EEG-Findings during long-term treatment with everolimus in TSC-associated and therapy-resistant epilepsies in children. (December 2022)

• Record Type: Journal Article
• Authors: Wiegand, Gert; Japaridze, Natia; Gröning, Kristina; Stephani, Ulrich; Kadish, Navah E.
• Is Part Of: Seizure. Volume 103(2022)
• Page Start: 101
• Page End: 107
• Publication Date: 2022-12
• Subjects: Tuberous sclerosis complex (TSC) -- Epilepsy -- Video-EEG-Monitoring -- Everolimus -- Long-term treatment -- Interictal epileptic discharges; ACTH adrenocorticotropic hormone -- AE adverse events -- ASM antiseizure medications -- BSID Bayley scales of infant development -- EVO Everolimus -- IED interictal epileptic discharge -- ILAE International League Against Epilepsy -- md median -- mTOR mammalian target of rapamycin complex -- NREM non-rapid eye movement -- SEGA subependymal giant cell astrocytoma -- TSC Tuberous sclerosis complex -- VABS Vineland scales for adaptive behavior -- VNS vagus nerve stimulator; Epilepsy -- Periodicals; Epilepsy -- Periodicals; Seizures -- Periodicals; Épilepsie -- Périodiques; Electronic journals; Electronic journals; 616.853
• DOI: 10.1016/j.seizure.2022.10.022
• Languages: English
• ISSNs: 1059-1311

Abstract

Highlights: In children with TSC, EVO was effective in reducing EEG abnormalities. Long-term treatment with EVO led to a further reduction of seizures and IED. The decrease in IED during sleep was particularly striking. The cessation of IED may be important for developmental progression. Abstract: Aim: : 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. Methods: : 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. Results: : 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. Discussion: : 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.