Abstracts

Rationale: To assess the outcomes of epilepsy surgery in pediatric patients with various intractable epileptic encephalopathies (EE). Methods: Ninety-nine children (67 boys, 32 girls) with intractable EE were treated with epilepsy surgery such as resective surgery, hemispherotomy, and corpus callosotomy (CC) at Severance Children’s Hospital from 2003 to 2007. The study included 76 children of Lennox-Gastaut syndrome (LGS), 19 children of West syndrome, 3 infants of early infantile epileptic encephalopathy (EIEE), and one patient of Rasmussen encephalitis. Clinical characteristics, findings of presurgical evaluation, type of surgery, pathologic findings, and seizure and developmental outcomes after epilepsy surgery, were reviewed. Results: Of 76 patients with LGS, CC was performed in 37 patients (48.7%), resective surgery in 29 (38.2%), and hemispherotomy in 10 (13.2%). Engel’s class I outcomes were achieved in 8 of 10 patients (80.0%) who had hemispherotomy, in 16 of 29 (55.2%) who had resective surgery, and in 10 of 37 (27.0%) who had CC. Of 19 with West syndrome, resective surgery was performed in 12 patients (63.2%), CC in 4 (21.1%), and hemispherotomy in 3 (15.8%). Engel’s class I outcomes were achieved in 8 of 12 (66.7%) who had resective surgery, and 1 of 3 (33.3%) who had hemispherotomy. Of 3 patients with EIEE, two had hemispherotomy and obtained Engel’s class I outcomes and one patient had resective surgery and obtained Engel’s class II outcome. Malformations of cortical development including microdysgenesis and cortical dysplasia were the most common pathology seen in children undergoing resective surgery or hemispherotomy, as follows: in 26 of 39 patients (66.7%) with LGS, in 11 of 15 (73.3%) with West syndrome, and in 2 of 3 (66.7%) with EIEE. In neuropsychiatric tests, 20 of 26 patients with LGS had improvement in cognitive function after surgery. Conclusions: Epilepsy surgery should be considered in catastrophic EE with suspected underlying focal epileptogenic region.