Lennox-Gastaut Syndrome

Severe form of epilepsy

A seizure is a brief abnormal burst of electrical activity in the brain. When a person has recurring seizures, it is called epilepsy, or seizure disorder. Lennox-Gastaut syndrome is a severe form of epilepsy that usually begins before 4 years of age. It occurs in about 0.3 in 1,000 live births and affects boys slightly more than girls.

Lennox-Gastaut syndrome may be caused by brain malformations, lack of oxygen during birth, severe head injury, nervous system infections, or inherited conditions. In 30% to 35% of affected children, no cause can be found.

Symptoms

The symptoms of Lennox-Gastaut syndrome include:

• Multiple seizure types - a child may have different types of seizures, and there may be periods of frequent seizures and brief, seizure-free periods. Seizures are usually repeated multiple times throughout the day and during sleep at night.
• Cognitive impairment - slightly to profoundly impaired intellectual functioning, developmental delays, and behavioral changes. By age 6, most children will have some degree of mental retardation.

Children with Lennox-Gastaut syndrome may fall due to either muscle stiffening or loss of muscle tone during seizures.

Diagnosis

Diagnosis of Lennox-Gastaut syndrome may be difficult because other seizure disorders may cause similar symptoms. Tests may be done to exclude other conditions. Looking at the brain structures with magnetic resonance imaging (MRI) will help identify any brain abnormalities.

A test called an electroencephalogram (EEG) is used to help diagnose seizures. An important sign of Lennox-Gastaut syndrome is a specific type of brain-wave pattern that is seen during an EEG.

Treatment

Treatment of Lennox-Gastaut syndrome focuses on controlling the seizures. However, this is difficult in this syndrome because of the different types of seizures that occur and because there may be periods of frequent seizures. Treatment may include medications such as Depakote (valproic acid), Lamictal (lamotrigine), Felbatol (felbamate), or Topamax (topiramate). As children with Lennox-Gastaut syndrome grow older, the types of seizures they have may change. So they may need to change medications or try new combinations of medications to control their seizures.

Children with Lennox-Gastaut syndrome may have behavioral problems such as hyperactivity or autistic behaviors. Special education programs can help each child reach his developmental potential.

_Sources:

"Lennox-Gastaut Syndrome." Epilepsy Syndrome. Epilepsy Foundation. 5 Dec 2008.

"NINDS Lennox-Gastaut Syndrome Information Page." Disorders. 13 Aug 2008. National Institute of Neurological Disorders and Stroke. 5 Dec 2008.