Ingo Borggraefe¹ and Soheyl Noachtar²

¹Department of Pediatric Neurology and Developmental Medicine, Children’s Hospital. ²Epilepsy Center, Department of Neurology, University of Munich, Germany.

Abstract

Lennox-Gastaut syndrome is an epileptic encephalopathy starting in early childhood consisting of the triad of cognitive impairment, multiple seizures types and slow spike-wave complexes in the electroencephalogram. Global developmental delay is usually evident before the onset in patients with symptomatic LGS whereas children with cryptogenic LGS might have a normal history of cognitive development before onset of the seizures. The seizure types most commonly associated with LGS are tonic seizures, myoclonic, dialeptic (absence) and astatic seizures. The seizures usually respond poorly to antiepileptic drugs. Cognitive deterioration occurs even in individuals who developed normally before onset of the encephalopathy. In this review, we will focus on the medical, dietary and surgical treatment of seizures associated with this syndrome. The treatment options for LGS have gained recently by emerging randomized controlled trials of new antiepileptic drugs and the introduction of vagal nerve stimulation.