Immune treatments Since the success of steroids in epileptic encephalopathy (Klein and Livingston 1950) and West syndrome (Sorel and Dusaucy-Bauloye 1958), these drugs have been systematically tried in refractory epilepsies, including LGS. Nevertheless, there have been few reports of steroid use in childhood epilepsy after the ﬁrst year of life. Some beneﬁts have been previously reported with ACTH (Yamatogi et al 1979) and corticosteroids (Roger et al 1989) in patients with LGS. Prednisone was added to regular antiepileptic medications for the treatment of 28 children (aged 18 months to 10 years) with intractable epilepsy, including 10 with LGS. Seven of these patients became seizure free (Sinclair 2003). Other small series have been published in patients with intractable epilepsy, with different protocols, products and epilepsy syndromes, with various but often favorable results (Verhelst et al 2005). Side effects and lack of objective long-term effects have limited the use of steroids. Furthermore, new AEDs have become available since these older publications. However, Schmidt and Bourgeois (2000) consider the use of corticotrophin or corticosteroids as a fourth-line treatment in acute or subacute deterioration of LGS (You et al 2008b). Intravenous immunoglobulins (IVIG) The immune system has been sometimes called the “circulating brain” because of the numerous, reciprocal and continuous interactions between the immune and neurological systems (Billiau et al 2005). Consequently, the concept that the immune system may play an active role in epileptogenesis is more than 25 years old (Aarli and Fontana 1980) and has been developed in experimental animal models of epilepsy. Pechadre et al in 1977 noted that epileptic children treated with intramuscular immunoglobulins for recurrent infections had fewer seizures. Since this observation, Ariizumi et al (1983) reported a dramatic improvement in infants with West syndrome treated with IVIG while numerous studies have reported on the existence of a variety of immunological alterations, such as hypoIgA, in epileptic patients as well as the association of some well-known immune-mediated disease states, eg, lupus erythematous disease, with epilepsy (Duse et al 1996; van Rijckevorsel 1999; Aarli 2000). The link seems substantial in some epileptic syndromes, eg, Rasmussen disease, with the presence of autoantibodies and a favorable evolution after immunomodulatory treatments. However, the mechanisms of action of IVIG remain hypothetical (such as antiviral effect, substitutive therapy in case of concomitant immune deficiency, idiotype-anti-idiotype interaction, Neuropsychiatric Disease and Treatment 2008:4(6) Lennox-Gastaut treatment neuromodulant effect). The available data come mostly from open studies (van Rijckevorsel et al 1986; van Engelen et al 1994a; Billiau et al 2007), case reports (Fois et al 1990; Echenne et al 1991; Sterio et al 1992; Gross-Tsur et al 1993; Espinosa et al 2002), one single-blind (Ilum et al 1990) and one double-blind (van Rijckevorsel et al 1994). Doses, duration, and schedule of infusion, were different and results from the more than 370 treated children cannot be combined for a global analysis (van Engelen et al 1994b; Duse et al 1996; van Rijckevorsel 1999). However, the global review of published cases suggests that IVIG might be effective in some patients with refractory epilepsy, including LGS (Orange et al 2006; Feasby et al 2007). Ketogenic and modiﬁed Atkins diet Descriptions of the beneﬁcial effects of fasting were already mentioned by Hippocrates and in the New Testament. Later, the concept of ‘diet’ evolved and it was clear that a high-fat diet can mimic ketosis secondary to fasting. The ketogenic diet was therefore “born” and ﬁrst developed in the 1920s (Swink et al 1997), when only a few AEDs were available. With the development of different AEDs this therapeutic option was used less frequently. This treatment may have serious long-term adverse effects and is difﬁcult to manage for prolonged periods (Hemingway et al 2001). More recently, there has been new interest in this diet for use after failure of drug therapy, above all in children with refractory seizures (Kinsman et al 1992; Freeman et al 1998; Freeman and Vining 1999; Lefevre and Aronson 2000; Henderson et al 2006). Alternative ketogenic diets compared to the ﬁrst description have been proposed in order to improve long-term compliance and to decrease adverse events, notably on growth (for a review see Hartman and Vining 2007). The ﬁrst published results may be difﬁcult to interpret because the description of clinical signs is limited and some patients with specific genetic disorders may be included, eg, GLUT-1 deﬁciency syndrome characterized by intractable epilepsy (atonic seizure, myoclonus, absences), slow SW on EEG, mental retardation (Leary et al 2003) and highly responsive to ketogenic diet (Wexlet et al 1997; Wang et al 2005). Nevertheless, Levy and Copper (2003) consider the diet as a possible option in the therapy of LGS. Treatment of status epilepticus (SE) Treatment of SE in LGS is the same as for other epileptic syndromes, at least for convulsive SE. However, BZDs 1011