Treatment of Lennox-Gastaut syndrome

Child and Familiy Health Services, Goldsworth Park Health Centre, Denton Way, Woking, UK, GU21 3LQ.
Cochrane database of systematic reviews (Online) (Impact Factor: 6.03). 02/2009; 2(3):CD003277. DOI: 10.1002/14651858.CD003277.pub2
Source: PubMed


The optimum treatment for Lennox-Gastaut syndrome has yet to be established. Lennox-Gastaut syndrome is a seizure (epilepsy) disorder that is commonly associated with behavioural and mental health problems. Many different treatments are currently used in the treatment of this disorder and many more have been tried in the past, often with little success. The review of trials found that there was no evidence to suggest that any one drug was more effective than another in the treatment of this disorder in terms of controlling the different seizure types. More research is needed to compare the therapies currently available.

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    • "This syndrome is characterized by various types of epileptic seizures (mainly tonic seizures), diffuse slow spike-andwave complex patterns on electroencephalogram (EEG), and impairment of cognitive function (Arzimanoglou et al., 2009; Beaumanoir, 1985). The long-term mental and seizure prognoses are generally devastating (Arzimanoglou et al., 2009; Beaumanoir, 1985; Blatter-Arifi, 1991; Oguni et al., 1996; Ohtsuka et al., 1990; Yagi, 1996), and appropriate and early intervention is crucial for the treatment of patients with LGS to prevent mental deterioration. However, therapeutic evidence for antiepileptic drugs (AEDs) in LGS treatment is not sufficiently established due to its complex symptoms and low prevalence rate (less than 1.0 per 10,000 population) (Cowan et al., 1989; Eriksson and Koivikko, 1997; Oka et al., 2006; Olafsson and Hauser, 1999), though several randomized controlled clinical trials have been conducted with some AEDs: felbamate (The Felbamate Study Group in Lennox—Gastaut Syndrome, 1993), lamotrigine (Motte et al., 1997), topiramate (Sachdeo et al., 1999), and clobazam (Ng et al., 2011). "
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    ABSTRACT: Purpose To evaluate the efficacy, safety, and pharmacokinetics of rufinamide as an adjunctive therapy for patients with Lennox–Gastaut syndrome (LGS) in a randomized, double-blind, placebo-controlled trial. Methods We conducted a multicenter clinical trial with a 4-week baseline, a 2-week titration, a 10-week maintenance, and either a follow-up visit or entry into an open-label extension. Patients with LGS (4 to 30 years old) taking between one and three antiepileptic drugs were recruited. After the baseline period, patients were randomly assigned to rufinamide or placebo. The primary efficacy variable was the percent change in the tonic-atonic seizure frequency per 28 days. Key findings: Of the 59 patients, 29 were randomized to the rufinamide group and 30 to the placebo group. The frequency of epileptic seizures was significantly decreased in the rufinamide group than in the placebo group; the median percent change in frequency of tonic-atonic seizures was -24.2% and -3.3%, respectively, (p = 0.003) and that of total seizures was -32.9% and -3.1%, respectively (p <0.001). Subgroup analyses indicated that the efficacy of rufinamide was consistent independent of clinical background characteristics. The common treatment-related adverse events in the rufinamide group were decreased appetite (17.2%), somnolence (17.2%), and vomiting (13.8%). Transient seizure aggravations were observed in 13 (22.0%) of the 59 patients, though a causal relationship with rufinamide was suspected in only one patient. All adverse events were mild to moderate in severity. The mean plasma concentration of rufinamide within 12 hours after administration was 17.2 μg/mL. Significance: The present results showed a favorable risk-benefit profile for rufinamide as an adjunctive therapy for patients with LGS.
    Epilepsy Research 09/2014; 108(9). DOI:10.1016/j.eplepsyres.2014.08.019 · 2.02 Impact Factor
  • Journal of Pediatric Neurosciences 01/2010; 5(1):86-8. DOI:10.4103/1817-1745.66666
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    ABSTRACT: To review the pharmacology, pharmacokinetics, efficacy, and safety of rufinamide in the treatment of epileptic seizures and describe its potential place in therapy. MEDLINE (1966-January 2010) and PubMed (1966-January 2010) literature searches were conducted to identify primary literature investigating rufinamide. References from selected publications discussing rufinamide, as well as the package insert, were reviewed. Published controlled trials describing the efficacy and safety of rufinamide were given preference. Available abstracts were also reviewed. Four published trials were identified and included and a retrospective review of the clinical use of rufinamide was also analyzed. The remainder of the information described is from 4 abstracts. Rufinamide is a new antiepileptic agent that differs structurally from other antiepileptic drugs and is approved as adjunctive therapy for Lennox-Gastaut syndrome (LGS). It presumably provides its antiseizure activity by prolonging sodium channel inactivity, stabilizing cell membranes. It is absorbed and metabolized extensively, then excreted renally as an inactive metabolite. Clinical trials show that adjunctive rufinamide is effective at reducing seizure frequency in patients with LGS and refractory partial seizures. Rufinamide showed no effect on cognitive function in patients with refractory partial seizures. Short-term adverse event rates were similar to those of placebo. Safety data from long-term studies show that rufinamide is well tolerated, causing headache, dizziness, and fatigue at rates of >10%. Rufinamide has few clinically relevant drug interactions, although it does increase phenytoin serum concentrations, while valproate increases rufinamide serum concentrations. Data show that rufinamide is safe and effective as an adjunctive agent for LGS and may be used to treat partial seizures. The benefits of rufinamide include its pharmacokinetics, limited drug interactions, and lack of effect on cognitive function, but its use is limited by the rarity of LGS and the lack of comparative data with other antiepileptic agents.
    Annals of Pharmacotherapy 03/2010; 44(4):658-67. DOI:10.1345/aph.1M679 · 2.06 Impact Factor
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