Abstracts

Rationale: Tuberous sclerosis complex (TSC) is a multisystem disorder with intractable epilepsy necessitating multi-staged invasive monitoring/resective surgery. We introduce multi-staged MRI-guided laser ablation in children with TSC. Methods: We present five TSC patients with intractable epilepsy. Initial ablation, targeting the most active tubers, is followed every 2 months by repeated evaluation to identify active epileptic tubers treated by subsequent stages of ablation. Post-operative video-EEG monitoring clarifies acute response to treatment. Six-year-old boy, with SEGA and refractory partial seizures, underwent laser-ablation of the SEGA, then three active tuber conglomerates in left superior/inferior frontoparietal, inferior temporal regions in 2 months, and left occipital tubers in 8 months. Two-year-old girl with seizures involving right temporal regions, underwent ablation of these areas, followed by ablation of bitemporal tuber conglomerates in 2 months, left occipital tubers in 3 months, right occipital tubers in 5 months. Seventeen-year-old girl, with intractable complex partial/generalized epilepsy with neurocognitive delays, underwent ablation of the most active left posterior parietal tubers. Four-year-old girl, with generalized/complex partial epilepsy (spasms, hemispasms, tonic seizures, staring episodes: 5-10 clusters/day), autism spectrum disorder, had ablation of right frontal, left anterior medial and anterior superior insula, and left inferior posterior cingulate tubers. Eight-year-old boy with autism spectrum disorder, history of infantile spasms, simple and complex partial seizures originating focally from the left posterior temporal, occipital regions and sleep potentiated interictal spikes solely involving the same regions, underwent MRI-guided thermal laser ablation of the left posterior quadrant tuber conglomerate. Results: First patient, with daily complex partial seizures, is seizure free with improving autistic features 16 months after the first procedure. The second patient, with daily myoclonic seizure clusters, hypsarrhythmia and regression, has occasional staring episodes with improving neurocognitive skills 16 months after the first ablation. The third patient has one complex partial episode every 3 to 4 months 10 months post procedure (45 seizures/day previously). The fourth patient has 1-2 brief myoclonic episodes with improving behavioral/neurocognitive difficulties, 3 months post procedure. The fifth patient has been seizure free with immediate improvement in his behavioral outbursts, 1week post procedure. Conclusions: Staged application of MRI-guided thermal laser ablation technology is a promising novel alternative treatment option for intractable epilepsy and neurocognitive difficulties in TSC patients.