Abstracts

Rationale: To describe successful, multi-staged MRI-guided thermal laser ablation in 5 children with tuberous sclerosis complex (TSC).Methods: Five TSC patients with intractable epilepsy underwent MRI-guided thermal laser ablation of active epileptogenic tuber conglomerates, initial ablation targeting the most active tubers, followed every 2 months by re-evaluation to identify active epileptic tubers treated by subsequent stages of ablation. A 6-year-old boy, with a subependymal giant cell astrocytoma (SEGA) and refractory partial seizures, underwent laser-ablation of the SEGA, then three active tuber conglomerates (left superior/inferior frontoparietal, inferior temporal) in 2 months, and left occipital tubers in 8 months. A 2-year-old girl with partial seizures (bitemporal), underwent initial ablation of right temporal, then bitemporal tuber conglomerates in 2 months, left occipital tubers in 3 months, right occipital tubers in 5 months. A 17-year-old girl, with intractable epilepsy, motor and intellectual disability, underwent ablation of the most active left posterior parietal tubers. A 4-year-old girl, with refractory epilepsy (spasms, hemispasms, tonic seizures, staring episodes), autism spectrum disorder, had ablation of right frontal, left anterior medial and anterior superior insula, and left inferior posterior cingulate tubers. An 8-year-old boy with autism spectrum disorder, infantile spasms, partial seizures (left posterior temporal, occipital sleep potentiated spikes), underwent MRI-guided thermal laser ablation of the left posterior quadrant tuber conglomerate.Results: Three patients at latest follow-up are seizure free. The other 2 patients have >90% reduction. More importantly, all patients have improved cognitive, behavioral abilities and quality of life. The first patient is seizure free with improving autistic features 30 months after the first procedure. The second patient has occasional staring episodes with improving neurocognitive skills 30 months after the first ablation. The third patient has one complex partial episode every 3-4 months 24 months post procedure. The fourth patient gradually became seizure-free, with improving behavioral/neurocognitive difficulties 17 months post procedure. The fifth patient has been seizure free with immediate improvement in behavioral outbursts, with continuing improvement for 14 months.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 resulting in seizure freedom or at least dramatic seizure reduction and in all cases significant degree of reversible epileptic encephalopathy.