What is Lennox-Gastaut syndrome?
Lennox-Gastaut syndrome is a severe form of epilepsy. Seizures begin in early childhood, usually before the age of 4 years.
Children, adolescents, and adults with Lennox-Gastaut syndrome have multiple types of seizures that vary among individuals. Common seizure types include:
- Tonic seizures (stiffening of the body, upward eye gaze, dilated pupils, and altered breathing patterns)
- Atypical absences (staring spells)
- Atonic seizures (brief loss of muscle tone, which could cause abrupt falls)
- Myoclonic seizures (sudden muscle jerks)
- Generalized tonic-clonic seizures (muscle stiffness and rhythmic jerking)
There may be periods of frequent seizures mixed with relatively seizure-free periods. Although not always present at the onset of seizures, most people living with Lennox-Gastaut syndrome experience some degree of impaired intellectual functioning or information processing, along with developmental delays and behavioral disturbances. A particular pattern of brain electric activity can be seen using electroencephalogram (EEG).
Lennox-Gastaut syndrome can be caused by a variety of conditions, including:
- Brain malformations
- Tuberous sclerosis
- Perinatal asphyxia
- Severe head injury
- Central nervous system infection
- Inherited genetic and inherited degenerative or metabolic conditions
No cause can be found in 30 to 35 percent of individuals and Lennox-Gastaut syndrome can be very difficult to treat. A combination of seizure medications and other treatments may be used to improve seizure control and other associated conditions.
The medication valproate is generally considered a first-line therapy for various seizure types. Other anticonvulsant medications may include clobazam, felbamate, lamotrigine, rufinamide, topiramate, and cannabidiol. The U.S. Food and Drug Administration (FDA) approved cannabidiol (Epidolex, derived from marijuana) for the treatment of seizures associated with Lennox-Gastaut syndrome in individuals ages 2 and older. The drug contains only small amount of the psychoactive element in marijuana and does not induce euphoria associated with the drug.
Other treatment options include:
- Dietary therapy with the ketogenic diet
- Vagus nerve stimulation
- Epilepsy surgery (typically a corpus callostomy, which involves severing the band of nerve fibers that connect the two halves of the brain to prevent seizures from spreading)
Medication may be combined with the other treatments to optimize seizure control. Children who improve initially may later show tolerance to a drug or have uncontrollable seizures.
The prognosis for individuals with Lennox-Gastaut syndrome varies. There is no cure for the disorder. Complete recovery, including freedom from seizures, is very rare.
How can I or my loved one help improve care for people with Lennox-Gastaut syndrome?
Consider participating in a clinical trial so clinicians and scientists can learn more about Lennox-Gastaut syndrome and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with Lennox-Gastaut syndrome at Clinicaltrials.gov.
Where can I find more information about Lennox-Gastaut syndrome?
More information is available from the following organizations: