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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 8-year-old boy with Lennox-Gaustaut syndrome demonstrating paroxysmal fast activity during sleep. There were no observable clinical changes noted during the discharge.
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f2-tcrm-5-271: This is an EEG of a 8-year-old boy with Lennox-Gaustaut syndrome demonstrating paroxysmal fast activity during sleep. There were no observable clinical changes noted during the discharge.

Mentions: Paroxysmal fast activity (Figure 2) is the second characteristic EEG pattern of Lennox-Gaustaut syndrome and occurs predominantly or sometimes exclusively during nonrapid eye movement sleep.15 Discharge frequency is usually between 10 and 25 Hertz, preceded or followed by generalized sharp and slow wave complexes, and has a duration of 1 to 9 seconds. The spike paroxysms show little change in frequency throughout the burst. The discharges are widespread in distribution and bilaterally synchronous over both hemispheres. Shifting asymmetries are common. When seen during the waking state, paroxysmal fast activity is usually ictal and associated with tonic seizures. However, in its most common finding during nonrapid eye movement sleep, the EEG pattern is usually not associated with obvious clinical signs. When not associated with clinical signs, this pattern may sometimes be subclinical seizures.


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 8-year-old boy with Lennox-Gaustaut syndrome demonstrating paroxysmal fast activity during sleep. There were no observable clinical changes noted during the discharge.
© Copyright Policy
Related In: Results  -  Collection

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

f2-tcrm-5-271: This is an EEG of a 8-year-old boy with Lennox-Gaustaut syndrome demonstrating paroxysmal fast activity during sleep. There were no observable clinical changes noted during the discharge.
Mentions: Paroxysmal fast activity (Figure 2) is the second characteristic EEG pattern of Lennox-Gaustaut syndrome and occurs predominantly or sometimes exclusively during nonrapid eye movement sleep.15 Discharge frequency is usually between 10 and 25 Hertz, preceded or followed by generalized sharp and slow wave complexes, and has a duration of 1 to 9 seconds. The spike paroxysms show little change in frequency throughout the burst. The discharges are widespread in distribution and bilaterally synchronous over both hemispheres. Shifting asymmetries are common. When seen during the waking state, paroxysmal fast activity is usually ictal and associated with tonic seizures. However, in its most common finding during nonrapid eye movement sleep, the EEG pattern is usually not associated with obvious clinical signs. When not associated with clinical signs, this pattern may sometimes be subclinical seizures.

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