." Treatment modalities include administration of anesthetic agents, anti-epileptic medications, magnesium infusion, pyridoxine, steroids and IVIG, initiation of the ketogenic diet and hypothermia as well as epilepsy surgeries including focal resections and placement of vagus nerve stimulators. A case has previously been reported of a patient with diffuse epileptogenesis in SE successfully treated with CC (1). CC has been projected to decrease epileptogenesis due to an overall reduction in post operative interictal and bisynchronous discharges (2). CC is a surgery with relatively low morbidity and mortality in experienced hands (3). We are presenting two super-refractory pediatric cases with focal epileptogenesis and associated structural lesions deemed not suitable for immediate focal resection or disconnection due to significant risk of incurring additional morbidity. Methods: Two male patients, K.B., age 14 years, (Sturge Weber syndrome) and C. S., age 11 years, (focal cortical dysplasia) required prolonged ICU admissions for super-refractory SE. K.B.'s case was a fulminant presentation of new onset seizures as SE in a high functioning athlete. Over the course of his initial status, he developed contralateral hippocampal signal changes/sclerosis. He was treated with pentobarbital, midazolam and with hypothermia. Over the course of three weeks, multiple attempts to wean pentobarbital resulted in recurrence of epileptiform activity, and a CC was performed. A multilobar disconnection was not offered due to the substantial risk of new motor and cognitive deficits in a previously high functioning patient. C.S., a left handed child with a mild right hemiparesis, had undergone two prior left frontal resections for cortical dysplasia, with resulting development of significant ADHD tendencies, declining school performance and depression. He initially did well following each of his two resective surgeries, although with continued seizures, he underwent placement of a vagus nerve stimulator (VNS), and was initiated on the ketogenic diet. After several days of increased seizures at home with lethargy, he was admitted to the floor, followed by transfer to the ICU. Due to recurrent increased seizures with attempts to wean midazolam, he underwent a CC,, nine days after his initial transfer to the ICU. A more widespread left hemisphere resection was avoided due to concerns of causing additional neuropsychological deficits. Results: Following CC, both patients were able to be weaned from coma inducing medications. K.B. had a recurrence of occipital seizures without dyscognitive features, and a VNS was placed. He has been well controlled for the past 6 months, and has successfully returned to pitching. C.S. continued to have frequent daily focal seizures, although post operatively these seizures were without dyscognitive features or post ictal effects. Seizures gradually abated, and he has been seizure free over the past 6 months, except for one seizure occurring in the setting of illness. Conclusions: CC can reduce overall epileptogenesis and secondary bisynchrony. In these two patients with super refractory SE, CC likely contributed to the resolution of SE. In addition to currently accepted available therapies, CC may provide additional benefit in the treatment of super refractory SE. Funding: None