Abstracts

Rationale: Not applicable Methods: Not applicable Results: BW is a 13 year old boy with NF type 1 and focal epilepsy. At an early age, he started having developmental regression as well as unexplainable nausea and vomiting, which were concerning for undiagnosed seizures. He started having focal seizures with impairment in consciousness when he was 8 years old. The semiology included staring with hand automatisms as well as loss of awareness and postural tone and/or eye movements followed by tonic-clonic activity. He is currently on oxycarbazepine and zonisamide. MRI of the brain showed abnormal signal in the amygdala and hippocampus on the left suggestive of mesial temporal sclerosis as well as multifocal areas of vacuolar demyelination and/or hamartomatous change in the right basal ganglia and left temporal lobe consistent with NF1. As part of the pre-surgical work-up, PET-MRI showed decreased uptake in the left temporal lobe, and ictal SPECT showed the seizure focus in the left mesial temporal lobe. Phase 1 admission to the EMU captured multiple seizures all arising from the left temporal region with interictal left temporal epileptiform discharges with semiology of right hand dystonic posturing. Neuropsychology testing showed impairment in left temporal region. WADA and fMRI showed left language dominance with right memory dominance. It was decided to pursue Phase II pre-surgical evaluation with subdural electrodes with left hemispheric coverage. EEG showed seizures arising from the left anterior and mesial temporal lobe with late secondary seizure focus of lateral posterior temporal gyrus. Therefore, after extensive discussion with ophthalmology regarding the optic glioma, it was decided to pursue left anterior temporal lobectomy with RNS strip electrode placement over the superior posterior lateral temporal gyrus for potential future use. Post-surgical specimen showed FCD III (Ia + HS). Since surgery, he has achieved complete seizure remission. He has been started on a slow taper off the oxycarbazepine and continues on the zonisamide.CD is a 26 year old woman with history of NF-1, optic glioma c/b obstructive hydrocephalus s/p bilateral VP shunts, and focal epilepsy. Her seizures were diagnosed at the age of 8 and treated with carbamazepine until 2008. Multiple MRIs of the brain showed left mesial temporal sclerosis along with optic chasm glioma. PET-MRI showed decreased uptake in the left hippocampus and left mesial temporal lobe. She was placed on lamotrigine but continued to have AED intolerance and frequent seizures so was admitted for Phase 1 evaluation in 2013. She had multiple admissions to the EMU. EEG showed multiple seizures originating from the left temporal region with interictal anterior temporal sharp/spike wave discharges. Levetiracetam was initiated due to side effects and ineffectiveness of carbamazepine but then later tapered off due to side effects so Zonisamide was added. WADA and neuropsychological testing showed mild risk for post-operative memory complications if she should undergo left temporal lobectomy but did show left language dominance. She was presented at epilepsy surgery conference and it was agreed to pursue left anterior temporal lobectomy without invasive monitoring with all of the concordant data above. Since surgery, she has had complete seizure remission with her last seizure prior to epilepsy surgery in 2014. She has been continued on all of her anti-epileptic medications to date. Conclusions: Given the outcome of complete seizure remission in both of these patients, these cases highlight the important role that epilepsy surgery, along with a thorough pre-surgical workup, can have in patients with medically refractory epilepsy and pre-existing brain abnormalities caused by a neurodevelopmental disorder.  Funding: Not applicable