{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T10:25:29Z","timestamp":1777803929848,"version":"3.51.4"},"reference-count":19,"publisher":"SAGE Publications","issue":"9","license":[{"start":{"date-parts":[[2013,7,9]],"date-time":"2013-07-09T00:00:00Z","timestamp":1373328000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Child Neurol"],"published-print":{"date-parts":[[2013,9]]},"abstract":"<jats:p>We examined flurothyl gas\u2013induced seizure latencies and phenotype in 2 mouse models of neuronal ceroid lipofuscinoses: the nclf ( Cln6 mutant) variant late-infantile model and the mnd ( Cln8 mutant) Northern epilepsy model. Mnd mice on postnatal days 35 to 42 had increased latency to loss of posture compared with wild-type controls. Nclf, mnd, and wild-type mice on postnatal days 21 days to 25 displayed similar latency profiles during repeated seizure induction (kindling) and retesting; seizure phenotypes were different, however. Kindled wild-type mice reexposed to flurothyl after a 28-day recovery displayed brainstem generalized seizures exclusively. Neuronal ceroid lipofuscinoses mutants demonstrated a lack of brainstem seizures at retesting after 28 days. Repeated induction of generalized seizures delayed weight gain in both nclf and mnd mice compared with wild-type mice. These and our previous results suggest that abnormal seizure-related neuronal connectivity and\/or plasticity are shared characteristics of the neuronal ceroid lipofuscinoses.<\/jats:p>","DOI":"10.1177\/0883073813493667","type":"journal-article","created":{"date-parts":[[2013,7,9]],"date-time":"2013-07-09T22:06:50Z","timestamp":1373407610000},"page":"1137-1141","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Seizure Susceptibility, Phenotype, and Resultant Growth Delay in the\n                    <i>nclf<\/i>\n                    and\n                    <i>mnd<\/i>\n                    Mouse Models of Neuronal Ceroid Lipofuscinoses"],"prefix":"10.1177","volume":"28","author":[{"given":"Elizabeth","family":"Kriscenski-Perry","sequence":"first","affiliation":[{"name":"Center for Neural Development and Disease, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA"},{"name":"Rochester Institute of Technology, Rochester, NY, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Attila D.","family":"Kov\u00e1cs","sequence":"additional","affiliation":[{"name":"Center for Neural Development and Disease, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA"},{"name":"Department of Biochemistry and Biophysics, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA"},{"name":"Sanford Children's Health Research Center, Sanford Research\/University of South Dakota, Sioux Falls, SD, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Pearce","sequence":"additional","affiliation":[{"name":"Center for Neural Development and Disease, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA"},{"name":"Sanford Children's Health Research Center, Sanford Research\/University of South Dakota, Sioux Falls, SD, USA"},{"name":"Department of 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