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Lennox-Gastaut Syndrome

Symptoms

Lennox-Gastaut syndrome is a severe type of epilepsy characterized by three factors:

  • multiple seizure types (myoclonic, tonic, atonic, atypical absence, etc.)
  • a distinctive brain-wave pattern 
  • mental deficiency that can range from slight to profound cognitive impairment

The peak age of onset is between the ages of 3 and 5 years. There is a slightly greater prevalence in boys than girls. It is estimated that between 20 and 60 percent of children with this syndrome have a prior history of infantile spasms. This syndrome is rare and occurs in about .3 in every 1,000 live births.

The first sign in about one-third of children with Lennox-Gastaut syndrome is a prolonged seizure. The child usually has multiple seizures throughout the day. Tonic seizures (muscle stiffening) are the most common and usually occur during sleep. They usually last about 10 seconds, but they can last up to one minute.

Diagnosis

Many of Lennox-Gastaut’s symptoms are common with other epileptic conditions, so a diagnosis of Lennox-Gastaut may be delayed until the child’s behavior is observed over a period of time. An EEG will show a slow spike and wave pattern that is specific to this syndrome. Brain abnormalities are common with this syndrome, so physicians will perform brain imaging tests. The physician will also check for signs of prior seizures, mental retardation, hearing and visual impairment, and tuberous sclerosis which are sometimes present in children with Lennox-Gastaut.

 

EEG of Lennox-Gastaut Syndrome

 

Treatment

Children with Lennox-Gastaut are typically treated with multiple epileptic drugs to control their seizures. Medications such Depakene® (valproate), Lamictal® (lamotrigine), Topamax® (topiramate), and Klonopin® (clonazepam), may be prescribed.

A vagus nerve stimulator, which is an implantable device that stimulates the vagus nerve within the left side of the neck, has shown to reduce the frequency and/or intensity of seizures of children with this type of epilepsy. Read more about vagus nerve stimulator implantation at Children’s.

Corpus callosotomy is also effective.

Drug information changes periodically. For the most updated information on drugs, visit www.drugs.com.

Last Update
July 2, 2012
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Last Update
July 2, 2012
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