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Both studies comprised a baseline period (2 weeks), 2\u2010week up\u2010titration period, 12\u2010week stable\u2010dose maintenance period, and down\u2010titration or entry into long\u2010term follow\u2010up study. Symptoms of myoclonus were assessed by Unified Myoclonus Rating Scale (<jats:styled-content style=\"fixed-case\">UMRS<\/jats:styled-content>). Primary efficacy end point was percent reduction from baseline in action myoclonus score (<jats:styled-content style=\"fixed-case\">UMRS<\/jats:styled-content> section 4) at last treatment visit. Safety assessments included treatment\u2010emergent adverse events (<jats:styled-content style=\"fixed-case\">TEAE<\/jats:styled-content>s).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>N01187: 50 patients randomized, 47 completed; N01236: 56 patients randomized, 54 completed. Median (min\u2013max) percent reduction from baseline in action myoclonus score is the following\u2014N01187: placebo 5.6 (\u221281.3 to 53.8), pooled <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> group (primary efficacy analysis) 21.4 (\u221250.0 to 73.6), <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> 50 mg\/day 26.3 (\u221235.8 to 69.2), <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> 150 mg\/day 16.9 (\u221250.0 to 73.6); N01236: placebo 17.5 (\u2212170 to 61.5), <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> 5 mg\/day \u22124.6 (\u2212430 to 81.8), <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> 150 mg\/day (primary efficacy analysis) 12.3 (\u221258.3 to 96.9). Estimated differences versus placebo were not statistically significant. <jats:styled-content style=\"fixed-case\">TEAE<\/jats:styled-content>s were reported by 72\u201375% placebo\u2010treated and 56\u201383% <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content>\u2010treated patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>Effect of <jats:styled-content style=\"fixed-case\">BRV<\/jats:styled-content> on action myoclonus was not statistically significant. However, action myoclonus score showed wide intrapatient variability and may not have been the optimal tool to measure severity of myoclonus in <jats:styled-content style=\"fixed-case\">EPM<\/jats:styled-content>1. Both studies had very high completion rates (95.3% overall), and a high percentage of patients (88.7% overall) entered long\u2010term follow\u2010up; both likely to be influenced by good tolerability. These studies demonstrate the feasibility of rigorous trials in progressive myoclonic epilepsy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.13275","type":"journal-article","created":{"date-parts":[[2015,12,15]],"date-time":"2015-12-15T11:33:28Z","timestamp":1450179208000},"page":"210-221","source":"Crossref","is-referenced-by-count":46,"title":["Brivaracetam in Unverricht\u2010Lundborg disease (<scp>EPM<\/scp>1): Results from two randomized, double\u2010blind, placebo\u2010controlled studies"],"prefix":"10.1111","volume":"57","author":[{"given":"Reetta","family":"K\u00e4lvi\u00e4inen","sequence":"first","affiliation":[{"name":"Kuopio Epilepsy Center Kuopio University Hospital and Institute of Clinical Medicine School of Medicine Faculty of Health Sciences University of Eastern Finland Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Genton","sequence":"additional","affiliation":[{"name":"Henri Gastaut\u2010Centre Saint Paul Hospital 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