History of Lennox-Gastaut syndrome

The clinical manifestations of Lennox-Gastaut syndrome (LGS) have been recognized and described for more than two centuries.7 Distinct from these clinical descriptions, in 1939 Gibbs and colleagues8,9 identified an electroencephalographic (EEG) pattern similar to, yet slower than, the classic 3-Hz spike and wave discharge (petit mal pattern) and proposed to call it a petit mal variant to distinguish it. In 1945, Dr. William Lennox assembled the clinical and the EEG manifestations into the first semblance of the electroclinical syndrome that we now call childhood epileptic encephalopathy with diffuse slow spike and waves or the Lennox-Gastaut syndrome.10 Lennox’s triad consisted of:7,10–12

• the slow spike and wave interictal EEG pattern
• mental retardation
• three types of seizures that were considered characteristic: myoclonic jerks, atypical absence, and drop attacks (also known as akinetic seizures or astatic seizures)

Initially, this constellation of symptoms was not given a formal syndrome name. Renewed interest began in 1964 when Doose13 and Sorel14 each described about 20 cases. Three international meetings in Marseille (1964–1968) led by Henri Gastaut examined the syndrome in depth, confirmed its worldwide existence, and bestowed the name Lennox-Gastaut, reinforcing the significant contributions of both the U.S. and French groups to the understanding of the syndrome.7

The importance of recognizing patients with LGS increased in the early 1990s when this population was identified as a target group to participate in trials of investigational antiepileptic drugs (AEDs). Patients with LGS were targeted because of their high daily seizure frequency, the lack of highly effective therapy, and the desire to examine safety and efficacy issues for investigational AEDs in pediatric populations before the general release of these agents.

Adapted from: Glauser TA and Morita DA. Lennox-Gastaut syndrome. In: Devinsky O and Westbrook LE, eds. Epilepsy and Developmental Disabilities. Boston: Butterworth-Heinemann; 2001;65–78.
With permission from Elsevier (www.elsevier.com).
Reviewed and revised May 2004 by Steven C. Schachter, MD, epilepsy.com Editorial Board.