Treatment of Lennox-Gastaut Syndrome (LGS)

Abstract Lennox-Gastaut Syndrome (LGS) is a severe form of epilepsy that usually starts in early to mid childhood and is characterized by multiple seizure types, abnormal electroencephalogram with slow spike-and-wave discharges and cognitive problems. Numerous approaches are currently used to treat...

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Bibliographic Details
Published in:European journal of paediatric neurology 2009-11, Vol.13 (6), p.493-504
Main Authors: Ferrie, Colin D, Patel, Amit
Format: Article
Language:English
Subjects:
Anticonvulsants - classification
Anticonvulsants - therapeutic use
Antiepileptic drug
Child
Cognition Disorders - complications
Cognition Disorders - drug therapy
Corpus Callosum - surgery
Diet Therapy - methods
Double-Blind Method
Epilepsy
Epilepsy - complications
Epilepsy - diagnosis
Epilepsy - epidemiology
Epilepsy - therapy
Epileptic encephalopathy
Humans
Management
Neurology
Pediatrics
Randomized Controlled Trials as Topic
Vagus Nerve Stimulation - methods
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Summary:Abstract Lennox-Gastaut Syndrome (LGS) is a severe form of epilepsy that usually starts in early to mid childhood and is characterized by multiple seizure types, abnormal electroencephalogram with slow spike-and-wave discharges and cognitive problems. Numerous approaches are currently used to treat LGS, including use of conventional antiepileptic drugs (most commonly sodium valproate, lamotrigine and topiramate), other drug interventions (corticosteroids and intravenous immunoglobulin) and nonpharmacologic treatments (ketogenic diet, corpus callosotomy and vagus nerve stimulation). Rufinamide is the most recent antiepileptic drug to have shown efficacy in the treatment of LGS. Despite the variety of therapeutic options, there have been only five double-blind, placebo-controlled clinical trials of antiepileptic drugs in LGS and none of these were head-to-head comparison trials. The evidence supporting the use of available treatments for LGS is, therefore, not robust. Here, we review the evidence supporting the use of specific therapies in LGS and provide recommendations on how to set appropriate treatment goals, select treatments and minimize polypharmacy. A suggested treatment algorithm is presented and discussed. Sodium valproate is recommended for first-line therapy; if seizures persist, alternative interventions should be trialed on an individually tailored basis.
ISSN:1090-3798
1532-2130
DOI:10.1016/j.ejpn.2008.12.005