Lennox-Gastaut syndrome (LGS) is a complex epilepsy of childhood characterised by an EEG demonstrating slow spike and wave complexes, multiple seizure types and cognitive impairment.
Control of seizures is difficult, and the effectiveness of established anticon-vulsants is often disappointing. In the past few years, many new medications have been introduced for the treatment of partial epilepsy in adults. Emerging evidence suggests that some of these agents are also useful in LGS.
Felbamate has been shown to be effective in a large controlled clinical trial, although its use is limited by life-threatening idiosyncratic reactions. Lamotrigine and topiramate have recently been shown in prospective controlled trials to be beneficial and well tolerated in patients with LGS. Vigabatrin appears to be particularly useful in children with underlying tuberous sclerosis. Novel neuroprotective strategies may be tested in the near future.
The ketogenic diet has empirical efficacy in some children with intractable seizures. Vagal nerve stimulation is currently being tested in LGS, and preliminary data are encouraging. Anterior callosotomy may provide an improved quality of life in children with frequent ‘drop attacks’.
These pharmacological and nonpharmacological approaches to the treatment of LGS offer new hope to patients and their families.
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