Resective surgery to treat refractory status epilepticus in children with focal epileptogenesis Abstract—Prolonged high-dose suppressive therapy (HDST) is a mainstay in the management of refractory status epilepticus (RSE), albeit with high mor- bidity and mortality. The authors studied 10 patients who were carefully selected for epilepsy surgery after failing prolonged (2 weeks) HDST. Status epilepticus was stopped acutely in all of them with no mortality and no sub- stantial morbidity. At follow-up (median 7 months), 7 (70%) of 10 patients were seizure free, and 3 (30%) of 10 had significant improvement in their epilepsy. NEUROLOGY 2005;64:567–570 A. Alexopoulos, MD; D.K. Lachhwani, MD; A. Gupta, MD; P. Kotagal, MD; A.M. Harrison, MD; W. Bingaman, MD; and E. Wyllie, MD Refractory status epilepticus (RSE) is status epilepti- cus (SE) that fails to respond to high-dose suppres- sive therapy (HDST). 1,2 Prolonged HDST is associated with significant mortality and morbidity. In a long-term study of previously healthy children who survived RSE treated with prolonged HDST, none returned to baseline neurologic function and all developed epilepsy. 3 The prognosis was worse with preexisting neurologic disease. Mortality rates in children after RSE range from 16% to 43.5%. 4-6 Patients with RSE of focal origin may be poten- tially amenable to resective surgery. However, the scant literature is limited to isolated case reports or small case series involving multiple subpial transec- tions, cortical resection, corpus callosotomy, or im- plantation of a vagus nerve stimulator. 7,8 The results have been mixed, and there is lack of consensus about epilepsy surgery in the midst of a medical emergency. Our current study highlights the role of resective epilepsy surgery in patients presenting with RSE due to focal epileptogenesis. Methods. From a pediatric epilepsy surgery database of 283 consecutive children who underwent epilepsy surgery at The Cleveland Clinic Foundation between 1997 and 2004, we identi- fied patients who underwent epilepsy surgery due to RSE. The majority of patients with RSE seen at our institution are not surgical candidates, but 10 patients had emergent resective epi- lepsy surgery to treat RSE after failing 2 weeks of aggressive pharmacotherapy in the pediatric intensive care unit. The consen- sus to perform surgery was arrived at after careful consideration of clinical details during patient management conferences, ap- proval from the bioethics committee, and informed parental con- sent. Relevant demographic, clinical, and follow-up information was obtained by chart review. Results. The 10 children (six girls, four boys) presented with RSE at 3 days to 16 years (median 4 years, 3 months) of age, after onset of epilepsy at 1 day to 11 years (median 2.5 months) of age (table). At the time of RSE, nine pa- tients had frequent motor seizures and one had severe combative epileptic encephalopathy requiring restraints. All patients required care in the pediatric intensive care unit, and nine needed mechanical ventilation. Six patients had lobar (1), multilobar (2), and hemi- spheric (3) malformations of cortical development; one of these patients had epidermal nevus syndrome and one had tuberous sclerosis with perinatal cardiac rhabdomyoma and positive tuberous sclerosis 1 mutation. One additional patient had tuberous sclerosis with typical skin findings and multiple bilateral cortical tubers. The remaining three patients had Rasmussen encephalitis (1), prenatal middle and anterior cerebral artery infarction (1), and unclear diagnosis (1) (presumed Rasmussen encephalitis with com- plicated findings). MRI and EEG abnormalities were congruent during the RSE in 8 of 10 patients and incongruent in 2 of 10 (one had hemispheric encephalomalacia opposite the side with ictal and interictal epileptiform abnormalities, whereas the other had bilateral diffuse cerebral volume loss and exclu- sively unihemispheric EEG abnormalities). Five of the six children with hemispheric processes (malformation of cor- tical development or Rasmussen syndrome) had hemipare- sis at the time of RSE. Patients had not been considered for epilepsy surgery before the onset of RSE due to one or more of the following reasons: infrequent seizures before RSE (2), presentation with RSE within 1 month of epilepsy onset (4), or presence of complicating factor(s) (four patients had bilateral in- volvement on EEG and/or MRI and three patients had onset of seizures from regions including the eloquent cor- tex). However, in all our patients, there was evidence (based on semiology, EEG, or neuroimaging) supporting a focal or unihemispheric origin of SE. The failure of aggres- sive medical management in controlling SE tipped the scales toward consideration of resective surgery, although some of the concerning preoperative factors had remained unchanged. Medical management of RSE in every case included HDST with more than one agent (phenobarbital, pentobar- bital, midazolam, propofol, fentanyl, isoflurane) given IV. In some cases, all HDST was performed at our institution, whereas others were transferred to our institution after failing HDST at an outside hospital. All the patients were obtunded or in a coma. Oral antiepileptic medications were also given via a nasogastric tube. Patients remained in From the Departments of Neurology (Drs. Alexopoulos, Lachhwani, Gupta, Kotagal, and Wyllie), Pediatric Critical Care Medicine (Dr. Harrison), and Neurosurgery (Dr. Bingaman), The Cleveland Clinic Foundation, Cleve- land, OH. Received April 30, 2004. Accepted in final form September 15, 2004. Address correspondence and reprint requests to Dr. Deepak K. Lachhwani, Section of Pediatric Epilepsy and Pediatric Neurology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, S-51, Cleveland, OH 44195; e-mail: lachhwd@ccf.org Copyright © 2005 by AAN Enterprises, Inc. 567