Patients with Neurofibromatosis Type-1 and temporal epilepsy can be good candidates for epilepsy surgery. (P6.283)

OBJECTIVE: We present two patients with Neurofibromatosis Type I (NF1), Refractory Epilepsy (RE), and atypical temporal lobe epilepsy features that underwent epilepsy surgery after extensive subdural evaluation with good outcome. BACKGROUND: RE in patients with NF1 are often related to complex pathologies such as tumors, cortical dysplasia and vascular complications and therefore epilepsy surgery is rarely contemplated. DESIGN/METHODS: Retrospective chart and video-EEG review. RESULTS: Case 1 is a 37 year old woman with NF1 and RE since age 13. Ictal semiology consisted of a non-specific aura followed by automotor manifestations. Interictal EEG revealed right fronto-temporal slowing and sharp waves while MRI showed right mesial temporal sclerosis. Subdural ictal EEG revealed right mesial abrupt paroxysmal fast followed by severe electro-decrement and reappearance of widespread paroxysmal fast. The patient underwent right temporal lobectomy and became seizure free and revealed cognitive improvement. Neocortical dysplastic changes and hippocampal atrophy were seen on pathology. Case 2 is a 10 year old boy with NF1 and RE for 1 year. Seizure consisted of auras of metallic taste followed by alteration of consciousness as well as left facial clonic seizures. MRI showed mass lesions in the right temporal lobe and posterior frontal lobe. Video-EEG revealed independent right temporal and right fronto-central ictal onsets. Following subdural evaluation a right temporal lobectomy as well as right frontal lesionectomy was performed. Pathology revealed DNET in both tissues. The patient remained seizure free for 1 year, and although recurrence of tumor was associated with new seizures, they were easily controlled. CONCLUSIONS: Patients with NF1 and RE may benefit from invasive EEG evaluation with high chance of obtaining seizure control following tailored epilepsy surgery especially if temporal seizure source is identified. Disclosure: Dr. Weinstock has received personal compensation for activities with Supernus Pharmaceuticals, Cyberonics, and Sunovion. Dr. Ale has nothing to disclose. Dr. Li has nothing to disclose. Dr. Mahfooz has nothing to disclose. Dr. Balos has nothing to disclose. Dr. Li has nothing to disclose.