레녹스 가스토 증후군에서 뇌량절제술

Abstract

Purpose: Lennox-Gastaut syndrome (LGS) is one of the most devastating pediatric epileptic syndromes characterized by various disabling seizures, generalized forms of abnormal epileptiform EEG features and progressive psychomotor retardation. Corpus callosotomy is one of treatment options in patients with intractable LGS without definitive focal cortical pathology. This study was designed to evaluate efficacy and safety of corpus callosotomy in patients with LGS. Patients and Methods: Thirty patients (21 boys, 9 girls) with LGS who had corpus callosotomy at Severance Children’s Hospital from October 2003 to January 2007, were enrolled with mean followup of 34.6±14.0 months. We retrospectively reviewed medical records, video-EEG monitoring, MRI, seizure outcome and postoperative complications. Results: Mean age of corpus callosotomy was 100.7±56.4 months. Twenty-four patient (80.0%) underwent total corpus callosotomy and 6 (20.0%) underwent resection of the anterior 4/5 of corpus callosum. Seizure outcomes were as follows: 5 patients (16.7%) were Engel class I, 9 (30.0%) were class II, 6 (20.0%) were class III and 10 (33.3%) were class IV. Postoperative complications were seen in 4 patients (13.3%), such as hemorrhage (2 patients), involuntary movement (1 patient) and transient ataxia (1 patient), but all were recovered completely. Subsequent resective surgery was performed in 5 patients of Engel class III and IV, whose EEG features became lateralized without complete seizure control. Satisfactory surgical outcomes (class I and II) were achieved in 46.7% of total patients. Conclusions: Corpus callosotomy could be effective and safe treatment in medically intractable LGS without focal pathology