Lennox-Gastaut Syndrome

Childhood epilepsy is a source of much pain both for children and parents. Unfortunately, Lennox-Gastaut syndrome is a type of childhood epilepsy that is particularly difficult to manage and treat. The syndrome's short-term effects often include physical injury and mental stress, while its long-term effects can debilitate a child's education and future.

Causes

• Lennox-Gastaut syndrome usually begins manifesting itself when a child is between 3 and 5 years old. In almost half of the cases, no definitive cause can be found. However, doctors sometimes trace it to a genetic condition called tuberous sclerosis. Other causes include meningitis and head injury.

Frequency

• In terms of being just another type of epilepsy, Lennox-Gastaut syndrome is fairly uncommon, occurring in about one to five children out of every 100 with epilepsy. However, in the realm of intractable epilepsies, Lennox-Gastaut is the most common. An intractable epilepsy is one that is particularly difficult to identify the cause of and treat.

Symptoms

• Lennox-Gastaut syndrome involves a number of types of seizures. Atonic seizures, or "drop attacks," involve a child dropping to the ground while seizing. This can result in physical injury. With absence seizures, a child can appear unresponsive and blank anywhere from 10 seconds to 10 minutes. These seizures can occur continuously to form a non-convulsive status epilepticus in which the child is unresponsive and unable to move or speak for a long time. In addition, Lennox-Gastaut can involve tonic seizures in which the arms or legs stiffen. These and many other types, such as complex partial, tonic-clonic, myoclonic and partial motor seizures, can lead to the development of learning problems in the child that are difficult to overcome.

Diagnosis

• One of the most useful tools for identifying the presence of Lennox-Gastaut syndrome is an electroencephalogram, or EEG. The EEG is especially useful because it can detect features in the brain that indicate the presence of the syndrome even when the child is not undergoing a seizure. In addition, if the child's seizures are caused by a head injury, genetic disorder or other disease, a doctor could identify it without the EEG.

Treatment

• Lennox-Gastaut syndrome is notoriously difficult to treat. However, in some cases the drugs lamotrigine, clobazam, sodium valproate, topiramate or phenytoin can be effective, as can the use of corticosteroid drugs. A ketogenic diet has been found effective, and in some cases, surgery is a viable option. The most common surgeries for Lennox-Gastaut are the corpus callostomy and vagus nerve stimulation procedures.