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Seizures were evaluated with the Unified Batten Disease Rating Scale, a disease\u2010specific quantitative assessment tool.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighty\u2010six children (44 males, 42 females) with JNCL were assessed at an average of three annual visits (range 1\u201311). Eighty\u2010six percent (<jats:italic>n<\/jats:italic>=74) experienced at least one seizure, most commonly generalized tonic\u2013clonic, with mean age at onset of 9\u00a0years 7\u00a0months (SD 2y 10mo). Seizures were infrequent, typically occurring less often than once every 3\u00a0months, and were managed with one to two medications for most participants. Valproate (49%, <jats:italic>n<\/jats:italic>=36) and levetiracetam (41%, <jats:italic>n<\/jats:italic>=30) were the most commonly used seizure medications. Myoclonic seizures occurred infrequently (16%, <jats:italic>n<\/jats:italic>=14). Seizure severity did not vary by sex or genotype. Seizures showed mild worsening with increasing age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>The neuronal ceroid lipofuscinoses (NCLs) represent a group of disorders unified by neurodegeneration and symptoms of blindness, seizures, motor impairment, and dementia. While NCLs are considered in the differential diagnosis of progressive myoclonus epilepsy, we show that myoclonic seizures are infrequent in JNCL. This highlights the NCLs as consisting of genetically distinct disorders with differing natural history.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.12634","type":"journal-article","created":{"date-parts":[[2014,11,12]],"date-time":"2014-11-12T05:12:14Z","timestamp":1415769134000},"page":"366-371","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Standardized assessment of seizures in patients with juvenile neuronal ceroid lipofuscinosis"],"prefix":"10.1111","volume":"57","author":[{"given":"Erika F","family":"Augustine","sequence":"first","affiliation":[{"name":"Department of Neurology University of Rochester School of Medicine and Dentistry Rochester NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather R","family":"Adams","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Rochester School of Medicine and Dentistry Rochester NY 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