What is Lennox-Gastaut Syndrome?

Lennox-Gastaut Syndrome (LGS) accounts for only 2 to 5% of childhood epilepsies. Yet children with this type of epilepsy are well-known to both the pediatric and adult neurologist, because their seizures are hard to control and they will need life-long treatment. The intellectual and behavioral problems add to the complexity of this syndrome. There are often difficulties in managing life with LGS.

 

This epilepsy syndrome usually persists into the adult years. Changing some in its presentation with age, it requires 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.

 

Quick Facts:

  • The Lennox-Gastaut syndrome (LGS) is a type of epilepsy with multiple different types of seizures, particularly tonic (stiffening) and atonic (drop) seizures
  • Intellectual development is usually, but not always, impaired
  • The EEG (electroencephalogram) shows a classic pattern of background slowing and spike-wave bursts at frequencies less than 2.5 per second
  • The cause of the disorder is unknown in 1 out of 4 children

 

How is Lennox-Gastaut Syndrome Treated?

 

Treatment of LGS is difficult because seizures often do not respond to seizure medications, anti-epileptic drugs (AEDs). In some cases, an operation to separate the two hemispheres of the brain, called corpus callosum surgery, is preformed to reduce seizures and injuries. This surgery may be done as a partial callosotomy, which involves cutting the front two-thirds of fiber bundle connecting the two halves of the brain, in hopes for a reduction in seizure frequency, or as a complete callostomy in which the additional one-third of the fiber bundle is cut. Statistically, seizure frequency is reduced by an average of 70% to 80% after partial callosotomy and 80% to 90% after complete callosotomy.

 

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