Editor’s note: This section was sponsored by the generosity of Eisai. However, in order to avoid any possible sponsorship bias, the sponsor was not known to either the author or the editor.

The Lennox-Gastaut syndrome designates a type of epilepsy with multiple different types of seizures, particularly including tonic (stiffening) and atonic (drop) types of seizures. Intellectual development is usually, but not always, impaired. The EEG shows characteristic patterns of background slowing and spike-wave bursts at frequencies less than 2.5 per second. There a many underlying causes of this condition, but in about a quarter of children, no cause can be identified. Treatment is difficult, because the seizures tend to be resistant to anti-epileptic drugs, and the intellectual changes do not respond to any currently available medicine. Partial relief of seizures, and also falls and injuries from seizures, may be obtained by valproic acid, lamotrigine, topiramate, felbamate, clonazepam, rufinamide and occasionally other medications. Stimulation of the vagus nerve in the neck, with an implanted pacemaker sometimes improves seizures in people with LGS. An operation to separate the two halves of the brain, called corpus callosum surgery, may reduce seizures and injuries, but obviously is a big undertaking. LGS continues to present great challenges to children with the syndrome, their families and the doctors who care for them. Much more research is needed to identify better therapies.

Lennox-Gastaut Syndrome (LGS) accounts for only 2 to 5% of childhood epilepsies, yet children with this epilepsy syndrome often are well known to their pediatric neurologist. This disparity is due to the tendency for seizures to be hard to control, the requirement for long-term treatment (often with medical and non-medical therapies), and the intellectual impairment, all of which cause children with LGS to make frequent visits to their physicians. Unfortunately, this epilepsy syndrome usually persists through childhood and adolescence to adult years (changing some in its presentation with age) requiring familiarity by all health care professionals. In the last few years, several new treatments have emerged. While none are a cure for LGS, this is good news for a group of children that historically has had few good treatment options.

Author: James Wheless, M.D.
Topic Editor: Robert Fisher, M.D., Ph.D.
Last Reviewed: 8/7/09

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