EEG-Findings during long-term treatment with everolimus in TSC-associated and therapy-resistant epilepsies in children.

This prospective observational study evaluated the long-term EEG changes in children treated with everolimus (EVO) for refractory TSC-associated epilepsy. Changes in EEG-abnormalities were related to developmental outcomes. Thirteen children treated with EVO were examined for EEG-recorded seizures and interictal epileptic discharges (IED) during a 72-hour-video-EEG-monitoring, which was performed at baseline and repeated at follow-up intervals of at least 9 months. Antiseizure medication was left unchanged for at least 27 months. Changes in cognitive developmental parameters were related to reduction of seizures and IED at the last monitoring. We found a significant reduction of recorded seizures and IED during sleep at the first as well as the last follow-up recording. The reduction of IED was especially prominent during sleep. For patients who continued for more than one monitoring under EVO (n = 8), number of seizures further decreased. In patients with developmental examination (n = 9), we observed that only (nearly) full cessation of IED was related to acquisition of new skills. In children with TSC, EVO was effective in reducing recorded seizures and IED; long-term EVO treatment led to a more pronounced reduction and an improvement of nocturnal IED even when the patient was initially not seizure-free. Cessation of IED in children with developmental improvement may point to the importance of healthy sleep for cognition.

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Copyright © 2022. Published by Elsevier Ltd.

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Declaration of Competing Interests The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: GW obtained honoraria for speaking engagements from Desitin (Hamburg, Germany) and Novartis (Nürnberg, Germany). He gave scientific advice for EISAI (Frankfurt am Main), PTC Therapeutics (Frankfurt, Germany), Biogen (München, Germany). NJ has received obtained honoraria for speaking engagements from Desitin (Hamburg, Germany). KG has received obtained honoraria for speaking engagements from Desitin (Hamburg, Germany). US has received honoraria for lectures and scientific advice from Zogenix. NK has received honoraria for a speaking engagement by Novartis (Nürnberg, Germany).