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Seizures in TSC typically first present in infancy or early childhood, including focal seizures and infantile spasms. Infantile spasms in TSC are particularly characteristic in its strong responsiveness to vigabatrin. Although a number of mouse models of epilepsy in TSC have been described, there are very limited electroencephalographic (EEG) or seizure data during the preweanling neonatal and infantile\u2010equivalent mouse periods. <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice are a well\u2010characterized mouse model of epilepsy in TSC, but whether these mice have seizures during early development has not been documented. The objective of this study was to determine whether preweanling <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice have developmental EEG abnormalities or seizures, including spasms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Longitudinal video\u2010EEG and electromyographic recordings were performed serially on <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO and control mice from postnatal days 9\u201021 and analyzed for EEG background abnormalities, sleep\u2010wake vigilance states, and spontaneous seizures. Spasms were also induced with varying doses of N\u2010methyl\u2010D\u2010aspartate (NMDA).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The interictal EEG of <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice had excessive discontinuity and slowing, suggesting a delayed developmental progression compared with control mice. <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice also had increased vigilance state transitions and fragmentation. <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice had spontaneous focal seizures in the early neonatal period and a reduced threshold for NMDA\u2010induced spasms, but no spontaneous spasms were observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Neonatal <jats:italic>Tsc1<\/jats:italic><jats:sup>GFAP<\/jats:sup>CKO mice recapitulate early developmental aspects of EEG abnormalities, focal seizures, and an increased propensity for spasms. This mouse model may be useful for early mechanistic and therapeutic studies of epileptogenesis in TSC.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.16495","type":"journal-article","created":{"date-parts":[[2020,4,10]],"date-time":"2020-04-10T08:53:41Z","timestamp":1586508821000},"page":"879-891","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Early developmental electroencephalography abnormalities, neonatal seizures, and induced spasms in a mouse model of tuberous sclerosis complex"],"prefix":"10.1111","volume":"61","author":[{"given":"Nicholas","family":"Rensing","sequence":"first","affiliation":[{"name":"Department of Neurology and Hope Center for Neurological Disorders Washington University School of Medicine St Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin J.","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Neurology and Hope Center for Neurological Disorders Washington University School of Medicine St Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas J.","family":"Foutz","sequence":"additional","affiliation":[{"name":"Department of Neurology and Hope Center for Neurological Disorders Washington University School of Medicine St Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph L.","family":"Friedman","sequence":"additional","affiliation":[{"name":"Department of Neurology and Hope Center for Neurological Disorders Washington University School of Medicine St Louis Missouri"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rafael","family":"Galindo","sequence":"additional","affiliation":[{"name":"Department of Neurology and Hope Center for Neurological Disorders Washington University School of Medicine St Louis 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