{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T22:34:27Z","timestamp":1789598067753,"version":"build-2803163510"},"reference-count":38,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2016,2,13]],"date-time":"2016-02-13T00:00:00Z","timestamp":1455321600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"French League Against Epilepsy"},{"name":"Association Thermalliance"},{"name":"Rothschild Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2016,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To describe the epileptic phenotype of <jats:italic>Tsc1<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice pups in comparison with age\u2010related seizures in human tuberous sclerosis complex (<jats:styled-content style=\"fixed-case\">TSC<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p><jats:italic>Tsc1<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> and control mice underwent intracranial electroencephalography (<jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content>) recording at postnatal ages (P)8 to P33, with linear silicon probe implanted in the somatosensory cortex of one or both hemispheres for 8\u201324 h. Ictal events were classified visually by independent analyzers; distinct <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content> patterns were related to age and analyzed to quantify field potential characteristics and signal dynamics between hemispheres. We collected retrospectively 20 infants with prenatally diagnosed <jats:styled-content style=\"fixed-case\">TSC<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content> before seizure onset, and analyzed the electroclinical course of epilepsy, taking into account a first\u2010line treatment by vigabatrin.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Spontaneous seizures were disclosed in 55% of <jats:italic>Tsc1<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice at P9\u201318. Three ictal patterns were identified: from P9 to P12 \u201cspike clusters\u201d consisted of recurring large spikes without clinical correlate; \u201cspasm\u2010like\u201d discharges dominated from P13 to P16 consisting of high amplitude large field potential superimposed with or followed by fast activity repeated every 2\u201310 s for at least 20 s, accompanied by rhythmic limb contractions; from P14 to P18 a \u201ctonic\u2013clonic like\u201d pattern comprised rhythmic spikes of increasing amplitude with tonic\u2013clonic movements. Early onset \u201cspike clusters\u201d were mainly unilateral, whereas \u201cspasm\u2010like\u201d and \u201ctonic\u2013clonic like\u201d patterns were bilateral. Interhemispheric propagation was significantly faster for \u201ctonic\u2013clonic like\u201d than for \u201cspasm\u2010like\u201d events. In infants diagnosed prenatally with <jats:styled-content style=\"fixed-case\">TSC<\/jats:styled-content>, clusters of sharp waves or spikes preceded the first seizure, and vigabatrin prevented the development of seizures. Patients treated after seizure onset developed spasms or focal seizures that were pharmacoresistant in 66.7% of cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p><jats:italic>Tsc1<\/jats:italic><jats:sup><jats:italic>+\/\u2212<\/jats:italic><\/jats:sup> mice pups exhibit an age\u2010dependent seizure pattern sequence mimicking early human <jats:styled-content style=\"fixed-case\">TSC<\/jats:styled-content> epilepsy features. Spike clusters before seizure onset in <jats:styled-content style=\"fixed-case\">TSC<\/jats:styled-content> should be considered as a first stage of epilepsy reinforcing the concept of preventive antiepileptic therapy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.13325","type":"journal-article","created":{"date-parts":[[2016,2,13]],"date-time":"2016-02-13T14:39:20Z","timestamp":1455374360000},"page":"648-659","source":"Crossref","is-referenced-by-count":29,"title":["Epilepsy in young <i>Tsc1<\/i><sup><i>+\/\u2212<\/i><\/sup> mice exhibits age\u2010dependent expression that mimics that of human tuberous sclerosis complex"],"prefix":"10.1111","volume":"57","author":[{"given":"Svetlana","family":"Gataullina","sequence":"first","affiliation":[{"name":"INSERM U1129 \u201cInfantile Epilepsies and Brain Plasticity\u201d Paris France"},{"name":"Paris Descartes University PRES Sorbonne Paris Cit\u00e9 Paris France"},{"name":"CEA Gif sur Yvette France"},{"name":"Neurology Department Mignot Hospital CH Versailles Le Chesnay France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Lemaire","sequence":"additional","affiliation":[{"name":"Innovations and Industrial Development Activsoft Antony France"},{"name":"Adpuerivitam Antony France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrice","family":"Wendling","sequence":"additional","affiliation":[{"name":"INSERM U1099 Rennes France"},{"name":"LTSI Rennes 1 University Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Kaminska","sequence":"additional","affiliation":[{"name":"INSERM U1129 \u201cInfantile Epilepsies and Brain Plasticity\u201d Paris France"},{"name":"Paris Descartes University PRES Sorbonne Paris Cit\u00e9 Paris France"},{"name":"CEA Gif sur Yvette France"},{"name":"Clinical Neurophysiology Necker\u2010Enfants Malades Hospital APHP Paris France"},{"name":"Reference Center for Rare Epilepsies Necker\u2010Enfants 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