Abstracts

Rationale: ome patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. Majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development. Methods: Surgically treated 10 patients using the posterior quadrantectomy (PQT) were enrolled in this study. Surgical outcome was analyzed as seizure-free or not at two years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (MDI) and psychomotor developmental index (PDI) in the Bayley scales of infant development-II preoperatively, at 6- and 12-months after the PQT. Results: Eight out of 10 patients resulted seizure free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12-month after the PQT in seizure free patients were 80.5 and 84.5, respectively (P < 0.01). PDIs at these post-operative intervals were 87.3 and 86.4, respectively (P < 0.05). Patients with residual seizures did not improve both in MDI and PDI. Conclusions: The PQT achieved good seizure control and improved psychomotor development in patients with SWS. The complete deafferentation of angiomatous areas is required for seizure free results and psychomotor developmental improvement.