Abstracts

RATIONALE: Refractory status epilepticus (RSE) is the persistence of seizure activity despite appropriate antiepileptic drug (AED) therapy. RSE has a high morbidity and mortality. Treatment is typically with high-dose anesthetic agents, aiming for complete control of clinical and electrographic seizures followed by a wean of suppressive therapy. The duration of therapy may vary and it is recommended that therapy be continued for as long as needed in a patient with a healthy pre-morbid state, a self-limited disease process, and the absence of a lesion on neuroimaging suggesting a poor prognosis. In reviews of adult RSE, approximately 29% of patients returned to baseline (RTB).
METHODS: We retrospectively reviewed cases of RSE treated in our own intensive care unit (N=22) and classified them by etiology: acute symptomatic (AS), remote symptomatic (RS), remote symptomatic with acute precipitant (RSAP), and progressive encephalopathy (PE). 8/22 had AS RSE. Other cases of RSE (N=2) were identifed from our own outpatient group. All had been treated with high-dose suppressive therapy for up to 146 days. Seizure frequency was determined from the last clinic visit.
RESULTS: The overall mortality was 7/22 in children treated at our institution. In the AS group, the mortality was 3/8 and none of the 5 survivors RTB. In the RS group (N=5), none died and 3/5 RTB. In the RSAP group (N=6), 2 died and 3/6 RTB. In our total group of AS survivors (n=7), none are seizure free and all have refractory epilepsy. The seizure types are partial in all, with the best control classified as either intermittent (N=1) or 5 per month (N=1). The remainder (N=5) all have daily seizures. All survivors are on multiple AEDs and vagal nerve stimulation (VNS) has been done on 2. All survivors have significant learning problems, none attend a regular school, 2 are mute, and 1 is vegetative. An evaluation for epilepsy surgery was done in two children, one with initial partial RSE, but bilateral foci were identified and VNS was done.
CONCLUSIONS: Despite the capability of prolonged treatment of RSE with suppressive therapy, there is a poor prognosis associated with acute symptomatic RSE in children. None of our children returned to baseline functioning and all have refractory epilepsy. We do not recommend changing current guidelines for the treatment of RSE, but it is important for physicians and families to be aware of the prognosis.