The first time I heard Tommy's diagnosis, Lennox-Gastaut syndrome, the words had no meaning to me. I asked the doctor for information, and he said there wasn't much written for parents, so I went to a medical library and spent the afternoon with a few textbooks and a medical dictionary. Sometimes, I had to ask one of the students to explain the definitions. I was in tears when I left. It sounded totally hopeless; Tommy had no future. Ten years later, Tommy's seizures are under much better control. He loves school (special education classes), has many friends, is an incredibly important part of our family, and gives us all great pleasure. He can almost beat me at tennis!
Lennox-Gastaut syndrome is serious but uncommon. It is defined by three things:
The seizures usually begin between 1 and 6 years of age, but can begin later. The syndrome involves some combination of tonic, atonic, atypical absence, myoclonic, and tonic-clonic seizures that are usually resistant to medications.
Useful medications for controlling the seizures of patients with Lennox-Gastaut syndrome include valproate, carbamazepine, clobazam (not available in the US), lamotrigine, and topiramate. Felbamate is also an effective drug and can often improve behavior and quality of life, but it carries a risk of life-threatening blood or liver disorders and must be used carefully.
In children or adults with frequent, poorly controlled seizures, it is often wise to avoid high doses of antiepileptic drugs because they may intensify the behavioral, social, and intellectual problems, especially when two or more drugs are used together. It may be better to tolerate slightly more frequent seizures in order to have a more alert and attentive family member. In those patients whose seizures are not controlled with medication, there are other options. These include the vagus nerve stimulator, the ketogenic diet or corpus callosotomy.
Vagus nerve stimulation or corpus callosotomy can be helpful treatments for some patients. However, experts typically recommend vagus nerve stimulation before consideration of corpus callosotomy because of lower risks.
Most children with Lennox-Gastaut syndrome have intellectual impairment ranging from mild to severe. Behavioral problems are also common and probably relate to a combination of the brain dysfunction, seizures, and antiepileptic drugs.
The course of the seizures varies greatly. Some children will later have fairly good seizure control. Others will continue to have multiple types of poorly controlled seizures throughout life. The intellectual and behavioral development of children whose seizures come under fair to good control may be almost normal, but the development of those who have frequent seizures and are given high doses of more than one drug may be severely delayed. This syndrome usually persists into adulthood and affected persons often need to live in a residential (adult foster care) group home when their parents are no longer able to care for them.