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      Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide

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          Abstract

          Lennox-Gaustaut syndrome is an electroclinical epilepsy syndrome characterized by the triad of electroencephalogram showing diffuse slow spike-and-wave discharges and paroxysmal fast activity, multiple intractable seizure types, and cognitive impairment. The intractability to seizure medications and cognitive impairment gives rise to eventual institutionalized patient care. Only a small subset of seizure medications has been shown to be helpful in seizure control. Most patients take up to 3 medications at high therapeutic dosing and are susceptible to medication-induced side effects. The lack of medication efficacy in seizure control has led one meta-analysis to conclude that there is no single medication that is highly efficacious in controlling seizures in this syndrome. On this background, a new and structurally novel seizure medication, rufinamide, has been found to be beneficial in the treatment of seizures in this syndrome. In a multicenter, double-blinded, randomized, placebo-controlled study, rufinamide was found to reduce seizures by over 30%. More importantly, it reduced the frequency of the seizure type that induces most of the morbidity of this syndrome, the drop seizure, by over 40%. There were few side effects, the medication was well tolerated, and in the open labeled extension study, tolerance was not found. In this review, we describe the main electroclinical features of Lennox-Gaustaut syndrome and summarize the few controlled studies that have contributed to its rational treatment. Currently, there is no single agent or combination of agents that effectively treat the multiple seizure types and co-morbidities in this syndrome. Our focus will be on the role of the new medication rufinamide in seizure reduction in patients with Lennox-Gaustaut syndrome.

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          Author and article information

          Journal
          Ther Clin Risk Manag
          Therapeutics and Clinical Risk Management
          Therapeutics and Clinical Risk Management
          Dove Medical Press
          1176-6336
          1178-203X
          2009
          2009
          4 May 2009
          : 5
          : 271-280
          Affiliations
          [1 ]Division of Pediatric Neurology, Seattle Children’s Hospital/University of Washington, Seattle, Washington, USA;
          [2 ]Department of Pharmacy, University of Washington, Seattle, Washington, USA
          Author notes
          Correspondence: Russell P Saneto, Associate Professor Neurology and Adjunct Associate Professor Pediatrics, Seattle Children’s Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA, Tel +1 206 987 2708, Fax +1 206 987 2649, Email russ.saneto@ 123456seattlechildrens.org
          Article
          tcrm-5-271
          2697537
          19536315
          © 2009 Saneto and Anderson, publisher and licensee Dove Medical Press Ltd.

          This is an Open Access article which permits unrestricted noncommercial use, provided the original work is properly cited.

          Categories
          Review

          Medicine

          lennox-gaustaut syndrome, rufinamide, catastrophic pediatric epilepsy

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