Low long-term efficacy and tolerability of add-on rufinamide in patients with Dravet syndrome

被引:33
|
作者
Mueller, A. [1 ,2 ]
Boor, R. [3 ,4 ]
Coppola, G. [5 ]
Striano, P. [6 ]
Dahlin, M. [7 ]
von Stuelpnagel, C. [1 ,2 ]
Lotte, J. [1 ,2 ]
Staudt, M. [1 ,2 ]
Kluger, G. [1 ,2 ]
机构
[1] Epilepsy Ctr Children & Adolescents, Neuropediat Clin, Schon Klin Vogtareuth, Vogtareuth, Germany
[2] Epilepsy Ctr Children & Adolescents, Clin Neurorehabil, Schon Klin Vogtareuth, Vogtareuth, Germany
[3] Univ Hosp Schleswig Holstein, No German Epilepsy Ctr Raisdorf, Kiel, Germany
[4] Univ Hosp Schleswig Holstein, Dept Neuropediat, Kiel, Germany
[5] Univ Naples 2, Clin Child Neuropsychiat, Naples, Italy
[6] Univ Genoa, Muscular & Neurodegenerat Dis Unit, G Gaslini Inst, Genoa, Italy
[7] Karolinska Hosp, Astrid Lindgren Childrens Hosp, S-10401 Stockholm, Sweden
关键词
Rufinamide; Dravet syndrome; SCN1A mutation; Refractory seizures; SEVERE MYOCLONIC EPILEPSY; LENNOX-GASTAUT-SYNDROME; INFANCY; SEIZURES; TRIAL; ENCEPHALOPATHIES; ADULTS; SCN1A;
D O I
10.1016/j.yebeh.2011.04.057
中图分类号
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要
In this retrospective European multicenter study we evaluated the efficacy and tolerability of rufinamide in patients with Dravet syndrome and refractory seizures. Twenty patients were included: in 16 patients a SCN1A mutation was detected. The responder rate after 6 months was 20%, and after 34 months, 5%. The retention rate was 45% after 6 months and 5% after 34 months. Rufinamide treatment was stopped because of aggravation of seizures (30%), no effect (45%), and side effects (10%). The efficacy and long-term retention rate were low in our patients with Dravet syndrome and refractory seizures, far lower than in patients with Lennox-Gastaut syndrome: one-third of our patients experienced seizure aggravation. Therefore, rufinamide does not seem to be a suitable option for long-term treatment in patients with Dravet syndrome. (C) 2011 Elsevier Inc. All rights reserved.
引用
收藏
页码:282 / 284
页数:3
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