Lennox-Gastaut syndrome is a form of severe childhood epilepsy, usually onsetting between five and six years of age. Children usually develop normally until the epilepsy appears, but begin to experience developmental setbacks as the seizures progress. Intellectual disabilities are common in children with Lennox-Gastaut syndrome and they may also develop behavioral problems and developmental delays. There are a number of treatment options available to control the seizures.
Children with Lennox-Gastaut syndrome can experience a variety of types of seizures. Tonic seizures, where the body stiffens, can occur, along with atonic or “drop” seizures, where muscle tone is lost. Myoclonic seizures with muscle jerks are very common. More rarely, patients can experience tonic-clonic seizure and absence seizures. Status epilepticus can also occur. The frequency and intensity of seizures may be highly variable in a patient with this condition.
One option for managing Lennox-Gastaut syndrome is medication. It may be necessary to try several medications at different dosages to find a combination that works for the patient. One concern is the possibility of having a seizure disorder resistant to medications, where the patient will continue experiencing severe seizures despite being on medication. Brain surgeries are also available; one option is an invasive procedure where the corpus callosum — the connection between the hemispheres of the brain — is severed to stop seizure activity. These measures are usually considered after more conservative means for controlling the seizures have failed.
Seizure activity associated with Lennox-Gastaut syndrome is usually observable and parents may also notice behavioral changes. Children experiencing seizures should be taken to a pediatric neurologist for evaluation. Brain scans and other diagnostic tools can be used to collect information about what is happening so a diagnosis can be developed. A treatment plan will require cooperation between parents, child, and physician, and this condition must be managed for life, making it important to get patients familiar with adhering to medication regimens.
Lennox-Gastaut syndrome can lead to disability and children may need educational interventions and other forms of assistance so they can acquire knowledge and skills and enjoy independence later in life. The degree of disability associated with this condition is variable, and it can be helpful to have a child evaluated to determine what kinds of interventions will be beneficial. Education professionals should be informed about how the disorder affects a child's ability to learn and provided with information on what to do if a child has a seizure in class.
