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

Saneto RP, Anderson GD - Ther Clin Risk Manag (2009)

Bottom Line: Most patients take up to 3 medications at high therapeutic dosing and are susceptible to medication-induced side effects.There were few side effects, the medication was well tolerated, and in the open labeled extension study, tolerance was not found.Our focus will be on the role of the new medication rufinamide in seizure reduction in patients with Lennox-Gaustaut syndrome.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurology, Seattle Children's Hospital/University of Washington, Seattle, Washington, USA;

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.

No MeSH data available.


Related in: MedlinePlus

This is an EEG of a 5-year-old girl with Lennox-Gaustaut syndrome showing generalized spike-and-wave discharges. The frequency of spike-and-wave activity is <2.5 Hertz.
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f1-tcrm-5-271: This is an EEG of a 5-year-old girl with Lennox-Gaustaut syndrome showing generalized spike-and-wave discharges. The frequency of spike-and-wave activity is <2.5 Hertz.

Mentions: The characteristic interictal EEG pattern of slow spike-and-wave complex (Figure 1) was initially described by Gibbs et al.7 The record is dominated by 1.5 to 2.5 Hertz spike/sharp-and-wave complex, which are diffuse and maximum bifrontally but sometimes can be confined to the anterior or posterior head regions. Although the slow spike-and-wave pattern is fairly symmetrical, a shifting asymmetry in different bursts is common.18 The duration of the complexes can vary, but they often appear in prolonged sequences as long as half of the routine EEG record without clinical change. For this reason, the slow spike-and-wave activity is usually considered an interictal pattern. The slow spike-and-wave complexes are sometimes not present on the EEG at the onset of seizures, but can appear over 1 year later.22 Non-rapid eye movement sleep augments slow spike-and-wave complex expression in about 95% of patients with longer duration of discharges.18


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

Saneto RP, Anderson GD - Ther Clin Risk Manag (2009)

This is an EEG of a 5-year-old girl with Lennox-Gaustaut syndrome showing generalized spike-and-wave discharges. The frequency of spike-and-wave activity is <2.5 Hertz.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2697537&req=5

f1-tcrm-5-271: This is an EEG of a 5-year-old girl with Lennox-Gaustaut syndrome showing generalized spike-and-wave discharges. The frequency of spike-and-wave activity is <2.5 Hertz.
Mentions: The characteristic interictal EEG pattern of slow spike-and-wave complex (Figure 1) was initially described by Gibbs et al.7 The record is dominated by 1.5 to 2.5 Hertz spike/sharp-and-wave complex, which are diffuse and maximum bifrontally but sometimes can be confined to the anterior or posterior head regions. Although the slow spike-and-wave pattern is fairly symmetrical, a shifting asymmetry in different bursts is common.18 The duration of the complexes can vary, but they often appear in prolonged sequences as long as half of the routine EEG record without clinical change. For this reason, the slow spike-and-wave activity is usually considered an interictal pattern. The slow spike-and-wave complexes are sometimes not present on the EEG at the onset of seizures, but can appear over 1 year later.22 Non-rapid eye movement sleep augments slow spike-and-wave complex expression in about 95% of patients with longer duration of discharges.18

Bottom Line: Most patients take up to 3 medications at high therapeutic dosing and are susceptible to medication-induced side effects.There were few side effects, the medication was well tolerated, and in the open labeled extension study, tolerance was not found.Our focus will be on the role of the new medication rufinamide in seizure reduction in patients with Lennox-Gaustaut syndrome.

View Article: PubMed Central - PubMed

Affiliation: Division of Pediatric Neurology, Seattle Children's Hospital/University of Washington, Seattle, Washington, USA;

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.

No MeSH data available.


Related in: MedlinePlus