{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T22:06:30Z","timestamp":1793829990910,"version":"4.6.1"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,10,28]],"date-time":"2016-10-28T00:00:00Z","timestamp":1477612800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100012739","name":"Zogenix","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100012739","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2017,2]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Dravet syndrome (<jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>) is a severe, drug\u2010resistant epilepsy. Fenfluramine has been reported to have a long\u2010term clinically meaningful anticonvulsive effect in patients with <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This prospective, open\u2010label study assessed the safety and effectiveness of low\u2010dose fenfluramine in a new cohort of patients with <jats:styled-content style=\"fixed-case\">DS<\/jats:styled-content>. Following a 3\u2010month baseline period, fenfluramine was added to each patient's current antiepileptic drug regimen at a dose of 0.25\u20131.0 mg\/kg\/day (max. 20 mg\/day). The incidence of major motor seizures (tonic, clonic, tonic\u2013clonic, atonic and myoclonic seizures lasting &gt;30 s) in both the baseline and treatment periods was assessed via a seizure diary. Periodic echocardiographic examinations during the treatment period were used to assess cardiovascular safety.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nine patients (aged 1.2\u201329.8 years) enrolled in the study and were treated with fenfluramine for a median duration of 1.5 (range, 0.3\u20135.1) years. Median frequency of major motor seizures was 15.0\/month in the baseline period. All patients demonstrated a reduction in seizure frequency during the treatment period with a median reduction of 75% (range, 28\u2013100%). Seven patients (78%) experienced a \u226550% reduction in major motor seizure frequency. The most common adverse events were somnolence (<jats:italic>n<\/jats:italic> = 5) and anorexia (<jats:italic>n<\/jats:italic> = 4). No evidence of cardiac valvulopathy or pulmonary hypertension was observed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The effectiveness and safety of low\u2010dose fenfluramine as an add\u2010on therapy for DS in this new prospective cohort supports previous findings.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13195","type":"journal-article","created":{"date-parts":[[2016,10,28]],"date-time":"2016-10-28T06:42:57Z","timestamp":1477636977000},"page":"309-314","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":95,"title":["Low\u2010dose fenfluramine significantly reduces seizure frequency in Dravet syndrome: a prospective study of a new cohort of patients"],"prefix":"10.1111","volume":"24","author":[{"given":"A.","family":"Schoonjans","sequence":"first","affiliation":[{"name":"Department of Neurology \u2013 Paediatric Neurology Antwerp University Hospital University of Antwerp Antwerp"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B. 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