Background: Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy, which starts in childhood with various seizure types. The children develop cognitive impairment and a typical EEG pattern. The aim of this article is to describe the clinical presentation of LGS, with particular emphasis on the course in adulthood.
Material and methods: The article is based on literature (up to November 2009) identified through a non-systematic search in PubMed and our own clinical experience.
Results: There are cases with unknown etiology and symptomatic cases with a wide spectrum of etiologies. While children with LGS have a high frequency of generalized seizures, seizure activity tends to decrease somewhat in adulthood and the seizures may become more focal. The prognosis is usually poor. The adult patient with LGS is clearly affected by global encephalopathy and is typically characterized by bluntness, apathy, progressive cognitive failure and motoric deficits. Valproate has been the first-line treatment for many years, but newer antiepileptic drugs; such as lamotrigine, topiramate and rufinamide, have shown efficacy as add-on therapy. Overtreatment with antiepileptic drugs is common.
Interpretation: For optimal treatment, a specialist should follow LGS patients at all ages. New treatment options with milder side effects may improve the quality of life for these patients.