Abstracts

Rationale: Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem disorder that causes intractable epilepsy in children. Most patients require multi-staged invasive monitoring/resective surgery. We introduce the use of multi-staged MRI-guided laser ablation in children with TSC.Methods: We present two patients with tuberosclerosis complex and intractable epilepsy. Following decision of surgical intervention, parents gave informed consent for laser ablation approach. Initial ablation was performed targeting the most active tubers, followed, in 2 months, by repeated evaluation to identify active epileptic tubers that were treated by subsequent second stage ablation. Immediate post-operative video-EEG monitoring was done in both stages to assess acute response to treatment. A 6-year-old TSC boy, with refractory partial seizures. Brain-MRI showed multiple tubers and SEGA. Hence, first stage laser-induced thermal ablation of the SEGA was performed to prevent hydrocephalus. In 2 months, second stage-ablation of three active tuber conglomerates located in the left superior/inferior frontoparietal region and inferior temporal region was done. A 2-year-old girl with TSC and seizures located the right anterior, mid-posterior temporal regions. Hence the patient underwent first stage laser ablation on these areas. In 2 months, second stage, ablation was performed targeting epileptogenic bitemporal tuber conglomerates. Results: The first patient had daily complex partial seizures preoperatively. One day post-ablation of the SEGA the patient s aggression, behavioral difficulties started improving. One day after the second stage, the patient became seizure free with significant improvement in autistic features. The patient has been seizure free without major autistic features over 4 months following the ablations. The second patient had multiple clusters of myoclonic seizures daily. Following the first stage, hypsarrhythmia pattern diminished except for mild staring episodes and seizures have been under control. She regained verbal, personal/social/motor milestones. Following the second ablation, her development improved; video EEG monitoring did not reveal any epileptiform correlation to the brief staring episodes. Conclusions: MRI-guided laser ablation technology is a novel alternative treatment option for TSC patients.