Lennox-Gastaut Syndrome

Abstract

Lennox-Gastaut syndrome is considered an epileptic encephalopathy. The encephalopathy is the result of epileptic activity (epileptic encephalopathy) with or without developmental problems (developmental encephalopathy). This syndrome is defined by a triad of multiple drug-resistant seizure types, a specific EEG pattern, and intellectual disability. The prevalence of Lennox-Gastaut syndrome is estimated between 1% and 2% of all patients with epilepsy. The etiology of Lennox-Gastaut syndrome is often divided into two groups: identifiable (genetic-structural-metabolic) in 65–75% of the patients and Lennox-Gastaut syndrome of unknown cause in others. The seizures in Lennox-Gastaut syndrome are usually drug-resistant. Reduction in the frequency of the most incapacitating seizures should be the major objective. Valproate, lamotrigine and topiramate are considered to be the first-line drugs by many experts. Other effective drugs include levetiracetam, clobazam, rufinamide, and zonisamide. New antiepileptic medications such as fenfluramine HCl and sulthiame are gaining interest due to their effects on seizure reduction in patients with Lennox-Gastaut syndrome. The ketogenic diet is an effective treatment option. For patients with drug-resistance, a further therapeutic option is surgical intervention. Corpus callosotomy is a palliative surgical procedure that aims at controlling the most injurious seizures. Vagus nerve stimulation also offers reasonable seizure improvement. The long-term outcome for patients with Lennox-Gastaut syndrome is generally poor.