Acquired, Lesional Lennox-Gastaut Syndrome

This chapter presents a patient with pharmacoresistant acquired Lennox-Gastaut s yndrome due to extensive bihemispheric polymicrogyria. The patient was treated with palliative neuromodulation via two different neurostimulation devices. We describe the surgical procedure for implanting a deep brain stimulation device targeting bilateral centromedian thalamic nuclei. We describe the intra-operative neurophysiological recording to confirm electrode placement within the centromedian nucleus. We highlight the evidence supporting the targeting of the centromedian nucleus in pharmacoresistant generalized epilepsy. Additionally, we review polymicrogyria-associated epilepsies, the epileptic networks involved, and the available surgical interventions in this cohort. We highlight the rare entity of hypertrophied corpus callosum and its possible association with cortical malformations and language lateralization. Furthermore, we shed light on the importance of language lateralization and handedness in predicting the risk of post-operative language deficits following corpus callosotomy. Finally, we examine semiologies such as generalized atonic seizures and auditory and visual phenomena, including palinopsia.