Six adult patients with septo-optic dysplasia and drug-resistant epilepsy: Clinical findings and course

Epilepsy Behav Case Rep. 2017 Apr 19:8:73-84. doi: 10.1016/j.ebcr.2017.04.001. eCollection 2017.

Abstract

Septo-optic dysplasia (SOD) is a rare disorder associated with optic nerve hypoplasia, pituitary abnormalities and agenesis/dysgenesis of midline brain structures including the septum pellucidum and corpus callosum. Though sometimes associated with drug-resistant epilepsy, this association has not been well studied. We report six SOD patients with associated malformation of cortical development (MCD) and drug-resistant epilepsy who underwent video-EEG telemetry at our centre between 1998 and 2016 for drug-resistant epilepsy. ‬Three then underwent surgery; right temporal neocortical resection, right functional hemispherectomy and placement of a vagus nerve stimulator. Clinical findings and the patients' ultimate courses are discussed.

Keywords: Cortical malformation; Drug-resistant; Epilepsy; Seizure surgery; Seizures; Septo-optic dysplasia.