Abstracts

Rationale: Refractory status epilepticus (RSE)-seizures lasting longer than 60 minutes despite treatment with at least 2 antiepileptic drugs (AED)-is a challenging clinical condition and there is no consensus on the duration of treatment with intravenous (IV) anesthesia, use of newer AED and other management approaches. A longer duration of status and acute symptomatic etiology have been associated with a poor outcome in children in the literature. Methods: We report a teenager who had a good cognitive and seizure outcome despite acute symptomatic RSE lasting for 14 weeks and prolonged treatment with IV anesthesia. Results: A 14 year old girl developed generalized convulsive status epilepticus preceded by a febrile illness for 5 days. She was previously healthy and reported to be an excellent student. CSF showed elevated protein and mild pleocytosis. MRI brain was normal. Continuous EEG monitoring showed diffuse slowing with runs of multifocal sharp and slow waves. She was presumed to have meningoencephalitis and treated with a course of antibiotics and acyclovir. No organism was identified and a specific etiology was not found despite extensive investigation. Seizures were initially treated with lorazepam, fosphenytoin, phenobarbital and valproic acid. As seizures remained refractory, high-dose suppressive IV anesthesia was used. Several attempts to wean her off anesthetic drugs resulted in recurrence of electrographic and clinical seizures. Concern regarding the morbidity with prolonged use of IV anesthesia led to the use of newer AED - levetiracetam, topiramate, zonisamide and felbamate -and unconventional treatments including IV immunoglobulin, steroids, pyridoxine, vagus nerve stimulation and the ketogenic diet. Surgery was not considered because of multifocal electrographic seizures. Seizures were finally controlled 101 days after onset and anesthetic agents were discontinued. Two weeks later, she had a score of 20/30 on mini-mental status examination and mild weakness in the extremities. EEG was normal. Two months later, neuro-psychological evaluation revealed improving reading skills and memory. She was able to function at fifth grade level. Seven months later, she only had relative attention deficit and difficulty with complex speed tasks, and was able to complete ninth grade with As and Bs. She continued to have seizures but, at her last follow-up visit 18 months after recovery from status, seizure frequency had decreased to an average of 1/month on a combination of levetiracetam and clorazepate. Neurologic examination showed mild difficulty with ambulation and brisk reflexes. EEGs done 2, 4 and 13 months after recovery were normal. Conclusions: Management of patients with RSE is difficult and prospective randomized trials are needed to determine the optimal approach. Prolonged RSE of acute symptomatic etiology is not always associated with a poor cognitive or seizure outcome.