Rufinamide is an orally active, structurally novel compound (1-[(2,6-difluorophenil1) methyl1]-1 hydro 1,2,3-triazole-4 carboxamide), which is structurally distinct from other anticonvulsant drugs. It was granted orphan drug status for the adjunctive treatment of Lennox-Gastaut syndrome (LGS) in the United States in 2004, and released for use in Europe in 2007. In January 2009, rufinamide was a...

We report a case with Lennox-Gastaut syndrome (LGS) who underwent staged total callosotomy with a favorable outcome. A 6-year-old boy began having myoclonic seizures at the age of 8 months and was diagnosed with LGS when he was 27 months old. Various antiepileptic drugs and a ketogenic diet failed to control his multiple types of seizures. At the age of 36 months, he underwent corpus callosotom...

The variable presentation and progression of Lennox-Gastaut syndrome (LGS) can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed b...

BACKGROUND AND PURPOSE Rufinamide (RUF) is a novel antiepileptic drug (AED) and its efficacy has been proven in Lennox-Gastaut syndrome (LGS). However, there is a lack of data regarding the efficacy in pediatric intractable epilepsies other than LGS. The purpose of the study was to explore the efficacy and tolerability of RUF in pediatric patients with intractable epilepsies as well as LGS. M...

Purpose: This study presents the characteristics of patients with mild malformation cortical development oligodendroglial hyperplasia in epilepsy (MOGHE) SLC35A2 somatic variants brain who underwent surgery and showed clinical improvement seizures. Methods: We collected 10 mutations to treat drug-resistant at Severance Children’s Hospital from 2014 2019 retrospectively reviewed their genetic pr...

Submit Manuscript | http://medcraveonline.com Abbreviations: CAPS-2: Calcium dependent Activator Protein for Secretion 2; BDNF: Brain Derived Neurotrophic Factor; CXCR4: Chemokine Receptor with CXC Motif 4; GABA: Gamma Amino-Butyric Acid; GABA A receptor: Gamma Amino-Butyric Acid Receptor A; GAERS: Genetic Absence Epilepsy Rats from Strasbourg; LGS: Lennox-Gastaut Syndrome; MAP-2: Microtubule A...

Purpose: Lamotrigine (LTG) is often used as adjunctive therapy in Lennox-Gastaut syndrome (LGS); however, it may worsen myoclonic and atypical absence seizures LGS patients. This study reviewed the overall efficacy safety of LTG children with LGS.Methods: retrospective included 38 patients (aged <18 years) who underwent between October 2020 March 2022 at Severance Children’s Hospital. The pr...

PURPOSE Vagus nerve stimulation (VNS) is approved for use for refractory partial seizures. Nevertheless, information regarding VNS therapy for special populations, including Lennox-Gastaut syndrome (LGS) is limited. We discuss the effectiveness, tolerability, and safety of VNS therapy in patients with LGS. METHODS A six-center, retrospective study evaluated the effectiveness of VNS therapy in...

Background: Lennox-Gastaut syndrome (LGS) is a severe form of childhood-onset epilepsy associated with serious injuries due to frequent and severe seizures. Of the antiepileptic drugs (AEDs) approved for LGS, clobazam is a more recent market entrant, having been approved in October 2011. Recent AED budget impact and cost-effectiveness analyses for LGS suggest that adding clobazam to a health pl...

We have examined brainstem lesions in patients with refractory epilepsy disorders, including West syndrome (WS), Lennox-Gastaut syndrome (LGS), and dentatorubral-pallidoluysian atrophy (DRPLA). Acetylcholinergic neurons (AchNs) in the pedunculopontine tegmental nucleus (PPN) are involved in mental development, and disruption of neuronal nicotinic acetylcholine receptors can lead to epilepsy. In...